Ophthalmology colour atlas 9781609133382, 2011025133

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CO LO R ATLAS & SYNO PSIS OF CLINICAL OPHTHALMOLOGY

W i l l s Ey e I n s t i t u t e

Co rn e a S ECON D EDITION

EDITOR

Christopher J. Rapuano, MD Director and Attending Surgeon, Cornea Service Co-Director, Refractive Surgery Department Wills Eye Institute Professor of Ophthalmology Jefferson Medical College of Thomas Jefferson University Philadelphia, Pennsylvania

SERIES EDITOR

Christopher J. Rapuano, MD Director and Attending Surgeon, Cornea Service Co-Director, Refractive Surgery Department Wills Eye Institute Professor of Ophthalmology Jefferson Medical College of Thomas Jefferson University Philadelphia, Pennsylvania

CO LO R ATLAS & SYNO PSIS OF CLINICAL OPHTHALMOLOGY

W i l l s Ey e I n s t i t u t e

Co rn e a S ECON D EDITION

Senior Executive Editor: Jona han W. Pine, Jr. Senior Product Managers: Emilie Moyer and Grace Capu o Senior Manufacturing Coordinator: Benjamin Rivera Marketing Manager: Lisa Lawrence Creative Director: Doug Smock Production Services: Ap ara, Inc. © 2012 by LIPPINCOT WILLIAMS & WILKINS, a Wolters Kluwer business First edition, © 2003

e McGraw-Hill Companies, Inc.

wo Commerce Square 2001 Market Street Philadelphia, PA 19103 USA LWW.com All righ s reserved. T is book is pro ec ed by copyrigh . No par o his book may be reproduced in any orm by any means, including pho ocopying, or u ilized by any in orma ion s orage and re rieval sys em wi hou writ en permission rom he copyrigh owner, excep or brie quo a ions embodied in cri ical ar icles and reviews. Ma erials appearing in his book prepared by individuals as par o heir of cial du ies as U.S. governmen employees are no covered by he above-men ioned copyrigh . Prin ed in China Library of Congress Cataloging-in-Publication Data Rapuano, Chris opher J. Cornea / Chris opher J. Rapuano. – 2nd ed. p. ; cm. – (Color a las & synopsis o clinical oph halmology–Wills Eye Ins i u e) Includes bibliographical re erences and index. ISBN 978-1-60913-338-2 (alk. paper) 1. Cornea–Diseases–A lases. 2. Cornea–Diseases–Handbooks, manuals, e c. I. and synopsis o clinical oph halmology series. [DNLM: 1. Corneal Diseases–A lases. WW 17] RE336.R37 2011 617.7'19–dc23

i le. II. Series: Color a las

2011025133 Care has been aken o con rm he accuracy o he in orma ion presen ed and o describe generally accep ed prac ices. However, he au hors, edi ors, and publisher are no responsible or errors or omissions or or any consequences rom applica ion o he in orma ion in his book and make no warran y, expressed or implied, wi h respec o he currency, comple eness, or accuracy o he con en s o he publica ion. Applica ion o he in orma ion in a par icular si ua ion remains he pro essional responsibili y o he prac i ioner. T e au hors, edi ors, and publisher have exer ed every e or o ensure ha drug selec ion and dosage se or h in his ex are in accordance wi h curren recommenda ions and prac ice a he ime o publica ion. However, in view o ongoing research, changes in governmen regula ions, and he cons an ow o in orma ion rela ing o drug herapy and drug reac ions, he reader is urged o check he package inser or each drug or any change in indica ions and dosage and or added warnings and precau ions. T is is par icularly impor an when he recommended agen is a new or in requen ly employed drug. Some drugs and medical devices presen ed in he publica ion have Food and Drug Adminis ra ion (FDA) clearance or limi ed use in res ric ed research set ings. I is he responsibili y o he heal h care provider o ascer ain he FDA s a us o each drug or device planned or use in heir clinical prac ice. o purchase addi ional copies o his book, call our cus omer service depar men a (800) 638-3030 or ax orders o (301) 223-2320. In erna ional cus omers should call (301) 223-2300. Visi Lippincot Williams & Wilkins on he In erne : a LWW.com. Lippincot Williams & Wilkins cus omer service represen a ives are available rom 8:30 am o 6 pm, ES . 10 9 8 7 6 5 4 3 2 1

To my wonderful wife, Sara, an essential partner at home and at work; we continue to make a perfect team; our children— Michael, Patrick, Daniel, and Megan— for always reminding me what is important in life; my parents, Cathie and Al, for all their love and support over the years; and my three brothers, sisters-in-law, brothers-in-law, and their children, who demonstrate how essential family really is in our lives.

Abou he Series

T

he beau y o he a las/ synopsis concep is he power ul combina ion o illus ra ive pho ographs and a summary approach o he ex . Oph halmology is a very visual discipline ha lends i sel nicely o clinical pho ographs. While he seven oph halmic subspecial ies in his series—Cornea, Re ina, Glaucoma, Oculoplas ics, Neurooph halmology, Uvei is, and Pedia rics—employ varying levels o visual recogni ion, a rela ively s andard orma or he ex is used or all volumes.

vi

T e goal o he series is o provide an up- oda e clinical overview o he major areas o oph halmology or s uden s, residen s, and prac i ioners in all he heal hcare pro essions. T e abundance o large, excellen -quali y phoographs and concise, ou line- orm ex will help achieve ha objec ive. Chris opher J. Rapuano, MD Series Editor

Pre ace

T

he main objec ive o basic oph halmic educa ion is o rain he user o discover he ull his ory o he pa ien ’s illness, recognize he abnormal physical signs, make a diagnosis, and sugges appropria e me hods o rea men . T e aim o higher raining is o ampli y hese capabili ies in bread h and dep h hrough prac ical experience and subspecial y raining. During case presen a ions and even clinical examina ions, i is no uncommon o encoun er residen s making he wrong diagnosis and arriving a he wrong rea men plan. T ere are wo principal reasons or his error. Firs , hey may ail o de ec all he abnormal signs, and, second, hey are unable o in egra e and in erpre he ac s ha are collec ed. T e f rs s ep in he managemen o any condi ion is making a correc diagnosis. One mus be able o de ec all he abnormal signs and know wha one is observing. T e goal o his book is o use color pho ographs o he impor an corneal, an erior segmen , and ex ernal diseases wi h ou line- orm ex o succinc ly illus ra e and describe hese condi ions. T is a las is in ended no only or oph halmic residen s and cornea ellows bu also or prac icing physicians. Each sec ion covers he clinical ea ures o he impor an cornea and ex ernal eye diseases, diagnos ic

es s, di eren ial diagnoses, and rea men . In addi ion o providing prac ical in orma ion on he approach and managemen o each condi ion, his book aids recogni ion o clinical signs by including a selec ion o classic phoographs. In he f eld o cornea s udy, he old saying, “A pic ure is wor h a housand words,” is inadequa e because no even a housand words can subs i u e or a good pic ure o he condi ion. I is hoped ha he ex ensive use o color pho ographs hroughou his a ordable a las will have a grea impac on he memory and acili a e learning. o emphasize he impor ance o sign recogniion and he powers o observa ion, he ollowing quo a ions may serve as use ul reminders or all o us: Credi mus be given o observa ion ra her han heories, and o heories only in so ar as hey are conf rmed by he observed ac s.

Aris o le T e more I see, he more I see here is o see.

John Sebas ian Chris opher J. Rapuano, MD Editor

vii

Acknowledgmen s

I

would like o acknowledge he many people who helped make his book a reali y. Mos o he clinical pho ographs came rom my pa ien s. I am gra e ul o hem or allowing me o subjec hem o ash pho ography. Several colleagues generously supplied addi ional pho os, including Kris in Hammersmi h, MD, Elisabe h Cohen, MD, Pe er Laibson, MD, Irving Raber, MD, Wee-Jin Heng, MD, and also Rolande Michaud, MD, via Pa ricia Laughrea, MD, rom Quebec, Canada. I also hank Jack Scully and Roger Barone o he Audio-Visual Depar men a Wills Eye

viii

or all heir help and exper ise wi h he phoographic needs or his book and or all he volumes in his series. I wish o hank Jona han Pine, Emilie Moyer, Grace Capu o, and he eam a Lippincot Williams & Wilkins or giving me he opporuni y o be par o his series and or keeping he process moving orward. Finally, I hank all o our ellows and residen s, pas and presen , or all hey do o encourage me o con inue eaching and learning in our wonder ul subspecial y o cornea and an erior segmen disease.

Con en s Abou he Series vi Pre ace vii Acknowledgmen s viii Ch a pt er 1 Conjunctival In ections and Inf ammations 2 Blephari is and Meibomi is 2 Chalazion (In ernal Hordeolum, S ye) 4 Bac erial Conjunc ivi is (Nongonococcal) 6 Gonococcal Bac erial Conjunc ivi is 8 Viral Conjunc ivi is ( ypically Adenovirus) 10 Chlamydial Conjunc ivi is (Adul Inclusion Conjunc ivi is) rachoma 16 Molluscum Con agiosum 18 Ligneous Conjunc ivi is 20 Pediculosis 21 Parinaud’s Oculoglandular Syndrome 22 Oph halmia Neona orum 24 Allergic Conjunc ivi is 26 A opic Kera oconjunc ivi is 28 Vernal Kera oconjunc ivi is 30 Superior Limbic Kera oconjunc ivi is 33 Floppy Eyelid Syndrome 35 oxic and Fac i ious Kera oconjunc ivi is (Kera i is Medicamen osa) 37 Ocular Rosacea 40

14

Ch a pt er 2 Conjunctival Degenerations and Mass Lesions 42 Pinguecula and P erygium 42 O her Conjunc ival Degenera ions 46 Amyloidosis 46 Calcium Concre ions 46 Melanocy ic Conjunc ival Lesions 48 Conjunc ival Epi helial Melanosis (Racial Melanosis) Oculodermal Melanosis (Nevus o O a) 48 Nevus 48 Primary Acquired Melanosis 48 Secondary Acquired Melanosis 49 Malignan Melanoma 49

48

ix

x

CO NT ENTS

Benign Amelanocy ic Conjunc ival Lesions 52 Granulomas 52 Epibulbar Dermoid 52 Lipodermoid 52 Heredi ary Benign In raepi helial Dyskera osis 52 Po en ially Malignan Amelanocy ic Conjunc ival Lesions 58 Squamous Papilloma 58 Conjunc ival In raepi helial Neoplasia 58 Squamous Cell Carcinoma 58 O her Carcinomas 58 Reac ive Lymphoid Hyperplasia and Non-Hodgkin’s Lymphoma 59 Cys ic Lesions 64 Primary Conjunc ival Cys 64 Ia rogenic Cys s 64 Vascular Lesions 67 elangiec asias 67 Hema ologic Disorders 67 Hemorrhagic Lymphangiec asia 67 Capillary Hemangioma 67 Lymphangioma 67 Kaposi’s Sarcoma 67 S urge-Weber Syndrome (Encephalo rigeminal Angioma osis) 67 Caro id–Cavernous Sinus and Dural-Sinus Fis ulas 67

Ch a pt er 3 Anterior Segment Developmental Anomalies 70 Anomalies o Corneal Size and Shape 70 Microcornea 70 Megalocornea 72 Nanoph halmos 74 Microph halmos 74 Buph halmos 76 Congeni al An erior S aphyloma/ Kera ec asia 78 Sclerocornea 79 Cornea Plana 81 An erior Segmen Dysgeneses 82 Pos erior Embryo oxon 82 Axen eld’s Anomaly 82 Rieger’s Anomaly 82 Rieger’s Syndrome 82 Pe ers’ Anomaly 82 Localized Pos erior Kera oconus 82 Aniridia 86 Iris Coloboma 88

CO NTENTS

xi

Ch a pt er 4 Ectatic Conditions of the Cornea 90 Kera oconus 90 Pellucid Marginal Degenera ion Kera oglobus 100

98

Ch a pt er 5 Corneal Dystrophies 102 An erior Corneal Dys rophies 102 Epi helial Basemen Membrane Dys rophy (An erior Basemen Membrane Dys rophy, Map-Do -Fingerprin Dys rophy, Cogan’s Microcys ic Dys rophy) 102 Meesmann’s Corneal Dys rophy ( Juvenile Heredi ary Epi helial Dys rophy) Reis-Bücklers Dys rophy 110 Gela inous Drop–Like Corneal Dys rophy 114 S romal Corneal Dys rophies 116 Granular Dys rophy 116 Lat ice Dys rophy 120 Macular Dys rophy 123 Avellino Corneal Dys rophy (Granular-Lat ice) 126 Schnyder’s Corneal Dys rophy 128 Pos erior Corneal Dys rophies 131 Endo helial Dys rophy and Fuchs’ Dys rophy 131 Pos erior Polymorphous Corneal Dys rophy 136 Congeni al Heredi ary Endo helial Dys rophy 140

Ch a pt er 6 Corneal Degenerations and Deposits 142 Involu ional Changes 142 Corneal Arcus 142 Whi e Limbal Girdle of Vog 142 Crocodile Shagreen 142 Cornea Farina a 143 Polymorphic Amyloid Degenera ion 143 Corneal Deposi s—Nonpigmen ed 148 Band Kera opa hy 148 Salzmann’s Nodular Degenera ion 151 O her Corneal Degenera ions 153 Spheroidal Degenera ion 153 Lipid Kera opa hy 153 Coa s’ Whi e Ring 153 Corneal Deposi s—Pigmen ed 156 Cornea Ver icilla a (Vor ex Kera opa hy) 156 Crys alline Kera opa hy 158 Corneal Iron Deposi s 160 Kayser-Fleischer Ring 163 Terrien’s Marginal Degenera ion 164 Iridocorneal-Endo helial Syndrome 166

108

xii

CO NTENTS

Ch a pt er 7 Corneal In ections, Inf ammations, and Sur ace Disorders 168 Bac erial Kera i is 168 Fungal Kera i is 174 Acanthamoeba Kera i is 178 Herpes Simplex Kera i is 182 Primary Ocular Herpes 182 Recurren Ocular Herpes Simplex 184 HSV: Epi helial Kera i is (Dendri ic Ulcer) 184 HSV: Nonnecro izing S romal Kera i is (Disci orm Kera i is) 188 HSV: Necro izing S romal Kera i is 192 HSV: Neuro rophic Kera i is (Me aherpe ic Kera i is) 194 Herpes Zos er Kera i is 196 In ers i ial Kera i is (Syphili ic, Nonsyphili ic) 202 Subepi helial Inf l ra es 206 Superf cial Punc a e Kera opa hy (Punc a e Epi helial Erosions) 208 T ygeson’s Superf cial Punc a e Kera opa hy 210 Kera oconjunc ivi is Sicca (Dry Eye Syndrome) 212 Filamen ary Kera opa hy 216 Exposure Kera opa hy 218 Neuro rophic Kera opa hy 220 Recurren Corneal Erosion 222 Bullous Kera opa hy 225 Acquired Immunodef ciency Syndrome 228 Con ac Lens Complica ions 230 oxic/ Allergic Conjunc ivi is 230 Gian Papillary Conjunc ivi is 230 Con ac Lens Kera opa hy (Con ac Lens–Associa ed Superior Limbic Kera oconjunc ivi is) 230 Con ac Lens Overwear Syndrome 230 igh Lens Syndrome 231 Corneal Warpage 231 Corneal Neovasculariza ion 231 S erile Kera i is 231 Microbial Kera i is 232

Ch a pt er 8 Systemic and Immunologic Conditions A ecting the Cornea 236 Wilson’s Disease (Hepa olen icular Degenera ion) 236 Vi amin A Def ciency 238 Cys inosis 240 Mucopolysaccharidoses and Lipidoses 242 Collagen Vascular Diseases 244 Ocular Cica ricial Pemphigoid 249 S evens-Johnson Syndrome (Ery hema Mul i orme Major) Mooren’s Ulcer 255

252

CO NTENTS

Phlyc enulosis 257 S aphylococcal Hypersensi ivi y 259 Corneal Gra Rejec ion 261

Ch a pt er 9 Anterior Sclera and Iris 266 Episcleri is 266 An erior Scleri is 269 Iris Cys s 274 Iris Pigmen Epi helial Cys 274 Iris S romal Cys 274 Iris umors 276 Iris Nevus 276 Malignan Melanoma 276 Me as a ic umor 276 Vascular umor 276

Ch a pt er 10 Surgery and Complications 278 Ca arac Ex rac ion and In raocular Lens Implan a ion 278 Full-T ickness Corneal ransplan a ion (Pene ra ing Kera oplas y) Endo helial Kera oplas y 290 An erior Lamellar Kera oplas y 295 Corneal Biopsy 298 Superf cial Kera ec omy 299 Excimer Laser Pho o herapeu ic Kera ec omy 300 Conjunc ival Flap 303 Limbal S em Cell ransplan a ion 306 Amnio ic Membrane ransplan a ion 308 Corneal Per ora ion 311 Re rac ive Surgery 313

Ch a pt er 11 Trauma 324 Chemical Burns 324 T ermal and Elec rical Burns 330 T ermal Burns 330 Elec rical Burns 330 Ul raviole Kera opa hy (Arc Welder’s Flash) 332 Corneal Abrasion 334 Corneal and Conjunc ival Foreign Bodies 336 Subconjunc ival Hemorrhage 340 Corneoscleral Lacera ion and Wound Dehiscence 342 rauma ic Hyphema 350 Epi helial Downgrow h 353 Desceme ’s De achmen 356

Index 359

282

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CO LO R ATLAS & SYNO PSIS OF CLINICAL OPHTHALMOLOGY

W i l l s Ey e I n s t i t u t e

Co rn e a S ECON D EDITION

C H AP T ER

Conjunc ival In ec ions and In amma ions BLEPH ARITIS AND MEIBOMITIS

C

hronic blephari is and meibomi is are very common, bila eral in amma ions o he eyelid margins ha may cause nonspecif c ocular irri a ion, which is o en worse in he morning. On he o her hand, some pa ien s have severe blephari is bu no symp oms.

Etiology S aphylococcal in ec ion, acne rosacea, seborrheic derma i is Symptoms Burning, i ching, discom or , oreign-body sensa ion, earing, crus ing, mild discharge, uc ua ion in vision Signs Associa ed a opic and seborrheic derma iis, and ocular rosacea Hyperemia, elangiec asias, crus ing, scaling, orma ion o collaret es around bases o

2

lashes (s aphylococcal), sleeves along eyelashes (seborrheic), and pou ing o meibomian gland orif ces, which can be expressed o produce a hickened lipid secre ion, some imes o oo hpas e-like consis ency ( Fig. 1-1) Fro hy and oamy ear f lm, conjunc ival injec ion, in erior superf cial punc a e kera opa hy, phlyc enulosis, corneal inf l ra es Treatment Warm compresses 5 o 10 minu es b.i.d., eyelid margin scrubs wi h mild commercially available cleansers (e.g., Ocuso Lid Scrub, Advanced Vision Research S erilid) ear supplemen s while awake, opical azi hromycin drops or ery hromycin, baci racin, or e racycline oin men a bed ime Oral e racycline 250 mg b.i.d. o q.i.d. or doxycycline 100 mg q.d. o b.i.d. in severe or recurren cases. T ese medica ions can o en be apered o a much lower dose or long- erm use (e.g., doxycycline 20 mg

Blepharitis and Meibomitis

b.i.d. or 50 mg q.d.). Oral ery hromycin (approxima ely 200 mg/ day) can be used or children. Judicious shor - erm use o opical cor icos eroids or phlyc enulosis or inf l ra es

3

Prognosis Good or signif can improvemen in symp oms over weeks, bu pa ien s need o unders and ha he condi ion is con rolled ra her han cured.

A

B FIGURE 1-1. A. Blepharitis.S ignif can crus ing a he base o he eyelashes is seen. A ew collaret es are presen . B. Meibomitis. Severe pou ing o he meibomian glands o he in erior eyelid can be seen. T e eyelid margin is hickened and in amed, wi h some conjunc ival injec ion visible.

4

1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMAT IO NS

CH ALAZION ( INTERNAL HORDEOLUM, ST YE)

A

chalazion is a ender eyelid mass, o en wi h surrounding ery hema and swelling. I may be small or large, and can cause signif can eyelid in amma ion when severe. Etiology Blockage o meibomian gland orif ces and s agna ion o sebaceous secre ions Associa ed wi h blephari is/ meibomi is and acne rosacea Symptoms Eyelid swelling, pain, and redness O en a his ory o previous chalazia Rarely, large, cen ral chalazia can cause corneal at ening, especially a er re rac ive surgery, or induced as igma ism. Signs Subcu aneous round, f rm, swelling in he arsal pla e ( Fig. 1-2) May have an associa ed pyogenic granuloma on eversion o eyelid Some imes may be associa ed wi h signif can eyelid in amma ion (presep al celluli is) Dif erential Diagnosis Ex ernal hordeolum: an acu e s aphylococcal in ec ion o a lash ollicle and i s associa ed gland o Zeis or Moll

Pyogenic granuloma: a vascularized mass pro ruding rom he conjunc iva Sebaceous carcinoma: suspec in recurren chalazia, eyelid margin excoria ion, or loss o lashes, especially i unila eral Diagnosis Eyelid biopsy i suspicious or sebaceous carcinoma Treatment Warm compresses, eyelid massage, and hygiene (see Blephari is/ Meibomi is above) opical azi hromycin drops or ery hromycin, baci racin, or e racycline oin men or blephari is/ meibomi is Oral e racycline 250 mg b.i.d. o q.i.d. or doxycycline 100 mg q.d. o b.i.d. in in amed, severe, or recurren cases, o preven recurren chalazia Cor icos eroid injec ion can be considered o reduce scarring in recalci ran cases. Incision and curet age i no improvemen wi h medical rea men . Prognosis Very good wi h medical rea men I medical rea men is unsuccess ul, surgical rea men is qui e e ec ive.

Chalazion (Internal Hordeolum, Stye)

5

A

B FIGURE 1-2. Chalazion. A. A large, in amed chalazion o he upper eyelid. Severe blephari is and crus ing o he eyelid margin, predisposing ac ors or developmen o chalazia, are also presen . B. Lower-eyelid eversion reveals a large indura ed mass consis en wi h a chalazion.

6

1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMAT IO NS

BACTERIAL CONJUNCTIVITIS ( NONGONO CO CCAL)

B

ac erial conjunc ivi is is a rela ively uncommon, usually bila eral condi ion, charac erized by a mucopurulen or purulen discharge. Etiology Staphylococcus aureus, Staphylococcus epidermidis Streptococcus pneumoniae Haemophilus inf uenzae (especially in children), o hers Symptoms Redness, discharge, oreign-body sensaion, burning, i chiness, pho ophobia Signs Purulen or mucopurulen discharge ( Fig. 1-3) Conjunc ival hyperemia, maximal in he ornices Pseudomembranes may be presen in severe in ec ions. Corneal punc a e epi heliopa hy

Preauricular lymphadenopa hy is usually absen . Diagnostic Evaluation Conjunc ival swab or Gram s ain, culures, and sensi ivi ies i severe or recurren Treatment Spon aneous resolu ion in days o 1 o 2 weeks is usual. Ar if cial ears o wash away discharge Empiric broad-spec rum opical an ibio ic drops (e.g., polymyxin B/ rime hoprim, uoroquinolones, gen amicin, obramycin, neomycin/ gramicidin/ baci racin) q.i.d. or 1 week or azi hromycin b.i.d. or 2 days hen q.d. or 5 days An ibio ic oin men s (e.g., cipro oxacin, obramycin, gen amicin, e racycline, baciracin, polymyxin B/ baci racin) can be used q.i.d. or 1 week in pa ien s in whom he drops wash ou very quickly, such as crying children. Prognosis Very good Severe in ec ions can cause permanen conjunc ival scarring.

Bacterial Conjunctivitis (Nongonococcal)

7

A

B

FIGURE 1-3. Bacterial conjunctivitis. A. Di use conjunc ival injec ion and a purulen discharge are presen in his eye wi h bac erial conjunc ivi is. B. A severe purulen discharge wi h crus ing can be seen in his pa ien who has bac erial conjunc ivi is. T ere is also modera e conjunc ival injec ion.

8

1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMAT IO NS

GONO CO CCAL BACTERIAL CONJUNCTIVITIS

G

onococcal conjunc ivi is is a rare, occasionally bila eral condi ion, charac erized by acu e onse o a severe purulen discharge. Etiology Primarily Neisseria gonorrhoeae Occasionally Neisseria meningitidis I is ypically sexually ransmit ed.

Symptoms Redness, severe purulen discharge, oreignbody sensa ion, burning, pho ophobia Hyperacu e onse (wi hin 12 o 24 hours) Signs Severe purulen discharge; pseudomembranes may be presen Marked conjunc ival in amma ion and chemosis ( Fig. 1-4A) Eyelid swelling Preauricular lymphadenopa hy o en presen Corneal punc a e epi heliopa hy, epi helial de ec , inf l ra e, ulcer, or per ora ion ( Fig. 1-4B) Diagnostic Evaluation Conjunc ival scraping or immedia e Gram s ain, cul ures, and sensi ivi ies. T e

diagnosis is conf rmed i he Gram s ain demons ra es gram-nega ive in racellular diplococci. Treatment Sys emic ce riaxone 1 g IM in a single dose i here is no corneal involvemen . I he pa ien is allergic o cephalosporins, uoroquinolones are he drugs o choice. I here is corneal involvemen or corneal involvemen canno be excluded because o a limi ed sli -lamp examina ion, he pa ien should be rea ed wi h ce riaxone 1 g IV q12h o q24h or 3 days. opical uoroquinolone (e.g., ciprooxacin) drops q2h, or q1h i he cornea is involved. Ocular irriga ion wi h saline q.i.d. o q2h o elimina e he discharge. Evalua e and rea or possible coin ec ion wi h Chlamydia (e.g., azi hromycin 1 g PO once). Evalua e sexual par ners or sexually ransmit ed in ec ions. Prognosis Very good i diagnosed and rea ed appropria ely be ore corneal involvemen occurs. I he cornea is involved, he prognosis is guarded.

Gonococcal Bacterial Conjunctivitis

9

A

B FIGURE 1-4. Gonococcal conjunctivitis. A. Severe in amma ion and chemosis are presen hroughou he conjunc iva in his righ eye. Some purulen discharge is presen on he eyelid and conjunc iva nasally. T e cornea is no involved. B. A large corneal ulcer wi h signif can issue loss is ound in he superior cornea; i is cri ical o examine he en ire cornea in eyes wi h gonococcal conjunc ivi is o de ermine whe her here is corneal involvemen .

10

1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

VIRAL CONJUNCTIVITIS ( T YPICALLY ADENOVIRUS)

V

iral conjunc ivi is is a common, highly con agious, usually bila eral condi ion, charac erized by he rapid onse o redness, i chiness, and earing, f rs in one eye and hen he o her. Etiology Adenovirus sero ypes 8, 19, 37: epidemic kera oconjunc ivi is Adenovirus sero ypes 3, 7: pharyngoconjunc ival ever, usually in children O hers: herpes simplex virus, en eroviruses, Newcas le disease virus, Eps ein-Barr virus Symptoms earing, i ching, burning, redness, oreignbody sensa ion, pho ophobia His ory o con ac wi h someone wi h a red eye, recen upper respira ory rac in ecion, or recen eye examina ion Signs Eyelid edema Wa ery discharge Generalized conjunc ival hyperemia, subconjunc ival hemorrhages Conjunc ival ollicles, which are requen ly mos apparen in he in erior ornices ( Fig. 1-5A) Membranes or pseudomembranes in severe cases Conjunc ival membranes consis o coagula ed exuda e adheren o in amed

conjunc ival epi helium. Clinically, a rue membrane causes bleeding on at emp ed removal and a pseudomembrane does no , bu his rule is no universal. T e causes o rue membranes and pseudomembranes are similar. Cen ral punc a e epi helial kera i is, and occasionally an epi helial de ec ( Fig. 1-5B). Mul iple small corneal inf l ra es wi h overlying punc a e s aining may also be seen in he acu e phase o severe in ec ions ( Fig. 1-5C). Preauricular lymphadenopa hy is o en presen . Subepi helial inf l ra es (SEIs) can occur weeks a er he onse o he acu e in ec ion and may persis or mon hs o years ( Fig. 1-5D) Treatment Ar if cial ears and cool compresses our o eigh imes a day An ihis amines (e.g., an azoline, naphazoline) q.i.d. or i ching Removal o membranes or pseudomembranes Cor icos eroid drops in severe cases wi h membranes or pseudomembranes or erosions. A long, slow aper o mild cor icos eroid drops can be used in eyes wi h SEIs ha a ec visual unc ion. S ric observa ion o hygienic measures is needed o avoid spreading he in ec ion. Prognosis Very good. I clinically signif can subepihelial inf l ra es develop, he rea men course can be prolonged. Severe in ec ions wi h membranes or pseudomembranes can cause permanen conjunc ival scarring ( Fig. 1-5E).

Viral Conjunctivitis (Typically Adenovirus)

11

A

B FIGURE 1-5. Viral conjunctivitis. A. Di use conjunc ival injec ion wi h a severe ollicular reac ion, grea es in eriorly, is presen in his eye wi h viral conjunc ivi is. B. A cen ral punc a e epi helial kera i is as seen in his eye is o en ound early in he course o viral conjunc ivi is, mos commonly caused by adenovirus. ( continued)

12

1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

C

D FIGURE 1-5. (Continued) Viral conjunctivitis. C. In he acu e phase, small superf cial corneal inf l ra es wi h overlying punc a e s aining can develop. No e he irregular ligh re ex. D. Mul iple subepi helial inf l ra es (SEIs) o he cornea can be seen 2 mon hs a er resolu ion o adenoviral kera oconjunc ivi is. T ese SEIs end o resolve on heir own. I hey are severe, hey can a ec visual acui y and cause glare symp oms. SEIs generally respond o low-dose opical cor icos eroid drops; however, i hey are s ar ed, hese drops need o be apered very slowly, over mon hs. ( continued)

Viral Conjunctivitis (Typically Adenovirus)

13

E

FIGURE 1-5. ( Continued) Viral conjunctivitis. E. In erior conjunc ival scarring is seen in his eye several mon hs a er adenoviral conjunc ivi is.

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

CHLAMYDIAL CONJUNCTIVITIS ( ADULT INCLUSION CONJUNCTIVITIS)

A

dul chlamydial conjunc ivi is is a relaively common, usually unila eral condiion ha is ypically ransmit ed sexually and generally a ec s young adul s. Etiology Chlamydia trachomatis sero ypes D hrough K ypically sexually ransmit ed Symptoms earing, i ching, burning, redness, oreignbody sensa ion, pho ophobia, discharge o longer han 3 o 4 weeks in dura ion May be associa ed wi h ure hri is, vaginiis, or cervici is Signs S ringy, whi e mucopurulen discharge Large ollicles in he in erior ornices ( Fig. 1-6) Superior arsal ollicles, occasionally ollicles a he limbus Superior limbal or peripheral nummular corneal inf l ra es and pannus

Mild preauricular lymphadenopa hy may be presen . Diagnosis His ory o sexual exposure; pa ien may have concomi an geni ourinary symp oms Direc immuno uorescen an ibody es o conjunc ival smears Giemsa s ain cy ology or basophilic cy oplasmic inclusion bodies o Halbers aed erProwazek; more common in newborns han adul s McCoy chlamydial cell cul ure Treatment Azi hromycin 1 g PO once, doxycycline 100 mg PO b.i.d., or e racycline, ery hromycin or clari hromycin 250 mg q.i.d. or 2 o 6 weeks opical azi hromycin drops b.i.d. or 2 days, hen q.i.d. or 1 o 6 weeks, or e racycline or ery hromycin oin men q.i.d. or 4 o 6 weeks Re erral or rea men o sexual par ners and o her sexually ransmit ed in ec ions should be done. Prognosis Very good

Chlamydial Conjunctivitis (Adult Inclusion Conjunctivitis)

15

FIGURE 1-6. Chlamydial conjunctivitis. A severe in erior conjunc ival ollicular reac ion can be seen in his eye wi h chronic chlamydial conjunc ivi is. T ere were similar conjunc ival ollicles superiorly. T ere is also di use bulbar conjunc ival injec ion.

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

TRACHOMA

T

rachoma is a bila eral conjunc ivi is ha is common in underdeveloped coun ries where hygiene is poor. I is endemic in A rica and cer ain par s o Asia and is one o he mos common causes o preven able blindness, a ec ing millions o people around he world.

Etiology Chlamydia trachomatis sero ypes A, B, Ba, and C Signs World Heal h Organiza ion Classif ca ion rachoma ous ollicular in amma ion ( F): more han f ve ollicles grea er han 0.5 mm on he upper arsus rachoma ous in ense in amma ion ( I): hickening obscuring more han 50% o arsal vessels rachoma ous scarring ( S): cica rizaion wi h whi e lines or bands in arsal conjunc iva (Arl ’s line) ( Fig. 1-7)

rachoma ous richiasis ( ): richiasis o a leas one eyelash Corneal opaci y (CO): corneal opaci y involving a leas par o he pupillary margin Cica riza ion o limbal ollicles resul s in depressions known as Herber ’s pi s. Diagnostic Evaluation Diagnos ic inves iga ion is similar o ha or adul inclusion conjunc ivi is. Treatment SAFE s ra egy: surgery or richiasis, an ibio ics (repea ed every 6 o 12 mon hs in endemic areas), acial and environmen al hygiene An ibio ic rea men similar o ha or adul inclusion conjunc ivi is Prognosis Very good unless signif can corneal scarring has developed. Rein ec ion is common i hygienic condi ions do no improve.

Trachoma

17

FIGURE 1-7. Trachoma. Whi e scarring o he superior arsal conjunc iva is presen . T e whi e line is called an Arl ’s line.

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

MOLLUSCUM CONTAGIOSUM

M

olluscum con agiosum is an uncommon cause o chronic ollicular conjunc ivi is, which is usually unila eral and may be missed i he eyelid margin is no examined closely. Etiology Viral par icles rom molluscum con agiosum lesions o he eyelid may cause a oxic response o he conjunc iva. Symptoms earing, i ching, burning, redness, oreignbody sensa ion. May be chronic. Signs Single or mul iple, dome-shaped, umbilica ed, molluscum con agiosum eyelid lesions

associa ed wi h ollicular conjunc ivi is. May be chronic ( Fig. 1-8). Wa ery or mucoid discharge Corneal micropannus i long-s anding In immunocompromised pa ien s, here may be ex ensive eyelid lesions wi h minimal conjunc ival in amma ion. Treatment Removal o eyelid lesion by incision and curet age, shaving, excision, cau eriza ion, or cryo herapy. I severe, consider a workup or an immune def ciency disorder such as HIV in ec ion. Prognosis Very good, bu i can ake many weeks or he ollicular conjunc ivi is o resolve a er he lesion is rea ed.

Molluscum Contagiosum

19

A

B FIGURE 1-8. Molluscum contagiosum. A. A severe in erior palpebral conjunc ival ollicular reac ion o several mon hs’ dura ion is apparen . Generally, he bulbar conjunc iva is much less injec ed han he palpebral conjunc iva. B. In he same pa ien , an umbilica ed creamy-colored nodule is seen a he upper eyelid margin. I hey are no searched or care ully, molluscum con agiosum lesions can easily be overlooked. Small incision and curet age in o he cen er o he lesion, causing bleeding, is o en cura ive.

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

LIGNEOUS CONJUNCTIVITIS

L

igneous disease is a very rare cause o chronic unila eral or bila eral conjunc ivi is wi h charac eris ic “woody,” hick membrane orma ion.

Etiology Due o a plasminogen def ciency, o en au osomal recessive, may be sporadic Symptoms earing, i ching, burning, redness, oreignbody sensa ion May have an eyelid mass Generally chronic Signs Chronic conjunc ivi is wi h woodlike, hick membrane orma ion on he upper

arsus and occasionally he lower arsus ( Fig. 1-9) May have similar involvemen o he mou h, nasopharynx, rachea, and geni ourinary mucous membranes. Treatment Removal o pseudomembranes opical cyclosporine, heparin, hyaluronidase, or cor icos eroids opical or sys emic lys-plasminogen replacemen herapy may be benef cial. Prognosis Poor wi h previous rea men s opical or sys emic lys-plasminogen replacemen herapy is a promising rea men or his rare condi ion.

FIGURE 1-9. Ligneous conjunctivitis. Prominen whi e, “woody” membranes at ached o he superior palpebral conjunc ivas o bo h eyes are presen in his baby wi h ligneous conjunc ivi is. (Cour esy o Rolande Michaud, MD.)

Pediculosis

PEDICULOSIS

P

ediculosis is a sexually ransmit ed in ec ion ha is caused by con ac wi h pubic lice.

Etiology Eyelid in ec ion wi h pubic lice Symptoms earing, i ching, burning, redness, oreignbody sensa ion. May be unila eral or bila eral May be chronic Signs Lice, ni s (eggs), and blood- inged debris on eyelids and lashes ( Fig. 1-10) Mild o severe chronic ollicular conjunc ivi is

21

Treatment Remove all lice and ni s under sli -lamp illumina ion. opical an ibio ic oin men (e.g., e racycline, baci racin, or ery hromycin) on eyelids .i.d. or 1 o 2 weeks o smo her he lice and ni s. Oral ivermec in, wo doses (exac dose depends on body weigh ), 1 week apar , has been shown o be e ec ive. Use an ilice shampoo and lo ion o rea nonocular areas. Wash and machine dry all clo hes and linens. rea sexual par ners. Prognosis Good i all he lice and ni s are removed. Rein ec ion can occur i sexual par ners and clo hes and linens are no rea ed appropria ely.

FIGURE 1-10. Pediculosis. Several lice can be seen at ached o he base o he eyelashes in his le eye wi h pediculosis. T e mos obvious one is loca ed emporally. Numerous ubular ni s are presen on he eyelash sha s. Some blood- inged debris can be seen a he base o he lashes.

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

PARINAUD’S OCULO GLANDULAR SYNDROME

P

arinaud’s oculoglandular syndrome is an uncommon, usually unila eral condi ion wi h diverse causes, charac erized by conjuncival granulomas and ex remely swollen preauricular and submandibular lymph nodes.

Etiology Ca -scra ch ever is he mos common cause. Mononucleosis ularemia uberculosis Rare causes: sporo richosis, syphilis, coccidioidomycosis, chancroid, lymphogranuloma venereum Symptoms Redness, oreign-body sensa ion, discharge Fever, malaise, skin rash

Signs Unila eral conjunc ival granulomas and large ollicles ( Fig. 1-11) Severe ipsila eral preauricular or submandibular lymphadenopa hy Diagnostic Evaluation Appropria e blood es s, conjunc ival s ains, cul ures, and conjunc ival biopsy Comple e blood coun , serology, and ches radiograph as needed Treatment opical an ibio ic oin men (e.g., e racycline, ery hromycin, cipro oxacin, baci racin, polymyxin B/ baci racin) q.i.d. or 4 weeks Sys emic rea men varies according o cause. Prognosis Generally good, al hough i depends on he specif c e iology.

Parinaud’s Oculoglandular Syndrome

23

FIGURE 1-11. Parinaud’s oculoglandular syndrome. Severe di use conjunc ival in amma ion along wi h a supero emporal conjunc ival granuloma is presen in his child wi h ca -scra ch disease. No e he skin abrasions near he nose, which were presumably caused by a ca scra ch. (Cour esy o Pe er Laibson, MD.)

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

OPHTH ALMIA NEONATORUM

N

eona al conjunc ivi is (oph halmia neona orum) is unila eral or bila eral conjunc ival in amma ion occurring during he f rs mon h o li e. Etiology Chemical: Usually causes rela ively mild di use injec ion wi hou discharge, which las s no longer han 24 hours. ypically due o prophylac ic silver ni ra e drops. Neisseria gonorrhoeae: Causes copious purulen discharge, which may be associa ed wi h membrane orma ion. Presen s wi hin f rs ew days o li e. Herpes simplex ype 2: Associa ed wi h eyelid margin vesicles. Presen s wi hin 1 week o bir h. Chlamydia trachomatis: Causes a purulen papillary conjunc ivi is because he in an canno produce ollicles. May have pseudomembranes. Presen s during he second week o li e. Simple bac erial (e.g., Staphylococcus, Streptococcus, gram-nega ive species): Presen s wi hin f rs ew days a er bir h. Signs Eyelid edema, conjunc ival injec ion, chemosis, purulen discharge ( Fig. 1-12) Kera i is is uncommon bu may occur i rea men or gonococcal conjunc ivi is is delayed. Dif erential Diagnosis Nasolacrimal duc obs ruc ion: earing, nonpurulen discharge, and no in ec ion

Diagnostic Evaluation Conjunc ival scrapings or Gram s ain, Giemsa s ain, bac erial cul ure, immuno uorescen es s, and viral cul ure Never assume ha only one pa hogen is responsible. Treatment Evalua e bo h paren s or geni al in ec ion and rea accordingly. Empiric: opical e racycline, cipro oxacin, baci racin, polymyxin B/ baci racin or ery hromycin oin men q.i.d. and ery hromycin 25 mg/ kg PO b.i.d. or 2 weeks Chemical: Ar if cial ears drops or gels, or no rea men Neisseria gonorrhoeae: opical saline irrigaion our o eigh imes a day. opical penicillin or a uoroquinolone (e.g., cipro oxacin) q1h. Sys emic single-dose ce riaxone 125 mg IM. Ery hromycin 12.5 mg/ kg PO q.i.d. or 2 weeks Herpes simplex ype 2: opical ganciclovir (e.g., Zirgan) gel drops or vidarabine (e.g., Vira-A) oin men f ve imes a day and aper over 1 o 2 weeks. Consider IV acyclovir. Chlamydia trachomatis: opical azi hromycin drops b.i.d. or 2 days, hen q.d. or e racycline or ery hromycin oin men q.i.d. plus ery hromycin 12.5 mg/ kg PO q.i.d. or 2 weeks Simple bac erial: Cipro oxacin, baci racin, polymyxin B/ baci racin, gen amicin, or obramycin oin men q.i.d. or 2 weeks Prognosis Generally good i diagnosed and rea ed appropria ely be ore any corneal or sys emic involvemen occurs

Ophthalmia Neonatorum

25

FIGURE 1-12. Ophthalmia neonatorum.T is in an had a severe di use conjunc ivi is rom a Chlamydia in ec ion. (Cour esy o Irving Raber, MD.)

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

ALLERGIC CONJUNCTIVITIS

Signs Eyelid edema Wa ery or mucoid discharge Conjunc ival hyperemia wi h a mild papillary response ( Fig. 1-13A) Chemosis ( Fig. 1-13B) T e cornea is generally no involved.

Treatment Avoid exposure o o ending allergen, including requen change o clo hes, showering, and washing o clo hes and bed linens. Cool compresses, ar if cial ears opical an ihis amines (e.g., emedas ine, levocabas ine, naphazoline, an azoline) q.i.d. opical mas cell s abilizers (e.g., cromolyn, lodoxamide, pemirolas ) q.i.d. opical an ihis amine/ mas cell s abilizers (e.g., azelas ine, epinas ine, ke o i en, nedocromil, olopa adine, bepo as ine, alca adine) q.d. o b.i.d. Oral an ihis amine (e.g., diphenhydramine 25 mg PO .i.d., ce irizine 5 o 10 mg PO q.d., exo enadine 30 o 60 mg PO b.i.d., lora adine 5 o 10 mg PO q.d.), especially i rhini is is presen Mild opical cor icos eroid i severe (e.g., lo eprednol 0.2%, uorome holone 0.1%) q.i.d. or shor dura ion Skin es ing and desensi iza ion herapy can be help ul in some cases.

Dif erential Diagnosis Perennial varian can occur a any ime o or hroughou he year.

Prognosis Good, bu mild chronic symp oms o en persis .

A

llergic conjunc ivi is (e.g., hay ever conjunc ivi is) is a very common ype I hypersensi ivi y reac ion ha causes conjuncival injec ion and i ching and generally occurs during he hay ever season. Etiology Pollen, grass, spores, hair, pe s, wool, dus , mi es, e c. Symptoms I ching, mucous discharge, earing, redness, his ory o allergy. Symp oms are ypically seasonal and vary wi h exposure.

Allergic Conjunctivitis

27

A

B FIGURE 1-13. Allergic conjunctivitis. A. Modera e conjunc ival injec ion and in erior papillary reac ion is presen in his eye wi h chronic allergic conjunc ivi is. B. Conjunc ival chemosis can be seen emporally, resul ing rom an acu e allergic reac ion o ca ur ouching he eye.

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

ATOPIC KERATO CONJUNCTIVITIS

A

opic kera oconjunc ivi is is an uncommon, bila eral perennial condi ion ha may also involve he eyelids and occurs primarily in pa ien s wi h a opic derma i is. Etiology Chronic ype I hypersensi ivi y allergic reac ion similar o vernal kera oconjunc ivi is, bu causing more prominen eyelid and periorbi al skin involvemen Symptoms I ching, earing, redness, discharge His ory o a opy Signs Eyelid crus ing, eczema, and s aphylococcal blephari is ( Fig. 1-14A) Mucoid discharge Small papillae on he palpebral conjunciva wi h edema giving a velve y appearance Conjunc ival scarring and symblepharon orma ion in advanced cases Corneal punc a e epi helial erosions, peripheral vasculariza ion and scarring ( Fig. 1-14B) May have associa ed kera oconus, ca arac , and re inal de achmen Dif erential Diagnosis Di ers rom vernal kera oconjunc ivi is in ha a opic kera oconjunc ivi is presen s

in adul li e, papillae are small, here is an absence o limbi is and ran as’ do s, and i may cause neovasculariza ion and cica riza ion. Treatment Cool compresses, ar if cial ears opical mas cell s abilizers (e.g., cromolyn, lodoxamide, pemirolas ) q.i.d. or an ihisamine/ mas cell s abilizers (e.g., azelas ine, epinas ine, ke o i en, nedocromil, olopa adine, bepo as ine, alca adine) q.d. o b.i.d. opical cor icos eroid i severe (e.g., uorome holone oin men b.i.d. on eyelids and/ or lo eprednol 0.2% o 0.5%, uorome holone 0.1%, or prednisolone 0.125% o 1.0% drops q.i.d.) as shor - erm rea men Pimecrolimus 1% cream (e.g., Elidel) or acrolimus 0.03% oin men (e.g., Pro opic) b.i.d. can be applied o a ec ed skin in pa ien s older han 2 years or several weeks. T ese medica ions have been associa ed wi h skin cancer and lymphoma. An oral an ihis amine may be help ul (e.g., diphenhydramine 25 mg PO .i.d., ce irizine 5 o 10 mg PO q.d., exo enadine 30 o 60 mg PO b.i.d., lora adine 5 o 10 mg PO q.d.). Cyclosporine drops (e.g., cyclosporine 0.05% o 2% b.i.d. o q.i.d.) can have a cor icos eroid-sparing e ec in severe cases. Prognosis Fair o good, depending on he severi y o he condi ion. In raocular pressure mus be moni ored regularly when cor icos eroids are used, even i only on he eyelids.

Atopic Keratoconjunctivitis

29

A

B

FIGURE 1-14. Atopic keratoconjunctivitis. A. Eyelid ery hema, hickening, and scaling are apparen in his pa ien wi h a opic disease. T e skin has a lea hery ex ure, and here is loss o lashes. Conjunc ival injec ion and an old in erior corneal scar can be apprecia ed. B. Severe superior and in erior corneal scarring has developed in his eye wi h chronic a opic kera oconjunc ivi is.

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

VERNAL KERATO CONJUNCTIVITIS

V

ernal kera oconjunc ivi is (spring ca arrh) is a seasonal, recurren , bila eral, ype I hypersensi ivi y reac ion ha usually presen s in childhood and resolves gradually a er puber y. Etiology and Epidemiology ype I hypersensi ivi y allergic reac ion similar o a opic kera oconjunc ivi is, bu wi h a seasonal exacerba ion and less eyelid and skin involvemen . Males are a ec ed more commonly han emales. Symptoms I ching, earing, oreign-body sensa ion, burning, discharge Signs S ringy, mucopurulen discharge Milky-whi e pseudomembranes Superior arsal papillae o medium o gian size, p osis ( Fig. 1-15A) Occasionally, limbal papillae (limbi is) ha may be associa ed wi h small whi e spo s con aining eosinophils ( ran as’ do s) ( Fig. 1-15B and C) Superior corneal punc a e epi helial erosions, corneal ulcera ion (“shield” ulcer) ( Fig. 1-15D)

Mild subepi helial corneal scarring Dif erential Diagnosis Gian papillary conjunc ivi is (GPC) is much less severe, and is charac erized by small- o medium-sized superior arsal papillae. I can be unila eral or bila eral, depending on he cause. Also, GPC is caused by an allergic reac ion o pro ein buildup on con ac lenses, par icularly so lenses; ocular pros he ics; or pro ruding su ures ollowing ocular surgery. Treatment I mild, rea as or a opic conjunc ivi is. An ihis amine/ mas cell s abilizers are e ec ive, especially i ini ia ed be ore he allergy season. T ey also have a cor icos eroid-sparing unc ion. I severe or in he presence o shield ulcer, use a shor course o opical cor icos eroids wi h an ibio ic drops or oin men s our o six imes a day. Prolonged use o cor icos eroids is discouraged, and moni oring o in raocular pressure and lens clari y should be per ormed regularly. Shield ulcers may require surgical removal o he adheren plaque. Prognosis Fair o good, depending on he severi y o he condi ion.

Vernal Keratoconjunctivitis

31

A

B FIGURE 1-15. Vernal conjunctivitis. A. Large, con uen papillae o he superior arsal conjunc iva are presen . T ese are a - opped and are ermed “cobbles one” papillae. B. Papillae can be more prominen a he limbus han he arsal conjunc iva in cer ain pa ien s, mos commonly o A rican heri age. In hese pa ien s he condi ion is called limbal vernal conjunc ivi is and he whi e spo s are ermed ran as’ do s. In his pa ien , limbal ollicles and ran as’ do s can be seen superiorly, especially a he 10 o’clock and 1 o 3 o’clock limbus. ( continued)

32

1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

C

D FIGURE 1-15. (Continued) Vernal conjunctivitis. C. Mul iple small whi e ran as’ do s are visible a he in erior limbus D. An ovoid epi helial de ec loca ed in he superior one- hird o he cornea (a “shield” ulcer) is apparen in his eye. T ere is also modera e surrounding punc a e kera opa hy. No e he poor corneal ligh re ex.

Superior Limbic Keratoconjunctivitis

33

SUPERIOR LIMBIC KERATO CONJUNCTIVITIS

Superior corneal f lamen s, punc a e erosions, and occasionally pannus

S

Treatment Preserva ive- ree ar if cial ear drops q2h. Consider emporary or permanen punc al occlusion. Cyclosporine 0.05% o 2% b.i.d. o q.i.d. may be help ul. Ace ylcys eine (e.g., Mucomys ) 10% drops q.i.d. or rea men o corneal f lamen s opical an ihis amine/ mas cell s abilizers (e.g., azelas ine, epinas ine, ke o i en, nedocromil, olopa adine, bepo as ine, alca adine) q.d. o b.i.d. may be help ul. Applica ion o silver ni ra e 0.5% solution o superior bulbar and palpebral conjunc iva or 15 o 30 seconds Local cau ery or surgical resec ion o superior bulbar conjunc iva ( Fig. 1-16B)

uperior limbic kera oconjunc ivi is (SLK) is an uncommon, usually bila eral, chronic, relapsing, in amma ory reac ion ha is requen ly associa ed wi h hyroid dys unc ion. I usually a ec s middle-aged emales.

Etiology Unknown, bu mos likely rela ed o mechanical rauma involving he superior palpebral and lax bulbar conjunc iva Symptoms Foreign-body sensa ion, burning, occasionally redness Signs Hyperemia, hickening, redundance, and laxi y o superior bulbar conjunc iva ( Fig. 1-16A) Lack o lus er and posi ive s aining o superior bulbar conjunc iva wi h uorescein, lissamine green, and rose bengal dyes Fine, velve y, superior arsal papillae

Prognosis Good or improvemen in symp oms, air or comple e resolu ion o symp oms. May be recalci ran o rea men

34

1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

A

B FIGURE 1-16. Superior limbic keratoconjunctivitis (SLK). A. In SLK, here is localized conjunc ival injec ion o he superior bulbar conjunc iva. T ere is pannus and hickened conjunc iva a he superior limbus. B. Medical rea men ailed. Localized conjunc ival cau ery a er injec ion o local anes hesia was per ormed. T is rea men is o en success ul in signif can ly improving he pa ien ’s symp oms.

Floppy Eyelid Syndrome

FLOPPY EYELID SYNDROME

35

F

Signs Easy eversion o upper eyelid. arsus eels so and rubbery ( Fig. 1-17A) Chronic papillary conjunc ivi is o he upper arsus, superf cial punc a e kera opa hy ( Fig. 1-17B)

Etiology An ex remely lax, oppy upper eyelid ever s, causing corneal exposure.

Treatment Pro ec he eye by aping i closed or placing an eye shield during sleep. Ar if cial ears or an ibio ic oin men or lubrica ion, especially a bed ime A horizon al eyelid- igh ening procedure may be necessary o e ec long- erm improvemen .

loppy eyelid syndrome, an uncommon condi ion, is due o a spon aneous eversion o he upper eyelid during sleep, hereby exposing he upper arsal conjunc iva and cornea o rauma rom pillows or bed linens. I mos o en a ec s obese men who have a his ory o sleep apnea. I is associa ed wi h kera oconus.

Symptoms Chronic redness, oreign-body sensa ion, discharge. O en worse in he morning Symp oms are mos commonly on he side on which he pa ien sleeps.

Prognosis Good o very good wi h a horizon al eyelid- igh ening procedure.

36

1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

A

B

FIGURE 1-17. Floppy eyelid syndrome. A. Li ing he upper eyelid easily demons ra es he severe laxi y o he eyelid in his pa ien wi h oppy eyelid syndrome. B.T e upper eyelid is ex remely lax and easily ever ed by pulling he eyelid margin superiorly. T ere is a f ne, di use papillary reac ion o he upper palpebral conjunc iva.

Toxic and Factitious Keratoconjunctivitis (Keratitis Medicamentosa)

TOXIC AND FACTITIOUS KERATO CONJUNCTIVITIS ( KERATITIS MEDICAMENTOSA)

C

hronic oxic conjunc ivi is may be unila eral or bila eral, depending on he cause. Fac i ious conjunc ivi is is caused by sel ins illa ion o ma erial o cause a red eye.

Etiology Aminoglycoside an ibio ics, especially orif ed medica ions An iviral agen s Glaucoma agen s, par icularly epinephrine, brimonidine, pilocarpine, carbonic anhydrase inhibi ors opical nons eroidal an i-in amma ory agen s opical anes he ics Any preserved eyedrops Sel - rauma, o en or secondary gain, such as missing work or school Dif erential Diagnosis Mucus f shing syndrome: a rare, unila eral or bila eral condi ion resul ing rom repea ed sel - rauma iza ion o he conjunc iva while rying o remove mucus rom he conjunc iva— o en a vicious cycle, as he rauma causes addi ional mucus produc ion. Pa ien s o en do no admi o his ac ivi y unless pressed. Symptoms Chronic redness, i ching, oreign-body sensa ion, mild discharge Signs Ini ially a papillary conjunc ival reac ion, la er ollowed by orma ion o ollicles,

37

predominan ly involving he in erior ornices ( Fig. 1-18A) In erior corneal punc a e epi heliopa hy In erior conjunc ival erosions. Conjunc ival necrosis can occur in severe cases. Severe corneal involvemen may cause a s erile s romal ring inf l ra e, which is some imes mis aken or in ec ious kera i is, especially wi h anes he ic abuse ( Fig. 1-18B). Rarely, s erile corneal, conjunc ival, or scleral mel ing can occur ( Fig. 1-18C). Diagnostic Evaluation De ailed his ory aking is mos impor an . Treatment Discon inue o ending medica ion or sel -abuse. Frequen preserva ive- ree ar if cial ear drops our o eigh imes a day Mild an ibio ic oin men (e.g., ery hromycin) i here is signif can epi heliopa hy Consider pressure pa ching (marking he pa ch o make sure i is no removed) or 1 o 2 days o preven he pa ien rom placing any hing in he eye. Hospi aliza ion may some imes be required or pa ien s who are unresponsive o rea men o ensure ha hey do no con inue o use he o ending medica ion or abuse he eye, especially or anes he ic abuse. A comple e emporary arsorrhaphy is rarely required o break he o ending cycle. Prognosis Very good i he o ending medica ion or sel -abuse can be s opped be ore signif can corneal damage has occurred.

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

A

B FIGURE 1-18. Toxic/ allergic conjunctivitis. A. A chronic ollicular conjunc ivi is secondary o an allergic reac ion o opical apraclonidine (Iopidine) . I resolved over weeks once he medica ion was discon inued. Topical anesthetic abuse. B.T is pa ien was rea ed or a ungal ulcer loca ed rom he 9 o 11 o’clock posi ions. I was resolving slowly un il he s ole proparacaine rom he physician’s o ce and developed a large ring inf l ra e and hypopyon consis en wi h opical anes he ic abuse. ( continued)

Toxic and Factitious Keratoconjunctivitis (Keratitis Medicamentosa)

39

C FIGURE 1-18. (Continued) Toxic/ factitious keratoconjunctivitis. C.T is eye has a localized conjunc ival abrasion and injec ion. T ere is some associa ed subconjunc ival hemorrhage. A er ob aining a sys emic workup ( which was nega ive) , and rying several medical regimens wi hou success, i was learned ha he pa ien was poking hersel in he eye wi h a needle o ge ou o work. She was re erred or a psychological evalua ion.

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1 CO NJUNCTIVAL INFECTIO NS AND INFLAMMATIO NS

OCULAR ROSACEA

A

cne rosacea is a common skin disease o unknown e iology wi h requen ocular involvemen , ypically occurring in middleaged adul s. I is o en associa ed wi h dry-eye syndrome. Etiology Rosacea is an idiopa hic derma ologic condi ion a ec ing he pilosebaceous uni s o he acial and eyelid skin. Symptoms Chronic, bila eral ocular irri a ion, redness, burning, earing, crus ing Signs Skin: chronic hyperemia, elangiec asias, papules, pus ules o nose, orehead, and cheeks. Rhinophyma in advanced s ages, especially in men ( Fig. 1-19A) Eye: blephari is, meibomi is, eyelidmargin elangiec asias, recurren chalazia, conjunc ival or episcleral injec ion ( Fig. 1-19B) Superf cial punc a e kera opa hy, peripheral corneal vasculariza ion, s erile marginal inf l ra es, phlyc enules, peripheral corneal

scarring, pannus, hinning, and occasionally corneal mel ing and per ora ion ( Fig. 1-19C) Treatment Warm compresses 5 o 10 minu es b.i.d. and eyelid hygiene or blephari is/ meibomi is Minimally preserved or preserva ive- ree ar if cial ears or dry eyes Judicious opical cor icos eroid or s erile kera i is. When in doub , rea an inf l ra e as in ec ious kera i is. opical azi hromycin drops a bed ime (q.h.s.), or e racycline, baci racin, or ery hromycin oin men b.i.d. or q.h.s. Oral e racycline or ery hromycin 250 mg b.i.d. o q.i.d. or doxycycline 100 mg q.d. o b.i.d. or 1 week, hen hal dose or 4 o 6 weeks. Can aper down o a minimum dosage (e.g., doxycycline 20 mg b.i.d. or 50 mg q.d.) or long- erm rea men ? Consider a derma ology consul i here is signif can skin involvemen . Prognosis Good or improvemen in symp oms, poor or o al resolu ion o symp oms. Pa ien s mus realize ha rosacea is a chronic condiion ha can be e ec ively rea ed in mos cases bu no “cured.”

Ocular Rosacea

41

A

B

C FIGURE 1-19. Ocular rosacea. A. T ere are signif can papules and pus ules o he cheeks, nose, and eyebrow areas. Rhinophyma is apparen . No e he peripheral corneal scarring in ero emporally on he le rom previous corneal inf l ra e and mel ing. B. T ere are severe elangiec asias o he eyelid margin, which is no iceably hickened. No pa en meibomian gland orif ces are visible. C. elangiec asias and hickening o he eyelid margin, along wi h crus ing o he eyelashes, are seen in his pa ien wi h severe ocular rosacea. Di use conjunc ival injec ion and a dense corneal scar rom previous rosacea-rela ed ulcera ion a he 7 o’clock posi ion are presen .

C H AP T ER

Conjunc ival Degenera ions and Mass Lesions PINGUECULA AND PTERYGIUM

P

ingueculae and p erygia are ex remely common conjunc ival/ corneal degenera ions ha ypically a ec pa ien s living in equa orial regions where here is high exposure o sunligh .

Etiology Ul raviole exposure Chronic dryness and ho , dus y environmen T ese ac ors lead o elas o ic degeneraion o he subs an ia propria o he conjunc iva, resul ing in subepi helial proli era ion o brovascular issue, ini ially on he conjunc iva and hen on he cornea. Symptoms Irri a ion, redness nasally or emporally, earing, occasionally decreased vision Occasionally, con ac lens in olerance Rarely, pain i in amed 42

Signs Pinguecula: yellow-whi e, of en riangular, sligh ly eleva ed conjunc ival lesion adjacen o he nasal or emporal side o he limbus ( Fig. 2-1A). T ey may become mildly o modera ely in amed ( Fig. 2-1B). P erygium: riangular, wing-shaped brovascular shee o issue ex ending on o he cornea a he 3 and 9 o’clock posi ions ( Fig. 2-1C). An iron line (S ocker’s line) in he corneal epi helium may be presen cen ral o he apex o he p erygium. An area o hinning due o desicca ion (delle) may be presen in he cornea adjacen o an eleva ed lesion. Large or recurren p erygia can cause symblepharon orma ion and even res ric ocular mo ili y ( Fig. 2-1D). Dif erential Diagnosis Pseudop erygium: An adhesion o conjunciva on o he corneal sur ace af er corneal injury ( Fig. 2-1E). Unlike a rue p erygium, he adhesion is only a he apex and no hroughou he underlying sur ace. I is ypically unila eral and of en no a he 3 and 9 o’clock posi ions.

Pinguecula and Pterygium

Fuchs’ marginal kera i is: Associa ed wi h mild o severe peripheral corneal hinning ( Fig. 2-1F). Conjunc ival papilloma, nevus, in raepihelial neoplasia, or squamous cell carcinoma: I no ypical or a p erygium or pinguecula, consider a conjunc ival biopsy. Diagnostic Evaluation Sli -lamp examina ion o look or unusual ea ures suspicious o o her diagnoses. Pingueculae and p erygia have classic appearances. Excisional biopsy in cases suspicious or malignancy Treatment Avoid excessive sunligh exposure and wear good-quali y ul raviole -blocking sunglasses. Ar i cial ears o preven dry eyes opical an ihis amines (e.g., emedas ine, levocabas ine, naphazoline, an azoline), nons eroidal an i-in amma ory agen s (e.g., ke orolac, brom enac, nepa enac), or, rarely,

43

cor icos eroids (e.g., lo eprednol 0.2%, uorome holone 0.1%) q.d. o q.i.d. o reduce redness or in amma ion. Surgical excision is indica ed i here is excessive irri a ion, di cul y wi h con ac lens wear, or cosme ic reasons, or when here is progression oward he visual axis. T e recurrence ra e is much lower when excision is combined wi h a conjunc ival au ograf . I inadequa e conjunc ival issue is available, an amnio ic membrane graf can be used o cover he bare sclera. In raopera ive applica ion o mi omycin C and pos opera ive use o be a radia ion may decrease he recurrence ra e, bu bo h are associa ed wi h an increased risk o corneoscleral necrosis and are usually no necessary when a conjunc ival au ograf is per ormed. Prognosis Good o very good, depending on severi y P erygia can recur in abou 10% o 15% o pa ien s, occasionally worse han he original p erygium.

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

A

B

C FIGURE 2-1. Pinguecula. A. A riangular, creamy-whi e eleva ed conjunc ival mass is seen a he nasal limbus rom he 3 o he 4:30 clock posi ion. B.T is pinguecula, also loca ed be ween he 3 o 4:30 posi ions, is sligh ly inf amed, as demons ra ed by mild surrounding conjunc ival injec ion. Pter ygium. C. A nasal wing-shaped brovascular grow h is apparen in his pa ien ’s righ eye wi h a classic nasal p erygium. T is p erygium is reaching in o he visual axis. ( continued)

Pinguecula and Pterygium

45

D

E

F FIGURE 2-1. ( Continued) Pter ygium. D. T is large recurren nasal p erygium has resul ed in signi can symblepharon orma ion and res ric ion o ocular mo ili y. Pseudopterygium. E. T is large nasal pseudop erygium developed as par o he healing process a er an episode o peripheral ulcera ive kera i is. Some residual peripheral scarring is seen in ero emporally. Fuchs’ marginal keratitis. F. T is eye has a his ory o unila eral chronic nasal peripheral corneal inf amma ion, hinning, and neovasculariza ion due o Fuchs’ marginal kera i is. Six years prior, i had developed a small Seidel-posi ive leak, which resolved wi h medical rea men . T e eye has remained quie on low-dose opical s eroids. No e he severe corneal hinning in eronasally.

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

OTHER CONJUNCTIVAL DEGENERATIONS AMYLOIDOSIS Amyloidosis is a degenera ive condi ion in which he noncollagenous pro ein amyloid is deposi ed in he conjunc iva ( Fig. 2-2A and B). I may be primary or secondary. I may be localized o he conjunc iva or be rela ed o a sys emic disorder such as amyloidosis, plasma cell dyscrasias, or, rarely, lymphoma. Primary localized amyloidosis is he mos common orm. Primary sys emic amyloidosis involves amyloid deposi ion hroughou he eye and eyelids and can a ec he hear and kidneys.

Rule ou sys emic amyloid condi ions.

CALCIUM CONC ETIONS Calcium concre ions are yellow-whi e calcium deposi s ha are embedded in he upper and/ or lower palpebral conjunc iva. Generally hey are loca ed below he surace o he conjunc iva and do no cause any symp oms. Occasionally, he concre ions erode hrough he sur ace o he conjunciva, s ain wi h uorescein dye, and cause oreign-body symp oms ( Fig. 2-2C). I hey are mild, he symp oms can be rea ed wi h opical lubrica ion; i hey are severe, he concre ions can be removed, bu hey of en recur.

A FIGURE 2-2. Conjunctival amyloidosis. A. An eleva ed yellow-colored conjunc ival mass is no ed in his elderly pa ien . I was mobile over he sclera, and i did no have he classic appearance o a p erygium or he papilloma ous pat ern o a squamous cell umor. Conjunc ival biopsy revealed amyloidosis. T ere is o en associa ed subconjunc ival hemorrhage, as seen in his case. ( continued)

Other Conjunctival Degenerations

47

B

C FIGURE 2-2. ( Continued) Conjunctival amyloidosis. B. A creamy whi e, rubbery conjunc ival lesion covered he superior and nasal bulbar conjunc iva o his le eye. Conjunc ival biopsy revealed amyloidosis. Conjunctival calcium concretions. C. Mul iple whi e calcium concre ions have eroded hrough he conjunc ival epi helial sur ace o he upper eyelid, resul ing in f uorescein s aining, seen upon eyelid eversion. T ese exposed concre ions cause a chronic oreign-body sensa ion and require removal.

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

MELANO CYTIC CONJUNCTIVAL LESIONS CONJUNCTIVAL EPITHELIAL MELANOSIS (R CIAL MELANOSIS) Common in pigmen ed races, usually bila eral, bu may have asymme ric ocular involvemen Becomes more pronounced during puber y. Fla , pa chy, brownish pigmen a ion sca ered over he conjunc iva, bu requen ly involves he perilimbal regions ( Fig. 2-3A). Mobile over he sclera. May be per ora ed by an erior ciliary ar eries or nerves. No malignan po en ial.

OCULODE MAL MELANOSIS (NEVUS OF OTA) A congeni al condi ion charac erized by blue-gray hyperpigmen a ion o skin and mucous membranes in he dis ribu ion o he f h cranial nerve Almos always unila eral. T ree varian s are seen: dermal, ocular, and oculodermal melanoses. Involves he dermis o he skin and episclera o he eye, hus he lesion does no move over he sclera. May a ec ipsila eral uveal issues, orbi , and cen ral nervous sys em. Malignan rans orma ion, uveal melanoma, and glaucoma can develop, and pa ien s should be ollowed up regularly.

NEVUS Develops during puber y or early adul hood. Mos are subepi helial or compound nevi.

Appears as a well-demarca ed, a or sligh ly eleva ed lesion, usually in he in erpalpebral areas. I is usually soli ary, and has a predilec ion or he limbus, plica, caruncle, and eyelid margin. Cys ic spaces wi hin he nevus are common and are he key o diagnosis. T e degree o pigmen a ion may vary and may increase a puber y ( Fig. 2-3B). Enlargemen can occur bu may be a sign o malignan rans orma ion. Nevi involving he cornea, arsal, or orniceal conjunc ivae are ex remely rare and should be excised or his opa hologic evalua ion. Periodic pho ographic documen a ion o he lesion may be help ul or ollow-up.

P IMA YACQUI ED MELANOSIS T is is an uncommon, unila eral, premalignan condi ion ha is usually seen in middleaged o elderly whi e pa ien s. Uni ocal or mul i ocal a pa ches wi h indis inc margins ha may involve any par o he conjunc iva. Cys ic spaces are absen ( Fig. 2-3C). Follow-up wi h clinical documen a ion (e.g., sli -lamp pho ography) should be perormed every 6 mon hs. Malignan change should be suspec ed i he pa ches become nodular. Local wide excision wi h cryo herapy is of en per ormed or suspicious lesions. Pos opera ive opical chemo herapy (e.g., wi h mi omycin C) may be bene cial. Incomple e excision and/ or recurrence is common, requiring more aggressive rea men (e.g., local radia ion herapy or opical chemo herapy). opical mi omycin C has also been used success ully o rea primary acquired melanosis wi hou wide excision.

Melanocytic Conjunctival Lesions

SECONDA YACQUI ED MELANOSIS Causes include: Adrenochrome deposi s: discre e clumps o melanin on arsal and orniceal conjunc iva associa ed wi h long- erm use o opical epinephrine—becoming rare Alkap onuria: in erpalpebral, bluishgray or black pigmen a ion o he conjunc iva, episclera, sclera, and endons o horizon al rec us muscles due o accumulaion o homogen isic acid Mascara deposi s Age-rela ed Addison disease Hemochroma osis Argyrosis: As a resul o long- erm use o drops con aining silver, becoming rare Dark oreign bodies

49

MALIGNANT MELANOMA Malignan melanoma is an uncommon, malignan umor ha may be pigmen ed or nonpigmen ed. I may arise de novo, rom preexis ing primary acquired melanosis, or rom a nevus. Eleva ed nodule ha can a ec any par o he conjunc iva, bu has a predilec ion or he limbus and may ex end on o he cornea. Feeder vessels may be seen ( Fig. 2-3D and E). Advanced melanomas may invade he eyelids and orbi . rea men is local excision wi h cryoherapy. Local radia ion herapy may also be bene cial. Exen era ion may be necessary or orbi al involvemen . Use pallia ion wi h chemo herapy i me as asis is presen (lymph nodes, cen ral nervous sys em, liver, e c.).

A FIGURE 2-3. Conjunctival epithelial (racial) melanosis. A. An area o poorly demarca ed conjunc ival epi helial melanin pigmen is seen in his A rican American pa ien . T ese lesions have minimal o no malignan po en ial. ( continued)

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

B

C FIGURE 2-3. ( Continued) Conjunctival nevus. B. A pigmen ed pa ch can be seen in he superior conjunc iva o his A rican American woman. I is well demarca ed, has no changed in size, and has numerous microcys s, all poin ing o he diagnosis o a nevus. Primar y acquired melanosis. C. An area o f a conjunc ival pigmen a ion is seen a he limbus rom he 3 o 5 o’clock posi ions. T ere is a mild increase in vasculariza ion. T is lesion is suspicious or malignan rans orma ion. ( continued)

Melanocytic Conjunctival Lesions

51

D

E FIGURE 2-3. ( Continued) Malignant melanoma o the conjunctiva. D. Biopsy o his large, solid conjunc ival mass revealed malignan melanoma. I is rela ively amelano ic, bu pigmen ed areas can be seen a he 3 and 9 o’clock aspec s o he lesion. T ere is also signi can surrounding vasculariza ion, indica ing an aggressive process. E. A small recurren conjunc ival malignan melanoma is seen a he limbus a he 5 o’clock posi ion a er surgical excision. I was reexcised and rea ed wi h a radioac ive plaque.

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

BENIGN AMELANO CYTIC CONJUNCTIVAL LESIONS GR NULOMAS Chalazion: Single nodule on he arsal conjunc iva (see Fig. 1-2A and B) Pyogenic granuloma: vascularized nodule(s) o he bulbar or palpebral conjunciva. T ey mos commonly occur af er conjunc ival injury such as surgery or a chalazion ( Fig. 2-4A). T ey may be rela ed o a oreign body, such as a punc al plug ( Fig. 2-4B). Sarcoidosis: mul iple yellow-colored nodules involving he arsal or orniceal conjunc iva ( Fig. 2-4C). When hese are presen , biopsy can con rm he diagnosis o sarcoidosis. Rhinosporidiosis: very rare ungal in ecion ha may cause conjunc ival granulomas Vasculi ides (e.g., polyar eri is nodosa, Churg-S rauss syndrome): very riable lesions

EPIBULBA DE MOID Epibulbar dermoid is an uncommon, congeni al lesion ha may occur in isola ion or in associa ion wi h o her ocular or sys emic anomalies. I may be unila eral or bila eral. Solid, smoo h, round whi e mass ypically loca ed a he in ero emporal limbus, bu may be elsewhere, even he cen ral cornea. Lesions may encroach on o he cornea, causing as igma ism. May have hair ollicles ( Fig. 2-4D, E, and H) Surgical resec ion may resul in corneoscleral hinning and may have o be combined wi h a corneal lamellar pa ch graf or, rarely, a pene ra ing graf ( Fig. 2-4F and G). Ul rasound biomicroscopy may be

help ul in de ermining he dep h o he lesion preopera ively. Ocular associa ions: Eyelid coloboma, ocular coloboma Sys emic associa ions: Goldenhar syndrome, mos common, of en bila eral dermoids; reacher Collins syndrome; Franceschet i syndrome

LIPODE MOID A lipodermoid is an uncommon and of en bila eral condi ion, ypically ound in adul s. Large, yellow, sof , movable, subconjuncival lesions consis ing o adipose and dermal issues mos commonly loca ed supero emporally. Hair ollicles may be seen on he surace. T e lesions can ex end in o he superior ornices, where i is impossible o visualize heir pos erior limi s. Comple e surgical excision is unnecessary and should be avoided o preven damage o he rec us muscle, lacrimal gland, and he leva or muscle.

HE EDITA YBENIGN INTR EPITHELIAL DYS ER TOSIS Heredi ary benign in raepi helial dyskera osis (HBID) is a rare disorder charac erized by marked conjunc ival and episcleral vessel injecion wi h overlying whi e plaques o acan ho ic and dyskera o ic epi helial cells ( Fig. 2-4I). I is loca ed primarily in he nasal and emporal in erpalpebral zones. I is mos commonly ound in members o he Haliwa Indian ribes o Nor h Carolina. No good rea men exis s curren ly, al hough occasionally a conjunc ival biopsy may be required o rule ou a conjunc ival umor.

Benign Amelanocytic Conjunctival Lesions

53

A

B FIGURE 2-4. Pyogenic granuloma. A.T is large collec ion o granula ion issue occurred as an inf amma ory response a er an in erior eyelid chalazion resolved. B. A small pyogenic granuloma developed in his punc um, rela ed o a silicone punc al plug. I resolved once he plug was removed. ( continued)

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

C

D FIGURE 2-4. ( Continued) Sarcoid granulomas. C. Mul iple yellowish nodules are seen in he upper bulbar conjunc iva in his pa ien wi h sarcoidosis. In pa ien s wi h suspec ed sarcoidosis and a conjunc ival nodule, he diagnosis o sarcoidosis can o en be con rmed wi h a simple conjunc ival biopsy. Epibulbar dermoid. D. An in eronasal dermoid can be seen in his 7-year-old girl. Al hough her uncorrec ed vision was 20/ 20, she and her paren s were unhappy wi h i s cosme ic appearance. ( continued)

Benign Amelanocytic Conjunctival Lesions

55

E

F FIGURE 2-4. ( Continued) Epibulbar dermoid. E. T is 6-year-old boy was essen ially asymp oma ic rom his in ero emporal dermoid. T ere are several modera ely large cilia pro ruding rom he cen er. F. T is 12-yearold girl was becoming very unhappy wi h he cosme ic appearance o his dermoid, which was subsequen ly removed. ( continued)

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

G

H FIGURE 2-4. ( Continued) Epibulbar dermoid. G. A 2 years a er excision o he dermoid wi h a lamellar corneal gra , he pa ien seen in F had an excellen cosme ic resul . H.T is in ero emporal dermoid encroaches on he cornea wi h secondary corneal scarring. ( continued)

Benign Amelanocytic Conjunctival Lesions

57

I FIGURE 2-4. ( Continued) Hereditar y benign intraepithelial dyskeratosis (HBID). I. Prominen conjunc ival and episcleral injec ion wi h an overlying eleva ed whi e plaque is seen emporally in his righ eye wi h HBID. T e pa ien was qui e pho ophobic.

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

POTENTIALLY MALIGNANT AMELANO CYTIC CONJUNCTIVAL LESIONS SQUAMOUS PAPILLOMA Peduncula ed squamous papilloma is an uncommon, benign umor caused by he human papillomavirus. I ypically occurs in children and young adul s. Papillomas have ngerlike projec ions and are loca ed in he palpebral conjunc iva, ornix, or caruncle ( Fig. 2-5A). T ey may be mul i ocal or bila eral. T ey of en resolve on heir own. T ey can be excised when hey are large or chronic, bu hey may recur, occasionally worse han he original lesion. Applica ion o cryo herapy a he base o he lesion af er excision may decrease he risk o recurrence. Oral cime idine has been repor ed o be e ec ive. A nonviral, sessile orm ha occurs in he elderly and involves he perilimbal conjunciva should be considered precancerous or cancerous and should be comple ely excised wi h wide margins and supplemen al cryoherapy and sen or his opa hologic evaluaion. Al erna ive rea men includes opical or subconjunc ival in er eron alpha 2b, generally af er biopsy con rma ion.

CONJUNCTIVAL INTR EPITHELIAL NEOPLASIA Conjunc ival in raepi helial neoplasia is a unila eral, premalignan condi ion ha is seen in older, air-skinned individuals. T is condi ion was ormerly re erred o as Bowen’s disease, in raepi helial epi helioma, and conjunc ival dyskera osis. T e lesions are usually loca ed a he limbus and may involve adjacen cornea ( Fig. 2-5B–D). T e hree clinical ypes are

(1) a eshy gela inous lesion wi h variable kera iniza ion, (2) a whi e plaque (leukoplakic ype), and (3) papillary. rea men is mos commonly comple e surgical excision wi h supplemen al cryo herapy. Success ul rea men has also been achieved wi h opical and subconjunc ival in er eron alpha 2b.

SQUAMOUS CELL CA CINOMA Invasive squamous cell carcinoma is a rare, slow-growing, locally invasive umor ha occurs mos requen ly a he limbus. I mos likely progresses rom conjunc ival in raepi helial neoplasia ha breaks hrough he conjunc ival basemen membrane. Papillary or gela inous umor. Frequen ly associa ed wi h eeder blood vessels ( Fig. 2-5E) rea men is mos commonly comple e surgical excision wi h supplemen al cryo herapy. A lamellar sclerec omy may be required o excise he lesion comple ely. Success ul rea men has also been achieved wi h opical and subconjunc ival in er eron alpha 2b. May be aggressive in immunocompromised pa ien s.

OTHE CA CINOMAS Mucoepidermoid carcinoma and spindle cell carcinoma are similar o squamous cell carcinoma bu are more aggressive, and hey may arise anywhere on he conjunc iva. Sebaceous carcinoma is an uncommon and aggressive umor ha ypically involves he upper eyelid o elderly pa ien s, bu may rarely arise de novo rom he arsal conjunciva as a papilloma ous or plaquelike lesion ( Fig. 2-5F). May masquerade as chronic unila eral conjunc ivi is or recurren chalazia.

Potentially Malignant Amelanocytic Conjunctival Lesions

May require mul iple biopsies. May have page oid spread wi h skip areas.

EACTIVE LYMPHOID HYPE PLASIA AND NON-HODG IN LYMPHOMA T e appearance o he wo condi ions is similar. Smoo h, eshy, subconjunc ival mass ha may involve a large area ( Fig. 2-5G–I). T e lesions may be single or mul iple, and hey

59

involve bo h eyes in abou 20% o cases. Small a ec ed areas are called “salmon pa ches,” and hey occur mos commonly in he bulbar or orniceal conjunc iva. Incisional or excisional biopsy should be per ormed and sen or immunohis ochemical s udies (which may require non xed issue). A sys emic evalua ion should be per ormed by an in ernis or oncologis .

A FIGURE 2-5. Conjunctival papilloma. A.T is squamous papilloma in a pedia ric pa ien is likely o viral origin. I had numerous papillary vascular ronds at ached o a s alk ( peduncula ed) . ( continued)

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

B

C FIGURE 2-5. ( Continued) Conjunctival intraepithelial neoplasia. B. No e he large, well-demarca ed, sessile papilloma ous lesion adjacen o he limbus rom he 6 o he 8 o’clock posi ion. Excisional biopsy revealed conjunc ival in raepi helial neoplasia. C. T is f eshy, sessile, mildly eleva ed mass lesion has a kera inized, leukoplakic componen . Excisional biopsy revealed conjunc ival in raepi helial neoplasia. ( continued)

Potentially Malignant Amelanocytic Conjunctival Lesions

61

D

E FIGURE 2-5. ( Continued) Conjunctival intraepithelial neoplasia. D.T is HIV-posi ive pa ien had a small limbal conjunc ival lesion in eriorly. T ickened, gray-whi e ronds and shee s o abnormal epi helium are indica ive o ex ensive corneal invasion rom he 3 o he 12 o’clock posi ions. Squamous cell carcinoma. E. A large, eleva ed conjunc ival mass is seen a he superior limbus. T e mass has vascularized papilloma ous ronds. T e lesion ex ends on o and covers mos o he cornea. Conjunc ival biopsy revealed squamous cell carcinoma. ( continued)

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

F

G FIGURE 2-5. ( Continued) Sebaceous carcinoma. F.T e righ upper eyelid is ever ed in his elderly pa ien wi h a chronic unila eral blepharoconjunc ivi is demons ra ing a di use hickened papilloma ous conjunc ival sur ace. Eyelid biopsy revealed sebaceous carcinoma. Conjunctival lymphoma. G. Mul iple eleva ed pinkish nodules are presen in he lower palpebral conjunc iva o his le eye. Similar nodules were seen in he righ eye. Biopsy revealed conjunc ival lymphoma. ( continued)

Potentially Malignant Amelanocytic Conjunctival Lesions

63

H

I FIGURE 2-5. ( Continued) Conjunctival lymphoma. H.T is f eshy, salmon-colored mass adjacen o he caruncle was a conjunc ival lymphoma. I was rea ed wi h surgical excision and local radia ion herapy, as workup did no reveal evidence o sys emic involvemen . I. A large f eshy, well-delinea ed, sligh ly nodular conjunc ival mass can be seen occupying he en ire in erior ornix o his le eye; biopsy was posi ive or lymphoma.

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

CYSTIC LESIONS P IMA YCONJUNCTIVAL CYST A common, ranslucen cys con aining clear uid on he bulbar conjunc iva. May be at ached o he conjunc iva or reely mobile under he conjunc iva ( Fig. 2-6A–C). May cause a oreign-body sensa ion. Di eren ial diagnosis includes conjuncival lymphangiocele, which is of en more or uous. rea men is usually unnecessary. I i is symp oma ic, he cys should be comple ely excised by shelling i ou rom under he

conjunc iva. Punc uring he cys wi h a needle or incomple e excision ypically resul s in recurrence.

IAT OGENIC CYSTS T ese cys s may ake he ollowing orms: Secondary implan a ion cys s ollowing surgery or rauma. Drainage bleb ollowing l ra ion surgery or blebs ha may be a and di use or localized af er ca arac surgery ( Fig. 2-6D). enon’s cys associa ed wi h a l ra ion bleb, charac erized by an eleva ed cys like cavi y wi h engorged sur ace vessels.

Cystic Lesions

65

A

B FIGURE 2-6. Conjunctival cyst. A. A large, mobile conjunc ival cys is seen near he limbus. I was big enough o cause chronic irri a ive symp oms. T ese can o en be removed in o o by care ully incising he conjunc iva and gen ly shelling ou he cys . B. A large mobile nasal conjunc ival cys covers he caruncle nasally in his righ eye. ( continued)

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2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

C

D FIGURE 2-6. ( Continued) Conjunctival cyst. C.T e same cys as in B can be seen o be pro ruding rom he palpebral ssure when he eye is closed. T is caused drying and kera iniza ion o he sur ace o he cys . T e en ire cys and i s kera inized sur ace were excised and did no recur. D. T is nonmobile conjunc ival cys was probably rela ed o superior limbal surgery. A nylon su ure can be seen a he base o he cys .

Vascular Lesions

VASCULAR LESIONS TELANGIECTASIAS T e ollowing me abolic disorders may rarely be associa ed wi h dila ed and or uous blood vessels o he bulbar conjunc iva: Diabe es melli us Fabry disease: Frequen ly associa ed wi h small aneurysm orma ion O her me abolic disorders (e.g., ucosidosis, GM1 gangliosidosis) Mul iple endocrine neoplasia IIb: associa ed wi h prominen paralimbal nerve bundles

HEMATOLOGIC DISO DE S T e ollowing hema ologic disorders may be associa ed wi h sludging o he blood: Dyspro einemias (e.g., mul iple myeloma) Sickle-cell anemia: isola ed, corkscrewor comma-shaped vessels Polycy hemia vera

HEMO HAGIC LYMPHANGIECTASIA Hemorrhagic lymphangiec asia is a rare condi ion ha may occur af er mild in amma ion or rauma. I may also be associa ed wi h vascular mal orma ions o he eyelid and paro id gland. Dila ed and or uous bulbar lympha ic vessels may become lled wi h blood i hey communica e wi h conjunc ival veins.

CAPILLA YHEMANGIOMA Capillary hemangioma is an uncommon umor ha may be associa ed wi h hemangiomas o he eyelids and orbi . Brigh red lesion ha blanches wi h pressure. I may bleed spon aneously or ollowing minor rauma.

67

LYMPHANGIOMA Lymphangioma is a rare umor ha may be associa ed wi h similar lesions o he orbi , ace, sinuses, and oropharynx. Brigh red lesion which is similar o, bu may be larger han, a hemangioma

K POSI’S SA COMA Kaposi’s sarcoma, occurring mos commonly on he skin, including he eyelids and occasionally on he conjunc iva, may be seen in AIDS pa ien s. A reddish vascular conjunc ival lesion ha may be di use or nodular (see Fig. 7-20B). A di use umor may resemble a subconjunc ival hemorrhage upon cursory examina ion. No speci c rea men is required. I severe, and sys emic AIDS rea men and chemoherapy have been maximized, local excision, cryo herapy, or radia ion can be used or ocular Kaposi’s sarcoma.

STU GE-WEBE SYND OME (ENCEPHALOT IGEMINAL ANGIOMATOSIS) Localized elangiec asias, probably associa ed wi h episcleral hemangiomas ( Fig. 2-7A) Associa ed wi h glaucoma, iris hyperchromia, and di use choroidal hemangioma.

CA OTID–CAVE NOUS SINUS AND DUR L-SINUS FISTULAS T ere are wo ypes o ar eriovenous s ulas ha a ec he eye. T ey produce reversal o ow hrough he superior oph halmic vein ha can be seen on color Doppler s udies and dila ion o he superior oph halmic vein ha can be seen on compu ed omography or magne ic resonance imaging. A caro id–cavernous sinus s ula is a high- ow communica ion be ween he in ernal caro id ar ery and he cavernous sinus.

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I mos commonly occurs af er rauma or surgery bu can arise spon aneously. I can produce severe conjunc ival vessel engorgemen and chemosis, eyelid swelling, pulsa ing exoph halmos, eleva ed in raocular pressure, and an orbi al brui . A dural-sinus s ula is a low- ow communica ion be ween he meningeal branches o he caro id ar ery and he cavernous sinus. T ey occur spon aneously, mos commonly in middle-aged and elderly women. T e clinical ndings are much milder han in caro id–cavernous sinus s ulas, al hough he

in raocular pressure can be qui e eleva ed. T e chronic red eye is of en mis aken or chronic conjunc ivi is ( Fig. 2-7B and C). Bo h orms can cause ar erializa ion o he conjunc ival blood vessels resul ing in he charac eris ic corkscrew pat ern. rea men is wi h closure o he s ula hrough an endoar erial balloon emboliza ion or surgery, al hough dural-sinus s ulas may close spon aneously or af er angiography. Signi can ly eleva ed in raocular pressure needs o be addressed.

A FIGURE 2-7. Sturge Weber syndrome. A. Prominen episcleral vessels are seen superiorly in his pa ien wi h S urge-Weber syndrome. Eleva ed episcleral venous pressure can cause glaucoma. ( continued)

Vascular Lesions

69

B

C FIGURE 2-7. ( Continued) Cavernous sinus f stula. B. T is pa ien had a low f ow dural-sinus s ula. No e he engorged corkscrew episcleral vessels. T e in raocular pressure was modera ely eleva ed. C. Mul iple prominen dila ed corkscrew episcleral vessels are seen in his eye wi h a low f ow dural-sinus s ula. (Cour esy o Wee-Jin Heng, MD.)

C H AP T ER

An erior Segmen Developmen al Anomalies ANOMALIES OF CORNEAL SIZE AND SH APE MIC OCO NEA

M

icrocornea is an uncommon congeni al unila eral or bila eral condiion. Inheri ance is au osomal dominan or recessive. Signs In an horizon al corneal diame er less han 10 mm; adul horizon al corneal diame er less han 11 mm ( Fig. 3-1)

Shallow an erior chamber, angle-closure or open-angle glaucoma, corneal f at ening, and hyperopia May have associa ed nanoph halmos ( Table 3-1) O her ocular dimensions are normal. Treatment Manage re rac ive error and search or o her ocular and sys emic anomalies. Prognosis Varies depending on associa ed ocular and sys emic abnormali ies

TABLE 3-1. Associa ion o Microcornea Ocular • Anterior segment dysgeneses • Congenital cataract • Congenital glaucoma • Corneal leukoma • Cornea plana • Hyperopia • Microphakia • Uveal coloboma

70

Systemic Syndromes • Cornelia de Lange’s • Ehlers-Danlos • Nance-Horan • Trisomy 13, 18, 21 • Turner’s • Waardenburg’s • Weill-Marchesani

Anomalies of Corneal Size and Shape

71

FIGURE 3-1. Microcornea.T is cornea measured 8.5 o 9.0 mm in diame er. O herwise he eye was essen ially normal.

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3 ANTERIO R SEGMENT DEVELO PMENTAL ANO MALIES

MEGALOCO NEA

M

egalocornea is an uncommon congenial, bila eral condi ion ha is usually inheri ed in an X-linked recessive manner and is here ore ound mos ly in males. Signs Clear cornea wi h a horizon al diame er o grea er han 12 mm in he neona e and 13 mm in adul s ( Fig. 3-2) Very deep an erior chamber Normal in raocular pressure Corneal s eepening, high myopia, and as igma ism, bu good visual acui y

Lens subluxa ion may occur as a resul o zonular s re ching. May develop glaucoma secondary o angle abnormali ies Treatment Manage re rac ive error and search or o her ocular and sys emic anomalies, especially glaucoma and lens abnormali ies. Prognosis Generally good, bu depends on associa ed ocular and sys emic abnormali ies ( Table 3-2) TABLE 3-2. Associa ions o Megalocornea Ocular • Astigmatism • Axenfeld-Rieger anomaly • Cataract • Congenital glaucoma • Ectopia lentis • Myopia

Systemic Syndromes • Albinism • Alport’s • Apert’s • Craniosynostosis • Down’s • Ehlers-Danlos • Marfan’s • Osteogenesis imperfecta • Progressive facial hemiatrophy

Anomalies of Corneal Size and Shape

73

FIGURE 3-2. Megalocornea. T is cornea measured 14 mm in diame er. T e cornea is clear excep or some calcif c degenera ion nasally and emporally.

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3 ANTERIO R SEGMENT DEVELO PMENTAL ANO MALIES

NANOPHTHALMOS

N

anoph halmos is an uncommon, congeni al, bila eral condi ion in which he globe has reduced volume bu is o herwise grossly normal. Signs Very high hyperopia (e.g., +12D o +15D) Adul corneal diame er is reduced, bu he lens has a normal volume. Shor axial leng h (e.g., 16–18 mm) Shallow an erior chamber T ick sclera Fundus may show a crowded disc, vascular or uosi y, and macular hypoplasia

Associated Problems Angle-closure glaucoma Uveal e usion Re inal de achmen Poorly olera ed in raocular surgery

MIC OPHTHALMOS

M

icroph halmos is an uncommon unila eral or bila eral condi ion in which he axial leng h o he eye is reduced and he eye is mal ormed ( Fig. 3-3). T e e ec s on vision depend on i s severi y and he presence o associa ed anomalies. T ere are wo ypes o microph halmos: noncoloboma ous and coloboma ous ( Table 3-3). TABLE 3-3. ypes o Microph halmos Noncolobomatous

Colobomatous

Isolated • Sporadic • Inherited (dominant, recessive, X-linked recessive)

Isolated • Sporadic • Inherited (dominant)

With anterior persistent hyperplastic primary vitreous

With systemic syndromes: • Patau’s (trisomy 13) • Edward’s (trisomy 18) • Cat-eye (partial trisomy 22) • CHARGE • Meckel • Lenz microphthalmia

Intrauterine infections (rubella, toxoplasmosis, cytomegalovirus, varicella)

CHARGE syndrome is coloboma, heart anomaly, choanal atresia, retardation, and genital or ear anomalies.

Anomalies of Corneal Size and Shape

75

FIGURE 3-3. Microphthalmos. T is microph halmic eye has a small cornea, abnormal iris, and overall small size. (Cour esy o Pe er Laibson, MD.)

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3 ANTERIO R SEGMENT DEVELO PMENTAL ANO MALIES

BUPHTHALMOS

B

uph halmos is an uncommon, usually bila eral condi ion in which he globe is enlarged as a resul o s re ching o he cornea and sclera because o increased in raocular pressure be ore bir h or during he rs 3 years o li e. Signs Large cornea wi h variable scarring; may develop corneal edema la er in li e Horizon al or curvilinear rup ures in Desceme ’s membrane (Haab’s s riae) ( Fig. 3-4) Very deep an erior chamber Angle anomalies Myopia Op ic disc cupping Associations o In antile Glaucoma Ocular Aniridia An erior segmen dysgeneses Congeni al ec ropion uveae

Sys emic Down’s syndrome Lowe’s syndrome Mucopolysaccharidoses Neuro broma osis ype 1 Nevus o O a Pa au’s syndrome ( risomy 13) Pierre Robin’s syndrome Rieger’s syndrome S urge-Weber syndrome Treatment Managemen o glaucoma by a glaucoma specialis Prognosis Guarded, depending on amoun o op ic nerve damage prior o diagnosis, e cacy o rea men , and associa ed ocular and sys emic disorders. Haab’s s riae o he cornea do no preven good vision.

Anomalies of Corneal Size and Shape

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A

B FIGURE 3-4. Haab’s striae. A.T ese breaks in Desceme ’s membrane occurred secondary o congeni al glaucoma. No e he mul iple parallel swirling lines, which are rolled-up edges o Desceme ’s membrane. B. Mul iple parallel swirling lines are eviden in his eye wi h congeni al glaucoma.

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3 ANTERIO R SEGMENT DEVELO PMENTAL ANO MALIES

CONGENITAL ANTE IO STAPHYLOMA/ KER TECTASIA

C

ongeni al an erior s aphyloma and keraec asia are ex remely rare, congeni al, usually unila eral condi ions resul ing in severe corneal pro rusion and occasionally per oraion ( Fig. 3-5). Etiology I is probably due o in rau erine kera i is. Signs Severe corneal opaci ca ion and pro rusion o corneal issue beyond he plane o he eyelids.

Endo helium, Desceme ’s, and pos erior corneal issue are absen . I may be lined by uveal issue pos eriorly. Treatment A pene ra ing kera oplas y or an erior segmen ransplan can be at emp ed in bila eral cases, bu he success ra e is ex remely poor. Mos eyes will undergo an enuclea ion. Prognosis Poor

FIGURE 3-5. Keratectasia. Gross specimen a er enuclea ion o an eye wi h a large corneal s aphyloma a er suspec ed in rau erine in ec ion. No e he massive pro rusion an erior o he corneal limbus. (Cour esy o Pe er Laibson, MD.)

Anomalies of Corneal Size and Shape

SCLE OCO NEA

S

clerocornea is a rare, congeni al, usually bila eral bu o en asymme ric, nonprogressive, noninf amma ory condi ion. I can be par ial or comple e. Etiology Unknown Mos cases are sporadic.

Signs Opaci ca ion and vasculariza ion o he peripheral or en ire cornea. I only he peripheral cornea is involved, he resul ing “scleraliza ion” makes he cornea appear smaller han normal ( Fig. 3-6). O en associa ed wi h cornea plana May have associa ed glaucoma

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Ocular Associations An erior segmen dysgeneses Blue sclera Congeni al ca arac Cornea plana Glaucoma Iris abnormali ies (e.g., aniridia, coloboma) Microph halmos Treatment I unila eral, may op o ollow he eye. I bila eral, consider a pene ra ing kera oplas y or kera opros hesis. Prognosis Pene ra ing kera oplas y has a relaively poor prognosis in sclerocornea. Kera opros hesis surgery in in an s is a recen developmen and has a guarded prognosis.

A FIGURE 3-6. Peripheral sclerocornea. A.T e en ire corneal periphery, bu especially he superior and in erior cornea, are scleralized. ( continued)

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3 ANTERIO R SEGMENT DEVELO PMENTAL ANO MALIES

B

C FIGURE 3-6. ( Continued) Peripheral sclerocornea. B.T e superior, nasal, and in erior peripheral cornea are vascularized in his eye wi h ex ensive peripheral sclerocornea. Total sclerocornea. C.T e en ire cornea is scleralized. T ere is a sligh ly less opaque area cen rally, which urned ou o be he cen ral cornea during pene ra ing kera oplas y.

Anomalies of Corneal Size and Shape

CO NEA PLANA

C

ornea plana is a rare, congeni al, bila eral condi ion. Many physicians consider i a mild orm o sclerocornea. Etiology Unknown

Symptoms None or poor vision Signs Hyperopia Severely f a corneal curva ure, where he sclera and cornea have he same curva ure ( Fig. 3-7) Shallow an erior chamber Glaucoma

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Ocular Associations Aniridia An erior segmen dysgeneses Microcornea Microph halmos Sclerocornea Treatment Correc re rac ive error. Con ac lenses can be di cul o due o f a ness o he cornea and he lack o di erence be ween he corneal and scleral curva ure. A scleral lens may be bene cial. Prognosis Good

FIGURE 3-7. Peripheral sclerocornea/ cornea plana. A sli -beam view o he eye seen in Figure 3 6A demons ra es he lack o change in corneal curva ure be ween he sclera and he cornea. T e cornea had a a corneal curva ure and he eye was hyperopic.

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ANTERIOR SEGMENT DYSGENESES POSTE IO EMB YOTOXON Pos erior embryo oxon is a creamy-whi e, hickened, an eriorly displaced Schwalbe’s line seen in he corneal periphery ( Fig. 3-8A). I is ypically a bila eral condi ion ha occurs o some ex en in approxima ely 15% o normals. I is presen in all pa ien s wi h Axen eld’s and Rieger’s anomaly.

AXENFELD’S ANOMALY Axen eld’s anomaly is a rare, bila eral, au osomal dominan or sporadic anomaly. I is charac erized by s rands o iris ha span across he angle and at ach o a pos erior embryo oxon ( Fig. 3-8B). Glaucoma develops in 50%o cases, and he disorder is hen known as Axen eld’s syndrome.

IEGE ’S ANOMALY Rieger’s anomaly is a rare, bila eral, au osomal dominan or sporadic anomaly. I is charac erized by pos erior embryo oxon wi h adheren iris s rands and iris s romal hypoplasia ( Fig. 3-8C). T ere may be pseudopolycoria, corec opia, and ec ropion uveae. O hose a ec ed, 50% o pa ien s develop glaucoma.

IEGE ’S SYND OME Au osomal dominan rai . Usually bila eral bu asymme ric. Consis s o Rieger’s anomaly plus den al (hypodon ia, microdon ia) or acial (maxillary hypoplasia, elecan hus, hyper elorism) mal orma ions. O hose a ec ed, 50% o pa ien s develop glaucoma.

PETE S’ ANOMALY Sporadic inheri ance, al hough au osomal recessive and dominan pat erns have been repor ed.

Bila eral in 80% o cases bu o en asymme ric. Cen ral corneal opaci y, pos erior Desceme ’s membrane or s romal de ec , o en iridocorneal adhesions, possible lens– cornea adhesion and shallow an erior chamber ( Fig. 3-8D and E). May be associa ed wi h lens displacemen and ca arac O hose a ec ed, 50% o pa ien s develop glaucoma. May have associa ed sys emic abnormaliies (e.g., skele al, den al)

LOCALIZED POSTE IO KER TOCONUS Very rare, sporadic, usually unila eral developmen al anomaly presen ing a bir h Nonprogressive pro rusion o cen ral area o he pos erior corneal sur ace ( Fig. 3-8F) May have a mild corneal scar Myopic as igma ism can occur. Diagnosis o Anterior Segment Dysgeneses Family his ory or similar condi ion and sys emic his ory or associa ed anomalies Examine under anes hesia i unable o do an adequa e evalua ion in he o ce, including a sli -lamp examina ion, measuremen o corneal diame er, in raocular pressure, gonioscopy, ophhalmoscopy, and re inoscopy. Ul rasound biomicroscopy and op ical coherence omography can be help ul in imaging he an erior segmen , A-scan ul rasonography is help ul o measure he axial leng h o moni or or glaucoma, and B-scan ul rasonography can be used o image he pos erior segmen when necessary. Treatment o Anterior Segment Dysgeneses Visual rehabili a ion: correc re rac ive errors, rea amblyopia, con rol glaucoma wi h medica ions or surgery, ca arac ex rac ion and corneal ransplan a ion as necessary. May need combined e or s o specialis s in cornea, glaucoma, re ina, and pedia ric oph halmology

Anterior Segment Dysgeneses

Re er o a pedia rician or managemen o sys emic abnormali ies. Chromosomal analysis and gene ic counseling Prognosis or Anterior Segment Dysgeneses Excellen or pos erior embryo oxon and localized pos erior kera oconus; air

83

o good or Axen eld’s and Rieger’s anomalies; guarded or Pe ers’ anomaly. he prognosis depends grea ly on he severi y o he glaucoma. Eyes wi h Pe ers’ anomaly can do well wi h corneal ransplan a ion; he success ra e is worse when he lens is involved.

A

B FIGURE 3-8. Posterior embryotoxon. A. A prominen Schwalbe’s line can be seen rom he 7 o he 9 o’clock posi ions. (Cour esy o Irving Raber, MD.) Axenfeld’s anomaly. B. A prominen Schwalbe’s ring wi h iris adhesions can be seen in eriorly in his eye wi h Axen eld’s anomaly. (Cour esy o Elisabe h Cohen, MD.) ( continued)

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3 ANTERIO R SEGMENT DEVELO PMENTAL ANO MALIES

C

D FIGURE 3-8. ( Continued) Rieger’s anomaly. C. A prominen Schwalbe’s ring is presen nasally and emporally in his eye wi h Rieger’s anomaly. T ere is iris a rophy wi h mild corec opia emporally. (Cour esy o Pe er Laibson, MD.) Peters’ anomaly. D.T is eye wi h Pe ers’ anomaly has a dense paracen ral corneal opaci y. T ere is a band o iris rom he 3 o’clock pupillary margin o he corneal opaci y. ( continued)

Anterior Segment Dysgeneses

85

E

F FIGURE 3-8. ( Continued) Peters’ anomaly. E. T is dense cen ral corneal opaci y was associa ed wi h iris adhesions rom he pupillary margin o he pos erior aspec o he corneal opaci y in his 4-year-old girl born in China. Her o her eye had a similar condi ion requiring corneal ransplan s in bo h eyes. Posterior keratoconus. F. An inden a ion o he pos erior cornea can be seen cen rally, which is charac eris ic o pos erior kera oconus. T ere is a mild associa ed corneal opaci y. T ere is minimal an erior corneal change, bu here may be as igma ism.

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3 ANTERIO R SEGMENT DEVELO PMENTAL ANO MALIES

ANI IDIA

A

niridia is a rare, bila eral condi ion ha is associa ed wi h glaucoma in 75% o cases. wo- hirds o pa ien s have an au osomal dominan orm ha is no associa ed wi h Wilms’ umor. Approxima ely one- hird o cases are sporadic; 25% o sporadic cases will develop Wilms’ umor. Signs Par ial or essen ially comple e absence o he iris Synechial angle-closure glaucoma occurs in 75% o cases as a resul o pulling orward o rudimen ary iris issue. Associa ed ocular and sys emic disorders Classif cation AN-1: isola ed; au osomal dominan : Vision is poor due o oveal hypoplasia. AN-2: isola ed; au osomal dominan : Vision is normal. AN-3 (Gillespie’s syndrome): au osomal recessive. Men al handicap, cerebellar a axia AN-4 (Miller’s syndrome): dele ion o he shor arm o chromosome 11, sporadic. Wilms’ umor, geni ourinary anomalies, menal re arda ion AN-5: variable inheri ance. Iris hypoplasia wi h o her ocular mal orma ions (e.g., Pe ers’ anomaly, microcornea, congeni al aphakia, ec opia len is) AN-6: variable inheri ance. Iris hypoplasia wi h o her sys emic syndromes (e.g., Biemond’s syndrome, absence o pa ella) Ocular Associations Glaucoma Corneal lesions: pannus, opaci y ( Fig. 3-9A–C), kera olen icular adhesions, microcornea, sclerocornea

Lens and iris changes: congeni al aphakia, an erior polar, pos erior subcapsular ca arac , subluxa ion, ec opia len is, persis en pupillary membranes Fundus lesions: oveal hypoplasia, disc hypoplasia, colobomas Nys agmus Diagnosis Family his ory or similar condi ion and sys emic his ory or associa ed anomalies. Examine under anes hesia i unable o do an adequa e evalua ion in he o ce, including a sli -lamp examina ion, measuremen o corneal diame er and in raocular pressure, gonioscopy, oph halmoscopy, and re inoscopy. Treatment Visual rehabili a ion: Correc re rac ive errors, rea amblyopia, and con rol glaucoma wi h medica ions or surgery. Ca arac ex rac ion and corneal ransplan / limbal s em cell ransplan / kera opros hesis as necessary Chromosomal analysis and gene ic counseling Renal evalua ion by pedia rician or pedia ric oncologis o moni or or Wilms’ umor Prognosis Fair, depending on severi y o glaucoma and corneal abnormali ies. T e limbal s em cell abnormali y o en leads o corneal haze and scarring. Corneal ransplan s o en ail because o limbal s em cell de ciency. Limbal s em cell ransplan a ion wi h chronic sys emic immunosuppression has a reasonably good prognosis. Kera opros hesis surgery also has a reasonably good prognosis.

Anterior Segment Dysgeneses

87

A

B

C FIGURE 3-9. Aniridia. A.T is eye wi h aniridia demons ra es corneal pannus and severe corneal scarring. Al hough here is a hazy view, no iris was seen upon sli -lamp examina ion. B.S ignif can peripheral pannus and di use pa chy an erior s romal corneal haze is presen in his eye wi h aniridia. C. Re roillumina ion o he eye in B reveals a well-posi ioned pos erior chamber in raocular lens implan and perhaps a very small amoun o peripheral iris.

88

3 ANTERIO R SEGMENT DEVELO PMENTAL ANO MALIES

IRIS COLOBOMA

I

ris coloboma is an uncommon, unilateral or bilateral condition caused by de ective closure o the embryonic f ssure, usually in eronasally. Isolated iris colobomas are either sporadic or dominantly inherited.

Signs Atotal coloboma is a segmental absence o iris rom pupil to root, giving rise to a “keyhole” pupil. A partial coloboma does not involve the iris root ( Fig. 3-10). Ocular Associations Colobomas o the ciliary body, lens, retina, choroid, and optic nerve Microphthalmos Systemic Associations Cat-eye syndrome (partial trisomy 22) CHARGE syndrome ( coloboma, heart anomaly, choanal atresia, retardation, and genital or ear anomalies) Edward’s syndrome (trisomy 18)

Patau’s syndrome (trisomy 13) Rubinstein-Taybi syndrome Diagnosis Family history or similar condition and systemic history or associated anomalies. Examine under anesthesia i unable to do an adequate evaluation in the o ce, including a slit-lamp examination, measurement o corneal diameter and intraocular pressure, gonioscopy, ophthalmoscopy, and retinoscopy. Ultrasound biomicroscopy and optical coherence tomography can be help ul in imaging the anterior segment, and B-scan ultrasonography can be used to image the posterior segment when necessary. Treatment Manage re ractive error and search or other anterior and posterior segment anomalies. Chromosomal analysis and genetic counseling Prognosis Depends on severity o the coloboma and on the extent o other ocular and systemic abnormalities

A FIGURE 3-10. Iris coloboma. A. An in erior iris coloboma is present in this newborn with numerous systemic abnormalities. T ere are f ne iris strands in eriorly. T e other eye, which had severe sclerocornea, is seen in Figure 3 6C. ( continued)

Anterior Segment Dysgeneses

89

B

C FIGURE 3-10. ( Continued) Irisc oloboma.B . A large in erior iris coloboma has been covered wi h an iris- in ed so con ac lens. C.T e con ac lens in he eye in B has been moved superiorly wi h a cot on- ipped applica or o reveal he large in erior iris coloboma. T ere is also a cor ical ca arac .

C H AP T ER

Ec a ic Condi ions o he Cornea KERATO CONUS

K

era oconus is a airly common condi ion charac erized by corneal hinning, prorusion, and irregulari y. I is usually bila eral, al hough he severi y o involvemen is o en asymme ric. Etiology Sporadic or au osomal dominan wi h incomple e pene rance Symptoms Gradually decreasing vision, ypically beginning in adolescence and progressing in o adul li e Pa ien s o en rela e a his ory o no being able o at ain good vision despi e mul iple changes o glasses or so con ac lenses. May have a his ory o eye rubbing Can develop acu ely decreased vision and pain due o hydrops wi h advanced disease

90

Signs Early Progressive myopia and as igma ism Scissors ref ex on re inoscopy Irregular mires on kera ome ry In erior s eepening on compu erized corneal opography ( Fig. 4-1A) and omography. Eyes wi h “low sagging cones” can demons ra e a mild crab-claw opographic pat ern ( Fig. 4-1B), which is similar o he pat ern seen in pellucid marginal degenera ion. Cen ral or paracen ral s romal hinning o he cornea wi h pro rusion a he apex o he hinning ( Fig. 4-1C) Fleischer’s ring: epi helial iron deposi s a he base o he cone ( Fig. 4-1D) Prominen corneal nerves ( Fig. 4-1E) La e Vog ’s s riae: ne ver ical deep s romal ension lines ha disappear emporarily

Keratoconus

wi h digi al pressure applied o he limbus ( Fig. 4-1F) Abnormal “oil drople ” red ref ex Rizut i’s sign: conical ligh ref ec ion on he nasal limbus when ligh is shone rom he emporal side Variable corneal scarring, depending on severi y ( Fig. 4-1G–I). May develop an eleva ed apical nodule ( Fig. 4-1J) Munson’s sign: bulging o he lower eyelid in downgaze Acu e hydrops: severe corneal edema resul ing rom a ear in Desceme ’s membrane ( Fig. 4-1K–M) Associations Ocular: vernal disease, blue sclera, re ini is pigmen osa, Leber’s congeni al amaurosis, f oppy eyelid syndrome Sys emic: Down’s syndrome, EhlersDanlos syndrome, Aper ’s syndrome, ocular allergies, os eogenesis imper ec a Dif erential Diagnosis Pellucid marginal degenera ion: in erior peripheral corneal hinning wi h pro rusion o he cornea above he area o maximal hinning Treatment Mild cases: glasses and so con ac lenses

91

Modera e cases: rigid gas-permeable con ac lens (RGPCL), hybrid lens, or scleral lens Severe and con ac lens–in oleran cases: Lamellar kera ec omy wi h a blade or excimer laser or an erior nodules Placemen o in racorneal ring segmen s Deep an erior lamellar kera oplas y Pene ra ing kera oplas y Epikera oplas y and hermokera oplas y are rarely per ormed. Re rac ive surgery in pa ien s wi h keraoconus is unpredic able and generally no recommended. Corneal collagen crosslinking: Generally per ormed by placing ribof avin drops on he cornea and hen rea ing he cornea wi h ul raviole ligh , i is being used o “s reng hen” he cornea o preven worsening o kera oconus in pa ien s wi h documen ed progression. While no curren ly FDA-approved, shor - erm resul s are promising. Prognosis Mos pa ien s do well wi h RGP or hybrid CLs. T e success ra e wi h corneal ransplana ion in kera oconus is high.

92

4 ECTATIC CO NDITIO NS O F THE CO RNEA

A

B

C FIGURE 4-1. Keratoconus corneal topography. A. Signif can irregular in erior corneal s eepening is apparen using compu erized corneal opographic analysis in his eye wi h modera e kera oconus. As seen on he color scale on he le , he red colors indica e corneal s eepening and blue colors indica e corneal at ening. T e f gure on he righ shows he Placido’s rings; his image is impor an o veri y he quali y and cen ra ion o he s udy. B. T e irregular in erior s eepening seen on compu erized corneal opographic analysis o his eye wi h kera oconus reveals a sligh crab-claw pat ern, which is reminiscen o pellucid marginal degenera ion. Eyes wi h “low sagging cones” can demons ra e his opographic pat ern. Keratoconus. C. Sli -beam view o his eye wi h signif can kera oconus demons ra es in erocen ral corneal hinning and s eepening. No e he hinnes and mos pro ruded areas o cornea coincide. ( continued)

Keratoconus

93

D

E FIGURE 4-1. ( Continued) Keratoconus. D. A prominen Fleischer’s ring, iron pigmen deposi ion a he base o he cone, is presen in his eye wi h kera oconus. E. Prominen corneal nerves can be seen in his eye wi h kera oconus. T ese nerves can be dis inguished rom corneal “ghos ” blood vessels because “ghos ” vessels have a lumen, making hem appear as wo parallel lines. ( continued)

94

4 ECTATIC CO NDITIO NS O F THE CO RNEA

F

G FIGURE 4-1. ( Continued) Keratoconus. F. Fain , ver ical pos erior s romal s ress lines, Vog ’s s riae, are visible a he apex o he cone. Gen le pressure on he limbus can cause hese lines o change direc ion or disappear. G. Signif can cen ral s romal scarring is presen in his eye wi h advanced kera oconus. ( continued)

Keratoconus

95

H

I FIGURE 4-1. ( Continued) Keratoconus. H. Modera e in erocen ral corneal scarring can be seen in his eye a er resolu ion o corneal hydrops. An iron line is visible a he superior edge o he scarring. I. Sli -beam image o he same eye demons ra es signif can hinning in he area o scarring. ( continued)

96

4 ECTATIC CO NDITIO NS O F THE CO RNEA

J

K FIGURE 4-1. ( Continued) Keratoconus. J. A hyper rophic nodule is presen a he apex o he cone. T ese nodules can occur de novo or rela ed o rigid con ac lens wear. Such a nodule can a ec vision and/ or in er ere wi h com or able con ac lens wear. T ese nodules can be removed wi h a superf cial kera ec omy wi h a blade or excimer laser pho o herapeu ic kera ec omy (P K). K. Acu e corneal hydrops occurs when he cornea s re ches o such a degree ha a ear develops in Desceme ’s membrane, allowing sudden in ow o aqueous uid in o he corneal s roma. T e corneal s roma can swell o grea er han f ve imes i s normal hickness. Acu e hydrops is associa ed wi h a sudden decrease in vision and increase in pain. ( continued)

Keratoconus

97

L

M FIGURE 4-1. ( Continued) Keratoconus. L. Sli -beam view o he same cornea. No e he severe corneal hickening. A prominen cle is apparen cen rally, where corneal lamellae are separa ed by a large degree o aqueous uid. M. Severe corneal whi ening rom acu e corneal hydrops is presen . T e ellow eye has scarring rom previous corneal hydrops (see Fig. 4-1H and I) .

98

4 ECTATIC CO NDITIO NS O F THE CO RNEA

PELLUCID MARGINAL DEGENERATION

P

ellucid marginal degenera ion is an uncommon, bila eral condi ion wi h in erior corneal hinning, pro rusion, and irregulari y. I usually presen s in early adul hood. Etiology Sporadic

Symptoms Gradually decreasing vision beginning in young adul hood. Can develop acu e decreased vision and pain due o hydrops wi h advanced disease Signs High irregular agains - he-rule as igmaism (f a a 90 degrees, s eep a 180 degrees) Recognizable “crab-claw” pat ern o irregular as igma ism on compu erized corneal opography and omography ( Fig. 4-2A), al hough a similar pat ern can be ound in eyes wi h kera oconus wi h “low sagging cones.” In erior, crescen -shaped band o peripheral corneal hinning, 1 o 2 mm in wid h, ex ending rom he 4 o’clock o he 8 o’clock posi ion, which is separa ed rom he limbus by normal cornea ( Fig. 4-2B). T e area o pro rusion is loca ed above he band o hinning ( Fig. 4-2C and D).

Fleischer’s ring and Vog ’s s riae are absen . Corneal hydrops occurs on rare occasions. Dif erential Diagnosis Kera oconus: in erocen ral corneal hinning wi h pro rusion o cornea in he area o grea es hinning. A Fleischer’s ring and Vog ’s s riae may be presen . Treatment Mild and modera e cases: RGPCL, hybrid lens, or scleral lens. Severe and con ac lens–in oleran cases Large in erior pene ra ing kera oplas y Deep an erior lamellar kera oplas y In erior crescen ic wedge resec ion Crescen ic pene ra ing kera oplas y (some imes ollowed by a cen ral pene ra ing kera oplas y) and placemen o in racorneal ring segmen s are occasionally per ormed. Re rac ive surgery in pa ien s wi h pellucid marginal degenera ion is unpredic able and generally no recommended. Prognosis Mos pa ien s do well wi h RGPCLs, al hough hey are harder o han in kera oconus pa ien s. T e success ra e wi h corneal ransplan a ion in pellucid marginal degenera ion is good, bu no as good as kera oconus, due o more peripheral disease.

A

FIGURE 4-2. Pellucid marginal degeneration corneal topography. A. Signif can irregular nasal and emporal corneal s eepening is apparen in his compu erized corneal opographic analysis o his eye wi h modera e pellucid marginal degenera ion. Classically, he s eepening is seen o curve around in eriorly in his condi ion. As seen on he color scale on he le , he red colors indica e corneal s eepening and blue colors indica e corneal at ening. T e f gure on he righ shows he Placido’s rings; his image is impor an o veri y he quali y and cen ra ion o he s udy. No e he severe dis or ion o he Placido’s rings in his eye, wi h pellucid marginal degenera ion. ( continued)

Pellucid Marginal Degeneration

99

B

C

D FIGURE 4-2. ( Continued) Pellucid marginal degeneration. B. Side view demons ra es corneal pro rusion in eriorly wi h signif can s eepening near he limbus. C. T is sli -beam pho o reveals in erior corneal hinning approxima ely 2 mm rom he limbus, below he area o maximal pro rusion. D. Sli -beam view o his eye wi h severe pellucid marginal degenera ion also reveals corneal hinning approxima ely 2 mm rom he in erior limbus. T ere is signif can corneal s eepening in eriorly. No e ha he mos pro ruded por ion o he cornea is above he hinnes area.

100

4 ECTATIC CONDITIO NS O F THE CORNEA

KERATO GLOBUS

K

era oglobus is an ex remely rare, bila eral condi ion o severe uni orm peripheral corneal hinning. I usually presen s a or shor ly a er bir h. Etiology Unknown

Symptoms Poor vision, occasionally pain due o hydrops Signs o al corneal hinning wi h maximal hinning in he midperiphery, resul ing in pro rusion o he en ire cornea ( Fig. 4-3) T e cornea can be very hin. Normal corneal diame er; very deep an erior chamber Acu e hydrops may occur in advanced cases.

Pa ien may develop a corneal per ora ion rom minimal rauma because o he severe corneal hinning. Systemic Associations A syndrome comprising blue sclera, hyperex ensible join s, den al and hearing abnormali ies Hyper hyroidism Rubins ein- aybi syndrome Treatment Mild and modera e cases: Spec acles, which improve vision and provide some proec ion agains rauma Severe cases: Some pa ien s do well wi h a scleral lens. Surgical rea men is problema ic. A large ec onic lamellar gra ollowed many mon hs la er by a smaller pene ra ing gra is an op ion. Prognosis Fair. Surgical rea men has a low success ra e.

Keratoglobus

101

A

B

FIGURE 4-3. Keratoglobus. A. A hin, bulging cornea is eviden in his eye wi h kera oglobus. B. A sli -beam view demons ra es ha he hinnes por ion o he cornea is in he periphery in his eye wi h kera oglobus.

C H AP T ER

Corneal Dys rophies ANTERIOR CORNEAL DYSTROPHIES

be a–induced [ GFBI] gene o chromosome 5q31)

EPI HELIAL BASEMEN MEMBR NE DYS OPHY (AN E IO BASEMEN MEMBR NE DYS OPHY, MAP-DO -FINGE P IN DYS OPHY, COGAN’S MIC OCYS IC DYS OPHY)

Symptoms

E

pi helial basemen membrane (EBM) dys rophy is a common epi helial dys rophy ha can cause pain ul recurren corneal erosions and/ or decreased vision.

Etiology and Pathology EBM dys rophy is due o an abnormali y o produc ion o epi helial basemen membrane ha ex ends in o he epi helium, leading o mul iple basemen membranes in he corneal epi helium. rapped epi helial cells can orm “Cogan’s microcys s.” ypically degenera ive, occasionally au osomal dominan ( rans orming grow h ac or

102

Mos commonly asymp oma ic Recurren erosion syndrome: may have unila eral or bila eral recurren episodes o pain in he middle o he nigh or upon opening he eyes af er sleep. Can occur af er rauma wi h a sharp objec such as a ngernail, ree branch, or paper edge May no e painless dis or ion o vision when he cen ral cornea is involved Signs Sli -lamp examina ion shows maplike lines, do s (microcys s), and/ or ngerprin like epi helial lesions, which may occur singly or in various combina ions ( Fig. 5-1). T ese ndings are bes seen wi h re roillumina ion and wi h a broad sli beam rom he side. “Nega ive s aining” rom sligh ly eleva ed areas o epi helium may be seen wi h uorescein dye.

Anterior Corneal Dystrophies

Eyes wi h recurren erosions may have minimal clinical ndings, localized areas o loose epi helium, or a rank epi helial de ec .

103

Dif erential Diagnosis O her an erior corneal dys rophies, such as Meesmann’s dys rophy and Reis-Bücklers dys rophy

Pain ul erosions can be rea ed wi h lubricaion, hyper onic drops and oin men (sodium chloride 5%), pressure pa ching, opical cor icos eroids and oral doxycycline, epi helial debridemen , bandage sof con ac lens, an erior s romal micropunc ure, diamond burr polishing o Bowman’s membrane, or excimer laser pho o herapeu ic kera ec omy (P K).

Treatment I vision is decreased due o cen ral involvemen , he irregular epi helium can be debrided.

Prognosis Very good wi h appropria e rea men , al hough some pa ien s have recalci ran recurren erosions

A FIGURE 5-1. Epi helial basemen membrane dys rophy. A. Reduplica ed epi helial basemen membrane causing maplike changes are readily visible cen rally. ( continued)

104

5 CO RNEAL DYST RO PHIES

B

C FIGURE 5-1. ( Continued) Epi helial basemen membrane dys rophy. B. Large maplike opaci ies are presen hroughou he cen ral cornea, causing complain s o monocular “diplopia,” bet er described as “shadow vision.” C. Maplike changes in his eye are almos conf uen cen rally, resul ing in signi can visual dis or ion. ( continued)

Anterior Corneal Dystrophies

105

D

E FIGURE 5-1. (Continued) Epi helial basemen membrane dys rophy. D. T e maplike changes here are o en re erred o as a “mare’s ail” pat ern. E. Fluorescein s ain and he cobal blue ligh view o he cornea shown in D. Signi can “nega ive s aining” is eviden because o areas o eleva ed epi helium. T ese eleva ed areas can cause oreign-body sensa ion and/ or decreased vision. ( continued)

106

5 CO RNEAL DYST RO PHIES

F

G FIGURE 5-1. ( Continued) Epi helial basemen membrane dys rophy. F. Do changes o EBM dys rophy. T ese creamy whi e Cogan microcys s are iny pocke s o sur ace epi helial cells rapped benea h an abnormal epi helial basemen membrane. G. A large area o epi helial microcys s is seen superiorly. ( continued)

Anterior Corneal Dystrophies

107

H

I FIGURE 5-1. ( Continued) Epi helial basemen membrane dys rophy. H. Re roillumina ion view o he same eye highligh s he epi helial microcys s. I. Fingerprin -like changes o EBM dys rophy in re roillumina ion. T ese parallel lines and bleblike changes are a resul o irregulari ies in he epi helial basemen membrane. T ey are causing irregular as igma ism and decreased vision.

108

5 CO RNEAL DYST RO PHIES

MEESMANN’S CO NEAL DYS OPHY(JUVENILE HE EDI A YEPI HELIAL DYS OPHY)

M

eesmann’s dys rophy is a rare bila eral epi helial disorder ha can cause ocular irri a ion and pho ophobia. Etiology and Pathology Meesmann’s dys rophy is an au osomal dominan (kera in K3 and K12 genes o chromosomes 12q13 and 17q12, respec ively) condi ion in which hundreds o iny vesicles con aining periodic acid-Schi (PAS)– posi ive “peculiar subs ance” are ound in he epi helium. Symptoms Pa ien s are usually asymp oma ic bu may no e irri a ion, glare, and pho ophobia. Mild

pain may develop in adul hood as a resul o recurring corneal erosions. Signs Re roillumina ion demons ra es myriad iny, ranslucen , epi helial cys s ha ex end o he limbus and are mos numerous in he in erpalpebral region. T e lesions appear gray or clear under direc illumina ion ( Fig. 5-2). Treatment Mos pa ien s require no rea men . Consider lubrica ion and sunglasses or mild symp oms. Rarely, a bandage sof con ac lens can be used or a super cial kera ec omy can be per ormed or severe symp oms, bu he dys rophy will recur. Prognosis Good, al hough rare pa ien s will have chronic symp oms

Anterior Corneal Dystrophies

109

A

B FIGURE 5-2. Meesmann’s dys rophy. A. Mul iple iny, ranslucen , epi helial cys s are apparen in re roillumina ion. T ey end o be more prominen in he in erpalpebral zone. B. On direc illumina ion, he microcys s are gray in color bu are di cul o see. On illumina ion o he iris, a he 3 o’clock edge o he pupil, myriad microcys s are visible.

110

5 CO RNEAL DYST RO PHIES

EIS-BÜCKLE S DYS

R

OPHY

eis-Bücklers dys rophy is an uncommon, bila eral, symme ric dys rophy o Bowman’s membrane ha causes pain and decreased vision early in li e.

Etiology Reis-Bücklers dys rophy is an au osomal dominan ( GFBI gene o chromosome 5q31) disorder ha causes damage and scarring o Bowman’s membrane and he an erior s roma. Symptoms Severe recurren corneal erosions rom a young age, even soon af er bir h Progression o he condi ion leads o reduced vision ha occurs in he second o hird decades o li e, al hough in severe cases i can occur in he rs decade. Signs Honeycomb appearance due o re icular, ring-shaped, subepi helial opaci ies ha are mos dense cen rally bu may involve he

en ire cornea. Wi h ime, hey can progress deeper in o he s roma ( Fig. 5-3A–D). Dif erential Diagnosis O her an erior or s romal dys rophies (e.g., epi helial basemen membrane dys rophy, granular dys rophy, macular dys rophy) Treatment Mild cases: lubrica ion More severe cases: bandage sof con ac lenses, super cial kera ec omy, excimer laser P K, mid-s romal or deep an erior lamellar kera oplas y, or pene ra ing kera oplas y may be necessary. Prognosis Excimer laser P K can be qui e success ul in improving vision and decreasing pain ul episodes in many cases. Kera oplas y may be required in advanced cases. Recurrence in he donor graf is common af er corneal ransplana ion and also af er P K ( Fig. 5-3E and F). P K can of en be repea ed or per ormed or recurrence af er kera oplas y.

Anterior Corneal Dystrophies

111

A

B FIGURE 5-3. Reis Bücklers dys rophy. A. A sligh re icular pat ern can be seen mainly in he cen ral cornea in his eye wi h rela ively mild Reis-Bücklers dys rophy. B.T is eye has modera e changes o Reis-Bücklers dys rophy. I primarily involves he cen ral cornea, bu he opaci y approaches he limbus in eriorly. ( continued)

112

5 CO RNEAL DYST RO PHIES

C

D FIGURE 5-3. ( Continued) Reis Bücklers dys rophy. C.T is eye wi h advanced Reis-Bücklers dys rophy has di use, re icular, limbus- o-limbus subepi helial and an erior s romal opaci y. T ere are ew i any clear spaces. D. T is eye also has advanced Reis-Bücklers dys rophy. For una ely, he vision improved signi can ly a er excimer laser P K. ( continued)

Anterior Corneal Dystrophies

113

E

F FIGURE 5-3. ( Continued) Reis Bücklers dys rophy. E. Recurren Reis-Bücklers a ew years a er a pene ra ing kera oplas y. Un or una ely, Reis-Bücklers recurs rela ively rapidly a er corneal ransplan a ion. F.T is eye also has recurren Reis-Bücklers dys rophy several years a er pene ra ing kera oplas y. No e he honeycomb opaci y cen rally and involvemen o he en ire corneal periphery.

114

5 CO RNEAL DYST RO PHIES

GELA INOUS D OP–LIKE CO NEAL DYS OPHY

G

ela inous drop–like corneal dys rophy is a rare condi ion ha causes signi can symp oms early in li e.

Etiology and Pathology Gela inous drop–like corneal dys rophy is an au osomal recessive condi ion ( umorassocia ed calcium signal ransducer 2 gene o chromosome 1p32) His opa hology: subepi helial and s romal amyloid deposi s Symptoms Severe decreased vision, pain, redness, pho ophobia, and earing beginning in he rs wo decades o li e. Signs T ere are a varie y o presen a ions, including rela ively a subepi helial opaciies similar o band kera opa hy, small or large groups o eleva ed nodules (“mulberry”

pat ern) ( Fig. 5-4A), and larger nodular lesions (“kumqua ” pat ern). Super cial and deep neovasculariza ion may develop. Super cial and deep s romal opaci ca ion may also develop. Dif erential Diagnosis O her an erior or s romal dys rophies (e.g., macular dys rophy, Reis-Bücklers dysrophy, granular dys rophy) Treatment Mild cases: lubrica ion More severe cases: bandage sof con ac lenses, super cial kera ec omy, excimer laser P K, mid-s romal or deep an erior lamellar kera oplas y, or pene ra ing kera oplas y may be necessary. Prognosis Poor, because he condi ion ypically recurs wi hin a ew years ( Fig. 5-4B). Rarely, a kera opros hesis may be required i here is good op ic nerve and macular unc ion.

Anterior Corneal Dystrophies

115

A

B FIGURE 5-4. Gela inous drop–like dys rophy. A.C onf uen severe cen ral “mulberry-like” eleva ed lesions are seen in his man wi h gela inous drop–like dys rophy. Ob aining good pho ographs was di cul due o in ense pho ophobia. B. Approxima ely 2 years a er pene ra ing kera oplas y, here was signi can symp oma ic recurrence o he nodules. T is pa ien is he sis er o he pa ien seen in A.

116

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STROMAL CORNEAL DYSTROPHIES GR NULA DYS

G

OPHY

ranular dys rophy is an uncommon disorder ha can cause decreased vision and recurren pain ul erosions in young adul s.

Etiology and Pathology Granular dys rophy is an au osomal dominan ( GFBI gene o chromosome 5q31) disorder ha becomes mani es during he rs or second decade o li e. His opa hology: Hyaline deposi s s ain brigh red wi h Masson richrome. Symptoms Pain ul recurren erosions are uncommon, bu hey may occur be ore vision is signi can ly a ec ed. Decreased vision occurs in young adul hood and middle age, when he corneal opaci ies become con uen . Signs Small, discre e, whi e granules (“crushed breadcrumbs”) wi hin he cen ral an erior s roma, separa ed by clear in ervening spaces. Wi h ime, he lesions ex end deeper wi hin

he s roma and become larger and more numerous. Wi h more ime, super cial lesions become con uen over he pupillary axis, severely a ec ing vision. T e periphery is spared ( Fig. 5-5). Dif erential Diagnosis O her an erior or s romal dys rophies (e.g., Reis-Bücklers dys rophy, macular dys rophy) Treatment Mild cases: lubrica ion More severe cases: bandage sof con ac lenses, super cial kera ec omy, excimer laser P K, mid-s romal or deep an erior lamellar kera oplas y, or pene ra ing kera oplas y may be necessary. Prognosis Excimer laser P K can be qui e success ul in improving vision and decreasing pain ul episodes in many cases. Lamellar or pene ra ing kera oplas y may be required in advanced cases. Recurrence in he donor graf is common af er corneal ransplan a ion and also af er P K, al hough i akes longer han af er surgery or Reis-Bücklers dys rophy. P K can of en be repea ed or per ormed or recurrence af er kera oplas y.

Stromal Corneal Dystrophies

117

A

B FIGURE 5-5. Granular dys rophy. A.T is eye wi h mild granular dys rophy has minimal opaci y and s ill re ains excellen vision. T ere are numerous discre e whi e, “crushed breadcrumb” opaci ies cen rally wi h clear in ervening spaces. B.T e same eye seen in re roillumina ion o he re ina; he granules are highligh ed. ( continued)

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C

D FIGURE 5-5. ( Continued) Granular dys rophy. C. T is eye wi h granular dys rophy has rela ively conf uen opaci ies al hough he granules are small and no very dense. D.T is sli -beam view demons ra es some o he granular opaci ies o be ra her super cial. ( continued)

Stromal Corneal Dystrophies

119

E

F FIGURE 5-5. ( Continued) Granular dys rophy. E. T is eye has a combina ion o he f a “crushed breadcrumb” opaci ies and he more hree-dimensional dense whi e s ella e opaci ies. T e in ervening spaces are s ill rela ively clear. F.T e larger, denser, deeper granules are hidden by almos conf uen , very an erior s romal opaci ies. T e Vision is poor. For una ely, he conf uen an erior opaci ies are o en rea able wi h excimer laser P K.

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LAT ICE DYS

L

OPHY

at ice dys rophy is an uncommon disorder ha ypically causes recurren pain ul erosions in young adul s and decreased vision la er in li e.

Etiology and Pathology Lat ice dys rophy can be subdivided in o several ypes: ype I: au osomal dominan inheriance ( GFBI gene o chromosome 5q31). Fine, branching, re rac ile lines wi hin he an erior or mid-s roma, sparing he corneal periphery. T is is by ar he mos common orm. ype II (Mere oja’s syndrome): associa ed wi h sys emic amyloidosis and has au osomal dominan inheri ance (gelsolin gene o chromosome 9q34). Lat ice lines are hicker bu less numerous han in ype I; he lines begin peripherally and progress cen rally. Visual acui y is usually good, wi h minimal recurren erosions. ypes III and IV: au osomal dominan inheri ance ( GFBI gene o chromosome 5q31). Lat ice lines are coarser or hinner han in ype I and go rom limbus o limbus. T ere may no be recurren erosions. His opa hology: amyloid deposi s, which s ain pinkish red wi h Congo red dye, me achroma ic wi h crys al viole s ain, and demons ra e apple-green bire ringence when viewed wi h polarized ligh . Symptoms Pain ul recurren corneal erosions are common and can occur in childhood or early

adul hood. Vision ypically declines af er early adul hood. Signs Cen ral, branching, re rac ile lines (seen well wi h re roillumina ion), subepi helial whi e do s, and di use an erior s romal haze can be seen early in he disease. La er, signi can subepi helial brosis and scarring can occur ( Fig. 5-6). Dif erential Diagnosis Polymorphic amyloid degenera ion (PAD): a condi ion o older pa ien s, wi h no pain ul erosions, no decreased vision, and no amily his ory o cornea problems. Few or many re rac ile amyloid do s or lines are seen in he s roma, ypically cen rally (see Fig. 6-1E–H in Chap er 6). Treatment Mild cases: lubrica ion More severe cases: bandage sof con ac lenses, super cial kera ec omy, excimer laser P K, mid-s romal or deep an erior lamellar kera oplas y, or pene ra ing kera oplas y may be necessary. Prognosis Excimer laser P K can be success ul in improving vision and decreasing pain ul episodes in some cases. Lamellar or pene ra ing kera oplas y may be required in o hers. Recurrence in he donor graf is common af er corneal ransplan and also af er P K, al hough i akes longer han af er surgery or ReisBücklers dys rophy. P K can of en be repea ed or per ormed or recurrence af er kera oplas y.

Stromal Corneal Dystrophies

121

A

B FIGURE 5-6. Lat ice dys rophy. A.T is eye has mild ype I lat ice dys rophy. No e he ne, branching lines ha appear gray-whi e on direc illumina ion and re rac ile on re roillumina ion o he iris. B.T is eye wi h ype I lat ice dys rophy has modera ely advanced disease. T ere are mul iple, rela ively hick lat ice lines cen rally and in hem idperiphery. ( continued)

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5 CO RNEAL DYST RO PHIES

C

D FIGURE 5-6. ( Continued) Lat ice dys rophy. C.T is eye wi h ype I lat ice dys rophy has cen ral an erior s romal haze due o numerous previous episodes o recurren erosions. T ere is subepi helial brosis cen rally. O en, his scarring leads o diminished recurren erosions bu grea ly impedes vision. Re rac ile lat ice lines are s ill visible. D. Re roillumina ion o he re ina highligh s he re rac ile amyloid deposi s in lat ice dys rophy.

Stromal Corneal Dystrophies

MACULA DYS

M

OPHY

acular dys rophy is a rela ively rare disorder ha ypically causes glare and decreased vision in young adul li e. Etiology and Pathology Macular dys rophy is an au osomal recessive (carbohydra e sul o rans erase 6 gene o chromosome 16q22) disorder. I can be subdivided in o wo ypes hrough blood es ing: ype I: presen s in childhood and is more common; lacks kera an sul a e in he cornea ype II: presen s in he second decade, and kera an sul a e is presen in he cornea His opa hology: acid mucopolysaccharide (glycosaminoglycan) deposi s, which s ain wi h colloidal iron and Alcian blue s ains Symptoms Glare and decreased vision in young adul s May have pain ul recurren erosion symp oms

123

Signs Cen ral, gray-whi e, ill-de ned bu relaively ocal opaci ies wi h di use cloudiness o he in ervening s roma (Fig. 5-7A–C). T e cornea is usually hinner han normal. T e lesions ex end rom limbus o limbus and even ually involve he en ire s romal hickness. T e cen ral lesions are super cial, while he peripheral lesions are deep ( Fig. 5-7D). May have associa ed cornea gut a a. Dif erential Diagnosis O her an erior or s romal dys rophies (e.g., Reis-Bücklers dys rophy, granular dys rophy) Treatment Vision is usually a ec ed by he hird decade and requires a corneal ransplan . Excimer laser P K may be help ul o remove super cial cen ral opaci ies. Prognosis Good wi h corneal ransplan a ion. Recurrence af er kera oplas y is uncommon and occurs la e.

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5 CO RNEAL DYST RO PHIES

A

B FIGURE 5-7. Macular dys rophy. A. Small paracen ral ovoid creamy-whi e opaci ies are seen in his eye wi h rela ively mild macular dys rophy. No e ha he en ire cornea is sligh ly hazy. B. T is eye wi h macular dys rophy has cen ral opaci ies o various shapes and sizes. Al hough hey are ain , hese opaci ies are also presen in he corneal periphery. T e en ire cen ral cornea is involved, wi h a conf uen s romal opaci y, so here are no clear zones be ween he dense macules. ( continued)

Stromal Corneal Dystrophies

125

C

D FIGURE 5-7. ( Continued) Macular dys rophy. C. In his eye he cen ral and peripheral opaci ies are very apparen . T e en ire cornea is involved wi h a di use ull hickness haze. D. Sli -beam view demons ra es ha he cen ral opaci ies are in he an erior s roma and he peripheral opaci ies are in he pos erior s roma. T is dis ribu ion is a classic nding in macular dys rophy. T e ull- hickness corneal haze can also be apprecia ed.

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5 CO RNEAL DYST RO PHIES

AVELLINO CO NEAL DYS (GR NULA -LAT ICE)

OPHY

A

similar o hose ound in lat ice dys rophy ( Fig. 5-8)

vellino dys rophy is a varian o granular dys rophy wi h signi can amyloid deposi ion similar o lat ice dys rophy. I causes sympoms similar o hose o granular dys rophy.

Dif erential Diagnosis O her an erior or s romal dys rophies (e.g., Reis-Bücklers dys rophy, granular dysrophy, lat ice dys rophy)

Etiology and Pathology Avellino dys rophy is an au osomal dominan ( GFBI gene o chromosome 5q31) disorder ha becomes mani es during he rs ew decades o li e. His opa hology: consis s o bo h hyaline and amyloid deposi s

Treatment Mild cases: lubrica ion More severe cases: bandage sof con ac lenses, super cial kera ec omy, excimer laser P K, mid-s romal or deep an erior lamellar kera oplas y, or pene ra ing kera oplas y may be necessary.

Symptoms Pain ul recurren erosions are more common han in granular dys rophy. Decreased vision occurs in middle age, when he cen ral corneal opaci ies become con uen . Signs An erior s romal “crushed breadcrumb” opaci ies sugges ive o granular dys rophy, associa ed wi h deeper s romal re rac ile lines

Prognosis Excimer laser P K can be qui e success ul in improving vision and decreasing pain ul episodes in many cases. Lamellar or pene ra ing kera oplas y may be required in advanced cases. Recurrence in he donor graf is common af er corneal ransplan and also af er P K, al hough i akes longer han af er surgery or Reis-Bücklers dys rophy. P K can of en be repea ed or per ormed or recurrence af er kera oplas y.

Stromal Corneal Dystrophies

127

A

B FIGURE 5-8. Avellino dys rophy. A.T is righ eye demons ra es charac eris ics o bo h granular and lat ice dys rophy. T ere are several “crushed breadcrumb” opaci ies along wi h he re rac ile lines o lat ice dys rophy. B.T e pa ien ’s le eye has similar ea ures.

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5 CO RNEAL DYST RO PHIES

SCHNYDE ’S CO NEAL DYS

S

OPHY

chnyder’s corneal dys rophy is a condi ion associa ed wi h choles erol deposi ion in he cornea. T ere are ew ocular symp oms un il la e in li e, al hough he deposi s may be seen early.

Etiology T is is an uncommon, au osomal dominan (UBIAD1 gene o chromosome 1p36) condi ion associa ed wi h hypercholes erolemia and hyper riglyceridemia. I can be associa ed wi h sys emic hyperlipidemia or hypercholes erolemia. I may also be associa ed wi h genu valgum and xan helasma. Symptoms Glare symp oms in adul hood. In advanced cases, pa ien s can develop decreased vision. Signs Fine ring o yellowish-whi e crys alline choles erol deposi s mainly involving he

cen ral an erior s roma in hal o pa ien s ( Fig. 5-9A–C) Of en associa ed wi h a prominen arcus lipoides A ull- hickness cen ral s romal haze develops in la er s ages ( Fig. 5-9D). Dif erential Diagnosis O her causes o corneal crys als (e.g., in ec ious crys alline kera opa hy, cys inosis, gou , mul iple myeloma, monoclonal gammopa hies) Treatment Check as ing choles erol and riglyceride levels. Excimer laser P K or corneal ransplan aion is occasionally needed la e in li e in eyes wi h severe corneal opaci y. Prognosis Vision is usually good, and corneal ransplan is ypically no necessary. P K can be used o remove he super cial crys als in pa ien s wi h severe glare. Recurrence af er P K or kera oplas y is rare.

Stromal Corneal Dystrophies

129

A

B FIGURE 5-9. Schnyder’s corneal dys rophy. A.T is young adul wi h Schnyder’s corneal dys rophy has he cen ral super cial crys als, bu minimal cen ral s romal haze and arcus lipoides. B. T e hree classic charac eris ics o Schnyder’s corneal dys rophy are eviden in his eye. T e cen ral, super cial crys alline opaci ies, ull- hickness s romal haze, and dense peripheral arcus lipoides are all apparen . T e cen ral crys als can have a denser annular pat ern as in his eye. ( continued)

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5 CO RNEAL DYST RO PHIES

C

D FIGURE 5-9. ( Continued) Schnyder ’s corneal dys rophy. C. In his high-magni ca ion view, he super cial crys als and underlying s romal haze are visible. T e edge o he arcus lipoides can also be seen. D.T is pa ien , wi h a amily his ory o Schnyder’s corneal dys rophy, had he noncrys alline orm. T e cen ral ull- hickness opaci y and arcus lipoides are presen . Approxima ely 50% o eyes wi h Schnyder’s corneal dys rophy do no have crys als.

Posterior Corneal Dystrophies

POSTERIOR CORNEAL DYSTROPHIES ENDO HELIAL DYS OPHYAND FUCHS’ DYS OPHY

E

ndo helial dys rophy and Fuchs’ dys rophy represen a con inuum o disease involving abnormali ies in Desceme ’s membrane ha a ec he endo helial cells. Be ore s romal edema occurs, he condi ion is ermed endo helial dys rophy; af er s romal edema develops, i is ermed Fuchs’ dys rophy. Etiology, Epidemiology, and Pathology Endo helial dys rophy is a common condiion ha may proceed o Fuchs’ dys rophy over a period o years. Fuchs’ dys rophy ypically occurs af er he f h or six h decade, more commonly in women. Inheri ance is ypically au osomal dominan bu can be recessive. Associa ed wi h gene ic varia ion in he ranscrip ion ac or 4 gene on chromosome 18q21. His opa hology: iny, cen ral excrescences o a hickened Desceme ’s membrane known as cornea gut a a; pigmen on he endo helium Specular microscopy: variable endo helial size (polymege hism) and shape (pleomorphism), numerous dark areas, reduced number o endo helial cells Symptoms Generally asymp oma ic in endo helial dys rophy. Mildly decreased visual acui y and poor-quali y vision can be seen in eyes wi h modera e endo helial dys rophy, which worsens in early s ages o Fuchs’ dys rophy. Modera e visual loss develops la er, as pos erior s romal edema increases. When epi helial edema develops, here is of en a signi can decrease in vision. Pa ien s gener-

131

ally have worse vision upon awakening in he morning, which improves over several hours. As epi helial edema worsens, bullae, which can rup ure, can cause severe pain. Signs iny, cen ral excrescences o Desceme ’s membrane known as cornea gut a a are seen. T e con uence o lesions gives rise o a “bea en-me al” appearance ( Fig. 5-10A and B). A variable amoun o pigmen on he endo helium and a gray, hickened appearance o Desceme ’s membrane ( Fig. 5-10C) S romal edema develops, giving rise o a hickened cornea (Fuchs’ dys rophy) ( Fig. 5-10D and E). Epi helial edema and bullae (bullous keraopa hy) orm, which may rup ure, causing irri a ion and pain ( Fig. 5-10F). Years o bullae orma ion can cause scarring and brosis, wi h ewer pain ul symp oms bu poorer vision ( Fig. 5-10G). Increased incidence o hyperopia, narrow angles, and glaucoma. Dif erential Diagnosis Aphakic and pseudophakic bullous keraopa hy: af er ca arac surgery Pos erior polymorphous corneal dys rophy: linear, bandlike, vesicular, or grouped con gura ions wi h irregular edges a he level o Desceme ’s membrane Treatment rea men in early s ages includes hyperonic saline, lubrica ion, and lowering in raocular pressure. When mild epi helial edema is presen , blowing warm air rom a hair dryer held a arm’s leng h over he eyes or 5 o 10 minu es each morning can improve some pa ien s’ vision earlier in he day.

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When vision is signi can ly impaired, endohelial or pene ra ing kera oplas y is indica ed. Prognosis Endo helial dys rophy uncommonly progresses o Fuchs’ dys rophy. Ca arac surgery

may precipi a e developmen o persis en corneal edema in eyes wi h endo helial dysrophy. Mild o modera e Fuchs’ dys rophy can of en be managed success ully wi hou surgery, bu i a corneal ransplan is required, he success ra e is very good.

A

B FIGURE 5-10. Fuchs’ dys rophy. A. A hickened Desceme ’s membrane wi h a corruga ed or “orange peel” pat ern o he endo helial cell layer is apparen in his eye wi h mild Fuchs’ dys rophy. B. Using re roillumina ion o he re ina, he “bea en-me al” pat ern o cornea gut a a can easily be apprecia ed. ( continued)

Posterior Corneal Dystrophies

133

C

D FIGURE 5-10. ( Continued) Fuchs’ dys rophy. C.T is eye wi h mild Fuchs’ dys rophy has mild s romal edema wi h some Desceme ’s olds. T ere are some secondary epi helial basemen membrane changes a he 5 o’clock edge o he pupil. Brown pigmen on he endo helium can be seen cen rally. D. In his eye wi h modera e Fuchs’ dys rophy, s romal edema has caused cen ral corneal clouding and decreased vision. ( continued)

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5 CO RNEAL DYST RO PHIES

E

F FIGURE 5-10. ( Continued) Fuchs’ dys rophy. E. Sli -beam view o he same eye demons ra es cen ral corneal hickening. T e irregular ligh ref ex is due o mild microcys ic edema. F. Sli -beam view o his eye wi h more advanced Fuchs’ dys rophy no only reveals cen ral s romal edema, bu also a large epi helial bulla in erocen rally. (continued)

Posterior Corneal Dystrophies

135

G FIGURE 5-10. ( Continued) Fuchs’ dys rophy. G.T is eye wi h advanced Fuchs’ dys rophy has developed s romal edema involving mos o he cen ral cornea. A large area o eleva ed subepi helial brosis is presen in he cen ral area o he edema.

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5 CO RNEAL DYST RO PHIES

POS E IO POLYMO PHOUS CO NEAL DYS OPHY

P

os erior polymorphous corneal dys rophy is an uncommon condi ion characerized by various abnormali ies o Desceme ’s membrane and he endo helium.

Etiology and Pathology T is is an uncommon, au osomal dominan , markedly variable condi ion a ec ing Desceme ’s membrane and endo helium. T e signs and symp oms can be very variable even wi hin members o he same amily. His opa hology: Endo helial cells look like epi helial cells in ha hey have microvilli and s ain posi ive or kera in. Symptoms Onse o symp oms may occur a bir h, bu many pa ien s are asymp oma ic. T e main symp om is decreased vision due o corneal edema. Pain can occur i corneal bullae develop. Signs Linear, bandlike, vesicular, or grouped con gura ions wi h irregular, of en scalloped edges a he level o Desceme ’s membrane. T e lesions are requen ly asymme ric. T ere

may be corneal edema in advanced cases ( Fig. 5-11). O hose a ec ed, 15% have glaucoma. May be associa ed wi h iridocorneal adhesions and corec opia May be associa ed wi h Alpor ’s syndrome (heredi ary nephri is and sensorineural hearing loss) Dif erential Diagnosis Iridocorneal endo helial syndrome: unila eral and nonheredi ary Treatment Mos pa ien s require no rea men . Pa ien s need o be moni ored or glaucoma. I corneal edema develops, i can be rea ed similarly o Fuchs’ dys rophy. When vision is signi can ly impaired, endo helial or pene ra ing kera oplas y is indica ed. Prognosis Very good or re aining good vision. When signi can iris changes are presen , he chances o glaucoma increase. Pos erior polymorphous corneal dys rophy rarely requires corneal ransplan a ion, bu i a corneal ransplan is required, he success ra e is good.

Posterior Corneal Dystrophies

137

A

B FIGURE 5-11. Pos erior polymorphous corneal dys rophy. A. A large cen ral horizon al scalloped band is visible in his eye wi h pos erior polymorphous corneal dys rophy. B. Mul iple small gray areas are eviden on he endo helial sur ace in his eye wi h pos erior polymorphous corneal dys rophy. ( continued)

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5 CO RNEAL DYST RO PHIES

C

D FIGURE 5-11. ( Continued) Pos erior polymorphous corneal dys rophy. C. On direc illumina ion, a scalloped band in he endo helium is visible jus superior o he visual axis. D. On re roillumina ion o he re ina, he scalloped band is more apparen . (continued)

Posterior Corneal Dystrophies

139

E

F FIGURE 5-11. ( Continued) Pos erior polymorphous corneal dys rophy. E. Mul iple gray-whi e opaci ies o various shapes and sizes can be seen on direc illumina ion. F. Sli -beam view o he same eye demons ra es ha he opaci ies are a he level o Desceme ’s and endo helium.

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5 CO RNEAL DYST RO PHIES

CONGENI AL HE EDI A Y ENDO HELIAL DYS OPHY

C

ongeni al heredi ary endo helial dys rophy (CHED) is an ex remely rare condi ion involving corneal edema a bir h or shor ly hereaf er.

Etiology and Pathology Au osomal recessive and au osomal dominan orms have been described. His opa hology: abnormal or absen endohelial cells Symptoms Au osomal dominan orm: presen s in he rs 1 o 2 years o li e, is progressive, wi h no nys agmus, bu pain and pho ophobia are common Au osomal recessive orm: presen s a bir h, is nonprogressive, nys agmus is presen , and here is no pain

Signs Bila eral, limbus- o-limbus corneal s romal edema wi h a blue-gray ground-glass appearance ( Fig. 5-12) Corneal hickness can be wo o hree imes normal No increase in corneal diame er or eleva ed in raocular pressure Dif erential Diagnosis Congeni al glaucoma: enlarged corneal diame er, eleva ed in raocular pressure Bir h rauma: unila eral, parallel oblique breaks in Desceme ’s membrane Treatment Depends on degree o corneal edema. Endo helial or pene ra ing kera oplas y may be indica ed i corneal edema is severe. Prognosis Fair, because o he di cul y o per orming corneal ransplan a ion in children.

Posterior Corneal Dystrophies

141

FIGURE 5-12. Congeni al heredi ar y endo helial dys rophy.D i use limbus- o-limbus corneal edema wi h a blue-gray ground-glass appearance is presen in his eye wi h congeni al heredi ary endo helial dys rophy.

C H AP T ER

Corneal Degenera ions and Deposi s INVOLUTIONAL CH ANGES CO NEAL A CUS Corneal arcus is a very common, bila eral condi ion ha may be ei her age-rela ed (arcus senilis) or associa ed wi h hyperlipidemia in younger individuals (arcus lipoides). Lipid deposi s begin in eriorly, hen superiorly, and la er ex end circum eren ially o orm a whi e perilimbal band abou 1 mm in diameer wi h a sharp ou line peripherally and a more dif use boundary cen rally. A clear zone o cornea separa es i rom he limbus ( Fig. 6-1A). May be accompanied by mild, nonprogressive hinning o he clear zone o he cornea ( urrow degenera ion) Check or hyperlipidemia in pa ien s under age 40 years. I unila eral, check or caro id disease on he uninvolved side. Pa ien s are asymp oma ic, and ocular rea men is no necessary.

142

WHITE LIMBAL GI DLE OF VOGT Whi e limbal girdle o Vog is a very common, bila eral, innocuous, age-rela ed condiion charac erized by chalky-whi e, crescen ic deposi s (elas o ic degenera ion) along he nasal and emporal perilimbal cornea. I may or may no be separa ed rom he limbus by a clear zone ( Fig. 6-1B). Pa ien s are asymp oma ic, and ocular rea men is no necessary.

C OCODILE SHAG EEN Crocodile shagreen is charac erized by grayish-whi e, polygonal s romal opaci ies separa ed by rela ively clear spaces. T e lesions usually involve he an erior s roma (an erior crocodile shagreen), bu hey may also be ound more pos eriorly (pos erior crocodile shagreen) ( Fig. 6-1C). Pa ien s are asymp oma ic, and ocular rea men is no necessary.

Involutional Changes

CO NEA FA INATA Cornea arina a is a rela ively common condi ion charac erized by bila eral, innocuous, minu e, “ our-dus ” lipo uscin-like deposi s in he deep s roma near Desceme ’s membrane. I is mos prominen cen rally. T ese opaci ies are bes seen wi h re roillumina ion of he iris ( Fig. 6-1D). Pa ien s are asymp oma ic, and ocular rea men is no necessary.

POLYMO PHIC AMYLOID DEGENER TION Polymorphic amyloid degenera ion is a airly common, bila eral, innocuous,

143

degenera ive condi ion usually seen a er he age o 50 years. I is charac erized by varying sizes o re rac ile, punc a e, comma-shaped, and lamen ous amyloid deposi s hroughou he s roma, bu is generally mos prominen cen rally and pos eriorly. T ese deposi s are bes seen wi h re roillumina ion of he re ina ( Fig. 6-1E–H). I is no associa ed wi h any sys emic disorder. Dif eren ial diagnosis: cornea arina a and lat ice dys rophy Pa ien s are asymp oma ic, and ocular rea men is no necessary.

144

6 CO RNEAL DEGENERATIO NS AND DEPO SITS

A

B FIGURE 6-1. Corneal arcus. A. A circular yellow-whi e lipid deposi ion is presen adjacen o he limbus or 360 degrees. No e he clear zone be ween he arcus and he limbus. Limbal girdle o Vogt. B. A crescen ic, rela ively dense whi e opaci y is seen a he limbus a he 9 o’clock posi ion. T ere is a small clear zone be ween he limbal girdle and he limbus. ( continued)

Involutional Changes

145

C

D FIGURE 6-1. ( Continued) Crocodile shagreen. C. Gray-whi e polygonal s romal opaci ies are eviden in his cornea. T ey may be loca ed in he an erior or he pos erior s roma. Cornea arinata. D. iny, “f our-dus ” deposi s are seen a he pupillary margin. T ese pinpoin opaci ies are loca ed in he deep s roma. T ey do no a ec vision. ( continued)

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6 CO RNEAL DEGENERATIO NS AND DEPO SITS

E

F FIGURE 6-1. ( Continued) Polymorphic amyloid degeneration. E. Amyloid deposi s in various shapes, including do s, commas, and lines, are seen in he corneal s roma. T is condi ion is a degenera ion, no a dys rophy. I is similar o lat ice dys rophy in ha hey bo h involve amyloid deposi ion; however, lat ice dys rophy is an inheri ed condi ion ha is ypically associa ed wi h recurren erosions and decreased vision in young adul hood. F.T is eye wi h polymorphic amyloid degenera ion has dense cen ral amyloid deposi s readily seen in re roillumina ion o he re ina. ( continued)

Involutional Changes

147

G

H FIGURE 6-1. ( Continued) Polymorphic amyloid degeneration. G. Mul iple small gray-whi e polymorphic amyloid degenera ion opaci ies are presen , primarily in he mid-periphery, in his elderly woman H.T e polymorphic amyloid degenera ion opaci ies in he same eye are highligh ed in re roillumina ion.

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6 CO RNEAL DEGENERATIO NS AND DEPO SITS

CORNEAL DEPOSITS— NONPIGMENTED BAND ER TOPATHY

B

and kera opa hy is a common condi ion charac erized by calcium deposi s in he subepi helial space, Bowman’s layer, and an erior s roma. Etiology Ocular Chronic ocular in amma ion (e.g., iridocycli is, juvenile rheuma oid ar hri is, corneal edema, in ers i ial kera i is, ph hisis bulbi) Silicone oil in he eye, especially he an erior chamber Me abolic Hypercalcemia or hyperphospha emia Gou Chronic renal ailure Heredi ary: amilial O her Chronic exposure o oxic vapors (e.g., mercury) Idiopa hic (age-rela ed)

Symptoms O en asymp oma ic. I cen ral, vision may be af ec ed. Ocular irri a ion can develop i hick calcium plaques ake of and cause an epi helial de ec .

Signs Peripheral, in erpalpebral plaque o calcium deposi usually separa ed rom he limbus by a hin line o clear cornea ( Fig. 6-2A–C) T e plaque ypically begins a he nasal and emporal cornea and ex ends cen rally. I o en con ains small holes and cle s, giving i a “Swiss cheese” appearance. Advanced lesions may become plaquelike, nodular, and eleva ed ( Fig. 6-2D). Treatment Mild cases may be observed or rea ed wi h lubrican s (e.g., ar i cial ear drops or oin men s). Severe cases (wi h visual, pain ul, or cosme ic indica ions) can be rea ed wi h chela ion using disodium e hylenediamine e raace ic acid 3% or super cial kera ec omy using he excimer laser (pho o herapeu ic kera ec omy, or P K) or a blade. Prognosis Excellen or he ocular calcium deposi s. T e band kera opa hy can recur, especially i he underlying condi ion persis s. Calcium chela ion can be repea ed i necessary. Epi helial healing problems may occur. Vision is o en limi ed, as a resul o residual corneal scarring or o her ocular pa hology.

Corneal Deposits—Nonpigmented

149

A

B FIGURE 6-2. Band keratopathy. A. A hin layer o calcium deposi ion can be seen adjacen o he limbus nasally and emporally. No e he hin line o clear cornea be ween he band kera opa hy and he limbus. B.T is eye had cen ral calcium deposi ion obscuring he view o he iris and pupil. ( continued)

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6 CO RNEAL DEGENERATIO NS AND DEPO SITS

C

D FIGURE 6-2. ( Continued) Band keratopathy. C. T is eye has he classic limbus- o-limbus horizon al “band” o band kera opa hy. D. T is eye wi h chronic corneal edema rom herpes zos er kera opa hy has a dense cen ral plaque o calcium deposi ion. Some o he plaque spon aneously f aked o cen rally.

Corneal Deposits—Nonpigmented

SALZMANN’S NODULA DEGENER TION

S

alzmann’s nodular degenera ion is a airly common, unila eral or bila eral condi ion charac erized by smoo h gray-whi e eleva ed lesions o he cornea.

Etiology I is o en ound in eyes wi h a his ory o chronic kera opa hy, such as in ers i ial kera iis, vernal kera oconjunc ivi is, kera oconjuncivi is sicca, phlyc enulosis, and rachoma, bu requen ly appears in o herwise normal eyes.

151

nodules anywhere on he sur ace o he cornea ( Fig. 6-3) Long-s anding nodules may have iron pigmen deposi ion in he epi helium a he base o he nodule. Dif erential Diagnosis Spheroidal degenera ion: small, globular, yellow-brown granules are ound in he supercial corneal s roma.

Symptoms O en asymp oma ic. May af ec vision i i involves he paracen ral or cen ral cornea; may cause a oreign-body sensa ion i i becomes very eleva ed

Treatment Mild cases are observed or rea ed wi h lubrica ion. I he nodules are causing symp oms, hey may be rea ed wi h super cial kera ec omy wi h a blade or excimer laser P K. opical mi omycin C a he ime o surgical excision may decrease he ra e o recurrence. Rarely, i severe, i may require a lamellar kera oplas y.

Signs Single or mul iple, discre e, whi e or graywhi e or occasionally bluish, smoo h, eleva ed

Prognosis Very good o excellen . Can recur a er surgical excision

A FIGURE 6-3. Salzmann’s nodular degeneration. A. A gray-whi e eleva ed lesion is seen in he peripheral cornea rom he 9 o 11 o’clock posi ions. T ese lesions may be single or mul iple and peripheral or cen ral. I hey are causing symp oms, hey can usually be rea ed wi h a super cial kera ec omy or excimer laser P K. ( continued)

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B

C FIGURE 6-3. ( Continued) Salzmann’s nodular degeneration. B.T is eye has approxima ely six Salzmann’s nodules superiorly. Flat er Salzmann’s opaci ies can be seen in be ween he more dis inc eleva ed nodules. C. Severe paracen ral and peripheral Salzmann’s nodular degenera ion is obvious rom approxima ely 9 o 6 o’clock.

Other Corneal Degenerations

OTHER CORNEAL DEGENERATIONS SPHE OIDAL DEGENER TION Also known as Labrador’s kera opa hy, ac inic kera opa hy, clima ic drople keraopa hy, corneal elas osis, and Biet i’s nodular dys rophy Spheroidal degenera ion is a rare, unila eral or bila eral condi ion ha ypically af ec s people who work ou doors. In erpalpebral, small, globular, yellowbrown granules are ound in he super cial corneal s roma ( Fig. 6-4A). T e lesions o en begin peripherally and progress cen rally. Advanced lesions can become nodular and eleva ed. Vision becomes af ec ed when lesions are cen ral. Pa ien s can develop a oreign-body sensa ion rom eleva ed nodules. When vision becomes impaired, rea men is wi h super cial kera ec omy wi h a blade, excimer laser P K, or lamellar or pene ra ing kera oplas y. Lesions may recur.

LIPID ER TOPATHY T is is a rela ively common unila eral condi ion mos requen ly associa ed wi h previous herpes simplex or herpes zos er kera i is.

153

Unila eral, ocal, whi e or yellowish deposi s wi h ea hery edges ( Fig. 6-4B). Secondary lesions are associa ed wi h corneal neovasculariza ion, whereas primary lesions (rare) are avascular. May appear crys alline ( Fig. 6-4C). Rarely, he lipid may involve he en ire cornea. Vision can be af ec ed by cen ral lipid deposi ion. rea men is wi h opical cor icos eroids o decrease in amma ion and vascularizaion. I he blood vessels diminish, he lipid may improve. Laser abla ion o vessels can be at emp ed, bu hey ypically reopen. opical or subconjunc ival vascular endohelial grow h ac or (VEGF) inhibi ors may decrease he blood vessels. Advanced cases causing poor vision may bene rom a corneal ransplan .

COATS’ WHITE ING Small, oval, whi e ring, 1 mm or less in diame er, usually loca ed in he in erior cornea a he level o Bowman’s layer wi h an in ac overlying epi helium ( Fig. 6-4D) Represen s old me allic oreign-body injury. Pa ien s are asymp oma ic, and ocular rea men is no necessary.

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A

B FIGURE 6-4. Spheroidal degeneration. A. Gray and brown deposi s are seen in he in erpalpebral area. T ese can be mildly or modera ely eleva ed. Lipid keratopathy. B. Creamy-whi e lipid deposi s in he corneal s roma are apparen in a ea hery pat ern. T e clearer lines are “ghos ” vessels, areas o previous corneal neovasculariza ion. ( continued)

Other Corneal Degenerations

155

C

D FIGURE 6-4. ( Continued) Lipid keratopathy. C. Whi e s romal lipid deposi s in a crys alline pat ern are no ed cen ral o an area o corneal neovasculariza ion. Coats’ white ring. D.T is small, brigh whi e oval ring is he resul o an old me allic oreign-body injury.

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6 CO RNEAL DEGENERATIO NS AND DEPO SITS

CORNEAL DEPOSITS— PIGMENTED CO NEA VE TICILLATA (VO TEX ER TOPATHY)

C

ornea ver icilla a is a condi ion ha occurs in pa ien s wi h Fabry’s disease, bu i is ound much more commonly caused by a varie y o drugs.

Etiology Fabry’s disease: an X-linked recessive sphingolipidosis charac erized by cornea ver icilla a, small conjunc ival aneurysms, lens opaci ies, papilledema, op ic a rophy, and macular and re inal edema. Cornea ver icilla a is seen in males wi h Fabry’s disease and he emale carriers. See also Mucopolysaccharidoses and Lipidoses in Chap er 8. Drugs ha can cause cornea ver icilla a: Amiodarone (mos common) ( Fig. 6-5)

Chloroquine Hydroxychloroquine Chlorpromazine Indome hacin A ovaquone Signs Bila eral, symme rical, golden-brownish epi helial deposi s arranged in a curvilinear ashion rom a poin below he pupil and swirling ou ward bu sparing he limbus Treatment T is corneal disorder is asymp oma ic, and no rea men is required. Prognosis Excellen . T e drug-rela ed deposi s end o resolve upon discon inua ion o he medica ion.

Corneal Deposits—Pigmented

157

FIGURE 6-5. Cornea verticillata. Super cial brown deposi s appearing o emana e rom a poin in he in erior cornea are apparen in his pa ien aking amiodarone. T ese deposi s do no a ec vision. T ey even ually disappear upon discon inua ion o he drug.

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6 CO RNEAL DEGENERATIO NS AND DEPO SITS

C YSTALLINE ER TOPATHY In ectious Crystalline Keratopathy In ec ious crys alline kera opa hy is an uncommon condi ion ha is usually caused by indolen organisms such as Streptococcus viridans. O her bac eria and ungi can also cause his condi ion. I may develop as a complica ion o pene ra ing kera oplas y and/ or he long- erm use o opical cor icos eroids. Discre e, whi e, branching crys alline deposi s in he an erior s roma wi hou signi can associa ed in amma ion are no ed ( Fig. 6-6A and B). rea men is wi h corneal smears and cul ures and in ensive opical an ibio ic herapy. Other Causes o Corneal Crystals Cys inosis: au osomal recessive condi ion resul ing in deposi s o he amino acid cys ine in conjunc iva, corneal s roma, iris, lens, and re ina, depending on severi y. T ere may

be grow h re arda ion, renal ailure, hepa osplenomegaly, and hypo hyroidism (see Fig. 8-3 in Chap er 8). Gou Monoclonal gammopa hies: include mul iple myeloma, lymphoma, and Waldens röm’s macroglobulinemia Chrysiasis: deposi s o gold par icles in pos erior corneal s roma and lens a er chronic usage o gold-con aining drugs or rea men o rheuma oid ar hri is Argyrosis: deposi ion o silver-gray par icles in pos erior corneal s roma as a resul o long- erm use o silver-con aining drops O her drugs: chloroquine, indome hacin, cipro oxacin ( Fig. 6-6C) Hyperlipidemia/ hypercholes erolemia: Schnyder’s corneal dys rophy (see Fig. 5-9 in Chap er 5)

A FIGURE 6-6. In ectious cr ystalline keratopathy. A. Mul iple ne ernlike opaci ies are presen in his corneal ransplan ; he pa ien was using chronic opical s eroids. ( continued)

Corneal Deposits—Pigmented

159

B

C FIGURE 6-6. ( Continued) In ectious cr ystalline keratopathy. B. A crys alline, branching in l ra e is seen cen rally in his corneal ransplan . Cul ure revealed Streptococcus viridans. Ciprof oxacin deposits. C.C onf uen whi e deposi s are seen in his cornea being rea ed wi h hourly ciprof oxacin drops or an in ec ious corneal ulcer. T ese deposi s resolve as he epi helium heals and as he medica ion requency is reduced.

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6 CO RNEAL DEGENERATIO NS AND DEPO SITS

CO NEAL I ON DEPOSITS Epithelial Rus ring: consis s o residual rus ollowing he removal o a me allic oreign body Hudson S ahli’s line: occurs a he juncion o he upper wo- hirds and he lower one- hird o an o herwise normal cornea Ferry’s line: occurs in ron o a l ering bleb ( Fig. 6-7A) S ocker’s line: occurs in ron o a p erygium ( Fig. 6-7A) Fleischer’s ring: occurs a he base o he cone in kera oconus (see Fig. 4-1D in Chap er 4)

O her iron lines may be ound adjacen o corneal eleva ions in Salzmann’s degenera ion, ( Fig. 6-7B), corneal gra s, and a er re racive surgery such as radial kera o omy ( Fig. 6-7C), pho ore rac ive kera ec omy (PRK), and laser-assis ed in si u kera omileusis (LASIK). Stromal Siderosis: as a resul o an in raocular iron oreign body Corneal blood s aining: due o hyphema, especially an “eigh -ball” hyphema. Clears slowly rom he periphery ( Fig. 6-7D and E)

A FIGURE 6-7. Iron lines adjacent to a trabeculectomy bleb and pter ygium. A. T is eye demons ra es iron lines jus cen ral o he superior rabeculec omy bleb (Ferry’s line) and nasal p erygium (S ocker’s line) . ( continued)

Corneal Deposits—Pigmented

161

B

C FIGURE 6-7. ( Continued) Iron line at the base o an elevated lesion. B. An iron line can be seen a he base o his long-s anding eleva ed Salzmann’s nodule. Iron line a er re ractive surger y. C. A s ella e iron line is presen cen rally in his eye wi h corneal f at ening several years a er radial kera o omy or myopia. ( continued)

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6 CO RNEAL DEGENERATIO NS AND DEPO SITS

D

E FIGURE 6-7. ( Continued) Corneal blood staining. D.T ere is signi can brown corneal s romal deposi ion in his eye. T e pa ien had previously undergone an ex racapsular ca arac ex rac ion and subsequen rauma resul ing in dehiscence o he ca arac wound and a o al hyphema. T e wound was repaired, bu signi can blood s aining developed. No e ha here is some clearing o he blood s aining rom he periphery. E.C orneal blood s aining can be seen resolving rom he corneal periphery in his eye 6 mon hs a er severe ocular rauma.

Corneal Deposits—Pigmented

K YSE -FLEISCHE

ING

Bila eral, greenish brown, peripheral band 1 o 3 mm in wid h a he level o Desceme ’s membrane, which occurs primarily in Wilson’s disease T e band rs appears in he ver ical meridian, hen ex ends o involve he en ire corneal circum erence. Early cases may require gonioscopy o visualize he deposi s ( Fig. 6-8; see also Fig. 8-1 in Chap er 8). May have associa ed subcapsular len icular deposi s resul ing in a “sun ower” ca arac in Wilson’s disease.

163

Etiology Wilson’s disease (hepa olen icular degenera ion): mos common cause o a KayserFleischer ring. A rare au osomal recessive condi ion caused by a de ciency o he enzyme ceruloplasmin. Charac erized by liver cirrhosis and mo or disorders. rea men wi h copper chela ing agen s such as D-penicillamine or e ra hiomolybda e may improve he condi ion and be ollowed by resolu ion o corneal deposi s. Primary biliary cirrhosis Chronic ac ive hepa i is Mul iple myeloma

FIGURE 6-8. Kayser Fleischer ring. Sli -beam view o he in erior cornea o a pa ien wi h Wilson’s disease. No e he brown pigmen a he level o Desceme ’s membrane peripherally. In early cases, he pigmen may be seen bes using gonioscopy. See also Figure8 -1.

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6 CO RNEAL DEGENERATIO NS AND DEPO SITS

TE IEN’S MA GINAL DEGENER TION

T

errien’s marginal degenera ion is an uncommon, o en bila eral, painless, slowly progressive peripheral corneal hinning condi ion. Etiology Unknown. Af ec s males more commonly han emales, generally in he second o our h decades

deposi s cen ral o he hinned edge. T e hinning usually begins superiorly and ex ends circum eren ially, al hough i can begin in eriorly. Epi helium remains in ac ( Fig. 6-9). Per ora ion is rare and is usually associa ed wi h blun rauma.

Symptoms Pa ien s are asymp oma ic wi h mild disease. More advanced disease causes decreased vision rom severe agains - he-rule as igmaism, which is commonly irregular.

Treatment Mild cases can be rea ed wi h glasses or so con ac lenses. Modera e cases can achieve good vision wi h rigid gaspermeable, hybrid, or scleral con ac lenses. Advanced cases may require a crescen ic inlay lamellar kera oplas y or ec onic purposes.

Signs Nonin amed, peripheral hinning associa ed wi h a vascularized pannus and lipid

Prognosis Good or mild and modera e disease, air or severe disease

A FIGURE 6-9. Terrien’s marginal degeneration. A. Superior peripheral corneal hinning wi h overlying pannus is seen. A dense arc o lipid deposi ion is seen a he cen ral edge o he hinning. ( continued)

Corneal Deposits—Pigmented

165

B

C FIGURE 6-9. ( Continued) Terrien’s marginal degeneration. B. Sli -beam view reveals corneal hinning, pannus, and lipid deposi ion superiorly. C. In his eye, which has signi can errien’s marginal degenera ion, sli -beam view demons ra es superior corneal hinning and neovasculariza ion wi h lipid a he leading edge.

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6 CO RNEAL DEGENERATIO NS AND DEPO SITS

I IDOCO NEAL-ENDOTHELIAL SYND OME

I

ridocorneal-endo helial (ICE) syndrome is a rare, unila eral, noninheri ed condi ion af ec ing he cornea, iris, and an erior chamber angle and is associa ed wi h glaucoma.

Etiology More common in young o middle-aged emales ICE syndrome consis s o hree overlapping ypes: Essen ial iris a rophy—iris hinning, iris holes, corec opia, pseudopolycoria Chandler syndrome—mild iris hinning and corec opia, marked corneal edema Cogan-Reese (iris nevus) syndrome— mul iple small pigmen ed iris nodules His opa hology: Epi helioid me aplasia o corneal endo helium, which can grow across he an erior chamber angle Symptoms Asymp oma ic in early s ages. Cosme ic symp oms can resul rom iris changes. Decreased vision occurs rom corneal edema and occasionally glaucoma.

Signs Common ea ures include an abnormal corneal endo helium wi h a ain ly hazy, bea en-me al appearance and broad peripheral an erior synechiae ex ending beyond Schwalbe’s line ( Fig. 6-10). Glaucoma resul s rom synechial angle closure and is mos severe in Chandler’s syndrome. Dif erential Diagnosis Axen eld-Rieger syndrome: prominen an eriorly displaced Schwalbe’s line, peripheral iris s rands, possible sys emic abnormali ies Pos erior polymorphous corneal dys rophy: au osomal dominan and bila eral, more corneal and ewer iris changes Treatment rea men includes medical or surgical herapy o lower in raocular pressure and corneal ransplan a ion or corneal decompensa ion. Prognosis Good o very good i he glaucoma can be con rolled. T e glaucoma can be di cul o con rol wi hou surgery and increases he risk o gra ailure.

A FIGURE 6-10. Iridocorneal endothelial syndrome. A.T is eye has mild essen ial iris a rophy. No e he ec ropion uveae and corec opia. ( continued)

Corneal Deposits—Pigmented

167

B

C

D FIGURE 6-10. ( Continued) Iridocorneal endothelial syndrome. B.T is eye has essen ial iris a rophy wi h peripheral an erior synechiae in eriorly and a large s re ch hole superiorly. C.T ere is an updrawn pupil due o progressive peripheral an erior synechiae orma ion superiorly and di use iris hinning in his eye wi h essen ial iris a rophy. Mild corneal edema is presen . D. Severe corec opia wi h he pupil drawn oward he 3 o’clock limbus can be seen in his eye wi h essen ial iris a rophy. T ere are several large s re ch holes. T is pa ien underwen a corneal gra or severe corneal edema.

C H AP

ER

Corneal In ec ions, In amma ions, and Sur ace Disorders BACTERIAL KERATITIS

B

ac erial kera i is is a serious, po en ially sigh - hrea ening corneal in ec ion ha ypically develops in pa ien s wi h a compromised corneal sur ace.

Predisposing Factors Con ac lens wear, especially ex endedwear so lenses Corneal rauma, oreign bodies Ocular sur ace disease (e.g., exposure/ neuro rophic kera opa hy, chronic bullous kera opa hy, dry eye syndrome, richiasis, dis ichiasis, en ropion) opical immunosuppressive herapy (e.g., cor icos eroids) Immunocompromised pa ien Pos opera ive: corneal wound or su urerela ed (e.g., corneal gra ) Etiology Staphylococcus Streptococcus Pseudomonas 168

Moraxella A ypical mycobac eria, o hers Symptoms Pain, irri a ion, redness, pho ophobia, discharge, decreased vision, con ac lens in olerance Signs Vary according o he severi y o he in ecion and, o a lesser ex en , he causa ive organism Whi e s romal inf l ra e associa ed wi h conjunc ival injec ion and ypically wi h an overlying epi helial de ec . T ere may be s romal loss (ulcer) ( Fig. 7-1A and B). T ere may be surrounding s romal edema, Desceme ’s olds, secondary reac ive iri is, and hypopyon ( Fig. 7-1C–H). S aphylococcal kera i is is charac erized by a well-def ned, whi e-gray or creamy s romal inf l ra e ha may enlarge o orm a dense s romal abscess. S rep ococcal kera i is may be suppura ive or have a crys alline appearance. Severe an erior uvei is and hypopyon orma ion are common.

Bacterial Keratitis

Pseudomonal kera i is ypically presen s as a rapidly progressive, suppura ive inf l ra e associa ed wi h hypopyon and a mucopurulen discharge. Corneal per ora ion may occur ( Fig. 7-1G). Dif erential Diagnosis S erile ulcers: vernal shield ulcer, neurorophic or exposure kera i is, au oimmune kera i is, con ac lens–induced s erile kerai is, medicamen osa kera i is. Usually less pain ul, minimal or no iri is or corneal edema, and cul ure is nega ive. S aphylococcal hypersensi ivi y kera i is: Inf l ra es may be bila eral; mul iple; peripheral; o en loca ed a he 2, 4, 8, or 10 o’clock posi ion; associa ed wi h blephari is; epi helial de ec is absen or is smaller han he inf lra e; and here is minimal an erior chamber ac ivi y. O her microbial (nonbac erial) kera i is: Bac erial cul ures are nega ive. Fungal and special cul ures and s ains are necessary or diagnosis. Diagnosis Corneal scraping or Gram’s s ain, Giemsa s ain, calco uor whi e s ain, cul ures, and sensi ivi y es ing. Rou ine media include blood, chocola e, Sabouraud’s agars, and hioglycola e bro hs. For deep lesions or when repea ed cul ures are nega ive in recalci ran cases, a corneal biopsy may be necessary. Treatment Empirical ou pa ien rea men wi h broad-spec rum, opical, non or if ed an ibio ic drops may be su cien or small (2 mm or less) peripheral ulcers wi h minimal sympoms and minimal an erior chamber ac ivi y. opical uoroquinolone (e.g., moxi oxacin, ga i oxacin, besi oxacin, levo oxacin, ciprooxacin, o oxacin) drops q30–60min around

169

he clock ini ially, a er a loading dose o 1 drop q5min or 15 minu es. For larger ulcers or when he ulcers involve he visual axis, or are associa ed wi h signif can discharge, an erior chamber ac ivi y, and hypopyon, rea men may require in ensive or if ed an ibio ic drops. Some pa ien s may need hospi aliza ion. For if ed ce azolin (50 mg/ mL) or vancomycin (25 mg/ mL) and or if ed gen amicin or obramycin (15 mg/ mL). Frequency o ins illa ion: 1 drop q5min or 30 minu es, hen q30–60min, o each drop. Wai 5 minu es be ween adminis ra ions o each medica ion. Subconjunc ival an ibio ics are necessary only i or if ed eye drops canno be s ar ed soon. Oral an ibio ics (e.g., moxi oxacin 400 mg q.d., cipro oxacin 500 mg b.i.d., or levo oxacin 500 mg q.d.) are help ul i he ulcer involves he sclera or has ex ended in o he eye. Sys emic an ibio ics are also required or Neisseria and Haemophilus in ec ion (e.g., ce riaxone 1 g IV or IM q12–24h). Cycloplegics are o en used o reduce ciliary spasm and o preven pos erior synechiae (e.g., scopolamine 0.25% or a ropine 1% .i.d.). Modi y regimen according o clinical response and cul ure and sensi ivi y resul s. opical cor icos eroids can be used or severe in amma ion only a er he organism is iden if ed and he in ec ion is under con rol. Urgen corneal ransplan a ion may be necessary in severe cases ha are progressing despi e aggressive rea men or or ulcers ha have per ora ed. Prognosis Close ollow-up is required. Prognosis is very good or small ulcers, good or modera e ulcers, poor or severe ulcers. Bet er prognosis or ulcers ou side he visual axis han ulcers in he visual axis.

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7 CO RNEAL INFEC IO NS, INFLAMMA IO NS, AND SURFACE DISO RDERS

A

B FIGURE 7-1. Bacterial keratitis. A.T is small in erior corneal inf l ra e in an overnigh so con ac lens wearer has some underlying corneal edema. Because i may be an early in ec ious kera i is, i should be rea ed wi h requen opical an ibio ics and ollowed closely. B.T is modera ely large, dense, paracen ral, con ac lens– rela ed corneal inf l ra e has an overlying epi helial de ec and surrounding edema. ( continued)

Bacterial Keratitis

171

C

D FIGURE 7-1. ( Continued) Bacterial keratitis. C. T is small o medium-sized paracen ral inf l ra e is modera ely dense. T e area o ac ive inf l ra ion is eleva ed as a resul o he in amma ory response. No e also he surrounding edema and he dis inc circular immune ring, bes seen cen rally. D.T is dense cen ral corneal ulcer has a large overlying epi helial de ec and modera e underlying corneal edema. T ere is a small hypopyon in eriorly, jus blun ing he in erior angle. ( continued)

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7 CO RNEAL INFEC IO NS, INFLAMMA IO NS, AND SURFACE DISO RDERS

E

F FIGURE 7-1. ( Continued) Bacterial keratitis. E.T is corneal in ec ion was due o Pseudomonas aeruginosa. T ere is a large circular corneal ulcer wi h overlying mucopurulen discharge, underlying corneal edema, and a modera ely large hypopyon. F.T is large dense corneal ulcer is associa ed wi h a hypopyon ha f lls approxima ely 50% o he an erior chamber. ( continued)

Bacterial Keratitis

173

G

H FIGURE 7-1. ( Continued) Bacterial keratitis. G.T is in ec ed corneal ulcer caused a per ora ion. Iris is plugging he wound. T e an erior chamber is shallow bu ormed. H. A o al corneal ulcera ion and epi helial de ec is presen in his eye. Sli -beam view demons ra es signif can cen ral hinning. T ere is no view o he an erior chamber.

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FUNGAL KERATITIS

F

ungal kera i is is a very serious, po enially sigh - hrea ening corneal in ec ion ha mos commonly develops in pa ien s a er rauma or in hose wi h a compromised corneal sur ace. Etiology Nonf lamen ous (e.g., Candida): Candida kera i is is a rare, unila eral, insidious ungal in ec ion ha usually occurs in eyes wi h preexis ing chronic corneal disease (e.g., dry eyes, herpes kera i is, exposure kera opa hy, pos kera oplas y, chronic use o cor icos eroid drops) or in severely debili a ed pa ien s. Fea ures include a gray-whi e s romal inf lra e similar o a bac erial ulcer. May have an an erior chamber reac ion and hypopyon ( Fig. 7-2A and B). Filamen ous (e.g., Aspergillus, Fusarium): Filamen ous kera i is is a rare, unila eral, insidious or aggressive ungal in ec ion ha requen ly a ec s normal eyes ollowing ocular rauma associa ed wi h vege a ive mat er and in wearers o so con ac lens. Fea ures include a grayish-whi e inf l ra e wi h indisinc ea hery borders, ypically surrounded by f ngerlike sa elli e inf l ra es in adjacen s roma. T e inf l ra es may ex end beyond he epi helial de ec . May have an associa ed ring inf l ra e, an erior chamber reac ion, and hypopyon ( Fig. 7-2C–F). Symptoms Pain, pho ophobia, earing, decreased vision; may have a his ory o rauma, con ac lens use or cor icos eroid eye drop usage Dif erential Diagnosis Fungal kera i is should be considered in he di eren ial diagnosis o bac erial or herpe ic kera i is ha does no respond o conven ional rea men or ha has an unusual his ory or suspicious appearance.

Diagnostic Evaluation His ory o rauma (which is o en minor) involving vege a ive mat er is highly sugges ive. Lack o response o conven ional an ibacerial herapy Corneal scrapings or Gram, Giemsa, calco uor whi e, or Gomori me henamine silver s ain, and cul ure (may ake up o a week or ungus o grow) Corneal biopsy may be required i smears and cul ures are nega ive. Treatment opical na amycin 5% (especially or f lamen ous ungi) and/ or ampho ericin B 0.15% (especially or Candida) q1h around he clock and aper over 4 o 6 weeks. Pa ien s may require hospi aliza ion ini ially. opical voriconazole 1% may also be e ec ive. Oral voriconazole 200 mg b.i.d. or i raconazole or uconazole 200 o 400 mg loading dose ollowed by 100 o 200 mg q.d. may be help ul in addi ion o he in ensive opical medica ions. Cycloplegics (e.g., scopolamine 0.25% or a ropine 1% .i.d.) Cor icos eroids are con raindica ed. Epi helial debridemen may acili a e opical herapy by enhancing pene ra ion o an i ungals. Modi y regimen according o clinical response and cul ure resul s. T erapeu ic corneal ransplan a ion may be necessary or unresponsive cases or per ora ed ulcers. Lamellar kera oplas y is rela ively con raindica ed because here is a high risk o recurrence o in ec ion. Prognosis Fair or mild o modera e in ec ions; poor or severe in ec ions

Fungal Keratitis

175

A

B FIGURE 7-2. Fungal keratitis. A. T is mul ilobula ed dense inf l ra e was caused by a Candida i n ec ion. T ere is an overlying epi helial de ec . Peripheral corneal neovasculariza ion sugges s ha i is a long-s anding ulcer. B.T is Candida corneal ulcer is slowly improving. T e denser inf l ra e a he in erior pupillary margin is surrounded by mul iple sa elli e lesions. ( continued)

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C

D FIGURE 7-2. ( Continued) Fungal keratitis. C.T is dense whi e inf l ra e wi h ea hery borders was a resul o a Fusarium in ec ion. A ring inf l ra e is beginning in eriorly. D.T is large cen ral pa chy corneal inf l ra e and hypopyon enlarged rapidly over several days, leading o an urgen corneal ransplan . His opa hology demons ra ed numerous f lamen ous ungi. ( continued)

Fungal Keratitis

177

E

F FIGURE 7-2. ( Continued) Fungal keratitis. E. Several mon hs a er removal o a corneal oreign body, a pa chy cen ral corneal inf l ra e is seen. T ere is also old in erior scarring and neovasculariza ion. Ini ial cul ures were nega ive. F. wo mon hs la er, he eye seen in E has worsened and has a much more dense cen ral inf l ra e. Cul ures a his poin grew Alternaria, which even ually responded o opical and oral voriconazole.

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ACANTHAMOEBA KERATITIS

A

canthamoeba kera i is is a rare parasi ic in ec ion o he cornea associa ed wi h he use o so con ac lenses and inadequa e con ac lens hygiene (e.g., using ap wa er or home-made saline solu ion, swimming or ho ub use while wearing con ac lenses), and occasionally, rauma. I should be considered in nonhealing, cul ure-nega ive kera i is. Etiology Acanthamoeba species Symptoms Severe pain ou o propor ion o severi y o kera i is, redness, earing, decreased vision, pho ophobia, minimal discharge. Symp oms ypically develop over a period o weeks, bu onse can be more rapid. His ory o so con ac lens use and occasionally rauma Signs Epi helial or subepi helial inf l ra es appearing as pseudodendri es early on ( Fig. 7-3A and B) Pa chy an erior s romal inf l ra es may be presen early on. Radial kera oneuri is ( Fig. 7-3C) A nonsuppura ive s romal ring inf l ra e, o en wi h variable epi helial breakdown, can develop over weeks. T e degree o in ammaion is dispropor iona ely mild rela ive o he amoun o pain ( Fig. 7-3D–F). In advanced cases, corneal hinning or per ora ion, scleri is, or hypopyon may develop.

Dif erential Diagnosis Herpes simplex kera i is Fungal kera i is Bac erial kera i is Diagnosis Pain dispropor iona e o severi y o in amma ion Lack o response o an ibac erial and an iviral herapy Ring inf l ra e and radial kera oneuri is are highly sugges ive. Corneal scrapings or Gram, Giemsa, or calco uor whi e s ain or amoebic cys s Cul ure on non-nu rien agar wi h Escherichia coli overlay or special media (e.g., bu ered charcoal yeas ex rac agar). Corneal biopsy may be necessary i smears and cul ures are nega ive. Treatment Polyhexame hylene biguanide (PHMB) 0.02% drops q1h. Chlorhexidine 0.02% can be used as an al erna ive o PHMB. Propamidine ise hiona e 1% (e.g., Brolene) drops q1h. Oral voriconazole 200 mg b.i.d. or i raconazole 200 o 400 mg q.d. may be used in addiion o he opical medica ions. O her drops (e.g., clo rimazole 1%) may be added, depending on he severi y or rea men response o he in ec ion. Cycloplegics (e.g., scopolamine 0.25% or a ropine 1% .i.d.) Low-dose opical cor icos eroids may be help ul in reducing in amma ion once he in ec ion appears o be under con rol. Oral nons eroidal an i-in amma ory agen s or narco ics or pain relie

Acanthamoeba Keratitis

Modi y regimen according o clinical response. Corneal ransplan a ion may be required i medical herapy ails, bu here is risk o recurrence.

179

Prognosis Fair o good i diagnosed and rea ed appropria ely wi hin he f rs mon h or so o developmen o symp oms; poor i signif can corneal involvemen is presen

A

B FIGURE 7-3. Acanthamoeba keratitis. A. A curvilinear, sligh ly eleva ed, “pseudodendri ic” epi helial irregulari y can be seen in his eye wi h con ac lens–rela ed Acanthamoeba kera i is. B.T is early Acanthamoeba in ec ion has several subepi helial inf l ra es in a linear pat ern reminiscen o a dendri e, hence he erm pseudodendri e. T ere was no rank epi helial de ec , bu here was epi helial irregulari y. ( continued)

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C

D FIGURE 7-3. ( Continued) Acanthamoeba k eratitis. C. Classic radial kera oneuri is is very apparen peripherally in his eye wi h con ac lens–rela ed Acanthamoeba kera i is. D.A er several weeks, a ring inf l ra e can develop, as can be seen especially superiorly. T ere is a small epi helial de ec in erocen rally. ( continued)

Acanthamoeba Keratitis

181

E

F FIGURE 7-3. ( Continued) Acanthamoeba keratitis. E. A large ring inf l ra e is presen in his eye. Despi e very aggressive medical rea men , his eye required a corneal ransplan . F.A er several mon hs o an iacan hamoeba rea men , his dense inf l ra e is f nally scarring. T e ac ive in ec ion even ually resolved, bu he eye was le wi h a signif can corneal scar.

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HERPES SIMPLEX KERATITIS

H

erpes simplex virus (HSV) in ec ion is an ex remely common condi ion ha a ec s a major propor ion o he popula ion, al hough mos in ec ions are subclinical. T e eyes may be a ec ed in primary ocular herpes or in recurren disease. Etiology HSV ype 1: causes in ec ion above he wais , especially o he ace, lips, and eyes. ransmit ed by close con ac . Much more common in he eye han ype 2 HSV ype 2: causes in ec ion below he wais , par icularly o he geni alia. ransmit ed sexually, bu neona es can be in ec ed during vaginal delivery. Uncommon in he eye

P IMA YOCULA HE PES Unila eral or bila eral acial and/ or eye in ec ion Etiology and Epidemiology Primary con ac wi h HSV Usually occurs in children or adolescen s Symptoms Fever, ulike symp oms Facial vesicular rash. Ocular redness, pain, decreased vision, and earing

Signs T ere may be vesicular blepharoconjunc ivi is or periorbi al derma i is. T e vesicles usually progress o orm crus s ( Fig. 7-4). T ere may be associa ed acu e ollicular conjunc ivi is wi h preauricular lymphadenopa hy. T e cornea may be involved in he orm o coarse macropunc a e epi helial kera i is or mul iple small branching epi helial dendri es wi hou s romal involvemen . Treatment Blepharoconjunc ivi is: ganciclovir (e.g., Zirgan) gel, ri uridine (e.g., Virop ic) drops, vidarabine (e.g., Vira-A) oin men , or acyclovir (e.g., Zovirax oph halmic) oin men f ve imes a day Corneal involvemen : ganciclovir (e.g. Zirgan) gel f ve imes a day or ri uridine drops (e.g., Virop ic) nine imes a day Consider acyclovir 200 o 400 mg PO f ve imes a day, valacyclovir 500 mg .i.d., or amciclovir 250 mg .i.d. or 7 o 14 days. Consider opical an ibio ic or acyclovir oin men o help heal skin lesions away rom he eyelid margin. Prognosis Good. T is is usually a benign and sel limi ed condi ion, bu he virus subsequen ly es ablishes a la en in ec ion in he rigeminal ganglion and may reac iva e, especially during periods o physical or emo ional s ress, causing recurren disease.

Herpes SimplexKeratitis

183

A

B FIGURE 7-4. Herpes simplex dermatitis. A.T is pa ien had recurren herpes simplex derma i is. No e he numerous ulcera ed skin lesions around he righ eye and cheek. T e righ eye appears uninvolved, bu i should receive prophylac ic an iviral rea men because o skin lesions on he eyelid margin. B. Mul iple ulcera ed skin lesions o herpes simplex can be seen in he upper eyelid. Con uen skin ulcera ions are presen in he lower eyelid wi h a mucoid discharge.

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ECU ENT OCULA HE PES SIMPLEX

R

ecurren ocular herpes may ake he orms o in ec ious epi helial kera i is, non-necro izing s romal kera i is (disci orm kera i is), necro izing s romal kera i is, neurorophic kera i is, and kera ouvei is.

Etiology and Epidemiology Recurren HSV is due o a reac iva ion o la en in ec ion in he rigeminal ganglion, especially during periods o physical or emoional s ress. I occurs in children and adul s. I is usually unila eral, bu i can be bila eral, especially in immunocompromised pa ien s and hose wi h a opy.

HSV: EPITHELIAL KER TITIS (DEND ITIC ULCE )

E

pi helial kera i is is a common, usually unila eral condi ion due o he presence o live virus wi hin corneal epi helial cells. Symptoms Unila eral redness, earing, irri a ion, decreased vision, pho ophobia, his ory o previous episodes Signs Single or mul iple branching, ulcera ing epi helial lesions wi h raised edges and erminal bulb orma ion ( Fig. 7-5A–C) Enlargemen o ulcers can lead o he orma ion o an amebic-shaped “geographic” ulcer ( Fig. 7-5D and E). T e ulcer bed s ains wi h uorescein. T e buil -up, swollen, opalescen margins o he lesion con aining virus-laden cells s ain wi h rose bengal. An erior s romal haze called “ghos dendri es” may develop below he epi helial lesions ( Fig. 7-5F). Corneal sensa ion is o en diminished.

Dif erential Diagnosis Herpes zos er kera i is: associa ed wi h a his ory o herpes zos er oph halmicus wi h ypical skin vesicles ound along derma omal dis ribu ion o he ace. May have eleva ed epi helial lesions wi h apered ends, which lack erminal bulbs. T e en ire “mucous plaque dendri e” s ains wi h rose bengal and mildly wi h uorescein. Prior o developmen o he ypical zos er rash, early zos er dendri es can look very similar o HSV dendri es. Acanthamoeba pseudodendri es Healing epi helial de ec s oxic epi heliopa hy Treatment Ganciclovir (e.g., Zirgan) gel f ve imes a day, ri uridine (e.g., Virop ic) drops q2h during he day, vidarabine (e.g., Vira-A) oin men f ve imes a day or acyclovir (e.g., Zovirax oph halmic) oin men f ve imes a day I he pa ien is already on cor icos eroids, he s eroids should be apered rapidly. Epi helial debridemen can help reduce viral load. I here is no response o rea men a er 1 week, hen poor compliance, resis ance o an iviral herapy, an iviral oxici y, or neurorophic disease should be considered. A shor course o sys emic acyclovir is unnecessary, because i does no preven subsequen developmen o s romal kera i is or uvei is, bu i can be used in place o requen opical an ivirals. Consider long- erm oral an iviral prophylaxis (e.g., acyclovir 400 mg b.i.d.) i a pa ien has had mul iple episodes o herpe ic eye disease. Prognosis Good, bu recurrences are common

Herpes SimplexKeratitis

185

A

B FIGURE 7-5. Herpes simplex keratitis. A. Fluorescein s aining wi h a cobal blue ligh o an ac ive herpes simplex epi helial dendri e. No e he “ ree branching” pat ern o he dendri e. T e cen ral bed s ains well wi h uorescein, while he eleva ed edges do no . T e ends o he dendri e have classic erminal “end bulbs.” B.R ose bengal dye on his ac ive dendri e s ains he heaped-up edges ha con ain virus-laden cells. ( continued)

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C

D FIGURE 7-5. ( Continued) Herpes simplex keratitis. C. A recurren epi helial dendri e is seen in his eye, which is 15 years s a us pos a corneal ransplan or herpes simplex kera i is scarring. D. T e cen ral area o a very large “geographic” lesion s ains readily wi h uorescein. T e edge o he epi helial de ec has herpes simplex dendri ic branching and erminal end bulbs. ( continued)

Herpes SimplexKeratitis

187

E

F FIGURE 7-5. ( Continued) Herpes simplex keratitis. E. Broad sli -beam view o he same eye highligh s he classic herpes simplex dendri ic ea ures. F.T is resolving epi helial dendri e barely s ains wi h uorescein. T ere is residual underlying corneal haze in he pat ern o he previous dendri e, o en ermed a “ghos dendri e.”

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HSV: NONNEC OTIZING ST OMAL KER TITIS (DISCIFO M KER TITIS)

D

isci orm kera i is is a primarily in amma ory condi ion caused by a hypersensi ivi y reac ion o he herpes simplex viral an igen in he cornea.

Acu e corneal hydrops o kera oconus Con ac lens overwear

Signs Cen ral disc o s romal and epi helial edema ( Fig. 7-6A and B) Small kera ic precipi a es localized o he underlying endo helium Folds in Desceme ’s membrane Surrounding s romal immune ring (Wessley ring) may be presen . T e limbal issue may be hickened and in amed (limbi is) ( Fig. 7-6C). An erior uvei is ( Fig. 7-6D and E) In raocular pressure may be eleva ed. Corneal sensa ion is ypically reduced.

Treatment I in amma ion is mild and vision is good, he condi ion can be observed. In more severe cases, opical cor icos eroids (e.g., prednisolone 1%, dexame hasone 0.1%, or lo eprednol 0.5% drops q.i.d.) can be s ar ed, main ained or several days o weeks, hen gradually apered over weeks or mon hs. O en, a very low dose o opical cor icos eroid (once or wice a week) may be required o preven recurren in amma ion. While on cor icos eroids more han once a day, concomi an oral an iviral herapy (e.g., acyclovir 400 mg b.i.d.) is o en used as prophylaxis. I an epi helial lesion is presen , i should be rea ed be ore s ar ing cor icos eroids. Recommend long- erm oral an iviral prophylaxis (e.g., acyclovir 400 mg b.i.d.) i a pa ien has had mul iple episodes o s romal kera i is.

Dif erential Diagnosis Herpes zos er disci orm kera i is Fuchs’ endo helial dys rophy

Prognosis Good. S romal scarring may occur and reduce vision ( Fig. 7-6F). O en recurs

Symptoms Unila eral redness, earing, irri a ion, blurred vision, pho ophobia, his ory o previous episodes

Herpes SimplexKeratitis

189

A

B FIGURE 7-6. Herpes simplex disci orm keratitis. A.T is eye has modera e cen ral corneal edema in a circular pat ern, hence he erm “disci orm.” T e sli -beam view demons ra es cen ral corneal hickening. T is disci orm kera i is represen s an in amma ory reac ion o previous herpes simplex in ec ion. I may resolve spon aneously, bu i o en responds ex remely well o opical cor icos eroids wi h an iviral coverage. B.T is eye has severe cen ral corneal edema wi h underlying kera ic precipi a es. ( continued)

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C

D FIGURE 7-6. ( Continued) Herpes simplex limbitis. C.T is eye, wi h a previous his ory o herpes simplex kera i is, has severe limbal in amma ion. No e he hickened, eleva ed limbal conjunc iva. T is limbi is responded o opical cor icos eroids and an iviral coverage. Herpes simplex iritis. D. Hundreds o granuloma ous kera ic precipi a es are presen in his eye wi h a his ory o previous herpes simplex kera i is. No e he ain cen ral corneal scarring o old herpes kera i is. O en he in raocular pressure is eleva ed in eyes wi h herpe ic iri is. Herpes simplex iri is responds o opical cor icos eroids wi h an iviral coverage. I o en benef s rom rea men wi h oral an iviral agen s in addi ion. ( continued)

Herpes SimplexKeratitis

191

E

F FIGURE 7-6. ( Continued) Herpes simplex keratitis. E. Re roillumina ion o he re ina reveals signif can iris s romal a rophy and iris ransillumina ion de ec s a er mul iple episodes o herpes simplex kera i is and iri is. F. A large, dense corneal scar wi h neovasculariza ion remains a er repea ed episodes o herpes simplex kera i is.

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HSV: NEC OTIZING ST OMAL KER TITIS

N

ecro izing s romal kera i is is unusual. I is mos likely caused by viral inf l ra ion and in amma ion o he corneal s roma.

Symptoms Unila eral redness, earing, irri a ion, blurred vision, pho ophobia, pain, his ory o previous episodes Signs Necro ic, cheesy, s romal inf l ra ion, usually associa ed wi h an epi helial de ec ( Fig. 7-7A) T e appearance o he inf l ra e can be con used wi h secondary bac erial or ungal kera i is. Corneal hinning, s romal neovascularizaion, scarring, or per ora ion may develop ( Fig. 7-7B). T ere may be associa ed kera ic precipia es, an erior uvei is, or hypopyon. In raocular pressure can be eleva ed even in he presence o minimal an erior chamber reac ion. Dif erential Diagnosis Primary or secondary bac erial or ungal kera i is: T ere is generally an overlying epi helial de ec . T ese condi ions should be considered when here is lack o response o an iviral rea men , and when here are increased or new signs o in ec ion and in amma ion.

Treatment T e f rs priori y is o rule ou a bac erial or ungal in ec ion and o rea any associa ed epi helial de ec . Once he epi helium has healed, opical cor icos eroids can be judiciously added o reduce s romal and an erior chamber in amma ion (e.g., prednisolone 1% or dexame hasone 0.1% drops q.i.d.), combined wi h opical or oral an iviral prophylaxis. Cor icos eroid drops should be apered gradually (s reng h and requency) over weeks or mon hs, depending on he level o in amma ion and he herapeu ic response. Cycloplegics (e.g., scopolamine 0.25% or cyclopen ola e 1% .i.d.). rea any eleva ed in raocular pressure. Avoid mio ics and pros aglandin analogs. Sys emic an iviral medica ions (e.g., acyclovir 400 mg f ve imes a day or weeks o mon hs) are ypically indica ed, especially when here is an erior uvei is. Corneal ransplan during acu e s ages o he in ec ion is discouraged because o he high ailure ra es Recommend long- erm oral an iviral prophylaxis (e.g., acyclovir 400 mg b.i.d.) i a pa ien has had pas episodes o s romal kera i is. Prognosis Fair. ypically, signif can s romal scarring remains, and i i is in he visual axis, i can severely a ec vision.

Herpes SimplexKeratitis

193

A

B FIGURE 7-7. Herpes simplex necrotizing keratitis. A. A necro izing s romal kera i is can be seen rom he 8 o’clock o 10 o’clock posi ions, reaching in o he visual axis. A prominen limbi is is presen . Old s romal scarring is presen superiorly and cen rally. B.T is necro izing herpes simplex kera i is caused a ull- hickness corneal mel and per ora ion. T is large per ora ion required an emergency pene ra ing kera oplas y.

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HSV: NEU OT OPHIC KER TITIS (METAHE PETIC KER TITIS)

N

euro rophic kera i is is no due o ac ive viral in ec ion bu is a healing problem caused by a combina ion o decreased sensa ion, drug oxici y, and a damaged basemen membrane rom epi helial in ec ion due o herpes. Symptoms Unila eral redness, earing, irri a ion, blurred vision, pho ophobia, his ory o previous episodes Signs Persis en epi helial de ec wi h heaped-up borders. T e edges do no s ain well wi h rose bengal. T e base s ains readily wi h uorescein ( Fig. 7-8). T ere may be mild o modera e corneal haze or scarring. Epi helial dendri es and erminal bulbs are absen .

May progress o corneal mel ing and per ora ion, especially i also using opical cor icos eroids Decreased corneal sensa ion Reac ive iri is may be presen . Dif erential Diagnosis Geographic herpes simplex ulcer: Edges have branching dendri ic ex ensions and s ain well wi h rose bengal. Treatment Discon inue oxic opical medica ions. O her measures o heal he epi helium are similar o hose or neuro rophic kera opa hy (see sec ion on Neuro rophic Kera opa hy in his chap er). Prognosis Good or small lesions, air or large lesions. O en corneal haze/ scar remains a er he epi helial de ec resolves.

Herpes SimplexKeratitis

195

FIGURE 7-8. Herpes simplex neurotrophic keratitis. A neuro rophic corneal ulcer is presen is his eye wi h recen herpes simplex kera i is. T e ac ive herpes in ec ion was rea ed wi h opical an iviral agen s and he ac ive dendri e resolved. However, he epi helial de ec remained, causing s romal hinning. No e he heaped-up edges o epi helium.

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HERPES ZOSTER KERATITIS

H

erpes zos er virus (HZV) in ec ion is caused by a reac iva ion o he chickenpox virus in he dorsal roo ganglion ha has migra ed down along he sensory nerves o a ec he skin o ha par icular derma ome. When he oph halmic division (V1) o he rigeminal nerve is involved, he condi ion is called herpes zos er oph halmicus. Etiology and Epidemiology Varicella zos er virus Unlike chickenpox, i is rare in children and ypically a ec s older pa ien s, bu i can also a ec young adul s, especially hose who are immunocompromised (e.g., hose wi h HIV, cancer). Symptoms Fever, malaise, and headache, which may precede he rash by a ew days. May be di cul o diagnose during he prodromal s age. Derma omal skin rash, ingling, burning, i ching sensa ion, pain Eye redness, irri a ion, earing, decreased vision, pho ophobia Signs Unila eral vesicular skin rash ha does no cross he midline ( Fig. 7-9A). T e rash subsequen ly orms blis ers and crus s ha heal wi h scarring. Hu chinson’s sign, vesicles on he ip o he nose, indica es presence o nasociliary nerve involvemen and may predic a higher risk o ocular disease. Periocular derma i is, conjunc ivi is, episcleri is, scleri is Corneal involvemen includes superf cial punc a e kera i is, microdendri ic kera iis, nummular kera i is, disci orm kera i is ( Fig. 7-9B), and neuro rophic kera i is.

Neuro rophic ulcers and persis en epi helial de ec s can lead o secondary in ec ion, hinning, and, even ually, corneal per ora ion ( Fig. 7-9C). Zos er pseudodendri es are eleva ed, “s uck-on” mucous-plaque lesions ha s ain wi h rose bengal bu do no s ain well wi h uorescein and do no have erminal end bulbs ( Fig. 7-9D and E). Iri is, glaucoma, re ini is, op ic neuri is, cranial nerve palsy, and cranial ar eri is may be seen. Pos herpe ic neuralgia (neuropa hic pain syndrome las ing longer han 3 mon hs a er he ini ial rash) may also develop. Dif erential Diagnosis Herpes simplex kera i is: Pa ien s are generally younger, have a his ory o recurren episodes, skin involvemen has no derma omal dis ribu ion, dendri es have erminal end bulbs, and he cen ral par s are depressed and s ain well wi h uorescein. Treatment Skin involvemen Oral acyclovir 800 mg f ve imes a day or amciclovir 500 mg .i.d. or valacyclovir 1000 mg .i.d. or 7 o 10 days o be s ar ed as soon as possible H2 an agonis (e.g., cime idine 400 mg b.i.d. PO) may reduce i ching and pain. An iviral (e.g., acyclovir) and/ or an ibac erial (e.g., e racycline, baci racin, or ery hromycin) oin men q.i.d. o he skin Conjunc ivi is and episcleri is T ese are sel -limi ed, and rea men is or symp oma ic relie . Cool compresses, ar if cial ears, or an ibio ic oin men (e.g., ery hromycin, baci racin, or e racycline) b.i.d. or .i.d.

Herpes Zoster Keratitis

Scleri is: Consider oral nons eroidal an iin amma ory agen s (e.g., urbipro en 100 mg .i.d.) or cor icos eroids (e.g., prednisolone 1 mg/ kg/ d q.d. or 2 weeks, hen aper) in severe cases. Disci orm kera i is: opical cor icos eroids in more severe cases (e.g., prednisolone 1%, dexame hasone 0.1%, or lo eprednol 0.5% our o eigh imes a day). aper slowly. A very low dose may be required chronically o preven recurrences. Pseudodendri es Generally sel -limi ed and rea ed wi h lubrica ion wi h ar if cial ear drops or oin men May respond o opical an iviral agen s (e.g., vidarabine oin men or ganciclovir gel), especially i he pa ien is immunocompromised Neuro rophic kera i is: See sec ion on Neuro rophic Kera opa hy in his chap er.

197

Re ini is, choroidi is, op ic neuri is: in ravenous acyclovir and oral cor icos eroids Pos herpe ic neuralgia: opical capsaicin or doxepin creams, sys emic neuropa hic pain medica ions (e.g., gabapen in or pregabalin), and/ or sys emic an idepressan medica ion may be help ul. In severe cases, re erral o a neurologis or pain specialis is indica ed. All pa ien s Con rol iri is wi h opical cor icos eroids. Con rol glaucoma. Beware o cor icos eroid-induced pressure rise as a possible cause. Prognosis Good o poor, depending on he severi y o he corneal involvemen ( Fig. 7-9F). Chronic recurren bou s o ocular in ammaion are common. Pos herpe ic neuralgia can be devas a ing in some pa ien s.

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7 CO RNEAL INFEC IO NS, INFLAMMA IO NS, AND SURFACE DISO RDERS

A FIGURE 7-9. Herpes zoster dermatitis. A. A herpes zos er derma i is (shingles) in ec ion in he V2 dis ribu ion. No e he ulcera ed skin vesicles. ( continued)

Herpes Zoster Keratitis

199

B

C FIGURE 7-9. ( Continued) Herpes zoster keratitis. B. Herpes zos er virus can cause a similar disci orm kera i is o herpes simplex virus. In his eye, cen ral corneal edema and mul iple small kera ic precipi a es are seen using he sli -bean view. Herpes zoster keratitis. C.S ignif can limbal in amma ion and an ac ive corneal mel are presen in his eye wi h a his ory o herpes zos er kera i is. I responded well o a conjunc ival ap procedure. ( continued)

200

7 CO RNEAL INFEC IO NS, INFLAMMA IO NS, AND SURFACE DISO RDERS

D

E FIGURE 7-9. ( Continued) Herpes zoster keratitis. D. Mul iple eleva ed, plaquelike pseudodendri es are presen in his eye several weeks a er a herpes zos er derma i is around his eye. E. Fluorescein s ain wi h a cobal blue ligh highligh s he pseudodendri es seen in D. No e ha hey lack he classic “ ree branch” pat ern, eleva ed edges, and erminal end bulbs o herpes simplex dendri es. (continued)

Herpes Zoster Keratitis

201

F FIGURE 7-9. ( Continued) Herpes zoster keratitis. F. Eigh years a er an episode o herpes zos er oph halmicus, here is signif can corneal neovasculariza ion and scarring. T ere is also some whi e lipid kera opa hy surrounding he larges blood vessels.

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7 CO RNEAL INFEC IO NS, INFLAMMA IO NS, AND SURFACE DISO RDERS

INTERSTITIAL KERATITIS ( SYPHILITIC, NONSYPHILITIC)

I

n ers i ial kera i is is an uncommon, bila eral in amma ion o he corneal s roma wi hou primary involvemen o he epi helium or endo helium. I has a wide varie y o causes.

Etiology Congeni al or acquired syphilis ( Treponema pallidum) Lyme disease ( Borrelia burgdorferi) uberculosis (Mycobacterium tuberculosis) Herpes simplex, herpes zos er, Eps einBarr, mumps virus Leprosy (Mycobacterium leprae) Cogan’s syndrome: inni us, ver igo, and dea ness. Associa ed wi h polyar eri is nodosa, Wegener granuloma osis, and rheuma oid ar hri is. Cogan’s syndrome is a rare condi ion. Symptoms Bila eral pain, redness, earing, blurred vision Nonspeci c Corneal Features Di use mids romal edema, neovasculariza ion, and nonsuppura ive inf l ra ion Mild iri is, kera ic precipi a es Inac ive signs include deep s romal scarring associa ed wi h nonper used (ghos ) vessels ( Fig. 7-10A–D) Speci c Ocular Features Syphilis Congeni al syphilis is associa ed wi h acu e in ers i ial kera i is in he f rs wo decades o li e. Acquired syphilis has ewer corneal f ndings and more pos erior segmen involvemen .

uberculosis: in ers i ial kera i is, phlycenulosis, granuloma ous iridocycli is, re inal vasculi is, choroidi is Lyme disease: enlarging skin rash (ery hema chronicum migrans), conjunc ivi is, episcleri is, in ers i ial kera i is ( Fig. 7-10E), granulomaous iridocycli is, in ermedia e uvei is, cranial nerve, re inal, and orbi al abnormali ies Leprosy: conjunc ivi is, episcleri is, scleri is, in ers i ial kera i is, hickened corneal nerves, corneal hypes hesia, granuloma ous iridocycli is, iris pearls, nodular leproma, iris a rophy, anisocoria, eyelid and lacrimal abnormali ies, ca arac , acial nerve palsy Diagnosis Syphilis Fluorescen reponemal an ibody absorp ion (F A-Abs) or microhemaggluina ion-Treponema pallidum (MHA- P) es (specif c es s or reponemal an ibodies). Usually remains posi ive or li e Venereal Disease Research Labora ory (VDRL) or rapid plasma reagin (RPR) es (nonspecif c es s are posi ive during acu e in ec ion). Use ul or screening and or moni oring ac ivi y o disease uberculosis: examina ion o spu um or acid- as bacilli, ches radiograph, purif ed pro ein deriva ive (PPD) skin es . New in ereron release assay (e.g., Quan iFERON- B Gold) may also be help ul. Lyme disease: his ory o exposure o deer, mouse, or ick bi e, direc immuno uorescen an ibody es Leprosy: skin scrapings or Ziehl-Neelsen s ain Cogan’s syndrome: Consul an o orhinolaryngologis . Treatment Kera ouvei is: opical cor icos eroids (prednisolone 1% or dexame hasone

Interstitial Keratitis (Syphilitic, Nonsyphilitic)

0.1% q2–4h) and cycloplegics (scopolamine 0.25% or cyclopen ola e 1% .i.d.) Syphilis, uberculosis, Lyme disease, leprosy: Re er o an in ernis , pulmonologis , or in ec ious disease specialis or appropria e rea men . Cogan’s syndrome: Re er o an o orhinolaryngologis or sys emic cor icos eroid

203

rea men o preven permanen hearing loss. Prognosis Generally good wi h appropria e rea men . Signif can permanen corneal scarring can resul and may require corneal ransplana ion when nonin amed.

A FIGURE 7-10. Interstitial keratitis. A.T is pa ien wi h a his ory o congeni al syphilis has old corneal s romal scarring. T e clearer lines are old blood vessels. T e scarring may be mid-s romal or pos erior s romal, and here is o en modera e corneal hinning. ( continued)

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7 CO RNEAL INFEC IO NS, INFLAMMA IO NS, AND SURFACE DISO RDERS

B

C FIGURE 7-10. ( Continued) Interstitial keratitis. B. Numerous corneal s romal “ghos ” vessels are seen. T e pa ien had a his ory o congeni al syphilis. C.T is le eye has signif can pa chy and ea herlike cen ral scarring rom in ers i ial kera i is. T ere are also many ghos vessels nasally. ( continued)

Interstitial Keratitis (Syphilitic, Nonsyphilitic)

205

D

E FIGURE 7-10. ( Continued) Interstitial keratitis. D. Sli -beam pho o o he same eye nicely demons ra es ha he scarring in in ers i ial kera i is is primarily pos erior. E. Nummular corneal inf l ra es are presen in his eye o a pa ien wi h Lyme disease. T e pa ien was rea ed sys emically or Lyme disease, and he inf l ra es responded o opical cor icos eroids.

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7 CO RNEAL INFEC IO NS, INFLAMMA IO NS, AND SURFACE DISO RDERS

SUBEPITHELIAL INFILTRATES

S

ubepi helial inf l ra es are a common unila eral or bila eral kera opa hy ha is ypically caused by viruses. Etiology

Adenovirus or Eps ein-Barr virus in ec ion Blephari is, ocular rosacea Con ac lens–induced reac ion, may be exacerba ed by preserva ives in lens care solu ions T ygeson’s superf cial punc a e kera opa hy S aphylococcal or chlamydial in ec ions Lyme disease Epi helial corneal gra rejec ion in donor cornea (Krachmer’s spo s) Symptoms Pho ophobia, oreign-body sensa ion, redness, earing, mild blurring o vision, may be asymp oma ic Signs Few or mul iple, unila eral or bila eral, granular, small, oval or circular, subepi helial

opaci ies, which may s ain wi h rose bengal bu s ain poorly wi h uorescein ( Fig. 7-11) Dif erential Diagnosis Superf cial punc a e kera opa hy: mul iple punc a e epi helial de ec s ha s ain well wi h uorescein bu no wi h rose bengal Treatment rea underlying condi ion. Discon inue con ac lens wear. Preserva ive- ree ar if cial ear drops or gels q2–6h and lubrica ing oin men q.h.s. or b.i.d. Mild opical cor icos eroid (e.g., lo eprednol 0.2% or uorome holone drops 0.1% q.i.d.) i vision is a ec ed or symp oms are severe. May require a slow aper o preven recurrence Prognosis Good, especially i underlying condi ion can be iden if ed. When cor icos eroids are used, hey o en mus be apered very slowly o preven recurrence. Long-s anding subepi helial inf l ra es can resul in permanen scars.

A

FIGURE 7-11. Subepithelial inf ltrates a er viral conjunctivitis. A.T is eye has numerous ac ive cen ral subepi helial inf l ra es a er adenoviral kera oconjunc ivi is. T ese responded well o opical s eroids. ( continued)

Subepithelial Inf ltrates

207

B

C FIGURE 7-11. ( Continued) Subepithelial inf ltrates a er viral conjunctivitis. B. Over 100 ac ive subepi helial inf l ra es recurred a er opical s eroids were apered rapidly. T ey improved grea ly on a slower s eroid aper. Some eyes need o be on low-dose opical s eroids or mon hs o years o preven recurrence. C.M ul iple subepi helial inf l ra es can be seen cen rally in his eye many mon hs a er adenoviral kera oconjunc ivi is causing glare, halos, and decreased vision. Some o hese inf l ra es appear sligh ly ac ive, while o hers appear mores carred.

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7 CO RNEAL INFEC IO NS, INFLAMMA IO NS, AND SURFACE DISO RDERS

SUPERFICIAL PUNCTATE KERATOPATHY ( PUNCTATE EPITHELIAL EROSIONS)

S

uperf cial punc a e kera opa hy is a common nonspecif c f nding seen in a wide varie y o corneal disorders. Etiology Primarily superior Sub arsal oreign body or concre ions Vernal kera oconjunc ivi is Superior limbic kera oconjunc ivi is rachoma Poorly f t ing con ac lens Floppy eyelid syndrome richiasis or dis ichiasis Primarily in erpalpebral Kera oconjunc ivi is sicca Neuro rophic kera opa hy Exposure o ul raviole ligh Con ac lens–rela ed (chemical oxici y, igh lens syndrome, overwear syndrome) Primarily in erior Rosacea, blephari is Exposure kera opa hy, including nocurnal lagoph halmos Lower eyelid margin lesions oxici y rom drops or chemical injury En ropion or ec ropion richiasis or dis ichiasis

rauma, chemical injury Sel -in ic ed, eye rubbing Symptoms Foreign-body sensa ion, earing, and decreased o vision i cen ral cornea is a ec ed Signs Mul iple, iny, pinpoin epi helial de ec s ha s ain well wi h uorescein. T ey may be con uen i severe ( Fig. 7-12). Dif erential Diagnosis Subepi helial inf l ra es: ew or mul iple, unila eral or bila eral, granular, small epi helial or subepi helial opaci ies ha may s ain wi h rose bengal bu s ain poorly wi h uorescein Treatment rea underlying disorder. Discon inue con ac lens wear and oxic medica ions. Preserva ive- ree ar if cial ear drops or gels q1–6h and lubrica ing oin men q.h.s. or b.i.d., depending on severi y. Consider puncal occlusion wi h plugs or cau ery. opical an ibio ic oin men (e.g., ery hromycin, baci racin, polymyxin B/ baci racin, or e racycline) wo o our imes a day may be added. Avoid opical medica ions con aining preserva ives. Prognosis Generally good, bu depends on he underlying condi ion.

Superfcial Punctate Keratopathy (Punctate Epithelial Erosions)

209

A

B FIGURE 7-12. Superf cial punctate keratopathy. A. Fluorescein dye and cobal blue ligh reveal signif can in erior corneal punc a e s aining. B. Fluorescein dye and cobal blue ligh demons ra e severe cen ral punc a e s aining. T e s aining is almos con uen in eriorly.

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7 CO RNEAL INFEC IO NS, INFLAMMA IO NS, AND SURFACE DISO RDERS

THYGESON’S SUPERFICIAL PUNCTATE KERATOPATHY

T e conjunc iva is no injec ed, and he an erior chamber is quie .

T

Treatment Ar if cial ear drops q2–6h and lubrica ing oin men q.h.s. or b.i.d., depending on severi y Mild opical cor icos eroid (e.g., lo eprednol 0.2% o 0.5% or uorome holone drops 0.1% q.i.d.) i vision is a ec ed or symp oms are severe. Cor icos eroid drops can usually be apered, bu recurrence is common. Pa ien s may need long- erm low-dose cor icos eroids o remain asymp oma ic. T erapeu ic so con ac lenses may provide symp oma ic relie i cor icos eroid herapy ails or is con raindica ed. opical cyclosporine drops have been repor ed o help in cer ain pa ien s and may allow he dosage o opical cor icos eroid o be reduced.

hygeson’s superf cial punc a e kera opahy is an uncommon, usually bila eral, idiopa hic condi ion ha has a chronic course wi h exacerba ions and remissions. I may resolve spon aneously a er many years.

Etiology Unknown. Some physicians believe i is o viral origin. Symptoms Pho ophobia, oreign-body sensa ion, earing, mildly decreased vision, may be asymp oma ic Signs A ew o hundreds o course, punc a e or s ella e, round o oval grayish-whi e clus ers o epi helial lesions ha occur hroughou he cornea, are sligh ly eleva ed, and s ain minimally wi h uorescein ( Fig. 7-13)

Prognosis Good or signif can ly improved symp oms, bu he condi ion is o en chronic and recurren .

T ygeson’s Superfcial Punctate Keratopathy

211

A

B FIGURE 7-13. T ygeson’s superf cial punctate keratopathy. A. Mul iple small epi helial lesions are presen in he cen ral cornea. T ey may s ain minimally wi h uorescein dye. B. T e same eye seen wi h re roillumina ion o he re ina. No e he coarse, sligh ly s ella e na ure o he epi helial lesions.

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7 CO RNEAL INFEC IO NS, INFLAMMA IO NS, AND SURFACE DISO RDERS

KERATO CONJUNCTIVITIS SICCA ( DRY EYE SYNDROME)

K

era oconjunc ivi is sicca re ers o a dry eye syndrome ha commonly causes chronic low-grade irri a ion o he eyes. I is caused primarily by aqueous ear def ciency. I is o en exacerba ed by blephari is and meibomi is.

Etiology Sjögren’s syndrome (primary or secondary) Drugs (e.g., an ihis amines, be a-blockers, a ropine) Collagen vascular diseases (e.g., rheumaoid ar hri is, sys emic lupus ery hema osus, polymyosi is) Conjunc ival scarring (e.g., ocular cica ricial pemphigoid, S evens-Johnson syndrome, rachoma, chemical injuries, chronic con ac lens wear) Lacrimal gland des ruc ion (e.g., umor, sarcoidosis, surgical removal, radia ion) Vi amin A def ciency (xeroph halmia) Idiopa hic (involu ional) Symptoms Burning, oreign-body sensa ion, dryness, i ching, ired eeling, mucous discharge Paradoxically, some pa ien s complain o episodes o earing, which is likely re ex earing rom severe kera opa hy. May complain o poor and/ or uc ua ing vision Symp oms end o be worse a he end o he day, wi h prolonged reading or compu er use or elevision viewing, in dry or dus y environmen s, and during con ac lens wear. Signs Reduced or absen ear meniscus

In erpalpebral s aining o conjunc iva and cornea wi h uorescein, rose bengal, or lissamine green ( Fig. 7-14A) Corneal f lamen s and mucous plaques May have corneal neovasculariza ion, hinning, scarring, ulcera ion or per ora ion, especially when associa ed wi h collagen vascular diseases ( Fig. 7-14B and C) Dif erential Diagnosis O her causes o superf cial punc a e keraopa hy (see sec ion on Superf cial Punc a e Kera opa hy, above) Diagnosis Kera oconjunc ivi is sicca is a clinical diagnosis based on a combina ion o his ory, clinical signs, and ancillary es s. Abnormal Schirmer es ( 3 mon hs) Course o disease Acu e (sudden onse and limi ed dura ion) Chronic ( persis en uvei is wi h relapse 251

Dense opaci ies

No endorsed by SUN. Adap ed rom Nussenblat RB, Whi cup SM. Uveitis: Fundamentals and Clinical Practice. 4 h ed. Loca ion: Elsevier; 2010.

SUN TERMINOLO GY FOR ACTIVIT Y OF UVEITIS

A

l hough he ul ima e goal o uvei is rea men is o suppress in amma ion comple ely, i is also impor an o assess shor - erm changes in in amma ion, especially when evalua ing he e cacy o rea men . Inac ive uvei is is def ned as: Zero o rare an erior chamber cells (less han one cell per high-power f eld) T ere was no consensus or def ni ion o inac ive vi ri is on he basic o vi reous cells. Worsening ac ivi y wo-s ep increase in in amma ion (an erior chamber cell or vi reous haze) or increase rom grade 3+ o 4+

12

2 ANATO MIC CLASSIFICATIO N O F UVEITIS

Improved ac ivi y wo-s ep decrease in in amma ion (an erior chamber cell or vi reous haze) or decrease o grade 0 Remission Inac ive disease or 3 mon hs or more a er discon inuing all rea men s or eye disease Def ni ion o success ul cor icos eroid sparing ou comes For adul s, long- erm s eroid use should be limi ed o 10 mg per day or less.

REFERENCES Davis JL, Madow B, Cornet J, e al. Scale or pho ographic grading o vi reous haze in uvei is. Am J Ophthalmol. 2010;150:637–641. Jabs DA, Nussenblat RB, Rosenbaum J . S andardiza ion o Uvei is Nomencla ure (SUN) Working Group. S andardiza ion o uvei is nomencla ure or repor ing clinical da a. Resul s o he Firs In erna ional Workshop. Am J Ophthalmol. 2005;140( 3):509– 516. Nussenblat RB, Pales ine AG, Chan CC, e al. S andardiza ion o vi real in amma ory ac ivi y in in ermedia e and pos erior uvei is. Ophthalmology. 1985;92( 4):467–471. Nussenblat RB, Whi cup SM. Uveitis: Fundamentals and Clinical Practice, 4 h ed. Philadelphia: Elsevier; 2010.

C H AP T ER

Episcleritis, Scleritis, and Keratitis EPISCLERITIS

injected, and the redness can be sectoral

eresa Larson and H. Nida Sen

E

piscleritis is a self-limited, generally benign

Etiology and Epidemiology Episcleritis occurs most frequently in young to middle-aged women (20 to 40 years old). It is frequently unilateral (70%). e etiology is unknown in most cases, but it is believed to be immune mediated, and it is occasionally associated with systemic disease. Symptoms Redness, minimal eye pain Foreign-body sensation Minimal to no changes in vision Signs Bright red or salmon-pink color in natural light ( Fig. 3-1) is contribution to the work was done as part of the the United States Government.

ontipped applicator and blanch with topical administration of 10% phenylephrine. Small peripheral corneal opacities are present in 10% of cases. erential Diagnosis Scleritis In contrast to scleritis, episcleritis has minimal pain, less common systemic disease association, and essentially no complications. Pinguecula Phlyctenule Subconjunctival hemorrhage Conjunctival neoplasia Anterior uveitis Diagnostic Evaluation Although systemic disease is not commonly associated with episcleritis, a thorough review of systems and targeted systemic 13

14

3 EPISCLERITIS, SCLERITIS, AND KERATITIS

workup is indica ed. Sys emic workup can be considered in recurren cases. Up o 30% o pa ien s are ound o have an underlying disease associa ion. Treatment Episodes are sel -limi ed and rarely require rea men beyond ar if cial ears, opical NSAIDs, and opical cor icos eroids. I he episcleri is does no resolve promp ly wi h herapy, consider ano her diagnosis.

A

Prognosis T e prognosis is usually very good. Episodes may recur bu rarely are here las ing sequelae. REFERENCES Fos er CS, and Sainz de la Maza M. Clinical considera ion o episcleri is and scleri is. In T e Sclera. New York: Springer-Verlag; 1994:107. Jabs DA, Mudun A, Dunn JP, e al. Episcleri is and scleri is: clinical ea ures and rea men resul s. Oph halmology. 2000;130:469–476. Sainz de la Maza M, Jabbur NS, Fos er CS. Severi y o scleriis and episcleri is. Oph halmology. 1994;101:389–396.

B

FIGURE 3-1. Episcleritis. A.T is patient has episcleritis with a salmon pink hue. B.T e same eye with blanching o the episclera af er administration o 10% phenylephrine.

Scleri is

SCLERITIS T eresa Larson and H. Nida Sen

S

cleri is is charac erized by inf amma ion and edema o scleral and episcleral issue. I is classi ed as an erior or pos erior, and ur her subclassi ed as di use, nodular, and necro izing ( Table 3-1 ).

ANTERIOR SCLERITIS Etiology and Epidemiology An erior scleri is is he mos common orm, making up 80% o 85% o all scleri is cases. Di use and nodular scleri is occur wi h equal requency. Scleri is occurs mos requen ly in middleaged women (40 o 60 years old) 25% o 50% o pa ien s have a his ory o sys emic disease, mos commonly rheumaoid ar hri is ( Table 3-2). Necro izing scleri is is he mos severe and des ruc ive orm o scleri is and is mos o en associa ed wi h sigh - hrea ening sequelae. Necro izing scleri is is divided in o “wi h inf amma ion” and “wi hou inf amma ion” (scleromalacia per orans). TABLE 3-1. Scleritis Clinical Subtypes and T eir Prevalence An erior scleri is Dif use Nodular

80%–85% 40%–45% 40%

Necro izing scleri is Wi h in amma ion Wi hou in amma ion (scleromalacia per orans)

10%–15% 10% 1%–5%

Pos erior scleri is

1%–5%

Adap ed rom Nussenblat RB, Whi cup SM. Uveitis: Fundamentals and Clinical Practice, 4 h ed. Philadelphia: Elsevier; 2010. T is con ribu ion o he work was done as par o he au hors’ o cial du ies as NIH employees and is a work o he Uni ed S a es Governmen .

15

Symptoms Pa ien s presen wi h a red, pain ul eye. T ey may have a boring eye pain. Vision loss is rare. Pa ien s wi h scleromalacia per orans ypically have no pain. Signs he sclera has a violaceous hue in na ural ligh wi h in lamed vessels ha are immobile wi h a co on- ipped applica or ( Fig. 3-2). I is easier o apprecia e scleri is by looking a he eye wi hou he sli lamp. Areas o repea ed at acks may demons ra e scleral hinning wi h a bluish hue. Eyes wi h di use scleri is have generalized edema, while eyes wi h sec oral or nodular scleri is have localized ery hema ( Fig. 3-3). Necro izing scleri is has a whi e avascular area surrounded by injec ion and edema ( Fig. 3-4). opical (10%) phenylephrine will no blanch he vessels in scleri is. Dif erential Diagnosis Episcleri is Subconjunc ival hemorrhage Conjunc ival mucosa–associa ed lymphoid issue lymphoma Sen inel vessels An erior uvei is or panuvei is Acu e angle-closure glaucoma Kera i is Endoph halmi is Caro id or dural sinus s ula Diagnostic Evaluation es ing is guided by his ory and review o sys ems. Rheuma oid ac or, an i-ci rullina ed cyclic pro ein (CCP), classical

16

3 EPISCLERITIS, SCLERITIS, AND KERATITIS

TABLE 3-2. Systemic Disease Associations Noninfectious

Infectious

Other

Connective tissue disease Rheumatoid arthritis Juvenile rheumatoid arthritis Reiter’s syndrome Systemic lupus erythematosus Relapsing polychondritis Polymyositis Infammatory bowel disease Spondyloarthropathy

Herpes zoster ophthalmicus Herpes simplex keratitis Acanthamoeba keratitis Syphilis Lyme disease Bartonellosis Tuberculosis

Gout Rosacea Foreign body reaction Drugs (bisphosphonates)

Vasculitides Wegner’s granulomatosis Polyarteritis nodosa Allergic angiitis o Churg-Strauss Cogan syndrome Takayasu disease Adamantiades—Behçet’s disease Sarcoidosis

an ineu rophil cy oplasmic an ibody (c-ANCA), pro oplasmic-s aining an ineurophil cy oplasmic an ibodies (p-ANCA), myeloperoxidase, an inuclear an ibody (ANA), and an i-dsDNA may be considered i an underlying rheuma ologic disease is suspec ed. Rule ou in ec ious causes wi h he appropria e es s, including: uorescen reponemal an ibody absorp ion (F A-Abs), rapid plasma reagin (RPR) or Venereal Disease Research Labora ory (VDRL), purif ed pro ein deriva ive (PPD) and/ or Quan iFERON, rauma, and oreign body. Orbi al C should be considered in a ypical cases. Treatment Oral NSAIDs or milder (nonnecro izing) scleri is cases Nodular scleri is o en can respond o an injec ion o riamcinolone over he nodule. Periocular riamcinolone can also be used selec ively or cases o di use scleri is.

Oral prednisone Immunosuppressive herapy is indica ed or recurren or severe cases (par icularly or necro izing scleri is). ANCA posi ive pa ien s may have a more severe course o disease and hus require more aggressive herapy. In pa ien s wi h necro izing scleri is associa ed wi h a sys emic disorder such as rheuma oid ar hri is ( Fig. 3-5) or Wegener’s granuloma osis, aggressive sys emic herapy is necessary because i is associa ed wi h a high mor ali y ra e wi hou sys emic rea men . Prognosis he prognosis varies based on he si e o in lamma ion, associa ed complica ions, underlying condi ions, and response o herapy. Di use an erior scleri is has he bes prognosis, whereas necro izing scleriis has he wors prognosis wi h he highes ra es o visual loss and complica ions.

Scleritis

REFERENCES Albini A, Zamir E, Read RW, e al. Evalua ion o subconjunc ival riamcinolone or nonnecro izing an erior scleri is. Oph halmology. 2005;112( 10): 1814–1820. Fos er CS, Sainz de la Maza M. Clinical considera ion o episcleri is and scleri is. In T e Sclera. New York: Springer-Verlag; 1994:107.

A

17

Fos er CS, Fors o SL, Wilson LA. Mor ali y ra e in rheuma oid ar hri is pa ien s developing necro izing scleri is or peripheral ulcera ive kera i is: e ec s o immunosuppression. Oph halmology. 1984;91: 1253–1263. Jabs DA, Mudun A, Dunn JP, e al. Episcleri is and scleri is: clinical ea ures and rea men resul s. Oph halmology. 2000;130:469–476.

B

FIGURE 3-2. A.D i use anterior scleritis is characterized by in ammation o the deep scleral and episcleral vessels, which have a dark red/ violaceous hue. B. Di use anterior scleritis with in erotemporal thinning. Note the scleral injection and adjacent scleral thinning evidenced by the blue color o the underlying choroid.

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3 EPISCLERITIS, SCLERITIS, AND KERATITIS

FIGURE 3-3. Nodular scleritis and peripheral ulcerative keratitis. T e immobile nodule is surrounded by scleral injection. T ere is a ocal, peripheral corneal opacity resulting rom past episodes o peripheral ulcerative keratitis.

FIGURE 3-4. Necrotizing scleritis in a patient with Wegener’s granulomatosis with one suture remaining rom a past scleral biopsy. Note the white, avascular patch o sclera with surrounding scleritis.

FIGURE 3-5.T is elderly woman had poorly treated rheumatoid arthritis that caused painless scleral thinning through which the underlying blue choroid is visible (necrotizing scleritis without in ammation, or scleromalacia per orans) . (Courtesy o Sunir J. Garg, MD.)

Scleritis

POSTERIOR SCLERITIS

I

is an in amma ory disease o he sclera ha begins pos erior o he spiral o illaux/ ora serra a and involves he pos erior aspec o he eye. Etiology and Epidemiology Pos erior scleri is is more common in women. I has he lowes incidence o he various sub ypes o scleri is; however, i is o en underrecognized because o i s many mani es a ions. Symptoms Pa ien s o en presen wi h an “achy, deep” pain, decreased vision, and redness. Signs Unlike an erior scleri is, he vision is o en a ec ed. Pain Eleva ed in raocular pressure, associa ed an erior scleri is, a swollen op ic disc, choroidal olds, serous re inal de achmen , and/ or a subre inal mass or lesion ( Fig. 3-6) -sign on B-scan ul rasound ( Fig. 3-7) Dif erential Diagnosis Choroidal umors Uveal e usion syndrome Rhegma ogenous re inal de achmen Vog -Koyanagi-Harada syndrome Cen ral serous choriore inopa hy Op ic neuri is Masquerade syndromes (lymphoma, me as a ic carcinoma, choroidal melanoma) Diagnostic Evaluation Labora ory evalua ion or an underlying sysemic disease is warran ed based on he resul s o a horough his ory and review o sys ems.

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Rule ou rea able in ec ious causes such as syphilis and uberculosis (RPR, VDRL, F A-Abs, PPD, Quan i eron) B-scan: T is is cri ical o es ablish he diagnosis. I demons ra es scleral wall hickness >2 mm in ei her a di use or nodular ashion. Classically, a -sign ha represen s a sonographically emp y space due o edema surrounding enon’s capsule and he op ic nerve is seen. Fluorescein angiography can be used o rule ou o her causes such as Vog -KoyanagiHarada syndrome and sarcoidosis. Treatment Mos pa ien s respond o oral NSAIDs; however, hose wi h more severe chronic disease will require more aggressive sys emic herapy wi h cor icos eroids and/ or immunosuppressive herapy. Prognosis T e prognosis depends on he imeliness o rea men and severi y o disease. Pa ien s older han 50 years, pa ien s wi h an associa ed sys emic disease, and pa ien s requiring more aggressive rea men have a grea er risk o vision loss. REFERENCES Benson WE. Pos erior scleri is (review) . Surv Oph halmol. 1988;32( 5):297–316. Fos er CS, Sainz de la Maza M. Clinical considera ion o episcleri is and scleri is. In T e Sclera. New York: Springer-Verlag; 1994:107. Fos er CS, Fors o SL, Wilson LA. Mor ali y ra e in rheuma oid ar hri is pa ien s developing necro izing scleri is or peripheral ulcera ive kera i is: e ec s o immunosuppression. Oph halmology. 1984;91:1253–1263. Jabs DA, Mudun A, Dunn JP, e al. Episcleri is and scleriis: clinical ea ures and rea men resul s. Oph halmology. 2000;130:469–476. McCluskey PJ, Wa son PG, Ligh man S, e al. Pos erior scleri is: clinical ea ures, sys emic associa ions, and ou come in a large series o pa ien s. Oph halmology. 1999;106:2380–2386.

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3 EPISCLERITIS, SCLERITIS, AND KERATITIS

B

FIGURE 3-6.T is patient has posterior scleritis. A.T ere are horizontal choroidal olds consistent with a mass (in this cases scleral inf ammation) behind the globe. B.T e f uorescein angiogram shows the choroidal striations. (Courtesy o William Benson, MD.)

A

B

C

D

FIGURE 3-7. Posterior scleritis causes disruption o the normal choroidal circulation as well as restriction o scleral outf ow through the vortex veins. T is causes subsequent dys unction o the retinal pigment epithelium. A.T is can result in a serious retinal detachment. Fluorescein angiography can demonstrate pinpoint choroidal leakage ( B) with pooling o dye in the late rames ( C) . D. B-scan ultrasonography demonstrates thickening o the sclera and choroid ( arrows) . Edema o enon’s capsule behind the globe, and along the optic nerve, creates the -sign. (Courtesy o William Benson, MD, and Eliza Hoskins, MD.)

Phylectenulosis

PHYLECTENULOSIS S. R. Ra hinam

P

hlyc enular kera oconjunc ivi is is a nonspecif c, delayed hypersensi ivi y ( ype IV) reac ion o he cornea and/ or conjunc iva oward a varie y o an igens.

Etiology and Epidemiology Phlyc enulosis is seen in he f rs wo decades o li e. I is more common in persons wi h poor personal hygiene and in hose o lower socioeconomic s a us. I is o en associa ed wi h chronic meibomi is and chalazia. Less commonly, i has been associa ed wi h pulmonary and ex ra pulmonary uberculosis, s aphylococcal in ec ion, worm in esa ion, and ocal sepsis.

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Foreign-body granuloma Scleral abscess Diagnostic Evaluation Examina ion o he lid margin or blephari is, meibomi is, and chalazia. In coun ries such as India, consider: Sys emic workup or pulmonary/ ex rapulmonary uberculosis Screening or palpable lymph node o biopsy Pulmonary radiological s udies o rule ou uberculosis S ool examina ion or worm in es a ion

Signs Phlyc enulosis is a ocal in amma ory disease charac erized by an eleva ed, ranslucen nodule or vesicle wi h an ulcera ed summi surrounded by a zone o hyperemia ( Figs. 3-8 and 3-9). O en, conjunc ival phlyc ens are ransien and asymp oma ic. However, larger phlyc ens can resul in rank pus ular conjunc ivi is. I he corneal phlyc en migra es rom i s limbal origin o he cen ral cornea, i is called fascicular kera i is, which causes severe vision loss.

Treatment T e primary rea men is good lid hygiene o elimina e s aphylococcal lid in ec ion; his includes warm mois compresses, lid scrubs wi h baby shampoo, and opical ery hromycin eye oin men . I ano her sys emic cause is ound, rea men o he underlying disease is essen ial: I due o a parasi e, albendazole 400 mg/ day can be used. In cases o uberculosis, mul idrug an iubercular rea men (ri ampicin 10 mg/ kg/ day, isoniazid 5 mg/ kg/ day q.d. or 6 o 9 mon hs, e hambu ol 15 mg/ kg/ day, and pyrazinamide 25 o 30 mg/ kg/ day q.d. or f rs 2 mon hs should be considered). Prednisolone ace a e 1% or dexame hasone 0.1% every wo hours or 1 week ollowed by a slow aper has ens resolu ion. In severe cases, or hose ha require longer- erm s eroid use, opical cyclosporine A 2% has been ound o be e ec ive.

Dif erential Diagnosis Pingueculum Episcleri is

Prognosis Conjunc ival phlyc ens heal wi hou scarring and carry a good prognosis.

Symptoms Lacrima ion, pho ophobia, decreased vision, and blepharospasm

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3 EPISCLERITIS, SCLERITIS, AND KERATITIS

Corneal phlyctens may cause stromal scarring, which can cause vision loss ( Fig. 3-10). Recurrences are more common in patients with tuberculosis ( Fig. 3-11).

REFERENCE Doan S, Gabison E, Gatinel D, et al. Topical cyclosporine A in severe steroid-dependent childhood phlyctenular keratoconjunctivitis. Am J Ophthalmol. 2006;141: 62–66.

FIGURE 3-8.T is is a typical, moderate to severe phlyctenule with an elevated, translucent nodule with an ulcerated summit surrounded by a ring o hyperemia.

A

B

D

C

FIGURE 3-9.T is child had bilateral phlyctenules. T ere is a small, elevated vesicle with surrounding hyperemia on the right ( A) and lef ( B) eyes. C. Given the lack o eyelid disease, a search or a systemic cause was undertaken. T e child had a positive Mantoux reaction. D.A f er receiving systemic antitubercular treatments and topical steroids, there was resolution o symptoms.

Phylectenulosis

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FIGURE 3-10. Multiple healed limbal phlyctenules with residual corneal scarring.

FIGURE 3-11.T is woman has an active phlyctenule in her lef eye and a healed cervical tubercular sinus, which has lef a scar above the lef clavicle. reatment o her underlying tuberculosis with concomitant topical steroids caused resolution o her eye disease.

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3 EPISCLERITIS, SCLERITIS, AND KERATITIS

HERPETIC KERATOUVEITIS Pa rick Prendergas and William Hodge

I

n amma ion o he cornea and uveal rac arising rom in ec ion wi h varicella zos er virus (VZV) or herpes simplex virus (HSV) in ec ion is a common cause o an erior uvei is.

HERPES SIMPLEX VIRUS Etiology and Epidemiology Nearly all people are in ec ed wi h HSV 1 and 2 during heir li e ime. T e ini ial in ecion is usually asymp oma ic. T e virus can hen become a la en in ec ion, o en residing in he rigeminal nerve ganglion. I can hen reac iva e a any ime along one o he rigeminal nerves, including hose ha go o he eye. Herpes simplex kera ouvei is mos commonly resul s rom reac iva ion o la en in ec ion, and is he mos common cause o in ec ious uvei is. I presen s a all ages and occurs in bo h sexes wi h equal requency. HSV is he leading cause o corneal blindness in developed coun ries. In he Uni ed S a es, here are 20,000 new ocular cases per year, and 28,000 reac iva ions per year. One in 10,000 in an s are born wi h neona al HSV annually. Risk ac ors or viral reac iva ion include primary or secondary immunosuppression, and less commonly illness or s ress, mens rua ion, local injury and UV ligh exposure. T e ocular in amma ion may be due o he viral in ec ion i sel or rom he in ammaory response o he in ec ion. Symptoms Pa ien s may develop redness, i ching, burning, earing, and/ or discharge. Pho ophobia, modera e o severe pain, and blurry vision are common. Blis ers may occur on or near he eyelid.

Signs T e hallmark o he in ec ion is epi helial dendri ic kera i is, which is mos no able wi h uorescein s aining ( Fig. 3-12). Conjunc ival injec ion, decreased corneal sensa ion, corneal scarring, and decreased visual acui y are common. A hickened, edema ous cornea (disci orm kera i is), f brinous are wi h heavy an erior chamber cell and medium-sized granulomaous kera ic precipi a es presen on he endohelium; synechiae and increased in raocular pressure arising rom rabeculi is may also be seen ( Figs. 3-13 and 3-14). Dif erential Diagnosis Bac erial, viral, ungal, or allergic conjuncivi is, acu e angle-closure glaucoma, iri is or scleri is, corneal abrasion, recurren corneal erosion, or oxic conjunc ivi is Diagnostic Evaluation Sli -lamp examina ion classically reveals branching dendri es wi h erminal end bulbs ha end o show uorescein s aining in he ulcer base and rose bengal s aining a he border. A corneal swab or HSV DNA can be es ed wi h polymerase chain reac ion (PCR) can be used or an ibody i ers rom an aqueous sample can be ob ained o assess exposure. Treatment HSV epi helial kera i is usually resolves wi hou rea men . opical an iviral medicaion such as ri uoro hymidine 1% drops q.i.d. or opical gancyclovir 0.15% f ve imes daily can reduce he dura ion o he episode. Pa ien s wi h HSV s romal kera i is or uvei is (wi hou epi helial disease) can be rea ed wi h cycloplegia wi h scopolamine 0.25% or cyclopen ola e 1% drops .i.d. Prednisolone ace a e 1% drops q.i.d. should be used wi h a slow aper. Sys emic s eroids

Herpetic Keratouveitis

should be considered in severe uvei is. ri uoro hymidine 1% drops q.i.d. should be used or prophylaxis while on opical s eroids. Oral acyclovir can reduce he risk o recurrence. rophic epi he hial de ec s can be preven ed wi h preserva ive- ree lubrican drops and oin men s. arsorrhaphy can acili a e healing and preven recurren sur ace breakdown. A bandage lens and issue adhesive can be used o promo e epi he hial healing and preven corneal mel s. Prognosis Mos VZV and HSV in ec ions respond o an iviral rea men , s eroids, or bo h.

FIGURE 3-12. Dendritic keratitis is characteristic o HSV epithelial keratitis. Dendritic keratitis has active virus replicating in the epithelium; there ore, it is typically treated with topical antiviral agents, while corticosteroids are avoided.

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Complica ions depend on he severi y o he eye disease as well as pa ien charac eris ics. Po en ial complica ions o HSV in ec ion includes corneal neovasculariza ion and scarring, ca arac orma ion, neuro rophic ulcers, bac erial or ungal in ec ion, secondary glaucoma, pos herpe ic neuralgia, or vision loss arising rom op ic neuri is or choriore ini is. Corneal ransplan a ion may be necessary in some individuals. REFERENCE Wilhelmus KR, Gee L, Hauck WW, e al. Herpe ic Eye Disease S udy. A con rolled rial o opical cor icos eroids or herpes simplex s romal kera i is. Oph halmology. 1994;101( 12):1883–1895.

FIGURE 3-13. Disci orm stromal keratitis is an immune-mediated condition resulting rom chronic HSV in ection. Disci orm keratitis is the most common cause o blinding in ectious keratitis in the United States. opical corticosteroids are the mainstay o treatment and antiviral medications are used to avoid breakthrough o active in ection, not to treat the disci orm keratitis.

FIGURE 3-14. Disci orm keratitis and anterior uveitis rom HSV. T e in ammatory disci orm corneal lesions of en occur along with a low-grade uveitis that produces small keratic precipitates as seen in eriorly in this photo.

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VARICELLA ZOSTER VIRUS Etiology and Epidemiology Herpes zoster ophthalmicus is caused by reactivation o latent varicella zoster virus. VZV also causes chicken pox and shingles. Over 90% o adults have been exposed to VZV. Exposure to a ected individuals, increasing age, and immunosuppression are important risk actors or VZV reactivation. Iridocyclitis occurs in approximately 40% to 60% cases o herpes zoster ophthalmicus. T e li etime risk o reactivation is 10% to 20%. Symptoms Onset o the disease may be preceded by a u-like illness with malaise, nausea, and mild ever, along with progressive pain, skin hyperesthesia, and tingling. Within a ew hours or days a er onset o symptoms, a di use erythematous or maculopapular rash presenting over a single dermatome appears. It progresses to vesicles and pustules, which rupture and orm crusts within 3 to 5 days. T e uveitis usually starts 1 to 2 weeks a er onset o the rash. Patients can develop oreignbody sensations, eye pain, decreased vision, and/ or photophobia. Signs Decreased visual acuity, increased intraocular pressure, and miosis Hutchinson’s sign is a unilateral vesicular rash along the nasociliary branch o trigeminal nerve distribution Anterior chamber cells and are, granular inf ltrates in the anterior corneal stroma, pseudodendrites, keratitis, ciliary injection, and corneal edema may occur ( Figs. 3-15 and 3-16).

Sector iris stromal atrophy is a specif c sign o herpetic keratouveitis. Mucous plaque keratopathy can occur late in the disease course. Patients may also develop rash and vesicles on the eyelids along with conjunctivitis, retinal necrosis, and optic nerve involvement. Compared to HSV, slit-lamp examination may reveal dendrites that are slightly elevated, broader, and have less regular branching; there are ewer terminal end bulbs, and there is less central rose bengal staining. Fluorescein pools along the edge. Dif erential Diagnosis Bacterial, viral, ungal, or allergic conjunctivitis, acute angle-closure glaucoma, iritis or scleritis, corneal abrasion, recurrent corneal erosion, or toxic conjunctivitis Diagnostic Evaluation T e diagnosis is made clinically, and no laboratory tests are routinely ordered, although PCR can be help ul in certain cases. Treatment Systemic antivirals: Immunocompetent patients ( >50 years o age) with keratouveitis should be treated with: Acyclovir 800 mg PO f ve times per day or 10 to 14 days or Valacyclovir 1 g t.i.d. PO or 7 to 10 days or Famciclovir 500 mg PO q8h or 7 to 10 days Patients with recurrent disease may benef t rom long-term therapy. Antivirals are typically used or primary disease within the f rst week o onset. opical steroids: Prednisolone acetate 1% q.i.d. on a gradually tapering regimen should be administered concomitantly with antiviral agents. Strong evidence exists or improved

Herpetic Keratouveitis

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quali y o li e wi h a combina ion o an an iviral medica ion and opical s eroid; however, no all pa ien s are candida es. Cycloplegic agen s such as scopolamine 0.25% .i.d. should be used. Increased in raocular pressure should be aggressively managed wi h aqueous suppressan s including imolol 0.5% b.i.d., brimonidine 0.2% .i.d., and dorzolamide 2% .i.d.

and scarring, ca arac orma ion, corneal ulcer orma ion, bac erial or ungal in ec ion, secondary glaucoma, pos herpe ic neuralgia or vision loss arising rom op ic neuri is or choriore ini is. Immunocompe en pa ien s may benei rom he herpes virus vaccina ion, as i can reduce he incidence o herpes zos er.

Prognosis Mos VZV in ec ions respond o an iviral rea men and/or s eroids. Complica ions depend on severi y o he eye disease as well as pa ien charac eris ics. Po en ial complica ions o VZV in ec ion include dry eye, corneal neovasculariza ion

REFERENCES

FIGURE 3-15. Granulomatous uveitis rom herpes zoster. Both herpes zoster and herpes simplex can cause a granulomatous uveitis. Both can also create nongranulomatous uveitis and produce spine-like keratic precipitates called stellate keratic precipitates (not shown) . Isolated uveitis rom HSV or HZV in the absence o cornea disease is rare but can occur.

FIGURE 3-16. Lipid and brotic keratopathy rom severe herpes zoster ophthalmicus. T ese are latestage complications o herpes zoster ophthalmicus that occur af er multiple episodes o in ammation and typically take years to develop.

Cobo LM, Foulks GN, Liesegang , e al. Oral acyclovir in he rea men o acu e herpes zos er oph halmicus. Oph halmology. 1986;93( 6):763–770. Gnann JW, Whi ley RJ. Herpes zos er. N Engl J Med. 2002;347( 5):340–346. seng HF, Smi h N, Harpaz R, e al. Herpes zos er vaccine in older adul s and he risk o subsequen herpes zos er disease. JAMA. 2011 12;305( 2):160–166.

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MOOREN’S ULCER Joseph R. Zelefsky and Emmet . Cunningham, Jr.

M

ooren’s ulcer is a rare, progressive, in amma ory kera opa hy charac erized by severe pain, conjunc ival and episcleral injec ion, and peripheral corneal ulcera ion wi hou loss o adjacen sclera. Epidemiology and Etiology Mooren’s ulcer is mos commonly seen in he developing world, par icularly in Wes ern A rica and India. Men are a ec ed more o en han women. I can develop in bo h young and old pa ien s. o a large ex en , age a presen a ion varies by region. I can a ec one or bo h eyes. Risk ac ors include prior corneal rauma or surgery, previous corneal in ec ion, and concurren in es inal hookworm in es a ion. Pa ien s wi h he HLA-DR17 haplo ype have an increased risk o developing Mooren’s ulcer, and bo h cell-media ed and humoral immune-media ed mechanisms an ibodies have been sugges ed.

overhanging and requen ly opacif ed “leading edge” ( Fig. 3-18) Complete peripheral: Ex ensive peripheral ulcera ion leaving a cen ral island o corneal issue Total corneal ulceration: Comple e loss o all s romal issue, which leaves a residual f brovascular membrane overlying an in ac Desceme ’s membrane. Bila eral involvemen occurs in up o 50% o pa ien s. Corneal per ora ion occurs in up o 15% o cases and ends o occur mos requen ly in pa ien s wi h he comple e peripheral pat ern o ulcera ion. Dif erential Diagnosis Peripheral ulcera ive kera opa hy (secondary o rheuma oid ar hri is, Wegener’s granuloma osis, polyar eri is nodosa, sys emic lupus ery hema osus, and relapsing polychondri is) Ocular rosacea In ec ious kera i is errien’s marginal degenera ion

Symptoms Severe pain Pho ophobia earing Decreased vision

Diagnostic Evaluation Review o sys ems and labora ory es s o rule ou underlying rheuma ologic diseases (e.g., rheuma oid ac or, ANCA, ANA, HSV) and or hookworm in es a ion are use ul. T ere are no es s specif c or Mooren’s ulcer. Al hough earlier work sugges ed an associa ion wi h hepa i is C, more recen work ound no such associa ion.

Signs Conjunc ival and episcleral injec ion in he absence o scleral injec ion. Ulcera ion may ollow hree specif c pa erns ( Fig. 3-17): Partial peripheral: Peripheral ulcera ion o mos , bu no all, o he cornea, charac erized by deep vessels ex ending in o he ulcer bed rom he limbus, and an

Treatment Aggressive immunosuppressive herapy is he mains ay o rea men . Ini ially, pa ien s may be rea ed wi h opical cor icos eroids, ace ylcys eine, or opical cyclosporine. Mos pa ien s require sys emic herapy including cor icos eroids and one or more noncor icos eroid immunosuppressive agen s.

Mooren’s Ulcer

In recalci ran cases, surgical in erven ions such as limbal conjunc ival excision have been employed wi h varying degrees o success, and corneal or scleral pa ch gra s may be necessary in cases wi h corneal per ora ion. ( Fig. 3-19). Prognosis T e prognosis is generally variable and likely depends on how quickly and aggressively herapy is ini ia ed. A recen large cohor s udy repor ed ha less han 15% o pa ien s main ained 20/ 40 vision or bet er.

A

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REFERENCES Chow C, Fos er CS. Mooren’s ulcer. In Oph halmol Clin. 1996;36:1–13. Srinivasan M, Zegans ME, Zele sky JR, e al. Clinical charac eris ics o Mooren’s ulcer in Sou h India. Br J Oph halmol. 2007;91:570–575. andon R, Chawla B, Verma K, e al. Ou come o rea men o Mooren ulcer wi h opical cyclosporine a 2%. Cornea. 2008;27( 8):859–861. Wa son PG. Managemen o Mooren’s ulcer. Eye. 1997; 11:349–356. Zegans ME, Srinivasan M, McHugh , e al. Mooren ulcer in Sou h India: serology and clinical risk ac ors. Am J Oph halmol. 1999;128( 2):205–210.

B

C FIGURE 3-17.T e clinical spectrum o Mooren’s ulcer. A. Partial peripheral Mooren’s ulcer showing conjunctival and episcleral injection, with ulceration up to, but sparing, the sclera, with deep vessels in the ulcer bed, and an overhanging and opaci ed central ulcer margin. T ere is a small descemetocele within the ulcer. B. A total peripheral Mooren’s ulcer with a central island o edematous, opaci ed cornea. C. Complete Mooren’s ulcer with loss o all stromal tissue, replaced here with a brovascular membrane overlying an intact Descemet’s membrane. (Reproduced with permission rom Srinivasan M, Zegans ME, Zele sky JR, et al. Clinical characteristics o Mooren’s ulcer in South India. Br J Ophthalmol. 2007; 91:570–575, with permission rom BMJ Publishing Group Ltd.)

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3 EPISCLERITIS, SCLERITIS, AND KERATITIS

B

FIGURE 3-18. A. Active Mooren’s ulcer showing extensive peripheral ulceration, vascularization o the ulcerated bed, and overhang o the active leading edge. B. Inactive Mooren’s ulcer in a di erent patient has residual corneal thinning with persistent vascularization o the ulcer bed and opaci cation o the leading edge.

A

B

FIGURE 3-19. A. A tectonic patch graf was used to treat peripheral per oration in a patient with Mooren’s ulcer. B. A total tectonic graf used to treat central per oration in a patient with complete Mooren’s ulcer.

Peripheral Ulcerative Keratitis

PERIPHERAL ULCERATIVE KERATITIS Roxana Ursea

P

eripheral ulcera ive kera i is (PUK) is an ocular in amma ory condi ion wi h po en ially devas a ing consequences. I is o en associa ed wi h a coexis ing sys emic collagen vascular disease, bu i may also be due o an in ec ious e iology.

Pathophysiology T e periphery o he cornea has unique ana omic and physiologic charac eris ics ha predispose i o involvemen in au oimmune and in amma ory condi ions. T e limbus and peripheral cornea are adjacen o he highly vascular conjunc iva and derive par o heir nu rien supply rom he capillary arcades. T e conjunc iva is rich in immunocompe en cells, including macrophages, lymphocy es, and plasma cells, and hese cells have easy access o he peripheral cornea. T e peripheral cornea also has a high concen ra ion o Langerhans cells, which are involved in an igen presen a ion and secre ion o in amma ory media ors. T is leads o recrui men o in amma ory cells and media ors wi h subsequen release o pro eases and collagenases ha resul in peripheral corneal s romal degrada ion, necrosis, and ulcera ion. T e release o hese kera oly ic enzymes is dysregula ed; he ma rix me allopro ease-2, (member o a amily o enzymes involved in he degrada ion o ex racellular ma rix) is overexpressed. T e conjunc ival lympha ic drainage begins a he limbus. T e limbal vessels ermina e a he peripheral cornea, which a ec s

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he di usion o high-molecular-weigh molecules. T is acili a es he deposi ion o immune complexes, IgM, and C1, which ur her con ribu es o he immunologic ac ivi y and in amma ory response. Etiology Sys emic diseases (nonin ec ious): Collagen vascular diseases are responsible or approxima ely 50% o nonin ec ious cases o PUK. Rheuma oid ar hri is is he mos commonly associa ed condi ion. O her commonly associa ed diseases are Wegener’s granuloma osis, relapsing polychondri is, polyar eri is nodosa, microscopic polyangii is, Churg-S rauss syndrome, and sys emic lupus ery hema osus Rarely: Crohn’s disease and emporal ar eri is are associa ed wi h PUK. Secondary o in ec ion: Organisms ha can cause PUK include S aphylococcus and S rep ococcus species ( he mos common pa hogens), Pseudomonas, Acan hamoeba, Neisseria species, gram-nega ive bacilli, uberculosis, syphilis, and human immunodef ciency virus (HIV). Mooren’s ulcer is a sub ype o PUK ha is idiopa hic and, by def ni ion, occurs wi hou scleral involvemen and in he absence o any sys emic f ndings. Mooren’s ulcer is a diagnosis o exclusion made only a er he presence o collagen vascular disease or in ec ious causes have been ruled ou . Symptoms Ocular pain and redness ( ypical presen a ion) earing, pho ophobia, and decreased visual acui y (due o corneal opaci y or as igma ism)

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3 EPISCLERITIS, SCLERITIS, AND KERATITIS

Signs A crescen -shaped corneal ulcer ound wi hin 2 mm o he limbus is he ypical lesion T ere is o en an epi helial de ec overlying he ulcer wi h hinning o he underlying s roma. T ere can be a s romal inf l ra e ollowed by progressive corneal hinning (also known as “corneal mel ”) ( Fig. 3-20). Desceme ocele orma ion leading o perora ion rarely occurs. One hird o pa ien s have associa ed scleri is. Diagnostic Evaluation A horough his ory and examina ion is he mos impor an par o he workup. Cul ures o he ulcer should be considered. Evalua ion or underlying sys emic diseases, including es ing or serum an ibodies should be considered. Occasionally a corneal biopsy is needed o es ablish he diagnosis. Dif erential Diagnosis Condi ions ha lead o peripheral corneal hinning or scarring include errien’s marginal ulcera ion, corneal degenera ion, pellucid marginal degenera ion, phlyc enulosis, rachoma ous pannus, marginal kera i is, rosacea kera i is, and vernal kera oconjunc ivi is. Local insul s ha lead o peripheral corneal pa hology include poor-f t ing con ac lenses, corneal exposure, richiasis, kera oconjunc ivi is sicca, and meibomian gland dys unc ion. Treatment T e goal o rea men is o hal progressive corneal ulcera ion, preserve globe in egri y, encourage healing o he epi helial de ec , and address he underlying cause.

I PUK is secondary o an in ec ious e iology, pa ien s should be rea ed wi h medicaions appropria e or he o ending agen . I PUK is associa ed wi h sys emic disease, pa ien s should be rea ed wi h sys emic immunosuppressive herapy. T e choice o cy o oxic agen depends on he underlying disease. I vision loss is imminen , pulsed, shor erm IV me hylprednisolone should be adminis ered. I pa ien s have underlying rheuma oid ar hri is, sys emic cor icos eroids plus and agen such as me ho rexa e can be e ec ive. Wegener’s granuloma osis and polyar eriis nodosa should be rea ed wi h sys emic cor icos eroids and ano her medica ion such as mycophenola e mo e il. Aza hioprine, cyclosporine A, and chlorambucil have also been used wi h variable e cacy depending on he underlying collagen vascular disease. Consider reserving agen s wi h higher oxici y, such as cyclophosphamide, or cases wi h herapeu ic ailure, drug in olerance, or rapidly progressive disease. I no underlying sys emic disease is ound, consider opical drops or surgery. Drops include: Inhibi ors o collagenase syn hesis (i.e., 1% medroxyproges erone drops) Compe i ive inhibi ors o collagenase (i.e., opical 20% N-ace ylcys eine and sysemic e racycline) Lubrica ing agen s should promo e epihelial healing. Surgical managemen is primarily used in cases o impending per ora ion in order o preserve globe in egri y. Conjunc ival resec ion decreases conjunc ival produc ion o pro eases and collagenases and reduces local access o

Peripheral Ulcerative Keratitis

immune cells and in amma ory media ors o he peripheral cornea. Ulcer debridemen and applica ion o cyanoacryla e adhesive may be benef cial. issue adhesive can be used o preven ur her s romal loss by excluding acu e in amma ory cells rom he cornea. I has been success ully used in cases o impending per ora ion. Con inuous-wear bandage so con ac lens may help preven per ora ion. Lamellar or pene ra ing kera oplas y generally carry a poor prognosis. Gra ailure due o a recurren mel occurs in 80% o eyes by 6 mon hs a er pene ra ing kera oplas y. Superior orniceal advancemen o a conjunc ival pedicle in conjunc ion wi h sys emic immunosuppressive herapy has been used wi h some success in recen case repor s. PUK ref actory o conven ional herapies should be rea ed wi h a umor necrosis ac or-alpha inhibi or such as in iximab or adalimumab or ri uximab, which is an an iCD20 an ibody. Prognosis T e f nding o PUK in he set ing o sysemic disease is ex remely signif can , as i can serve as a marker or he presence o

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a po en ially le hal sys emic vasculi is. T e progression is rapid and visual loss can occur over a mat er o days. T e mos eared ocular complica ion is corneal per ora ion (corneal mel ), which can resul in abrup and permanen loss o vision. opical glucocor icoids are con raindica ed. T ey may ur her aggrava e he disease process and produce accelera ed corneal mel ing hrough inhibi ion o collagen syn hesis. Local rea men o PUK alone wi hou simul aneous sys emic rea men will almos invariably ail. Cy o oxic agen s no only improve he sys emic disease bu have also been shown o improve gra survival in pa ien s wi h rheuma oid ar hri is. REFERENCES Galor A, T orne JE. Scleri is and peripheral ulcera ive kera i is. Rheum Dis Clin N Am. 2007;33:835–854. Ladas JG, Mondino BJ. Sys emic disorders associa ed wi h peripheral corneal ulcera ion. Curr Opin Ophthalmol. 2000;11:468–471. Messmer EM, Fos er CS. Vasculi ic peripheral ulcera ive kera i is. Surv Ophthalmol. 1999;43( 5):379–396. Sainz de la Maza M, Fos er CS, Jabbur NS, e al. Ocular charac eris ics and disease associa ions in scleri isassocia ed peripheral kera opa hy. Arch Ophthalmol. 2002;120( 1):15–19. auber J, Sainz de la Maza M, Hoang-Xuan , e al. An analysis o herapeu ic decision making regarding immunosuppressive chemo herapy or peripheral ulcera ive kera i is. Cornea. 1990;9( 1):66–73.

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A

3 EPISCLERITIS, SCLERITIS, AND KERATITIS

B

D

C FIGURE 3-20. A. A 39-year-old woman with Crohn’s disease and peripheral ulcerative keratitis. Note the circum erential pannus with injected conjunctival vessels and active PUK with a “bullous-like” lesion in the peripheral cornea. B and C. Residual thinning in the area o in ammation af er the resolution o active in ammation. D. T e same patient has 360 degrees o pannus af er multiple episodes o PUK.

C H AP T ER

An erior Uvei is HUMAN LEUCO CYTE ANTIGEN B27–ASSOCIATED UVEITIS Julie Gueudry and Bahram Bodaghi

A

n erior uvei is cons i u es up o 75% o all cases o uvei is and human leucocy e an igen (HLA)-B27–associa ed uvei is is he mos commonly diagnosed cause o acu e an erior uvei is. T is haplo ype is requen ly associa ed wi h sys emic diseases such as ankylosing spondyli is, inf amma ory bowel disease, reacive ar hri is, psoria ic ar hri is, and undi erenia ed spondyloar hropa hies. T is group o diseases is also re erred o as seronega ive spondyloar hropa hies (“seronega ive” meaning nega ive rheuma oid ac or, and “spondylo” meaning spine). Etiology and Epidemiology Gene ic, geographic, and environmen al ac ors are involved. T e prevalence o HLA-B27 is 5% o 8% in Wes ern popula ions. HLA-B27 is less requen in nonwhi e popula ions.

T e li e ime cumula ive incidence o acu e an erior uvei is is 0.2% in he general populaion, bu increases o 1% in he HLA-B27– posi ive popula ion. Depending upon he popula ion s udied, he HLA-B27 haplo ype accoun s or 40% o 70% o cases o acu e an erior uvei is. More han hal o he pa ien s wi h an HLA-B27–associa ed acu e an erior uvei is presen wi h an associa ed sys emic disease. HLA-B27–associa ed uvei is has been hough o be more common in males han in emales, bu recen work has called his in o ques ion. I more commonly occurs in younger people. T e risk o developing spondyloar hri is or uvei is in a B27-posi ive pa ien is 25%. In pa ien s wi h spondyloar hropa hy, he prevalence o uvei is is as high as 32.7%. Symptoms Sudden onse o redness, pain, pho ophobia, and blurred vision. Associa ed sys emic complain s may include low back pain, ar hri is, psoriasis, oral ulcers, chronic diarrhea, and ure hri is. 35

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Signs Mos commonly, pa ien s have acu e and/ or recurren episodes o uvei is, usually las ing several days o weeks. However, i may be chronic in 25% o cases. arely are bo h eyes simul aneously inf amed. Fine kera ic precipi a es (KPs) and endohelial dus ing occur, bu he uvei is is always nongranuloma ous. Severe an erior chamber reac ion wi h brin can occur, and a hypopyon is common and is associa ed wi h disease severi y ( Figs. 4-1 and 4-2). A brin ne may orm across he pupillary margin. Pos erior synechiae are requen ly presen . Pos erior segmen involvemen is underrecognized, even hough vi ri is, vasculi is, papilli is, and macular edema may occur, especially in chronic, under rea ed cases.

SERONEGATIVE SPONDYLOARTHROPATHIES ( Figs. 4-3 to 4-7) Ankylosing spondyli is is a chronic ar hri is ha mainly a ec s he spine and sacroiliac join s. T e major symp oms are lower back pain and s i ness. 90% o pa ien s are HLA-B27–posi ive. Uvei is may be he rs mani es a ion o he disease and may occur prior o onse o join pain. Nons eroidal an i-inf amma ory medicaions and physical herapy are he mains ays o rea men . Me ho rexa e and an i- NF agen s have also been success ully used. eac ive ar hri is syndrome ( ei er’s syndrome) T e classic riad is papillary conjunc iviis, ure hri is, and polyar hri is (“can’ see,

can’ pee, can’ climb a ree”). However, hese symp oms may be mild or absen . An erior uvei is is usually less common (10% o cases). Mos o he pa ien s are young male adul s. T e HLA-B27 posi ivi y ra e is 60%. Bac eria such as Chlamydia, Salmonella, Yersinia, and Shigella have been associa ed wi h he disease, and may rigger he disease in a suscep ible pa ien , however heir role remains con roversial. Kera oderma blennorrhagicum (scaling skin), circina e balani is (rash around he penis), aph hous s oma i is, plan ar ascii is, and uncommonly iri is are addi ional diagnos ic cri eria. Inf amma ory bowel disease (IBD) Ulcera ive coli is and Crohn’s disease are he main diagnos ic en i ies. T e risk o developing uvei is is up o ve imes higher in pa ien s wi h ulcera ive coli is han hose wi h Crohn’s disease. Pa ien s wi h IBD who develop uvei is may develop sacroilii is and are HLA-B27 posi ive in 60% o cases. Pa ien s may also have ery hema nodosum and pyoderma gangrenosum. Behçe ’s disease and Whipple’s disease are he main di eren ial diagnoses o consider. Psoria ic ar hri is One- h o pa ien s wi h psoria ic ar hri is may develop sacroilii is. Pa ien s presen wi h cu aneous, join , and ungual involvemen . T e ypical skin lesions are eleva ed, well-circumscribed plaques. Pa ien s may have cen ral ar hri is a ec ing he spine, or dis al ar hri is a ec ing he ngers. In advanced cases, pa ien s may have “sausage digi de ormi y.”

Human Leucocyte Antigen B27–Associated Uveitis

Nail changes include nail pit ing, ridging, and discolora ion can occur. T e ra e o uvei is in pa ien s wi h psoria ic ar hri is is 25%. Uvei is in his subgroup o pa ien s has some speci c charac eris ics such as bila erali y, chronici y, and severi y. Pos erior segmen involvemen (CME, re inal vasculi is, and papilli is) is no uncommon. Undi eren ia ed spondyloar hropa hies T ere is an HLA-B27 posi ivi y ra e o 25%, and uvei is occurs somewha less requen ly in his group. Dif erential Diagnosis Idiopa hic an erior uvei is Sarcoidosis O her nongranuloma ous uvei is Behçe ’s disease–associa ed uvei is In ec ious uvei is (herpe ic uvei is, syphilis, Lyme disease, Whipple’s disease, or in ec ious endoph halmi is) Drug-induced uvei is: ri abu in, biphosphona es, pros aglandin analogues, and cido ovir ubuloin ers i ial nephri is and uvei is ( INU) Lens-induced uvei is Masquerade syndromes (re inoblasoma and me as a ic umors) Diagnostic Evaluation HLA-B27 yping ES , C-reac ive pro ein Work-up o rule ou i ems on he di erenial diagnosis, including serum angio ensinconver ing enzyme (ACE), ches radiograph, Lyme i er, VD L/ P / F A-Abs, uberculin skin es

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I indica ed: M I o he sacroiliac join and lumbar spine Swab or chlamydia, Shigella, Yersinia, and o her gram-nega ive bac eria. Specialized consul a ions: rheuma ology, gas roin es inal, derma ology, and in ec ious disease Treatment Cycloplegic and mydria ic drops will relieve pain and break pos erior synechiae. opical cor icos eroids are he mains ay o rea men or ocular disease and usually need o be adminis ered every hour or he rs 48 hours, hen slowly apered. I he uvei is is severe, subconjunc ival injec ions o dexame hasone can be considered daily or 3 consecu ive days. Sub- enon’s riamcinolone injec ion can also be used. I here is no improvemen on opical/ periocular s eroids, sys emic cor icos eroids and/ or sys emic immunosuppressive agen s may be proposed. Sys emic NSAIDs may decrease he recurrence ra e as well as exposure o cor icos eroids. An i- NF-α rea men may be use ul in rea men -resis an and/ or sigh - hrea ening cases. Prophylac ic rea men using sul asalazine remains con roversial. Prognosis Generally avorable wi h aggressive herapy Uvei is o en recurs and may become chronic. Presence o chronic inf amma ion is he main prognos ic ac or. Pos erior iris synechiae, band kera opa hy, pos erior subcapsular ca arac , ocular hyperension, hypo ony, cys oid macular edema, and epire inal membrane orma ion are he major complica ions.

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EFE ENCES Braun J, Baraliakos X, Lis ing J, e al. Decreased incidence o an erior uvei is in pa ien s wi h ankylosing spondyli is rea ed wi h he an i- umor necrosis ac or agen s inf iximab and e anercep . Arthritis Rheum. 2005; 52( 8):2447–2451. Chang JH, McCluskey PJ, Wake eld D. Acu e an erior uvei is and HLA-B27. Surv Ophthalmol. 2005;50:364–388. Durrani K, Fos er CS. Psoria ic uvei is: a dis inc clinical en i y? Am J Ophthalmol. 2005;139:106–111.

A

Loh A , Acharya N . Incidence ra es and risk ac ors or ocular complica ions and vision loss in HLA-B27associa ed uvei is. Am J Ophthalmol. 2010;150: 534–542. Zamecki KJ, Jabs DA. HLA yping in uvei is: use and misuse. Am J Ophthalmol. 2010;149( 2):189–193. Zeboulon N, Dougados M, Gossec L. Prevalence and charac eris ics o uvei is in he spondyloar hropa hies: a sys ema ic li era ure review. Ann Rheum Dis. 2008;67: 955–959.

B

FIGURE 4-1. A. Slit-lamp photograph shows severe acute anterior uveitis with f brin in the anterior chamber and 360 degrees o posterior synechiae in a patient with reactive arthritis. B. Remission occurred with maximal topical corticosteroids, cycloplegic and subconjunctival injection o dexamethasone each day or 3 days.

Human Leucocyte Antigen B27–Associated Uveitis

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FIGURE 4-2. Slit-lamp photograph showing acute anterior uveitis with f brin in the anterior chamber and a hypopyon in a patient who is HLA-B27 positive with ankylosing spondylitis.

FIGURE 4-3. Right ankle arthritis in a patient with ankylosing spondylitis.

FIGURE 4-4. Sausage toes o the right oot in a case o seronegative spondyloarthritis. (Courtesy o P. Quartier.)

FIGURE 4-5. Pelvis radiograph shows irregular margins and sclerosis o the sacroiliac joints. (Courtesy o P. Quartier.)

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A

4 ANTERIO R UVEITIS

B

FIGURE 4-6. A. Lateral lumbar spine radiograph demonstrating a syndesmophyte. (Courtesy o P. Quartier.) B.T is person has vertical syndesmophytes on multiple vertebrae causing a “bamboo spine.” (Courtesy o V. Vuillemin.)

FIGURE 4-7. Plaque psoriasis on the elbow o a patient with HLA-B27–associated uveitis and psoriatic arthritis.

Posner-Schlossman Syndrome

POSNER-SCHLOSSMAN SYNDROME Bahram Bodaghi

T

he Posner-Schlossman syndrome (PSS), also known as recurren glaucoma ocycli ic crisis syndrome, is an unusual clinical en i y ha occurs in young o middle-aged adul s. Ini ially considered an immune-media ed condi ion, i may be due o a viral in ec ion. Epidemiology I predominan ly occurs in young o middleaged pa ien s bu i may also be diagnosed in he elderly. T ere seems o be a clear male preponderance. T e disease remains rare bu mus be considered in all cases o unila eral uvei is associa ed wi h high in raocular pressure (IOP). Etiology Since i s ini ial descrip ion in 1948, here has been much specula ion regarding i s pa hogenesis. Despi e lack o evidence, developmen al abnormali ies o he angle, allergic ac ors, primary vascular abnormali ies, sympa he ic nervous sys em de ec s, and inf amma ory mechanisms have all been proposed as possible mechanisms or his disorder. ecen da a based on speci c in raocular an ibody produc ion and molecular biology sugges cy omegalovirus in ec ion as he inci ing agen in PSS. Symptoms Pa ien s presen wi h modera e blurred vision, o en due o mild corneal edema secondary o an acu e rise in IOP. I is almos always unila eral wi h recurren at acks in he same eye. Pa ien s may experience very mild pain or discom or .

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Signs Mild decrease in vision Dila ed conjunc ival vessels Whi e kera ic precipi a es o di eren sizes predominan ly loca ed a he cen ral cornea ( Fig. 4-8) Minimal aqueous f are wi hou cells Pos erior synechiae are no presen . T e IOP is markedly eleva ed, ranging rom 40 o 60 mm Hg. T e angle is open, al hough an erior synechiae may be presen . T ere is usually no iris he erochromia. Vi ri is is absen and here is no pos erior segmen involvemen . Be ween he at acks, he examina ion is unremarkable (wi h he excep ion o glaucoma ous op ic a rophy). Dif erential Diagnosis CMV-induced an erior uvei is Herpe ic an erior uvei is A ypical cases o Fuchs’ iridocycli is Nonspeci c hyper ensive iridocycli is Sarcoidosis uberculosis Mul iple sclerosis Diagnostic Evaluation An an erior chamber ap may be perormed or viral PC and analysis o speci c an ibody produc ion, con rming he presence o CMV. Visual eld es ing and/ or re inal nerve ber layer analysis can be used o iden i y glaucoma ous visual eld abnormali ies ha may occur in severe or recurren orms o he disease. Ancillary es s o exclude o her causes o unila eral uvei is and secondary glaucoma should be per ormed as clinically indica ed.

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Treatment rea men o lower he IOP is usually required during at acks in order o pro ec he op ic disc. Be ore he iden i ca ion o PSS as a viral disorder, many au hors ound he use o shor - erm opical cor icos eroids use ul. In CMV-associa ed PSS, speci c an iviral herapy wi h opical or sys emic drugs may be ini ia ed. T e dura ion o an iviral herapy depends on he clinical presen a ion and he severi y o visual eld al era ion. In severe cases wi h high clinical suspicion, a 2- o 3-mon h regimen may be considered. Cycloplegic agen s are no required. Fil ering surgery is usually no recommended bu may be success ully used o rea glaucoma ha progresses despi e maximum medical herapy.

Prognosis Generally speaking, recurrences decrease wi h increasing age, so he visual prognosis is usually good. However, in he absence o speci c ocular an ihyper ensive medica ions or surgery, permanen visual loss may occur in approxima ely 25% o cases due o chronic ocular hyper ension. EFE ENCES Bloch-Michel E, Dussaix E, Cerque i P, e al. Possible role o cy omegalovirus in he e iology o Posner-Schlossman syndrome. Int Ophthalmol. 1987;11:95–96. Chee SP, Bacsal K, Jap A, e al. Clinical ea ures o cy omegalovirus an erior uvei is in immunocompe en pa ien s. Am J Ophthalmol. 2008;145( 5):834–840. Posner A, Schlossman A. rea men o glaucoma associa ed wi h iridocycli is. JAMA. 1949; 139:82–86. eoh SB, T ean L, Koay E. Cy omegalovirus in ae iology o Posner-Schlossman syndrome: evidence rom quani a ive polymerase chain reac ion. Eye (Lond). 2005; 19( 12):1338–1340.

FIGURE 4-8. ypical white keratic precipitates that are most concentrated in the central cornea in a case o CMV-induced Posner-Schlossman syndrome.

Fuchs’Uveitis Syndrome (Fuchs’Heterochromic Iridocyclitis)

FUCHS’ UVEITIS SYNDROME ( FUCHS’ HETERO CHROMIC IRIDOCYCLITIS) Bahram Bodaghi and Phuc LeHoang

I

n 1906, Erns Fuchs described he clinical charac eris ics o a series o 38 pa ien s wi h a previously undescribed condi ion ha now bears his name. Usually considered a benign disease, he diagnosis o Fuchs’ uvei is syndrome (FUS) may be challenging, leading o he misuse o cor icos eroids, which can resul in ur her complica ions. ecen s udies have highligh ed he role o a viral agen in he pa hogenesis o FUS, expanding he spec rum o viral-induced an erior uvei is. Epidemiology T e prevalence o he disease varies rom 1.2% o 5% o pa ien s wi h uvei is. T e disease a ec s men and women equally and has no racial predilec ion, even hough i may be more di cul o recognize in individuals wi h brown eyes. FUS generally occurs in young adul s. Etiology and Pathogenesis Di eren heories have been proposed o explain he pa hogenesis o his disease. S udies on HLA associa ions and o her gene ic ac ors have been con radic ory. Due o he common clinical ea ures observed in pa ien s wi h congeni al Horner’s syndrome, a congeni al paralysis o he sympa he ic sys em was considered, bu convincing evidence is lacking. Based on di eren immunologic ndings i was hough o be an immunemedia ed disorder riggered by some inci ing even . T e in ec ious heory has always been preerred. As choriore inal scars are observed in

43

33% o 56% o pa ien s wi h FUS, i was iniially hough o be rela ed o a Toxoplasma in ec ion. However, compelling evidence sugges ing in ec ion wi h Rubella virus in pa ien s wi h FUS has been recen ly repor ed. Symptoms Pa ien s usually have minimal symp oms, and he disease is usually discovered during a rou ine eye examina ion. Floa ers are he main complain o pa ien s wi h FUS. Visual loss occurs la e in he disease course and is usually due o ca arac progression. T e pa ien may no e he erochromia ( Fig. 4-9). Signs FUS is a chronic, an erior, granuloma ous uvei is wi h vi reous involvemen . FUS is mainly unila eral (90% o cases) wi h a long, insidious course. Care ul bila eral sli -lamp examina ion is impor an . Impor an ly, ciliary injec ion and pos erior synechiae are never presen and should be viewed as exclusion cri eria. Kera ic precipi a es (KP) are presen in mos cases and are ypically small, whi e, and s ella e, and are scat ered over he en ire corneal endo helium ( Fig. 4-10). T ey are never conf uen . In mos o her diseases, KPs are more prominen on he in erior cornea. T e o her condi ion ha can cause di use KPs is herpe ic iridocycli is. He erochromia is a major sign bu may be absen in brown-eyed pa ien s. Dayligh (or na ural ligh ) examina ion be ore dila ion acili a es he diagnosis. Sec oral iris a rophy is no observed in FUS.

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Iris nodules may be observed a he level o he papillary margin (Koeppe nodules) or he iris sur ace (Busacca nodules). Due o iris a rophy, he iris blood vessels are more visible and narrower han in normal eyes. Peripheral synechiae and abnormal angle vessels are seen in abou 20% o 30% o cases. A hyphema may occur wi h a minor rauma or spon aneously (Amsler’s sign). An erior chamber f are and cells are minor. Ini ially, he ca arac s are usually pos erior subcapsular, bu may progress and become hyperma ure, requiring urgen surgery. Secondary glaucoma may occur in wohirds o cases. T is is o en due o misuse o opical cor icos eroids. Vi ri is wi h large cellular aggrega es and debris is a ypical nding ( Fig. 4-11). Fundus examina ion is usually normal even hough op ic disc hyperf uorescence may be observed on f uorescein angiography. Macular edema is absen in phakic eyes. Small, ocal choriore inal scars may be observed in he periphery. Dif erential Diagnosis Viral an erior uvei is In ermedia e uvei is Primary in raocular lymphoma Diagnostic Evaluation O en i is a clinical diagnosis. However, ancillary labora ory es s, including PC , o exclude o her e iologies o an erior uvei is can be considered. An erior chamber paracen esis may induce a mild hyphema (Amsler’s sign).

Visual eld es ing should be per ormed in cases o suspec ed glaucoma. Treatment opical and sys emic cor icos eroids or immunosuppressan s should be avoided. Da a on he e cacy o opical NSAIDs are con roversial. Ca arac surgery can be per ormed wi h an excellen visual ou come, however pa ien s may develop a hyphema. Care should be aken o avoid manipula ions o he iris and angle. opical and sys emic glaucoma medicaions are necessary in up o 60% o cases. Fil ering surgery wi h adjunc ive wound modula ors may be considered in resis an cases. Vi rec omy is rarely needed o clear vi reous debris and aggrega es. Prognosis T e prognosis o FUS is usually excellen in he absence o secondary glaucoma. EFE ENCES Birnbaum AD, essler HH, Schul z KL, e al. Epidemiologic rela ionship be ween Fuchs he erochromic iridocycliis and he Uni ed S a es rubella vaccina ion program. Am J Ophthalmol. 2007;144( 3):424–428. Fuchs E. Über Komplica ionen der He erochromic. Z. Augenheilkd. 1906;15:191–212. Liesegang J. Clinical ea ures and prognosis in Fuchs’ uvei is syndrome. Arch Ophthalmol. 1982;100:1622–1626. Liesegang J. Fuchs uvei is syndrome. In: Pepose JS, Holland GN, Wilhelmus K . Ocular In ection and Immunity. S . Louis: Mosby; 1996:495–506. Quen in CD, eiber H. Fuchs he erochromic cycli is: rubella virus an ibodies and genome in aqueous humor. Am J Ophthalmol. 2004;138( 1):46–54. Van Gelder N. Idiopa hic no more: clues o he pa hogenesis o Fuchs he erochromic iridocycli is and glaucomaocycli ic crisis. Am J Ophthalmol. 2008;145:769–771.

Fuchs’Uveitis Syndrome (Fuchs’Heterochromic Iridocyclitis)

A

45

B

FIGURE 4-9. A. Heterochromia in a young male patient with Fuchs’ uveitis syndrome o the right eye. B. Heterochromia in a young woman with FUS o the le eye.

FIGURE 4-10. T is patient has the white, small, di usely distributed stellate keratic precipitates typical o FUS.

FIGURE 4-11. Occasionally, patients with FUS may have vitreous cells and debris.

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JUVENILE IDIOPATHIC ARTHRITIS–ASSO CIATED UVEITIS ( JUVENILE RHEUMATOID/ CHRONIC ARTHRITIS)

TABLE 4-1. Risk Factors or Developing Uveitis in Children with JIA • • • •

Oligoarticular orm o JIA Young girls ANApositivity Young age at disease onset

Karina Julian and Bahram Bodaghi

J

uvenile idiopa hic ar hri is ( JIA) is he main cause o bo h uvei is and ar hri is in children. I is a bila eral, nongranuloma ous, chronic an erior uvei is. As i is usually asymp oma ic, he diagnosis may be delayed and major visual complica ions may have already occurred a he ime o presen a ion. Children wi h JIA mus have regular eye exams in order o iden i y he onse o in raocular inf ammaion and o ini ia e e ec ive rea men . Epidemiology and Etiology JIA is de ned as ar hri is o unknown e iology occurring in children under he age o 16 years and las ing or a leas 6 weeks. Seven subgroups o he disease have been de ned. Among hem, oligoar hri is (pauciar icular ar hri is) is he mos commonly associa ed wi h uvei is. Oligoar hri is is de ned as ar hri is a ec ing 1 o 4 join s wi hin he rs 6 mon hs o disease onse , and uvei is may occur in a subs an ial minori y o hese pa ien s. T e level o ac ivi y o he ar hri is does no necessarily ref ec he level o uvei is. Young girls ( 12,000 Albumin: abnormal i 450,000 a er 7 days o illness Hema ocri : abnormal i anemic or age I hree or more o hese addi ional labora ory es s are abnormal, he child should receive an echocardiogram and pharmacologic rea men . I ewer han hree o he addi ional laboraory es s are abnormal, an echocardiogram alone is per ormed. I posi ive, pharmacologic rea men should be given. I nega ive bu ever persis s, an echocardiogram may be repea ed. I nega ive and ever aba es, Kawasaki’s disease is unlikely. Treatment In ravenous gamma globulin is now he mains ay o rea men ( ypically 2 g/ kg in used over 12 hours). rea men is ini ia ed 5 o 7 days a er onse o ever. Aspirin is ypically given, hough i s bene in addi ion o in ravenous gamma globulin is unclear. T e dose is 80 o 100 mg/ kg/ day divided q.i.d. or 2 weeks, hen 3 o 5 mg/ kg once daily or 6 o 8 addi ional weeks. Aspirin

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herapy is con inued longer i coronary vessel abnormali ies are presen . Pa ien s are admit ed o an inpa ien service or moni oring and rea men , along wi h cardiac consul a ion. Ibupro en should be avoided, as i an agonizes aspirin’s an ipla ele ac ivi y. Prognosis I pa ien s do no develop coronary ar ery aneurysms, he disease usually resolves wi hou sequelae. Pa ien s who develop coronary ar ery aneurysms require prolonged moni oring and rea men . Coronary ar ery bypass gra ing and possibly cardiac ransplan a ion may be indica ed or gian aneurysms resis an o pharmacologic herapy. EFE ENCES Puglise JV, ao NA, Weiss R , e al. Ocular ea ures o Kawasaki’s disease. Arch Oph halmol. 1982;100( 7): 1101–1103. owley AH, Shulman S . Pa hogenesis and managemen o Kawasaki disease. Exper Rev An i In ec T er. 2010;8( 2):197–203. Smi h LB, Newburger JW, Burns JC. Kawasaki syndrome and he eye. Pedia r In ec Dis J. 1989;8( 2): 116–118.

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RELAPSING POLYCHONDRITIS S. R. Ra hinam

R

elapsing polychondri is is a mul isys em connec ive issue disease ha causes recurren episodes o in amma ion o he car ilaginous issues o he nose, ear lobes, respira ory rac , and join s, as well as o pro eoglycanrich issues including he media o he ar eries, conjunc iva, and sclera.

Etiology and Epidemiology T e cause is unknown. Immune complex deposi ion, -cell–media ed changes, and au oan ibodies o collagen ypes II, IX, and XI, which are ound in he cornea and sclera, may be responsible or he ocular complica ions. elapsing polychondri is occurs in all races, and is more common during hird o h decades o li e wi h a sligh emale predominance. Symptoms In ermit en ever, weigh loss, a igue, and skin rash Abrup onse o nasal and ear pain, swelling, and redness Ocular redness and pain Join pain Coughing, hoarseness o voice, shor ness o brea h Signs ( Figs. 7-53 to 7-55) In amma ion o he pinna o he ear, wi h pain, redness, and swelling, is presen in he vas majori y o pa ien s. Sudden hearing loss, ver igo, inni us ecurren episodes o eyelid edema, episcleri is, scleri is, peripheral ulcera ive kerai is, iri is, and rarely re inopa hy may occur. Pa ien s may also develop ocular muscle paresis or op ic neuri is.

Nasal in amma ion causes pain, redness, and a s uf y and/ or runny nose. Chronic in amma ion can cause a saddle-nose de ormi y. Voice hoarseness, epiglot i is, and laryngoracheal-bronchial s ric ure In amma ion o he aor ic ring can cause aor ic regurgi a ion, aor ic dissec ion, and cardiac conduc ion de ec s. Pa ien s may develop mi ral valve regurgi a ion. Ar hri is, mos commonly o he hands and knees, can even ually cause join de ormi y. Glomerulonephri is wi h associa ed renal ailure and anemia Nonspeci c skin rashes Dif erential Diagnosis Pa ien s may have concomi an connec ive issue diseases such as: heuma oid ar hri is Behçe ’s disease Wegener’s granuloma osis Polyar eri is nodosa Sys emic lupus ery hema osus In ec ious causes: Orbi al celluli is syphilis leprosy Lyme disease Diagnostic Evaluation I is a clinical diagnosis, bu biopsy o involved car ilage is help ul. T e diagnos ic cri eria consis o a leas hree o he ollowing clinical ea ures: Bila eral auricular chondri is Nasal chondri is espira ory rac chondri is Nonerosive seronega ive in amma ory polyar hri is Ocular in amma ion

Relapsing Polychondri is

Cochlear and/ or ves ibular dys unc ion Or one o he above wi h compa ible his ologic ea ures rom a car ilage biopsy Consider ches imaging, EKG/ ECG, ES , and serologies as needed o narrow he dif eren ial diagnosis. Treatment Prednisone (1 mg/ kg body weigh daily) alone or in combina ion wi h oral me horexa e (15 o 20 mg weekly) or aza hioprine (2 o 3 mg/ kg body weigh daily) is ef ec ive. A number o o her drugs, including dapsone, mycophenola e mo e il, and in liximab have been used wi h success. ES , urinalysis, and pulmonary unc ion es s can also be used o ollow rea men response.

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o preven signi can morbidi y and morali y, early diagnosis and aggressive rea men is impor an . Signi can cardiovascular or renal involvemen is associa ed wi h a poorer ou come overall. EFE ENCES Gergely P, Poor G. elapsing polychondri is. Bes Prac ice & Research Clinical Rheuma ology. 2008;18( 5): 723–738. McAdam CM, O’Hanlan MA, Blues one , e al. elapsing polychondri is: prospec ive s udy o 23 pa ien s and a review o he li era ure. Medicine Bal imore. 1976;55: 193–215. Miche CJ Jr, McKenna CH, Lu hra HS, e al. elapsing polychondri is. Survival and predic ive role o early disease mani es a ions. Annals o In ernal Medicine. 1986;104:74–78.

Prognosis Prognosis depends on he organ involvemen and response o rea men .

FIGURE 7-53.T is young girl has auricular redness and edema wi h mild scleri is.

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B

A

C

FIGURE 7-54. A. Severe auricular edema o he righ ear along wi h scleri is o he righ eye. B. On higher magni ca ion, he scleri is is associa ed wi h peripheral corneal in l ra es. C.T e lef ear shows in amma ion o he pinna wi h edema and ery hema. T e ear lobe is spared, which is ypical o his disease.

Relapsing Polychondri is

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B

A

C

FIGURE 7-55. A.T is person has developed a “saddle-nose” de ormi y due o long-s anding chondri is. She has cushingoid “moon acies” secondary o prolonged s eroid rea men . (Her eyes have been blurred or pa ien privacy.) B.T ere is scleral necrosis wi h resul an hinning o he lef eye due o chronic scleri is. C. Several mon hs la er, he ac ive scleri is o he lef eye has resolved, bu he scleral hinning remains. She has recurren ac ive scleri is in he righ eye. T e nasal de ormi y is more apparen rom he side view.

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SCLERODERMA S. R. Ra hinam

S

cleroderma is a chronic, mul isys em au oimmune disease, clinically charac erized by progressive brosis. Pa hologically, i is charac erized by chronic in amma ion, microvascular injury, and excessive ex racellular ma rix produc ion and deposi ion o ype I and ype III collagens. I also af ec s he eye, lungs, hear , kidneys, and gas roin es inal rac . Etiology and Epidemiology Scleroderma is a connec ive issue disorder o unknown e iology. I occurs worldwide in all races, bu people o A rican descen are af ec ed more requen ly. Women are our imes more likely o be af ec ed han men. T e peak onse occurs be ween 30 and 50 years. Symptoms Sys emic: a igue, dysphagia, progressive dyspnea, and ar hralgia. CNS symp oms can also occur. Ocular: redness and pain Signs ( Figs. 7-56 to 7-60) Sys emic Scleroderma is he major diagnos ic cri erion and is charac erized by igh , shiny skin wi h a charac eris ic loss o hair and loss o abili y o make a skin old. Indura ion o he skin on he ngers, ace, neck, and runk. Sclerodac yly (sclerosis o he ngers and oes) Micros omia due o perioral involvemen

Ischemic digi al ulcers and/ or digi al pit ing scars Decreased swea ing Ar hri is, join con rac ures Ocular Kera oconjunc ivi is sicca: T e mos common ocular mani es a ion, and may be severe Episcleri is, scleri is, scleral pi s, and peripheral corneal ulcer Eyelid skin brosis, which can cause lid s if ness, and shallowing o he ornices Nonspeci c al era ions o he re inal pigmen epi helium elangiec asis o he eyelids and o her par s o he ace Children may develop similar signs as adul s. In addi ion, hey may develop an erior uvei is. T ey also may have linear scleroderma o he ace called “en coup de sabre,” which looks like a sword cu across he ron oparie al lobe and may involve he periorbi al area, including he eye. Dif erential Diagnosis O her collagen vascular disorders Sjögren syndrome Diagnostic Evaluation T e diagnosis is mainly clinical; however, an icen romere an ibodies (ACAs), an iopoisomerase an ibodies, and an i- NA polymerase III an ibodies are repor ed o be speci c au oan ibodies associa ed wi h disinc clinical subse s o scleroderma. Treatment Sys emic rea men : Cor icos eroids are he rs -line drugs; oral prednisone (1 mg/ kg body weigh daily) alone or in combina ion wi h oral me ho rexa e (10 o 15 mg weekly)

Scleroderma

or aza hioprine (2 o 3 mg/ kg body weigh daily) is o en success ul. Ocular rea men : Lubrican s and punc al occlusion or dry eye Prognosis Pulmonary involvemen , including in ers i ial lung disease and/ or pulmonary hyper ension, develops in up o 80%o pa ien s and is curren ly he leading cause o dea h in scleroderma. Ocular prognosis is good i sys emic rea men is s ar ed early.

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EFE ENCES Subcommit ee or Scleroderma. Cri eria o he American heuma ism Associa ion Diagnos ic and T erapeu ic Cri eria Commit ee, Preliminary cri eria or he classi ca ion o sys emic sclerosis (scleroderma). Subcommit ee or scleroderma cri eria o he American heuma ism Associa ion Diagnos ic and T erapeu ic Cri eria Commit ee. Ar hri is Rheum. 1980;23:581–590. ailor , Gup a A, Herrick A, e al. Ocular mani es aions o scleroderma. Survey o Oph halmology. 2009; 54( 2):292–304. Zannin ME, Mar ini G, A hreya BH, e al. Ocular involvemen in children wi h localized scleroderma: a mul icen re s udy. Br J Oph halmol. 2007;91:1311–1314.

A

B

FIGURE 7-56. A. T is woman has many o he classic ea ures o scleroderma including nasal beaking, micros omia, perioral skin puckering, and skin depigmen a ion. (Her eyes have been blurred or pa ien privacy.) B.T ere are pit ing scars on he skin o he dis al phalanges.

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FIGURE 7-57. A.T is 70-year-old woman has loss o acial skin creases and igh skin. (Her eyes have been blurred or pa ien privacy.) B. She also has exion con rac ures o he nger join s and bony resorp ion o he erminal phalanges.

A

B

FIGURE 7-58.T ere is an area o scleral mel ing nasal o he cornea.

Scleroderma

A

C

E

117

B

D

FIGURE 7-59. A.T is girl has absence o acial skin creases, and she has marked conjunc ival injec ion, mild scleri is, and a nasal corneal ulcer. B. Higher magni ca ion o he righ eye. C.T ere is sclerodac yly wi h ypical hickened shiny skin o he ngers wi h marked edema. D.A f er promp rea men wi h sys emic s eroids, here is marked improvemen o bo h her sys emic and ocular condi ion. E.T e sclerodac yly has grea ly improved as a resul o sys emic rea men .

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7 PO S ERIO R UVEI IS AND CO LLAGEN VASCULAR DISEASES

A

B FIGURE 7-60. A. T ere are numerous digi al pi s on he dorsal sur ace o he hand, as well as resolving areas o indura ion. B. T ere is an ischemic digi al ulcer on he ip o he index nger, and digi al pit ing on he ip o he ring nger.

Derma omyosi is and Polymyosi is

DERMATOMYOSITIS AND POLYMYOSITIS S. R. Ra hinam

P

olymyosi is is a mul isys em au oimmune disorder charac erized by in amma ion and degenera ion o he s ria ed muscles. When i is associa ed wi h a skin rash, i is re erred as derma omyosi is, in which he muscle, skin, and surrounding connec ive issues are also af ec ed.

Etiology and Epidemiology Polymyosi is has been hough o be an immune-media ed syndrome caused by de ec ive cellular immuni y direc ed oward myo bers, however more recen work sugges s a role or an an igen-driven response as well. Viral in ec ions, malignancies, or connecive issue disorders all have been implica ed as riggering ac ors. Derma omyosi is has been hough o be a humoral at ack agains he capillaries and small ar erioles resul ing in microin arc ion, a rophy, and calci ca ion o muscle and subcu aneous issue; recen work sugges s ha i is a mul imechanism disorder. T ese diseases occur mos o en in children be ween 5 and 15 years o age and in adul s be ween 50 and 70 years. Women are af ec ed wice as o en as men. I is prevalen hroughou he world. Symptoms Di cul y get ing up rom a chair or climbing up s eps Fa igue, myalgias, ar hralgias, and muscle cramps ed eye, earing Signs ( Figs. 7-61 to 7-64) Polymyosi is Proximal muscle weakness and enderness

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Nondes ruc ive ar hri is Cardiomyosi is In ers i ial lung disease aynaud’s phenomenon Dif use cu aneous, subcu aneous, and some imes muscular calci ca ion T e eye muscles are spared and he acial muscles are involved only in severe disease. Derma omyosi is T e rash consis s o a helio rope (i.e., blue-purple) discolora ion on various par s o he body including he eyelids. Pa ien s may have ery hema ous, scaly, eleva ed skin lesions called Got ron rash. Pa ien s may also have widespread scaling, hyperpigmen a ion, and depigmen a ion o he skin. Ocular involvemen is usually con ned o he eyelids. Some pa ien s may have a well-circumscribed a rophic lid scar. Corneal scarring can resul rom he eyelid disease. e inal vascular disease is rare. Dif erential Diagnosis Muscular dys rophies T yroid hormone disorders Drug-induced myopa hy (s a in drugs or chloroquine/ hydroxychloroquine) Diagnostic Evaluation Eleva ion o enzymes including crea inine phosphokinase, aldolase, SGO , SGP , and lac a e dehydrogenase M I and muscle biopsy Some serum au oan ibodies like myosi isassocia ed an ibodies, and myosi is-speci c an ibodies are ound in approxima ely 40% o pa ien s. Muscle s reng h es ing and pulmonary unc ion es ing can be use ul o ollow disease progression.

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Treatment Cor icos eroids are he rs -line drug. Prednisolone (1 mg/ kg body weigh daily) alone or in combina ion wi h oral me ho rexa e (10 o 15 mg weekly) or aza hioprine (2 o 3 mg/ kg body weigh daily) is ef ec ive. In ravenous immunoglobulin (IVIG), mycophenola e mo e il, aciolimus, and ri uximab have shown some bene in preliminary s udies. Prognosis Spon aneous remission has been repor ed in one- h o cases. Early rea men mainains he muscle s reng h and reduces he relapse ra e. In he long erm, myosi is has a major ef ec on quali y o li e. Pa ien s wi h polymyosi is may have o her diseases such as Sjögren’s disease or scleroderma.

Cancer is ound in up o 15% o pa ien s wi h derma omyosi is.

EFE ENCES Akikusa JD, ennankore DK, Levin AV, e al. Eye ndings in pa ien s wi h juvenile derma omyosi is. JRheuma ology. 2005;32( 10):1986–1991. Allanore Y, Vignaux O, Arnaud L, e al. Ef ec s o cor icos eroids and immunosuppressors on idiopa hic in amma ory myopa hy rela ed myocardi is evalua ed by magne ic resonance imaging. Ann Rheum Dis. 2006; 65:249–252. Dalakas MC. Immuno herapy o myosi is: issues, concerns and u ure prospec s. Na Rev Rheuma ol. 2010;6( 3):129–137. Hengs man GJ, van den Hoogen FH, van Engelen BG. rea men o he in amma ory myopa hies: upda e and prac ical recommenda ions. Exper Opin Pharmaco her. 2009;10( 7):1183–1190.

FIGURE 7-61. Scarring and pigmen a ion over he cheeks, and hyperpigmen ed pa ches are seen on he skin o nose and ear lobes.

Derma omyosi is and Polymyosi is

A

121

B

FIGURE 7-62. A.T is pa ien has he charac eris ic helio rope rash ( blue-purple) and edema on he dorsum o hands, wi h a scaly, ery hema ous erup ion on he knuckles (Got ron rash) . B. No e he muscle a rophy o he orearms and palms.

A

B

FIGURE 7-63. A.T is pa ien has elangiec asis o he upper eyelid skin. T is elangiec asia can appear as a purple line, which may precede a helio rope rash. B. T ere is chronic meibomiani is and corneal in l ra es.

FIGURE 7-64.T ere is cu aneous calci ca ion a he elbow join . (Cour esy o Dr. Par hiban, Derma ology, Madurai.)

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POLYARTERITIS NODOSA Julie Lew and Shree Kurup

P

olyar eri is nodosa (PAN), also known as periar eri is nodosa, is an uncommon mul isys em, necro izing vasculi is ha af ec s medium- and small-sized ar eries hroughou he body ha can resul in re inal and choroidal in arc s. PAN mos commonly af ec s he skin, join s, gas roin es inal rac , kidneys, and peripheral nerves. T e eye is af ec ed in 10% o 20% o cases, and PAN can af ec bo h he an erior and pos erior segmen s. Etiology and Epidemiology PAN occurs more o en in males (60%) han in emales (40%). Mean age a onse is 45 years. I is an immune complex-media ed disease ha has also been linked wi h seroposi ivi y o hepa i is B sur ace an igen.

Mononeuropa hy or polyneuropa hy Dias olic BP >90 mm Hg Eleva ed blood urea ni rogen or crea inine Hepa i is B virus Ar eriographic abnormali y Biopsy o small or medium-sized ar ery con aining polymorphonuclear neu rophils Ocular signs ( Figs. 7-65 and 7-66) Vasculi is af ec ing he choroidal vessels is he mos common ocular nding in PAN. Choroidal and pos erior ciliary ar ery ischemia can cause an ischemic op ic neuropa hy as well. Cen ral re inal ar ery occlusion Vascular or uosi y Cot on wool spo s Hard exuda es Marginal corneal ulcera ion and kera i is Scleri is and episcleri is

Symptoms Fever, weigh loss, malaise Abdominal pain Myalgias Skin rash including livedo re icularis, nodules, purpura, and aynaud’s phenomenon ed, pain ul eye Blurry vision, oa ers Epididymi is or ovarian pain

Dif erential Diagnosis Wegener’s granuloma osis Syphilis Behçe ’s syndrome SLE Mixed connec ive issue disease Derma omyosi is heuma oid ar hri is

Signs T e American College o heuma ology (AC ) has de ned 10 cri eria or classi caion o PAN. A pa ien is said o have PAN i a leas hree o hese cri eria are presen : Weigh loss ≥4 kg Livedo re icularis es icular pain or enderness Myalgias, weakness, or leg enderness

Diagnostic Evaluation No es is diagnos ic o PAN, bu a workup should include: Comple e blood coun , me abolic panel, rheuma oid ac or, ES , ANA, hepa i is B an igen, ANCA, rapid plasma reagin ( P ), and uorescen reponemal an ibody-absorp ion (F A-Abs). Urinalysis or kidney involvemen Biopsy o issue wi h involved ar eries

Polyar eri is Nodosa

Fluorescein angiography i re inal vasculiis is suspec ed Kidney ar eriogram i kidney involvemen suspec ed Treatment Sys emic cor icos eroids are rs -line herapy. S eroid-sparing agen s may be used alone or in combina ion wi h s eroids or re rac ory cases. All s eroid-sparing agen s, including cy o oxic agen s such as cyclophosphamide, have been used wi h some success. Aza hioprine has been shown o be an ef ec ive main enance herapy o preven disease relapse. Plasmapheresis has also been shown o be bene cial when used as an adjunc herapy wi h cor icos eroids.

FIGURE 7-65. PAN-associa ed sclerokera i is wi h pannus and scleral hinning.

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Prognosis Early immunosuppressive herapy is he key o ob aining a good prognosis. T e 5-year survival ra e increases rom 12% o 80% wi h promp ins i u ion o sys emic immunosuppressive herapy.

EFE ENCES Galet a SL. Vasculi is. In: Miller N , Newman NJ, eds. Walsh and Hoy ’s Clinical Neuro-Oph halmology. 5 h ed. Vol. 3. Bal imore: Williams & Wilkins; 1998: 3744–3760. Hsu C , Kerrison JB, Miller N , e al. Choroidal in arcion, an erior ischemic op ic neuropa hy, and cen ral re inal ar ery occlusion rom polyar eri is nodosa. Re ina. 21( 4):348–351. Morgan CM, Fos er CS, D’Amico DJ, e al. e inal vasculi is in polyar eri is nodosa. Re ina. 1986;6: 205–209.

FIGURE 7-66.T ere is scleral hinning (scleromalacia) due o old necro izing scleri is (no e he area o scleral hinning and avasculari y ex ending 3 o 4 mm rom he limbus) .

C H AP T ER

White Dot Syndromes ACUTE POSTERIOR MULTIFO CAL PLACOID PIGMENT EPITHELIOPATHY Céline Terrada and Bahram Bodaghi

D

terior multifocal placoid pigment epitheliopathy (APMPPE or AMPPE) in 1968. e clinical presentation is usually characterized by multiple, bilateral, cream-colored, placoid lesions at the level of the outer retina/ retinal pigment epithelium (RPE). It is caused by ischemic changes occurring within the choriocapillaris.

Epidemiology and Etiology It predominantly occurs in young individuals that are in the second to fourth decades of life. ects men and women equally. APMPPE is usually bilateral, but it can be quite asymmetric and the second eye may not

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APMPPE is idiopathic, but it has been associated with mumps, secondary syphilis, Lyme disease, streptococcal group A infection and anti–hepatitis B virus vaccination. e disease has also been called acute multifocal ischemic choroidopathy. Symptoms Patients complain of visual disturbances, blurred vision, photopsias and scotomas. e degree of visual impairment can be variable depending upon the location of the lesions. Visual loss is due to macular involvement, and there is usually a gradual improvement in vision over the course of a few weeks. Signs and headache. Episcleritis, scleritis, anterior uveitis, vitreous haze, papillitis, and retinal vasculitis can occur. On fundus examination during the acute phase, the lesions are characterized

Acute Posterior Multifocal Placoid Pigment Epitheliopathy

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by mul iple yellow-whi e deep plaques, ranging rom one-hal o one op ic disk diame er in size. Lesions appear o be a he level o he ou er re ina and he PE even hough he ischemic al era ions involve he choriocapillaris. A er a ew days, healing s ar s a he cen er o he plaques, leaving a pigmen ed scar or mot led PE. New lesions can appear in he pos erior pole wi hin he rs 3 weeks o disease onse . APMPPE may be associa ed wi h serous re inal de achmen s, mimicking Vog Koyanagi-Harada (VKH) disease.

Op ical coherence omography (OC ) demons ra es nodular hyperref ec ivi y a he level o he pho orecep ors and PE. Visual eld es ing objec ively iden i es he sco oma a he pa ien s repor . In eres ingly, he elec rore inogram (E G) and elec rooculogram (EOG) are normal.

Dif erential Diagnosis Whi e do syndromes: Presumed ocular his oplasmosis syndrome, punc ua e inner choroidopa hy (PIC), mul i ocal choroidi is Sarcoidosis, syphilis, uberculosis ( B) VKH syndrome Sympa he ic oph halmia Subre inal brosis/ uvei is syndrome Serpiginous choroidi is Birdsho choriore inopa hy

Prognosis T e prognosis is good wi h a visual acui y o 20/ 25 a er 6 mon hs al hough small sco omas may persis . ecurrences or exacerba ions are rare. Macular localiza ion may be more aggressive. Subre inal neovasculariza ion remains a rare complica ion.

Diagnostic Evaluation ( Figs. 8-1 to 8-4) Fluorescein angiography Acu e s age Early and in ermedia e hypof uorescence ollowed by s aining and pooling in he la e rames Delay in choriocapillaris circula ion La e s age: Hyperf uorescence in early and la e rames wi hou leakage (window de ec ) Indocyanine green angiography (ICGA) shows hypof uorescence during he in ermedia e and la e ransi rames.

Gass JD. Acu e pos erior mul i ocal placoid pigmen epiheliopa hy. Arch Oph halmol. 1968;80:177–185. Jones BE, Jampol LM, Yannuzzi LA, e al. elen less placoid choriore ini is: a new en i y or an unusual varian o serpiginous choriore ini is? Arch Oph halmol. 2000;118:931–938. Senanayake SN, Selvadurai S, Hawkins CA, e al. Acu e pos erior mul i ocal placoid pigmen epi heliopa hy associa ed wi h ery hema nodosum and a f u-like illness. Singapore Med J. 2008;49:e333–335. Souka AA, Hillenkamp J, Gora F, e al. Correla ion be ween op ical coherence omography and au of uorescence in acu e pos erior mul i ocal placoid pigmen epiheliopa hy. Grae es Arch Clin Exp Oph halmol. 2006; 244:1219–1223.

Treatment Usually no rea men is required. In pa ien s wi h macular involvemen , sys emic cor icos eroids can be ini ia ed o has en visual recovery and immunosuppressive agen s are rarely required.

EFE ENCES

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B

FIGURE 8-1. Color (A) and red- ree (B) undus pho ographs show mul iple, deep yellow-whi e placoid lesions.

A

B

FIGURE 8-2. Fluorescein angiography (FA) . T e early rame shows hypof uorescence (A) , and he la e rame shows s aining and pooling (B) .

Acute Posterior Multifocal Placoid Pigment Epitheliopathy

A

B

FIGURE 8-3. ICG demons ra es hypof uorescence in bo h he in ermedia e (A) and la e (B) rames.

FIGURE 8-4. Color undus pho ographs in a case o APMPPE wi h bila eral involvemen .

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SERPIGINOUS CHORIORETINOPATHY Céline Terrada and Bahram Bodaghi

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erpiginous choroidopa hy, also known as serpiginous choroidi is, geographic choroidopa hy or geographic helicoid peripapillary choroidopa hy, is an inf amma ory disorder involving he choroid, he choriocapillaris and he PE. I ge s i s name due o he serpen ine pat ern i develops as i progresses rom he op ic disk.

Epidemiology and Etiology Serpiginous choroidi is is a rare cause o pos erior uvei is ( 125 µm) appear as a rophic scars. In eres ingly, here are o en hundreds o smaller lesions ( 80) .T e visual acui y did no improve a er vi rec omy.

Ocular Toxocariasis

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FIGURE 9-58. T is 17-year-old girl had a subre inal granuloma wi h cen ral scar orma ion. Her vision was 20/ 400. She had a high serum IgG i er or Toxocara canis.

A

C

B

FIGURE 9-59. A.T is 8-year-old girl had progressive vision loss down o coun ngers. She described an overall “darkness” o her vision. She had a submacular lesion wi h op ic neuri is. B.T e f uorescein angiogram reveals la e s aining o he granuloma wi h disk hyperf uorescence. C. She did no receive rea men . Five mon hs la er, her vision had improved o 20/ 60 wi h involu ion o he lesion. T ere is a subre inal brosis wi h al era ion o he re inal pigmen epi helium. (Cour esy o Sunir J. Garg, MD.)

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CAT-SCRATCH DISEASE Julie Gueudry and Bahram Bodaghi

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a -scra ch disease (CSD), or ocular Bar onella in ec ion, is a bac erial in ecion ha causes wo major ypes o ocular involvemen : conjunc ivi is and neurore iniis. Generally occurring in children, adolescen s, and young adul s, he diagnosis is based on a ypical clinical presen a ion and a speci c serology. Mos cases are benign and are sel -limi ed. Preven ion remains impor an in order o reduce he incidence o he disease and i s po en ial complica ions. More severe cases require an ibio ics. Etiology and Epidemiology I is caused by Bar onella henselae, a small gram-nega ive rod ha is prevalen worldwide. T e domes ic ca or kit en is he principal animal vec or. Mos pa ien s will have a recen his ory o ca bi es, ca scra ches, and/ or ca ea bi es. I can also be ransmit ed i ca saliva or he eces o he ca ea comes in o con ac wi h he conjunc iva or wi h an exposed wound. Kit ens have a higher ra e o in ec ion han adul ca s. I occurs in immunocompe en individuals o all ages bu is more requen in children and young adul s. Symptoms Unila eral eye redness, oreign body sensaion, and epiphora Blurred vision Pa ien s may develop a ever, nausea, vomi ing, and sore hroa , which occurs wi hin 2 weeks o onse o ocular symp oms. Pa ien s may hen develop regional lymphadenopa hy.

Signs ( Figs. 9-60 to 9-62) Usually, bu no exclusively, unila eral Conjunc ivi is is seen wi h parinaud oculoglandular syndrome, which is charac erized by: Palpebral swelling Granuloma ous nodule on he conjunc iva Discharge is o en presen and ends o be serous Preauricular, submandibular, or cervical lymphadenopa hy is he classic ea ure o his disease. Mild sys emic symp oms o malaise, a igue, and nausea may occur in 10% o 30% o pa ien s Neurore ini is Possible cause o Leber idiopa hic s ella e neurore ini is May be unila eral or bila eral Op ic nerve swelling wi h peripapillary hemorrhages may occur. T e classic s ella e macular hard exuda es may ollow a ew weeks la er. Vi reous cells Granuloma ous an erior uvei is may occur wi hou op ic neuri is Neurore ini is is no a complica ion o CSD-rela ed conjunc ivi is Focal re inochoroidi is wi h whi e in lra es and re inal necrosis may also occur. I usually is associa ed wi h vi reous and/ or an erior chamber cells, and op ic disk swelling may be presen . Dif erential Diagnosis Parinaud oculoglandular conjunc ivi is: ularemia B Syphilis Sarcoidosis

Cat-Scratch Disease

Lymphogranuloma venereum due o Chlamydia rachoma is Ocular sporo richosis O her causes o op ic disk edema associa ed wi h a macular s ar ha should be considered: Malignan hyper ension Pseudo umor cerebri wi h a macular hemi-s ar Sarcoidosis Syphilis B oxoplasmosis oxocariasis Lyme disease Lep ospirosis Diagnostic Evaluation Bar onella henselae serology wi h indirec immuno uorescen assays or ELISA es ing. T ese have a modera e alse-nega ive ra e. War hin-S arry silver impregna ion s ain Direc iden i ca ion hrough issue cul ure is challenging. PC or he de ec ion o B. henselae 16S ribosomal DNA in ocular uids can be considered. Treatment T e disease is usually sel -limi ed and he ideal rea men has no been de ned. Parinaud oculoglandular conjunc ivi is: azi hromycin 500 mg by mou h he rs day hen 250 mg daily or 4 more days. Severe in raocular in ec ion: doxycycline (100 mg given PO b.i.d.) or rime hoprimsul ame hoxazole can be used, occasionally

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in conjunc ion wi h ri ampin (300 mg PO b.i.d.). Doxycycline and ri ampin appear o shor en he course o disease and has en visual recovery. Doxycycline is no used in children below 12 years o age, as oo h discolora ion is a concern. Dura ion o rea men is usually 4 weeks in immunocompe en pa ien s and 4 mon hs in immunocompromised pa ien s. Preven ion is paramoun ; curren ly, here is no vaccine available and any ca bi e or scra ch mus be immedia ely washed and disin ec ed. Prognosis Long- erm prognosis is excellen in mos cases and mos pa ien s will recover heir vision wi hin 1 o 4 weeks. T e macular s ar may ake 6 o 12 mon hs o ully resolve. Some individuals may develop a mild or severe visual loss due o op ic neuropa hy or macular a rophy. EFE ENCES Cunningham E , JE Koehler. Ocular bar onellosis. Am J Oph halmol. 2000;130:340–349. Curi AL, Machado D, Heringer G, e al. Ca -scra ch disease: ocular mani es a ions and visual ou come. In Oph halmol. 2010;30( 5):553–558. Drancour M, Berger P, errada C, e al. High prevalence o as idious bac eria in 1520 cases o uvei is o unknown e iology. Medicine (Bal imore). 2008;87:167–176. Jones DB. Ca -scra ch disease. In: Pepose JS, Holland GN, Wilhelmus K , edi ors. Ocular in ec ion and immuni y. S . Louis: Mosby Year Book; 1996:1389–1397. Solley WA, Mar in DF, Newman NJ, e al. Ca scra ch disease: pos erior segmen mani es a ions. Oph halmology. 1999;106( 8):1546–1553.

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A

B

C FIGURE 9-60. A. Pa ien wi h s ella e neurore ini is caused by Bartonella henselae.T e vision a presen a ion was hand mo ion. B. T ere was progressive improvemen several weeks a er an ibio ic herapy. C. One year la er, he nal vision was 20/ 200 due o op ic and macular a rophy.

Cat-Scratch Disease

A

B

C

D

E

F

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FIGURE 9-61. Color undus pho ograph (A) and red- ree pho ograph (B) show op ic disk edema wi h a macular s ar. T ere are some in rare inal hemorrhages and wo small areas o choriore ini is emporal o he ovea. C, D. FA shows s aining o he op ic disk and choriore inal lesions in he la e rames. E, F. ICG angiography shows hypocyanescence in he areas o choriore ini is. T ere are also larger areas o hypocyanescence around he disk and under he macula.

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FIGURE 9-62. T is pa ien has Parinaud’s oculoglandular syndrome. T e le eye has conjunc ival injec ion wi h epiphora. T e le preauricular node is enlarged.

Whipple’s Disease

WHIPPLE’S DISEASE Valérie Toui ou and Bahram Bodaghi

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hipple’s disease is a sys emic in ec ious disease caused by Tropheryma whipplei. Ocular involvemen remains rare bu he diagnosis should be considered in pa ien s wi h uvei is or neuro-oph halmologic manies a ions who also have chronic diarrhea and weigh loss and who do no improve, and may even worsen, despi e use o cor icos eroids. Epidemiology T e incidence is approxima ely 18 and 30 cases o sys emic disease per 100,000 people annually. T is incidence is likely an underes ima e. Ocular disease occurs in approxima ely 3% o cases o sys emic Whipple’s disease. T e mean age a onse o he disease is 50 years. T ree- our hs o in ec ed pa ien s are men. Etiology Whipple disease is caused by he bac eria Tropheryma whipplei, bu i ook nearly 50 years o isola e he organism. 1952: Firs pa ien cured by an ibio ics (chloramphenicol) sugges ing a bac erial origin o he disease 1992: Firs iden i ca ion o he bacillus 2000: Firs cul ure o Tropheryma whipplei achieved Signs and Symptoms ( Figs. 9-63 and 9-64) Sys emic Usually precedes ocular involvemen and includes: Chronic weigh loss is he mos common symp om. Pa ien s may also have nonspeci c evers.

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Chronic diarrhea, abdominal pain, s ea orrhea Ar hralgias (usually more peripheral han axial, and is polyar icular ra her han monoar icular). T e ar hralgias are o en migra ory. Chronic lymphadenopa hy (which is o en medias inal) Asci es and pleuri is Cu aneous hyperpigmen a ion, hrombopenic purpura Hear murmurs, endocardi is, myocardi is, arrhy hmia Cen ral mo or de ci , demen ia, hypo halamopi ui ary involvemen , epilepsy, poly- or mono-neuri is Ocular Findings may occur in he absence o any sys emic symp oms. Kera i is Pa ien s may develop a chronic, bila eral, pos erior, or panuvei is. I is usually granuloma ous wi h a ew kera ic precipi a es and limi ed pos erior synechiae. Choroidi is Scleri is Oph halmoplegia, supranuclear gaze palsy Oculomas ica ory myorhy hmia (pendular nys agmus associa ed wi h ongue or mandibular myoclonus) Papilledema, re robulbar neuri is, op ic nerve a rophy Dif erential Diagnosis Sarcoidosis B Behçe ’s disease Ulcera ive coli is His oplasmosis

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Mul i ocal choroidi is In raocular and sys emic lymphoma Amyloidosis Mycobac erium avium in racellulare in ec ion Ankylosing spondyli is Diagnostic Evaluation Mos pa ien s have he diagnosis made la e in he disease course due o he nonspeci c signs and symp oms. issue is required o es ablish he diagnosis. T e mos common es s are: Immunohis ochemis ry: PAS-posi ive “ oamy” macrophages are seen in issue biopsies (duodenal, vi reous, lymph node) PC : PC o saliva and s ool specimens are use ul as screening es s. PC o duodenal biopsies, lymph node biopsies, aqueous humor, vi reous, or CSF specimens can be used o iden i y he 16S-r NA gene. Treatment An ibio ics mus cross he blood–brain barrier and be adminis ered or a prolonged period o ime in order o reduce he risk o relapses. Cerebral Whipple’s disease: Induc ion (2 weeks) Ei her penicillin 1.2 million uni s or ce riaxone IV 2 g b.i.d. in addi ion o s rep omycin 1 g/ day or 2 weeks, or

IV rime hoprim (800 mg)-sul ame hoxazole (160 mg) ( MP-SMX) b.i.d. or .i.d. or 1 o 2 weeks Follow-up rea men (1 year) MP-SMX (960 mg b.i.d.), or Oral ce xime (400 mg once daily). Usually MP-SMX is pre erred or long- erm rea men . Due o Whipple’s ef ec on gas roin esinal absorp ion, pa ien s should also receive olic acid supplemen a ion. Ocular Whipple’s disease: T ere is no consensus on rea men . Prolonged an ibio ic herapy or a leas 1 year is required in o order o avoid relapses. Prognosis T e prognosis is variable as mos pa ien s are diagnosed la e in he disease course, when signi can CNS or cardiac changes may have occurred. EFE ENCES Chan Y, Yannuzzi LA, Fos er CS. Ocular Whipple’s disease: earlier de ni ive diagnosis. Oph halmology. 2001;108( 12):2225–2231. Drancour M, aoul D, Lépidi H, e al. Cul ure o ropheryma whipplei rom he vi reous uid o a pa ien presen ing wi h unila eral uvei is. Ann In ern Med. 2003;16;139( 12):1046–1047. Lagier JC, Lepidi H, aoul D, e al. Sys emic ropheryma whipplei: clinical presen a ion o 142 pa ien s wi h in ec ions diagnosed or con rmed in a re erence cen er. Medicine (Bal imore). 2010;89( 5):337–345.

Whipple’s Disease

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FIGURE 9-63. Chronic pos erior uvei is wi h papilli is and vascular leakage a he pos erior pole in a pa ien wi h Whipple’s disease.

FIGURE 9-64. Periodic acid-Schi posi ive macrophage inclusions in he vi reous specimen o a pa ien wi h chronic uvei is who was diagnosed wi h Whipple’s disease.

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DIFFUSE UNILATERAL SUBACUTE NEURORETINITIS Carlos Alexandre de Amorim Garcia

D

if use unila eral subacu e neurore ini is (DUSN) is a unila eral ocular in ec ious disease caused by nema odes capable o in lra ing he subre inal space. I can cause a di use choriore ini is, or “wipe-ou ” o he PE.

Etiology and Epidemiology DUSN usually occurs in heal hy children and young adul s wi h no signi can pas ocular his ory. I mos commonly occurs in people in ropical clima es and is he second leading cause o unila eral blindness in nor heas Brazil. I is also ound in he Uni ed S a es, India, and some Asian and European coun ries. Several species o nema odes o various sizes may cause DUSN, including: Toxocara canis, A. caninum, S rongyloides s ercoralis, Ascaris lumbricoides, and B. procyonis. Iden i ca ion o he organism is based on a combina ion o care ul measuremen o he parasi e’s dimensions, serologic es ing (limi ed role), and epidemiologic s udies. Symptoms T e clinical course is charac erized by periods o ac ivi y and remission. Less commonly, pa ien s may have pain, pho ophobia, and redness. Decreased visual acui y and cen ral or paracen ral sco omas ha can be severe in he la e s ages o he disease. Signs ( Figs. 9-65 to 9-68) Early s age Mild o modera e vi ri is, mild op ic disk edema, and recurren crops o

evanescen , mul i ocal, whi e-yellowish lesions a he level o he ou er re ina, PE and choroid which are clus ered in only one region o he re ina. Less requen signs include: iridocycli is, perivenous exuda ion, subre inal hemorrhages, and serous re inal de achmen . T e worm can be seen during any s age o he disease. When presen , hey are ypically in he vicini y o he ac ive whi eyellowish lesions. A live nema ode is seen as a whi e, mobile, o en glis ening worm ha is gen ly apered a bo h ends and varies in leng h rom 400 o 2,000 µm. La e s age Mos pa ien s in whom DUSN is suspec ed clinically are in he chronic phase. T ese pa ien s have severe vision loss, wi h he vas majori y o pa ien s having less han 20/ 200 vision. Eyes o en have dif use depigmen a ion o he PE, mos prominen in he peripapillary and peripheral re ina. T e dif use PE loss leads o op ic nerve a rophy and severe re inal ar eriole narrowing. T ere is an increased re inal inner limi ing membrane re ex. Occasionally, recurren crops o evanescen , mul i ocal, whi e-yellowish lesions a he level o he ou er re ina and choroid occur. Evidence o whi e-yellowish subre inal unnels or racks, which are sugges ive o larva migra ion in he subre inal space Dif erential Diagnosis Early s age Mul i ocal choroidi is Acu e pos erior mul i ocal placoid pigmen epi heliopa hy

Di use Unilateral Subacute Neuroretinitis

Mul iple evanescen whi e do syndrome Birdsho choriore ini is Sympa he ic oph halmia Nonspeci c op ic neuri is and papilli is La e s age Post rauma ic choriore inopa hy e ini is pigmen osa Occlusive vascular disease Sarcoidosis oxic re inopa hy Diagnostic Evaluation T e main es is biomicroscopy using he hree-mirror Goldman lens or 78-diop er lens. Blood serologies, blood smears and s ool samples are no help ul. Occasionally eosinophilia is ound. FA in he early s age o he disease demons ra es hypo uorescence o he ocal whi e yellowish lesions o ac ive re ini is ollowed by la e s aining. In he advanced s a es, here is an irregular increase in background choroidal uorescence. ICGA shows hypo uorescen dark spo s. Elec rore inogram: DUSN is charac erized by a dif use re inal in amma ion ha causes signi can elec rophysiologic al era ions o bo h he a- and b-waves. Goldman perime ry is use ul or evalua ing he visual eld be ore and a er rea men .

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Treatment When a live worm is ound, laser rea men o kill he nema ode is use ul a any disease s age. Success ul rea men can improve visual acui y and reduce in amma ory ocular signs. Oral rea men : Albendazole 400 mg/ day or 30 days can be used when a live worm is no ound. Prognosis Good i larva is visualized in he early s age. Poor i he diagnosis is delayed. EFE ENCES Cor ez , Denny JP, Mendoza M, e al. Dif use unila eral subacu e neurore ini is in Venezuela. Oph halmology. 2005;112:2110–2114. Garcia CA, Gomes AH, Garcia Filho CA, e al. Early-s age dif use unila eral subacu e neurore ini is: improvemen o vision a er pho ocoagula ion o he worm. Eye. 2004;18:624–627. Garcia CA, Gomes AH, Vianna N, e al. La e-s age di use unila eral subacu e neurore ini is: pho ocoagulaion o he worm does no improve he visual acui y o af ec ed pa ien s. In Oph halmol. 2005;26:39–42. Gass JD, Brauns ein R . Fur her observa ions concerning he dif use unila eral subacu e neurore ini is syndrome. Arch Oph halmol. 1983;101:1689–1697. Gass JDM. Dif use unila eral subacu e neurore ini is. In: Gass JDM, ed. S ereoscopic A las o Macular Disease: Diagnosis and Trea men . 4 h ed. S . Louis: Mosby-Year Book Inc.; 1997:622–628. Souza EC, Casella AMB, Nakashima Y, e al. Clinical eaures and ou comes o pa ien s wi h dif use unila eral subacu e neurore ini is rea ed wi h oral albendazole. Am J Oph halmol. 2005;140:437.

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FIGURE 9-65. No e he ac ive whi e-yellowish evanescen lesions in he early s age wi h a small live worm (A) and a er ocal laser pho ocoagula ion (B) .

A

B

C

D

FIGURE 9-66. A–C. No e he ac ive whi e-yellowish evanescen lesions in he early s age o a pa ien in whom no live worm could be ound. D. T e same pa ien 60 days a er albendazole rea men .

Di use Unilateral Subacute Neuroretinitis

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FIGURE 9-67. No e he op ic a rophy, narrowing o he re inal ar erioles and widespread mot led depigmen a ion o he re inal pigmen epi helium. T e subre inal worm was loca ed (and highligh ed in he inse ) .

FIGURE 9-68. Fundus ndings in he la e s ages o di use unila eral subacu e neurore ini is. No e he op ic a rophy and ar eriolar at enua ion wi h presence o a live worm (enlarged in he inse ) .

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ONCHO CERCIASIS Jason F. Okulicz

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nchocerciasis (river blindness) is a chronic in ec ion caused by he nemaode Onchocerca volvulus and primarily af ec s he skin and eye.

Etiology and Epidemiology Onchocerciasis is endemic in A rica, where approxima ely 95% o all in ec ed people live. I also occurs in La in America and Yemen. In ec ion reduces li e expec ancy by an average o 10 years. Onchocerciasis is a major cause o blindness, wi h approxima ely wo million people having blindness or severe visual impairmen . T e nema ode Onchocerca volvulus is ransmit ed rom person o person by he bi e o a black y (genus Simulium), which breeds near reely owing wa erways, hus he name “river blindness.” Micro lariae en er he skin and ma ure in o adul s, orming nodules in he subdermal connec ive issue, o en a bony prominences. Micro lariae released rom he adul worms migra e o various si es, mos commonly o he subepidermal lympha ics and he eye. Ocular pene ra ion by micro lariae begins hrough he bulbar conjunc iva a he limbus, hen hey invade he cornea, an erior chamber, and iris. T e micro lariae en er he pos erior segmen via hema ogenous spread or via he ciliary nerves. When he micro lariae die, he hos inci es an immune response, which is responT is con ribu ion o he work was done as par o he au hor’s o cial du ies as an NIH employee and is a work o he Uni ed S a es Governmen .

sible or mos o he ocular and derma ologic complica ions. Symptoms Early symp oms include ever, ar hralgias, and ransien ur icaria o he ace and runk. Pruri us is common; however, some pa ien s have no pruri us, while o her have severe, con inuous i ching. Conjunc ivi is or pho ophobia may occur early, wi h blindness becoming increasingly common as ime goes on. Signs ( Figs. 9-69 to 9-75) Onchocercoma, which are subcu aneous nodules, can be ound anywhere. A maculopapular rash is common, while skin licheni ca ion, hypopigmen a ion, or hyperpigmen a ion can also occur. More severe disease is charac erized by skin ulcera ions, epidermal a rophy, hanging groin (due o a rophic inguinal skin), emoral and inguinal lymphadeni is, and generalized corporal a rophy. Micro lariae may be seen wi hin he cornea or migra ing reely in he an erior chamber and vi reous humor. Corneal in l ra ion o micro lariae causes punc a e kera i is ha over ime can cause sclerosing kera i is. Corneal opaci ca ion and neovascularizaion secondary o in amma ion induced by dead micro lariae occur as lymphocy es and eosinophils in l ra e he peripheral cornea and lead o a sclerosing kera i is. An erior uvei is Nongranuloma ous or granuloma ous in amma ion occurs early and may resul rom invasion o he iris and ciliary body by he micro lariae or as a response o dead micro lariae. Iri is occurs in abou 10% o 20% o cases wi h ocular involvemen .

Onchocerciasis

A pseudo-hypopyon composed o micro lariae can occur. Pos erior synechiae may cause in erior pupil dis or ion giving he classic pearshaped iris, and may lead o pupillae occlusio e seclusio, iris bombe, iris a rophy, in amma ory or angle closure glaucoma, and ca arac . Choriore ini is is presen in 10% o 25%o pa ien s wi h ocular involvemen , and dif use PE mot ling wi h subre inal brosis can occur. T e macula is generally spared, wi h cenral visual acui y main ained un il la e in he disease. Op ic a rophy can be ound in 25% o cases wi h ocular involvemen Dif erential Diagnosis Syphilis Yaws Scleroderma Uvei ic glaucoma or chronic angle closure glaucoma HSV in ec ion An erior ischemic op ic neuropa hy Sarcoidosis rachoma B In ers i ial kera i is A opic kera oconjunc ivi is Neuro rophic kera opa hy Diagnostic Evaluation radi ionally, he diagnosis is based on a “skin snip,” which en ails ob aining a 3- o 5-mg skin snip rom an af ec ed area and examining he issue or direc visualiza ion o micro lariae. Mos sensi ive and speci c es overall Less sensi ive or early or mild in ec ions

225

Becoming increasingly unaccep able o local popula ions due o invasiveness PC es s can be used o ampli y parasi e DNA sequences rom skin snips and increase sensi ivi y. Direc visualiza ion o micro lariae by sli lamp examina ion o he cornea and an erior chamber o he eye. O her es s apid- orma an ibody cards U ilize serum specimens o de ec an ibodies, such as IgG4 an ibodies o recombinan Onchocerca volvulus an igen Ov16 Dips ick assays De ec oncho-C27 an igen in urine or ears, and have high sensi ivi y and speci ci y Treatment Ivermec in is he drug o choice given i s high e cacy and low oxici y. Preven s ocular disease and elimina es skin disease A single 150 mcg/ kg dose clears microlariae rom skin or several mon hs. Does no af ec adul worms Adverse reac ions are similar o he sysemic responses o dying micro lariae Fever, edema, pruri us, lymphadeni is, and body aches Frequency o adminis ra ion is con roversial Up o 33% o pa ien s in nonendemic areas cured wi h a single dose Mos pa ien s require addi ional herapy given he li espan o adul worms is 12 o 15 years. Annual ivermec in appears o reduce in raocular in amma ion.

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Doxycycline arge s symbio ic Wolbachia bac eria essen ial or worm er ili y Usual course is doxycycline 100 mg PO b.i.d. or 6 weeks in addi ion o ivermec in Combina ion ivermec in and doxycycline herapy has been shown o reduce micro larial load, which may af ec ransmission and may reduce or preven blindness. Prognosis Generally is good in pa ien s who receive proper herapy be ore irreversible ocular lesions develop.

Ivermec in rea s skin mani es a ions and hereby reduces morbidi y and improves quali y o li e. EFE ENCES CDI S udy Group. Communi y-direc ed in erven ions or priori y heal h problems in A rica: resul s o a mul icoun ry s udy. Bull World Heal h Organ. 2010;88( 7): 509–518. Enk CD. Onchocerciasis—river blindness. Clin Derma ol. 2006;24:176–180. Hopkins AD. Ivermec in and onchocerciasis: is i all solved? Eye. 2005;19:1057–1066.

FIGURE 9-69. Pho ograph demons ra ing punc a e kera i is ha resul s rom in l ra ion o micro lariae.

FIGURE 9-70. Pho ograph showing sclerosing kera i is.

Onchocerciasis

FIGURE 9-71.T e “leopard-spo ” pat ern o skin depigmen a ion.

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FIGURE 9-72. An onchocercoma adjacen o he knee.

FIGURE 9-73. A rophic inguinal skin can resul in he complica ion o hanging groin.

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9 INFECTIO US PO STERIO R UVEIT IS

B

FIGURE 9-74. A. Pho omicrograph o a skin biopsy specimen demons ra ing an adul worm in cross-sec ion ( hema oxylin and eosin s ain) . B. Pho omicrograph o a gravid adul emale worm rom skin biopsy specimen ( hema oxylin and eosin s ain) .

FIGURE 9-75. T is pa ien has di use choriore inal scarring due o onchocerciasis. (Cour esy o Nida Sen, MD, and Rober Nussenblat , MD.)

Loiasis

LOIASIS Rajeev Jain and Dinesh Selva

L

oiasis, also known as Loa loa lariasis, A rican eye worm, Calabar swellings, and ugi ive swellings, is due o a subcu aneous nema ode (roundworm), Loa loa, which causes bo h cu aneous and ocular disease. In ec ed pa ien s may have pruri us, subcu aneous swelling, migra ing lesions, and marked eosinophilia. Adul worms are 25 o 70 mm long.

Etiology and Epidemiology Loiasis is endemic in he rain ores areas o wes ern and cen ral A rica. Sporadic cases occur in migran s o and ravelers rom hese areas. I is a chronic in ec ion, in ec ing millions o people. ransmission occurs hrough he bi e o an in ec ed emale y rom he genus Chrysops, which is also known as he mango y, deer y, or horse y. T ese blood-sucking ies in roduce larial larvae in o he skin, where hey develop in o adul s. T e adul s migra e subcuaneously and may be seen moving undernea h he conjunc iva. Humans are he only known reservoir, and he nema ode can live or several years in he subcu aneous issue. Signs and Symptoms ( Figs. 9-76 to 9-78) Al hough he majori y o in ec ions are asymp oma ic, skin and eye involvemen may mani es clinically. Sys emic Calabar swellings are localized, in amma ory, nonery hema ous subcu aneous swellings ha are 15 o 20 mm in size. T ey occur in he ex remi ies o en adjacen o he join s, and las or a ew days o a ew weeks. T ey are areas o localized angioedema secondary o an immune response and can be associa ed wi h ur icaria and pruri us.

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Pa ien s may have migra ory myalgias, ar hralgias, and pit ing edema o he ex remi ies. O her hypersensi ivi y reac ions include glomerulonephri is, mo or and sensory de ci s, as hma, endomyocardial brosis, and eosinophilia. Ocular T e worm can migra e under he conjunc iva. Pa ien s may presen wi h a red and pain ul eye wi h conjunc ival injec ion, chemosis, or subconjunc ival hemorrhage. However, pa ien s may be asymp oma ic or no e only mild ocular irri a ion or a sensaion o movemen across he ocular sur ace. T e adul worm may be seen moving undernea h he conjunc iva or as an immobile, coiled ranslucen s ruc ure. Pain ul eyelid swelling has also been repor ed. Less commonly, he worm may be visible in he an erior chamber. Diagnostic Evaluation Direc isola ion o Loa loa worm rom he calabar swellings or subconjunc ival issue provides he mos de ni ive diagnosis. T e diagnosis may also be made by demons ra ion o micro lariae in he blood. Blood collec ion should be per ormed during he day ime, be ween 10 am and 2 pm, when micro larial densi y is highes . However, micro lariae may be absen in bloods ream in cases o unisexual in ec ion and unde ec able in up o 30% o pa ien s who have bisexual in ec ions. Signi can eosinophilia is o en presen . Pa ien s may also have eleva ed IgE. Al hough an igen de ec ion using immunoassay or circula ing an igens is possible, a high level o an igenic cross reac ivi y be ween dif eren helmin hic an igens has limi ed i s use.

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Treatment Drugs T e drug o choice remains die hylcarbamazine ci ra e (DEC) 6 mg/ kg/ day divided .i.d. and aken or a leas 2 weeks. I is ef ec ive agains micro lariae and less ef ec ive agains adul worms, so repea ed rea men s may be required or comple e cure. However, his should be used wi h cau ion, as DEC may precipia e encephalopa hy i here is high micro larial load. I DEC is no olera ed or here is high risk o precipi a ing encephalopa hy, albendazole 200 mg aken b.i.d. or 3 weeks should be considered. epea ed courses may be necessary. Ivermec in may also be given in a single dose o 200 o 400 µg/ kg. However, here is a higher risk o precipi a ing encephalopa hy, especially in areas ha also are endemic or Onchocerca.

An ihis amines and oral cor icos eroids may also be required o rea allergic reacions ha may develop as a consequence o dying micro lariae. Surgery Surgical ex rac ion rom he subconjunc ival space or excision biopsy o calabar swelling can also be per ormed prior o ins i u ion o drug herapy. When he worm is visualized, subconjunc ival or opical lidocaine can be used o anes he ize he eye. T e visible worm can hen be removed hrough a small conjunc ival peri omy. EFE ENCES Barua P, Barua N, Hazarika NK, e al. Loa loa in he an erior chamber o he eye: a case repor . Indian J Med Microbiol. 2005;23:59–60. Boussinesq M. Loiasis. Annals o Tropical Medicine & Parasi ology. 2006:100( 8):715–731. Jain , Chen JY, Bu cher A , e al. Subconjunc ival Loa loa worm. In J In ec Dis. 2008;12( 6):e133–135. Khe an VD. Subconjunc ival Loa loa wi h Calabar swelling. Indian J Oph halmol. 2007;55:165–166.

FIGURE 9-76. A subconjunc ival worm is visible as a ranslucen , whi e, mobile hread-like s ruc ure under he in erior bulbar conjunc iva. (Reproduced rom Jain R, Chen JY, Bu cher AR, e al. Subconjunc ival Loa loa worm. Int J Infect Dis.2008; 12( 6) :e133–135.)

Loiasis

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FIGURE 9-77.T e emale Loa loa worm is seen ollowing surgical ex rac ion hrough a conjunc ival peri omy. (Reproduced rom Jain R, Chen JY, Bu cher AR, e al. Subconjunc ival Loa loa worm. Int J Infect Dis.2008; 12( 6) :e133–135.)

t

h

FIGURE 9-78. T e adul emale Loa loa excised rom he pa ien in Figure 9-77. T e leng h rom he head ( h) and ail ( ) was 57.7 mm. I is approxima ely 0.5 mm wide. (Reproduced rom Jain R, Chen JY, Bu cher AR, e al. Subconjunc ival Loa loa worm. Int J Infect Dis. 2008;12( 6):e133–135.)

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OCULAR CYSTICERCOSIS Kim Ramasamy

C

ys icercosis is a sys emic illness caused by dissemina ion o he larval orm o he pork apeworm, Taenia solium. When humans inges con amina ed soil, wa er, or undercooked pork ha con ains T. solium cys icerci, he individual becomes he de ni ive hos and carries an in es inal adul apeworm ( aeniasis). A person wi h apeworm sheds eggs and apeworms in heir eces. When humans inges eggs o T. solium, hey develop cys icercosis wi hin organs (similar o wha happens in pigs), and are an acciden al in ermedia e hos . I is his ype o inges ion ha can lead o he ocular ndings.

Etiology and Epidemiology In ec ion wi h eggs resul s rom inges ion o he larvae hrough in ec ed ood handlers, inges ion o rui and vege ables con amina ed wi h human was e, or by eces-con amina ed wa er supplies in endemic areas. T e eggs become larvae ha cross he in es inal wall, en er he blood or lympha ic circula ion, and hen go o he eye, muscles, and neural issue. Cys icercosis af ec s an es ima ed 50 million people worldwide. I is ound in areas wi h poor sani a ion, and endemic areas include La in America including Mexico, subSaharan A rica, India, and Eas Asia. Ocular and orbi al cys icercosis mos commonly occurs in children and young adul s. Symptoms Blurry vision and oa ers. In cases o rupured cys icercosis, pa ien s may be much more symp oma ic rom he ensuing in amma ory reac ion. Pa ien s may have diplopia or s rabismus. Pa ien s wi h neurocys icercosis can develop epilepsy.

Signs ( Figs. 9-79 to 9-83) ranslucen cys wi h or wi hou characeris ic undula ing movemen s may be presen in he subre inal space, vi reous cavi y, conjunc iva, an erior segmen , ex raocular muscles, eyelid, or orbi . Occasionally, he worm crosses he macula causing signi can vision loss. I he cys rup ures, he pa ien may develop pro ound vi ri is, proli era ive vi reore inopa hy, uvei is, rhegma ogenous or exuda ive re inal de achmen , re inal hemorrhages, disk edema, cycli ic membrane ormaion, and ph hisis. Orbi al cys icercosis is charac erized by prop osis, globe displacemen , s rabismus, and res ric ed mo ili y. Dif erential Diagnosis oxocara Masquerade syndromes Endogenous endoph halmi is Severe oxoplasmosis Diagnostic Evaluation T e organism is o en visible via sli -lamp exam and indirec oph halmoscopy. I a hazy media is presen , B-scan ul rasonography can be used o visualize he cys . C scan o he brain is used o diagnose neurocys icercosis, and whole-body C can be used or sys emic screening. T e parasi e can be ound in s ool samples, and an ibodies can be de ec ed rom he blood or s ool. Treatment Medical: Niclosamide and praziquan el are used o rea adul worms and praziquan el and me ri ona e can be used or cys icercosis. T ese medica ions should be adminis ered wi h concomi an s eroids as dea h o he worm can resul in signi can in raocular in amma ion. Surgical removal o he parasi es is use ul. I he cys is presen in an erior chamber, a

Ocular Cysticercosis

paracen esis may be at emp ed. Pars plana vi rec omy wi h removal o cys is use ul or in raocular cys icercosis. Prognosis T e prognosis is poor in cases o rup ured cys icercosis wi h severe in amma ion.

A

C

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EFE ENCES Madigubba S, Vishwana h K, eddy G, e al. Changing rends in ocular cys icercosis over wo decades: an analysis o 118 surgically excised cys s. Indian J Med Microbiol. 2007;25( 3):214–219. a h S, Honavar SG, Naik M, e al. Orbi al cys icercosis: clinical mani es a ions, diagnosis, managemen , and ou come. Oph halmology. 2010;117( 3):600–605, 605.e1. Epub 2010 Jan 8.

B

FIGURE 9-79. A. ranslucen cys wi h charac eris ic undula ing movemen s ha appears like a “living mobile pearl.” B. T e scolex ( he “head” o he worm) pro ruding ou o he cys . C.T e cys is in he subre inal space.

FIGURE 9-80. T e subre inal cys icercosis has caused a exuda ive re inal de achmen .

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FIGURE 9-81. T e B-scan ul rasonogram demons ra es he subre inal cys icercosis and he scolex.

A

B

FIGURE 9-82. A.T e cys bored a hole rom he subre inal space, hrough he re ina and en ered he vi reous cavi y. T is passage inci ed inf amma ion, leaving behind a choriore inal scar. B.T e rup ured cys icercosis caused in ense vi reous reac ion.

FIGURE 9-83. In racranial cys icercosis shown in FLAIR MRI image in which f uid appears dark. T e CSF in he ven ricles appears dark as do he numerous round black “holes” which are f uid- lled cys s. T e brigh signal around he cys s is edema in he adjacen brain parenchyma. T e degree o disease is ex ensive, wi h cor ical, subcor ical, and deep brain lesions. (In erpre a ion cour esy o Michael Du ka, MD.)

Rhinosporidosis

RHINOSPORIDOSIS S. R. Ra hinam

235

corrodes he sclera, resul ing in hinning and s aphyloma orma ion. Pa ien s may develop dacryocys i is.

hinosporidiosis is a chronic granuloma ous disease ha af ec s he mucosa o he nose, conjunc iva, lacrimal sac, or ure hral mea us. T e lesion presen s as a discre e, riable, painless, slow-growing, polypoidal, peduncula ed, or sessile mass.

R

Dif erential Diagnosis Episcleri is Scleri is S aphyloma Gian papillary conjunc ivi is

Etiology and Epidemiology T e causa ive agen , Rhinosporidium seeberi, is an endosporula ing microorganism. Spores are ransmit ed rom dus and con amina ed wa er; ba hing in s agnan wa er in endemic areas is considered a major risk ac or. T e presumed mode o in ec ion is rom i s na ural aqua ic habi a hrough he mucosa or raumaized epi helium. I is endemic in India, Sri Lanka, Sou h America, Malawi, Kenya, Uganda, and he Congo.

Diagnostic Evaluation His opa hology is cri ical o con rm he diagnosis, as i very hard o cul ure. S aining he his opa hology o he conjunc ival scrapings wi h hema oxylin and eosin shows sporangia o dif eren sizes con aining spores.

Symptoms hini is, epis axis, and nasal obs ruc ion Foreign body sensa ion ed eye Dark-colored swelling on he eye Signs ( Figs. 9-84 to 9-87) T e mos common involvemen is he nasal and nasopharyngeal mucosa, ollowed by he eye. T e polyps are a red, eshy, conjunc ival lesion wi h small yellow-whi e do s on i s surace represen ing he sporangia. When he in ec ion is in he bulbar conjunc iva, here is no space or he oculosporidium o grow ou as a polyp as he af ec ed area is compressed by he lids. Hence, hese lesions are usually sessile and spread along he bulbar conjunc iva and grow deep oward he sclera. I has been pos ula ed ha some enzyma ic subs ance produced by he organism

Treatment Simple excision o he polyp wi h cryo herapy can work or smaller, circumscribed lesions. I here is lacrimal sac rhinosporidiosis, comple e excision o he sac may be necessary. I a s aphyloma occurs, cau eriza ion o he base o s aphyloma and homologous sclera gra can be per ormed. Oral dapsone 100 mg once or wice a day or 3 o 6 mon hs may rea he in ec ion. Prognosis ecurrence, chronici y, spread o ana omically close si es and secondary bac erial in ecions are he mos requen complica ions. EFE ENCES Arseculera ne SN. ecen advances in rhinosporidiosis and Rhinosporidium seeberi. Indian J Med Microbiol. 2002;20( 3):119–131. Capoor M, Khanna G, ajni, e al. hinosporidiosis in Delhi, Nor h India: Case Series rom a Non-endemic Area and Mini-review. Mycopa hologia. 2009;168: 89–94. Fredricks DN, Jolley JA, Lepp PW, e al. Rhinosporidium seeberi: a human pa hogen rom a novel group o aqua ic pro is an parasi es. Emerg In ec Dis. 2000;6( 3):273–282.

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A

B

C

FIGURE 9-84. A. Conjunc ival rhinosporidiosis approxima ely 5 mm in diame er wi h charac eris ic pale, yellow-whi e spo s scat ered on he sur ace. B.T e pa ien underwen excision o he grow h, and a pos excision pho ograph shows in ac scleral issues (arrow). C. His opa hologic sec ions wi h hema oxylin and eosin s ain shows he sporangia in various s ages o ma uri y enclosed in a hick chi inous wall wi h in l ra ion o chronic inf amma ory cells. (H and E, × 400.)

FIGURE 9-85.T is is an advanced conjunc ival polyp wi h he charac eris ic pale yellow-whi e spo s wi h associa ed early scleral ec asia/ s aphyloma.

Rhinosporidosis

FIGURE 9-86.T is person has more signi can scleral ec asia wi h he uveal issue apparen as he dark blue color.

FIGURE 9-87.T is person has comple e scleral hinning superior o he cornea in he le eye wi h uveal prolapse.

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C H AP T ER

Endophthalmitis POSTOPE TIVE ENDOPHTH ALMITIS Stephen G. Schwartz , Harry W. Flynn, Jr., and Roy D. Brod

ENDOPHTHALMITIS

TIVE

E

ndophthalmitis is characterized by marked

etiology and most likely infecting organisms e largest category is acute-onset postoperative ing within 6 weeks of intraocular surgery. Etiology and Epidemiology Presents within 6 weeks of intraocular surgery Reported incidence rates are variable. In a recent large single-center series, reported rates were 0.025% overall, 0.028% following cataract surgery, 0.2% following secondary intraocular lens (IOL) implantation, 0.108%

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following penetrating keratoplasty, and 0.011% following 20-gauge pars plana vitrectomy ( PPV) . e Endophthalmitis Vitrectomy Study (EVS) recruited patients with acute-onset postoperative endophthalmitis following cataract surgery or secondary IOL implantation. In the EVS, 69% of patients had positive vitreous cultures. Of these, the most common etiologic organisms were coagulase-negative staphylococci. Preoperative risk factors include immune compromise (including diabetes mellitus), active systemic infection, active blepharitis or conjunctivitis, and disease of the lacrimal drainage system. Intraoperative risk factors include prolonged or complicated surgery, secondary IOL implantation, posterior capsular rupture, vitreous loss, iris prolapse, contaminated irrigating solutions or IOLs, and inferotemporal placement of clear corneal incisions. Some authors have suggested that a clear corneal, sutureless incision for cataract surgery is a risk factor for endophthalmitis.

Postoperative Endophthalmitis

Pos opera ive risk ac ors include wound leak, vi reous incarcera ion in he wound, and con amina ed eye drops. Symptoms apid onse o visual loss, redness, and pain Signs ( Figs. 10-1 and 10-2) Marked in raocular (an erior chamber and vi reous) inf amma ion wi h an erior chamber brin and hypopyon Eyelid edema, conjunc ival conges ion, corneal edema, and re inal periphlebi is may occur o a variable degree. Dif erential Diagnosis oxic an erior segmen syndrome ( ASS) generally occurs earlier (wi hin 1 or 2 days) and may be associa ed wi h lit le or no pain, as well as lit le or no pos erior segmen inf amma ion. e ained lens ma erial Flare-up o pre-exis ing uvei is riamcinolone ace onide par icles Long-s anding (dehemoglobinized) vi reous hemorrhage Diagnostic Evaluation Acu e-onse pos opera ive endoph halmiis is a clinical diagnosis, ollowed by laboraory con rma ion. I he pos erior segmen canno be visualized, B-scan ul rasonography may be help ul o rule ou re inal de achmen , suprachoroidal hemorrhage, or re ained lens ragmen . Aqueous and vi reous cul ures. Vi reous samples are more likely o yield a posi ive culure han aqueous samples. Vi reous cul ures may be ob ained ei her wi h a needle ( ap) or wi h PPV ins rumen a ion. Commonly used cul ure media include 5% blood agar (mos common organisms), chocola e agar ( as idious organisms, such as N. gonorrhoeae and H. inf uenzae), Sabouraud

239

agar ( ungi), hioglycolla e bro h (anaerobes), and anaerobic blood agar (anaerobes). Blood cul ure bot les may also be used and are help ul in a er-hours cases. Treatment T e EVS repor ed ha or pa ien s wi h acu e-onse pos opera ive endoph halmi is ollowing ca arac surgery or secondary IOL implan a ion and presen ing visual acui y o ligh percep ion, PPV was associa ed wi h improved visual ou comes when compared o vi reous ap. For pa ien s wi h hand mo ion vision or bet er, he resul s o in ravi real ap wi h injec ion o an ibio ics were similar o PPV. In diabe ic pa ien s wi h presen ing visual acui y o hand mo ions or bet er, here was a rend oward bet er visual ou comes in pa ien s rea ed wi h PPV, bu his was no s a is ically signi can . T e EVS rea ed all pa ien s wi h in ravi real vancomycin (1 mg in 0.1 mL) and amikacin (0.4 mg in 0.1 mL). However, o reduce he risk o aminoglycoside oxici y, ei her ce azidime (2.25 mg in 0.1 mL) or ce riaxone (2 mg in 0.1 mL) may be considered. T e EVS repor ed no addi ional visual beneassocia ed wi h he adjunc ive use o sys emic amikacin and ce azidime. However, our hgenera ion sys emic f uoroquinolones such as moxif oxacin achieve in raocular pene ra ion and may be considered or adjunc ive use, al hough suppor ing evidence o e cacy is lacking. In pa ien s wi h acu e-onse pos opera ive endoph halmi is wi h suspec ed bac erial e iology, in ravi real dexame hasone (0.4 mg in 0.1 mL) may be considered. T e EVS rea ed all pa ien s wi h subconjunc ival vancomycin (25 mg in 0.5 mL), ce azidime (100 mg in 0.5 mL), and dexame hasone (6 mg in 0.25 mL). However, subsequen clinical rials have demons ra ed ha here may be no addi ional bene associa ed wi h subconjunc ival an ibio ics.

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T e EVS rea ed all pa ien s wi h sys emic prednisone (30 mg b.i.d. or 5 o 10 days). However, sys emic cor icos eroids should be used wi h cau ion in cer ain a -risk pa ien s, including diabe ics and he elderly. T e EVS rea ed all pa ien s wi h or i ed opical vancomycin (50 mg/ cc) and or i ed opical amikacin (20 mg/ cc), up o every hour. As a subs i u e, commercially available opical an ibio ics (such as our h-genera ion f uoroquinolones) may be considered. In addi ion, he EVS rea ed all pa ien s wi h opical cor icos eroids and cycloplegics. I he clinical s a us appears o be worsening a 48 o 72 hours, considera ion should be given o recul uring and reinjec ion o an ibio ics based on he ini ial cul ure resul s. I he ini ial cul ure was a needle ap, PPV can be considered or he subsequen procedure. Prognosis T e s ronges predic or o nal visual ou come in he EVS was presen ing visual acui y. T ere ore, promp ini ia ion o rea men is

FIGURE10- 1. T is person has conjunc ival chemosis, injec ion, hypopyon, and f brin in he an erior chamber, all indica ive o acu e-onse pos opera ive endoph halmi is.

more impor an han any o her ac or, including vi reous ap versus PPV. O her predic ors o less avorable ou comes in he EVS included older age, diabe es melli us, corneal in l ra e, abnormal in raocular pressure, an erior segmen neovascularizaion, absen red ref ex, and open pos erior capsule. EFE ENCES Endoph halmi is Vi rec omy S udy Group. esul s o he Endoph halmi is Vi rec omy S udy: a randomized rial o immedia e vi rec omy and o in ravenous an ibio ics or he rea men o pos opera ive bac erial endoph halmi is. Arch Ophthalmol. 1995;113:1479–1496. Lalwani GA, Flynn HW Jr, Scot IU, e al. Acu e-onse endoph halmi is a er clear corneal ca arac surgery (1996–2005). Clinical ea ures, causa ive organisms, and visual acui y ou comes. Ophthalmology. 2008;115: 473–476. Schwar z SG, Flynn HW Jr, Scot IU. Endoph halmi is: classi ca ion and curren managemen . Exp Rev Ophthalmol. 2007;2:385–396. Wyko CC, Parrot MB, Flynn HW Jr., e al. Nosocomial acu e-onse pos opera ive endoph halmi is a a universi y eaching hospi al (2002–2009) . Am J Ophthalmol. 2010 Jul 7 [Epub ahead o prin ].

FIGURE10- 2. T e pa ien has acu e pos opera ive endoph halmi is. In his case, here is a pro use amoun o purulen ma erial wi h an opaque cornea, sugges ive o an organism such as Pseudomonas aeruginosa.

Postoperative Endophthalmitis

DELAYED-ONSET (CH ONIC) POSTOPER TIVE ENDOPHTHALMITIS

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ndoph halmi is is charac erized by marked inf amma ion o in raocular issues and f uids. Delayed-onse (chronic) pos opera ive endoph halmi is is de ned as endoph halmi is presen ing more han 6 weeks a er in raocular surgery.

Etiology and Epidemiology Presen s more han 6 weeks ollowing in raocular surgery In one single-cen er series, he repor ed ra e o delayed-onse pos opera ive endoph halmiis ollowing ca arac surgery was 0.017%. Common causa ive organisms in cases o chronic pos opera ive endoph halmi is include P. acnes, ungi, and various less virulen gram-posi ive and gram-nega ive organisms. Symptoms Insidious onse o visual loss, redness, pho ophobia, and pain. Symp oms are ypically less severe han in pa ien s wi h acu eonse pos opera ive endoph halmi is. Signs ( Fig. 10-3) Slowly progressive in raocular inf ammaion wi h variable occurrence o hypopyon and kera ic precipi a es. A whi e in racapsular plaque may be presen and may be indica ive o P. acnes in ec ion. Signs are ypically less severe han in pa ien s wi h acu e-onse pos opera ive endoph halmi is. Eyelid edema, conjunc ival conges ion, corneal edema, and aqueous and vi reous inf amma ion may occur, bu generally o a lesser degree han in acu e-onse pos operaive endoph halmi is. Dif erential Diagnosis Nonin ec ious uvei is e ained lens ma erial

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riamcinolone ace onide par icles Longs anding (dehemoglobinized) vi reous hemorrhage Diagnostic Evaluation Delayed-onse (chronic) pos opera ive endoph halmi is is a clinical diagnosis, conrmed wi h labora ory es ing. I he pos erior segmen canno be visualized, B-scan ul rasonography may be help ul o rule ou re inal de achmen , suprachoroidal hemorrhage, and re ained lens ragmen . Cul ures: Vi reous samples are more likely o yield a posi ive cul ure han are aqueous samples. Vi reous cul ures may be ob ained ei her wi h a needle ( ap) or wi h PPV ins rumen a ion. Commonly used cul ure media include 5% blood agar (mos common organisms), chocola e agar ( as idious organisms, such as N. gonorrhoeae and H. inf uenzae), Sabouraud agar ( ungi), hioglycolla e bro h (anaerobes), and anaerobic blood agar (anaerobes). I delayed-onse (chronic) pos opera ive endoph halmi is is suspec ed, he labora ory should be ins ruc ed o hold he cul ures or a leas 2 weeks o allow isola ion o as idious organisms. Blood cul ure bot les may also be used. Treatment Many au hors recommend ini ial PPV wi h capsulec omy in cases o chronic pos operaive endoph halmi is. Al erna ively, one may consider ini ial ap and injec , ollowed by more invasive surgery i he clinical examinaion does no improve. T e Endoph halmi is Vi rec omy S udy (EVS) did no enroll pa ien s wi h delayed-onse (chronic) pos opera ive endoph halmi is, so i s ndings regarding vi reous ap versus PPV are no necessarily applicable o hese pa ien s. I PPV wi h capsulo omy or capsulec omy is no success ul,

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explan a ion o he IOL and removal o he capsular bag may be necessary. Similar o acu e-onse pos opera ive endoph halmi is, a reasonable ini ial rea men would include in ravi real vancomycin (1 mg in 0.1 mL) and amikacin (0.4 mg in 0.1 mL). However, o reduce he risk o aminoglycoside oxici y, ei her ce azidime (2.25 mg in 0.1 mL) or ce riaxone (2 mg in 0.1 mL) may be considered. I ungal endoph halmi is is suspec ed, ei her in ravi real ampho ericin B (0.005 mg in 0.1 mL) or in ravi real voriconazole (0.1 mg in 0.1 mL) may be used. Adjunc ive sys emic an iungals may be used, ypically in consul a ion wi h an in ernis or in ec ious disease specialis . In pa ien s wi h suspec ed bac erial e iology, in ravi real dexame hasone (0.4 mg in 0.1 mL) may be considered. However, ungal in ec ions are rela ively more common in pa ien s wi h delayed-onse (chronic) pos opera ive endoph halmi is, so in ravi real dexame hasone should be used wi h cau ion in hese pa ien s. Similar o acu e-onse pos opera ive endoph halmi is, subconjunc ival vancomycin (25 mg in 0.5 mL), ce azidime (100 mg in 0.5 mL), and dexame hasone (6 mg in 0.25 mL) can be given. Similar o acu e-onse pos opera ive endoph halmi is, sys emic cor icos eroids should be used wi h cau ion in cer ain a -risk pa ien s, including diabe ics and he elderly. In addi ion, ungal in ec ions are rela ively more common in hese pa ien s, so sys emic

cor icos eroids should be used wi h cau ion i ungal in ec ion is suspec ed. For i ed opical vancomycin (50 mg/ cc) and or i ed opical amikacin (20 mg/ cc) may be bene cial. However, or i ed opical an ibio ics may require access o a compounding pharmacy, which may no be available in all loca ions. As a subs i u e, commercially available opical an ibio ics (such as our h-genera ion f uoroquinolones) may be considered. In addi ion, opical cor icos eroids and cycloplegics are sugges ed. Prognosis Because delayed-onse (chronic) pos opera ive endoph halmi is is requen ly caused by indolen organisms, he prognosis may be sligh ly more avorable han or pa ien s wi h acu e-onse pos opera ive endoph halmi is caused by more virulen species. EFE ENCES Al-Mezaine HS, Al-Assiri A, Al- ajhi AA. Incidence, clinical ea ures, causa ive organisms, and visual ou comes o delayed-onse pseudophakic endoph halmi is. Eur J Ophthalmol. 2009;19:804–811. Clark WL, Kaiser PK, Flynn HW Jr, e al. rea men s ra egies and visual acui y ou comes in chronic pos opera ive Propionibacterium acnes endoph halmi is. Ophthalmology. 1999;106:1665–1670. Doshi , Arevalo JF, Flynn HW Jr, e al. Evalua ing exaggera ed, prolonged, or delayed pos opera ive in raocular inf amma ion. Am J Ophthalmol. 2010 July 12 [Epub ahead o prin ]. Schwar z SG, Flynn HW Jr, Scot IU. Endoph halmi is: classi ca ion and curren managemen . Exp Rev Ophthalmol. 2007;2:385–396.

FIGURE 10-3. T is eye had delayed-onse pos opera ive endoph halmi is. Ex ernally, he eye is no injec ed, and no an erior chamber kera ic precipi a es or hypopyon is no ed. T e whi e plaque wi hin he capsular bag is sugges ive o P. acnes, which was he organism even ually isola ed on cul ures.

Postoperative Endophthalmitis

FILTE ING BLEB-ASSOCIATED ENDOPHTHALMITIS

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ndoph halmi is is charac erized by marked inf amma ion o in raocular issues and f uids. In a pa ien wi h endoph halmi is, he e iology and mos likely in ec ing organisms may be predic ed by he clinicalset ing.Endoph halmi is associa ed wi h l ering blebs has cer ain unique and impor an charac eris ics. Etiology and Epidemiology I may presen mon hs o years ollowing rabeculec omy. epor ed incidence ra es are variable. In a large single-cen er series, he repor ed ra e was 0.2%. Preopera ive risk ac ors include ac ive blephari is or conjunc ivi is, con amina ed eye drops, con ac lens wear, and lacrimal drainage sys em abnormali ies. In raopera ive risk ac ors include an in erior l ering bleb and use o an ime aboli es. Pos opera ive risk ac ors include bleb leak, hin-walled blebs, bleb manipula ions, or blebi is. Symptoms Vision loss, redness, and pain. A er he onse o ini ial symp oms, progression may be ei her rapid or insidious. Signs ( Figs. 10-4 and 10-5) Purulen (whi e) bleb Marked in raocular inf amma ion wi h an erior chamber brin and hypopyon Eyelid edema, conjunc ival conges ion, corneal edema, vi ri is, and re inal periphlebiis may occur. Dif erential Diagnosis Blebi is: Generally associa ed wi h less inf amma ion, less pain, and lit le or no vi reous inf amma ion

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Nonin ec ious uvei is riamcinolone ace onide par icles Longs anding (dehemoglobinized) vi reous hemorrhage Diagnostic Evaluation Fil ering bleb-associa ed endoph halmi is is a clinical diagnosis, ollowed by labora ory con rma ion. I he pos erior segmen canno be visualized, B-scan echography may be help ul o rule ou re inal de achmen or suprachoroidal hemorrhage. Ocular cul ures: Vi reous samples are more likely o yield a posi ive cul ure han aqueous samples. Vi reous cul ures may be ob ained ei her wi h a needle ( ap) or wi h PPV ins rumen a ion. Commonly used cul ure media include 5% blood agar (mos common organisms), chocola e agar ( as idious organisms, such as N. gonorrhoeae and H. inf uenzae), Sabouraud agar ( ungi), hioglycolla e bro h (anaerobes), and anaerobic blood agar (anaerobes). Blood cul ure bot les may also be used and are help ul in a er-hours cases. Treatment Some au hors recommend ini ial PPV in cases o l ering bleb-associa ed endoph halmi is. Al erna ively, one may consider ini ial ap and injec , ollowed by more invasive surgery i he clinical examina ion does no improve. T e EVS did no enroll pa ien s wi h l ering bleb-associa ed endoph halmi is, so i s ndings regarding vi reous ap versus PPV are no necessarily applicable o hese pa ien s. Similar o acu e-onse pos opera ive endoph halmi is, ini ial rea men includes in ravi real vancomycin (1 mg in 0.1 mL) and amikacin (0.4 mg in 0.1 mL). However, o reduce he risk o aminoglycoside oxici y,

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ei her ce azidime (2.25 mg in 0.1 mL) or ce riaxone (2 mg in 0.1 mL) may be considered. In pa ien s wi h l ering bleb-associa ed endoph halmi is, in ravi real dexame hasone (0.4 mg in 0.1 mL) may be considered. Similar o acu e-onse pos opera ive endoph halmi is, subconjunc ival vancomycin (25 mg in 0.5 mL), ce azidime (100 mg in 0.5 mL), and dexame hasone (6 mg in 0.25 mL) can be used. Similar o acu e-onse pos opera ive endoph halmi is, sys emic cor icos eroids should be used wi h cau ion in cer ain a -risk pa ien s, including diabe ics and he elderly. For i ed opical vancomycin (50 mg/ mL) and or i ed opical amikacin (20 mg/ mL) may be bene cial. However, or i ed opical an ibio ics may require access o a compounding pharmacy, which may no be available in all loca ions. As a subs i u e, commercially available opical an ibio ics (such as our h-genera ion f uoroquinolones) may be considered. In addi ion, opical cor icos eroids and cycloplegics are sugges ed. Prognosis Pre-exis ing visual loss (e.g., end-s age glaucoma) may limi visual recovery in hese pa ien s.

FIGURE10- 4. T e supero emporal f l ering bleb is ischemic cen rally, and is surrounded by injec ion o he bleb and conjunc iva. T ere is corneal edema, hypopyon, and f brin in he an erior chamber.

Because s rep ococcal and gram-nega ive organisms are more requen in his ca egory, he visual ou comes may be worse han in cases o acu e-onse pos opera ive endoph halmi is. Similar o acu e-onse pos opera ive endoph halmi is, he s ronges predic or o nal visual ou come is presen ing visual acui y. T ere ore, promp ini ia ion o rea men is more impor an han any o her ac or, including vi reous ap versus PPV. EFE ENCES Busbee BG, ecchia FM, Kaiser , e al. Bleb-associa ed endoph halmi is: clinical charac eris ics and visual ou comes. Ophthalmology. 2004;111:1495–1503. Endoph halmi is Vi rec omy S udy Group. esul s o he Endoph halmi is Vi rec omy S udy: a randomized rial o immedia e vi rec omy and o in ravenous an ibio ics or he rea men o pos opera ive bac erial endoph halmi is. Arch Ophthalmol. 1995;113:1479–1496. Schwar z SG, Flynn HW Jr, Scot IU. Endoph halmi is: classi ca ion and curren managemen . Expert Review Ophthalmol. 2007;2:385–396. Sharan S, rope GE, Chipman M, e al. La e-onse bleb in ec ions: prevalence and risk ac ors. Can JOphthalmol. 2009;44:279–283. Smiddy WE, Smiddy J, Ba-Ar h B, e al. Subconjunc ival an ibio ics in he rea men o endoph halmi is managed wi hou vi rec omy. Retina. 2005;25: 751–758.

FIGURE10- 5. T e in ec ed supero emporal bleb is whi e, surrounded by an injec ed, red background, he ypical appearance o bleb-associa ed endoph halmi is. T ere is mild corneal edema wi h a small hypopyon.

Endogenous Endophthalmitis

ENDO GENOUS ENDOPHTH ALMITIS Manohar Babu Balasundaram and S. R. Rathinam

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ndogenous endoph halmi is (EE) occurs due o hema ogenous dissemina ion o baceria or ungi o he eye. While many pa ien s will have o her evidence o sys emic in ec ion, such as endocardi is, urinary rac injec ion, pneumonia, skin in ec ions, or meningi is, a number o pa ien s may appear o be heal hy. A good his ory and high clinical suspicion is very impor an in hese cases. Etiology and Epidemiology Compromised hos de ense and sep ic ocus anywhere in he body T ose wi h a his ory o IV drug abuse, indwelling ca he ers, diabe es, hepa obiliary in ec ions, alcoholism, immunosuppressive disorders, and abdominal surgery are a higher risk. A number o organisms can cause EE, including: gram-posi ive organisms such as Streptococcus species, Staphylococcus aureus, and Bacillus cereus; gram-nega ive organisms such as Klebsiella pneumonia, Escherichia coli, and Pseudomonas aeruginosa, and ungi such as Candida albicans and Aspergillus species. Gram-posi ive organisms are predominan ly responsible or EE in Wes ern coun ries while gram-nega ive organisms and ungi are responsible or EE in Eas ern coun ries. Symptoms Sys emic symp oms are nonspeci c and depend on he primary source o sys emic sepsis, and can include ever, chills, myalgias, and pain in he speci c organ sys em involved. Ocular symp oms can include pain, blurry vision, pho ophobia, f oa ers. T e disease is

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usually unila eral, bu can be bila eral in up o 25% o cases. Signs ( Figs. 10-6 to 10-11) An erior ocal: Discre e oci o in ec ion, seen as iris nodules or microabscesses, wi h injec ion, and an erior chamber cell An erior di use: Severe inf amma ion, wi h lid edema, chemosis, corneal edema, and/ or hypopyon Pos erior ocal: Whi ish nodules or plaques, usually in he choroid, ha rapidly involve he re ina, wi h or wi hou o h spo s, cot on wool spo s, re inal vasculi is, vi ri is, and/ or vi reous snowballs Pos erior di use: Wi h in ense vi reous inf amma ion Dif erential Diagnosis Severe panuvei is oxoplasmosis Lep ospirosis Masquerade syndromes Pos opera ive endoph halmi is Diagnostic Evaluation Cul ures: T roa , nasal, blood, urine, indwelling ca he ers, and cerebrospinal f uid (CSF) and any o her clinically eviden sep ic oci should be considered or cul ure be ore ini ia ion o an ibio ic herapy. O en mul iple si es are cul ured a once. Abdominal ul rasound can be help ul o look or abscess orma ion, especially in he hepa obiliary sys em. Ches imaging can be used o iden i y abscess orma ion or pneumonia. In raocular cul ures rom aqueous and vi reous can be help ul i here is no o her posiive specimen. rans horacic and ransesophageal echocardiograms can be use ul o evalua e pa ien s or endocardi is.

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es ing or immunocompromised s a es, including HIV should be considered. Treatment rea men is ailored o he organism and organ sys em involved, and mainly depends on iden i ca ion o he causa ive agen . Sys emic broad-spec rum IV an ibio ics, such as vancomycin and an aminoglycoside/ or hird-genera ion cephalosporin rea s no only he sys emic in ec ion, bu has reasonable in raocular pene ra ion due o breakdown o he blood–eye barrier. In ravi real an ibio ics such as vancomycin 1 mg in 0.1 mL and amikacin 0.4 mg in 0.1 mL or ce azidime 2.25 mg in 0.1 mL wi h or wi hou PPV may be needed. In cases o proven or suspec ed ungal in ec ion, a combina ion o oral or IV drug (ampho ericin B 20 mg/ day, f uconazole 400 mg/ day, i raconazole 200 mg/ day, voriconazole 400 mg b.i.d. × 1 day, hen 200 mg b.i.d., or caspo ungin), and in ravi real agen s such as ampho ericin B 5 µg/ 0.1 mL or voriconazole 100 µg/ 0.1 mL wi h or wi hou

PPV are needed. Cases wi h more signi can vi ri is end o do bet er wi h vi rec omy plus an i ungal agen s. opical s eroids and cycloplegics are used o con rol an erior segmen inf amma ion. Prognosis Poor prognosis is associa ed wi h virulen organisms, compromised sys emic heal h and delay in diagnosis. rea men o he primary ocus o in ec ion improves he prognosis. EFE ENCES Arevalo JF, Jap A, Chee SP, e al. Endogenous endoph halmi is in he developing world. Int Ophthalmol Clin. 2010 Spring;50( 2):173–187. Jackson L, Eykyn SJ, Graham EM, e al. Endogenous bac erial endoph halmi is: a 17-year prospec ive series and review o 267 repor ed cases. Surv Ophthalmol. 2003;48:403–423. Smi h S , Kroll AJ, Lou PL, e al. Endogenous bac erial and ungal endoph halmi is. Int Ophthalmol Clin. 2007 Spring;47(2):173–183. Wong JS, Chan K, Lee HM, e al. Endogenous bac erial endoph halmi is. An Eas Asian experience and a reappraisal o a severe ocular a ic ion. Ophthalmology. 2000; 107:1483–1491.

FIGURE10- 6. T is person developed an erior di use EE wi h severe conjunc ival injec ion, corneal edema, hypopyon, and pos erior synechiae a er IV uid herapy or dehydra ion.

Endogenous Endophthalmitis

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A

FIGURE 10-7. A. An HIV-posi ive pa ien developed EE wi h a hypopyon a er experiencing sepsis secondary o an IV ca he er. B. T e pa ien developed sepsis due o an IV ca he er rom which Pseudomonas was cul ured.

B

A

B

FIGURE10- 8. A. EE occurred as a resul o an in ec ed oo wound o a diabe ic pa ien . B.T ere was a pos erior ocal subre inal abscess wi h hemorrhagic borders in his pa ien .

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B

FIGURE10- 9. A.T is pa ien had mul i ocal pos erior endogenous endoph halmi is. T e choroid shows purulen ma erial wi h some break hrough in o he vi reous cavi y be ore an ibio ic herapy. B.T e same pa ien a er an ibio ic rea men shows involu ion o he lesions wi h clearer media.

A

B

FIGURE10- 10. A. Pos erior di use EE is charac erized by in ense vi reous in amma ion. Prior o s ar ing an ibio ic herapy, he pa ien had di use vi ri is, subre inal inf l ra es, and hemorrhage. B.A er rea men , he vi reous in amma ion is less in ense and he vi ri is appears o be “f rming up.”

FIGURE10- 11. T is person has candidemia, and developed vi reous “snowballs,” which are o en seen wi h endogenous candida endoph halmi is.

Endogenous Fungal Endophthalmitis

ENDO GENOUS FUNGAL ENDOPHTH ALMITIS Bahram Bodaghi and Phuc LeHoang

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ndogenous ungal endoph halmi is usually occurs secondary o hema ogenous dissemina ion o ungi in pa ien s who have cer ain well-iden i ed risk ac ors. Early diagnosis is impor an in order o avoid subsequen complica ions and a poor visual ou come.

Epidemiology Endogenous ungal endoph halmi is is uncommon. Pa ien s o all ages, rom neona es o he elderly, can develop i . I is impor an o iden i y risk ac ors such as IV hyperalimen a ion, ca he er in ec ion, immunosuppression, longerm sys emic use o an ibio ics, diabe es melli us, cancer surgery, abdominal surgery, abor ion, or IV drug abuse. T ese can acilia e early diagnosis, as well as help guide rea men . Approxima ely one- hird o pa ien s wi h candidemia will develop choriore ini is or endogenous endoph halmi is. Ocular involvemen should be considered a marker or a dissemina ed in ec ion. Etiology and Pathogenesis Aspergillus species, Candida species, Cryptococcus neo ormans, and Histoplasma capsulatum are he major causes o endogenous ungal endoph halmi is. Endogenous ungal endoph halmi is develops slowly when compared o endogenous bac erial endoph halmi is. Lesions s ar a he level o he choroid, and subsequen ly break hrough in o he vi reous, especially wi h Candida species.

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Candida species involve he vi reous as he prominen ocus o in ec ion, whereas Aspergillus species are predominan ly a he level o re ina, subre inal space, subre inal pigmen epi helial space, and choroid. Symptoms Pa ien s may be asymp oma ic, and pa ien s who are seriously ill may no be able o voice any visual changes. In o her cases, symp oms are due o choriore inal and vi reous involvemen and may include: Floa ers, blurred vision Sco oma a and pho ophobia A red and pain ul eye Visual loss due o macular involvemen or dense vi ri is Signs ( Figs. 10-12 to 10-17) Lesions are ini ially loca ed a he level o he choroid and/ or re ina, and spread in o he vi reous cavi y in he la e phase. T e an erior segmen ini ially may be normal. Over ime, pa ien s may develop an erior uvei is wi h ciliary injec ion, nongranulomaous kera ic precipi a es, pos erior synechiae, f are cells, and in severe cases, a hypopyon. Early on, unduscopy shows small creamywhi e lesions ha may become di cul o see a he la e s age due o vi reous involvemen . e inal hemorrhages may surround small necro ic lesions and appear similar o o h spo s. Vi reous abscess o en have a “s ring-o pearls” appearance. Periorbi al and eyelid edema can rarely occur. Orbi al abscess, orbi al apex syndrome, and an op ic neuropa hy may be associa ed wi h Aspergillus e hmoid sinusi is.

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Dif erential Diagnosis T e early s age o he disease may mimic o her ypes o in raocular inf amma ion, including: T e whi e do syndromes, speci cally mul i ocal choroidi is Primary in raocular lymphoma or o her masquerade syndromes Acu e re inal necrosis syndrome In ec ious choriore ini is such as oxoplasmosis Syphilis Diagnostic Evaluation Cul ures should be ob ained rom sources such as blood, urine, and IV lines in order o iden i y he organism and a possible underlying source. Ches imaging can also be considered as indica ed by his ory. Iden i ca ion o he ungus rom an aqueous, vi reous, or re inal specimen can be ob ained in selec circums ances, bu is no rou inely needed. PC applied o ocular f uids is a promising diagnos ic ool bu is no ye rou inely used. Per ormed a an early s age, f uorescein angiography can help o con rm he presence o choriore ini is. B-scan ocular ul rasonography should be used o assess he re ina and vi reous in cases wi h severe media opaci y. Treatment When evalua ing pa ien s wi h ocular inf amma ion, looking or a rea able in ecion remains paramoun . Sys emic evaluaion looking or an underlying source is cri ical. Early diagnosis o ungal endoph halmi is enables earlier rea men and a bet er nal visual ou come.

Spon aneous resolu ion remains ex remely rare. IV and in ravi real adminis ra ion o an iungal agen s is he main herapeu ic s ra egy. Pa ien s wi h Candida endoph halmi is Oral f uconazole and voriconazole are rs -line agen s. In severe or imminen ly vision hrea ening cases, in ravi real ampho ericin B, voriconazole, or caspo ungin can be adminis ered. Pa ien s wi h Aspergillus endoph halmi is Oral voriconazole is e ec ive. In ravi real ampho ericin B, voriconazole, or caspo ungin can be used or more vision hrea ening cases. PPV wi h in ravi real an i ungal injec ion should be per ormed in cases wi h signi can vi reous involvemen , or bo h diagnos ic and herapeu ic e ec . T e use o in ravi real cor icos eroids remains con roversial. Prognosis T ere is a wide range o visual ou comes in cases o ungal endoph halmi is, depending on how soon he rea men is s ar ed, he s age o he disease, and he organism involved. T e in erac ion be ween he hos immune response and he burden o he in ec ious agen play a major role in a ec ing visual ou come. Despi e aggressive rea men , he visual prognosis is o en poor because o requen macular involvemen . Sys emic ungal in ec ion is a li ehrea ening condi ion.

Endogenous Fungal Endophthalmitis

EFE ENCES Cassoux N, Bodaghi B, Lehoang P, e al. Presumed ocular candidiasis in drug misusers a er in ravenous use o oral high dose buprenorphine (Subu ex) . Br J Ophthalmol. 2002;86:940–941. Khan FA, Slain D, Khakoo R . Candida endoph halmi is: ocus on curren and u ure an i ungal rea men op ions. Pharmacotherapy. 2007;27:1711–1721.

FIGURE10- 12. T ere are many small vi reous abscesses in a case o ungal endoph halmi is due o Candida albicans. (Cour esy o MidA lan ic Re ina, he Re ina Service o Wills Eye Ins i u e.)

A

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ao NA, Hidaya AA. Endogenous myco ic endoph halmi is: varia ions in clinical and his opa hologic changes in candidiasis compared wi h aspergillosis. Am J Ophthalmol. 2001;132:244–251. Shen X, Xu G. Vi rec omy or endogenous ungal endoph halmi is. Ocul Immunol Inf amm. 2009;17: 148–152.

FIGURE10- 13. T is pa ien had Candida albicans– associa ed choriore ini is wi h a prominen whi e abscess emporal o he macula.

B

FIGURE10- 14. Aspergillus fumigatus–associa ed EE be ore (A) and a er (B) vi rec omy shows a large area o choriore ini is along he supero emporal arcade.

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10 ENDO PHTHALMITIS

B

FIGURE10- 15. FA (early phase [A] and mid-phase [B] ) showing he ypical pat ern o choriore ini is ( blocks early and s ains la e) in a case o Candida albicans–associa ede ndoph halmi is.

A

B

FIGURE10- 16. A. T e color pho ograph shows mul iple ocal, round, whi e vi reous abscesses sugges ive o candida. B. FA demons ra es nonspecif c s aining o he op ic disk and vessels, as well as some blocking due o he vi reous in amma ion.

FIGURE10- 17. T ere is modera e vi ri is as well as a yellow-whi e subre inal abscess wi h associa ed subre inal hemorrhage in his pa ien wi h Aspergillus. (Cour esy o Arunan Sivalingam, MD, and Eliza Hoskins, MD.)

C H AP T ER

AIDS-Related Eye Disease Annal D. Meleth and Allison Dublin

A

s of 2007, over one million people in the United States, and over 33 million people worldwide are infected with the HIV and men who have sex with men and racial e HIV virus itself can cause retinopathy. Many of the more serious ocular manifestations of AIDS are due to opportunistic infections.

HIV RETINOPATHY

H

retinopathy is the most common ocular manifestation of HIV. Epidemiology and Etiology It is uncommon if the CD4 is above 200, but it occurs in approximately 50% of patients with a CD4 less than 50. On histopathology, loss of pericytes, narrowing of the capillary lumen, and thickening of the basement membrane are the earliest manifestations of the disease. Symptoms Patients are typically asymptomatic, but they may describe alterations of color vision,

sensitivity. Signs on wool spots ( Fig. 11-1) Intraretinal hemorrhages Capillary microaneurysms, nonperfusion, and telangiectasis erential Diagnosis Diabetic retinopathy Hypertensive retinopathy Radiation retinopathy Venous occlusive disease Collagen vascular disease ( lupus) Prognosis e prognosis is almost uniformly good. Treatment usually regresses with highly active antiretroviral therapy ( H AART) . R EF E R EN C ES rst quarter century. Am JOphthalmol. 2008;145( 3) :397–408. Jabs DA. Ocular manifestations of HIV infection. Trans Am Ophthalmol Soc. 1995;93:623–683.

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FIGURE 11-1. A ew scat ered cot on wool spo s in a pa ien wi h HIV.

Cytomegalovirus Retinitis

CYTOMEGALOVIRUS RETINITIS

C

y omegalovirus (CMV) re ini is is he mos common ocular oppor unis ic in ec ion in AIDS, and is charac erized by yellow-whi e re inal necrosis and perivascular in amma ion.

Epidemiology and Etiology T e primary risk ac or is he degree o immune compromise. Pa ien s wi h a CD4 coun below 50 cells/ µL have more han a our old higher risk o developing CMV re ini is compared o pa ien s wi h CD4 grea er han 100 cells/ µL. CMV re ini is is he mos common ocular oppor unis ic in ec ion in pa ien s wi h AIDS, and is i sel an AIDS-de ning illness. In he pre-HAAR era, i occurred in up o 40% o pa ien s wi h AIDS and mos pa ien s died wi hin 1 o 2 years a er diagnosis. For una ely, here has been a drama ic decrease in incidence in HAAR era. Rhegma ogenous re inal de achmen s (RRDs) are also an impor an cause o vision loss in pa ien s wi h CMV re ini is, developing in approxima ely 20% o pa ien s. Symptoms Painless vision loss Floa ers Pho opsias Signs ( Figs. 11-2 to 11-7) Perivascular in amma ion wi h irregular pa ches o uf y whi e re ini is and necrosis wi h associa ed scat ered hemorrhages. I usually s ar s as a single lesion and hen spreads ou ward as granular do s rom ha ocus. Pa ien s o en have a granular lesion wi h mul iple do s around he edge (represen ing

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advancing lesions) wi h cen ral clearing ha leads o a s ippled re inal pigmen epi helium. Addi ional ea ures include: Fros ed branch angii is Chronic vi ri is Cys oid macular edema Progression ypically occurs via expansion o previous re inal lesions, which can advance a a ra e o up o 250 microns per week. I ypically advances as er an eriorly han pos eriorly. Vision loss occurs via macular or op ic nerve involvemen rom primary re ini is or secondarily wi h macular edema associa ed wi h paramacular involvemen or due o re inal de achmen . Dif erential Diagnosis Acu e re inal necrosis (ARN) s ar s as mul iple lesions in he mid-peripheral ha rapidly progress and is associa ed wi h subs an ial vi ri is. Progressive ou er re inal necrosis (PORN) Syphilis oxoplasmosis Diagnostic Evaluation Fundus pho os can be used o ollow disease progression. Fluorescein angiography (FA) demons ra es mot led hyper uorescence o he expanding borders. T ere can be la e s aining o he re inal vessels near he area o in ec ion. Au o uorescence: Advancing border o en displays hyperau o uorescence. AC ap or vi reous ap wi h polymerase chain reac ion (PCR) may be considered. Treatment HAAR wi h immune recovery arres s CMV re ini is progression.

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Wi hou HAAR , 50% o pa ien s experience CMV reac iva ion a main enance dose o an i-CMV herapy. T e clinician mus consider concurren CMV disease in o her organ sys ems. Sys emic herapy Gancyclovir (5 mg/ kg IV b.i.d.) or an induc ion period o 2 weeks ollowed by long- erm main enance (5 mg/ kg IV q.d.) Foscarne (90 mg/ kg IV b.i.d. or 60 mg/ kg IV .i.d.) or an induc ion period o 2 weeks ollowed by main enance (90 mg/ kg q.d.) Cido ovir (5 mg/ kg every week) or an induc ion period o 2 weeks ollowed by long- erm main enance (3 o 5 mg/ kg IV q2wk) Valgancyclovir (900 mg PO b.i.d.) is an orally adminis ered prodrug o gancyclovir, which has an induc ion period o 2 weeks ollowed by main enance (900 mg PO q.d.) In ravi real herapy can be use ul as sysemic herapy can cause bone marrow suppression (gancyclovir and valgancyclovir) and renal oxici y ( oscarne and cido ovir). However, in ravi real injec ions need o be per ormed requen ly. I a pa ien needs longerm herapy and sys emic rea men canno be con inued, a gancyclovir implan (see below) should be considered. Gancyclovir (2 mg/ 0.05 mL) Foscarne (1.2 mg/ 0.05 mL) Fomivirsen (330 g in ravi real every week); wi hdrawn rom he marke Combina ion Surgical herapy Gancyclovir implan (Vi raser , Bausch and Lomb) is surgically placed

in he pars plana and delivers gancyclovir or 6 o 8 mon hs a + 4 concen ra ion o IV injec ion. May be a good op ion in pa ien s wi h resis ance and unila eral disease. Re inal de achmen s in hese cases are of en complex wi h mul iple re inal holes and of en require vi rec omy wi h silicone oil amponade. Resis ance Pheno ypic and geno ypic resis ance can occur o gancyclovir, oscarne , and cido ovir. Gancyclovir resis ance of en secondary o mu a ions in he UL97 gene. Ra e o resis ance has declined in HAAR era ( rom 28% in pre-HAAR o approxima ely 9% in HAAR era). Repor s exis o gancyclovir-resis an CMV in serum wi h suscep ible CMV in aqueous and vi reous samples. Prognosis Dependen on ex en o re ina involved, bu has drama ically improved in HAAR era. REFERENCES Jabs DA, van Nat a ML, T orne JE, e al. Course o cy omegalovirus re ini is in he era o highly ac ive an ire roviral herapy: Second eye involvemen and re inal de achmen . Ophthalmology. 2004;111:2232–2239. Musch DC, Mar in DF, Gordon JF, e al. rea men o cy omegalovirus re ini is wi h a sus ained-release ganciclovir implan . T e Ganciclovir Implan S udy Group. N Engl J Med. 1997;337( 2):83–90. S udies o Ocular Complica ions o AIDS Research Group, in collabora ion wi h he AIDS Clinical rials Group. Mor ali y in pa ien s wi h he acquired immunode ciency syndrome rea ed wi h ei her oscarne or ganciclovir or cy omegalovirus re ini is. N Engl J Med. 1992;326( 4):213–220.

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FIGURE 11-2. Ac ive CMV re ini is in a pa ien who underwen bone marrow ransplan a ion.

FIGURE 11-3. CMV re ini is in his case is limi ed o perimacular area wi h necro izing re ini is along he in erior arcade, in rare inal hemorrhage, and granulari y surrounding ovea.

A

B

FIGURE 11-4.T is pa ien wi h bila eral CMV re ini is conf rmed wi h PCR has ex ensive whi e areas o re ini is in he mid-periphery wi h scat ered hemorrhages in bo h eyes.

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FIGURE 11-5. No e he area o CMV re ini is superior o ovea (upper righ ) and vascular s aining and leakage on FA (upper le ) . Fundus au o uorescence shows hyperau o uorescence in he corresponding area ( lower le ) . Lower righ rame shows resolu ion o re ini is ollowing ganciclovir implan .

FIGURE 11-6. CMV re ini is wi h “brush-f re” appearance along he in eronasal quadran in a pa ien wi h HIV.

Cytomegalovirus Retinitis

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A

B

FIGURE 11-7. A.T is pa ien had CMV re ini is wi h he classic “pizza” undus appearance o re inal whi ening wi h scat ering re inal hemorrhages. B. Wi hin 1 mon h o s ar ing HAAR herapy and oral valgancyclovir, here was marked improvemen . (Cour esy o Paul Baker, MD.)

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IMMUNE RECOVERY UVEITIS

I

mmune recovery uvei is (IRU) represen s a response o an igens and i occurs in pa ien s who have immune-recons i u ion a er having had re ini is. Al hough i mos commonly occurs in pa ien s wi h CMV re ini is, i can also occur ollowing in ec ion wi h uberculosis or oxoplasmosis.

Epidemiology and Etiology I ypically occurs in pa ien s who have had prior CMV re ini is. T e risk increases wi h he ex en o re inal involvemen . Incidence es ima es vary widely, bu ypically occurs in approxima ely 10% o pa ien s wi h immune recovery. Symptoms Vision loss Floa ers Pho opsias Signs Pos erior synechiae Ca arac Vi ri is Op ic disk edema Cys oid macular edema Epire inal membrane Proli era ive vi reore inopa hy Vi reous hemorrhage

Dif erential Diagnosis CMV re ini is ARN oxoplasmosis Diagnostic Evaluation IL-12 is ypically eleva ed in vi reous (vs. CMV re ini is). T e CMV PCR rom an an erior chamber or vi reous ap may be nega ive. Treatment Bo h periocular and in ravi real riamcinolone, as well as low-dose oral s eroids, can be adminis ered depending on immune s aus. T e e cacy o hese rea men s is variable. Generally, he CMV does no reac iva e wi h s eroid rea men , bu pa ien s should be ollowed or his. Prognosis T e ou come is variable and depends upon he degree o in amma ion and presence o pos erior segmen complica ions. Pa ien s may have some long- erm vision loss as a resul o IRU. REFERENCES Kempen JH, Min YI, Freeman WR, e al. Risk o immune recovery uvei is in pa ien s wi h AIDS and cy omegalovirus re ini is. Ophthalmology. 2006;113:684–694. Morrison VL, Kozak I, LaBree LD, e al. In ravi real riamcinolone ace onide or he rea men o immune recovery uvei is macular edema. Ophthalmology. 2007;114:334–339.

Acute Retinal Necrosis

ACUTE RETINAL NECROSIS

A

cu e re inal necrosis (ARN) is rare and consis s o a ull- hickness re inal necrosis associa ed wi h vi ri is and occlusive vasculopa hy. I is caused by he herpes simplex virus (HSV) and ypically occurs in immunocompe en pa ien s. Epidemiology and Etiology T e herpes viruses, including varicella zoser virus (VZV), HSV-1, HSV-2, EBV, and CMV all have caused ARN. T e mean age o onse is age 50, bu i can occur a any age. ARN is ypically unila eral a onse . ARN is bila eral (BARN) in 10% o 36%, and he second eye becomes involved usually wi hin 6 weeks o he rs , al hough several years o separa ion are possible. Symptoms Mild o modera e eye pain Vision loss Floa ers Pho opsias Signs ( Fig. 11-8) American Uvei is Socie y diagnos ic cri eria One or more oci o re inal necrosis wi h discre e borders in he peripheral re ina Rapid circum eren ial progression o necrosis in he absence o rea men Evidence o occlusive vasculi is wi h ar eriolar involvemen Prominen in amma ion in he an erior and pos erior chambers O her mani es a ions Op ic neuri is Scleri is, episcleri is

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Neovasculariza ion and vi reous hemorrhage rela ed o occlusive disease Dif erential Diagnosis PORN CMV re ini is oxoplasmosis Syphilis Sarcoidosis uberculosis Diagnostic Evaluation PCR o aqueous or vi reous wi h demons ra ion o virus Color undus pho ography can be used o documen disease progression. FA demons ra es hypo uorescence in he areas o occlusive vasculopa hy, la e hyper uorescence in he areas o ac ivi y, and hyperuorescence o neovasculariza ion (i presen ). Treatment IV acyclovir, along wi h sys emic cor icos eroids wi h or wi hou in ravi real an iviral herapy, has been he s andard herapy or a number o years. In addi ion o rea ing he af ec ed eye, i reduces risk o bila eral involvemen and ex ension o disease. rea men does no reduce he risk o RRD. Recen repor s sugges ha rea men wi h oral valacyclovir, he prodrug o acyclovir, achieves similar bioavailabili y and rea men success and avoids inpa ien admission. RRDs usually need o be rea ed wi h vi rec omy and long-ac ing gas/ silicone oil amponade. Some inves iga ors recommend prophylacic laser re inopexy in order o reduce he ra e

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o re inal de achmen ; however, he e cacy o his approach is variable. Prognosis T e nal visual ou come is usually poor. Fi y percen o pa ien s are 20/ 200 or worse 3 mon hs a er disease onse , and his increases o 75% by 3 years. T e prognosis is rela ed o ini ial visual acui y. RRD occurs in 50% o pa ien s and por ends a poor visual ou come.

T e newer an iviral agen s do no appear o of er a bet er nal visual ou come. REFERENCES Aizman A, Johnson MW, Elner SG. rea men o acu e re inal necrosis syndrome wi h oral an iviral medicaions. Ophthalmology. 2007;114( 2):307–312. Epub 2006 Nov 21. ibbet s MD, Shah CP, Young LH, e al. rea men o acu e re inal necrosis. Ophthalmology. 2010;117( 4):818–824. Epub 2010 Jan 15.

A

B

FIGURE 11-8. A. ARN wi h large areas o whi e re inal necrosis wi h overlying vi ri is. B. FA demons ra es signif can occlusive vasculopa hy and neovasculariza ion.

Progressive Outer Retinal Necrosis

PRO GRESSIVE OUTER RETINAL NECROSIS

263

P

Dif erential Diagnosis ARN involves he en ire re ina, while PORN is ou er re inal. ARN has modera e o severe vi ri is and vasculi is, and uncommonly involves he pos erior pole. CMV re ini is Syphilis uberculosis

Epidemiology and Etiology I occurs in severely immunocompromised pa ien s, mos commonly in hose wi h AIDS. In he pre-HAAR era i occurred in up o 2% o pa ien s, bu is less common now. ypically, i is caused by varicella zos er and herpes simplex viruses. I is bila eral in over wo- hirds o cases. wo- hirds o pa ien s have a his ory o cu aneous herpes zos er.

Diagnostic Evaluation Pa ien s should be es ed or HIV i an underlying diagnosis is unknown. PCR o de ec he virus in he vi reous FA: Demons ra es ou er re inal de ec ha blocks early and s ains la e. Unlike ARN, here is a lack o vasculi is. Fundus au o uorescence: Early hypo uorescence ollowed by a s ippled au o uorescence pat ern.

rogressive ou er re inal necrosis (PORN) is charac erized by ou er re inal necrosis. Like ARN, i is associa ed wi h herpes virus, bu unlike ARN, i occurs in immunocompromised pa ien s. T e visual ou come is poor.

Symptoms Painless vision loss “Cur ain” visual eld de ec Signs ( Fig. 11-9) Presen ing vision ranges rom near-normal o NLP. Pa chy, mul i ocal ou er re inal whi ening, ypically ou side o he pos erior pole wi h macular involvemen in one- hird a he ime o presen a ion. Ex remely rapid progression (hours o days) o ull- hickness re inal necrosis Minimal o no vi ri is No vasculi is Af eren pupillary de ec

Treatment Combina ion in ravi real and in ravenous an iviral herapy wi h long- erm main enance herapy in pa ien s who are persis en ly immunocompromised should be considered, bu he visual ou come remains poor. T e underlying cause o immunosuppression should be rea ed. Aggressive managemen o RRDs wi h vi rec omy and silicone oil amponade. Prognosis Despi e aggressive rea men , he visual ou come is poor wi h 67% o pa ien s progressing o NLP in one series. wo- hirds o pa ien s may also develop a re inal de achmen .

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REFERENCES Engs rom RE Jr, Holland GN, Margolis P, e al. T e progressive ou er re inal necrosis syndrome. A varian o necro izing herpe ic re inopa hy in pa ien s wi h AIDS. Ophthalmology. 1994;101( 9):1488–1502. Kim SJ, Equi R, Belair ML, e al. Long- erm preserva ion o vision in progressive ou er re inal necrosis rea ed wi h combina ion an iviral drugs and highly ac ive an ire roviral herapy. Ocul Immunol Inf amm. 2007; 15( 6):425–427.

Spaide RF, Mar in DF, eich SA, e al. Success ul rea men o progressive ou er re inal necrosis syndrome. Retina. 1996;16(6):479–487. Yin PD, Kurup SK, Fischer SH , e al. Progressive ou er re inal necrosis in he era o highly ac ive an ire roviral herapy: success ul managemen wi h in ravi real injec ions and moni oring wi h quan i a ive PCR. J Clin Virol. 2007;38( 3):254–259. Epub 2007 Feb 5.

FIGURE 11-9. PORN wi h large areas o ou er re inal whi ening involving he macula. No e he lack o vi ri is and vasculi is.

Fungal Retinitis

FUNGAL RETINITIS

F

ungal re ini is is a rare cause o visual compromise in pa ien s wi h AIDS, and i is ypically associa ed wi h sys emic ungemia ( Fig. 11-10). Cryptococcus neoformans I is a yeas ha causes in ec ion hrough inhala ion. His orically, his was a common cause o meningi is in AIDS pa ien s. I af ec s he eye ei her hrough hema ogenous spread or hrough ex ension rom he op ic nerve. T e mos common ocular mani es a ions are op ic neuropa hy and choroidi is, bu i can also cause op ic a rophy, endoph halmiis, vi reore inal abscesses, and ex raocular muscle palsies (usually he six h nerve).

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rea men wi h in ravenous ampho ericin B plus in ravi real ampho ericin B wi h or wi hou vi rec omy should be considered. Paracoccidiomycosis T is ungal in ec ion is more common in Cen ral and Sou h America. I is he mos common cause o sys emic mycosis in Brazil. I is rarely seen in AIDS pa ien s. ypical mani es a ions include parinaud oculoglandular syndrome. I can also cause choroidal granuloma, vi ri is, and endoph halmi is. rea men wi h in ravenous ampho ericin B plus in ravi real ampho ericin B wi h or wi hou vi rec omy should be considered. Candida Candida endoph halmi is is uncommon among AIDS pa ien s.

A

FIGURE 11-10. T is pa ien wi h AIDS presen ed wi h ever and a headache. A. Dila ed undus exam revealed mul iple, deep, yellow plaques scat ered hroughou he pos erior pole is ypical o Cryptococcus neoformans. (continued)

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B

C

FIGURE 11-10. (Continued) B. FA revealed nonspecif c la e s aining o he choroidal lesions. C. One mon h a er s ar ing ampho ericin, here was resolu ion o he yellow lesions. Di use mot ling o he re inal pigmen epi helium is presen , mos no ably in he macula. (Cour esy o Paul Baker, MD.)

PneumocystisCarinii (Jirovecii) Choroiditis

PNEUMOCYSTIS CARINII ( JIROVECII) CHOROIDITIS

P

neumocystis carinii (PC) choroidi is is a rare cause o choroidopa hy in pa ien s wi h AIDS and o en serves as a marker o dissemina ed sys emic disease. Epidemiology and Etiology I is rare. PC causes pneumoni is. For a number o years, he prophylaxis or PC pneumoni is was aerosolized pen amidine. However, his did no provide prophylaxis o he eyes. T ere has been a drama ic decrease in ocular PC since he use o sys emic prophylaxis. T e majori y o pa ien s ( hree- our hs) have bila eral disease.

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Dif erential Diagnosis uberculosis In raocular lymphoma Sarcoidosis Me as a ic disease Syphilis Cryptococcus Diagnostic Evaluation FA demons ra es early hypo uorescence o he lesions wi h la e s aining. Treatment Double-s reng h rime hoprimsul ame hoxazole IV or oral or 21 days, ollowed by long- erm prophylaxis is very ef ec ive.

Symptoms As he lesions cause a choroidi is and usually have no associa ed in amma ion, pa ien s are ypically asymp oma ic.

Prognosis PC ypically resolves wi h rea men .

Signs Af ec ed pa ien usually exhibi deep, muli ocal cream-orange plaques ha range in size rom 300 o 3,000 microns and are loca ed in he midperipheral and pos erior choroid.

Gup a A, Hus ler A, Herieka E, e al. Pneumocys is choroidi is. Eye (Lond). 2010;24( 1):178. Morinelli EN, Dugel PU, Rif enburg R, e al. In ec ious mul i ocal choroidi is in pa ien s wi h acquired immune de ciency syndrome. Ophthalmology. 1993;100: 1014–1021.

REFERENCES

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KAPOSI’S SARCOMA

K

aposi’s sarcoma (KS) is a common umor rela ed o human herpes virus-8 in ec ion. I ypically occurs in pa ien s wi h AIDS and af ec s he eyelids or he adnexal skin. Epidemiology and Etiology

Oculocu aneous KS may precede, ollow, or occur concomi an ly wi h sys emic disease. Associa ed wi h HHV-8 I is mos commonly seen in pa ien s wi h AIDS, bu can also occur in persons o Medi erranean or A rican descen , as well as in organ ransplan pa ien s. Symptoms Ocular irri a ion secondary o ear lm abnormali ies Signs ( Figs. 11-11 and 11-12) Pa ien s usually have a violaceous umor ha af ec s he conjunc iva, lid margin, or eyelid skin. Conjunc ival lesions usually appear as an eleva ed subconjunc ival reddish mass, and are more common in he in erior ornix and on he arsal conjunc iva. Eyelid lesions usually presen as a rm subcu aneous purplish nodule. One s aging sys em de nes KS as: S age 1: A pa ch ha is less han 3 mm in heigh and presen or less han 4 mon hs S age 2: A larger plaque ha is a , which is also less han 3 mm in heigh and presen or less han 4 mon hs S age 3: A nodule grea er han 3 mm in heigh and presen or more han 4 mon hs Dif erential Diagnosis Subconjunc ival hemorrhage

Conjunc ival melanosis Pyogenic granuloma Squamous cell carcinoma Conjunc ival lymphoma Lymphangioma Diagnostic Evaluation His opa hology demons ra es spindleshaped cells wi h in amma ory cells and ex ravasa ed red blood cells. S age 1: Fla dila ed vascular channels S age 2: Plump usi orm endo helial cells lining dila ed vascular channels, wi h a ew oci o imma ure spindle cells S age 3: Densely packed spindle cells wi h hyperchroma ic nuclei and mi o ic gures Sys emic evalua ion should be per ormed o iden i y any ex raocular umors. Treatment T e mos ef ec ive rea men is immune recons i u ion. In ralesional injec ion o α-in er eron, vinblas ine, and riamcinolone have all been used. Ex ernal beam radia ion can be ef ec ive. Prognosis ypically, mos pa ien s have an indolen course. umor recurrence is airly common. REFERENCES Chang Y, Cesarman E, Pessin MS, e al. Iden i ca ion o herpesvirus-like DNA sequences in AIDS-associa ed Kaposi’s sarcoma. Science. 1994;266:1865–1869. Dugel PU, Gill PS, Frangieh G , e al. rea men o ocular adnexal Kaposi’s sarcoma in acquired immune de ciency syndrome. Ophthalmology. 1992;99:1127–1132. In erna ional Collabora ion on HIV and Cancer. Highly ac ive an ire roviral herapy and incidence o cancer in human immunode ciency virus-in ec ed adul s. J Natl Cancer Inst. 2000;92:1823–1830.

Kaposi’s Sarcoma

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FIGURE 11-11. Kaposi’s sarcoma appears as an eleva ed red umor on he lid margin in a pa ien wi h AIDS. (Cour esy o Rober Penne, MD.)

FIGURE 11-12. Kaposi’s sarcoma can o en appear as a pseudo-subconjunc ival hemorrhage as in his pa ien wi h AIDS. (Cour esy o Ajay Manchandia, MD.)

C H AP T ER

Drug-Induced Uvei is Nehali V. Saraiya and Debra A. Goldstein

U

vei is is usually due o immune or in ecious causes; however, cer ain sys emic or local drugs may also precipi a e in raocular inf amma ion. In general, he pa hogenesis o drug-induced inf amma ion is no well unders ood. I is hypo hesized ha direc and/ or indirec mechanisms are involved. Direc mechanisms are hough o play a role wi h opically or in racamerally ins illed drugs and usually are observed soon a er medica ion use. Indirec mechanisms resul rom immune complex deposi ion in uveal issues, immune reac ions o an igens libera ed rom an ibio ic-induced dea h o a microorganism, or drug-induced al era ion o melanin’s abili y o scavenge ree radicals. T ese mechanisms may resul in in raocular inf amma ion weeks o mon hs a er ini ial use o he drug. T e mos common medica ions implica ed in drug-induced uvei is are discussed in he ollowing pages.

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RIFABUTIN

R

i abu in is prescribed or he rea men and prophylaxis o Mycobacterium avium complex (MAC) in ec ion in HIV-posi ive pa ien s. Symp oms o acu e uvei is may presen 2 weeks o more han 7 mon hs ollowing ini ia ion o herapy. Uvei is has been repor ed o occur wi h ri abu in alone as well as in combina ion wi h o her an imicrobial agen s such as azi hromycin, ery hromycin, clari hromycin, e hambu ol, and f uconazole. I has been repor ed wi h doses as low as 300 mg per day. I recurs wi h re-challenge and increases in severi y wi h dose escala ion. Symptoms Unila eral or bila eral Pain Redness Pho ophobia Blurred vision

Rifabutin

Signs Conjunc ival injec ion Kera ic precipi a es An erior chamber cell/ f are wi h or wi hou hypopyon Vi reous cell Perivascular re inal in l ra es Treatment Discon inue ri abu in. rea inf amma ion wi h opical s eroids and cycloplegic agen s.

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Prognosis In mos cases, uvei is resolves wi hin 1 o 2 mon hs o discon inua ion o ri abu in and adminis ra ion o opical cor icos eroids, wi h avorable ou comes (comple e resolu ion o symp oms and normaliza ion o vision in many cases). REFERENCES Jacobs DS, Piliero PJ, Kuperwaser MG, e al. Acu e uvei is associa ed wi h ri abu in use in pa ien s wi h human immunode ciency virus in ec ion. Am J Ophthalmol. 1994;118( 6):716–722. Moor hy RS, Valluri S, Jampol LM. Drug induced uvei is. Surv Ophthalmol. 1998;42( 6):557–570.

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12 DRUG-INDUCED UVEIT IS

CIDOFOVIR

C

ido ovir has been used in ravenously and in ravi really or he rea men o cy omegalovirus (CMV) re ini is. An erior uvei is has been repor ed o occur in 26% o 59% o pa ien s receiving IV cido ovir a er a median o 4 o 11 doses. Hypo ony and uvei is have also been repor ed ollowing IV cido ovir in a pa ien wi h nonocular CMV in ec ion (encephali is) and an o herwise normal undus exam, sugges ing a direc e ec o he drug on he ciliary body. An erior uvei is also occurs wi h in ravi real cido ovir or rea men o CMV re ini is. One cases series repor ed an erior uvei is in 26% o pa ien s a er a single in ravi real cido ovir injec ion. Concomi an use o sys emic probenecid decreased he requency o inf amma ion. Because o i s associa ion wi h immune recovery uvei is, cido ovir should no be used i immune recovery is expec ed.

Signs and Symptoms Pain, redness, pho ophobia, earing, and decreased vision

Unila eral or bila eral nongranuloma ous an erior uvei is wi h or wi hou kera ic precipi a es, pos erior synechiae, hypopyon, and an erior vi reous cell T e uvei is may be severe and brinous, and accompanied by hypo ony. Treatment and Prognosis Aggressive opical s eroid and cycloplegic agen s Cessa ion o cido ovir is usually required. Ou come is variable wi h po en ial or permanen s ruc ural complica ions such as poserior synechiae and hypo ony. REFERENCES Amba i JK, Wynne KB, Angerame MC, e al. An erior uvei is associa ed wi h in ravenous cido ovir use in pa ien s wi h cy omegalovirus re ini is. Br JOphthalmol. 1999;83( 10):1153–1158. Kempen JH, Min YI, Freeman WR, e al. S udies o Ocular Complica ions o AIDS Research Group. Risk o immune recovery uvei is in pa ien s wi h AIDS and cy omegalovirus re ini is. Ophthalmol. 2006;113( 4): 684–694. Moor hy RS, Valluri S, Jampol LM. Drug induced uvei is. Surv Ophthalmol. 1998;42( 6):557–570.

Bisphosphonates

BISPHOSPHONATES

B

isphosphona es are used o inhibi bone resorp ion in pa ien s wi h os eoporosis and o manage hypercalcemia associa ed wi h os eoly ic bone cancer, me as a ic disease o bone, and Page ’s disease o bone. Ocular inf amma ion has been repor ed mos commonly wi h pamidrona e disodium, bu is also seen wi h o her agen s in his class including zoledronic acid, alendrona e sodium, risedrona e sodium, and e idrona e disodium. Onse has been repor ed anywhere rom 24 hours o weeks a er ini ia ion o herapy. Symptoms Unila eral or bila eral Redness Pain Pho ophobia Blurred vision

Signs Conjunc ivi is Episcleri is Scleri is ( Fig. 12-1) Iri is: An erior chamber reac ion wi h cell/ f are

273

Treatment Nonspeci c conjunc ivi is seldom requires rea men ; NSAID eye drops may provide symp oma ic relie . In hese cases, he bisphosphona es may be con inued. Episcleri is may be rea ed wi h opical s eroids or NSAIDS. An erior uvei is can vary markedly in severi y, and may be rea ed wi h opical or sys emic s eroids depending on severi y. In mos ins ances, he bisphosphona e mus be discon inued or he uvei is o resolve, and hey mus be discon inued or cases wi h scleri is. Prognosis Good, wi h resolu ion o symp oms in all cases, and resolu ion o scleri is wi h medical managemen and discon inua ion o bisphosphona e herapy REFERENCES Fraun elder FW, Fraun elder F , Jensvold B. Scleri is and o her ocular side e ec s associa ed wi h pamidrona e disodium. Am J Ophthal. 2003;135( 2):219–222. Moor hy RS, Valluri S, Jampol LM. Drug induced uvei is. Surv Ophthalmol. 1998;42( 6):557–570.

FIGURE 12-1. Mild dif use scleritis in a patient taking a bisphosphonate.

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12 DRUG-INDUCED UVEIT IS

SULFONAMIDES

S

ul onamide deriva ives are a mains ay or he rea men o many gram-posi ive and gram-nega ive bac erial in ec ions, including urinary rac in ec ions, o i is media, bronchiis, sinusi is, and pneumonia. Visual dis urbances, kera i is, conjunc ivi is, and periorbi al edema have been repor ed.

Signs and Symptoms Unila eral or bila eral acu e iri is has been described wi hin 24 hours o s ar ing he medica ion as well as wi h rechallenge. O her au oimmune dys unc ion such as ery hema mul i orme minor, di use macular vesicular rash, s oma i is, glossi is, conjunc ival or scleral injec ion, or granuloma ous hepa i is may be presen concurren ly. T e mos serious complica ion is S evens-Johnson syndrome (SJS)/ oxic epidermal necrolysis ( EN). SJS/ EN are acu e hypersensi ivi y reac ions o he skin ha are usually drug

FIGURE 12-2. T is patient with Stevens-Johnson syndrome has dry eye and extensive symblepharon ormation. (Courtesy o Charles Bouchard, MD, Loyola University.)

induced ( Figs. 12-2 to 12-6). Mos pa ien s are young adul s. Pa ien s have a f u-like prodrome o malaise, a igue, and headache ha is ollowed in a ew days wi h di use ery hemaous pa ches ha can coalesce o orm blis ers wi h ull hickness epidermal necrosis. T ere can also be bac erial superin ec ion. Mucosal involvemen , including he conjunc iva, occurs in nearly all pa ien s. Treatment and Prognosis Discon inue agen i uvei is is severe, and rea iri is wi h opical s eroids and cycloplegic agen s as necessary. Pa ien s wi h S evens–Johnson syndrome may need hospi aliza ion and care in an in ensive care or burn uni . Pa ien s may develop hypo ension, renal and respira ory ailure, and corneal scarring. REFERENCE Moor hy RS, Valluri S, Jampol LM. Drug induced uvei is. Surv Ophthalmol. 1998;42( 6):557–570.

FIGURE 12-3. Another patient with StevensJohnson syndrome who has conjunctivitis and sloughing o the conjunctival epithelium. (Courtesy o Charles Bouchard, MD, Loyola University.)

Sulfonamides

FIGURE 12-4. A 3-year-old girl developed StevensJohnson syndrome and suf ered a desquamative eruption. Note the extensive mucositis (conjunctivitis and stomatitis) characteristic o Stevens-Johnson syndrome. Her medication was changed and she was success ully treated with intravenous immunoglobulin (IVIG) . (Courtesy o Vanessa A. London, MD.)

A

275

FIGURE 12-5. T e palms o a 59-year-old man diagnosed with Stevens-Johnson syndrome. Note the sharp demarcation o erythema at the edges o the palms and the classic targetoid lesions with dusky centers along his wrists. His medication was discontinued and he was treated with 60 mg prednisone or 3 days with resolution o his symptoms. (Courtesy o Vanessa A. London, MD.)

B

FIGURE 12-6. A. A 48-year-old man with AIDS presented with a dif use morbilli orm eruption that evolved into blistering with prominent palm and sole involvement. B. He also had conjunctivitis, stomatitis, and involvement o the glans penis. Skin biopsy con rmed a diagnosis o Stevens–Johnson syndrome. With discontinuation o trimethoprim/ sul amethoxazole, his rash and conjunctivitis eventually resolved. (Courtesy o Vanessa A. London, MD.)

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12 DRUG-INDUCED UVEIT IS

METIPRANOLOL

M

e ipranolol is a nonselec ive β1/ β2 blocker used opically in he rea men o glaucoma via suppression o aqueous humor. I is he mos commonly repor ed be a-blocker o cause uvei is. Incidence is rare. Signs and Symptoms Unila eral or bila eral granuloma ous an erior uvei is wi h mut on- a kera ic precipia es, an erior chamber cell and f are, absence o iris nodules, wi h or wi hou increased in raocular pressure.

Treatment and Prognosis rea iri is wi h opical s eroid and cycloplegic agen s. Discon inue me ipranolol and subs i u e ano her hypo ensive agen or pressure conrol. Using ano her be a-blocker is usually sa e. Symp oms usually resolve wi hin 3 o 5 weeks. REFERENCES Akingbehin , Villada JR. Me ipranolol-associa ed granuloma ous an erior uvei is. Br J Ophthalmol. 75( 9): 519–523. Moor hy RS, Valluri S, Jampol LM. Drug induced uvei is. Surv Ophthalmol. 1998;42( 6):557–570.

Brimonidine

BRIMONIDINE

B

rimonidine ar ra e is a highly selec ive α2 adrenorecep or agonis ha lowers in raocular pressure by reducing aqueous produc ion and increasing uveoscleral aqueous ou f ow. I is generally well olera ed. T e mos common ocular adverse even s are allergic reac ions severe enough o necessi a e disconinua ion o herapy in 7% o 15% o pa ien s and conjunc ival ollicles in 8% o pa ien s. An erior uvei is secondary o brimonidine is rare, and develops be ween 11 and 15 mon hs a er s ar ing herapy. In many cases, a previous his ory o allergic derma oconjunc ivi is or ollicular conjunc ivi is is presen . Signs and Symptoms Acu e-onse redness, pho ophobia, blurred vision due o granuloma ous iri is wi h

277

mut on- a kera ic precipi a es, wi h or wi hou iris nodules or pos erior synechiae, and mild an erior chamber cell and f are ( Fig. 12-7). Treatment and Prognosis Cessa ion o brimonidine opical s eroids and cycloplegics o rea in lamma ion generally leads o resolu ion o symp oms; permanen s ruc ural changes such as pos erior synechiae may remain. REFERENCES Ka z LJ. Brimonidine ar ra e 0.2% wice-daily vs imolol 0.5% wice daily: 1-year resul s in glaucoma pa ien s. Brimonidine S udy Group. Am J Ophthalmol. 1999; 127( 1):20–26. Moor hy RS, Valluri S, Jampol LM. Drug induced uvei is. Surv Ophthalmol. 1998;42( 6):557–570.

FIGURE 12-7. Small and medium granulomatous keratic precipitates in a person using topical brimonidine.

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12 DRUG-INDUCED UVEIT IS

PROSTAGLANDIN ANALO GUES

been described wi h use o bima opros and ravopros .

P

Signs and Symptoms Eye pain and redness wi h a mild an erior chamber reac ion

ros aglandin analogues are used in rea men o open-angle glaucoma and ocular hyper ension, and ac via increasing uveoscleral ou f ow. T ey are he newes class o hypo ensive agen s, and are o en rs -line agen s in rea ing glaucoma and ocular hyperension. In one re rospec ive case series, an erior uvei is (iri is) was seen in 4.9% o pa ien s rea ed wi h la anopros wi hin 6 mon hs o s ar ing he medica ion. T is series also repor ed a 2.1% incidence o cys oid macular edema due o la anopros . A previous his ory o cys oid macular edema, iri is, in raopera ive vi reous loss, or an erior chamber in raocular lens are risk ac ors. An erior uvei is has also

Treatment and Prognosis Good prognosis wi h comple e resolu ion o iri is wi hin 1 o 2 weeks o discon inuing medica ion REFERENCES Moor hy RS, Valluri S, Jampol LM. Drug induced uvei is. Surv Ophthalmol. 42( 6):557–570. Warwar RE, Bullock JD, Ballal D. Cys oid macular edema and an erior uvei is associa ed wi h la anopros use. Experience and incidence in a re rospec ive review o 94 pa ien s. Ophthalmology. 1998;105( 2):263–268.

C H AP T ER

Masquerade Syndromes PRIMARY INT LYMPHOMA

O CULAR

H. Nida Sen and Bahram Bodaghi

P

rimary intraocular lymphoma (PIOL), also known as primary retinal lymphoma (PRL) or reticulum cell sarcoma, is a subset of primary CNS lymphoma (PCNSL) that involves the retina, vitreous, and optic nerve head with or without simultaneous CNS involvement. Most PIOLs are extranodal,

Epidemiology and Etiology More commonly seen in immunocompromised individuals Incidence in the United States has tripled over the last 20 years both in immunocompromised and immunocompetent individuals. 100 new cases of PIOL each year e incidence of PCNSL is 4 to 5 per 1000 person-years among patients with is contribution to the work was done as part of the

AIDS and 0.3 per 100,000 persons-years in immunocompetent patients. 25%of PCNSL patients have eye involvement at the time of presentation, whereas up to 85%of PIOL develop PCNSL. It t ypically affect s an older populat ion, usually in t he fift h to sixt h decades of life. Slight male preponderance Bilateral in approximately 80%, but can be very asymmetric at presentation Immunosuppression ( secondary to AIDS or transplantation) is a risk factor. Infectious agents (Epstein-Barr virus, human herpesvirus 8, Toxoplasma) have been associated with PIOL. However, there have been no clear genetic or infectious markers to suggest susceptibility to PIOL. Symptoms Photophobia, ocular pain (rare) Signs ( Figs. 13-1 to 13-5) Vitreous cells (occurring in sheets) and haze Multifocal, cream-colored, subretinal

the United States Government.

279

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13 MASQ UERADE SYNDRO MES

AC cells, kera ic precipi a es (KP) Visual acui y is ar bet er han expec ed based on he amoun o in amma ion Dif erential Diagnosis Sarcoidosis Viral re ini is (cy omegalovirus, varicella zos er virus, herpes simplex virus), acu e re inal necrosis oxoplasmosis Syphilis uberculosis Endoph halmi is Me as a ic cancer Diagnostic Evaluation T e diagnosis is di cul , and requires malignan cells or issue or diagnosis. Fluorescein angiography demons ra es di use re inal pigmen epi helium ( PE) per urba ion and la e s aining a he PE level wi h a granular or “mot led” pat ern, early blockage wi h la e s aining o re inal/ subre inal lesions, op ic nerve s aining or leakage, and pigmen epi helial de achmen s. Lack o cys oid macular edema (CME) and re inal vascular leakage is an in eres ing ea ure o PIOL. Indocyanine green angiography shows small, round hypo uorescen areas ha disappear in he la e phase. OC : Nodular hyperre ec ive lesions in he PE issue diagnosis: Cy ology (large a ypical lymphoma cells), ow cy ome ry, cy okine analysis, and molecular analyses rom ocular uids (aqueous or vi reous) Brain/ spinal M I and lumbar punc ure (LP), ( or ow cy ome ry and cy ology) o iden i y CNS involvemen Treatment Curren herapies are no cura ive. Sys emic chemo herapy is he mains ay o rea men even when associa ed PCNSL is

no ound. Adjunc local chemo herapy can be adminis ered. Sys emic herapy: High-dose me horexa e; cyclophosphamide, adriamycin, vincris ine, and prednisone; cy arabine; radio herapy, and ri uximab have all been ried wi h various degrees o success. Local herapy: In ravi real me ho rexa e (400 mcg/ 0.1 mL), in ravi real ri uximab (1 mg/ 0.1 mL), and radia ion can all be considered. ecurrence and complica ions end o be higher wi h radia ion. Prognosis Prognosis is poor. Median progression- ree survival is less han 3 years, and overall survival is approxima ely 5 years and appears o be unaf ec ed by rea men ype. Sys emic spread ou side o he CNS is ex remely rare ( 1

Muscles

SR, MR

IR > MR > SR > LR

Muscle border

Irregular

Regular

Involved (Fig. 13 7B)

Spared

Involved

Relatively clear

endon Orbital at

Extraocular muscles: SR, superior rectus; MR, medial rectus; IR, in erior rectus; LR, lateral rectus.

misdiagnosis o as hma. Ocular examinaion may reveal eyelid and conjunc ival nodules, evidence o previous uvei is (kera ic precipi a es), iris nodules, vi reous debris, and cot on-wool spo s. Op ic neuri is may occur. Angio ensin-conver ing enzyme (ACE) and ches X-ray may be help ul bu can prove nega ive in a localized varian known as “orbi al sarcoid” or orbi al granuloma ous in amma ion. Orbi al me as asis: Me as a ic disease o he orbi ypically occurs in a more indolen and less in amma ory ashion han classic IOIS. However, in amma ory signs may cer ainly accompany orbi al me as asis. T e empiric diagnosis o IOIS should be used wi h grea cau ion in any pa ien wi h a known his ory o cancer, wi h a low hreshold or at emp ed orbi al biopsy. Special orm: One rare varian o orbi al in amma ion, known as sclerosing pseudoumor, is more chronic and causes signif can issue des ruc ion rom secondary f brosis. T is orm o orbi al in amma ion is poorly responsive o cor icos eroid herapy and may

require more dras ic measures, including chemo herapeu ic agen s, biologics, repea ed surgical debulking, and on occasion, exen eraion. T e clinical and his opa hologic pic ure is so a ypical o classic IOIS ha some exper s have appropria ely ques ioned whe her his diagnosis even belongs wi hin he rubric o orbi al in amma ion. IgG4 sclerosing disease is a newly described en i y in he orbi and may represen a sub ype o sclerosing pseudoumor. IgG4 orbi opa hy may be associa ed wi h f brosis o o her viscera and a higher risk o even ual lymphoma developmen .

DIAGNOSTIC EVALUATION C or M imaging will reveal a poorly circumscribed, inf l ra ing enhancemen o orbi al issue and may be limi ed o specif c issue (muscle, lacrimal gland). Bone des ruc ion is very a ypical and necessi a es issue biopsy. On occasion, B-scan ul rasonography is help ul in cases o pos erior scleri is, which may no be visible on C or M I.

Idiopathic Orbital Infammatory Syndrome (Orbital Pseudotumor)

Sys emic work-up is rarely help ul and is usually no indica ed. In pa ien s where di eren ia ion rom in ec ion is di cul , a CBC may be ob ained. An eleva ed whi e coun and le shi may be seen in bo h in ec ious and nonin ec ious orbi al in amma ion. Bac erial in ec ion will usually resul in neu rophilia wi h le shi , while in some cases o IOIS, eosinophilia may be presen . As already no ed, a care ul his ory o previous cancer mus be sough in all cases o presumed IOIS. A ypical cases o IOIS warran ur her inves iga ion, including serologies or sarcoidosis and Wegener granuloma osis and work-up or po en ial malignan sources (pros a e specif c an igen, mammography, ches imaging, e c.). T e need or ini ial orbi al biopsy o ei her conf rm IOIS or re u e o her con ounding diagnoses is con roversial. T ere is no “ ypical” his opa hology ha conclusively proves he diagnosis o IOIS; ra her, biopsy is usually done o rule ou o her dis inc pa hology. Cer ainly, biopsy should be considered in a ypical cases o IOIS or in pa ien s wi h a known his ory o malignancy. Because o he low morbidi y o lacrimal gland biopsy, some exper s also recommend biopsy o all cases o suspec ed in amma ory dacryoadeni is. Biopsy may no be easible i he a ec ed issue is no readily accessible (e.g., orbi al apex) or i surgery carries a signif can morbidi y (e.g., biopsy o an ex raocular muscle). wo addi ional poin s mus also be considered by he clinician. Firs , rea men o IOIS wi h sys emic cor icos eroids prior o at emp ed biopsy may mask he rue his opa hology o he process. Second, a “nega ive” biopsy does no necessarily rule ou a po en ially dangerous diagnosis: he biopsy may simply have been inadequa e. T is is a dis inc possibili y in biopsy o he orbi al apex and ex raocular muscles, where an at emp on ob aining more issue also increases he risk o permanen morbidi y rom he procedure.

339

In cases ha presen a ypically (subacu e/ chronic, bila erally in adul s) or respond poorly o cor icos eroid rea men (recalciran or recurren cases), a lower hreshold or a comple e sys emic work-up and issue biopsy in search o ano her cause should be aggressively pursued.

TREATMENT Cor icos eroids: In adul s, prednisone 80 o 100 mg daily usually resul s in rapid and drama ic clinical improvemen , o en a er only one dose; pedia ric doses should be calcula ed on he basis o body weigh (1 mg/ kg/ day). T is drama ic response o appropria e doses o cor icos eroids bols ers he presumpive diagnosis o IOIS bu is no conclusive: o her pa hologies, mos no ably lymphoproli era ive disease, may mani es similar drama ic responses. elapses are common and are usually rela ed o a rapid aper o cor icos eroids. Nons eroidal an i-in amma ory medicaions (NSAIDs). In pa ien s who are in oleran o cor icos eroids, he ini ial cor icos eroid dose may be apered more rapidly while a s eady dose o NSAIDs is main ained as a herapeu ic bridge, ollowed by a slow NSAID aper. adia ion herapy. A course o low-dose (2000 cGy) orbi al radio herapy may be considered. Many exper s manda e issue biopsy be ore proceeding wi h radio herapy. I any doub is presen be ween IOIS and orbi al celluli is, as occurs more o en in children han in adul s, i is pruden o f rs begin a rial o in ravenous an ibio ics wi h close clinical ollow-up. I symp oms ail o improve a er 48 hours, a “ es dose” o cor icos eroids may be given while s ill main aining an ibio ic coverage. A rapid response o cor icos eroids is indica ive o a nonin ec ious in amma ion. are cases o IOIS may no respond o cor icos eroids and NSAIDs, and in such

340

13 O RBI AL DISEASE O F NEURO -O PH HALMIC SIGNIFICANCE

cases an ime aboli e or biologic herapy may be considered, bu biopsy should be perormed f rs when easible. T e use o cor icos eroids injec ion in o orbi al issue or IOIS mirrors he discussion o his modali y in ED (see previous sec ion) on ED.

All pa ien s wi h IOIS, even hose wi h classic presen a ions and rapid, sus ained response o sys emic cor icos eroids mus be warned abou he limi a ions o empiric diagnosis and ollowed over he long erm o assure ha no con ounding diagnosis mani es s.

Idiopathic Orbital Infammatory Syndrome (Orbital Pseudotumor)

341

A

B

C FIGURE 13-6. Inf amma ory dacryoadeni is. A, B. Di use injec ion o he lacrimal gland wi hou purulen discharge. C. Axial C shows di use enlargemen o he lacrimal gland wi hou bone erosion.

342

13 O RBI AL DISEASE O F NEURO -O PH HALMIC SIGNIFICANCE

A

B FIGURE 13-7. Myosi is. A. T ickening o he medial rec us muscle inser ion wi h overlying conjunc ival injec ion. B. Axial MRI 1, gadolinium, a suppression reveals di use enlargemen o he muscle, including he inser ion.

Idiopathic Orbital Infammatory Syndrome (Orbital Pseudotumor)

343

A

B FIGURE 13-8. enoni is. A. Clinical signs o prop osis, ex ernal oph halmoplegia, and conjunc ival injec ion. B. On C , di use hickening and enhancemen o enon’s capsule are obvious.

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13 O RBI AL DISEASE O F NEURO -O PH HALMIC SIGNIFICANCE

A

B

C FIGURE 13-9. A. ypical pink, boggy eyelids o IOIS. No e limi a ion in upgaze. B. Coronal C shows di use in raconal enhancemen . C. Comple e clinical resolu ion 1 week a er oral cor icos eroids.

Cavernous Sinus Fistulas

CAV CAVERN VE RN N O US US S SIN INU US S FIS ULAS U LA AS

A

cquired ar eriovenous (AV) communicaions (f s ulas) a ec ing he orbi mos commonly occur in he cavernous sinus.

ETIOLO GY In simple erms, ar eriovenous f s ulas wi hin he cavernous sinus generally all in o wo ca egories: High- ow f s ulas are usually he resul o rauma, occur in younger pa ien s, and involve an abnormal connec ion be ween he caro id siphon and he cavernous sinus venous plexus (Barrow ype A). T e erm caro id-cavernous f s ula is used in erchangeably wi h high- ow f s ula. Low-f ow f s ulas ypically occur spon aneously in older pa ien s. T e abnormali y occurs be ween one o he small ar erial eeders o he la eral dural wall rom he in ernal caro id, ex ernal caro id, or bo h (Barrow ype B, C, D) and he venous plexus o he cavernous sinus, hence he erm “dural-sinus f s ula.” Al hough prac ical or he oph halmologis s, his rubric is a gross oversimplif caion o he s ra if ca ion o cavernous sinus f s ulas used by our neurosurgical colleagues. O her cri ical parame ers ha usually require angiography o elucida e include he source (in ernal versus ex ernal caro id ar ery), la erali y (ipsila eral, con rala eral, or bo h), and, mos impor an ly, he presence or absence o pos erior cor ical venous ou ow. I is impossible or an oph halmologis o assess his las ea ure on a clinical basis, ye remains a cri ical concep , since signif can pos erior cor ical venous ou ow by he f s ula markedly increases he risk o hemorrhagic s roke.

345

CLINICAL CH ARACTERISTICS Symp oms Visual acui y varies rom normal o poor Diplopia ed eye Headache may be presen A “whooshing” or “rushing” sound in he head Signs Prop osis Ex ernal oph halmoplegia a imes wi h ocular misalignmen (Fig. 13-10A) Auscul a ion over he superior orbi al rim may reveal a brui , al hough his is an admit edly uncommon f nding. T e conjunc ival vessels associa ed wi h f s ulae are di usely engorged and or uous (ar erializa ion, corkscrewing). T e vascular engorgemen o en ex ends o he limbus (Fig. 13-10B). In raocular pressure may be eleva ed. Applana ion onome ry o en shows a signif can pulsa ili y o he Myers prisms. Iris neovasculariza ion may occur rom chronic ocular ischemia. R PD and dyschroma opsia are presen i op ic neuropa hy develops. Venous engorgemen or cen ral re inal vein occlusion may be seen on unduscopic examina ion (Fig. 13-10C). Exuda ive re inal or choroidal de achmen s are less common. Ocular or macular ischemia

DIAGNOSTIC EVALUATION Orbi al color Doppler ul rasonography shows engorgemen o he superior

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13 O RBI AL DISEASE O F NEURO -O PH HALMIC SIGNIFICANCE

oph halmic vein (SOV), reversal o ow, and an ar erial wave orm wi hin he SOV (Fig. 13-11). Doppler ul rasonography is rela ively sensi ive in his disorder and should be u ilized in all cases o rule in or rule ou he diagnosis. An enlarged, S-shaped SOV in he superior orbi , jus benea h he superior rec us/ leva or complex on C or M I (Fig. 13-12). Bo h SOVs may be enlarged wi h asymme ric clinical f ndings, depending on ow characeris ics. T e ex raocular muscles may also be enlarged because o decreased venous ou ow and resul an orbi al conges ion. No e ha ex raocular muscle and SOV enlargemen may be seen in o her conges ive orbi opahies, mos no ably ED. When suspec ed, pa ien s wi h cavernous sinus f s ulas should undergo six-vessel ar eriography o bet er charac erize he abnormali y, assess or pos erior cor ical venous ou ow, and allow or ransar erial or ransvenous closure o he abnormali y (Fig. 13-13A).

TREATMENT In high- ow f s ulas ha presen wi h severe orbi al signs, including progressive op ic neuropa hy, rea men is indica ed. rea men is per ormed by neuroradiologic in erven ionalis s. T e loca ion o he abnormal connec ion is delinea ed using convenional ar eriography, and pre erably a he same ime, he f s ula is closed wi h a varie y

o echniques, including glue, balloons, or hrombogenic coils (Fig. 13-13B). On occasion, he f s ula canno be approached using conven ional ransar erial rou es. In such cases, he f s ula may be closed rom an SOV approach u ilizing a lid crease incision. T e pa ien is always warned abou he po en ial complica ions o ar eriography, including s roke and dea h, as well as he risks o f s ula closure, including visual loss, worsening o orbi al conges ion, marked in raocular pressure rise and orbi al compar men syndrome rom sudden closure o orbi al venous egress, cen ral re inal vein/ ar ery occlusion, and neovasculariza ion due o ocular ischemia. Low- ow f s ulas may be ollowed over ime unless he in raocular pressure is unconrolled, pos erior cor ical venous ou ow is presen , he f ndings persis over mon hs wi hou improvemen , or progressive ocular f ndings develop, since hese f s ulas may close spon aneously. A paradoxical worsening o symp oms may be seen in low- ow f s ulas. As hrombosis orms in he SOV, he pa ien may presen wi h a marked increase in signs and symp oms. I possible, conserva ive managemen is indica ed or 48 o 72 hours, a which ime he symp oms should begin o aba e as al erna e orbi al venous drainage orms. I no improvemen occurs a er his ime, he pa ien should undergo repea workup o assure ha a low- ow s a e has no conver ed in o a high- ow abnormali y.

Cavernous Sinus Fistulas

347

A

B

C FIGURE 13-10. Clinical signs. A. Mild prop osis and an abduc ion de ci are no ed on he righ . B. Ar erializa ion o conjunc ival vessels. C. Cen ral re inal vein occlusion.

348

13 O RBI AL DISEASE O F NEURO -O PH HALMIC SIGNIFICANCE

FIGURE 13-11. Color Doppler ul rasonography. Reversal o f ow red ins ead o he usual blue is no ed in he SOV. Also no e he presence o an ar erial wave orm.

FIGURE 13-12. Coronal C imaging reveals an engorged SOV arrow along wi h di use enlargemen o he ex raocular muscles on he a ec ed side.

Cavernous Sinus Fistulas

349

A

B FIGURE 13-13. Ar eriography. A. An abnormal connec ion is no ed be ween he caro id siphon small arrow and he cavernous sinus double arrow , wi h secondary engorgemen o he SOV large arrow . B. Flow in o he SOV ceases a er placemen o a de achable balloon arrow in o he cavernous sinus.

Index Note: Page number ollowed by and t indicates f gure and table respectively.

B

A Aberrant regeneration, 234, 243 Acetazolamide, 121 Acetylcholine receptor antibody assay, or MG, 289 Acquired bilateral color loss, 4 Acquired dyschromatopsia, 4 Acquired unilateral color loss, 4 Acromegaly, 178 Adie pupil, 304 Adie (tonic) pupil, 321–322, 323 –325 clinical characteristics o , 321, 323 –325 diagnostic evaluation o , 322, 324 di erential diagnosis o , 322 epidemiology o , 321 etiology o , 321 Amiodarone optic neuropathy, 107–108, 108 clinical characteristics o , 107, 108 diagnostic evaluation o , 107 di erential diagnosis o , 107 disc edema o , 108, 108 epidemiology o , 107 etiology o , 107 natural history o , 107–108 treatment o , 107t vs. NAION, 107t Amsler grid, 8, 9 Anisocoria, 231, 236 , 303 pathologic, 304, 309 physiologic, 303, 308 Anticoagulation therapy, 106 Anti-VEGF agents, intravascular, 63 Anton’s syndrome, 197 Apraclonidine, or Horner syndrome, 312, 316 Aquaporin-4, 74 Arcuate bundles, 12 Arcuate f eld de ect, 16 , 57 Argyll Robertson pupil, 326–327 clinical characteristics o , 326, 327 diagnostic evaluation o , 327 etiology and pathogenesis o , 326, 326t treatment or, 327 Arteriovenous mal ormations (AVM), 207, 211 Arteritic anterior ischemic optic neuropathy (A-AION), o GCA, 98–99, 100 Artery, cilioretinal, 56 Atheroma, carotid, 54, 60

350

Bailey-Love chart, 3 Benedikt syndrome, 232, 232t Bielschowsky head-tilt test, 244 Bilateral altitudinal f eld de ects, 197 Bilateral hyperemia with peripapillary hemorrhage, in toxic optic neuropathy, 113 Bilateral occipital lobe disease, 196–197, 203 –204 Binasal visual f eld de ect, 169 Binocular visual f eld test, 213 Biologic agents, use o , in TED, 332 Bitemporal hemianopia, 169, 171 Blind conjunctival biopsy, 79 Blood oxygen-level–dependent unctional MRI (BOLD- MRI), 26 Branch retinal artery occlusion (B O), 54, 56, 57 . See also Retinal artery occlusion recurrent, 60, 61 Brightness comparison, 8 Bromocriptine, 178, 181

C Cabergoline, 178 Calcium emboli, 54, 55 Campylobacter jejuni, and GBS, 285 “Candle wax spots”, 83 Cantholysis, or orbital hemorrhage, 163, 167 Cardiac abnormalities, 54 Cardiac echography, 60, 60t Cardiac myxoma, 54 Carotid atheroma, 54, 60 Carotid Doppler, 60, 60t Carotid endarterectomy, 63 Carotid occlusion, 56, 59 Carotid patency, 60 Carotid stenosis, 63 Cat-scratch disease (Bartonella henselae), 76 Cavernous sinus, 168, 260 anatomy o , 260, 262 lesions in, 260 Cavernous sinus f stulas, 345–346, 347 –349 arteriography, 346, 349 clinical characteristics o , 345, 347 diagnostic evaluation o , 345–346, 348 etiology o , 345 high- ow, 345

low- ow, 345 treatment o , 346 Cavernous sinus lesions, 244–245 Cavernous sinus meningioma, with diplopia, 39 Cavernous sinus syndrome, 232, 250, 260 arteriovenous f stula and, 260, 266 causes o , 260 cavernous sinus thrombosis and, 260, 267 –268 clinical characteristics o , 260–261 diagnostic evaluation o , 261 metastatic lesions and, 260, 263 –264 mucormycosis and, 260, 265 Cavernous sinus thrombosis, bilateral, 41 CD4-type T cells, 97 Cecocentral scotoma le eye, 12, 17 Central retinal artery, 54 Central retinal artery occlusion (C O), 54, 56. See also Retinal artery occlusion with cherry-red spot, 58 and visual loss in GCA, 98 Central scotoma right eye, 12, 17 Cerebrospinal uid (CSF) examination, 65 in syphilitic optic neuropathy, 87, 88t Checkerboard f eld de ect, 197, 203 –204 Chest imaging, or MG, 289 Chiasmal glioma, 179, 185 Cholesterol emboli, 54, 55 , 60 asymptomatic, 56 Choroidal ischemia, 98, 101 Chronic progressive external ophthalmoplegias (CPEO), 279 clinical characteristics o , 279, 281 etiology o , 279 inheritance o , 279 Cigare e smoking, cessation o , in Graves’ disease, 331 Cilioretinal artery, 56 Clinically def nite multiple sclerosis (CDMS), 66 CN palsies, multiple, 260–261, 262 –268 Cocaine 10%, or Horner syndrome, 312, 317 Color comparison, 5, 6 Color vision, 4–5

INDEX 3 5 1 Computed tomography (CT), 22 Computed tomography venography (CTV), 120 Con rontation visual f elds, 13–14 Congenitally anomalous discs, 141 , 142. See also Pseudopapilledema Contrast sensitivity, 8, 10 Contrast sensitivity impairment, in optic neuritis, 65 Controlled High-Risk Subjects AVONEX Multiple Sclerosis Prevention Study (CHAMPS), 66. See also Optic neuritis Convergence retraction nystagmus, 296 Cortical blindness, 197 Corticosteroids or GBS, 285 or GCA, 99–100 or IOIS, 339 or optic neuritis, 66 or radiation optic neuropathy, 106 or sarcoidosis, 80 or TED, 331 traumatic optic neuropathy and, 163 Cranial nerve III (CN III), 230 anatomy o , 230 Cranial nerve III palsy, 230–243 aberrant regeneration o CN III, 234, 243 primary, 234 clinical characteristics o , 231, 235 –237 diagnostic evaluation o , 232–233, 233t, 240 di erential diagnosis o , 233, 241 , 242 etiology o , 230–231 natural history o , 234, 234t pathophysiology o , 230–231 syndromes associated with, 231, 235 , 236 cavernous sinus syndrome, 232 CN III ascicle syndromes, 231 lesions in subarachnoid space, 232 nuclear III nerve lesions, 231, 232t orbital syndromes, 232 Cranial nerve IV (CN IV), 244 Cranial nerve IV palsy bilateral, 245 clinical characteristics o , 244, 246 –248 etiology o , 244 investigations o , 245 treatment or, 245 Cranial nerve VI, 249 anatomy o , 249 Cranial nerve VI palsy, 249 clinical characteristics o , 250, 252

congenital, 250 diagnostic evaluation o , 250–251, 254 –256 etiology o , 249 pseudo, 251 subarachnoid involvement in, 249 syndromes associated with cavernous sinus syndrome, 250 Foville syndrome, 249 Millard–Gubler syndrome, 249 orbital lesions, 250 petrous apex syndrome, 249–250 treatment o , 251 vasculopathic, 251 Craniopharyngiomas, 178–179, 183 C-reactive protein (CRP), or GCA, 99

D Decibels (dB), 14 De Morsier’s syndrome. See Septooptic dysplasia Depot corticosteroid, or TED, 332 Depth perception problems, 172 Devic disease, 74 Diabetes, and carotid occlusion, 56, 59 Diabetic papillopathy, 105, 105 clinical characteristics o , 105 clinical course o , 105 etiology o , 105 treatment o , 105 Di usion tensor imaging (DTI), 26, 50 Di usion-weighted imaging (DWI), 25 or acute cerebral cortical in arction, 25, 44 , 48 or def ning abscess, 25, 43 Diplopia, 216 evaluation binocular, 218 binocular single vision visual f eld or, 228, 229 comitant or incomitant, 218–219, 219 –222 ductions and versions or, 223, 223 orced duction test in, 224, 226 –227 lids and pupils in, 224 monocular, 216–217, 217 nystagmus and, 225 ocular cephalic movements in, 224 primary and secondary deviation in, 218, 220 prism cover test, 218, 219 , 220 red lens or Maddox rod in, 218–219, 221 , 222 saccades and pursuits in, 224 ocular motility disturbances without, 228 parachiasmal lesions and, 172, 173

Diplopia test, or monocular blindness, 213 “Disc at risk”, 90, 91 Disulf ram, 110t DOA. See Dominant optic atrophy (DOA) Dominant optic atrophy (DOA), 136, 137 –141 clinical characteristics o , 136 diagnostic evaluation o , 136 di erential diagnosis o , 136 epidemiology o , 136 treatment o , 136 Dorsal midbrain (Parinaud) syndrome, 296, 297 clinical characteristics o , 296 diagnostic evaluation o , 296 etiology o , 296 treatment o , 296 Double vision, 99, 279 Droopy lid, 279 Duane retraction syndrome, 250, 253 Dyschromatopsia, 90, 197 Dyschromatopsia, acquired, 109

E E erent system, neuro-ophthalmologic examination o , 216–229 Electromyography (EMG), or MG, 289 Emboli calcium, 54 cholesterol, 54 at, 54 in ectious, 54 neoplasm, 54 platelet–f brin, 54 retinal, 54 talc, 54 visible, 60 Encephalopathy, 56 Endarterectomy, 63 Erythrocyte sedimentation rate (ESR), 99 Esotropia, 250, 252 Ethylene glycol, 110t

F Farnsworth Munsell 100 Hue, 4–5, 5 , 136, 140 Farnsworth panel D15, 4 Fiber tractography, 26, 50 Finger counting con rontation visual f elds, 13, 18 –19 , 20 Fluid-a enuated inversion recovery (FLAIR), 24–25, 34 Fluorescein angiography, 76 Fogging, 213 Forced duction test, 224, 226 –227

3 5 2   INDEX Foville syndrome, 249 Functional MRI, 25–26 Functional tests, or no vision, 212

G Gass plaque, 61 Gaze palsies, 275–276, 275t, 277 , 278 clinical characteristics o , 276, 277 etiology o , 275–276 lesions location and, 275, 275t abducens nucleus, 275 rontal lobe, 275 PPRF, 275 special orms o , 276 Gaze pareses, 275 GBS. See Guillain-Barré syndrome (GBS) GCA. See Giant cell arteritis (GCA) Gerstmann syndrome, 195–196 Ghost image, 216 Giant cell arteritis (GCA), 56, 60, 97–100, 100 –102 age actor and, 97 clinical characteristics o , 97–99 diagnosis o etiology o , 97 incidence o , 97 ischemia in, 97 occult orm, 97 pathogenesis o , 97 and polymyalgia rheumatica, 97 signs o , 98–99 symptoms o , 97–98 treatment or, 99–100 Glaucoma, 146 Glioma malignant, 127, 127 , 128 optic nerve, 123–125, 125 , 126 Goldmann manual perimetry. See Kinetic manual perimetry Gradenigo syndrome, 250 Granulomatous uveitis, anterior, 79 Graves’ disease. See yroid eye disease (TED) Guillain–Barré syndrome (GBS), 285, 286 clinic characteristics o , 285, 286 etiology o , 285 treatment or, 285

H Hallucinations, occipital lobe, 197 Halogenated hydroxyquinolines, 110t Hardy–Rand–Ri ler (HRR) plate, 4 Heart block, KSS and, 280 Hemianopia bitemporal, 169, 171 homonymous, 169 Hemi-f eld slide phenomenon, 172 Hemihypoplasia, 154, 156

Hollenhorst plaque, 55 , 60 Homonymous hemianopia, 169, 194 complete, 194 incomplete, 194 Horizontal raphé, 12 Horner syndrome, 302, 304, 305 , 309 , 311–313, 311t, 314 –317 carotid artery dissection and, 313 clinical characteristics o , 311–312, 314 –315 anhidrosis, 312 anisocoria, 311–312 dilation lag, 312 iris heterochromia, 312 paradoxical pupillary dilation, 312 ptosis, 311 skin changes, 312 diagnostic evaluation o , 312–313, 316 , 317 etiology o , 311 treatment o , 313 Hour-glass–shaped embolus, 55 Humphrey visual f eld test, NAION and, 94 Hydroxyamphetamine, or Horner syndrome, 313 Hydroxycobalamin, intramuscular injections o , 110 Hypertensive optic neuropathy, 76, 103, 104 clinical characteristics o , 103 di erential diagnosis o , 103 etiology o , 103 with hemimacular star, 78 increased intracranial pressure and, 103 and swollen optic discs, 103, 104 treatment o , 103 Hyperviscosity syndromes, 54

I Ice test, 289, 295 Idiopathic intracranial hypertension (IIH), 120–121, 121 , 122 Idiopathic orbital in ammatory syndrome (IOIS), 336–340, 341 –344 boggy eyelids o , 344 clinical characteristics o , 336 dacryoadenitis in, 341 diagnostic evaluation o , 338–339 di erential diagnosis o , 336–338, 337t, 338t adenoviral conjunctivitis, 337 lymphoproli erative disease, 336–337 orbital cellulitis, 336 orbital metastasis, 338 sarcoidosis, 337–338 Wegener granulomatosis, 337

etiology o , 336 myositis in, 342 tenonitis in, 343 treatment in, 339–340 vs. cellulitis, 337t vs. orbital lymphoproli erative disease, 337t vs. thyroid eye disease, 337, 338t Immunosuppressive therapy, or NMO, 74, 75 INO. See Internuclear ophthalmoplegia (INO) Internal carotid artery stenosis, 56–57 Internuclear ophthalmoparesis, 269, 271 Internuclear ophthalmoplegia (INO), 269–270, 270 –274 bilateral, 269, 273 clinical characteristics o , 269, 270 diagnostic evaluation o , 269, 272 etiology o , 269 MRI o , 269, 272 special orms o , 270, 274 treatment o , 269 Intraocular pressure, lowering o , 63 Intravenous uorescein angiography (IVFA), 99 Intravenous immune globulin (IVIg), 285 IOIS. See Idiopathic orbital in ammatory syndrome (IOIS) Ipsilateral internal carotid artery occlusion, 56–57 Ischemic optic neuropathy, 107, 107t, 132 Ischemic Optic Neuropathy Decompression Trial (IONDT), 90 Ishihara pseudo-isochromatic plates, 4, 4

J Jaw claudication, 98 Junction scotoma, 169, 170 , 171

K Kearns-Sayre syndrome (KSS), 279–280, 282 Kinetic manual perimetry, 15 Kinetic red target, 13

L Lacrimal glands, 79, 85 Lacunar in arct, in midbrain, 48 Lateral geniculate nucleus (LGN) lesions, 195

INDEX 3 5 3 Leber hereditary optic neuropathy (LHON), 132–133, 134 , 135 acute phase, 132 chronic phase, 132 clinical characteristics o , 132 with dense central scotomas, 135 diagnostic evaluation o , 132–133 di erential diagnosis o , 132 etiology o , 132 mutations in mitochondrial DNA in, 132 optic disc changes in, 132, 134 prognosis o , 133 Leber stellate neuroretinitis, 76, 77 , 78 clinical characteristics o , 76 diagnostic evaluation o , 76 epidemiology o , 76 etiology o , 76 natural history o , 76 treatment or, 76 Lesions in corpus callosum, 60, 62 lateral geniculate nucleus (LGN), 195 o optic disc, 13 o optic nerve, 12 optic tract, 194–195, 198 –199 o parietal lobe, 195–196 in retinal pigment epithelium, 83 o temporal lobe, 195, 199 LHON. See Leber hereditary optic neuropathy (LHON) Lid-gaze dyskinesis, 234 Light–near dissociation, pupillary, 326–327, 326t, 327 Lumbar puncture, 88, 120 Lumboperitoneal shunt, 121 Lyme disease, and neuroretinitis, 76

M Macular disease, 10 Macular sparing, 196, 201 Macular star, 76, 77 Magnetic resonance angiography (M ), 25, 60, 60t or arterial stenosis, 25, 48 or ICA dissection, 25, 49 or intracranial aneurysms, 25, 47 Magnetic resonance imaging (MRI), 22–26, 27 –50 claustrophobia during, 22 di usion tensor imaging, 26 di usion-weighted imaging, 25 uid-a enuated inversion recovery, 24–25, 34 unctional, 25–26 undamentals o , 23 gadolinium-enhanced T1, 24 high-f eld strength unit, 22

imaging protocol or, 51, 52 , 53 lesions in corpus callosum, 60, 62 low-f eld strength unit, 22–23 or optic neuritis, 22, 65 proton density weighted image contrast, 25 pulse sequences o , 23 short TI inversion recovery and T2 at saturated sequence, 24 so tissues accentuation in, 22 or subclinical demyelinating plaques, 65, 71 or subtle abnormalities a ecting optic pathways, 22 T1-weighted imaging, 23–24, 27 T2-weighted imaging, 24, 27 T2W gradient echo, 25, 46 or visual pathways assessment, 22 Magnetic resonance venography (MRV), 25, 120 Malignant glioma, 127, 127 , 128 clinical characteristics o , 127, 127 etiology o , 127 MRI o , 127, 128 treatment o , 127 Medial longitudinal asciculus (MLF), 269 Methotrexate, or sarcoidosis, 80 Methylprednisolone, intravenous or neuromyelitis optica, 75 or optic neuritis, 66 Methylprednisolone, intravenous, in GCA, 63 MG. See Myasthenia gravis (MG) Migraine, 207–208, 209 –211 aura o , 207, 209 classif cation o , 207 clinical characteristics o , 207 diagnostic evaluation o , 208 di erential diagnosis o , 207 treatment o , 208 vs. arteriovenous mal ormation, 207, 211 Millard–Gubler syndrome, 249 Miller Fisher syndrome, 287 Mirror test, 213 Mitochondrial myopathies, 279. See also Chronic progressive external ophthalmoplegias (CPEO); Kearns-Sayre syndrome (KSS) Mobility testing, or no vision, 212 Möbius syndrome, 250 Monocular diplopia, 216–217, 217 Morning glory disc anomaly, 157, 159 MRI. See Magnetic resonance imaging (MRI) Mucormycosis, 60 Multiple sclerosis, 34 Multiple sclerosis (MS), 64

Myasthenia gravis (MG), 233, 288–289, 290 –295 bilateral ptosis in, 288, 291 clinical characteristics o , 288–289, 290 –294 diagnostic evaluation o , 289, 295 etiology o , 288 ophthalmoplegia in, 289, 293 pathogenesis o , 288 and pseudo-INO, 288, 292 treatment or, 289 Mydriasis, 318, 319 , 320 Adie’s tonic pupil, 318, 321 cranial nerve III palsy, 318 pharmacologic, 318, 320 trauma, 318, 319

N NAION. See Nonarteritic anterior ischemic optic neuropathy (NAION) Nasal nerve f ber bundles, 12–13 Nelson syndrome, 178 Neurof bromatosis type 1 (NF-1), 123 eatures o , 124t Neuromyelitis optica (NMO), 74–75 clinical characteristics o , 74 diagnostic evaluation o , 74–75, 74t epidemiology o , 74 etiology o , 74 neuro-ophthalmic eatures, 74 treatment o , 75 Neuro-ophthalmic examination, 2 procedures be ore, 2 purpose o , 2 Neuroretinitis, 76. See also Leber stellate neuroretinitis in syphilis, 86 Neurosarcoidosis, with sudden vision loss, 37 NMO. See Neuromyelitis optica (NMO) NMO-IgG antibody titer, 74 Nonarteritic anterior ischemic optic neuropathy (NAION), 90–91, 91 –96 clinical characteristics o , 90, 92 diagnosis and investigations o , 91 edema o , 91 etiology o , 90, 91 incidence o , 90 natural history o , 91, 92 –96 pathogenesis o , 90 treatment or, 91 Nonphysiologic visual loss, 212–215 decreased vision and, 213 no vision and, 212–213 visual f eld loss and, 214, 215 Nonsteroidal anti-in ammatory medications (NSAIDs), or IOIS, 339

3 5 4   INDEX Nothnagel syndrome, 231, 232t Nuclear- ascicular lesions, 244 Nutritional optic neuropathy, 109–110, 112 , 113 bilateral visual loss in, 109 di erential diagnosis o , 109t clinical presentation o , 109 diagnostic evaluation o , 109 etiology o , 109 treatment o , 110 Nystagmus, 298 binocular end-point, 300 optokinetic, 300 vestibular, 300 congenital, clinical characteristics o , 299t dissociated convergence-retraction, 300 see-saw, 300 jerk, 298 localization o , 299t mimicking conditions o , 300t monocular, 298–299 Heimann-Bielchowsky phenomenon in, 298 internuclear ophthalmoplegia in, 298 spasm nutans in, 298–299 superior oblique myokymia in, 299 nondissociated acquired pendular, 301 Brun’s, 301 congenital, 301 downbeat, 301 gaze evoked, 301 latent, 301 periodic alternating, 301 rebound, 301 upbeat, 300 vestibular, 301 pendular, 298

O Occipital abscess, with visual def cit, 43 Occipital in arct, 44 Occipital lobe hallucinations, 197 Occipital lobe lesions, 207, 210 Occipital lobe visual f eld de ects, 196–197 Occluder, 3 Occlusion carotid, 56, 59 ophthalmic artery, 56 retinal artery, 54–63 Ocular ischemic syndrome, 56–57, 99 clinical eatures o , 57 prognosis o , 57 treatment o , 63

Ocular massage, 63 Ocular misalignment, 99 Ocular torsion, 246 One-and-a-hal syndrome, 276 ON . See Optic Neuritis Treatment Trial (ON ) Ophthalmic artery occlusion, 56 Ophthalmoplegia, 231 Ophthalmoscopy, 11 Optical coherence tomography (OCT), 145 buried drusen by, 153 retinal nerve f ber layer measurement by, 186, 189 –191 Optic atrophy, 79 Optic chiasm, 168–193 anatomic eatures o , 168 con uence o optic nerves at, 168 demyelinating plaque in, 192 disorders o , 168–169 band optic atrophy with bitemporal visual f eld de ect, 173, 176 –177 clinical characteristics o , 172–173, 173 –177 craniopharyngiomas and, 178–179, 183 etiology o , 172, 172t gliomas and, 179, 185 meningioma and, 179, 184 nontumor lesions and, 191, 192 , 193 pituitary tumors and, 178, 180 –182 parachiasmal tumors, ollow-up o , 186 position o , 168 postf xed, 168 pref xed, 168 prolapse o , 186, 188 radiation necrosis o , 186, 187 visual f eld abnormalities in, 169, 170 , 171 binasal visual f eld de ect, 169 bitemporal hemianopia, 169, 171 homonymous hemianopia, 169 junction scotoma, 169, 170 , 171 and visual loss, 168–169 visual recovery ollowing chiasmal decompression, 186, 189 –191 Optic disc colobomas o , 157, 158 , 159 granuloma o , 79, 82 normal, 142 Optic disc colobomas, 157, 158 , 159 clinical characteristics o , 157, 158 diagnostic evaluation o , 157, 159 etiology o , 157

ocular anomalies associated with, 157t treatment o , 157 Optic disc drusen, 90, 145–146, 146 –153 auto uorescence o , 151 B-scan ultrasonography or, 145, 150 calcif ed, 150 circumpapillary subretinal hemorrhage, 148 clinical characteristics o , 145 crescent-shaped hemorrhage, 149 diagnostic evaluation o , 145 di erential diagnosis o , 145 epidemiology o , 145 etiology o , 145 as glistening masses, 146 OCT or buried drusen, 153 peripapillary pigment atrophy, 148 progression o , 147 as white spot, 150 Optic disc edema, 90, 92 Optic disc swelling in neuroretinitis, 76, 77 in optic neuritis, 65, 71 Optic nerve abnormalities o , in syphilis, 86 (See also Syphilitic optic neuropathy) in ammation o (See Optic neuritis) Optic nerve glioma, 42 , 123–125, 125 , 126 . See also Neurof bromatosis type 1 (NF-1) clinical characteristics o , 123, 125 epidemiology o , 123 etiology o , 123 MRI o , 123, 126 treatment o , 123–124 chemotherapy, 124 radiation therapy, 124, 125t surgical resection, 123 Optic nerve hypoplasia, 154–156 clinical characteristics o , 154, 155 diagnostic evaluation o , 154 etiology o , 154 orms o hemihypoplasia, 154, 156 septooptic dysplasia, 155 treatment o , 154 Optic nerve sheath enestration, 121 Optic nerve sheath hematoma, 162 Optic nerve sheath meningiomas, 33 , 129, 130 , 131 clinical characteristics o , 129, 130 diagnostic evaluation o , 129 epidemiology o , 129 etiology o , 129 MRI o , 131 treatment o , 129

INDEX 3 5 5 Optic neuritis, 8, 35 –36 , 64–73, 132 acute, 64 clinic characteristics o , 64–65 demyelinating, 64 diagnosis o , 65, 65t epidemiology o , 64 etiology o , 64 MRI or, 22, 65 natural history o , 66–68 optic nerve visual f eld de ect in, 65, 70 progression to MS, 68 studies on, 66 CHAMPS, 67–68, 69t, 73 ON , 66–67, 67t, 68t, 72 treatment or, 66 e Optic Neuritis Treatment Trial (ON ), 66, 66t baseline data o , 67t on benef ts o corticosteroids, 66 details o , 66t risk at yearly ollow-up periods., 68t on risk o development o multiple sclerosis, 68t, 72 visual f eld abnormalities in, 70 visual recovery, 67t Optic neuropathies. See also specif c optic neuropathy anterior, 64 posterior, 64 Optic neuropathies with loss o color perception, 4 Optic perineuritis (perioptic neuritis), 86 Optic pit, 160, 160 , 161 clinical characteristics o , 160, 160 , 161 etiology o , 160 retinal elevation in, 160, 161 Optic tract, 194 lesions, 194–195, 198 –199 Optic tract syndrome, 169 Optociliary shunt vessels, 115 Optokinetic response, blind eye and, 213 Orbital apex syndrome, 261 Orbital hemorrhage, with optic neuropathy, 166 Orbital in ammation, 233 etiology o , 331t Orbital in ammatory disease, 251, 258 Orbital lesions, 245, 250 Orbital radiation, in TED, 331–332 Orbital syndromes, 232 Orbital trauma, 233, 241 Outstretch arm-to-nose test, 212

P Palinopsia, 197 Panphotolasercoagulation, 63

Papilledema, 76, 87, 107, 115–116, 117 –119 , 132, 296, 297 acute, 115, 117 atrophic, 115, 118 chronic, 115, 118 clinical characteristics o , 115–116 def nition o , 115 diagnostic evaluation o , 116 di erential diagnosis o , 116 etiology o , 115 with uid and exudates in macula, 119 requent causes o , 116t insipient, 115, 117 stages o , 115 treatment o , 116 Papillitis, 86, 89 Papillomacular bundle, 12 Paracentral homonymous scotomas, 196, 202 Parachiasmal meningiomas, 179, 184 Paranasal sinus ungal in ection, 60 Parasellar tumors, and photophobia, 172 Parietal lobe, lesions o , 195–196 Parks ree-Step Test, 244, 246 Patelet-derived growth actors, 97 PCOM artery aneurysm, 232 Pelli–Robson chart, 10 Periocular pain, dull, 57 Peripapillary hemorrhages, 105 Periventricular white ma er lesions, 62 Petrous apex syndrome, 249–250 Petrous bone racture, 250 Phosphodiesterase type 5 drugs, and NAION, 90 Photophobia, 172 Photostress recovery test, 8, 10 Pigmentary retinopathy o KSS, 280, 282 Pituitary apoplexy, 178, 182 Pituitary tumors, 31 and chiasmal syndrome, 178, 180 –182 visual f eld de ect with, 189 Plasmapheresis, 74 and GBS, 285 Platelet–f brin emboli, 54 Polymyalgia rheumatica (PMR), 97, 98 Polyopia, 197, 205 –206 Pontine paralytic exotropia, 276, 278 Pontine paramedian reticular ormation (PPRF), 275 Positive visual phenomena, 64–65 Posterior communicating artery aneurysm (PCoA), with oculomotor nerve palsy, 47 Posterior ischemic optic neuropathy (PION), 98 Postf xation blindness, 172, 174 bitemporal hemianopia and, 172, 175

Prednisone, oral, or optic neuritis, 66 Prism cover test, 218, 219 , 220 Progressive multi ocal leukoencephalopathy (PML), and visual f eld def cit, 32 Progressive supranuclear palsy (PSP), 283, 284 clinical characteristics o , 283, 284 di erential diagnosis o , 283 etiology o , 283 natural history o , 283 Prolactin-secreting pituitary tumors, 178 Proptosis, 123 Pseudodrusen, 115 Pseudo-Grae e sign, 234 Pseudopapilledema, 142, 143 , 144 clinical characteristics o , 142 etiology o , 142 with visible drusen, 143 vs. papilledema, 142t white areas around optic discs o myelin, 142, 144 Pseudotumor cerebri (PTC), 120–121, 121 , 122 clinical characteristics o , 120 diagnostic criteria or, 120 diagnostic evaluation o , 120 etiology o , 120 idiopathic variety o , 120 MRI or, 45 , 120, 121 treatment o , 120–121 PSP. See Progressive supranuclear palsy (PSP) PTC. See Pseudotumor cerebri (PTC) Pupil, 302 examination o , technique o , 303, 307 near response, testing or, 303, 304 pupillary reactivity, 303, 304 pupillary size, 303–304, 308 relative a erent pupillary de ect, 303, 304 parasympathetic innervation o , 302–303, 306 sympathetic innervation o , 302, 305 Pupil-gaze dyskinesis, 234 Pupillary mydriasis, 296 Pupillary testing, 5–8, 6 , 7

R Radiation optic neuropathy, 106 clinical characteristics o , 106 diagnosis o , 106 di erential diagnosis o , 106 epidemiology o , 106 etiology o , 106 prognosis o , 106 treatment or, 106

3 5 6   INDEX Radiation therapy or IOIS, 339 or optic nerve glioma, 124 prognosis o , 125 side e ects o , 125t PD. See Relative a erent pupillary de ect ( PD) Red comparison, 13–14 Red lens/ Maddox rod, 218–219, 221 , 222 Relative a erent pupillary de ect ( PD), 5–8, 7 , 56, 90, 213 Rest test, 289 Retinal artery occlusion, 54–63 diagnostic evaluation o , 60, 60t epidemiology o , 54 etiology o , 54 in GCA, 98–99, 100 signs o , 56–57 symptom o , 56 treatment o , 63 Retinal edema, 56 Retinal emboli, 54 Retinal nerve f ber layer, thinning o , 93 , 95 Retinal nerve f bers, 12 arcuate nerve f ber bundles, 12 nasal nerve f ber bundles, 12–13 papillomacular bundle, 12 Retinal pigment epithelium (RPE) atrophy o , 83 white lesions in, 83 Retinal veins, 57 Retrobulbar optic neuritis, 86–87 Retrochiasmal disorders, 194–211 homonymous hemianopia, 194 LGN lesions, 195 o occipital lobe, 196–197 bilateral occipital lobe disease, 196–197, 203 –204 cortical blindness, 197 dyschromatopsia, 197 in arcts o , 203 –204 macular sparing o , 196, 201 palinopsia, 197 paracentral homonymous scotomas o , 196, 202 polyopia, 197, 205 –206 temporal crescent sparing or involvement o , 196, 200 optic tract syndrome type I, 194–195, 198 –199 optic tract syndrome type II, 195 o parietal lobe, 195–196 o temporal lobe, 195, 199

Ring scotoma, 196–197 Rituximab, or sarcoidosis, 80 Rule o the pupil, 231

S Sarcoid granulomas, 79, 82 Sarcoidosis, 79–80, 81 –85 clinical characteristics o , 79 diagnostic evaluation o , 79 etiology o , 79, 81 MRI or, 79, 84 nonoptic nerve mani estations o , 80t ocular eatures o , 80t treatment o , 80 Scalp tenderness, 98 Sclerosing pseudotumor, 338 Scotomas, 56 cecocentral, 12, 17 central, 12, 13, 17 , 79 centrocecal, 109 gross, 13 See-saw nystagmus, 173 Septooptic dysplasia, 155 Serological testing, in NMO, 74 SITA (Swedish Interactive reshold Algorithm), 14 Skew deviation, 245–246 Snellen acuity, 2, 8 Spasm o the near re ex (SNR), 251, 259 Spectral domain optical coherence tomography, o optic nerve head, 93 Static automated perimetry, 14–15 Static f nger wiggle, 14 Stereopsis, 213 Strabismus comitant, 218 incomitant, 218 Subacute superior sagi al sinus (SSS) thrombosis, 30 Subarachnoid space lesions, 244 Superf cial temporal arteries abnormal, GCA and, 99, 101 color Doppler o , 99 Superior ophthalmic vein (SOV), 345–346 Superior orbital f ssure syndrome, 261 Susac syndrome, 56, 60, 61 Syphilis, and neuroretinitis, 76 Syphilitic optic neuropathy, 86–89 bilateral papillitis rom, 89 clinical characteristics o , 86–87, 86t CSF testing in, 88t diagnostic evaluation o , 87, 88t etiology o , 86 ocular mani estations o , 86t serologic tests or, 87t

T Taboparetic pupils, 326 Tangent screen, 15 TED. See yroid eye disease (TED) Temporal artery biopsy, 60, 63 or GCA diagnosis, 99, 102 Temporal lobe, lesions o , 195, 199 Ten-diopter prism test, 213 Tensilon test, 289 iamine def ciency, 110 rombocytosis, GCA and, 99 rombolytics, or C O, 63 yroid eye disease (TED), 233, 257 , 328–332, 333 –335 acute in ammatory, with lid retraction, 329, 333 clinical characteristics o , 329–330 conjunctival chemosis, 329, 334 diagnostic evaluation o , 330, 335 di erential diagnosis o , 330, 331t epidemiology o , 328 etiology o , 328 incidence o , 328–329 lid lag in downgaze, 329, 333 strabismus, 330, 334 treatment o , 330–332 upper eyelid retraction in, 329, 333 yroid unction studies, or MG, 289 yroid-stimulating antibody (TSI) test, 330 Tobacco–alcohol amblyopia, 111 TON. See Traumatic optic neuropathy (TON) Topiramate, 121 Topless disc syndrome. See Hemihypoplasia Toxic optic neuropathies, 110t ethambutol, 111 medications producing, 111t methanol, 110–111, 114 tobacco, 111 Toxoplasmosis, and neuroretinitis, 76 Transient ischemic a acks (TIA), 63 Transient visual loss, 97 Traumatic optic neuropathy (TON), 162–164, 164 –167 clinical characteristics o , 162–163 diagnostic evaluation o , 163, 167 epidemiology o , 162 etiology o , 162 blunt head trauma, 162 complete/ partial avulsion o optic nerve, 162 compression by bony ragment, 162, 164 optic nerve sheath hematoma, 162 penetrating injury, 162, 164 orbital hemorrhage with, 163, 166 treatment or, 163–164, 167

INDEX 3 5 7 Tritanopia, 136 Tuberculum sellae meningioma, 40

U Uhtho phenomenon, 65 Uncal herniation, 232 Upright–supine test, 246

V Vascular endothelial growth actor (VEGF), 97 Vasculitis, 54 Venous stasis retinopathy, 57 Ventriculoperitoneal shunt, 121 Vertical misalignment, 244 Viral illness, and neuroretinitis, 76 Visible emboli, 60

Visual acuity (VA), 2–3, 3 improvement in, methods or, 2 Visual allesthesia, 197 Visual-evoked responses (VER), 65 Visual f elds de ects, 56 arcuate, 57 optic disc lesions and, 13 optic nerve lesions and, 12 examinations, types o con rontation visual f elds, 13–14 kinetic manual perimetry, 15 static automated perimetry, 14–15 tangent screen, 15 extent o , 12 and retina, relationship between, 12–13 testing or, 12

Visual loss in GCA, 98–99 in giant cell arteritis, 97 nonphysiologic, 212–215, 215 Vitamin B12 def ciency, 110 Vitamin B12 injections, 110 Vitreous traction, 207

W Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO), 270, 274 Weber syndrome, 231, 232t Weight loss, 121 Wernicke’s hemianopic pupil, 195 Westergren method, 99 Wilbrand’s knee, 169 Worth our-dot test, 213

CO LO R ATLAS & SYNO PSIS OF CLINICAL OPHTHALMOLOGY

W i l l s Ey e I n s t i t u t e

Oc u lo p la s tic s S ECON D EDITION

EDITOR

Robert B. Penne, MD Director and Attending Surgeon, Oculoplastics Service Co-Director, Ocular Cicatricial Pemphigoid Clinic Wills Eye Institute Philadelphia, Pennsylvania

SERIES EDITOR

Christopher J. Rapuano, MD Director and Attending Surgeon, Cornea Service Co-Director, Refractive Surgery Department Wills Eye Institute Professor of Ophthalmology Jefferson Medical College of Thomas Jefferson University Philadelphia, Pennsylvania

CO LO R ATLAS & SYNO PSIS OF CLINICAL OPHTHALMOLOGY

W i l l s Ey e I n s t i t u t e

Oc u lo p la s tic s S ECON D EDITION

Senior Executive Editor: Jona han W. Pine, Jr. Senior Product Managers: Emilie Moyer and Grace Capu o Senior Manufacturing Coordinator: Benjamin Rivera Marketing Manager: Lisa Lawrence Creative Director: Doug Smock Production Services: Ap ara, Inc. © 2012 by LIPPINCOT WILLIAMS & WILKINS, a Wolters Kluwer business First edition, © 2003 e McGraw-Hill Companies, Inc. wo Commerce Square 2001 Market Street Philadelphia, PA 19103 USA LWW.com All righ s reserved. T is book is pro ec ed by copyrigh . No par o his book may be reproduced in any orm by any means, including pho ocopying, or u ilized by any in orma ion s orage and re rieval sys em wi hou writ en permission rom he copyrigh owner, excep or brie quo a ions embodied in cri ical ar icles and reviews. Ma erials appearing in his book prepared by individuals as par o heir of cial du ies as U.S. governmen employees are no covered by he above-men ioned copyrigh . Prin ed in China 978-1-60913-265-1 1-60913-265-3 Library o Congress Ca aloging-in-Publica ion Da a available upon reques Care has been aken o con rm he accuracy o he in orma ion presen ed and o describe generally accep ed prac ices. However, he au hors, edi ors, and publisher are no responsible or errors or omissions or or any consequences rom applica ion o he in orma ion in his book and make no warran y, expressed or implied, wi h respec o he currency, comple eness, or accuracy o he con en s o he publica ion. Applicaion o he in orma ion in a par icular si ua ion remains he pro essional responsibili y o he prac i ioner. T e au hors, edi ors, and publisher have exer ed every e or o ensure ha drug selec ion and dosage se or h in his ex are in accordance wi h curren recommenda ions and prac ice a he ime o publica ion. However, in view o ongoing research, changes in governmen regula ions, and he cons an ow o in orma ion rela ing o drug herapy and drug reac ions, he reader is urged o check he package inser or each drug or any change in indica ions and dosage and or added warnings and precau ions. T is is par icularly impor an when he recommended agen is a new or in requen ly employed drug. Some drugs and medical devices presen ed in he publica ion have Food and Drug Adminis ra ion (FDA) clearance or limi ed use in res ric ed research set ings. I is he responsibili y o he heal h care provider o ascer ain he FDA s a us o each drug or device planned or use in heir clinical prac ice. o purchase addi ional copies o his book, call our cus omer service depar men a (800) 638-3030 or ax orders o (301) 223-2320. In erna ional cus omers should call (301) 223-2300. Visi Lippincot Williams & Wilkins on he In erne : a LWW.com. Lippincot Williams & Wilkins cus omer service represen a ives are available rom 8:30 am o 6 pm, ES . 10 9 8 7 6 5 4 3 2 1

To Devany, Daniel, and Mara— the source of pride and balance in my life

Abou he Series he beau y o he a las/ synopsis concep is he power ul combina ion o illus ra ive pho ographs and a summary approach o he ex . Oph halmology is a very visual discipline ha lends i sel nicely o clinical pho ographs. While he seven oph halmic subspecial ies in his series—cornea, re ina, glaucoma, oculoplas ics, neurooph halmology, uvei is, and pedia rics—employ varying levels o visual recogni ion, a rela ively s andard orma or he ex is used or all volumes.

vi

T e goal o he series is o provide an up- oda e clinical overview o he major areas o oph halmology or s uden s, residen s, and prac i ioners in all he heal h care pro essions. T e abundance o large, excellen -quali y phoographs and concise, ou line- orm ex will help achieve ha objec ive. Chris opher J. Rapuano, MD Series Editor

Pre ace his ex is aimed a assis ing physicians (oph halmologis s and nonoph halmologis s) in recognizing he mos common oculoplas ic condi ions. Many oculoplas ic condi ions can be diagnosed on simple visual examina ion, which makes his a las an ideal resource o have in emergency depar men s and in he of ce. I provides a solid basis o pho ographic and descrip ive in orma ion o diagnose oculoplas ic

condi ions. Once hese condi ions are recognized, he ex describes o her es s ha may be needed and he di eren ial diagnoses ha should be considered. T e managemen op ions or hese condi ions are also described. Rober B. Penne, MD Editor

vii

Acknowledgmen s

S

pecial hank o my colleagues who provided assis ance: Edward Bedrossian, MD; Jurij Bilyk, MD; Richard Her le, MD; Ka e Lane, MD; and Mary A S e anyszyn, MD.

viii

Con en s Abou he Series vi Pre ace vii Acknowledgmen s viii

SECT IO N I: EYELIDS Ch a pt er 1 Benign Eyelid Lesions 2 Papilloma 2 Seborrheic Kera osis 4 Cu aneous Horn 6 Epidermal Inclusion Cys 8 Molluscum Con agiosum 10 Xan helasma 12 Syringoma 14 Apocrine Hydrocys oma 16 richoepi helioma 18 Nevi (Nevocellular Nevi) 20 Hemangioma o he Eyelid (Cherry Angioma)

22

Ch a pt er 2 Eyelid Inf ammation 24 Chalazion 24 Hordeolum 26 Floppy Eyelid Syndrome 28

Ch a pt er 3 Eyelid Neoplasms 30 Kera oacan homa 30 Ac inic Kera osis 32 Len igo Maligna 34 Basal Cell Carcinoma 36 Squamous Cell Carcinoma 40 Sebaceous Adenocarcinoma 42 Malignan Melanoma 44 Kaposi’s Sarcoma 46

Ch a pt er 4 Eyelid rauma 48 Marginal Eyelid Lacera ion 48 Canalicular Eyelid Lacera ion 50 Dog Bi es 52 Eyelid Burns 54

ix

x

CO NT ENTS

Ch a pt er 5 Eyelid Malpositions 56 En ropion 56 Acu e Spas ic En ropion 56 Involu ional En ropion 58 Cica ricial En ropion 60 Ec ropion 62 Involu ional Ec ropion 62 Paraly ic Ec ropion 64 Cica ricial Ec ropion 66 Mechanical Ec ropion 68 Symblepharon 70 richiasis 72 P osis 74 Congeni al Myogenic P osis 74 Acquired Myogenic P osis 76 Aponeuro ic P osis 78 Neurogenic P osis 80 T ird Nerve Palsy 80 Myas henia Gravis 82 Marcus Gunn Jaw-Winking Syndrome 84 Horner’s Syndrome 88 Mechanical P osis 90 rauma ic P osis 92 Pseudop osis 94 Brow P osis 96 Derma ochalasis 98 Blepharochalasis 100 Eyelid Re rac ion 102 Eyelid Dyskinesis 104 Benign Essen ial Blepharospasm 104 Hemi acial Spasm 106

Ch a pt er 6 Congenital Eyelid Anomalies 108 Blepharophimosis 108 Epican hus 110 Epiblepharon 112 Congeni al En ropion 114 Congeni al Coloboma 116 Congeni al Dis ichiasis 118 Ankyloblepharon 120

Ch a pt er 7 Miscellaneous Eyelid Conditions 122 Ocular Cica ricial Pemphigoid 122

CO NTENTS

SECT T IO N II: L LACRIMAL ACR R I M AL LA APPARAT PP PAR R AT T US S Ch a pt er 8 Lacrimal Obstructions 126 Congeni al Obs ruc ions 126 Congeni al Nasolacrimal Duc Obs ruc ion 126 Dacryocys ocele 128 Lacrimal Fis ula 129 Acquired Obs ruc ions 130 Acquired Nasolacrimal Duc Obs ruc ion 130 Canalicular Obs ruc ion 132

Ch a pt er 9 Lacrimal In ections 134 Dacryocys i is 134 Canaliculi is 137

Ch a pt er 10 Lacrimal Sac umors 140

SECT S ECT T IO N III: III I : T H E O R RBIT BIT T Ch a pt er 11 Orbital In ections 142 Orbi al Celluli is 142 Orbi al Abscess 146 Phycomycosis (Mucormycosis) Aspergillosis 152

150

Ch a pt er 12 Orbital Inf ammation 154 T yroid-Rela ed Oph halmopa hy 154 Idiopa hic Orbi al In amma ion (Orbi al Pseudo umor) Sarcoidosis 164 Wegener’s Granuloma osis 168

Ch a pt er 13 Congenital Orbital Anomalies 170 Microph halmos 170

Ch a pt er 14 Orbital Neoplasms 174 Congeni al Orbi al umors 174 Dermoid Cys s 174 Lipodermoids 178 Vascular Orbi al umors 180 Capillary Hemangiomas 180 Cavernous Hemangiomas 184 Lymphangiomas 188

160

xi

xii

CO NTENTS

Hemangiopericy oma 192 Orbi al Varices 196 Ar eriovenous Mal orma ions 200 Neural umors 204 Op ic Nerve Gliomas 204 Neuro bromas 208 Meningiomas 210 Schwannomas 218 Mesenchymal umors 222 Rhabdomyosarcoma 222 Fibrous His iocy oma 226 Lymphoproli era ive umors 228 Lymphoid Hyperplasia and Lymphomas 228 Plasmacy oma 232 His iocy ic Disorders 236 Lacrimal Gland umors 240 Epi helial umors o he Lacrimal Gland 240 Miscellaneous Orbi al umors 246 Secondary Orbi al umors 246 Me as a ic Orbi al umors 252

Ch a pt er 15 Orbital rauma 258 Orbi al Frac ures 258 Orbi al Floor Frac ure 258 Medial Wall Frac ure 262 Orbi al Roo Frac ure 266 Zygoma ic Frac ure 268 Miscellaneous rauma 272 Orbi al Hemorrhage 272 Orbi al Foreign Bodies 276 Mucocele 282

Index 285

CO LO R ATLAS & SYNO PSIS OF CLINICAL OPHTHALMOLOGY

W i l l s Ey e I n s t i t u t e

Oc u lo p la s tic s S ECON D EDITION

C H AP T ER

Benign Eyelid Lesions PAPILLOMA

A

papilloma is a common benign, of en asymp oma ic, skin lesion ha occurs mos commonly in he in er riginous areas (axillae, in ramammary, and groin) bu is also commonly seen on he neck and eyelids. Papillomas are of en numerous on he eyelids when presen , and he number ends o increase wi h age. Synonyms: skin ag, acrochordon.

Epidemiology and Etiology Age: More common in middle-aged and elderly people Gender: More common in emales E iology: Unknown History Mos commonly asymp oma ic bu may become ender af er rauma. Wi h ime, lesions may become crus ed or hemorrhagic. Examination

Lesions are sof ; skin-colored, an, or brown; round or oval, peduncula ed 2

papillomas ( Fig. 1-1A & B). T e lesion is of en cons ric ed a he base. Size ranges rom less han 1 mm o 10 mm. Special Considerations May grow or become more numerous during pregnancy More common in obese pa ien s Dif erential Diagnosis Peduncula ed seborrheic kera osis Dermal nevus Soli ary neuro broma Molluscum con agiosum Conjunc ival papillomas ( Fig. 1-1C) can appear on he eyelid margin bu have a di eren appearance and he base o he lesion is rom he conjunc ival sur ace. Treatment Excision by simply snipping he lesion a he base Prognosis Excellen . Pa ien s may develop o her papillomas wi h ime.

Papilloma

3

A

B

C FIGURE 1-1. Papilloma. A. Multiple small papillomas o the upper eyelid. B. Larger papilloma o the right lower eyelid. Conjunctival papilloma. C. Papillomatous lesions may grow rom the conjunctival sur ace and protrude onto the eyelid margin. T ese papillomas are f esh colored and more riable than cutaneous papillomas. Conjunctival papillomas can be associated with a viral origin.

4

1 BENIGN EYELID LESIO NS

SEBORRHEIC KERATOSIS

T

he seborrheic kera osis is one o he mos common benign epi helial umors. T ese lesions are heredi ary, are rarely seen be ore he age o 30 years, and will con inue o increase over a li e ime. Some pa ien s will only have a ew, and o hers can have hundreds over heir body. Epidemiology and Etiology Age: More common as pa ien s age. Rare be ore age 30 years. Gender: More common and more ex ensive in males E iology: Unknown Inheri ance: Probably au osomal dominan

Lesions vary in size rom 1 mm o 6 cm. Special Considerations Mos common on lower lids Dif erential Diagnosis Pigmen ed ac inic kera osis Verruca vulgaris Pigmen ed basal cell carcinoma Pathophysiology Epidermal lesion. Benign proli era ion o kera inocy es, melanocy es, and orma ion o horn cys s.

History Lesions are of en presen or mon hs o years and are of en asymp oma ic. T ey are mos common on he ace, runk, and upper ex remi ies.

Treatment Excision-ligh elec rocau ery or cryo herapy will permi he lesion o be easily rubbed or curet ed o . T e underlying base can hen be re rea ed wi h cau ery.

Examination Lesion s ar s as a a , ligh an lesion. Wi h ime, he lesion becomes more pigmen ed and will become eleva ed ( Fig. 1-2A). As hey age, he lesion’s sur ace becomes “war y” ( Fig. 1-2B).

Prognosis Excellen wi h rare recurrence Pa ien s will of en have many lesions and will develop addi ional lesions over ime.

Seborrheic Keratosis

5

A

B FIGURE 1-2. Seborrheic keratosis (A&B). Seborrheic keratosis are common lesions o the eyelids. T ey tend to get darker as they have been present longer, as seen in B.

6

1 BENIGN EYELID LESIO NS

CUTANEOUS HORN

C

u aneous horn is a clinically descrip ive erm or lesions wi h exuberan hyperkera osis. T e e iology o his hyperkera osis can be variable and biopsy o de ermine he cause is required.

Epidemiology and Etiology Age: Older adul s Gender: Equal in males and emales E iology: Hyperkera osis is associa ed wi h a varie y o underlying lesions History Lesion may grow slowly or rapidly. Examination Raised lesion, of en on a s alk, usually whi e in color. T e sur ace is hyperkera o ic ( Fig. 1-3).

Special Considerations Biopsy o hese lesions is required o rule ou malignan lesion a he base o he lesion such as basal cell carcinoma or squamous cell carcinoma. Dif erential Diagnosis T is is a descrip ive erm and no a pa hologic, diagnos ic erm. T e base o his lesion may be a seborrheic kera osis, verruca vulgaris, basal cell carcinoma, or squamous cell carcinoma. Laboratory Tests Pa hologic evalua ion Treatment Excisional biopsy wi h pa hologic evalua ion Prognosis Good

Cutaneous Horn

7

A

B FIGURE 1-3. Cutaneous horn. A cutaneous horn has a hard, rough sur ace that is white in color. A. T is lesion is less pointed, but some end in a point, giving them their name o cutaneous “horn.” B. Cutaneous horn o the lower eyelid.

8

1 BENIGN EYELID LESIO NS

EPIDERMAL INCLUSION CYST

C

ommon whi e o yellow cys seen around he eyes and elsewhere on he ace. Easily rea ed wi h excision.

Epidemiology and Etiology Age: Any Gender: Equal in males and emales E iology: Arises spon aneously rom he in undibulum o he hair ollicle or ollowing rauma ic implan a ion o epidermal issue in o he dermis. History May have his ory o rauma o he area Lesions usually grow slowly or a period o ime and hen remain s able. Examination Smoo h, round, eleva ed cys

T e underlying cys is whi e and can of en be visualized hrough he hin eyelid skin ( Fig. 1-4). Special Considerations T ese cys s may become secondarily in ec ed and cause a celluli is. Dif erential Diagnosis Molluscum con agiosum Chalazion Syringoma Treatment Excision; at emp should be made o ei her excise he en ire cys wall or i lef a he base, i should be des royed wi h cau ery. Prognosis Excellen . Recurrence is rare.

Epidermal Inclusion Cyst

9

A

B FIGURE 1-4. Inclusion cyst. A. Inclusion cyst o the le upper eyelid. B. A smaller cyst o the le lower lid. Patients with inclusion cysts on the eyelids o en seek treatment be ore they become very large.

10

1 BENIGN EYELID LESIO NS

MOLLUSCUM CONTAGIOSUM

M

olluscum con agiosum is a sel -limi ed viral in ec ion charac erized by skincolored papules ha are of en umbilica ed a he cen er. In immunocompromised individuals, his may no be sel -limi ed and can lead o large cosme ically dis guring lesions. I hese lesions are loca ed on he eyelid margin, hey may cause a ollicular conjunc ivi is. Epidemiology and Etiology Age: Children and young adul s Gender: More common in males han emales E iology: Viral lesions spread by skin- oskin con ac History Spon aneously occurring lesions Known con ac wi h o her person wi h lesions is no usual Examination Single or mul iple small 1- o 2-mm papules ( Fig. 1-5). Rarely, hese lesions can become larger. T ey are pearly whi e or skin colored wi h a cen ral kera in plug ha gives hem heir cen ral umbilica ion.

Special Considerations I hese lesions are loca ed on he eyelid margin, hey may cause a mild o severe, chronic ollicular conjunc ivi is. In immunocompromised pa ien s, his viral in ec ion may no be sel -limi ed and can lead o large, cosme ically dis guring lesions, especially on he ace. Dif erential Diagnosis Epidermal inclusion cys Syringoma Kera oacan homa Laboratory Tests Direc microscopy o he kera in plug wi h Giemsa s ain shows “molluscum bodies.” Treatment T ese lesions will regress spon aneously over ime excep in immunocompromised pa ien s. I removal is desired, small lesions can be rozen or he core can be rea ed wi h elec rodesicca ion. Curet age or direc excision is also e ec ive. Prognosis Good in heal hy people T e chance o in ec ing o her people is low when he lesions are presen bu in ec ed pa ien s should avoid skin- o-skin con ac .

Molluscum Contagiosum

11

A

B FIGURE 1-5. Molluscum contagiosum. A. T ere are three lesions on the upper eyelid. I the lesions are on the eyelid margin, the eye itsel may be injected with a ollicular conjunctivitis. T is patient also had similar lesions on her leg. B. Multiple lesions o the eyelid margin. T ere was a mild ollicular reaction in the in erior ornix. (Courtesy o Jurij Bilyk, MD.)

12

1 BENIGN EYELID LESIO NS

XANTHELASMA

X

an helasma are yellowish plaques ha occur medially on he upper or lower lids and are classic in appearance. T ey end o enlarge wi h ime and may or may no be associa ed wi h hyperlipidemia. Synonyms: xan homa.

Special Considerations I LDL is eleva ed in he lipid pro le, i is a sign o a amilial lipopro ein disorder. Dif erential Diagnosis I he xan helasmas are presen bila erally, no o her lesions look like his. Early, a xan helasma can look like an inclusion cys or syringoma.

Epidemiology and Etiology Age: More han 50 years o age. I younger, mus consider a amilial lipoproein disorder. Gender: Ei her E iology: May or may no be associa ed wi h hyperlipopro einemia

Laboratory Tests Labora ory evalua ion o lipid pro le

History T e lesions are no ed or mon hs o years wi h slow enlargemen .

Treatment Excision is mos common. Elec rodesicca ion, laser, and applica ion o richloroace ic acid are o her rea men s.

Examination Sof , yellow-orange plaques loca ed medially on he upper and/ or lower eyelids ( Fig. 1-6)

Pathophysiology Macrophages con aining drople s o lipids orm xan homa cells. T ese xan homa cells hen accumula e orming he xan helasma.

Prognosis Good, bu wi h ime addi ional deposi ion may occur and he lesions reappear.

Xanthelasma

13

A

B FIGURE 1-6. Xanthelasma. T ese lesions are in the classic area o the upper eyelids. T ey are still relatively small but with time, the lipid deposition will continue and they will enlarge. Less commonly, they can occur in a similar position on the lower eyelids.

14

1 BENIGN EYELID LESIO NS

SYRINGOMA

S

yringoma presen as mul iple lesions on he lower lids o women. T e onse is usually insidious. Pa ien s ypically presen wi h cosme ic concerns because o he numerous “bumps” on he lower eyelids. T e challenge can be excision o he large number o lesions presen wi hou causing scarring or an ec ropion.

Lesions occur commonly on he lower eyelids bu may occur elsewhere on he ace, axillae, umbilicus, upper ches , and vulva. Dif erential Diagnosis Very ew o her lesions look similar or presen wi h numerous lesions on he lower eyelids. A single lesion can look like an inclusion cys , basal cell carcinoma, or richoepi helioma.

Epidemiology and Etiology Age: Begins in puber y Gender: Occurs in women and may be amilial E iology: An adenoma o he in raepidermal eccrine duc s

Pathophysiology Benign adenoma o he in raepi helial eccrine duc s Pa hology shows many small duc s in he dermis wi h comma-like ails wi h he appearance o adpoles.

History Lesions no ed on he lower eyelids wi h insidious onse . Lesions may be presen elsewhere on he ace, axillae, umbilicus, upper ches , and vulva.

Treatment Pa ien s of en reques removal on a cosme ic basis. Removal is by elec rosurgery or direc excision.

Examination Lesions are 1 o 2 mm, skin colored or yellowish, and usually appear in mul iples ( Fig. 1-7).

Prognosis A large number on he ace can be di cul o remove. Addi ional lesions may grow af er excision.

Syringoma

15

FIGURE 1-7. Syringoma. Multiple lesions in the classic area o the lower eyelids. T ere can be just a ew lesions or even more than in this patient.

16

1 BENIGN EYELID LESIO NS

APO CRINE HYDRO CYSTOMA

A

pocrine hydrocys oma is a very common lesion arising along he eyelid margin. I is a clear, cys ic lesion ha ransillumina es, al hough he overlying skin may give i a bluish color. Epidemiology and Etiology Age: Adul s Gender: Equal E iology: Cys orma ion rom he glands o Moll along he eyelid margin History Cys no ed and may slowly enlarge Examination Cys ic lesion near or on he eyelid margin ( Fig. 1-8).

Lesions are ranslucen or bluish and ransillumina e. T ere may be mul iple lesions. Dif erential Diagnosis Cys ic basal cell carcinoma Eccrine hydrocys oma (re en ion cys o eccrine glands) Pathophysiology T is lesion is an adenoma o he secre ory cells o Moll and no a re en ion cys . Treatment Marsupializa ion o he cys may be adequa e or super icial lesions, bu deeper lesions require comple e cys wall excision. Prognosis Excellen . Rare recurrence af er excision.

Apocrine Hydrocystoma

17

A

B FIGURE 1-8. Apocrine hydrocystoma. A. T is lesion on the upper eyelid transilluminates with the slit beam. On excision there will be a gush o clear f uid. B. Multiple lesions. Lesions are o en smaller than these and are di cult to photograph.

18

1 BENIGN EYELID LESIO NS

TRICHOEPITHELIOMA

T

richoepi helioma is a benign leshcolored papule ha arises rom an immaure hair ollicle. I can occur on he eyelid margin bu more commonly elsewhere on he ace, scalp, neck, and upper runk. Epidemiology and Etiology Age: Firs appears a puber y Gender: More common in males E iology: Benign appendage umor wi h hair di eren ia ion History Lesions o he eyelid and orehead appear a puber y and can slowly increase in size and number.

Examination Small pink or skin-colored papules ha can increase in size and become qui e large ( Fig. 1-9). Special Considerations May be con used wi h a basal cell carcinoma, especially i i appears as a soli ary umor. Dif erential Diagnosis Epidermal inclusion cys Basal cell carcinoma Syringoma Treatment Excision wi h pa hologic evalua ion Prognosis Excellen

Trichoepithelioma

19

FIGURE 1-9. Trichoepithelioma. T ese pink or skin-colored lesions can occur on the skin or eyelid margin. T ey can enlarge and be con used with a basal cell carcinoma. (From Fitzpatrick B, Johnson R , Wol K, et al. Color Atlas and Synopsis of Clinical Dermatology, 4th ed. New York, McGraw-Hill, 2001.)

20

1 BENIGN EYELID LESIO NS

NEVI (NEVOCELLULAR NEVI)

N

evocellular nevi are small (less han 1 cm), circumscribed, acquired pigmen ed lesions ha are made up o melanocy ic nevus cells loca ed in he epidermis and dermis bu rarely deeper.

Epidemiology and Etiology Age: Appear in early childhood and reach a maximum size in young adul hood. T ese lesions gradually involu e and disappear by age 60 years. T e excep ion is he dermal nevus, which does no involu e. Gender: Equal E iology: Groups o melanocy ic nevus cells loca ed in he epidermis, dermis, or, rarely, in he subcu aneous issue. History Pigmen ed lesion ha is s able or involu ing Lesions are asymp oma ic. Examination Nevi can be grouped as ollows ( Fig. 1-10): Junctional nevi: Round or oval, a , or very sligh ly raised lesion, less han 1 cm in diame er. an or brown in color wi h smoo h regular borders. Compound nevi: Round, eleva ed, dome-shaped lesion wi h smoo h or papilloma ous sur ace. Dark brown in color bu

becomes mot led as he lesion evolves in o a dermal nevus; of en has hairs growing ou o he lesion. Dermal nevi: Round, dome-shaped, eleva ed nodule, skin-colored, an, or brown wi h elangiec asias. T ese do no disappear wi h age and may become more peduncula ed. Special Considerations Any enlarging lesions, hose changing color, or becoming irri a ed in any way af er age 20 years need o be biopsied o rule ou malignan change. Dif erential Diagnosis Seborrheic kera osis Malignan melanoma Derma o broma Basal cell carcinoma Laboratory Tests His ologic examina ion i biopsied Treatment Observa ion, unless he lesion changes color, i s borders become irregular, or he lesion begins o i ch, hur , or bleed. Any o hese are indica ions or excisional biopsy wi h his ologic evalua ion. Prognosis Rare chance o malignan rans orma ion

Nevi (Nevocellular Nevi)

21

A

B FIGURE 1-10. Nevi. A.T is small nevus o the lower eyelid is amelanotic except or a ew pigmented spots. Nevi on the eyelid margin will o en mold against the eyeball, as in this picture, but cause no discom ort or corneal changes. B. A split nevus where nevi cells were split congenitally as the eyelid ssure ormed. T is nevus is very dark and shows the variation that can occur in the color o these lesions.

22

1 BENIGN EYELID LESIO NS

HEMANGIOMA OF THE EYELID (CHERRY ANGIOMA)

H

emangiomas o he eyelid are raised, red, benign lesions ha appear on he eyelids in adul hood and can increase in size bu usually remain less han 3 o 5 mm. Epidemiology and Etiology Age: Adul hood Gender: Equal E iology: Unknown History Usually appear spon aneously and can increase in size over a shor ime. Pa ien s may have o her similar lesions elsewhere on heir body.

Examination Raised, brigh red, blood- lled lesions ha can occur anywhere on he body ( Fig. 1-11A) Lesions may be single or mul iple. Dif erential Diagnosis Pyogenic granuloma ( Fig. 1-11B) Melanoma Laboratory Tests Pa hologic evalua ion af er excision Treatment Excision usually or cosme ic reasons, less commonly o have he lesion evalua ed pa hologically. Prognosis Excellen

Hemangioma of the Eyelid (Cherry Angioma)

23

A

B FIGURE 1-11. Eyelid hemangioma. A. Very red, blood- lled lesion that may be just slightly raised or very elevated as in this picture. On excision, there is usually a small gush o blood, but these lesions usually do not bleed excessively. arely, these can bleed actively so the surgeon must be prepared. Pyogenic granuloma. B. A pyogenic granuloma can look similar to a hemangioma, but it usually is solid, not blood lled, and o en somewhat papillomatous.

C H AP T ER

Eyelid Inf amma ion CH ALAZION

A

ype o ocal inf amma ion o he eyelid; i is a common lesion o he eyelid. T e mos common cause is blockage o a meibomian gland o he eyelid. Chalazia can presen wi h an inf amed, ender, red eyelid or as a discre e non ender lump in he eyelid. Epidemiology and Etiology Age: Any Gender: Equal E iology: Focal inf amma ion o he eyelid resul ing rom he obs ruc ion o he meibomian glands History O en presen wi h acu e onse o ocal eyelid inf amma ion. T e inf amma ion will resolve bu may urn in o a chronic cys -like lesion. T e onse may be more insidious wi h appearance o he cys -like lesion wi h minimal inf amma ion. Examination In he acu e process, he eyelid may be di usely in lamed wi h pain ocally over 24

he involved area ( Fig. 2-1A). here may be poin ing over he blocked meibomian gland. As he inf amma ion resolves, he resul ing lesion is a rm mass in he arsal pla e wi h or wi hou residual inf amma ion ( Fig. 2-1B). Special Considerations Chronic, nonresolving chalazion needs o be biopsied o rule ou a carcinoma. Dif erential Diagnosis Sebaceous adenocarcinoma Squamous cell carcinoma Basal cell carcinoma Pathophysiology Blockage o he eyelid glands resul s in release o he gland con en s in o he arsus and eyelid, resul ing in an inf amma ory process. he in lamma ory process is hen walled o wi h ime, resul ing in he cys -like lesion. T e exac role o bac eria in his process is unclear.

Chalazion

Treatment When in he inf amma ory phase, ini ial rea men is warm compresses and s eroid an ibio ic drops or oin men . As he lesion becomes cys ic, rea men is hen excision via a conjunc iva incision. Injec ion o s eroid in o he lesion may also be e ec ive. S eroid injec ions need

25

o be used cau iously in pa ien s wi h darkly pigmen ed skin, as hey can cause depigmen a ion. Prognosis Good T ese lesions can be mul iple and are rarely resis an o rea men .

A

B FIGURE 2-1. Chalazion. A. A f rm, ormed lump o the le lower eyelid. T ere is still some in ammation o the chalazion. T e eye is red rom blepharoconjunctivitis, which o en is part o a chalazion. Most o the time, the eye is white and quiet. B. A chronic chalazion o the le upper eyelid with some crusting over the chalazion rom external drainage.

26

2 EYELID INFLAMMATIO N

HORDEOLUM

A

n acu e in ec ion o he glands o Zeis (ex ernal hordeolum) or meibomian glands (in ernal hordeolum). I presen s as a red, inf amed, ender eyelid. In prac ice, he erms chalazion, hordeolum, and stye are o en used in erchangeably (and incorrec ly). Synonym: s ye. Epidemiology and Etiology Age: Any Gender: Equal E iology: Acu e bac erial in ec ion o he glands o Zeis or he meibomian glands History Sudden onse o ocal inf amma ion o he eyelid cen ered around a gland o he eyelid

Examination Red, swollen, ender eyelid, o en wi h a ocal area o in ec ion around a gland o he eyelid ( Fig. 2-2) Dif erential Diagnosis Presep al celluli is Eyelid abscess Pathophysiology Eyelid gland becomes in ec ed probably associa ed wi h blockage o he gland. Treatment Warm compresses and opical s eroid/ an ibio ic drops or oin men Rarely, his can evolve in o an abscess, which needs drainage, or a celluli is ha requires sys emic an ibio ics. Prognosis Excellen

Hordeolum

27

A

B FIGURE 2-2. Hordeolum. A. Acute in ammation o the le lower eyelid caused by blockage and in ection/ in ammation o a meibomian gland. T is lesion may resolve as the acute in ammation resolves or evolve into a chalazion. B. Blockage with in ection/ in ammation o the glands o Zeis is involved in this eyelid lesion. T is lesion is on the external eyelid in the area o the eyelash ollicles. Hordeola usually resolve without sequelae but can sometimes become chronic and take many weeks to resolve.

28

2 EYELID INFLAMMATIO N

FLOPPY EYELID SYNDROME

F

loppy eyelid syndrome is seen in obese pa ien s, many o whom have sleep apnea. T e eyelids become very loose and f oppy ei her as a primary process or secondary o chronic rubbing o he eyelids a nigh . T is syndrome mus be considered as a cause or chronic, papillary conjunc ivi is.

Epidemiology and Etiology Age: Adul s Gender: Males more commonly a ec ed E iology: Unknown. T e eyelid laxi y and loss o s ruc ure may be rela ed o chronic mechanical eyelid rubbing or may be due o some inna e abnormali y o he pa ien ’s eyelids. History Pa ien presen s wi h chronic papillary conjunc ivi is ha is usually bila eral and may give he his ory o his eyelids spon aneously ever ing a nigh . T e pa ien may complain o chronic nonspeci c irri a ion. T e symp oms are o en worse on he side he pa ien sleeps on. Examination Eyelids are f accid and easily ever ed ( Fig. 2-3).

T ere is chronic papillary conjunc ivi is wi h a kera i is o en wi h di use super cial punc a e kera i is. ypically, he palpebral conjunc iva has a velve y appearance. T ere is a high incidence o obesi y in hese pa ien s. Special Considerations T ere is a signi can incidence o sleep apnea in pa ien s wi h f oppy eyelid syndrome. All pa ien s need o have sleep s udies. Dif erential Diagnosis O her orms o conjunc ivi is in a pa ien wi h eyelid laxi y Pathophysiology T ere is loss o elas in bers in he arsus, bu he cause remains specula ive. Treatment Pa ching or a shield over he eye can be at emp ed bu is usually no help ul in he long erm. Horizon al eyelid igh ening is usually required. Prognosis T e laxi y will recur wi h ime. Horizon al igh ening will relieve sympoms or a ime.

Floppy Eyelid Syndrome

29

A

B

C FIGURE 2-3. Floppy eyelid syndrome. A. Patient with mild ptosis but complains o chronic irritation o the eyes. His eyes are white but he has moderate corneal superf cial punctate keratitis. B. Upper eyelids are easily everted and the undersides o the eyelids are red with a di use papillary reaction. C. T e eyelids are very loose and, once everted, will o en remain everted even with blinking.

C H AP T ER

Eyelid Neoplasms KERATOACANTHOMA

K

eratoacanthoma presents as an isolated lesion on the ace with a very unique appearance. he lesion is dome shaped with a central keratin- illed crater. It grows rapidly over weeks and may undergo spontaneous regression over months. Once considered benign, most pathologists now consider this a low-grade squamous carcinoma. Epidemiology and Etiology Age: Most of en older than 50 years o age; rare younger than 20 Gender: More common in males than emales by a ratio o 2 to 1 Etiology: Unknown; ultraviolet radiation and chemical carcinogens may have a causative role. History Rapid onset o growth over a ew weeks T e lesion is of en asymptomatic except or cosmetic changes. T ere may be occasional tenderness. 30

Examination Single, dome-shaped nodule with a central keratotic plug T e lesion is rm and is slightly red to light brown in color ( Fig. 3-1). Special Considerations Once considered a benign lesion, there may still be some con usion in the literature about whether this is a squamous carcinoma. T e lesion must be treated as a low-grade squamous carcinoma. Dif erential Diagnosis Basal cell carcinoma Hyperkeratotic actinic keratosis Squamous carcinoma Laboratory Tests Histopathology o the excised lesion Should be excised with rozen section guidance or with Mohs surgery Treatment Excision with pathologic evaluation T ese lesions will sometimes spontaneously regress over a ew months to a year.

Keratoacanthoma

T e need to rule out a squamous cell carcinoma and the cosmetic appearance should lead to biopsy and excision be ore spontaneous regression, especially around the eyelids.

31

Prognosis Good Depending on the size o the lesion, reconstruction o the de ect may leave some eyelid changes.

A

B FIGURE 3-1. Keratoacanthoma. A. Lesion o the lef upper eyelid that grew over 2 to 3 weeks. It was excised without recurrence. B. Large lesion o the lef lower eyelid in a 40-year-old patient. T e appearance could be that o a squamous cell carcinoma; however, the history o growth over 4 weeks and the patient’s younger age point to a keratoacanthoma. T is lesion was excised without recurrence.

32

3 EYELID NEO PLASMS

ACTINIC KERATOSIS

T

hese lesions may be single or multiple on chronically sun-exposed skin. T ey appear as dry, rough, scaly lesions that are stable but can rarely disappear spontaneously. Synonym: solar keratosis. Epidemiology and Etiology Age: Older than age 40; rare younger than 30 years Gender: Higher incidence in males Etiology: Sun exposure over time in a airskinned white population results in actinic keratosis. History Extensive sun exposure in youth Lesions present or months. Examination Rough, slightly elevated, skin-colored or light brown lesions with hyperkeratotic scale ( Fig. 3-2) Special Considerations It is estimated that one squamous cell carcinoma will develop per 1000 actinic keratoses. Dif erential Diagnosis Squamous cell carcinoma Discoid lupus

Laboratory Tests Pathologic evaluation i biopsied Pathophysiology Repeated solar exposure results in damage to the keratinocytes by the cumulative e ects o ultraviolet radiation. Treatment Prevention through early and li elong use o sunscreen Excise nodular lesions and submit or pathologic evaluation. Most at lesions respond to liquid nitrogen or topical application o 5% 5- uorouracil cream over a ew days to weeks. opical imiquimod cream has also been approved or treatment o actinic keratosis. T ese three treatments (liquid nitrogen, 5- uorouracil, and imiquimod) must be used with caution around the eye and should be avoided in lesions at or near the lid margin. Prognosis Some actinic keratoses may disappear spontaneously but others remain or years unless treated. Incidence o squamous cell carcinoma developing in these lesions is unknown but has been estimated to be one squamous cell carcinoma in every 1000 actinic keratoses.

Actinic Keratosis

33

A

B FIGURE 3-2. Actinic keratosis. A. Multiple actinic keratoses on the cheek and brow with signs o chronic sun damage. B. Lesion involving the lower eyelid. (Courtesy o Jurij Bilyk, MD.)

34

3 EYELID NEO PLASMS

LENTIGO MALIGNA

L

entigo maligna is a at intraepidermal neoplasm and the precursor lesion o lentigo maligna melanoma. T e lesion has striking variations o brown and black ( Fig. 3-3), of en described as a “stain.” Epidemiology and Etiology Age: Median age is 65 years Gender: Equal incidence in males and emales Etiology: Sun exposure is a de nite actor.

Of en appears like a dark “stain” on the skin Special Considerations T is is a premalignant lesion and should be excised because o the chance o development into a lentigo maligna melanoma. Dif erential Diagnosis Seborrheic keratosis Actinic keratosis Malignant melanoma Laboratory Tests Histopathologic evaluation

History History is usually not help ul, as exact onset o lesion is unclear.

Treatment Excision with margins sent or pathologic evaluation

Examination Flat, dark brown or black color, sharply de ned edges

Prognosis Excellent i excised be ore developing into a melanoma

Lentigo Maligna

35

A

B FIGURE 3-3. Lentigo maligna. A. A large macule with irregular borders and di erent shades o brown. (From Fitzpatrick B et al. Color Atlas & Synopsis of Clinical Dermatology, 4th ed. New York, McGraw-Hill, 2001.) B. Recurrent lentigo maligna o lef brow.

36

3 EYELID NEO PLASMS

BASAL CELL CARCINOMA

B

asal cell carcinoma is the most common type o skin cancer. It is locally invasive and aggressive but has very limited capacity to metastasize. I neglected, it can invade the orbit, especially i located in the medial canthal area. Most commonly, it occurs on the lower eyelid and is treated by complete surgical excision.

Epidemiology and Etiology Age: More than 40 years o age. Rare cases do occur in the 20s and 30s Gender: Males more than emales Etiology: Sun exposure and air skin with poor ability to tan are risk actors. reatment with x-ray ( or acne) increases the risk. Incidence: 500 to 1000 per 100,000 people History Slowly enlarging lesions in sun-exposed areas T e lesions may be associated with bleeding. Examination Round or oval, rm lesions with depressed center T e lesions are pink or red with ne thread-like telangiectasia. T e center may be ulcerated. Basal cell carcinoma may also appear scar-like or be cystic ( Fig. 3-4A–D). Special Considerations Aggressive treatment o basal cell carcinoma o the medial canthal area is indicated because o the risk o orbital extension rom the medial canthal area.

Basal cell carcinomas almost never metastasize. Sclerosing basal cell carcinomas have poorly de ned margins and may recur. Basal cell nevus syndrome is an autosomal dominate syndrome in which patients develop multiple basal cells at a very young age ( Fig. 3-4E and F). Dif erential Diagnosis Squamous cell carcinoma richoepithelioma Laboratory Tests Lesions are sent or pathologic evaluation. Treatment Complete surgical excision with pathologic evaluation Frozen sections or Mohs surgery is needed to assure complete excision. Reconstruction o the de ect is then completed at the same time. reatment with radiation should not be used or lesions around the eye unless the patient is not a surgical candidate. Imiquimod cream can be used as a nonsurgical option i the lesion is not close to the eyelid margin. Prognosis Good when promptly and completely excised Neglected cases can invade the orbit and brain and have the potential, in rare cases, to be atal.

Basal Cell Carcinoma

37

A

B FIGURE 3-4. Basal cell carcinoma. A. Classic appearance o a basal cell carcinoma. T is lesion does not involve the eyelid margin, but large lesions such as this one are a challenge or reconstruction because o the chance o lower eyelid ectropion. B. Notching o the eyelid margin is a sign o an eyelid neoplasm. T is basal cell carcinoma has caused a notch and demonstrates the smooth pearly borders o a basal cell carcinoma. ( continued)

38

3 EYELID NEO PLASMS

C

D FIGURE 3-4. (Continued) Basal cell carcinoma. C. Basal cell carcinoma may present as pigmented lesions, especially in patients with darker pigmented skin. Note the pearly edges on the in erior part o the lesion. D. A cystic lesion can be a basal cell carcinoma. T is lesion is larger than most hydrocystomas and has a slightly violaceous hue. T is cystic basal cell carcinoma was lled with a thick clear gel-like material, which is classic. (continued)

Basal Cell Carcinoma

39

E

F FIGURE 3-4. (Continued) Basal cell carcinoma. E. Basal cell nevus syndrome with many basal cell carcinomas all over the ace occurring at a young age. F. Pits o the palms o the hands that are of en seen in basal cell nevus syndrome.

40

3 EYELID NEO PLASMS

SQUAMOUS CELL CARCINOMA

S

quamous cell carcinoma is a malignant tumor o epithelial keratinocytes. It is of en the result o exogenous carcinogens (ultraviolet exposure, exposure to ionizing radiation, arsenic). T ese lesions are much less common than basal cell carcinoma on the eyelids and are usually success ully treated with excision. Epidemiology and Etiology Age: Older than age 55 years Gender: Males more commonly involved than emales Etiology: Sun exposure and air skin with poor ability to tan are risk actors. reatment with x-ray ( or acne) increases the risk. Incidence: 12 per 100,000 white males; 7 per 100,000 white emales; 1 per 100,000 blacks History Persistent keratotic lesion or plaque that does not resolve af er 1 month must be considered a potential carcinoma, especially in sunexposed areas. Examination wo types o lesions: Di erentiated lesions are keratinized, rm, and hard.

Undi erentiated lesions are eshy, granulomatous, and sof . T ese can present rom very small to large. T ey may be crusted with bleeding or smooth ( Fig. 3-5). Dif erential Diagnosis Actinic keratosis Basal cell carcinoma Keratoacanthoma Laboratory Tests Pathologic evaluation Treatment Complete surgical excision with controlled margins Frozen sections and Mohs surgery are both appropriate options. Imiquimod cream can be used as a nonsurgical option i not close to the eyelid margin. Prognosis Excellent unless the lesion is neglected Squamous cell carcinoma rarely spreads via lymphatics, blood vessels, or along nerves.

Squamous Cell Carcinoma

41

A

B FIGURE 3-5. Squamous cell carcinoma. A.T is is a very large squamous cell carcinoma that was neglected. It now in ltrates the entire lower eyelid. Note the crusting on the lesion, which is usually present with squamous cell carcinoma and is less common with basal cell carcinoma. B. Smaller lesion o the lower eyelid that shows crusting and an irregular, erosive central area.

42

3 EYELID NEO PLASMS

SEBACEOUS ADENO CARCINOMA

S

ebaceous adenocarcinoma is a highly malignant and potentially atal tumor that arises rom the sebaceous glands o the eyelid. Early, this tumor can be di cult to recognize and once it grows, it is di cult to contain, as it can have skip areas. Early recognition and aggressive excision are the keys to success ul treatment. Epidemiology and Etiology Age: Usually greater than 50 years o age Gender: More common in emales than males Etiology: Arises rom the meibomian glands, glands o Zeis, or rom the sebaceous glands o the caruncle, eyebrow, or acial skin. History Of en starts as a chronic blepharitis or nonresolving chalazion Patients may have a chronic red, irritated eye or months to years. Examination Multiple potential presentations: Nodular lesion simulating a chalazion Unilateral chronic blepharitis Cellular membrane growing over the conjunctiva Destructive, of en ulcerated, lesion on the eyelid margin ( Fig. 3-6) Occurrence in the upper eyelid is twice as common as the lower eyelid.

Special Considerations T is lesion is the great masquerader. Delay in diagnosis as a carcinoma and subsequent growth o the lesion add to the poor prognosis or this tumor. Dif erential Diagnosis Basal cell carcinoma Squamous cell carcinoma Chronic blepharitis Chronic chalazion Pathology It is important to alert your pathologist i this sebaceous adenocarcinoma is suspected. Special stains/ studies are per ormed on lesions that are suspected to be sebaceous adenocarcinoma. Without these stains, the lesion may be misdiagnosed. Treatment Diagnosing the lesion is of en the biggest challenge. Biopsy o any suspicious lesion is the key. Once diagnosed, complete excision with wide, controlled margins is the treatment o choice. T ere can be skip areas, so care ul ollowup or recurrence is needed. Prognosis T is is a potentially lethal tumor that must be treated aggressively.

Sebaceous Adenocarcinoma

43

A

B FIGURE 3-6. Sebaceous adenocarcinoma. A.T e eyelid margin is red and in amed with notching. B. When the eyelid is everted, there is an in ltrative lesion o the tarsal conjunctiva.

44

3 EYELID NEO PLASMS

MALIGNANT MELANOMA

M

alignant melanoma is a rare but very dangerous malignant lesion o the eyelids. Sun exposure as a child is an etiologic actor. Despite aggressive surgical excision, this can still be a atal tumor.

Dif erential Diagnosis Nevus Pigmented basal cell carcinoma Laboratory Tests Specimens are sent or pathologic evaluation.

History Pigmented lesion with recent growth or change in appearance

Treatment Complete excision with aggressive, controlled surgical margins T e deeper the lesion, the wider the margins required. Sentinel lymph node biopsy is usually required. Melanomas are best excised via a team approach at centers experienced with melanoma treatment.

Examination Pigmented lesion with irregular pigment deposition, irregular margins, or just increase in size ( Fig. 3-7) T ere may be ulceration and bleeding.

Prognosis Dependent on the depth o the tumor Eight-year survival rate is 33% to 93%, depending on depth o melanoma invasion ( Table 3-1).

Epidemiology and Etiology Age: T ird decade and beyond Gender: Equal between males and emales Etiology: Sun exposure and genetic predisposition

TABLE 3-1. Survival in Relation to umor Depth Depth (mm)

8-Year Survival (%)

3.60

33.3

Malignant Melanoma

45

A

B FIGURE 3-7. Malignant melanoma. A. Lesion o the right eyebrow that has grown over a ew months. T e lesion has irregular areas o lighter and darker pigmentation. B. Malignant melanoma o the lower eyelid.

46

3 EYELID NEO PLASMS

KAPOSI’S SARCOMA

K

aposi’s sarcoma is a vascular neoplasia that can involve multiple systems. It is a rare lesion o the eyelids but when present, is usually associated with a compromised immune system, most commonly HIV disease. Epidemiology and Etiology Age: Any Gender: More common in males Etiology: Vascular neoplasia is of en associated with immune compromise in the United States.

History Rapid growth o lesion may occur. Patients most commonly are HIV-positive, although other orms o immune compromise may predispose patients to these lesions. Examination Elevated dermal lesions that are red or purple ( Fig. 3-8)

Dif erential Diagnosis Pyogenic granuloma Chalazion Hemangioma Melanocytic nevus Laboratory Tests Pathologic evaluation i biopsied Evaluation o immune system i indicated Treatment Excision with pathologic evaluation Cryotherapy or intralesional chemotherapeutic agents may be used or local control o the lesions. Radiation treatment or some large lesions Prognosis Patients who develop lesions associated with HIV of en have a short survival and die rom advancement o the HIV disease. Patients with primary Kaposi’s sarcoma may survive or years.

Kaposi’s Sarcoma

A

B FIGURE 3-8. Kaposi’s sarcoma. Lesion o the lower lid in a patient with AIDS.

47

C H AP T ER

Eyelid rauma MARGINAL EYELID LACERATION

M

arginal eyelid lacera ions are mos commonly associa ed wi h rauma o he en ire orbi al area, and of en here are o her associa ed injuries. T e ex en o laceraion can vary grea ly. Promp , me iculous closure is he rea men o choice. Epidemiology and Etiology Age: Any age. Second hrough our h decades mos common. Gender: Males are more commonly a ec ed. E iology: Blun rauma (e.g., s ), direc cu (e.g., glass, kni e), or dog bi e mos commonly History rauma his ory is variable rom minor o major injuries. I is impor an o de ermine he cause o he rauma o know whe her o suspec oreign bodies.

48

he amoun o orce causing he injury will help de ermine he likelihood o more signi ican injuries o he orbi and globe. Examination Mus evalua e globe and orbi or injuries. Evalua e he ex en o he injury o he eyelid and be sure ha he lacrimal sys em is no injured ( Fig. 4-1). C scanning may be required i o her injuries or oreign bodies are suspec ed. Special Considerations Dog bi es (also ca s and humans) require copious irriga ion o he wound and special care because o he grea er risk o in ec ion. e anus immuniza ion mus be up o da e. Treatment Me iculous closure o he wound wi hin 24 o 48 hours

Marginal Eyelid Laceration

Surgery can be done in he o ce or emergency room set ing unless he lacera ions are complex or in children, where an opera ing room set ing wi h general anes hesia is required.

49

Prognosis Good. T e more complex he wound, he grea er he chance o scarring, which may hen require secondary repair a a la er da e.

FIGURE 4-1. Marginal eyelid laceration. Cen ral eyelid lacera ion rom an umbrella ca ching under he eyelid. Isola ed marginal lacera ions wi hou canalicular involvemen are more likely due o some objec direc ly cut ing he eyelid. Canalicular lacera ions are more of en because o earing and s re ching, as he medial lid is he weakes area and he rs o ear.

50

4 EYELID TRAUMA

CANALICULAR EYELID LACERATION

Probing o he canalicular sys em may be necessary i a lacera ion is suspec ed ( Fig. 4-2).

T

Special Considerations T e ar her he lacera ion is medially rom he lacrimal punc a, he more di cul i is o nd he dis al cu end.

he medial eyelid is he weakes area o he eyelid, so any horizon al rac ion on he eyelid is more likely o resul in damage o he medial eyelid and he canaliculus. Eyelid rauma requires care ul inspec ion o he medial can hal area o recognize he lacera ed canaliculus. Repair wi h silicone in ubaion is he rea men o choice.

Epidemiology and Etiology Age: Any age. Second hrough our h decades mos common. Gender: Males are more commonly a ec ed. E iology: Usually a earing injury, as he medial eyelid is he weakes area o he eyelid. History rauma his ory is variable, including blun orce, dog bi es, and, rarely, sharp objec s. Examination Evalua e eye and orbi or injuries. Any cu medial o he lacrimal punc a mus be evalua ed or a canalicular lacera ion.

Treatment Surgical repair wi h anas omosis o he canalicular ends and in uba ion o he lacrimal sys em Loca ing he dis al end o he canaliculus may be di cul and of en requires loupes or an opera ing microscope. Depending on he severi y o he injury and he pa ien ’s coopera ion, surgical repair is usually per ormed in he opera ing room set ing wi h local or general anes hesia. T e ubing is lef in he lacrimal sys em or 6 weeks o 6 mon hs, depending on severi y and he individual prac i ioner. Prognosis Good. Even he injuries ha resul in a scarred canaliculus usually do well, as mos pa ien s do well wi h one unc ioning canaliculus.

Canalicular Eyelid Laceration

51

A

B FIGURE 4-2. Canalicular laceration. A. Any cu or ear medial o he punc um, no mat er how super cial i appears, needs o be explored or involvemen o he canaliculus. T is lacera ion involved bo h he upper and lower canaliculi. B. T e punc um and cu canaliculus can be seen a he medial edge o he cu eyelid. T is eyelid was nearly comple ely avulsed.

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4 EYELID TRAUMA

DO G BITES

D

og bi es are highly variable in heir ex en bu generally do no involve injury o he globe i sel . Promp repair wi h copious irrigaion of en gives airly good resul s, depending on he ex en o he original injury.

Epidemiology and Etiology Age: Mos commonly children; adul s less common. Gender: Equal E iology: In children, he dog may be rying o bi e he child on he nose o show domina ion, which is wha happens be ween dogs, and usually does no represen an at ack. T e eyelid lacera ion is he resul o he dog’s canine oo h ca ching he eyelid and earing he medial lid ra her han a rue bi e o he eyelid. History Of en, he child knows he dog and here is a single bi e and no a vicious at ack. Examination Evalua ion o he eye and orbi or o her injuries

T ere is mos commonly a single bi e wi h mul iple areas o injury. T ere may be punc ure wounds or larger ears and gashes ( Fig. 4-3). Special Considerations T e bi e mus be repor ed o he heal h depar men or ollow up o be sure he dog is properly vaccina ed or rabies. e anus immuniza ion mus be up o da e. Treatment T e eyelid lacera ion mus be rea ed as ou lined previously. T e risk o in ec ion in animal and human bi es is high because o he bac eria in he mou h. Copious irriga ion o he wounds is he only rea men o proven bene o decrease he risk o in ec ion. T e use o broad-spec rum, sys emic an ibio ics has no been proven o lessen he chance o in ec ion bu should be considered. Prognosis Good. T e more severe he injury, he grea er he risk o pos opera ive de ormi y.

Dog Bites

53

FIGURE 4-3. Dog bite with eyelid lacerations. Dog bi es are usually a single bi e, bu he amoun o damage can be highly variable rom mild o severe. T e eyelid damage is usually rela ed o earing as he dog pulls away and he ee h ge caugh on he eyelid. Bo h he medial and la eral can hi are orn and mul iple punc ure wounds are seen.

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4 EYELID TRAUMA

EYELID BURNS

E

yelid burns are usually associa ed wi h signi can burns o he res o he ace and body unless hey are elec rical or chemical. All burns ake days o weeks or he ull issue dea h and necrosis o mani es . Recons ruc ion can be very di cul because o poor vasculariza ion.

Epidemiology and Etiology Age: Any Gender: Males more commonly a ec ed E iology: Burns ha involve he eyelids are usually associa ed wi h burns over a large percen age o he body.

T e concern is o pro ec he cornea wi h lubrica ion. Wi h ime, he eyelids will scar, resul ing in poor closure and more corneal exposure ( Fig. 4-4). Treatment An ibio ic oin men and copious corneal lubrica ion Sys emic an ibio ics are usually par o he sys emic care. As he burns heal, cica ricial changes become more prominen and he use o skin graf s is required.

History Generally associa ed wi h o her acial burns unless he e iology is elec rical or chemical.

Prognosis Dependen on he severi y o he burns

Examination Burns o he eyelid vary in dep h and severi y.

Severe burns may require mul iple surgeries and skin graf s o pro ec he cornea.

Eyelid Burns

55

A

B FIGURE 4-4. Eyelid burn. A. Elec rical burn wi h necrosis o he upper eyelid and underlying scleral necrosis. Elec rical burns can ake weeks or he o al amoun o issue necrosis o become apparen . B. Mol en lead was splashed on o he eyelid and in o he eye. Wi h hermal burns, he ex en o damage is eviden more quickly. No e he rela ive lack o vasculariza ion along he lower eyelid margin rom he burn. T ere was par ial necrosis and loss o par o he eyelid margin over ime.

C H AP T ER

Eyelid Malposi ions ENTROPION ACUTE SPASTIC ENT OPION

A

cu e spas ic en ropion is he resul o eyelid swelling along wi h orbicularis spasm ha resul s in a emporary in- urning o he eyelid. A cycle o corneal irri a ion rom he en ropion, causing more eyelid spasm, causing more irri a ion, mus be broken so he eyelid can re urn o normal. Some o hese pa ien s will have underlying involu ional changes (laxi y) ha may resul in a recurren en ropion. Epidemiology and Etiology Age: More common in older pa ien popula ion Gender: Equal occurrence in males and emales E iology: Ocular irri a ion or in ammaion causes con inued orced blinking and closure o he eye. T is will lead o in- urning o he lower eyelid in eyelids ha have involu ional changes predisposing hem o en ropion (see “Involu ional En ropion”).

56

History ecen surgery on he eye or recen onse o ocular irri a ion Examination Lower eyelid en ropion ( Fig. 5-1) wi h associa ed involu ional ac ors such as horizon al laxi y and orbicularis override. In addi ion, here is a separa e iden if able irri an o he eye. his irri an may be kera i is, oreign body, su ure, or jus in lamma ion pos opera ively. Dif erential Diagnosis Involu ional en ropion Cica ricial en ropion Pathophysiology Involu ional changes o he eyelid allow he orced closure o he eyelid orbicularis muscle o override he arsus and drive he eyelid margin inward oward he eye.

Entropion

Treatment rea men o he underlying ocular irri aion or in amma ion will resolve some cases. T is involves rea ing he ocular irri a ion and s abilizing he eyelid o hal he addiional irri a ion he eyelid is causing. S abilizing he eyelid may involve aping he eyelid ou or Quicker su ures.

57

Some cases will hen resolve; o hers will become an involu ional en ropion and need more ex ensive surgery. Prognosis Excellen . ecurrence in pa ien s wi h signif can involu ional ac ors o he eyelid may develop an involu ional en ropion a a la er ime.

FIGURE 5-1. Acute spastic entropion. Pa ien wi h a corneal abrasion. Con inued irri a ion and blinking leads o an en ropion.

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5 EYELID MALPO SITIO NS

INVOLUTIONAL ENT OPION

E

yelid laxi y bo h horizon ally and ver ically predisposes o he ins abili y o he lower eyelid. T e addi ional ac or required is he abili y o he pa ien ’s orbicularis muscle o override he arsus and drive he eyelid inward. Pa ien s presen wi h red, irri a ed eyes rom he eyelid margin and he eyelashes in con ac wi h he eye i sel .

Epidemiology and Etiology Age: More common in older pa ien popula ion Gender: Equal occurrence in males and emales E iology: Horizon al laxi y and orbicularis override resul in inversion o he eyelid. History Acu e onse o eye irri a ion. T is irri aion is some imes in ermit en in na ure and becomes more cons an . Examination Inver ed lower eyelid wi h in erior corneal superf cial punc a e kera i is (SPK) or corneal abrasion ( Fig. 5-2) En ropion is usually associa ed wi h horizon al eyelid laxi y.

Orbicularis muscle override is o en no ed as ullness over he arsal pla e when he lid is en ropic. T e en ropion can be in ermit en and no always presen on examina ion. Placing opical anes he ic drops in he eye, having he pa ien close he eyes orce ully, and look downward will usually bring ou he en ropion. Dif erential Diagnosis Cica ricial en ropion Acu e spas ic en ropion Pathophysiology Aging o eyelid issues resul s in laxi y and s re ching o suppor ing s ruc ures. Treatment Surgical correc ion is based on correc ing he ac ors con ribu ing o he en ropion, usually horizon al shor ening o he eyelid and igh ening he eyelid re rac ors in any o mul iple ways. Prognosis Excellen . T ere is a 5% o 10% chance o recurrence over 5 o 10 years.

Entropion

59

FIGURE 5-2. Involutional entropion. Lef lower eyelid en ropion wi h involu ional changes. T e rolled in orbicularis muscle can be seen driving he eyelid margin inward on he lef .

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5 EYELID MALPO SITIO NS

CICAT ICIAL ENT OPION

C

ica ricial en ropion is caused by conjuncival scarring pulling he eyelid inward. Generally, rea men is surgical, bu def ning and rea ing he cause o he conjunc ival scarring mus be done f rs or mos cases will recur. Occurs in he upper or lower eyelid.

Epidemiology and Etiology Age: Any age Gender: Equal occurrence in males and emales E iology: Scar issue on he conjunc ival sur aces resul s in shor ening o he pos erior lamella, physically pulling he eyelid inward. Fac ors include: Surgery Conjunc ival scarring diseases (e.g., ocular cica ricial pemphigoid, S evens-Johnson syndrome, rachoma) rauma Conjunc ival burns (e.g., chemical) An iglaucoma drops History Chronic low-grade in amma ion over mon hs o years resul s in he en ropion ha hen causes more irri a ion. T e o her scenario is a his ory o rauma or surgery resul ing in an en ropion and associa ed irri a ion. Examination Care ul evalua ion o he conjunc iva or signs o scarring causing inversion o he eyelid.

T is may include evalua ion o he o her eyelids o de ermine i he en ropion is isola ed or involving all our eyelids, which may help de ermine he e iology ( Fig. 5-3). Special Considerations Mus de ermine he e iology o he conjunc ival scarring be ore rea ing. Any progressive disease mus be quie ed be ore surgery can be done on he eyelids. Dif erential Diagnosis Acu e spas ic en ropion Involu ional en ropion Laboratory Tests Conjunc ival biopsy wi h immuno uorescence es ing i ocular cica ricial pemphigoid is suspec ed. Treatment De ermine he e iology o he conjunc ival scarring. Quie any ac ive disease. Surgical correc ion o he en ropion wi h a marginal ro a ion or buccal mucosal gra is hen he rea men o choice. Prognosis Variable depending on he e iology En ropion secondary o rauma and surgery usually do very well. Progressive disease processes, such as ocular cica ricial pemphigoid, can make i much more di cul o preven recurrence o he en ropion.

Entropion

61

A

B FIGURE 5-3. Cicatricial entropion. Ex ernally (A), i is di cul o di eren ia e his cica ricial en ropion rom an involu ional en ropion un il he eyelid is ever ed (B), and he cica ricial changes are no ed pulling he eyelid inward.

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5 EYELID MALPO SITIO NS

ECTROPION INVOLUTIONAL ECT OPION

I

nvolu ional ec ropion has he same involuional ac ors as in an involu ional en ropion (e.g., horizon al laxi y, ver ical ins abili y). T ese pa ien s do no have hyper rophic, spasic orbicularis muscle o override, so he uns able eyelid sags ou ward ins ead o being driven inward. Symp oms are less acu e and no as severe as in involu ional en ropion. Many pa ien s will have mild involu ional ec ropions and may be asymp oma ic. Epidemiology and Etiology Age: Incidence increases as age increases. Gender: Equal occurrence in males and emales E iology: Eyelid issue laxi y, especially horizon al laxi y History Insidious onse o ocular irri a ion and/ or earing Pa ien may no e redness and in ammaion o he eyelid margin. Examination Eyelid sagging in eriorly and away rom he globe sur ace ( Fig. 5-4)

Mus look or he amoun o horizon al laxi y, corneal exposure, and s enosis o he lacrimal punc a. Special Considerations arsal ec ropion is comple e eversion o he eyelid and indica es de achmen o he lower eyelid re rac ors. T is condi ion mus be recognized, as i requires bo h horizon al igh ening and rea achmen o he re rac ors. Dif erential Diagnosis Cica ricial ec ropion Paraly ic ec ropion Treatment Mild ec ropion wi h only mild exposure symp oms can some imes be rea ed wi h ocular lubrica ion. De ini ive rea men involves horizon al eyelid shor ening and possible punc oplas y. Prognosis Excellen . ecurrence a er surgery is es ima ed a 5% o 10%, bu is higher he longer he ollow-up is done and he more severe he ec ropion was a he ime o he repair.

Ectropion

FIGURE 5-4. Involutional ectropion. Bila eral ec ropions wi h very lax eyelids. No e he red palpebral conjunc iva rom chronic exposure.

63

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5 EYELID MALPO SITIO NS

PAR LYTIC ECT OPION

P

araly ic ec ropion is he resul o emporary or permanen seven h cranial nerve palsy. T e lower eyelid sags away rom he globe, resul ing in loss o pro ec ion o he eye and inabili y o he lacrimal sys em o collec ears. Pa ien s wi h less severe palsy and o her eye pro ec ive mechanisms in ac presen wi h earing. Pa ien s wi h more severe palsies and poor eye pro ec ion mechanisms presen wi h corneal breakdown. Epidemiology and Etiology Age: Any age Gender: Equal occurrence in males and emales E iology: Facial palsy e iologies include: Bell’s palsy Surgery: in racranial or acial S roke umor

Evalua e severi y o acial palsy, degree o ec ropion, amoun o corneal exposure, amoun o lagoph halmos, and presence o an in ac Bell’s phenomenon. Special Considerations Mus check or corneal sensa ion as loss o corneal sensa ion will make all exposure symp oms much worse. Any unexplained acial palsy mus be worked up. Dif erential Diagnosis Bell’s palsy versus nonresolving acial palsy

History Previous onse o acial palsy Depending on he severi y o he acial palsy, he ec ropion may have onse a he same ime or he eyelid may slowly sag wi h ime. T e severi y o he condi ion depends on he severi y o he paralysis, corneal sensa ion, and ocular lubrica ion.

Treatment rea men depends on he an icipa ed dura ion o he paralysis. I spon aneous improvemen is an icipa ed, hen rea men wi h lubrica ion and a emporary arsorrhaphy i severe corneal problems are presen is indica ed. I corneal exposure is s ill a problem wi h lubrica ion use and he paralysis is long erm, hen horizon al eyelid igh ening is used o rea he paraly ic ec ropion. Placing a gold weigh in he upper eyelid may also be required. arely, a permanen arsorrhaphy may be needed.

Examination T e lower eyelid is ound o be sagging away rom he globe ( Fig. 5-5).

Prognosis Variable. T e ec ropion ends o recur over ime i he paralysis is permanen .

Ectropion

FIGURE 5-5. Paralytic ectropion. Righ lower eyelid ec ropion as he resul o a acial palsy.

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5 EYELID MALPO SITIO NS

CICAT ICIAL ECT OPION

C

ica ricial ec ropion is caused by mechanical shor ening o he an erior lamellae o he eyelid pulling he eyelid down and ou ward. T is resul s in earing and corneal exposure. More common in he lower eyelid bu can occur in he upper eyelid.

Epidemiology and Etiology Age: Any age Gender: More common in males because o higher incidence o rauma ic even s. E iology: Scarring o he an erior lamellae o he eyelid pulls he eyelid ou ward. T e e iologies include: rauma Surgery Derma i is Skin carcinoma History May include a specif c his ory such as rauma or surgery I a chronic derma ologic condi ion is he cause o he scarring, his may be a known or a previously unrecognized condi ion. Examination Ex ernal scarring or skin changes are no ed on he upper or more commonly he lower eyelid.

T is scarring resul s in shor ening o he eyelid skin and ou - urning o he eyelid margin ( Fig. 5-6A). Special Considerations Mus always consider a skin carcinoma as he possible cause o scarring o he skin. I he cause is unclear, a biopsy is needed. Dif erential Diagnosis Impor an o di eren ia e involu ional ec ropion rom hose wi h cica ricial changes. Treatment rea men o any underlying derma ologic condi ion is impor an . In rauma ic or pos surgical cases, he scarring should be le or 6 mon hs or longer unless exposure or o her problems necessi a e earlier rea men . rea men involves lysis o any deep scar issue wi h horizon al igh ening. I he skin shor ening is severe, ull- hickness skin gra s will be required. Skin gra s have he po en ial or scarring and a cosme ically no iceable area a he gra si e ( Fig. 5-6B). Prognosis rauma or surgically induced cases do well wi h repair. Chronic condi ions o he skin end o resul in recurrences.

Ectropion

67

A

B FIGURE 5-6. Cicatricial ectropion. A. rauma o he lef lower eyelid resul s in scarring o he skin wi h ver ical shor ening as well as scarring in ernally wi hin he eyelid. B.A f er repair, using a skin graf .

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5 EYELID MALPO SITIO NS

MECHANICAL ECT OPION

M

echanical ec ropion is a rare cause o ec ropion in which a mass o some ype pushes he eyelid ou ward. here are usually associa ed involu ional changes ha allow he eyelid o be pushed ou ward. Epidemiology and Etiology Age: Older pa ien s Gender: Equal occurrence in males and emales E iology: Gravi y pulls he eyelid away rom he eye or pushes he eyelid away rom he eye secondary o a mass. Causes o he mass e ec include: Derma ochalasis Edema Chalazion Eyelid umor (e.g., hemangioma, inclusion cys )

History Pa ien may be asymp oma ic, have symp oms o corneal irri a ion, or have redness and irri a ion o he eyelid. Examination Mus de ermine he degree o involu ional changes o he eyelid as well as he e iology o he mass dis or ing he eyelid. T e amoun o corneal exposure and any corneal scarring should also be no ed ( Fig. 5-7). Dif erential Diagnosis Involu ional ec ropion Cica ricial ec ropion Paraly ic ec ropion Treatment Excision o he mass and correc ion o he involu ional ac ors o he eyelid Prognosis Good i he mass can be elimina ed

Ectropion

69

A

B FIGURE 5-7. Mechanical ectropion. A. Chemosis rom an in amma ory process mechanically pushes he lower eyelid ou ward. T ere are usually some involu ional changes presen o allow he eyelid o be pushed ou . Resolu ion o he chemosis allowed he eyelid o re urn o a normal posi ion. B. Mechanical ec ropion rom a chalazion. Even wi h chalazion resolu ion, he ec ropion may remain.

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SYMBLEPH ARON

S

ymblepharon is scarring be ween he bulbar and palpebral conjunc iva. T is may be associa ed wi h ac ive in amma ion or here may be no in amma ory signs. Epidemiology and Etiology

Age: Any age Gender: More requen in women E iology: T e ollowing can resul in scarring o wo conjunc ival sur aces: Chronic blephari is Previous rauma Conjunc ival scarring diseases (e.g., ocular cica ricial pemphigoid, S evensJohnson syndrome) A opic disease Eyelid surgery Conjunc ival burns Chronic glaucoma drops, especially mio ics History T ere may be no his ory, jus asymp oma ic symblepharon no ed on examina ion. Pa ien s wi h his ory o eye or eyelid rauma or in amma ion may also have symblepharon. Examination Scarring o he conjunc ival sur aces may be very sub le wi h sligh in erior ornix shor ening or i may be very obvious wi h large conjunc ival bands be ween he eye and eyelid ( Fig. 5-8).

Mus be sure o examine under he upper lid or conjunc ival scarring, as early signs are some imes more obvious here. Special Considerations I is impor an o de ermine he cause o he symblepharon. I asymp oma ic, he symblepharon may require no rea men excep looking or he cause o he scarring. uling ou a progressive conjunc ival scarring disease, such as ocular cica ricial pemphigoid, is impor an . Dif erential Diagnosis T e di eren ial diagnosis involves de ermining he cause o he symblepharon, no whe her he process is a symblepharon. Laboratory Tests Conjunc ival scarring o unknown e iology requires a conjunc ival biopsy wi h immunouorescence es ing o rule ou ocular cica ricial pemphigoid. In rare cases, squamous cell carcinoma may cause symblepharon; here ore, pa hologic evalua ion should be considered in selec cases. Treatment None or mild symblepharon Moni oring or progression is impor an . Signif can symblepharon may cause richiasis and cica ricial en ropion ha hen may require rea men . Prognosis Variable depending on he cause o he symblepharon

Symblepharon

A

B FIGURE 5-8. Symblepharon. A. Scarring is seen be ween he eyelid and he in erior cornea. B.E arly symblepharon may be no ed only as shor ening o he ornix.

71

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5 EYELID MALPO SITIO NS

TRICHIASIS richiasis is an acquired misdirec ion o eyelashes. richiasis may be ocal, as is seen a er eyelid rauma in he area o he lacera ion. T e process may be di use wi h eyelid scarring and lashes along he en ire eyelid margin. Epidemiology and Etiology Age: Any age. Non rauma ic causes are rare in childhood. More common wi h increasing age. Gender: More common in emales E iology: Lash ollicles are dis or ed and become misdirec ed wi h scarring o he eyelid. Chronic eyelid in amma ion may resul in grow h o misdirec ed lashes. Chronic blephari is, eyelid rauma, and conjunc ival scarring diseases can all cause richiasis. History Pa ien s will o en have a his ory o chronic eye irri a ion and in amma ion. T ey may also have a long his ory o eyelash problems. T ere may be a his ory o eyelid rauma or surgery. Examination Eyelashes are seen rubbing on he eyelid sur ace ( Fig. 5-9). T e amoun o corneal changes depends on he number o lashes and dura ion. T ere may be jus SPK or here may be corneal scarring. Special Considerations Impor an o di eren ia e richiasis rom abnormal eyelid posi ions, such as en ropions, which secondarily resul in eyelashes rubbing on he cornea.

Dif erential Diagnosis Spas ic en ropion Involu ional en ropion Cica ricial en ropion Congeni al dis ichiasis Laboratory Tests Conjunc ival scarring o unknown e iology requires a conjunc ival biopsy wi h immuno uorescence es ing o rule ou ocular cica ricial pemphigoid. Treatment Lashes can be epila ed or emporary relie bu hey always grow back. Elec rolysis or cryo herapy will abla e lashes on a more “permanen ” basis. A bes , 50% o he lashes will no regrow so mul iple rea men s are required. In cases o severe scarring, eyelid surgery will be needed o correc he problem. T is may include marginal ro a ion, excision o he abnormal lashes, and a buccal mucosal gra . Prognosis Dependen on he cause o he richiasis Chronic progressive in amma ory diseases, such as ocular cica ricial pemphigoid, will o en have recurren lashes ha can be very di cul o comple ely eradica e. richiasis rela ed o rauma or o her nonprogressive scarring usually responds well o rea men .

Trichiasis

73

A

B FIGURE 5-9. richiasis. A. Eyelashes are growing pos eriorly, con ac ing he cornea rom he upper eyelid. In rue richiasis, he eyelid margin is normal. Of en, wi h conjunc ival scarring disease, here will be some accompanied in- urning o he eyelid margin. B. Lower eyelid richiasis rom conjunc ival scarring.

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PTOSIS CONGENITAL MYOGENIC PTOSIS

C

ongeni al myogenic p osis is he mos common congeni al p osis and resul s rom a dysgenesis o he leva or muscle. I may be unila eral or bila eral and can vary in severi y rom very mild p osis o very severe.

Epidemiology and Etiology Age: Bir h Gender: Equally a ec s males and emales E iology: T e leva or muscle developmen is abnormal, resul ing in f brosis and at y inf l ra ion o he leva or muscle. History P osis no ed a bir h or soon a er. Child may have chin up head posi ion, especially i bila eral. Paren s may no e he child’s eyes are open while asleep. Examination P osis is no ed o be ei her unila eral or bila eral. T ere is lit le or no leva or unc ion, resul ing in a f bro ic, s i eyelid wi h a airly f xed posi ion as he eye moves rom up o down gaze. T e lid crease is o en poorly ormed. Depending on he severi y o he p osis, here may be amblyopia wi h unila eral p osis ( Fig. 5-10). Special Considerations T ere will be abnormal superior rec us unc ion in 16% o pa ien s; his makes exposure problems a er repair and s rabismus a concern.

Dif erential Diagnosis I he p osis is congeni al wi h poor leva or unc ion, here is lit le else in he di erenial. Bir h rauma can resul in a p osis, bu here is usually good leva or unc ion and he leva or muscle is no f bro ic. Marcus Gunn jaw wink needs o be considered in all cases o congeni al p osis (see sec ion “Marcus Gunn Jaw Wink”). T ere are o her orms o myogenic p osis ha are acquired, no congeni al, such as in muscular dys rophy, chronic progressive ex ernal oph halmoplegia (CPEO), myas henia gravis, or oculopharyngeal dys rophy. Laboratory Tests Skele al muscle biopsy and elec rophysiologic es ing may be needed. An elec rocardiogram should be done i CPEO is suspec ed. Treatment Fron alis suspension surgery using su ure, a silicone rod, or ascia la a T e age o do surgery depends on he severi y o he p osis and any underlying amblyopia. Amblyopia may need rea men wi h pa ching a er he eyelid is li ed. T ere is con roversy in rea ing unila eral congeni al p osis regarding whe her o do a ron alis suspension only on he p o ic eye or i he normal eye should have excision o he leva or and a ron alis suspension as well o provide symme ry. Prognosis Surgery is very success ul in li ing he lid above he pupillary axis. T e eyelid will someimes all wi h ime and repea surgery may be needed la er.

Ptosis

75

FIGURE 5-10. Congenital myogenic ptosis. Modera e congeni al p osis in a child. No e he ex reme use o he eyebrows o lif he eyelids. T ere is a prominen eyelid crease in his child, bu i is usually poorly de ned in congeni al p osis. Leva or unc ion was 3 mm.

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5 EYELID MALPO SITIO NS

ACQUI ED MYOGENIC PTOSIS

A

cquired myogenic p osis is an unusual cause o p osis rela ed o developmen o a muscular disease ha can be localized or may be sys emic. Epidemiology and Etiology Age: Acquired bu can presen in children or adul s E iology: Sys emic muscular diseases ha can cause acquired myogenic p osis include muscular dys rophy, CPEO, myas henia gravis, and oculopharyngeal dys rophy. History Progressive p osis ha is o en associa ed wi h o her muscular dys unc ion Examination P osis is no ed wi h decreased leva or unc ion. T ere may be abnormal eye movemen s and abnormal acial one. Myas henia gravis may have double vision as par o he presen a ion. Chronic progressive ex ernal oph halmoplegia has decreased eye movemen s, bu here is no diplopia. Care ul evalua ion o he abili y o close he eyes is needed, as poor closure will increase he risk o pos opera ive corneal exposure ( Fig. 5-11). Special Considerations Chronic progressive ex ernal oph halmoplegia is a gradual, bila eral p osis ha begins in childhood or young adul hood and is progressive wi h involvemen o ex raocular muscles.

I is heredi ary in 50% o he cases. I is progressive un il he eyes are f xed in a sligh ly downward direc ion wi h a severe p osis. Hear block, re ini is pigmen osa, abnormal re inal pigmen a ion, and various neurologic signs have been associa ed wi h his syndrome. Dif erential Diagnosis Congeni al p osis Neurogenic p osis Laboratory Tests Skele al muscle biopsy and elec rophysiologic es ing may be needed. An ECG should be done i CPEO is suspec ed. Treatment Surgery is needed or correc ion. Evalua ion and rea men o any sys emic abnormali ies mus be addressed f rs . Depending on he severi y o he p osis and he amoun o leva or unc ion, ei her leva or resec ion or ron alis suspension is indica ed. Fron alis suspension wi h a silicone rod will work well in many o hese pa ien s. Prognosis Mos pa ien s can achieve an eyelid level ha is unc ional. Mos will no be able o achieve an eyelid level or unc ion ha is considered normal.

Ptosis

77

A

B FIGURE 5-11. Acquired myogenic ptosis. A. Pa ien wi h muscular dys rophy and severe p osis. T ere is very lit le leva or unc ion, and he pa ien is barely able o keep his eyelids above he pupil wi h ex reme eyebrow eleva ion. B.T e same pa ien af er ron alis suspension surgery. He can now e ec ively lif his eyelids by eleva ing his eyebrows.

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APONEU OTIC PTOSIS

A

poneuro ic p osis is he mos common ype o p osis and is he resul o disinser ion o he leva or aponeurosis. T is ype o p osis may be caused by normal aging changes, swelling, or repe i ive s re ching o he upper eyelid. T e onse o p osis is gradual. Epidemiology and Etiology Age: arely congeni al. Mos common in older pa ien s. Gender: Equal E iology: Aponeuro ic p osis is due o an abnormali y o he leva or aponeurosis or i s inser ion. T is resul s rom normal involu ional changes and/ or repe i ive rac ion such as eyelid rubbing, eyelid swelling, and eye surgery. History Gradual, progressive droopiness o he eyelids is he mos common his ory. ecen eye surgery or eyelid swelling can exacerba e he p osis. Examination Mild o severe p osis wi h normal leva or unc ion and o en a high eyelid crease or less commonly a poor eyelid crease.

T e p osis may be worse in downgaze ( Fig. 5-12). Special Considerations Myas henia gravis mus be considered in all cases. Dif erential Diagnosis Congeni al p osis (di eren ia e by poor leva or unc ion) Myas henia gravis rauma ic p osis Laboratory Tests None Treatment Ex ernal leva or resec ion and müllerecomy are bo h good surgical approaches or success ul repair. Dry eyes, poor eye closure, and poor Bell’s phenomenon mus all be recognized preopera ively. T ese condi ions make pos opera ive corneal exposure more likely. Prognosis Excellen prognosis or success ul surgical correc ion

Ptosis

79

FIGURE 5-12. Aponeurotic ptosis. Bila eral p osis rom dehiscence o he leva or aponeurosis. No e he high, very de ned upper eyelid crease. Leva or unc ion was 18 mm.

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NEUROGENIC PTOSIS THI D NE VE PALSY hird cranial nerve palsy is usually mani es as sudden or progressive onse o p osis wi h an underlying s rabismus. De ermining he e iology is he f rs priori y, as some causes o a hird cranial nerve palsy are li e- hrea ening. rea men is di cul . Epidemiology and Etiology Age: Any age. are in children. Gender: No emale versus male di erence no ed. Etiology Ischemic microvascular disease Compressive: aneurysm, umor rauma Oph halmoplegic migraine: children History Acu e onse o p osis wi h double vision when he eyelid is li ed. May or may no be associa ed wi h pain. Examination Comple e p osis wi h he eye posi ioned down and ou ( Fig. 5-13). T ere is an inabili y o eleva e, depress, or adduc he eye. T e pupil may or may no be dila ed. Aberran regenera ion o he hird nerve should be ruled ou . Special Considerations I he pupil is dila ed, he pa ien needs emergen neuroimaging o rule ou a pos erior communica ing ar ery aneurysm. Nonresolving hird nerve palsies, incomple e hird nerve palsies, and any hird nerve palsy wi h aberran regenera ion requires neuroimaging.

Pa ien s younger han 50 years o age need neuroimaging unless hey have signif can vascular disease. Vasculopa hic causes o hird nerve palsies should resolve wi hin 3 mon hs. Dif erential Diagnosis Myas henia gravis Chronic progressive ex ernal oph halmoplegia Laboratory Tests M I wi h MR , or an angiogram i he hird nerve palsy involves he pupil Pathophysiology In errup ion o he hird nerve may be caused by compression o he nerve or ischemia. Ischemia will no cause pupillary dilaion and will resolve wi hin 3 mon hs. Treatment T e majori y o pupil-sparing hird nerve palsies will resolve in 3 mon hs. T ese pa ien s should be given adequa e ime or spon aneous resolu ion be ore surgical correc ion is per ormed. T e underlying s rabismus mus be rea ed be ore at emp ing o li he eyelid. P osis surgery requires ron alis suspension, bu here is risk o corneal exposure. Fron alis suspension wi h a silicone rod is a sa er surgical approach. Prognosis Many hird nerve palsies will resolve in 3 o 6 mon hs. T ose ha do no resolve are di cul o ge in o normal eyelid posi ion wi hou causing an unaccep able amoun o corneal exposure. Pa ien s will o en have residual diplopia rom he mo ili y problems when he eyelid is raised.

Neurogenic Ptosis

81

A

B FIGURE 5-13. T ird nerve palsy. A. Comple e p osis o he upper eyelid wi h no leva or unc ion. B.E leva ion o he p o ic eyelid reveals ocular misalignmen consis en wi h a hird nerve palsy.

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MYASTHENIA GR VIS

M

yas henia gravis is an au oimmune disorder in which au oan ibodies at ack he recep ors o he neuromuscular juncion. T e resul is usually a sys emic muscular weakness ha can be li e- hrea ening due o respira ory compromise. P osis wi h or wi hou diplopia can o en be he ini ial presena ion. rea men o he sys emic disease is maximized be ore any surgery is done on he eyelids. Epidemiology and Etiology Age: Any age Gender: More common in emales E iology: Au oimmune disease; may be associa ed wi h hymoma or an eceden in ec ion. History Insidious onse o droopy eyelids, double vision, or bo h, which are o en worse a he end o he day. Pa ien s may have acial weakness, proximal limb weakness, or di cul y swallowing and brea hing. Symp oms are ypically in ermit en . Examination T e disease is mos o en generalized and sys emic bu may presen ini ially wi h p osis and/ or double vision. P osis is worsened wi h sus ained upgaze and diplopia is worse wi h con inual eye movemen s ( a igue). Weakness o he orbicularis muscles is usually ound. T ere may also be acial and proximal limb weakness. Diagnosis may be made wi h he ice es , ace ylcholine recep or an ibody es ing, single-f ber EMG, or ensilon es ing.

T e ice es involves placing an ice pack on he eyelids or 2 minu es, i he p osis is secondary o myas henia gravis, i will improve. ensilon es ing involves IV adminis raion o edrophonium chloride ( ensilon). Improvemen o he p osis or diplopia indica es he e iology is myas henia gravis. T e use ulness o ensilon es ing is limi ed because o he po en ial adverse e ec s including bradycardia and even respira ory arres ( Fig. 5-14). A combina ion o single-f ber EMG and an ibody es ing is mos commonly used o make he diagnosis. Special Considerations Pa ien s wi h newly diagnosed myas henia gravis need a C or M I o he ches o rule ou hymoma. Pa ien s wi h any signs or symp oms o respira ory compromise need immedia e neurologic evalua ion or possible hospi al admission and rea men . Dif erential Diagnosis Ea on–Lamber syndrome Chronic progressive ex ernal oph halmoplegia T ird nerve palsy Laboratory Tests Ace ylcholine recep or an ibody assay and single-f ber EMG Pathophysiology An au oimmune disorder in which au oanibodies at ack he recep ors o he neuromuscular junc ion Treatment rea men o he disease is sys emic and may include pyridos igmine bromide (Mes inon), prednisone, and possible hymec omy.

Neurogenic Ptosis

rea men should be coordina ed by a neuro-oph halmologis or neurologis . Once maximum medical improvemen has been achieved, surgical correc ion o he p osis can be at emp ed.

83

Fron alis suspension is usually required. Prognosis Variable depending on he severi y o he disease

FIGURE 5-14. Myasthenia gravis. Bila eral p osis wi h inabili y o keep he lids rom covering he pupils. T ere is also decreased one o he acial muscles.

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MA CUS GUNN JAW-WINKING SYND OME his syndrome may be very mild or very drama ic wi h eleva ion o he eyelid wi h each jaw movemen when chewing ood. rea men is required i he p osis or he eyelid movemen is signif can . Epidemiology and Etiology Age: Presen a bir h E iology: A congeni al synkine ic syndrome caused by a congeni al aberran connec ion o he oculomo or nerve f bers ha innerva e he leva or muscle and he rigeminal nerve f bers o he muscles o mas ica ion.

Movemen o he mandible la erally, o he con rala eral side mos commonly, resul s in eleva ion o he eyelid ( Fig. 5-15). Special Considerations T e amoun o p osis and he amoun o synkine ic movemen will de ermine he rea men . Dif erential Diagnosis Congeni al p osis Laboratory Tests None

History A care aker o en f rs no ices his condi ion when eeding he baby. T e a ec ed eyelid will move up and down wi h he jaw movemen during eeding.

Treatment I he amoun o synkine ic movemen is small, hen a ron alis suspension is done. I he synkine ic movemen is large, hen he leva or muscle mus be disinser ed and excised be ore he ron alis suspension can be done.

Examination A unila eral p osis wi h poor leva or uncion. T e unila erally p o ic eyelid eleva es wi h movemen o he jaw.

Prognosis I may be di cul o ge good symme ry in he eyelids unless bo h eyes are opera ed on.

Neurogenic Ptosis

85

A FIGURE 5-15. Marcus Gunn jaw winking syndrome. A. Pa ien wi h severe p osis wi h ull eyebrow eleva ion and chin up posi ion o keep his eyelids above he pupil. ( continued)

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B

C FIGURE 5-15. (Continued) Marcus Gunn jaw winking syndrome. B. Wi h opening o his mou h, he eyelids go up and he is able o normalize his head posi ion. C and D. More common unila eral jaw winking where lef upper lid open wi h opening o he mou h. ( continued)

Neurogenic Ptosis

D FIGURE 5-15. (Continued)

87

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HO NE ’S SYND OME

H

orner’s syndrome classically presen s wi h mild p osis (2 mm) and pupillary miosis. A er any serious e iology has been ruled ou , hese cases respond very well o surgical correc ion wi h a müllerec omy.

Epidemiology and Etiology Age: May be congeni al or acquired E iology: E iology o acquired cases includes rauma, surgical procedures in he neck area, apical lung malignancies, aneurysm, dissec ion o he caro id ar ery, and idiopa hic. History Mild p osis is no ed. T e pupillary miosis may or may no be no ed un il he examina ion. Examination P osis, pupillary miosis, and anhidrosis are he hree f ndings. T e p osis is usually mild (1 o 2 mm). In congeni al Horner’s syndrome, here is also decreased pigmen a ion o he iris on he involved side ( Fig. 5-16). Special Considerations Cocaine es ing is used o conf rm he diagnosis. A 4% o 10% cocaine solu ion will dila e a normal pupil bu will ail o dila e a pupil a ec ed by Horner’s syndrome.

O her pharmacologic es ing will di erenia e f rs - and second-order neuron in errupion rom hird-order neuron. Hydroxyamphe amine drops will no dila e he pupil o a hird-order neuron Horner’s syndrome. A hird-order neuron Horner’s syndrome is generally o benign e iology. Dif erential Diagnosis Aponeuro ic p osis wi h pupillary anisocoria Laboratory Tests Ches C and M I/ MR wi h gadolinium o he neck and brain in all pa ien s wi h f rs - or second-order neuron involvemen Many neurologis s will image all pa ien s wi h Horner’s syndrome. Pathophysiology In errup ion o he sympa he ic innervaion o Müller’s muscle resul s in he p osis, whereas he dila or muscle o he iris resul s in he miosis. Treatment Once de ermined o be o benign e iology, a müllerec omy is he procedure o choice. Prognosis T e p osis responds well o surgical correc ion.

Neurogenic Ptosis

89

A

B FIGURE 5-16. Horner’s syndrome. A. P osis wi h miosis on he lef side. T e amoun o p osis is more han is of en seen in Horner’s syndrome and here may be some aponeuro ic dehiscence as well. B. Congeni al Horner’s syndrome wi h p osis, miosis, and iris hypopigmen a ion.

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MECH ANICAL PTOSIS

M

echanical p osis is drooping o he eyelid rela ed o res ric ion o he eyelid rom ei her scar issue or he weigh o a mass or swelling. Epidemiology and Etiology Age: Any age Gender: Equal E iology: T e increased weigh o any mass will weigh he eyelid down. T is can include chalazion, skin carcinoma, gian papillary conjunc ivi is, hemangiomas, neurof bromas, and so or h. es ric ion o eyelid movemen by scar issue will also produce his orm o p osis. History apidi y o onse varies according o he process Chalazion will have rapid onse , whereas a large basal cell carcinoma may slowly worsen over years. Examination P osis wi h an eyelid mass or evidence o scar issue ( Fig. 5-17) T e cause can be ex ernal or under he eyelid and di cul o see such as in severe gian papillary conjunc ivi is.

Eyelid scarring in ernally requires palpaion and s re ching and eversion o he eyelid o iden i y i . Special Considerations Imaging, such as C , may be needed o de ermine he ex en o he mass or o rule ou a oreign body or a rac ure a er rauma. Dif erential Diagnosis rauma ic p osis Aponeuro ic p osis Laboratory Tests None Treatment Addressing he cause is he primary rea men . Managemen can include medical rea men or surgical excision o a lesion or scar issue. Some pa ien s may require a second surgery o correc he p osis i removing he mechanical cause is no cura ive. Prognosis ela ed o prognosis o he mass or swelling ha is causing he p osis. I i is he resul o a recurren process, he prognosis is poor.

Mechanical Ptosis

91

FIGURE 5-17. Mechanical ptosis.N euro broma o he lef upper lid weighing down he eyelid and causing a p osis.

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TRAUMATIC PTOSIS rauma ic p osis has mul iple causes; however, exac ly which causes are presen in each case can be di cul o de ermine. Allowing 6 mon hs or spon aneous resoluion be ore correc ion is he rule in all rauma ic cases. Epidemiology and Etiology Age: All ages Gender: Males more common E iology: May include mul iple causes including myogenic, neurogenic, and aponeuro ic injuries History rauma o he eyelid wi h residual p osis a er he swelling resolves Examination Documen a ion o he severi y o p osis will allow moni oring or improvemen . Assessmen o leva or unc ion also helps de ermine e iology.

Any residual swelling, scarring, and lagoph halmos should also be documen ed ( Fig. 5-18). Special Considerations Mus wai 6 mon hs or possible resolu ion o he p osis be ore repairing. A number o cases will improve or resolve during his ime. Any injury ha sugges s direc cut ing o he leva or should have explora ion o he leva or a he ime o rauma repair i possible. Treatment Moni or or improvemen over he 6 mon hs ollowing he rauma. I he lid is s ill p o ic a er 6 mon hs, surgical correc ion wi h ex ernal leva or resec ion or ron alis suspension depending on leva or unc ion is indica ed. Prognosis Good i here is good leva or unc ion. I here is poor leva or unc ion wi h scarring, he prognosis is no as good.

Traumatic Ptosis

93

FIGURE 5-18. raumatic ptosis. Eyelid and eyebrow lacera ions resul ing in a rauma ic p osis. T is p osis did no improve over 6 mon hs and required surgical repair.

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PSEUDOPTOSIS

P

seudop osis is he alse illusion o p osis caused by malposi ion o he eye, conrala eral eyelid, or lack o orbi al volume. I is very impor an o recognize a pseudop osis o avoid doing unnecessary or he wrong surgery. Epidemiology and Etiology Age: Any age Gender: Equal E iology: T e eyelid posi ion is normal bu o her ac ors resul in he appearance o p osis. T e mos common is an abnormal posi ion o he eye. Eleva ion o he eye or he eye sinking in will bo h give a alse p osis. Derma ochalasis (see “Derma ochalasis”) is also a common cause o pseudop osis. History Pa ien presen s wi h he complain o a droopy eyelid or a variable period. Examination In all pa ien s wi h p osis, evalua ion mus include no ing he posi ion o he eye. Enoph halmos, hyper ropia, and a globe pushed superiorly all cause pseudop osis.

Evalua ion o he eyelid skin rela ive o he rue eyelid margin posi ion is impor an ( Fig. 5-19A). Eyelid re rac ion on he con rala eral side mus also be considered. Special Considerations C scanning may be needed i a mass pushing he globe up is suspec ed. Dif erential Diagnosis Enoph halmos ( Fig. 5-19B) Hyper ropia Globe malposi ion Lid re rac ion o he con rala eral eyelid Derma ochalasis Treatment Depends on he cause o he pseudop osis Enoph halmos, microph halmos, ph hisis bulbi: build up he orbi Hyper ropia: consider s rabismus surgery Globe eleva ion: remove mass Con rala eral eyelid re rac ion: correc eyelid re rac ion Derma ochalasis: blepharoplas y Prognosis Good

Pseudoptosis

95

A

B FIGURE 5-19. Pseudoptosis. A. Derma ochalasis is he mos common cause o pseudop osis. I he eyelid skin is eleva ed, he eyelid is in a normal posi ion under he skin. B. Ano her orm o pseudop osis is enoph halmos resul ing in drooping o he eyelid. T is pa ien may also have some componen o leva or aponeurosis disinser ion.

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BROW PTOSIS

B

row p osis is drooping o he eyebrows. I is o en a par o derma ochalasis bu mus be recognized and rea ed separa ely or he bes resul s. Many pa ien s will have some degree o brow p osis and no have symp oms. Symp oms can include loss o superior visual f eld, brow ache and a igue, and rhy ids o he orehead and brow. T ose wi h signif can brow p osis may require eyebrow surgery wi h or wi hou eyelid surgery.

Epidemiology and Etiology Age: More common as pa ien s age Gender: Equal. Females are more likely o be symp oma ic. Epidemiology: Wi h aging, gravi y, involu ional changes, and loss o elas ici y resul in drooping o he eyebrows. Depending on mul iple ac ors, his drooping will become symp oma ic in hose 50 years o age or older.

T is may give he appearance o excess upper eyelid skin. Pa ien s will also develop urrows o he orehead rom chronically eleva ing heir eyebrows ( Fig. 5-20). Special Considerations Brow droop will add o redundan skin o he upper eyelids. I is impor an o recognize how much o he excess skin on he upper eyelids will go away i he eyebrows are eleva ed. T is excess rom he brow droop should no be excised during blepharoplas y or he eyebrows will appear oo close o he eyelids. Ideally, he brow should be li ed and hen he blepharoplas y per ormed.

History Pa ien s will at emp o li heir eyebrows resul ing in deep urrows o he orehead, brow ache, and even headaches.

Treatment Brow li using one o he ollowing echniques; each has i s own indica ions and advan ages. Endoscopic eyebrow li Coronal eyebrow li Mid orehead eyebrow li Direc eyebrow li

Examination Eyebrows si below he superior orbi al rim.

Prognosis Good

BrowPtosis

97

FIGURE 5-20. Brow ptosis.T e eyebrows are well below he orbi al rim in his pa ien , adding o he apparen amoun o derma ochalasis.

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DERMATO CH ALASIS

D

erma ochalasis is a very common condi ion which increases as people age. Loss o elas ici y rom ul raviole exposure and aging resul s in excess skin o he upper and lower lids. I severe enough, he upper eyelid skin may cause unc ional blockage o he superior visual f eld. More commonly, he excess skin and he associa ed an erior prolapse o orbi al a is a cosme ic de ormi y.

Epidemiology and Etiology Age: Older pa ien s Gender: Equal male- o- emale ra io E iology: E iology is loss o eyelid skin elas ici y rom aging and ul raviole ligh exposure. T ere may be a heredi ary componen o derma ochalasis, especially when i occurs a a younger age.

History Symp oms are brow ache, heaviness around he eyes, and loss o superior visual f eld. T ese have a slow, insidious onse . Examination Excess skin o he upper eyelids o varying degrees I becomes especially bo hersome a er he skin con ac s he eyelashes. Derma ochalasis is o en associa ed wi h an erior prolapse o orbi al a . Underlying he excess skin, he possibili y o a rue p osis mus be evalua ed ( Fig. 5-21). Treatment Blepharoplas y, which may be unc ional or cosme ic in na ure. Prognosis Good

Dermatochalasis

99

FIGURE 5-21. Dermatochalasis.T ere is a large amoun o overhanging skin o bo h upper eyelids. T e underlying eyelid posi ion is normal. T ere is also some brow droop, which was addressed surgically a he same ime as he blepharoplas y.

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BLEPH ARO CH ALASIS

B

lepharochalasis is a rare, amilial varian o angioneuro ic edema ha occurs in younger individuals. I is charac erized by recurren episodes o in amma ory edema o he eyelids ha resul s in s re ching o he issues; over ime, he eyelids ake on he appearance o derma ochalasis commonly seen in much older pa ien s.

Epidemiology and Etiology Age: Onse in eens o 20s Gender: More common in emales E iology: Unknown; a varian o angioneuro ic edema

T ere may also be rue p osis, lacrimal gland prolapse, and prominen vessels o he lids. Mos commonly, hese f nding are unila eral. Pa ien s may also be seen a he ime o swelling wi h a swollen, uid-f lled lid wi h very lit le in amma ory signs. Dif erential Diagnosis Derma ochalasis T yroid-rela ed oph halmopa hy Orbi al in amma ory disease Pathophysiology Unknown

History ecurren episodes o eyelid swelling, usually unila eral

Treatment rea men is surgical excision o he skin and correc ion o p osis. Surgical repair can be complica ed by recurren edema, which may resul in recurrence o he problem.

Examination Excess skin o he eyelids ha is very hin and paper-like ( Fig. 5-22)

Prognosis Variable, depending on whe her he edema con inues o recur or burns ou

Blepharochalasis

101

FIGURE 5-22. Blepharochalasis.T is is a 25-year-old pa ien wi h recurren swelling o he righ eyelids resul ing in he hin, s re ched, redundan skin o he upper and lower eyelid.

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EYELID RETRACTION

E

yelid re rac ion is displacemen o he eyelid oward he respec ive superior or in erior orbi al rim, resul ing in scleral show. T is condi ion can be mild and wi hou symp oms or can resul in corneal exposure. T yroid oph halmopa hy is he mos common cause and eyelid re rac ion is o en he ini ial sign o his disease. Epidemiology and Etiology Age: Adul hood. are in children. Age o onse dependen on e iology. Gender: Females more common E iology: Eyelid re rac ion is caused by hyroid oph halmopa hy mos commonly, ollowed by overaggressive eyelid surgery and ver ical rec us muscle surgery ( Fig. 5-23). Upper eyelid re rac ion may be he resul o con rala eral p osis. Lower lid re rac ion can be a normal ana omic varian . Parinaud syndrome is a cen ral nervous sys em cause o upper eyelid re rac ion. History In hyroid oph halmopa hy, he pa ien no es slow onse o one or bo h eyes appearing oo wide open or having he “hyper hyroid s are” O en, redness and irri a ion o he a ec ed eye accompany hese symp oms. Pa ien s may have a his ory o sys emic hyroid abnormali ies. T ose pa ien s wi h a surgical cause will give he his ory o eyelid or eye muscle surgery. Examination Documen he amoun o eyelid re rac ion and whe her here is re rac ion or p osis on he o her side. Upper eyelid lag on down gaze, prop osis, and dysmo ili y all go along wi h hyroid ophhalmopa hy. No e signs o corneal exposure and previous eye or eyelid surgery.

Dif erential Diagnosis Malposi ion o he globe Hypo ropia or hyper ropia Con rala eral p osis Laboratory Tests T yroid unc ion es s unless pa ien has known, con rolled hyroid abnormali ies or here is a known surgical cause o he eyelid re rac ion. Pathophysiology T yroid oph halmopa hy resul s in chronic in amma ion o he lid re rac ors leading o scar issue orma ion and re rac ion o he eyelids. Treatment In hyroid oph halmopa hy, he disease mus be rea ed so i is inac ive be ore any surgical correc ion. rea corneal exposure wi h lubrica ion while wai ing or he disease o become inac ive. Mos causes o eyelid re rac ion will require surgical rea men i hey are signif can . ecession o he re rac ors is he mos common procedure used. T is works or mild o modera e re rac ion. More severe re rac ion o he lower eyelids requires eyelid spacer ma erial o be implan ed. Excess excision o skin o he upper or lower lids during blepharoplas y may require in ernal spacers or rarely skin gra s. Prognosis Generally, re rac ion can be rea ed success ully wi h surgery. Corneal exposure is o en an ongoing problem ha is improved wi h rea men bu no comple ely cured.

Eyelid Retraction

103

A

B FIGURE 5-23. Eyelid retraction. A. Lef , lower eyelid re rac ion rom scarring o he eyelid o he orbi al rim and a i anium pla e af er rauma and orbi al rac ure repair. B.T yroid-rela ed oph halmopa hy wi h upper eyelid re rac ion.

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EYELID DYSKINESIS BENIGN ESSENTIAL BLEPHA OSPASM

B

enign essen ial blepharospasm is a bila eral condi ion charac erized by involunary spasms o he orbicularis oculi, procerus, and corruga or muscles. T is condi ion may s ar as mild wi ching o he eyelids and can progress so ha hese con rac ions leave he pa ien s unc ionally blind during he con racion. Pa ien s canno predic when he spasms will occur and he orced closure o he eyelids can be li e- hrea ening i hey occur during driving, crossing s ree s, and so or h. Epidemiology and Etiology Age: Onse is when pa ien s are 40 years o age or older. Gender: Women more commonly a ec ed han men E iology: Unknown, bu probably o cenral nervous sys em origin, mos likely in he basal ganglia. T e process causes involun ary spasms o he orbicularis oculi, procerus, and corruga or muscles. History T e spasms s ar as mild wi ches and progressively worsen wi h ime. Pa ien s o en do no presen un il he spasms are severe enough o in er ere wi h ac ivi ies o daily living. Examination Pa ien s have in ermit en episodes o orced eyelid closure ha usually las or minu es ( Fig. 5-24). Be ween spasms, he examina ion may be normal. T us, he diagnosis is o en based on his ory.

T e spasms are bila eral, al hough hey can some imes be more severe on one side han he o her. Spasms can involve he lower ace and neck wi h ime. Spasms do no occur during sleep, unlike in hemi acial spasm. Special Considerations Mus rea any condi ion ha may cause ocular irri a ion and hus worsen he blepharospasm such as dry eyes. Blepharospasm can have a al consequences i no con rolled and he pa ien drives. Dif erential Diagnosis Hemi acial spasm Severe dry eyes or o her ocular irri a ion Treatment Bo ulinum oxin injec ion. T ese injec ions are e ec ive or mos pa ien s and las 3 o 4 mon hs be ore reinjec ion is required. Wi h ime, in some pa ien s hese injecions may become less e ec ive and par ial surgical excision o orbicularis muscle and o her pro rac ors will be required. Bo ulinum oxin injec ion may s ill be needed bu will be more e ec ive a er surgery. Muscle relaxan s and seda ives are occasionally used in his disease bu are o lit le benef . Prognosis Good wi h bo ulinum oxin injec ion are cases may no respond o rea men .

Eyelid Dyskinesis

105

FIGURE 5-24. Benign essential blepharospasm.T is pa ien would develop blepharospasm wi h any at emp o ouch he eyes. T e spasm can go on o involve o her acial muscles.

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5 EYELID MALPO SITIO NS

HEMIFACIAL SPASM Epidemiology and Etiology Age: Adul hood Gender: Equal male o emale ra io E iology: Vascular compression o he acial nerve a he level o he brains em History Unila eral spasm o one side o he ace Examination T e pa ien may have mild acial palsy on he a ec ed side. T e spasm may be seen on examina ion or no ed by he pa ien ’s his ory ( Fig. 5-25). Special Considerations Spasms are presen during sleep, unlike benign essen ial blepharospasm where hey are absen .

Dif erential Diagnosis Benign essen ial blepharospasm Myokymia Treatment T e pa ien needs M I o he cerebellar– pon ine angle o rule ou a mass lesion. Bo ulinum oxin is hen generally he rea men o choice. Neurosurgical decompression o he acial nerve is some imes considered. Prognosis Bo ulinum A oxin con rols he spasm bu requires repea injec ion every 3 o 6 mon hs.

Eyelid Dyskinesis

107

FIGURE 5-25. Hemifacial spasm. Hemi acial spasm o he lef side o he ace. T e spasm is usually in ermit en .

C H AP T ER

Congeni al Eyelid Anomalies BLEPH AROPHIMOSIS

B

lepharophimosis is a congeni al eyelid syndrome ha has a charac eris ic eyelid appearance and includes elecan hus, epicanhus inversus, and severe myogenic p osis. Epidemiology and Etiology Age: Congeni al Gender: Equal Inheri ance: Au osomal dominan E iology: Unknown

History O en have amily members wi h he same syndrome Examination Charac eris ic eyelid f ndings include elecan hus, epican hus inversus, and severe p osis. O her f ndings, which may or may no be presen , include lower eyelid ec ropion, a poorly developed nasal bridge, hypoplasia o he superior orbi al rims, hyper elorism, 108

mo ili y disorders, and various degrees o men al def ciency ( Fig. 6-1). Dif erential Diagnosis No o her syndrome gives hese characeris ic changes. Mus di eren ia e rom simple epican hus (see Fig. 6-2) and elecan hus. Treatment Mul iple s ages o recons ruc ion are required. Ini ial surgery is aimed a he elecan hus and epican hus inversus. T is may require a simple Z-plas y, Y–V plas y, or ransnasal wiring. T e second s age is correc ion o he p osis, which usually requires ron alis suspension. Finally, o her eyelid abnormali ies are addressed. Prognosis Signif can improvemen can be made wi h surgery. Depending on he severi y, here will always be some eyelid changes ha remain.

Blepharophimosis

109

FIGURE 6-1. Blepharophimosis. T is child has classic changes o blepharophimosis with ptosis, telecanthus, and epicanthus inversus. T is must be dif erentiated rom simple epicanthus ( see Fig. 6-2) .

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6 CO NGENITAL EYELID ANO MALIES

EPICANTHUS

E

pican hus is a medial can hal old ha is usually caused by imma ure mid acial bones. T e condi ion is usually bila eral and will resolve as he child’s ace ma ures. Epican hus is associa ed wi h cer ain eyelid syndromes.

Epidemiology and Etiology Age: Congeni al Gender: Equal E iology: Imma ure mid acial bones are considered o be he cause. History No ed a bir h Examination T ere is an ex ra old o skin and subcu aneous issue medially involving he eyelids. T is may make he child appear eso ropic. Four ypes o epican hus have been described: Epican hus arsalis: Fold is more prominen on he upper eyelid. Epican hus inversus: Fold is more prominen on he lower eyelid.

Epican hus palpebralis: Fold is equally on he upper and lower eyelids ( Fig. 6-2). Epican hus superciliaris: Fold runs rom he eyebrow region o he lacrimal sac. Special Consideration Epican hus arsalis can be a normal varian o he Asian eyelid. Epican hus inversus is par o blepharophimosis syndrome. Dif erential Diagnosis Blepharophimosis Treatment Mos cases o epican hus resolve wi h normal acial ma ura ion, so any po en ial rea men should be delayed un il he child is ma ure. T e excep ion is epican hus inversus, which rarely disappears wi h acial ma ura ion. I surgical rea men is required, hese cases respond well o a Y–V-plas y or Z-plas y. Prognosis Excellen . Mos cases resolve as he child grows. I surgery is required, he resul s are good.

Epicanthus

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FIGURE 6-2. Epicanthal folds. T ese olds can o en be seen as an isolated nding in young children. Unless severe, these epicanthal olds will lessen and even disappear as the child’s ace matures.

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6 CO NGENITAL EYELID ANO MALIES

EPIBLEPH ARON

E

piblepharon is override o he pre arsal muscle and skin, which causes he cilia o assume a ver ical posi ion al hough he eyelid margin is in a normal posi ion. T is disorder is usually asymp oma ic wi h no corneal s aining and requires no rea men .

Epidemiology and Etiology Age: Congeni al Gender: Equal E iology: Imma ure acial bones are el o allow or his excess skin and muscle. History T ere are usually no symp oms. Examination T ere is an excess o skin overriding he eyelid margin, which may even come in con ac wi h he eye. I his skin can be pulled back, he eyelid margin under i is in a normal posi ion. T ere is rarely any corneal s aining.

I here are corneal changes, considera ion mus be given o surgical correc ion ( Fig. 6-3). Special Considerations I is o en very di cul o di eren ia e epiblepharon rom congeni al en ropion, especially in an awake child who is squeezing his or her eyes shu . I here are signif can corneal changes, hen i is likely he eyelid is en ropic and surgery is required. Dif erential Diagnosis Congeni al en ropion Treatment No rea men is needed in mos cases. I here are corneal changes, hen excision o he excess skin and muscle is he rea men o choice. Prognosis Excellen . Mos cases resolve as he acial bones ma ure. T e rare case ha requires surgery will respond well.

Epiblepharon

113

A

B FIGURE 6-3. Epiblepharon (A&B). Excess skin o the lower eyelid rolls in and touches the cornea. T e eyelid is in a normal position and the cornea is normal. T is condition must be dif erentiated rom congenital entropion (see Fig. 6-4).

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6 CO NGENITAL EYELID ANO MALIES

CONGENITAL ENTROPION

C

ongeni al en ropion is rare bu can also be di cul o diagnose in an in an . An eye ha is always irri a ed and ha he child does no wan o open is a clue o look or an epi helial de ec or corneal scar. Epidemiology and Etiology

Age: A bir h Gender: Equal E iology: Usually rela ed o an abnormali y o he eyelid re rac ors or arsus. Very rarely, here can be conjunc ival scarring causing he en ropion. History T e child’s eye is always irri a ed, and he child does no wan o open he eye. Examination I is di cul o examine he eyelid o an in an unless hey are asleep. When he child

is awake and an at emp is made o examine he eyelid, he child squeezes he eye shu and a normal eyelid may urn in. Evidence o corneal scarring or an epi helial de ec on he in erior cornea is enough o suspec an en ropion ( Fig. 6-4). Dif erential Diagnosis Epiblepharon Treatment Surgical correc ion is required. Excision o pre arsal skin and orbicularis wi h igh ening o he re rac ors is he rea men o choice. Prognosis Good. Some chance o corneal scarring i no diagnosed early.

Congenital Entropion

115

A

B

C FIGURE 6-4. Congenital entropion. A. It is di cult to examine a child’s eyelid to determine whether it is entropic, especially when the eye is already irritated. In this child, there is corneal scarring rom a congenital entropion. B. Child immediately a er placing rotating sutures in the lower eyelid. C. Postoperative picture 4 weeks a er entropion surgery. T e eyelid is in a normal position and the corneal opacity is resolving.

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6 CO NGENITAL EYELID ANO MALIES

CONGENITAL COLOBOMA

C

ongeni al colobomas are ull hickness de ec s in he eyelid. Even larger de ec s are usually well olera ed over he shor erm un il he de ec can be repaired. Upper eyelid colobomas are usually no associa ed wi h o her sys emic abnormali ies, whereas lower eyelid colobomas are more commonly associa ed wi h acial cle syndromes.

Epidemiology and Etiology Age: Apparen a bir h Gender: Equal E iology: Abnormal embryonic developmen resul s in hese eyelid de ec s. History Eyelid de ec is usually no ed a bir h or soon a er. T ere are ew symp oms. Examination T e ull- hickness de ec is mos commonly medially on he upper eyelid.

Colobomas in his loca ion are no usually associa ed wi h any o her abnormali y. A coloboma o he lower eyelid is more likely o be par o a acial cle syndrome and may have o her acial de ec s and lacrimal abnormali ies. At en ion mus be given o he cornea or signs o exposure, al hough exposure is rare ( Fig. 6-5). Dif erential Diagnosis Bir h rauma o he eyelid Treatment Surgical repair o he coloboma is usually s raigh orward and can be done wi hou any aps ha would occlude he eye and cause amblyopia. Prognosis Colobomas do very well wi h surgical repair. O her acial de ec s may no be as easy o repair.

Congenital Coloboma

117

A

B FIGURE 6-5. Congenital coloboma. A. Child born with a coloboma o the upper eyelid. T is may be a totally isolated nding, but coloboma and a preauricular skin tag (B) are consistent with Goldenhar’s syndrome.

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6 CO NGENITAL EYELID ANO MALIES

CONGENITAL DISTICHIASIS

D

is ichiasis is a rare condi ion where an ex ra row o eyelashes replaces he meibomian gland openings on he eyelids. Epidemiology and Etiology Age: Presen a bir h

Gender: Equal E iology: Embryonic pilosebaceous uni s improperly di eren ia e in o hair ollicles. History T e ex ra row o lashes may be no ed or eye irri a ion may promp oph halmic evaluaion a which ime he problem is discovered. Examination A second row o eyelashes is no ed growing pos erior o he normal eyelash posi ion ( Fig. 6-6). T ese lashes may be in con ac wi h he cornea causing symp oms o eye irria ion, corneal punc a e s aining, and scarring. A good corneal evalua ion is impor an .

Dif erential Diagnosis Congeni al en ropion Conjunc ival scarring causing richiasis Treatment rea men is based on he symp oms and individualized. I rea men is required, op ions are variable. Conserva ive rea men wi h lubrica ion and con ac lenses is o en no success ul. Eyelash abla ion wi h cryo herapy or elec rolysis o en resul s in recurrence o eyelashes bu will be adequa e in some pa ien s. Surgical excision o he eyelashes and recons ruc ion—some imes using buccal mucosal gra s—works in he mos severe cases. Prognosis Usually good, bu mul iple procedures may be required and here may be undesirable cosme ic de ec s a er surgery.

Congenital Distichiasis

119

FIGURE 6-6. Congenital distichiasis. All our eyelids have these extra rows o eyelashes growing out o the position where the meibomian glands should be.

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6 CO NGENITAL EYELID ANO MALIES

ANKYLOBLEPH ARON

C

ongeni al ankyloblepharon is a ailure o he eyelids o separa e during embryonic developmen . Ankyloblepharon may also be acquired due o scarring, which resul s in adherence o he eyelids o each o her and o he globe. Epidemiology and Etiology Age: Congeni al. Acquired cases can occur a any age depending on he cause. Gender: Equal E iology: Congeni al ailure o he eyelids o separa e during embryonic developmen . Acquired ankyloblepharon is mos commonly he resul o progressive conjunc ival scarring resul ing in usion o he eyelids. Some causes include ocular cica ricial pemphigoid, S evens–Johnson syndrome, chemical burns, and herpes zos er. History Congeni al cases no ed a bir h Acquired cases will usually have a his ory o progressive scarring rela ed o he primary disease. Examination Congeni al ankyloblepharon may have comple e usion o he eyelids or jus a ew bands holding he eyelids oge her.

T e eye and orbi may be normal or have associa ed abnormali ies ( Fig. 6-7A). Acquired ankyloblepharon shows usion o he eyelids rom scar issue. T e eye i sel is no able o be seen ( Fig. 6-7B). Dif erential Diagnosis Cryp oph halmos Microph halmos Treatment Congeni al ankyloblepharon: lysis o he bands holding he eyelids oge her O her recons ruc ive procedures may be needed depending on severi y. Acquired ankyloblepharon: de ermining he e iology o he scarring is done f rs . I his process needs rea men o quie any in amma ion, hen ha mus be done f rs . At emp s o recons ruc he eyelids and resur ace he cornea can hen be at emp ed. T is requires coordina ion be ween oculoplas ic and corneal surgeons. Prognosis Congeni al ankyloblepharon: good Acquired ankyloblepharon: poor in mos cases. T e process causing he scarring will o en hamper he healing a er eyelid and corneal recons ruc ive surgery.

Ankyloblepharon

121

A

B FIGURE 6-7. Congenital ankyloblepharon. A. Unilateral combined ankyloblepharon and cryptophthalmos. (Courtesy Richard W. Hertle, MD.) Ankyloblepharon. B. Acquired ankyloblepharon as the result o scarring rom ocular cicatricial pemphigoid. T e eyelids are used, and there is likely scarring o the eyelids to the globe as well.

C H AP T ER

Miscellaneous Eyelid Condi ions OCULAR CICATRICIAL PEMPHIGOID

O

cular cica ricial pemphigoid (OCP) is a conjunc ival scarring disease ha occurs in older adul s. I can be mild or can be progressive and lead o corneal scarring and blindness. OCP con inues o be a con using, poorly unders ood condi ion ha can be very di cul o rea in some pa ien s. Epidemiology and Etiology Age: Older adul s Gender: More common in emales E iology: An au oimmune process in which an ibodies bind o he conjunc ival basemen membranes, resul ing in in ammaion and scarring. History T ere may be a long his ory o ocular irri a ion and epila ion o eyelashes over many years. T e o her ex reme is rapidly progressive conjunc ival and even corneal scarring wi h very red in amed eyes. 122

Some pa ien s will have ulcera ion o o her mucosal sur aces such as oral, esophageal, or geni al lesions. Skin lesions may also be par o he presen a ion. A signif can number o hese pa ien s have used or are curren ly using an iglaucoma drops. Examination Findings range rom mild, sub le conjunc ival scarring in he early s ages o severe scarring where he eyelid is s uck o he cornea. Cica ricial en ropion, richiasis, and severe dryness all add o he poor ocular sur ace. T e condi ion o he cornea is impor an in guiding rea men . Evalua ion o he mou h and skin or o her lesions is impor an ( Fig. 7-1). Special Considerations Some pa ien s on an iglaucoma medicaions will ge conjunc ival scarring ha is no progressive i he medica ion is s opped. T is f nding was more common in pa ien s using mio ics, such as pilocarpine, bu also seems o be associa ed wi h some o he modern an iglaucoma drops.

Ocular Cicatricial Pemphigoid

I is no clear whe her hese pa ien s have OCP or i he scarring is en irely rela ed o he drops. Dif erential Diagnosis S evens–Johnson syndrome Acid and alkali burns Previous eyelid surgery rachoma A opic disease Laboratory Tests Immuno uorescence es ing o he conjunc iva will reveal immunoglobulins a he basemen membrane in OCP. A posi ive biopsy is diagnos ic, bu a negaive biopsy does no rule ou OCP because here are a signif can number o biopsynega ive OCP cases. Pathophysiology An au oimmune process in which immune complexes bind a he conjunc ival basemen

123

membrane ha resul s in in amma ion and even ual scarring. T is des roys he ear glands o he conjunc iva and causes in- urned eyelids, lashes and corneal scarring. Treatment T e in amma ion mus be quie ed f rs . T is may require only doxycycline in very mild cases or s rong medica ions such as cyclophosphamide, mycophenola e mo e il or aza hioprine in re rac ory cases. A er he in amma ion is quie , eyelid problems, such as richiasis or en ropions, can be addressed surgically. All pa ien s will require aggressive lubrica ion and/ or punc al occlusion. Prognosis Variable. Some pa ien s’ disease will burn ou or respond o rea men wi hou signif can ocular injury. In o her pa ien s, he disease can progress no mat er wha rea men is used.

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7 MISCELLANEO US EYELID CO NDITIO NS

A

B FIGURE 7-1. Ocular cicatricial pemphigoid. A. Scarring o the eyelid to the cornea in this advanced case. B. Earlier in the disease, the conjunctival scarring is less obvious and may even be overlooked i the conjunctiva in the ornices is not care ully examined. ( continued)

Ocular Cicatricial Pemphigoid

125

C

D FIGURE 7-1. ( Continued) Ocular cicatricial pemphigoid. C.T e lower eyelid is entropic secondary to conjunctival scarring rom pemphigoid. D. Oral ulcerations are of en ound in active ocular cicatricial pemphigoid and help solidi y the diagnosis.

C H AP T ER

Lacrimal Obs ruc ions CONGENITAL OBSTRUCTIONS

Mos obs ruc ions will spon aneously resolve by he age o 6 o 12 mon hs.

CONGENITAL NASOLACRIMAL DUCT OBSTRUCTION ongeni al nasolacrimal duc obs ruc ion is seen in 2% o 6% o newborns bu resolves in he f rs 3 o 4 weeks in mos in an s. T e chronic purulen discharge is he main problem or caregivers, bu , wi h addi ional ime, a large percen age o hese obs ruc ions will resolve on heir own.

Examination Diagnosis is based mainly on he his ory. Examina ion may reveal increased ear f lm and some crus ing o he eyelashes. Mucus re ux wi h pressure over he lacrimal sac conf rms he diagnosis, bu is no always presen . Examina ion mus rule ou a dacryocys ocele or any sign o in ec ion ( Fig. 8-1).

Epidemiology and Etiology Age: Congeni al Gender: Equal in males and emales E iology: Incomple e developmen o he dis al lacrimal passage wi h a membranous block a he valve o Hasner

Dif erential Diagnosis Chronic conjunc ivi is Punc al dysgenesis En ropion richiasis

History Paren s will no e a chronic mucous discharge wi h mat ing o he eyelashes a 3 o 4 weeks o age in 2% o 6% o ull- erm in an s in one or bo h eyes.

Treatment iming o he rea men is con roversial. Nine y percen o all congeni al nasolacrimal duc obs ruc ions will resolve by age 12 mon hs.

C

126

Congenital Obstructions

Many physicians will use conserva ive rea men un il his ime. T is managemen consis s o massage wi h opical an ibio ics as needed o con rol he mucus discharge. Probing and irriga ion under general aneshesia will success ully rea 90% o pa ien s. T ose pa ien s no responsive o probing and irriga ion may require in uba ion wi h silicone ubes wi h or wi hou a balloon dacryoplas y.

127

T e rare pa ien will require a dacryocys orhinos omy. Prognosis rea men is very success ul. Wai ing or spon aneous resolu ion while he child has chronic discharge is o en di cul or he caregivers.

FIGURE 8-1. Congenital nasolacrimal duct obstruction. T ere is redness, crusting, and irritation o the right eyelids rom the chronic discharge. T e tear f lm is also increased. In many patients with congenital nasolacrimal duct obstruction, there will be no external signs and the diagnosis is based on the history the caregivers report.

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8 LACRIMAL O BSTRUCTIO NS

DACRYOCYSTOCELE

A

dacryocys ocele is a rare lesion no ed a bir h in he medial can hal area. I represen s uid and mucus rapped in he lacrimal sac. Dacryocys oceles will resolve bu mus be observed care ully because hey can become in ec ed. Epidemiology and Etiology Age: Congeni al Gender: Equal in males and emales E iology: Blockage o he lacrimal sys em dis ally, a he valve o Hasner, and proximally, a he valve o Rosenmüller, resul ing in rapped amnio ic uid and/ or mucus produced by he lacrimal sac goble cells.

I a mass is no ed above he medial can hal endon, ano her e iology mus be considered. I bila eral consider nasal exam and chance o respira ory obs ruc ion. Dif erential Diagnosis Hemangioma Meningoencephalocele Dacryocys i is

History Cys ic swelling o he medial can hus below he endon no ed a bir h

Treatment Observa ion or he f rs 1 o 2 weeks wi h massage Many will resolve on heir own. Probing is required i here is any sign o in ec ion or i here is no resolu ion a er 2 weeks.

Examination Prominen cys ic mass below he medial can hal endon ( Fig. 8-2)

Prognosis Excellen

FIGURE 8-2. Dacryocystocele. T e large, distended right lacrimal sac is easily seen and is f rm to palpation. T is child underwent probing and irrigation, which resolved the obstruction.

Congenital Obstructions

LACRIMAL FISTULA

A

lacrimal f s ula is an ex ra opening o he lacrimal sys em on o he skin usually loca ed in erior-nasal o he lacrimal punc um. One hird o f s ulas will have an associa ed lacrimal obs ruc ion wi h chronic mucous discharge. T e o her pa ien s are o en asymp oma ic. Epidemiology and Etiology Age: ypically congeni al, bu acquired f sulas can occur a an age. Gender: Equal in males and emales E iology: Abnormal embryonic developmen o he lacrimal sys em. Cases o acquired f s ulas are rela ed o dacryos enosis wi h dacryocys i is. History O en asymp oma ic unless here is an associa ed dacryos enosis

129

Examination Small cu aneous opening in erior and nasal o he medial can hal angle May or may no have ears exi ing rom i ( Fig. 8-3) Dif erential Diagnosis Mus de ermine i here is associa ed dacryos enosis Treatment I symp oma ic, he epi helial lined is ula can be excised. I here is also an associa ed dacryos enosis, a dacryocys orhinos omy and excision o he is ula is indica ed. Acquired f s ulas will disappear when he dacryocys i is resolves. Prognosis Excellen

FIGURE 8-3. Congenital lacrimal f stula. Note the very small opening in erior-nasal to the puncta, which is connected to the lacrimal system.

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8 LACRIMAL O BSTRUCTIO NS

ACQUIRED OBSTRUCTIONS ACQUIRED NASOLACRIMAL DUCT OBSTRUCTION

A

cquired nasolacrimal duc obs ruc ion becomes more common as pa ien s age. T e obs ruc ion mos commonly occurs in he nasolacrimal duc . Pa ien s may presen wi h earing or an in ec ion. Many pa ien s may have an obs ruc ion and be wi hou symp oms. Epidemiology and Etiology Age: Older pa ien s Gender: Females mos commonly E iology: Involu ional changes in he lacrimal duc / sac is he mos common cause. Naso-orbi al rauma or surgery, sinusi is, and dacryocys i is are also causes. History Con inual earing, which may have been preceded by in ermit en episodes o earing. T e process is mos commonly unila eral bu may be bila eral. Examination Increased ear f lm on sli lamp examinaion wi h abnormal dye disappearance es

Def ni ive diagnosis made wi h irriga ion o he lacrimal sys em, which will demons ra e obs ruc ion o ow ( Fig. 8-4). Dif erential Diagnosis O her causes o earing such as: Kera i is sicca Blephari is Ec ropion Punc al abnormali ies Special Tests Dacryocys ography may help def ne lacrimal s enosis in di cul cases and in par ial obs ruc ions. Treatment Symp oma ic comple e obs ruc ion requires a dacryocys orhinos omy. Par ial obs ruc ions can be rea ed wi h balloon dacryoplas y. Prognosis Dacryocys orhinos omy is success ul 90% o he ime or more o en. Balloon dacryoplas y is 70% o 80% success ul bu is less invasive.

Acquired Obstructions

131

FIGURE 8-4. Acquired nasolacrimal duct obstruction. T ere are o en no external signs o acquired nasolacrimal duct obstruction. T ere are excess tears running down the cheek and slight injection o the right eye. I there is some dacryocystitis associated with the blockage, the eye may be red.

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8 LACRIMAL O BSTRUCTIO NS

CANALICULAR OBSTRUCTION Epidemiology and Etiology Age: Any Gender: More common in emales E iology: rauma, ex ernal conjunc ival in ec ions (Epidemic Kera oconjunc ivi is, herpes), canaliculi is, sys emic chemo herapy History Onse o earing may be gradual or acu e. Examination Increased ear f lm, normal eyelid posi ion, and evidence o canalicular obs ruc ion on probing o he canaliculi ( Fig. 8-5)

Special Considerations T ere may be an addi ional, more dis al, obs ruc ion in he lacrimal sac and duc in some o hese cases. Dif erential Diagnosis Kera i is sicca Blephari is Ec ropion O her lacrimal sys em abnormali ies Treatment Silicone in uba ion wi h or wi hou a dacryocys orhinos omy Prognosis Canalicular obs ruc ions have a poorer prognosis han more dis al obs ruc ions. Success is in he range o 50%, depending on he e iology.

Acquired Obstructions

133

FIGURE 8-5. Canalicular obstruction. External signs o herpes simplex are the only sign o the canalicular obstruction. Probing demonstrates canalicular scarring as a result o herpes simplex.

C H AP T ER

Lacrimal In ec ions DACRYOCYSTITIS Epidemiology and Etiology Age: Mos common in older adul s bu can be seen a any age. Gender: More common in emales E iology: Nasolacrimal obs ruc ion rom various causes wi h s asis o f uid in he lacrimal sac and even ual in ec ion. History May have acu e onse o pain and swelling over he lacrimal sac. O hers may give he hisory o chronic earing wi h chronic mucous discharge and a ender lump over he lacrimal sac. T ere may be a prolonged his ory o a chronic conjunc ivi is. Examination enderness over he lacrimal sac is he mos common nding. T e lacrimal sac may be enlarged wi h signi can swelling, or i may be rela ively small ( Fig. 9-1A). Similarly, he amoun o periorbi al swelling varies wi h he severi y o he in ec ion. 134

Orbi al celluli is mus be considered i he in ec ion is severe ( Fig. 9-1B). Pa ien s wi h a low-grade chronic in ec ion may have mucus/ pus expressible hrough he canaliculi wi h pressure over he lacrimal sac. T e conjunc iva may be injec ed. Probing and irriga ion should no be done in he set ing o an in ec ion. Special Considerations I a pa ien complains o blood expressed rom he lacrimal sys em, a lacrimal sac umor mus be considered and imaging done. In ec ions can give bloody discharge as well. Dif erential Diagnosis Lacrimal sac umor Laboratory Tests Cul ure and sensi ivi y o any ma erial expressed or drained rom he lacrimal sac Imaging C or MRI scanning may be needed i a lacrimal sac umor is suspec ed.

Dacryocystitis

135

I here is a ormed abscess o he sac, incision and drainage is indica ed.

Rare pa ien s will have an open lacrimal sys em a er he in ec ion is gone and will no require a dacryocys orhinos omy. Pa ien s who have had a dacryocys i is and have an obs ruc ed lacrimal sys em have an increased risk o recurren dacryocys i is.

Ul ima ely, when he in ec ion has resolved, mos pa ien s will require a dacryocys orhinos omy.

Prognosis Excellen unless he pa ien is immunocompromised

Treatment rea men o he acu e in ec ion is he rs priori y. Sys emic an ibio ics and warm compresses are he rea men o choice.

136

9 LACRIMAL INFECTIO NS

A

B FIGURE 9-1. Dacryocystitis. A.T is 68-year-old man has a ormed lacrimal sac mass that is tender with a surrounding mild cellulitis. B. A more severe dacryocystitis with surrounding cellulitis.

Canaliculitis

CANALICULITIS

C

analiculi is is a rare in ec ion involving he proximal lacrimal sys em. T e in ecion can be bac erial or ungal and is usually indolen . Diagnosising canaliculi is can be di cul because i o en presen s as a chronic conjunc ivi is and no un il la e does he lacrimal in ec ion become apparen . Epidemiology and Etiology Age: Usually older adul s E iology: Some abnormali y o he lacrimal sys em leads o concre ion orma ion and a chronic in ec ion. In ra-canalicular plugs have become a more common cause o canaliculi is. History Chronic mucous discharge, earing, and conjunc ivi is unresponsive o opical an ibio ics Ask abou any his ory o punc al plug placemen and he ype o plug placed. Examination T e diagnosis can be di cul o con rm unless i is suspec ed. An ery hema ous, pou ing, dila ed puncum, which is o en ender o palpa ion and very ender o probing, is o en presen .

137

T ere may be a ollicular conjunc ivi is and a chronic mucous discharge. Pressure over he canaliculus may express pus or concre ions ( Fig. 9-2A and B). T ere is more likely o be enderness over he canaliculus wi h an in ec ious e iology and less likely when rela ed o re ained puncal plugs ( Fig. 9-2C). Dif erential Diagnosis Chronic conjunc ivi is Migra ed punc al plug Laboratory Tests Cul ure and sensi ivi y o ma erial in canaliculus is help ul in de ermining rea men . Treatment Warm compresses and opical and sysemic an ibio ics are he ini ial rea men . Mos pa ien s will have concre ions or a plug in he canaliculus and he process will recur un il hese are removed wi h incision and drainage o he canaliculus. Prognosis Good once recognized. A second obs rucion lower in he lacrimal sys em may resul in a recurrence.

138

9 LACRIMAL INFECTIO NS

A

B FIGURE 9-2. Canaliculitis. A. A red, tender upper canaliculus with expression o pus with pressure over the canaliculus. B.T e lacrimal stones ound on opening the canaliculus. ( continued)

Canaliculitis

C FIGURE 9-2. (Continued) Canaliculitis. C. Intra-canalicular plug removed af er causing canaliculitis.

139

C H AP T ER

Lacrimal Sac umors

L

acrimal sac umors are rare and he e iology is widely varied rom benign o malignan . Any dacryos enosis or dacryocys i is has he po en ial o be a lacrimal sac umor. When

Classically, he mass may be above he medial can hal endon bu early in he course may presen like a dacryocys i is. Examination Findings vary rom being iden ical o dacryocys i is o a palpable mass in he lacrimal sac area. T e umor may be ound during dacryocys orhinos omy when here was no evidence o a umor preopera ively. I a umor is suspec ed, nasal examina ion by an o olaryngologis may help def ne i s ex en , along wi h C and/ or MRI scanning ( Fig. 10-1). Dif erential Diagnosis Dacryocys i is Laboratory Tests Biopsy o he lacrimal sac or any abnormal appearing lacrimal sac. Dacryocys ogram may be help ul. Imaging C or MRI scanning is needed i a lacrimal sac umor is suspec ed. I may no be able o di eren ia e a umor rom an enlarged sac

140

Lacrimal Sac Tumors

141

secondary o in ec ion bu will show a large erosive mass.

Care ul long- erm ollow-up is impor an or any lacrimal sac umor.

Treatment Comple e excision o any benign or malignan umor is impor an . Frozen sec ion con rol is required o ry o assure comple e excision. Benign papillomas may recur wi h malignan rans orma ion. Lymphomas are sensiive o irradia ion.

Prognosis Recurrence is no uncommon. Fi y percen o ransi ional and squamous cell carcinomas will recur, and 50% o hese recurrences will be a al.

A

B FIGURE10. 1 Lacrimal sac tumor. A.T e patient has ullness o the lef lacrimal sac area and bloody discharge. B. An axial C scan showing a mass in the lacrimal sac ossa, which was a lymphoma on biopsy.

C H AP T ER

Orbi al In ec ions ORBITAL CELLULITIS

O

rbi al celluli is is a real oph halmic emergency ha needs promp recogni ion and rea men . T e in ec ion can progress rapidly over a ew hours in severe cases wi h po en ial li e- hrea ening complica ions.

Epidemiology and Etiology Age: All ages Gender: Equal incidence in males and emales E iology: Sinusi is is he mos common cause bu o her causes include skin in ecions or skin wounds, den al in ec ions, and dacryocys i is. History One o 3 days o progressive swelling around he eye T e process may be preceded by an upper respira ory in ec ion. T e pa ien may have a his ory o sinus in ec ions. 142

Examination Ery hema, swelling, chemosis, res ric ed mo ili y, pain on eye movemen , and prop osis charac erize orbi al celluli is. T ese symp oms are progressive over 24 o 48 hours. As he in ec ion advances, vision can be af ec ed. Pa ien s may or may no have a ever and leukocy osis. I is very impor an o make he dis incion be ween he signs o orbi al celluli is and presep al celluli is where here is jus swelling and redness o he eyelids ( Fig. 11-1). Imaging C scanning is no required o make he diagnosis o orbi al celluli is bu is needed o look or he source o in ec ion (e.g., sinusi is, orbi al abscess) and o rule ou o her processes such as an orbi al umor. A C scan will show sinusi is, which can require drainage. Orbi al oreign bodies or an orbi al abscess can require addi ional surgery.

Orbital Cellulitis

Special Considerations Aggressive and promp rea men o orbi al celluli is is required o preven poserior ex ension o he in ec ion, which can resul in cavernous sinus hrombosis, which is li e- hrea ening. Dif erential Diagnosis Presep al celluli is Orbi al pseudo umor Orbi al abscess Phycomycosis Me as a ic orbi al umor

143

Laboratory Tests Comple e blood coun : Whi e coun may be normal. Blood cul ures are o ques ionable value. Treatment Immedia e broad-spec rum IV an ibio ics, orbi al imaging, and care ul moni oring or improvemen in he rs 24 o 48 hours. Prognosis Good. Rare complica ions rom developmen o an abscess or cavernous sinus hrombosis.

A FIGURE 11-1. Preseptal cellulitis. A. Child with a scratch on the lateral lef upper eyelid that resulted in preseptal cellulitis 2 days later. Ocular motility is normal. T e patient responded to antibiotics within 48 hours. ( continued)

144

11 O RBITAL INFECTIO NS

B

C

D FIGURE 11-1. (Continued) Preseptal cellulitis. B. Early cellulitis related to a subconjunctival abscess that required drainage and oral and topical antibiotics. C–F. Patient with 2 days o swelling o the lef eye with orbital cellulitis. T e eye is swollen shut but, with lif ing, the eyelid ocular motility is limited and there is chemosis. T e patient responded with improvement in 48 hours on IV antibiotics. ( continued)

Orbital Cellulitis

145

E

F

G FIGURE 11-1. (Continued) Preseptal cellulitis. G. C scan shows proptosis and sinusitis and is consistent with the clinical diagnosis o orbital cellulitis.

146

11 O RBITAL INFECTIO NS

ORBITAL ABSCESS

A

n orbi al abscess is a rare complica ion o sinusi is and orbi al celluli is. Orbi al celluli is ha does no improve on broadspec rum IV an ibio ics needs care ul imaging o look or an orbi al abscess. Epidemiology and Etiology Age: Any Gender: Equal E iology: Sinus disease is he mos common source o a subperios eal abscess. Rarely, an orbi al oreign body can be he cause and mus be suspec ed i he abscess is in raorbi al (in raconal). History Orbi al celluli is wi h no sign o improvemen on appropria e an ibio ics Examination Signs are hose o orbi al celluli is ha do no improve on appropria e IV an ibio ics. T e globe may be displaced away rom he abscess. T e abscess is diagnosed on C scanning ( Fig. 11-2).

Imaging C scanning will demons ra e a subperios eal opaci y usually adjacen o an in ec ed sinus. Rarely, he abscess may be in raconal. Dif erential Diagnosis Orbi al celluli is Phycomycosis Cavernous sinus hrombosis Orbi al pseudo umor Laboratory Tests Comple e blood coun ; cul uring o he abscess con en s. Treatment Mos pa ien s will require immedia e surgical drainage o he abscess and rea men wi h broad-spec rum IV an ibio ics. Some abscesses have been rea ed wi h IV an ibio ics alone and close observa ion in children younger han age 9 years. Prognosis Promp and aggressive rea men usually allows success ul rea men . An orbi al abscess does have he po en ial o resul in visual loss, mo ili y problems, or even severe CNS morbidi y.

Orbital Abscess

147

A

B FIGURE 11-2. Orbital abscess. A. A patient with a 2- to 3-day history o swelling o the lef eye. B.T ere is 5 mm o proptosis and limited motility. ( continued)

148

11 O RBITAL INFECTIO NS

C FIGURE 11-2. (Continued) Orbital abscess. C. C scan shows pan sinusitis with a medial orbital abscess that required surgical drainage. ( continued)

Orbital Abscess

149

D

E FIGURE 11-2. (Continued) Orbital abscess. D. A patient with weeks o a red irritated eye. E. C scan shows an abscess around an old orbital oor implant.

150

11 O RBITAL INFECTIO NS

PHYCOMYCOSIS ( MUCORMYCOSIS)

P

hycomycosis is a rare, o en a al ungal in ec ion ha occurs in very sick, immunocompromised pa ien s, mos commonly in poorly con rolled diabe ics. T is in ec ion s ar s in he nasopharynx or sinuses and secondarily invades he orbi . Aggressive rea men has improved he survival in his o en a al condi ion.

Epidemiology and Etiology Age: Adul s Gender: Equal male and emale occurrence E iology: Fungi invade he orbi rom he sinuses or nose. T e ungi invade blood vessel walls and produce hrombosis, ischemia, and allow spread o he ungi. History Pa ien s o en have a his ory o severe sinus pain and progressive orbi al swelling. T e pa ien s who develop his in ec ion are immunocompromised in some way. T e mos common underlying condi ion is severe diabe es wi h poor con rol bu o hers include malignancy, chemo herapy, and chronic s eroid use. Examination Prop osis is he mos common nding wi h an orbi al apex syndrome. Black eschar in he nasal cavi y is a la e nding and is no a reliable diagnos ic sign. Pa ien s are very sick sys emically ( Fig. 11-3A).

Imaging C scanning will show evidence o sinus disease, which a imes can be very mild ( Fig. 11-3B). MRI wi h gadolinium should be done o look or evidence o ex ension in o he cavernous sinus. Pathology Diagnosis is made on biopsy. Nonsep a e, large branching hyphae ha s ain on hema oxylin and eosin s aining, unlike mos ungi, are ound. Dif erential Diagnosis Orbi al celluli is Orbi al pseudo umor Cavernous sinus hrombosis Laboratory Tests Evalua ion or diabe ic con rol, leukocy e coun Pathophysiology Oppor unis ic ungal in ec ion ha grows in an immunocompromised hos . Treatment Con rol sys emic disease, IV ampho ericin B, and surgical debridemen o necro ic issue, which can involve orbi al exen era ion. Prognosis Poor Depending on s a e o he pa ien ’s sys emic disease, his condi ion can o en be a al. Even i he disease is con rolled, vision is o en los in he af ec ed eye.

Phycomycosis (Mucormycosis)

151

A

B FIGURE 11-3. Phycomycosis. A. A patient with poorly controlled diabetes with a 1-week history o sinusitis. T e patient has a rozen globe and a central retinal artery occlusion. T ere is a dusky erythema o the cheek. B. C scan shows di use sinus disease with orbital involvement. Biopsy o the sinus revealed ungus consistent with phycomycosis.

152

11 O RBITAL INFECTIO NS

ASPERGILLOSIS

A

spergillosis occurs in wo orms. One orm, very similar o phycomycosis, occurs in immunocompromised pa ien s and has a poor prognosis. T e second orm occurs in heal hy pa ien s wi h chronic sinus disease and allergies. T is orm has a good prognosis. Epidemiology and Etiology Age: Adul s Gender: Equal male and emale incidence E iology: An oppor unis ic in ec ion ha grows in he sinuses and secondarily invades he orbi . I can occur in wo orms. One orm ac s like phycomycosis and hus occurs in immunocompromised hos s. T e second, “allergic” orm occurs in immune compe en hos s wi h chronic sinus disease and allergies. T e sinus is lled wi h mucin and ungus and may have bone erosion. History Aspergillosis occurring in immunocompromised hos s presen s similar o phycomycosis. T e allergic orm will presen as chronic sinus problems bu will invade he orbi wi h ime in 17% o pa ien s, resul ing in orbi al signs depending on he sinus involved. Examination Findings on examina ion are dependen on he orm o in ec ion. T e presen a ion o aspergillosis in he immunocompromised hos is he same as ha o phycomycosis and is only dif eren ia ed on biopsy. T e allergic orm will only presen wi h orbi al ndings in he minori y o cases. T e signs vary rom displacemen o he globe o an orbi al apex syndrome, depending on he loca ion o he in ec ion and exac direc ion o invasion ( Fig. 11-4). Imaging C scan will show sinus disease wi h secondary orbi al invasion.

MRI can be help ul in de ning ex en o he disease in he orbi and looking or possible CNS ex ension. T e allergic orm shows he sinus lled wi h mot led area o increased at enua ion on nonenhanced C scan. T ere may be areas o bone remodeling and even erosion. MRI imaging shows a signal void on 2 images. Pathology Diagnosis is made by biopsy. Sep a e branching hyphae o uni orm wid h are seen on Gomori’s me henamine silver s aining. Dif erential Diagnosis Sinusi is wi h mucocele Phycomycosis Me as a ic orbi al umor Laboratory Tests Immunocompromised pa ien s will have associa ed blood nding such as ke oacidosis, leukopenia, and so or h, depending on he e iology o he immunode ciency. Pa ien s wi h allergic orm may have a peripheral blood eosinophilia, eleva ed o al immunoglobulin E, and posi ive allergy skin es ing or ungus. Treatment Immunocompromised pa ien s wi h aspergillosis are rea ed he same as phycomycosis (see previous discussion). Cleaning ou he mucin and ungus rom he af ec ed sinus and orbi de ni ively rea s he allergic orm. Prognosis Poor in he immunocompromised orm Good in he allergic orm wi h appropria e rea men

Aspergillosis

153

A

B

C FIGURE 11-4. Aspergillosis. A. A 45-year-old patient with loss o vision in the lef eye and very mild proptosis on the lef . T ere are no other orbital signs. B. C scan shows a large mass o the sphenoid sinus with erosion into the cavernous sinus. C. On MRI, the central area o signal void is classic or aspergillosis. T is mass was simply cleaned out via transnasal sinus surgery and the vision returned to normal.

C H AP

ER

Orbi al Inf amma ion THYROID-RELATED OPHTH ALMOPATHY hyroid-rela ed oph halmopa hy ( RO) is he mos common cause o prop osis in adul s. T e disease can range rom mild eyelid re rac ion o severe prop osis wi h op ic nerve compression and corneal exposure. Early in he disease course, RO can be di cul o diagnose bu la er he ocular signs become classic. Epidemiology and Etiology Age: Rare in children, mainly adul s Gender: Women a ec ed ve o eigh imes more o en han men E iology: Poorly unders ood au oimmune inf amma ory process ha a ec s he eyelid and orbi al issues History Ini ial onse o nonspeci c ocular irri a ion ollowed by eyelid re rac ion, lid lag, eyelid swelling, and bulging o he eyes. Pa ien s will no e symp oms o be worse in he morning and improve over he day. Many pa ien s will have he his ory o a sys emic 154

hyroid imbalance, bu up o 30% may be eu hyroid a he onse o symp oms. Examination T e earlies signs o RO are very nonspeci c and i can be di cul o make he diagnosis early in he disease. Eyelid re rac ion and eyelid lag are also early signs ha will help con rm he diagnosis. As he disease progresses, chemosis, prop osis, and mo ili y res ric ion wi h diplopia will become apparen . La e signs are decreased vision rom op ic nerve compression and severe corneal exposure ( Fig. 12-1A–F). Imaging C scan will show enlargemen o he rec us muscles wi h endon sparing. T e in erior rec us is he mos commonly involved muscle ollowed by medial rec us and superior rec us. T e la eral rec us is rarely involved. C scan is no needed o make he diagnosis o RO, as his is a clinical diagnosis.

T yroid-Related Ophthalmopathy

C scanning is help ul o con rm unusual cases, evalua e op ic nerve compression, and be ore surgery or irradia ion ( Fig. 12-1G, H). Special Considerations T e course and severi y o disease is widely variable. Pa ien s may have a ew mon hs o mild inf amma ion wi hou any sequelae, whereas o hers can have severe inf amma ion ha can lead o severe prop osis, double vision, and visual loss over a ew mon hs or years. Pa ien s who smoke have a longer and more severe course. Dif erential Diagnosis Orbi al pseudo umor Orbi al celluli is Orbi al lymphoma Laboratory Tests T yroid-s imula ing hormone Pathophysiology Chronic inf amma ory process leads o deposi ion o glycosaminoglycans in he muscles and orbi al a wi h even ual scarring and dys unc ion o hese issues. Treatment Limi ing he inf amma ion will limi he scarring and severi y o he disease. Sys emic s eroids will decrease inf amma ion, bu because o he side e ec s rom

155

long- erm use, hey are usually limi ed o use as a emporary, shor - erm rea men . Orbi al irradia ion in some pa ien s is e ec ive a s opping he progression o he disease bu no e ec ive a reversing any o he changes ha have occurred. Any pa ien wi h signi can , ac ive disease is a po en ial candida e or irradia ion (excep pa ien s wi h diabe ic re inopa hy). T e use o orbi al irradia ion is con roversial. S eroid injec ions in o he orbi and a shor course o high-dose IV s eroids are o her rea men s. Immune modula ors such as ri uximab are also being explored o s op his au oimmune process. A er he inf amma ory phase is over, surgical correc ion o residual prop osis, diplopia, and eyelid de ormi ies can be considered. T is is done via a combina ion o orbi al decompression and eye muscle and eyelid surgery. Pa ien s presen ing wi h severe inf amma ion and an op ic neuropa hy or corneal decompensa ion can require an urgen orbi al decompression. Prognosis Good, bu some pa ien s may require mul iple surgical procedures over years as par o he rea men . Pa ien s wi h signi can disease o en have a prolonged course o rea men .

156

12 O RBI AL INFLAMMA IO N

A

B

C FIGURE 12-1. T yroid-related ophthalmopathy. A. A patient with very early thyroid-related ophthalmopathy with slight lid retraction on the lef . B. In down gaze, there is eyelid lag. C. A 20-year-old patient with severe proptosis, eyelid retraction, and corneal exposure. ( continued)

T yroid-Related Ophthalmopathy

157

D

E

F FIGURE12- 1. (Continued) T yroid-related ophthalmopathy. D–F. A 45-year-old patient with progressive swelling o the eyes with double vision and recent decreased vision. T ere is proptosis, chemosis, and limitation o motility. Vision was 20/ 80 rom optic nerve compression. ( continued)

158

12 O RBI AL INFLAMMA IO N

G

H FIGURE12- 1. (Continued) T yroid-related ophthalmopathy. G and H. C scans show enlargement o all rectus muscles with crowding at the orbital apex. T e patient required an orbital decompression and her vision returned to normal. ( continued)

T yroid-Related Ophthalmopathy

I

J FIGURE12- 1. (Continued) T yroid-related ophthalmopathy. I. A patient with severe thyroid-related ophthalmopathy. J.A f er 3 years and multiple surgeries, there is signi cant improvement.

159

160

12 O RBI AL INFLAMMA IO N

IDIOPATHIC ORBITAL INFLAMMATION (ORBITAL PSEUDOTUMOR) Epidemiology and Etiology Age: Children and adul s Gender: Equal incidence in males and emales E iology: T is inf amma ory process is by de ni ion unrela ed o any sysemic abnormali y and he cause remains unknown. History Acu e onse o orbi al pain o en associa ed wi h prop osis, ery hema, swelling, and res ric ed eye movemen s. T e symp oms depend on he exac locaion o he process bu pain is common o all presen a ions. Adul s more commonly have unila eral disease bu in children his can be a bila eral process. Examination T e acu e inf amma ory process can occur an eriorly and presen wi h acu e ery hema and swelling o he lids and globe. I may presen as a myosi is wi h res ric ed mo ili y and pain wi h eye movemen , as a scleri is, a dacryoadeni is, or in he orbi al apex wi h ew ex ernal signs bu signi can pain, dysmo ili y, and decreased vision. T e presen a ion is variable depending on he issues a ec ed. Pa ien s wi h orbi al pseudo umor can have a ever and a leukocyosis ( Fig. 12-2). Imaging C scanning will show hickening o he a ec ed issues such as enlarged muscles, hickened sclera, enlarged lacrimal gland, or an in l ra e in he orbi al a .

Special Considerations Rarely, here may be very ew inf ammaory signs and a more chronic bro ic process ha is ermed sclerosing inf amma ory orbi al pseudo umor. T is condi ion is no very responsive o rea men as rea men is geared oward elimina ing inf amma ion and here is very lit le inf amma ion in his process. Sys emic condi ions, such as sarcoidosis, may cause a very similar pic ure. Dif erential Diagnosis Orbi al celluli is T yroid-rela ed oph halmopa hy Lymphoma Sarcoidosis Wegener’s Granuloma osis Rup ured dermoid cys Me as a ic disease Laboratory Tests Pa ien s may have a leukocy osis, peripheral blood eosinophilia, eleva ed ESR, and a posi ive ANA. None o hese are diagnos ic. Pathophysiology A pleomorphic cellular inf amma ory response occurs and i no rea ed or no responsive o rea men here will be a resulan bro ic response ha will progress wi h ime and resul in chronic scarring. Treatment Sys emic s eroids are he mains ay o rea men . T ere should be an improvemen in symp oms in 24 o 48 hours. T e longer he process has been presen , he longer i can ake or a clinical response. A er here is a good clinical response, he s eroids are apered over 4 o 6 weeks. Pa ien s wi hou response o s eroids or wi h mul iple recurrences o he inf amma ion

Idiopathic Orbital Inf ammation (Orbital Pseudotumor)

require an orbi al biopsy o con rm he diagnosis. I con rmed, hey are hen candida es or orbi al irradia ion. Immune suppressive agen s may also be used in some pa ien s wi h recurrences.

161

Prognosis Excellen prognosis in mos acu e cases. T ere may be recurrences. Cases ha are chronic wi h less inf ammaory response are less responsive o rea men and can be progressive.

A

B FIGURE 12-2. Orbital pseudotumor. A. A 33-year-old man with a 5-day history o swelling, erythema, and pain that is worse with eye movement. T e eye is red with orbital swelling and tenderness to palpation. Eye movements are limited by pain. B.D i use in ltration o the orbit and slight enlargement o the medial rectus. T e clinical presentation along with the C scan are consistent with orbital pseudotumor. T e patient responded within 24 hours to oral prednisone. ( continued)

162

12 O RBI AL INFLAMMA IO N

C

D FIGURE 12-2. (Continued) Orbital pseudotumor. C.T is is a scleritis: Scleritis with some anterior orbital swelling. D.D i use scleral thickening on lef side. ( continued)

Idiopathic Orbital Inf ammation (Orbital Pseudotumor)

163

E

F FIGURE 12-2. (Continued) Orbital pseudotumor. E. C shows a di use enlargement o the lateral rectus muscle consistent with a myositis. T e patient had limited adduction and abduction as well as pain with eye movement. F. C scan showing in ammation at the orbital apex. On examination, the eye may be white and quiet with minimal proptosis. T ere is of en decreased vision and motility dys unction consistent with an orbital apex syndrome.

164

12 O RBI AL INFLAMMA IO N

SARCOIDOSIS

S

arcoidosis can occur in he orbi in mul iple orms and wi h varying amoun s o inf amma ion.Mos commonly,i presen s wi h lacrimal gland enlargemen wi h very mild inf amma ory signs. Sarcoidosis may have a much more acu e swelling and can a ec he sclera, ex raocular muscles, or o her orbi al issues. Epidemiology and Etiology Age: Any age bu mos common in adul hood Gender: Equal E iology: Mul isys em inf amma ory disease ha occurs primarily in individuals o A rican and Scandinavian descen . History Mos commonly presen s wi h lacrimal gland enlargemen wi h varying amoun o inf amma ory signs Examination Bila eral lacrimal gland enlargemen is he mos common presen a ion. Ex raocular muscles, he op ic nerve, and eyelid skin are less commonly a ec ed. Inf amma ion in adjacen sinuses may secondarily a ec he orbi . T e en ire eye mus be evalua ed or signs o sarcoidosis causing uvei is (an erior or pos erior), iris nodules, or re inal vascular changes. Conjunc ival granulomas as well as sarcoid skin lesions can help con rm he diagnosis ( Fig. 12-3).

Imaging C scanning will show enlargemen o he lacrimal gland, muscle, or o her a ec ed s ruc ure.

Ches x-ray or a ches C is needed o evalua e he lungs or possible pulmonary sarcoidosis. Special Considerations Mos pa ien s wi h sarcoidosis will have sys emic disease wi h pulmonary ndings. Some pa ien s will have he disease isola ed o he orbi wi hou sys emic indings. Dif erential Diagnosis Idiopa hic orbi al pseudo umor Dacryoadeni i is Laboratory Tests Angio ensin-conver ing enzyme may be helpul in es ablishing he diagnosis. Treatment A biopsy o he a ec ed issue is usually required o con rm he diagnosis o sarcoidosis. When presen , a conjunc ival nodule is simple o biopsy. O herwise, he a ec ed issue is biopsied. A er he diagnosis is es ablished, careul sys emic evalua ion is needed o look or sarcoid. rea men is mos commonly sys emic prednisone al hough o her immunosuppressive agen s have been used. rea men is usually aimed a disease conrol whe her i is or con rol o orbi al inf amma ion or o con rol pulmonary disease. Prognosis Mos pa ien s do well, bu rare pa ien s can have signi can sys emic mani es a ions. T e orbi al disease can be chronic and recurren .

Sarcoidosis

165

A

B FIGURE 12-3. Orbital sarcoidosis. A and B. A patient with proptosis and double vision with some mild aching o the right eye. Motility shows poor adduction o the right eye. ( continued)

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C

D FIGURE 12-3. (Continued) Orbital sarcoidosis. C. C scan shows an enlarged medial rectus muscle. T is myositis responded to oral prednisone but recurred. Biopsy showed sarcoidosis. D. Bilateral lacrimal gland enlargement on the external photograph. ( continued)

Sarcoidosis

167

E

F

G FIGURE 12-3. (Continued) Orbital sarcoidosis. E and F. Axial and coronal C scans show enlarged lacrimal glands. Biopsy showed sarcoidosis. G.I n ltration o the eyelid and anterior lacrimal tissue by sarcoidosis. Under the eyelid, the in ltration is yellow-brown with prominent blood vessels.

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WEGENER’S GRANULOMATOSIS

W

egener’s granuloma osis can involve he eye and orbi as a secondary ex ension rom he sinuses or i can presen involving he eye i sel wi h scleri is, kera i is, uvei is, and so or h. T e sys emic disease can be li ehrea ening and he ocular involvemen can cause blindness and loss o he eye. Epidemiology and Etiology Age: Mainly adul s Gender: Equal occurrence in males and emales E iology: A sys emic necro izing granuloma ous vasculi is ha classically a ec s he upper and lower respira ory rac and can a ec he small vessels o any major organ sys em. History Diagnosis may or may no already be made when he pa ien presen s wi h eye ndings. Mos commonly, here is bony erosion via ex ension o he disease in o he orbi rom he sinus cavi y. Pa ien s can also have a necro izing scleriis, which can be severe.

Examination Findings include scleri is, which may be an erior or pos erior and is o en necro izing. Prop osis wi h or wi hou orbi al inf amma ion may be presen ( Fig 12-4A). Imaging C scans show bone erosion rom sinus ex ension o he disease ( Fig 12-4B and C). Dif erential Diagnosis Malignan umor o he sinus Laboratory Tests An ineu rophil cy oplasmic an ibodies (speci cally c-ANCA) are o en presen wi h Wegener’s disease. Pathology Vasculi is, granuloma ous inf amma ion, and issue necrosis are ound on pa hologic evalua ion. Treatment Immunosuppressive medica ion, speci cally cor icos eroids and cyclophosphamide, is he rea men o choice. Prognosis Variable. T e disease can be progressive and a al.

A FIGURE 12-4. Wegener’s granulomatosis. A. A patient with orbital pseudotumor-like picture. ( continued)

Wegener’s Granulomatosis

169

B

C FIGURE 12-4. (Continued) Wegener’s granulomatosis. B and C. C scans show in ltration along the in erior– medial orbit and into the sinus. Poor response to prednisone and a positive ANCA led to a biopsy, which was consistent with Wegener’s granulomatosis.

C H AP T ER

Congenital Orbital Anomalies MICROPHTH ALMOS

M

icrophthalmos is a de ect in the eye development. T e eye is small and there are usually structural de ects. T e microphthalmos can be mild or the eye can be so small that it cannot be seen at all. Epidemiology and Etiology Age: Congenital Gender: Equal occurrence in males and emales Etiology: Developmental de ect with ailure o the choroidal ssure to close as an embryo. T is results in a small eye with structural abnormalities ( Fig. 13-1) Examination T e eye may be small to virtually nonexistent. T ere are structural abnormalities within the eye and the eye usually has poor or no vision.

170

T ere may be an accompanying cyst, which may be quite large. T is condition is usually unilateral, rarely bilateral. Dif erential Diagnosis Anophthalmos Treatment reatment is aimed at stimulating the orbit to grow and mature normally. I the eye is only slightly small or i there is a large cyst, orbital growth may continue as normal. However, i the eye is very small, expanding con ormers should be used to try to stimulate orbital growth. Dermis at graf s are used sometimes. Prognosis T ese patients of en have some orbital asymmetry even with aggressive treatment. T e cosmetic result is generally acceptable.

Microphthalmos

171

A FIGURE 13-1. Microphthalmos. A. A child wi h microph halmos wi h cys . ( continued)

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13 CO NGENITAL O RBITAL ANO MALIES

B FIGURE 13-1. (Continued) Microphthalmos. B. CT scan shows a small eye and at ached cys . ( continued)

Microphthalmos

173

C FIGURE 13-1. (Continued) Microphthalmos. C. Pa hologic specimen shows he cys ic ou pouching coming from he abnormally developed eye.

C H AP T ER

Orbital Neoplasms CONGENITAL ORBITAL TUMORS DERMOID CYSTS

D

ermoid cysts are relatively common, benign, orbital tumors in children. Classically, they are present at birth, are located superior temporally at the orbital rim, and enlarge with time. Epidemiology and Etiology Age: Congenital and enlarge with age Gender: Equal occurrence in males and emales Etiology: Epidermal elements are le during embryonic development in deeper tissues. T ese epidermal elements then orm a cyst that enlarges with time.

History More superf cial dermoids are o en noted in the f rst 1 to 2 years o li e as they grow and become more noticeable (Fig. 14-1A, B). T e dermoids that are deeper, such as in the orbit, may not become symptomatic until 174

adulthood when they have become large, start to leak, or rupture rom trauma. Examination T e classic location or a superf cial dermoid cyst is at the lateral brow over the rontozygomatic suture. Less commonly, they can be superior medial or even in the lower lid. T ey are smooth, painless masses that slowly enlarge. T ey can be reely mobile or f xed to the bony suture. Deeper dermoids can be in the superior and/ or lateral orbit. “Dumbbell” dermoids occur in the temporal ossa and have a component in the orbit and a part in the temporal ossa. Deeper dermoids present with proptosis or with symptoms o orbital in ammation as the dermoid cyst either leaks or ruptures ( Fig. 14-1C). Imaging C scan: nonenhancing cystic mass (Fig. 14-1D).

Congenital Orbital Tumors

MRI: hypoin ense on 2(Fig. 14-1E).

; hyperin ense on

1

Dif erential Diagnosis When loca ed super icially and emporally, here are very ew lesions his can be con used wi h. Imaging usually helps make he diagnosis i loca ed deep in he orbi . Pathology T e cys is lined by kera inizing epidermis wi h dermal appendages such as hair ollicles and sebaceous glands. T e cys is f lled wi h kera in and oil.

175

Treatment Comple e surgical excision wi h an in ac capsule is he surgery o choice. T is procedure should be done when he po en ial or cys rup ure becomes a risk. T is mos o en occurs when he child begins o walk and be more ac ive. Prognosis Excellen or superf cial dermoids. Good or deep dermoids as long as he en ire cys is removed

A FIGURE 14-1. Dermoid cyst. A. Sof , mobile mass along he superior emporal rim in a 1-year-old pa ien . T is has been presen since bir h. ( con inued)

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B

C FIGURE 14-1. (Con inued) Dermoid cyst. B. Excision o he dermoid hrough a lid crease incision. Ca nd D. Prop osis and globe displacemen caused by a deep orbi al dermoid, which was no ed a age 5 years. T e ossa orma ion caused by hese lesions is seen on he C scan. T e deep orbi al loca ion means hey are of en no no iced un il he child is older. T is was comple ely excised and he pa ien did well wi hou any ur her problems. ( con inued)

Congenital Orbital Tumors

177

D

E FIGURE 14-1. ( Con inued) Dermoid cyst. D. C scan show cys ic lesion wi h bony ossa orma ion. E. MRI o a dermoid cys . On a 2-weigh ed image, he cys is hyperin ense o a and muscle.

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LIPODERMOIDS

L

ipodermoids are congeni al solid umors loca ed emporally below he conjunc iva. T ese are some imes no no ed un il la er in li e. T ey should be le alone in almos all cases.

Dif erential Diagnosis Fa prolapse Lymphoma Prolapsed lacrimal gland

Epidemiology and Etiology Age: Congeni al Gender: Equal occurrence in males and emales E iology: Developmen al anomaly

Pathology Kera inizing squamous epi helium wi h adnexal s ruc ures T e underlying dermis usually con ains a and connec ive issue.

History Presen a bir h and generally does no change wi h ime Examination Yellowish, pink lesion over he la eral sur ace o he globe deep o he conjunc iva ( Fig. 14-2) T ey vary in size and o en have hairs on he sur ace.

Treatment No rea men At emp ed excision can damage he adjacen lacrimal duc s and rec us or leva or muscles. In rare cases when he lipodermoid is very large, he an erior por ion can be debulked leaving he conjunc iva unresec ed.

Imaging I large, C scan will show a mass wi h a densi y.

Prognosis Excellen i le alone

Congenital Orbital Tumors

179

A

B FIGURE 14-2. Lipodermoid. A. Classic loca ion or a lipodermoid, which has been presen since bir h. B. Close inspec ion of en shows hairs on he lesion. Despi e he cosme ic appearance, hese are bes lef alone.

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VASCULAR ORBITAL TUMORS CAPILLARYHEMANGIOMAS

C

apillary hemangiomas are benign umors o he orbi ha appear in he f rs ew weeks o li e and enlarge over he f rs 6 o 12 mon hs. T ey hen end o shrink over ime bu he ini ial presen a ion can be drama ic.

Epidemiology and Etiology Age: No ed in he f rs year o li e Gender: Equal occurrence in males and emales E iology: Abnormal grow h o blood vessels wi h varying degrees o endo helial cell proli era ion History Lesions are o en no ed in f rs ew weeks o li e and hey grow, some imes rapidly, over weeks o mon hs. T ey can presen deeper in he orbi wi h prop osis or more superf cially as an expanding mass. T e hemangioma will hen involu e over mon hs o years. Seven y-f ve percen o lesions will resolve over 4 years. Examination T e lesion appearance is dependen on he loca ion (Fig. 14-3A-C, F, G). T e more common superf cial lesions produce an eleva ed, dimpled, s rawberrycolored lesion. Deeper lesions may give a bluish discolora ion. Deep orbi al lesions may only give sympoms o an expanding orbi al mass.

Di eren ia ion be ween rhabdomyosarcoma and deep capillary hemangioma can only be made wi h biopsy. Imaging C scan reveals a mass ha can be well or poorly margina ed wi h enhancemen wi h con ras ( Fig. 14-3D, E). MRI is hypoin ense on 1 and hyperinense on 2. T e lesion enhances wi h gadolinium. Dif erential Diagnosis Rhabdomyosarcoma Pathology Proli era ion o endo helial cells organized in o a ne work o basemen membrane–lined vascular channels. Treatment T ese lesions will regress so hemangiomas are observed or regression unless hey cause visual obs ruc ion or as igma ism leading o amblyopia. In his case, rea men is required. Orbi al lesions causing severe prop osis may also require rea men . Orbi al biopsy is required i he lesion canno be di eren ia ed rom a rhabdomyosarcoma. rea men op ions include in ralesional s eroid injec ion, sys emic s eroids, or, in selec cases, surgical excision. Recen s udies sugges sys emic propranolol may become he rea men o choice. Care ul moni oring o hese babies on propranolol mus be done by pedia ric cardiology. Prognosis Good

Vascular Orbital Tumors

181

A

B FIGURE 14-3. Capillary hemangioma. A. Subcu aneous capillary hemangioma o he righ eyebrow ha increased in size over 6 mon hs. T e lesion becomes more prominen and red wi h crying. T is lesion resolved over 3 years. B. A small hemangioma on he child’s arm. ( con inued)

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C

D

E FIGURE 14-3. (Con inued) Capillary hemangioma. C.S uper cial orbi al hemangioma ha had increased in size and was causing amblyopia rom 7 diop ers o induced as igma ism. D and E. C scans show his an erior orbi al mass, which is well circumscribed and enhances wi h con ras . T is was excised because o he as igma ism and amblyopia. ( con inued)

Vascular Orbital Tumors

F

183

G

FIGURE 14-3. (Con inued) Capillary hemangioma. F. Large cu aneous capillary hemangioma wi h visual obs ruc ion. G.T is lesion responded well o a series o in ralesional s eroid injec ions.

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CAVERNOUS HEMANGIOMAS

C

avernous hemangiomas can presen as asymp oma ic, very insidious onse prop osis. More commonly, hese lesions presen wi hou any symp oms and are ound on imaging done or unrela ed reasons. T ey are slowly growing masses ha are generally easy o remove depending on heir loca ion.

Epidemiology and Etiology Age: Adul s Gender: Mos commonly middle-aged women E iology: Unknown History Very slow grow h usually means he pa ien is unsure o he onse or dura ion o he lesion. Mos commonly, he presen a ion is prop osis bu rarely here can be symp oms o visual loss. Examination Axial prop osis is he common presen a ion. I he lesion is a he apex or is very large, i can cause op ic nerve compromise ( Fig. 14-4A-C) or s rabismus. Lesions can rarely cause orbi al pain or he appearance o a choroidal mass.

Imaging C scan shows an encapsula ed, homogeneous, round mass wi h variable enhancemen . MRI: isoin ense on 1 and hyperin ense on 2(Fig. 14-4D, E). Marked enhancemen wi h gadolinium Special Considerations Rarely, lesions may grow rapidly during pregnancy. Dif erential Diagnosis Hemangiopericy oma Schwannoma Fibrous his iocy oma Pathology Encapsula ed umor consis ing o large endo helial lined channels wi h abundan , loosely dis ribu ed smoo h muscle in he vascular walls and smoo h muscle. Treatment Surgical excision is he rea men o choice. T ese lesions are easily removed once exposed. T ey do no regress and slowly enlarge so observa ion only delays surgery. Prognosis Excellen

Vascular Orbital Tumors

185

A

B FIGURE 14-4. Cavernous hemangioma. A. A pa ien wi h prop osis o he righ eye o unknown dura ion and no o her visual or orbi al complain s. B. C scan shows a well-circumscribed in raconal orbi al mass. ( con inued)

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C

D FIGURE 14-4. (Con inued) Cavernous hemangioma. C.T e mass was excised and was a cavernous hemangioma. D. MRI o a cavernous hemangioma. T e 1-weigh ed image shows he lesion isoin ense o muscle and hypoin ense o a . ( con inued)

Vascular Orbital Tumors

187

E FIGURE 14-4. ( Continued) Cavernous hemangioma. E. On the T 2-weighted image, the lesion is hyperintense to fat and muscle.

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LYMPHANGIOMAS

L

ymphangiomas are rare vascular hamar omas ha can behave in many di eren ways depending on loca ion and grow h pat erns. T is condi ion can vary rom mild ra her asymp oma ic lesions, o progressively growing, inf l ra ive lesions, o acu e prop osis and visual loss rom bleeding in o hese lesions. Epidemiology and Etiology Age: Usually no ed in he f rs decade o li e Gender: More common in emales E iology: Congeni al lesion History T ese lesions are o en no ed associa ed wi h a spon aneous bleed o he lesion al hough hey likely were presen or years prior. T ey can grow slowly and hen suddenly hemorrhage. Lymphangiomas can mani es as pain, subconjunc ival hemorrhage, or as prop osis. Less commonly, he cys s o hese lesions are no ed subconjunc ivally. T ese lesions enlarge wi h upper respiraory in ec ions. Examination T e f ndings on examina ion are dependen on he loca ion o he lesion. T e mos common presen a ion is associa ed wi h sudden bleeding in o he lymphangioma. I he bleed is superf cial, hen a subconjunc ival bleed is seen and he cys s o he lymphangioma are o en ound ( Fig 14-5A, B). I he hemorrhage is in he orbi , he f ndings may only be prop osis ( Fig 14-5C).

Care ul evalua ion or evidence o a lymphangioma superf cially should be done in hese cases. Imaging will aid in he diagnosis i he lesion is en irely orbi al. Imaging C scan: Poorly circumscribed, he erogeneous mass MRI: Hyperin ense on 1; very hyperinense on 2, wi h possible area o uid and blood ( Fig. 14-5D, E) Special Considerations Surgery per ormed on a lymphangioma increases he chances o spon aneous bleeds wi hin he lesion. Surgery should only be done i absolu ely necessary. Dif erential Diagnosis Diagnosis can usually be made wi h MRI. Pathology Nonencapsula ed mass wi h large serum- illed spaces lined by la endo helial cells T e in ers i ium has scat ered lymphoid ollicles. Treatment Observa ion unless he spon aneous bleeding causes visual loss, corneal exposure, or severe cosme ic disf guremen . Generally, wi h ime he blood will resorb. When an orbi al hemorrhage causes visual loss, drainage o he hemorrhage should be per ormed. Debulking o he lesion or orbi al decompression are o her rea men op ions.

Vascular Orbital Tumors

Lymphangiomas are inf l ra ive so excision is very di cul and here is usually signif can bleeding associa ed wi h excision, which is only per ormed as a las resor .

189

Prognosis Variable depending on he grow h o he lymphangioma Progressive lesions have a high incidence o visual disabili y and poor cosme ic resul .

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A

B FIGURE 14-5. Lymphangioma. A. Pa ien wi h sudden onse o orbi al discom or and prop osis. Medially, a small area o hemorrhage is no ed wi h subconjunc ival cys s consis en wi h a lymphangioma. B. More obvious hemorrhage was no ed along wi h he onse o deep orbi al pain. Mul iple cys s can be seen in he hemorrhage. Imaging was consis en wi h a lymphangioma. ( con inued)

Vascular Orbital Tumors

191

C

D

E FIGURE 14-5. (Con inued) Lymphangioma. C. Prop osis o he lef eye wi h recurren episodes o orbi al pain. T e pain was usually associa ed wi h an increase in he prop osis. D and E. MRIs show a superior orbi al mass wi h area o resh and old blood consis en wi h a lymphangioma.

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HEMANGIOPERICYTOMA

H

emangiopericy oma is a rare lesion ha can mimic a cavernous hemangioma bu has more rapid grow h and is more likely o cause symp oms. T ese can recur and have a chance o me as asis. Epidemiology and Etiology Age: Middle age Gender: Equal occurrence in males and emales E iology: umor origina es rom he pericy e. T is is a rare orbi al umor. History Insidious onse o prop osis and mass e ec bu usually more rapid onse han a cavernous hemangioma Examination Prop osis is o en he only f nding. T ese lesions appear more o en in he superior orbi bu in raconal loca ion is also common ( Fig. 14-6A, B). Imaging C scan: well-circumscribed, encapsula ed mass

MRI: isoin ense on 1; hyperin ense on (Fig. 14-6C, D). Enhances wi h gadolinium

2

Special Considerations T ese lesions have he po en ial or recurrence locally whe her he pa hology is benign or malignan . Malignan lesions can recur and can also me as asize. Dif erential Diagnosis Cavernous hemangioma Fibrous his iocy oma Schwannoma Pathology Uni orm spindle-cell umor wi h a sinusoidal vascular pat ern; can be divided in o benign, in ermedia e, and malignan orms. Treatment Comple e excision in he capsule is he bes rea men . Recurrence, i malignan , may require exen era ion. Prognosis Variable. Pa ien s mus be ollowed or a leas 10 years or local recurrence or me as asis.

Vascular Orbital Tumors

193

A

B FIGURE 14-6. Hemangiopericytoma. A and B. A 55-year-old man presen ed wi h increasing prop osis over 6 mon hs and diplopia. T ere is axial prop osis on he lef and a well-circumscribed mass on C scan. Pa hologic examina ion revealed a hemangiopericy oma. ( con inued)

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C FIGURE 14-6. ( Con inued) Hemangiopericytoma. C. 1-weigh ed coronal MRI wi h con ras and a suppression shows a hemangiopericy oma nex o he righ lacrimal gland. ( con inued)

Vascular Orbital Tumors

195

D FIGURE 14-6. (Con inued) Hemangiopericytoma. D. nex o he righ lacrimal gland.

-weigh ed coronal MRI o he same hemangiopericy oma

2

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ORBITAL VARICES

O

rbi al varices will presen in he 20s and 30s wi h a his ory o years o in ermi en prop osis. T ese lesions can be superf cial and no iceable or deep wi h only prop osis as a sign o he lesion. Mos lesions should be le alone unless here is ex reme orbi al pressure wi h unc ional def ci or a severe cosme ic disf guremen .

Epidemiology and Etiology Age: Usually no ed in he f rs hrough hird decades o li e Gender: Equal occurrence in males and emales E iology: Dila a ion o pre-exis ing venous channels History Pa ien s wi h nondis ensible varices presen wi h recurren episodes o hrombosis and hemorrhage in he lesion ( Fig. 14-7E, F). T is leads o prop osis, pain, mo ili y res ric ion, and even decreased vision. T ese symp oms resolve as he hemorrhage resolves. T e dis ensible varices presen wi h pain, prop osis, and pressure symp oms associa ed wi h s raining, bending orward, or Valsalva ( Fig. 14-7A, B). T e changes in he orbi and lids associa ed wi h his venous dis ension are also no ed. Examination Dis ensible varices are diagnosed easily by having he pa ien s bring heir head in o a dependen posi ion and no e he f lling o he varix.

Nondis ensible varices are more di cul o diagnose. T e pa ien will presen wi h symp oms o an acu e hemorrhage in o he lesion as no ed previously. T ere is generally no ex ernal hemorrhage presen or any sign o a varix in his ype. Imaging C scan may appear rela ively normal or wi h jus a small di use mass on axial cu s. In he dependen posi ion (coronal cu s), he mass will enlarge as he varix f lls wi h blood ( Fig. 14-7C, D). Nondis ensible varices will show a di use mass ha enhances wi h con ras . Dif erential Diagnosis Lymphangioma is he main di eren ial and di eren ia ion rom he nondis ensible varix is no always possible. Pathology Well-def ned venous channels Treatment Conserva ive observa ion in mos cases I a nondis ensible varix bleeds and visual or exposure symp oms require in erven ion, drainage o he blood clo is usually he rea men o choice. Prognosis Variable. Progressive lesions can be disf guring and success ul rea men is di cul .

Vascular Orbital Tumors

197

A

B FIGURE 14-7. Distensible orbital varix. A. A 55-year-old woman wi h a dis ensible varix in her superior medial orbi . B. Valsalva resul s in massive enlargemen and closure o he eye. ( con inued)

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C

D FIGURE 14-7. (Con inued) Distensible orbital varix. C. C scan showing he medial orbi al varix. D.W hen he head is placed in a dependen posi ion or he coronal C , he varix lls wi h blood, accoun ing or he enlargemen o he lesion on he coronal cu s. ( con inued)

Vascular Orbital Tumors

199

E

F FIGURE 14-7. ( Con inued) Distensible orbital varix. E. Nondis ensible varix may be deep in he orbi wi h only a small an erior componen . F.D i use orbi al involvemen wi h mul iple varices.

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ARTERIOVENOUS MALFORMATIONS

A

r eriovenous mal orma ions (AVM) presen wi h variable severi y. All cases involve he connec ion o an ar erial ow in o a venous drainage area such as he cavernous sinus. T ere may be sub le swelling and redness o he eye and orbi or he presen a ion may be severe prop osis, exposure, and in raocular vascular conges ion. Epidemiology and Etiology Age: Older adul s, excep a er rauma, which can occur a any age Gender: Equal occurrence in males and emales E iology: rauma (basal skull rac ure) resul s in high- ow f s ulas Degenera ive vascular process in pa ien s wi h hyper ension and a herosclerosis resul s in a low- ow f s ula. History Abrup onse o prop osis, chemosis, ar erializa ion o he conjunc ival vessels in one eye. T is occurs in a high- ow AVM. High- ow AVMs will have more severe sympoms bu o en have he his ory o head rauma. Low- ow AVMs are in older pa ien s, are slower in onse , and he symp oms are less drama ic. Examination Prop osis, chemosis, dysmo ili y, ar erializa ion o he conjunc ival vessels (corkscrew

pat ern) ( Fig. 14-8A, B), and eleva ed in raocular pressure are seen in AVMs. In high- ow s a es, he re inal vessels are a ec ed wi h venous conges ion. Imaging C scan and MRI show an enlarged superior oph halmic vein and here may be enlargemen o he ex raocular muscles ( Fig. 14-8D and E). Orbi al Doppler shows reversal o ow in he superior oph halmic vein and is diagnosic o an AVM ( Fig. 14-8C). Dif erential Diagnosis Orbi al pseudo umor Orbi al celluli is T yroid-rela ed oph halmopa hy Chronic conjunc ivi is Treatment Low- ow AVMs will o en resolve spon aneously. T e signs may worsen as he f s ula closes o . High- ow lesions o en require at emp ed selec ive emboliza ion o close he f s ula. T is may also be needed in low- ow lesions ha resul in uncon rolled glaucoma, diplopia, or vascular occlusion. Prognosis Variable. Many low- ow AVMs will close on heir own. rea men or AVMs is successul bu does have a risk o visual loss.

Vascular Orbital Tumors

201

A

B FIGURE 14-8. Arteriovenous mal ormation. A and B. A pa ien wi h a 3- o 4-week his ory o swelling and redness o he lef eye. Mo ili y is limi ed as no ed in at emp ed upgaze. (con inued)

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C

D FIGURE 14-8. ( Con inued) Arteriovenous mal ormation. C. Color Doppler imaging shows ar erializa ion o he superior oph halmic vein, which is diagnos ic o an ar eriovenous mal orma ion. D and E. C scan shows enlarged superior oph halmic vein and engorged rec us muscles, which is usually seen wi h an AVM. ( con inued)

Vascular Orbital Tumors

E FIGURE 14-8. ( Con inued)

203

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NEURAL TUMORS OPTIC NERVE GLIOMAS

O

p ic nerve glioma is a glial umor ha mos commonly presen s in children and includes painless prop osis and visual loss. T ese can ini ially involve he op ic chiasm or grow o involve i . rea men remains con roversial.

Epidemiology and Etiology Age: Predominan ly in children during he f rs decade o li e. Malignan gliomas occur in middle-aged males. Gender: Equal occurrence in males and emales E iology: Unknown History In children, gliomas presen wi h gradual, painless, unila eral, axial, prop osis wi h loss o vision and an a eren pupillary de ec . T e malignan orm in adul s presen s wi h symp oms o op ic neuri is bu rapidly progress o blindness and dea h. Examination Axial prop osis wi h visual loss, a eren pupillary de ec , op ic a rophy, or nerve swelling are all f ndings ( Fig. 14-9). T ere are no in amma ory signs or pain. Diagnosis is usually made on he basis o orbi al imaging.

T e malignan orm in adul s may show in amma ory signs along wi h signs o an op ic neuropa hy and prop osis. Imaging C scan demons ra es usi orm enlargemen o he op ic nerve ( Fig. 14-9B). MRI is he imaging o choice o evalua e he ex en and grow h o an op ic nerve glioma. 1 imaging is iso- o hypoin ense, whereas 2 imaging shows prolonged relaxa ion imes ( Fig. 14-9C, D). Special Considerations Neurof broma osis ype 1 is associa ed wi h 25% o 50% o op ic nerve gliomas. Dif erential Diagnosis Op ic nerve meningioma; he di eren ial diagnosis is more rela ed o how ex ensive he umor is and no wha i is. Pathology T ese are in radural lesions ha are juvenile pilocy ic as rocy omas. Treatment Con roversial and mus be individualized. Mos gliomas can be observed because hey are usually very slow-growing lesions. I here is signif can grow h, surgical excision is he bes rea men . I he umor is unresec able, radia ion is considered. Prognosis Variable. Some gliomas grow aggressively; o hers can remain s able or years.

Neural Tumors

205

A

B FIGURE 14-9. Optic nerve glioma. A. A 6-year-old girl wi h painless prop osis and visual loss. B. C scan shows usi orm enlargemen o he op ic nerve consis en wi h an op ic nerve glioma. ( con inued)

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C FIGURE 14-9. (Con inued) Optic nerve glioma. C. enlargemen .

-weigh ed axial MRI shows again shows usi orm

1

( con inued)

Neural Tumors

207

D FIGURE 14-9. (Con inued) Optic nerve glioma. D. cour esy o Ka e Lane, MD.)

-weigh ed axial MRI o he same pa ien . ( C and D

2

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NEUROFIBROMAS

N

eurof bromas are composed o proli era ing Schwann cells wi hin heir nerve shea h. T ere are mul iple orms o neurof bromas. Plexi orm neurof bromas are o en associa ed wi h neurof broma osis ype 1. Epidemiology and Etiology Age: Plexi orm neurof bromas are usually seen in he f rs decade. Isola ed lesions occur in he hird hrough f h decades. Gender: Equal occurrence in males and emales E iology: Plexi orm neurof bromas are he mos common neurof broma o involve he orbi and are associa ed wi h neurof bromaosis ype 1. History Pa ien s will o en already have he diagnosis o neurof broma osis and will develop hickening and hyper rophy o he a ec ed nerve. T ey may presen wi h hickening o eyelid or periorbi al skin, or wi h prop osis. Isola ed neurof bromas are no usually associa ed wi h neurof broma osis. Examination Findings will vary depending on he nerve or nerves ha are involved. T e involved nerves grow as a or uous, ropy angle o nerves. T is grow h is usually slow bu progressive and resul s in hickening o involved periorbi al and orbi al issues, prop osis, and orbi al bony abnormali ies ( Fig. 14-10A). T ese bony changes include orbi al enlargemen , abnormali ies o he sphenoid wing, and hypoplasia o he e hmoid and maxillary sinuses ( Fig. 14-10B). Isola ed neurof bromas presen wi h mass e ec . Prop osis, diplopia, and decreased vision may occur.

Imaging C and MRI show a di use inf l ra ing lesion in plexi orm neurof bromas. An isola ed neurof broma will be wellcircumscribed wi h charac eris ics similar o a schwannoma. Special Considerations Any pa ien wi h a plexi orm neurof broma mus be care ully evalua ed or neurof broma osis i hey are no known o have he disease. Soli ary neurof bromas are rare orbi al umors ha can be excised and are unlikely o be associa ed wi h neurof broma osis. T ese end o occur in middle age. Dif erential Diagnosis Lymphangioma Orbi al pseudo umor Isola ed lesions mus consider schwannoma, cavernous hemangioma, f brous his iocy oma Pathology Proli era ing, in er wining bundles o Schwann cells, axons, and endoneural f broblas s wi hin he nerve shea hs. Treatment Observa ion wi h surgical debulking only as a las resor . T ese umors canno be comple ely excised and recur and regrow wi h ime. Rare, isola ed lesions can be comple ely excised. T ese umors are o en very vascular and bleed during excision. Prognosis Generally poor cosme ic and unc ional resul s because o he progressive na ure o hese inf l ra ive umors. Isola ed lesions have a good prognosis.

Neural Tumors

209

A

B FIGURE 14-10. Neurof broma. A. Severe prop osis, comple e p osis, and orbi al in l ra ion by a plexi orm neuro broma. B. C scan shows orbi al in l ra ion as well as absence o par o he sphenoid bone o he orbi . All is consis en wi h neuro broma osis.

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MENINGIOMAS

M

eningiomas are invasive umors ha arise in racranially and secondarily invade he orbi . T ey are usually slowly progressive umors ha are very di cul o comple ely excise because o heir inf l ra ive na ure. Epidemiology and Etiology Age: Bimodal peak in he second and f h decades Gender: More common in women E iology: T ese umors arise rom arachnoid villi. Mos commonly, hese umors s ar as in racranial umors and ex end in o he orbi secondarily. A primary orbi al orm arises rom he op ic nerve shea h arachnoid issue. History Meningiomas will have a gradual onse o symp oms as hey slowly ex end rom heir in racranial origin in o he orbi . Oph halmic mani es a ions are dependen on he loca ion o he umor. Mos common orbi al presen a ion is umors arising near he p erion ha presen as a emporal ossa mass and prop osis ha can o en be suddenly no iced bu have been presen or years ( Fig. 14-11A–D). Op ic nerve meningiomas presen wi h slow, painless, progressive visual loss and prop osis. Examination Findings on examina ion depend on he loca ion o he meningioma. I loca ed in he emporal ossa, f ndings include emporal ossa ullness, prop osis, eyelid edema, and chemosis. I he meningioma arises near he sella and op ic nerve, early f ndings will be visual loss wi h op ic nerve edema or a rophy.

Op ic nerve meningiomas presen wi h decreased vision, an a eren pupillary de ec , prop osis, and possible oph halmoplegia ( Fig. 14-11E–J). T e op ic nerve may be normal, swollen, a rophic, or have shun vessels. Imaging C scan: Hyperos osis, calcif ca ion, wi h adjacen so issue ullness MRI: Use ul o de ec grow h along he dura Gadolinium enhancemen and a suppression echniques help def ne hese lesions in he orbi . Dif erential Diagnosis Op ic nerve glioma Lymphangioma Pathology hese umors are composed o cells ha can be round, polygonal, or spindle shaped. In addi ion, here are varying admix ures o blood vessels, f broblas s, and psammoma bodies. T e di eren pat erns o meningiomas show varying mix ures o hese componen s. Treatment In racranial meningiomas ha ex end in o he orbi are usually rea ed surgically. I well encapsula ed, hese can be comple ely excised wi h a neurosurgical and orbi al approach. Meningiomas can be inf l ra ive and involvemen o vi al s ruc ures may preven comple e excision and allow or debulking only. rea men o op ic nerve shea h meningiomas is required i here is aggressive

Neural Tumors

grow h, hrea o in racranial spread, or visual loss. Radia ion is he rea men o choice in progressive umors. Radia ion has been shown o slow grow h o hese umors. Surgical excision or biopsy is rarely needed.

211

Prognosis umors are generally progressive bu very slowly. Radia ion will slow or some imes s op grow h. More rapid grow h is unusual and he diagnosis should be ques ioned.

A

B FIGURE 14-11. Sphenoid wing meningioma. A. A pa ien wi h a lef -sided prop osis o gradual progressive onse . No e he emporal ossa ullness. B. C scan shows hyperos osis and an associa ed sof issue mass, all consis en wi h a sphenoid wing meningioma. ( con inued)

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C FIGURE 14-11. (Con inued) Sphenoid wing meningioma. C. suppression shows lef sphenoid wing meningioma.

1

-weigh ed coronal pos con ras MRI wi h a ( con inued)

Neural Tumors

213

D

E FIGURE 14-11. ( Con inued) Sphenoid wing meningioma. D. 2-weigh ed coronal MRI shows lef sphenoid wing meningioma. E. A pa ien wi h axial prop osis and visual loss. (con inued)

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F

G FIGURE 14-11. ( Continued) Sphenoid wing meningioma. F. MRI scan shows a usi orm enlargement o the optic nerve and is consistent with a meningioma. G. More commonly, there is dif use thickening o the optic nerve. T e thickened right optic nerve has a central lucency, termed the “railroad track” sign. ( continued)

Neural Tumors

215

H FIGURE 14-11. ( Continued) Sphenoid wing meningioma. H.T e hyperintense to at and muscle as seen on the lef side.

-weighted image can be hypointense to

2

( continued)

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I FIGURE 14-11. ( Con inued) Sphenoid wing meningioma. I. MRI 1-weigh ed coronal image wi h con ras and a suppression shows a more globular op ic nerve meningioma on he lef . ( con inued)

Neural Tumors

217

J FIGURE 14-11. ( Con inued) Sphenoid wing meningioma. J. MRI globular op ic nerve meningioma on he lef .

2

-weigh ed coronal image shows a more

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SCHWANNOMAS

S

chwannomas presen as well-encapsula ed, slowly growing masses ha ac very much like a cavernous hemangioma. T ese masses are usually easily excised and cause no subsequen problems.

Epidemiology and Etiology Age: 20 o 50 years o age Gender: Equal occurrence in males and emales E iology: Eccen ric grow hs rom peripheral nerves History Slow, insidious onse o prop osis over years Examination Prop osis wi h he direc ion dependen on he umor posi ion (mos commonly in raconal) ( Fig. 14-12A). Less commonly, here may be eyelid swelling, diplopia, visual dis or ion. Imaging C and MRI scan show a well-circumscribed, round lesion ( Fig. 14-12B–D).

Special Considerations Eigh een percen o pa ien s wi h schwannomas have neurof broma osis. Dif erential Diagnosis Capillary hemangioma Hemangiopericy oma Fibrous his iocy oma Pathology Proli era ion o Schwann cells in a perineural capsule is seen. T ese may be in a igh ly ordered arrangemen (An oni A) or loose arrangemen (An oni B). Treatment Surgical excision is he rea men o choice. Because hey are ou pouchings o a nerve, hey can o en be s ripped o he nerve. Recurrence o he umor, even i here is only par ial resec ion, is very rare. Prognosis Excellen

Neural Tumors

219

A

B FIGURE 14-12. Schwannoma. A and B. A 45-year-old woman wi h gradual onse o righ eye prop osis. C scan shows a well-circumscribed mass in he superior orbi . T ere is some bony ossa orma ion. On excision, his was a schwannoma. Schwannomas are mos commonly in raconal. ( con inued)

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C FIGURE 14-12. ( Con inued) Schwannoma. C. MRI shows a well-circumscribed lesion. T is shows he lesion isoin ense o muscle and hypoin ense o a .

-weigh edi mage

1

( con inued)

Neural Tumors

221

D FIGURE 14-12. ( Con inued) Schwannoma. D. On he muscle.

-weigh ed image, he lesion is hyperin ense o a and

2

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MESENCHYMAL TUMORS RHABDOMYOSARCOMA

R

habdomyosarcoma is he mos common primary orbi al malignancy o childhood. Classically, he child presen s wi h sudden onse o prop osis over days o weeks wi h an orbi al mass ound on imaging. Once suspec ed, he lesion should be immedia ely biopsied so rea men can be s ar ed as quickly as possible. Epidemiology and Etiology Age: Average age 7 o 8 years Gender: Equal occurrence in males and emales E iology: Rhabdomyosarcomas develop rom undi eren ia ed pluripo en ial mesenchymal cells. History Classically, rapid onse o unila eral prop osis is seen over days o a week, bu some pa ien s may presen less rapidly over many weeks. Examination Prop osis is seen wi h variable amoun s o adnexal response, such as edema, ery hema, and globe displacemen , and some imes a palpable mass ( Fig. 14-13A, C, D). T e superior nasal orbi is he mos common loca ion. Imaging C scan: Homogenous mass ha may have bony erosion ( Fig. 14-13B, E). MRI: Hypoin ense on 1 and hyperinense on 2 ( Fig. 14-13F). Variable enhancemen wi h gadolinium

Special Considerations Acu e prop osis in a child is an emergency. A er a rhabdomyosarcoma is suspec ed, he lesion needs o be biopsied wi hin 24 hours ollowed by promp ini ia ion o rea men . Dif erential Diagnosis Capillary hemangioma Orbi al pseudo umor Orbi al celluli is Rup ured dermoid cys Me as a ic umor Pathology T ere are our dis inc orms o rhabdomyosarcoma. Embryonic: poorly di eren ia ed spindle cells Alveolar: shows rounded rhabdomyoblas s Pleomorphic: rounded or s rap-like cells wi h cross-s ria ions Bo ryoid: rare orm wi h grape-like clus ers Treatment Once suspec ed, a C scan is done o iden i y he lesion. Urgen orbi al biopsy wi h pa hologic evalua ion is hen done o conf rm he diagnosis and classi y he ype o rhabdomyosarcoma. Sys emic evalua ion by a pedia ric oncologis is hen done. Radia ion and sys emic chemo herapy are he mains ays o rea men . Prognosis Survival ra es are 90% wi h his rea men .

Mesenchymal Tumors

223

A

B FIGURE 14-13. Rhabdomyosarcoma. A. An 8-year-old girl wi h 3-week his ory o progressive swelling o he righ eye. B. C scan shows large mass o he orbi , which on biopsy was a rhabdomyosarcoma. ( con inued)

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C

D FIGURE 14-13. ( Con inued) Rhabdomyosarcoma. C. and D. A 3-mon h-old girl wi h 1- o 2-week his ory o swelling o he righ eye. ( con inued)

Mesenchymal Tumors

225

E

F FIGURE 14-13. (Con inued) Rhabdomyosarcoma. E. C scan shows well-circumscribed mass wi h inden a ion o he globe. Biopsy o he mass revealed a rhabdomyosarcoma. T e ac ha his pa ien is younger han he ypical rhabdomyosarcoma pa ien shows ha rhabdomyosarcoma can presen a various ages. F. MRI o a rhabdomyosarcoma. T e 2-weigh ed image shows he lesion is hyperin ense o muscle and a .

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FIBROUS HISTIOCYTOMA

F

ibrous his iocy oma is a umor ha can be benign, locally aggressive, or malignan . I no comple ely excised, he benign orms can become malignan . T e umor is usually inf l ra ing and no encapsula ed. I usually presen s wi h prop osis and is o en accompanied by various orms o orbi al dys unc ion depending on he umor loca ion.

Epidemiology and Etiology Age: Middle age Gender: Equal occurrence in males and emales E iology: Arises de novo rom mesenchymal issue. T e umor can be benign, in ermedia e, or malignan . History Usually slow onse o prop osis wi h no def ni ive onse in he leas aggressive orm. T e malignan orm can presen more rapidly accompanied by diplopia, pain, swelling, and res ric ed eye movemen s. Examination Prop osis wi h ew o her orbi al signs in he benign orm T e more aggressive orms show signs o in amma ion, res ric ed eye movemen s, chemosis, and swelling ( Fig. 14-14A). Imaging C scan: Well-circumscribed orbi al mass bu he in ermedia e and malignan orms can be more inf l ra ive ( Fig. 14-14B). MRI: isoin ense on 1 and hyperin ense on 2 ( Fig. 14-14C) Enhances wi h gadolinium

Special Considerations Incomple e excision can lead o recurrence and he recurrence can be malignan . T e malignan , mos aggressive orm can me as asize. Dif erential Diagnosis Hemangiopericy oma Capillary hemangioma Schwannoma Pathology Fibrous-appearing his iocy ic cells ha orm a charac eris ic car wheel or s ori orm pat ern T e availabili y o immunohis ochemical s udies has allowed or more accura e classif ca ion o mesenchymal umors. Many o hese umors in he pas may have been incorrec ly classif ed. T is brings in o ques ion some o he prognos ic predic ions or hese umors. For example, some hemangiopericy omas ha are aggressive and even po en ially malignan umors may have classif ed as f brous his iocy omas. Treatment Comple e surgical excision. T e his ology will reveal he prognosis. Prognosis Depends on his ologic ype. I he benign or locally aggressive orms are comple ely excised, he prognosis is usually good.

Mesenchymal Tumors

227

A

B

C FIGURE 14-14. Fibrous histiocytoma. A. A 42-year-old man wi h progressive prop osis o he lef eye over 2 o 3 mon hs, increasing diplopia, and blurred vision. B. C scan shows an in raconal mass wi h possible in l ra ion o he op ic nerve. C. On MRI, he mass abu s bu does no in l ra e he op ic nerve. T e mass was removed and was a benign brous his iocy oma.

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LYMPHOPROLIFERATIVE TUMORS LYMPHOID HYPERPLASIA AND LYMPHOMAS

L

ymphoid lesions include a spec rum o lesions rom benign o malignan . Presence o even he benign lymphoid hyperplasia implies a risk in he u ure or he developmen o a lymphoma somewhere in he body. T ese lesions can occur in he orbi or subconjunc ival area and end o mold around s ruc ures ra her han displacing s ruc ures.

Epidemiology and Etiology Age: Older adul s Gender: Equal occurrence in males and emales E iology: Clonal expansion o abnormal precursor cells. T ere is a con inuum o disease rom he benign, localized lymphoid hyperplasia hrough malignan lymphoma. History A his ory o a painless, progressive mass T e exac his ory depends on he loca ion o he mass. An eriorly, i can presen as a visible, palpable mass. More pos eriorly, he symp oms will be more o prop osis or globe displacemen depending on he loca ion. Examination An eriorly, a visible, subconjunc ival salmonpa ch mass can be observed ( Fig. 14-15A, B). A so , di use mass can be palpa ed i he mass is in he an erior orbi bu no visible ( Fig. 14-15C). More pos erior umors will cause prop osis wi h symp oms depending on he umor loca ion ( Fig. 14-15E). T ese umors end o mold around orbi al s ruc ures and rarely displace or inf l ra e s rucures, so mo ili y or visual dis urbances are rare.

Imaging C scan: A mass ha molds around orbi al s ruc ures ra her han displacing or inf l ra ing ( Fig. 14-15D). MRI: Shows ex en o umor bu does no di eren ia e orbi al in amma ion and canno separa e lymphoid hyperplasia rom lymphoma ( Fig. 14-15F). PE scanning is used o look or lymphoid lesions elsewhere in he body. Special Considerations T ere are mul iple ways o evalua e a lymphoid lesion o de ermine whe her i is a benign or a malignan lesion. No all lesions can be clearly de ermined o be benign or malignan . Even he pa ien wi h benign lymphoid hyperplasia mus be observed sys emically or he developmen o a lymphoma elsewhere in he body over u ure years. Dif erential Diagnosis Orbi al pseudo umor Me as a ic orbi al umor Lymphangioma Pathology A collec ion o lymphocy es is seen, which iden if es he lesion as lymphoid. Microscopic appearance will give some evidence o he lesion as benign or malignan . Fresh issue is evalua ed o iden i y cell sur ace markers. Polyclonal lymphocy ic popula ions are less likely o develop sys emic disease; monoclonal lesions are more likely o accompany lymphoma elsewhere in he body. T e at emp o separa e lymphoid lesions in o benign and malignan is no exac and is bo h con roversial and con using; however, a his ime, i is he bes sys em available. Treatment T e lesions require biopsy o iden i y whe her hey are benign or malignan .

Lymphoproliferative Tumors

A sys emic workup is done o look or evidence o lymphoid lesions elsewhere in he body. rea men or localized benign orbi al disease is low-dose radia ion. More malignan lesions or sys emic disease usually requires radia ion and sys emic chemo herapy.

229

Prognosis T e prognosis is dependen on he ype o lymphoma. Many lymphomas are very responsive o rea men bu a high-grade lymphoma can be rapidly a al, even wi h rea men .

A

B FIGURE 14-15. Lymphoid hyperplasia and lymphoma. A and B. Subconjunc ival lymphoid in l ra es. T ese may be isola ed or here may be orbi al ex ension. T ey may be a reac ive process or a lymphoma. Where hese lesions all on he spec rum o benign versus malignan can only be di eren ia ed on biopsy. A was benign reac ive lymphoid hyperplasia, and B was a low-grade lymphoma. ( con inued)

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C

D FIGURE 14-15. ( Con inued) Lymphoid hyperplasia and lymphoma. C. A 65-year-old woman wi h swelling and redness o he lef eye. D. C scan shows a di use orbi al process, which on biopsy was a lymphoma. Pa ien was rea ed wi h radia ion and chemo herapy. ( con inued)

Lymphoproliferative Tumors

231

E

F FIGURE 14-15. ( Con inued) Lymphoid hyperplasia and lymphoma. E. Massive lid and orbi al in l ra ion by a lymphoma on he righ . F. MRI o a lymphoma. T e 2-weigh ed image shows he lesion o be hyperin ense o muscle and a .

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PLASMACYTOMA

lasmacy oma is an isola ed mass o plasma cells ha occurs in he bone. T is lesion can ex end rom he bone in o he orbi al so issue. Progression o a sys emic plasma cell umor is ermed mul iple myeloma.

Special Considerations As wi h lymphoma, plasma cell umors can be benign or malignan . T ey are di eren ia ed rom mul iple myeloma on he basis o sys emic involvemen in mul iple myeloma.

Epidemiology and Etiology Age: Six h and seven h decades Gender: Males more commonly a ec ed E iology: Rare proli era ion o plasma cells in so issues or bone o he orbi

Dif erential Diagnosis Mul iple myeloma Me as a ic disease His iocy ic disorders Malignan umor o he sinus

History Pa ien s presen wi h slow onse o a mass e ec wi h some in amma ory signs bu very rarely pain. Symp oms depend on he loca ion o he umor.

Pathology Classic plasma cells make up he umor. Vary rom ma ure o larger, imma ure cells depending on he umor. Di eren ia ion rom mul iple myeloma is on he basis o sys emic workup; mul iple myeloma having o her sys emic mani es a ions.

P

Examination I loca ed an eriorly, here is a palpable mass over or adjacen o an orbi al bone. T ere may be prop osis or globe displacemen depending on he loca ion ( Fig. 14-16A). Imaging C scan shows a lesion in or adjacen o bone wi h bony des ruc ion ( Fig. 14-16B, C).

Treatment Biopsy o he lesion, and hen a comple e sys emic workup. I he lesion is isola ed, higher dose irradia ion is indica ed. Chemo herapy may be indica ed. Prognosis Variable depending on he aggressiveness o he umor.

Lymphoproliferative Tumors

233

A FIGURE 14-16. Plasma cell tumor. A. A 70-year-old woman was no ed o have swelling around he lef eye. Examina ion shows prop osis wi h downward displacemen o he lef eye. ( con inued)

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B FIGURE 14-16. (Con inued) Plasma cell tumor. B. C scan shows a superior emporal lesion ha has eroded bone and may even be cen ered in bone. Biopsy revealed a plasmacy oma. ( con inued)

Lymphoproliferative Tumors

C FIGURE 14-16. ( Con inued) Plasma cell tumor. C. Axial C scan o plasmacy oma.

235

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HISTIOCYTIC DISORDERS

H

is iocy ic disorders are a rare group o abnormali ies o he mononuclear phagocy ic sys em. In he orbi , hey mos commonly presen as a uni ocal lesion o he superior bone o he orbi wi h secondary progressive prop osis. Epidemiology and Etiology Age: Children. Children younger han age 2 years are more likely o have sys emic disease, which is up o 50% a al. Over he age 2 years, he disease involves he bone wi hou sys emic involvemen bu is o en mul i ocal. T e older he child, he more likely he disease will be uni ocal and less severe. Gender: Males more commonly a ec ed E iology: Abnormal immune regula ion resul ing in an accumula ion o proli era ing dendri ic his iocy es History Orbi al swelling mos commonly superiorly over days o weeks. Examination Superior orbi al swelling wi h a variable amoun o mass e ec is he mos common presen a ion ( Fig. 14-17A). Younger children are more likely o have more swelling, mul i ocal bony involvemen , and sys emic involvemen . Imaging C scan will show a lesion adjacen o bone wi h bone erosion ( Fig. 14-17B, C).

Mos commonly in he superior, emporal orbi . Special Considerations T e older erm or hese disorders was hisiocy osis X wi h specif c mani es a ions ermed Let erer–Siwe disease, Hand–Schüller–Chris ian disease, and eosinophilic granuloma o bone. T ese erms are replaced by dif use so - issue his iocy osis, mul iple eosinophilic granuloma o bone, and uni ocal granuloma o bone. Dif erential Diagnosis Choles ea oma Repara ive granuloma Pathology Proli era ion o dendri ic his iocy es along wi h granulocy es and lymphocy es Treatment Bony lesions require a conf rma ory biopsy and hen debulking. T is rea men is o en cura ive bu in younger children, evidence o sys emic disease mus be sough . Rarely, a s eroid or low-dose radia ion is needed. rea men or sys emic disease in younger children may include s eroids, irradia ion, or cy o oxic agen s. In some cases, he disease may no respond o any hing. Prognosis Excellen in uni ocal disease in older children. Very young children wi h sys emic disease have a 50% mor ali y ra e.

Lymphoproliferative Tumors

237

A FIGURE 14-17. Histiocytic disorder. A. An 8-year-old boy wi h a 1- o 2-week his ory o swelling o his righ eye. Mild ery hema and swelling superiorly and downward displacemen o he globe on he righ is shown. ( con inued)

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B FIGURE 14-17. ( Con inued) Histiocytic disorder. B and C. C scans shows a superior in l ra e wi h bony erosion. Biopsy revealed a uni ocal granuloma o bone, which was rea ed wi h curet age. ( con inued)

Lymphoproliferative Tumors

C FIGURE 14-17. ( Con inued)

239

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LACRIMAL GLAND TUMORS EPITHELIAL TUMORS OF THE LACRIMAL GLAND

T

he lacrimal gland can harbor mul iple disease processes. T e mos common is in amma ory disease. Benign and malignan processes inheren o he lacrimal gland also occur. Biopsy is o en required o iden i y hese processes. Epidemiology and Etiology T is group includes a number o en i ies ha involve he lacrimal gland. T is does no include idiopa hic in amma ion or lymphoid inf l ra ion o he lacrimal gland. Age: Pleomorphic adenoma occurs in he our h and i h decades. Malignan mixed umors occur a an older age. Adenoid cys ic carcinoma has a peak incidence in he second and our h decades. Gender: Equal occurrence in males and emales E iology: Proli era ion o epi helial cells History Pleomorphic adenomas presen wi h progressive, downward, and inward displacemen o he globe, some imes wi h axial prop osis( Fig. 14-18A). T e process is painless, unlike he malignan umors o he lacrimal gland. Malignan mixed umors usually arise rom exis ing pleomorphic adenomas. Adenoid cys ic carcinoma will presen wi h more rapid grow h associa ed wi h signif can pain( Fig. 14-18E). T is pain is wha easily separa es many malignan lacrimal gland umors rom a benign pleomorphic adenoma. Examination Palpable mass in he superior, emporal quadran wi h displacemen o he globe down and in.

T e presence o in amma ion and he amoun o globe displacemen is variable depending on he e iology o he lacrimal gland mass. Imaging C scan: Pleomorphic adenomas show a globular, circumscribed mass. T e mass at ens and de orms he globe. T ere can be pressure expansion o he lacrimal ossa bu no erosion. Malignan lesions are no globular, are less well def ned, and may have bony erosions and calcif ca ions ( Fig. 14-18B, C, E, I). MRI: Valuable o def ne he ex en o in racranial ex ension in aggressive, malignan umors ( Fig. 14-18D, G). Special Considerations Mus comple ely excise a pleomorphic adenoma or i can recur as a malignan umor. Dif erential Diagnosis Idiopa hic in amma ion o he lacrimal gland Lymphoid inf l ra ion o he lacrimal gland ( Fig. 14-18H). Sarcoidosis Pathophysiology Pleomorphic adenoma: Proli era ion o epihelial cells wi h duc al and secre ory elemen s. Adenoid cys ic carcinoma: Small, benignappearing cells arranged in nes s, ubules, or in a cribri orm, Swiss-cheese pat ern Treatment Pleomorphic adenoma: Comple e excision wi hin he capsule Malignan umors: Individualize rea men . Generally ex ensive excision, especially wi h adenoid cys ic carcinoma and high-dose radia ion. Some umors will require orbi al exen era ion. Prognosis Pleomorphic adenoma: Excellen i comple ely excised Malignan umors: High ra e o recurrence over ime

Lacrimal Gland Tumors

241

A

B FIGURE 14-18. Lacrimal gland tumor. A. A 33-year-old man wi h slowly progressive, painless prop osis over a ew years. B and C. C scans show a round, well-circumscribed mass replacing he lacrimal gland. Comple e excision showed a pleomorphic adenoma. ( con inued)

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C

D FIGURE 14-18. (Con inued) Lacrimal gland tumor. D. MRI o a pleomorphic adenoma. T e shows he lesion is hyperin ense o a and muscle.

2

-weigh edi mage ( con inued)

Lacrimal Gland Tumors

243

E

F FIGURE 14-18. ( Con inued) Lacrimal gland tumor. E. A 58-year-old man wi h 6-mon h his ory o swelling and pain around he lef eye. Massive prop osis and swelling wi h downward displacemen o he eye are seen. F.C scan shows a large mass in he lacrimal gland area wi h bony des ruc ion. T is was adenoid cys ic carcinoma o he lacrimal gland. ( con inued)

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G

H FIGURE 14-18. (Con inued) Lacrimal gland tumor. G. MRI o an adenoid cys ic carcinoma. T e 2-weigh ed image shows he lesion is hyperin ense o muscle and a . H.T is 75-year-old man had swelling and downward displacemen o he lef eye or 2 o 3 mon hs. ( con inued)

Lacrimal Gland Tumors

245

I FIGURE 14-18. ( Con inued) Lacrimal gland tumor. I. C scan shows an enlarged lacrimal gland, which was a lymphoma on biopsy. Con ras he shape o his mass wi h he very round con our in B and C.

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MISCELLANEOUS ORBITAL TUMORS SECONDARYORBITAL TUMORS

S

econdary orbi al umors are umors ha invade he orbi rom adjacen s ruc ures including sinus umors, eyelid umors, and umors ha ex end in o he orbi rom wi hin he globe.

Epidemiology and Etiology E iology: Includes umors ha invade he orbi rom he sinus, eyelid, or globe. Sinus processes include mucoceles and squamous cell carcinoma. Eyelid umors include basal cell carcinoma ( Fig. 14-19A–C), sebaceous adenocarcinoma, and squamous cell carcinoma ( Fig. 14-19E–G). Re inoblas oma and choroidal melanoma ( Fig. 14-19D) can ex end rom he globe in o he orbi . Age and gender: Variable based on primary umor History O en, here is a his ory o ei her a neglec ed primary malignancy or a his ory o previous rea men o he primary malignancy. T e ime course o symp oms and grow h is dependen on he primary malignancy. Examination T ere may be obvious ex ernal signs o he primary umor such as in a neglec ed basal cell carcinoma. Likewise, in raocular umors ha ex end ou side he globe usually have ex ernal signs o in amma ion.

Sinus umors ha ex end in o he orbi may only show prop osis, o en wi h some direc ional displacemen o he globe. Imaging C scan: Dependen on he primary source. A umor rom he sinuses will show sinus changes. MRI: May help o def ne ex raocular ex ension o a primary ocular umor. Dif erential Diagnosis I he primary umor is known, he secondary process is easily suspec ed. Pathology T e pa hology is specif c or each o he individual processes. Treatment rea men is aimed a comple e excision i possible. I he umor is resec able and here is no evidence o dis an me as asis, hen ha is he rea men o choice. Depending on he umor, irradia ion a er excision can be used. For hose umors unresec able, he use o irradia ion and chemo herapy can be considered. rea men mus be individualized. Prognosis Generally poor. Even i he umor is hough o be removed, here is o en recurrence.

Miscellaneous Orbital Tumors

247

A

B FIGURE 14-19. Basal cell carcinoma with orbital invasion. A. A pa ien wi h a neglec ed medial can hal basal cell carcinoma presen s wi h prop osis. B and C. Axial and coronal C scans show a large medial orbi al mass ha was basal cell carcinoma on biopsy. T e pa ien required an orbi al exen era ion. ( con inued)

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C

D FIGURE 14-19. (Con inued) Basal cell carcinoma with orbital invasion. D. A pa ien wi h a large limbal mass. On undus examina ion, here was a large choroidal melanoma ha had ex ended ex rasclerally. ( con inued)

Miscellaneous Orbital Tumors

249

E FIGURE 14-19. ( Con inued) Squamous cell carcinoma with perineural spread. E. A 77-year-old man wi h an orbi al apex syndrome. ( con inued)

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F FIGURE 14-19. ( Con inued) Squamous cell carcinoma with perineural spread. F. C scan shows an in l ra ing mass a he apex wi h hickening along he superior orbi . Biopsy showed squamous cell carcinoma ha had spread in o he apex along he ron al nerve. T e pa ien had he his ory o a squamous cell carcinoma o he orehead excised 2 years prior. ( con inued)

Miscellaneous Orbital Tumors

251

G FIGURE 14-19. ( Con inued) Squamous cell carcinoma with perineural spread. G. Coronal C showing lesion a apex.

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METASTATIC ORBITAL TUMORS

M

e as a ic orbi al umors usually presen wi h orbi al in amma ion, pain, prop osis, and bony des ruc ion. Mos cases will have a known primary malignancy bu in up o 25%, he primary si e is unknown ( Fig. 14-20A–G). Epidemiology and Etiology Age: Mos commonly in he f h, six h, and seven h decades E iology and gender: Breas me as asis is he mos common in women. Lung me as asis is he mos common in men and second in women. O her e iologies include pros a e in men, gas roin es inal, and many have an unknown primary si e. History T e majori y o pa ien s will have a known primary malignancy a he ime o orbi al occurrence. T e onse o symp oms ends o be more rapid han in mos orbi al umors and can be accompanied by pain. Examination Prop osis is he mos common f nding. T is can be axial or displace he globe. A palpable mass may be presen ha is usually f rm. P osis, mo ili y dis urbances, and decreased vision may be presen because o he inf l ra ive na ure o he me as asis.

Imaging C scan: Highly variable appearance o hese lesions. May be discre e or invasive, cause bony erosion or hyperos osis (pros a e), and may only show muscle enlargemen . MRI: No diagnos ic and does no de ine bone well. May show ex en in o so issues. Dif erential Diagnosis Lymphoma Wegener’s granuloma osis Orbi al pseudo umor Pathology Special s ains and marker s udies can help iden i y he issue o origin in hose cases ha do no have a primary malignancy already iden if ed. Treatment Biopsy o iden i y he umor as me as a ic Sys emic rea men o he carcinoma is hen ha o he primary malignancy. Orbi al irradia ion will o en shrink he orbi al umor and diminish symp oms, ei her alone or in combina ion wi h chemo herapy. Prognosis T e prognosis is ha o he primary umor wi h me as asis. In mos cases, he prognosis is poor.

Miscellaneous Orbital Tumors

253

A

B FIGURE 14-20. Metastatic breast carcinoma. A and B. A 65-year-old woman wi h known his ory o breas cancer presen s wi h swollen, pain ul righ eye. T e pa ien has prop osis wi h a rozen globe and a corneal ulcer. C scan shows di use in l ra ion o he orbi wi h me as a ic breas carcinoma. Fur her workup revealed o her areas o me as a ic disease. ( con inued)

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C FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. C. MRI hypoin ense o a and muscle.

1

-weigh ed image shows he lesion ( con inued)

Miscellaneous Orbital Tumors

255

D FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. D.T e hyperin ense o a and muscle.

2

-weigh ed image shows he lesion ( con inued)

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E

F FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. E and F. A 68-year-old man wi h known me as a ic lung adenocarcinoma presen s wi h prop osis and pain. No e he la eral mass on he righ . C scan shows a la eral orbi al mass wi h bone des ruc ion. ( con inued)

Miscellaneous Orbital Tumors

257

G FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. G. An 8-year-old child wi h known neuroblas oma wi h orbi al me as asis. T e pho ograph shows he classic orbi al ecchymosis along wi h prop osis.

C H AP T ER

Orbi al rauma ORBITAL FRACTURES O BITAL FLOO FR CTU E

O

rbi al oor rac ures are he mos common ype o orbi al rac ure. T is is he resul o a blow o he eye i sel or o he bony rim. Many rac ures only resul in swelling and ecchymosis o he orbi al issues. T ose wi h en rapped issue and persis en diplopia, or wi h a large rac ure and enoph halmos, will require repair. Epidemiology and Etiology Age: Mos common in second o our h decades Gender: More common in males E iology: Direc orce o he in erior orbi al rim wi h buckling and rac ure o he oor is one mechanism. T e second mechanism consis s o orces ha raise he in raorbi al pressure and hen “blow-ou ” he hin orbi al oor. History rauma such as s , ngers, elbow, hi wi h a ball, and so or h 258

T e pa ien will of en have double vision af er he injury. Less commonly, he pa ien may no e orbi al swelling af er he rauma rom orbi al emphysema af er blowing he nose. Examination Orbi al swelling and ecchymosis are variable. Some rac ures have very lit le. In raorbi al hypes hesia and res ric ed mo ili y wi h diplopia are he mos speci c signs. As he orbi al swelling decreases, large rac ures will develop enoph halmos. Variable degrees o crepi ance may be presen as an indica ion o he rac ure. Imaging C scanning shows a rac ure o he orbi al loor o en wi h blood in he sinuses. A rac ure ha is very small is more likely o have en rapmen o orbi al issue han a very large rac ure. T e in erior rec us is almos never in he rac ure i sel bu issues around he muscle are en rapped.

Orbital Fractures

MRI does no image bone well and should no be used ini ially af er rauma. T e excep ion is he Whi e-Eyed Blowou Frac ure(WEBOF) where he muscle may be in he rac ure(below). Special Considerations Children and eenagers may sus ain an orbi al oor rac ure wi h no ecchymosis, bu wi h severe en rapmen o he in erior rec us muscle and associa ed pain, nausea, and vomi ing. T is is called a whi e-eyed blowou rac ure (WEBOF) ( Fig. 15-1). T ese pa ien s are very uncom or able and di cul o examine. T e en rapmen needs o be released wi hin 24 o 48 hours, as he muscle is severely en rapped and will become ischemic i no released. T is requires emergen surgery.

259

Treatment Open repair is required or pa ien s wi h unc ional diplopia ha does no improve as he swelling resolves. Frac ures involving more han 50% o he oor will resul in signi can enoph halmos and also should be considered or repair. Frac ures should be repaired wi hin 2 weeks o rauma wi h he excep ion o a WEBOF, which is an emergency. Mos rac ures ha are repaired will require an implan o some ype. Prognosis Good i repaired wi hin 2 weeks Some pa ien s will have direc muscle or nerve injury and ei her will no improve or may ake mon hs o improve.

A FIGURE 15-1. White-eyed blowout racture. A and B. A 9-year-old boy wi h a his ory o being hi wi h an elbow 1 day prior. He has pain, worse wi h eye movemen ; double vision; and has had nausea and some vomi ing. T e pho ograph shows his lack o ecchymosis and swelling as well as severe res ric ion o upgaze. ( continued)

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B

C FIGURE 15-1. (Continued) White-eyed blowout racture. C. C scan shows a small rap-door f oor rac ure wi h issue en rapmen . T is rac ure needs o be repaired promp ly as he en rapped muscle may become ischemic. ( continued)

Orbital Fractures

261

D

E FIGURE 15-1. (Continued) Orbital f oor racture. D. T is 72-year-old woman ell and hi her eye on her bedpos . She has ull mo ili y bu signi can swelling, ecchymosis, and in raorbi al hypes hesia. E. C scan shows a large orbi al f oor rac ure. T e pa ien is a risk or developmen o enoph halmos rom he large rac ure.

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MEDIAL WALL FR CTU E

M

edial wall rac ures can be isola ed racures o he medial wall only or hey can be a par o larger rac ures involving he nose and sinuses. Isola ed rac ures are rea ed much like orbi al oor rac ures ( Fig. 15-2). Larger rac ures usually involve a mul idiscipline approach o he repair o he rac ures. Epidemiology and Etiology Age: Mos common in second hrough our h decades Gender: More common in males E iology: Direc rac ures occur rom s riking a solid objec . Indirec (blow-ou ) racures occur in associa ion wi h and by similar mechanisms as orbi al oor rac ures. History rauma his ory is variable. Symp oms include diplopia and cosme ic de ormi ies depending on he ex en o he nasal rac ures. Examination Medial rec us en rapmen wi h diplopia and even ual enoph halmos are he wo ocular mani es a ions ha may occur. Direc rac ures of en have signi can damage o he nasal bridge and medial orbi . T e nasal bridge may be depressed wi h elecan hus. O her ndings ha can occur include epis axis, orbi al hema oma, cerebral spinal

uid rhinorrhea, and damage o he lacrimal drainage sys em. Imaging C scanning will show he ex en o he rac ure and assis wi h po en ial planning o he repair. MRI does no image bone well and should no be used ini ially af er rauma. Special Considerations Medial wall rac ures wi h en rapmen o he medial rec us need o be repaired sooner han oor rac ures (wi hin 1 week) i possible. Treatment I isola ed, medial wall rac ures of en do no need repair. Medial rec us en rapmen wi h diplopia is one indica ion or repair. I he rac ure is large, enoph halmos can develop and require surgery o build up he orbi . Implan s are some imes placed. Larger rac ures involving he nasal bridge and medial orbi require repair and pla ing, usually in conjunc ion wi h an o olaryngology specialis . Prognosis Good. Larger rac ures may require muliple surgeries and revisions.

Orbital Fractures

263

A

B

C FIGURE 15-2. Medial wall racture. A o C. A 55-year-old man s ruck in he ace wi h an unknown objec presen ed wi h horizon al diplopia. Mo ili y is res ric ed in he righ eye in bo h adduc ion and abduc ion. ( continued)

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D FIGURE 15-2. (Continued) Medial wall racture. D and E. C scans show a medial wall orbi al rac ure wi h he medial rec us muscle pulled in o he rac ure. ( continued)

Orbital Fractures

E FIGURE 15-2. (Continued)

265

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15 O RBITAL TRAUMA

O BITAL OOF FR CTU E

O

rbi al roo rac ures ( Fig. 15-3) are rare rac ures ha need o be recognized because o he po en ial or li e- hrea ening neurologic sequelae. T ere may jus be a small rac ure wi h no neurologic problems or here may be signi can in racranial air and bleeding. rea men is in conjunc ion wi h neurosurgery. Epidemiology and Etiology Age: Mos common in second hrough our h decades Gender: More common in males E iology: Blun rauma or direc injury by a hin objec ha goes above he globe under he superior orbi al rim. An isola ed roo racure is rare. History rauma his ory will of en sugges highenergy orces ha caused he injury. T ese include hydraulic air hoses, a blun objec wi h high veloci y, and so or h. Examination Poor upgaze, supraorbi al hypes hesia, and more swelling superiorly han in eriorly sugges an orbi al roo rac ure.

En rapmen o he superior rec us or superior oblique muscle is ex remely rare. Imaging C scanning will show he rac ure usually jus inside he orbi al rim. MRI does no image bone well and should no be used ini ially af er rauma. MRI can be o value o evalua e in racranial injury. Special Considerations Impor an o consul neurosurgery or he po en ial o CNS complica ions wi h a roo rac ure Treatment Repair o a roo rac ure is usually done or neurologic reasons ra her han ocular. Any pla ing and repair is done via cranio omy. Nondisplaced rac ures do no require repair. Prognosis Variable depending on he ex en o associa ed CNS injuries

Orbital Fractures

267

A

B FIGURE 15-3. Orbital roo racture. A.T e pa ien was s ruck in he eye wi h a hydraulic air hose. Examina ion shows signi can swelling, wi h decreased upgaze and supraorbi al hypes hesia. B. C scan shows an orbi al roo rac ure wi h in racranial hemorrhage.

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15 O RBITAL TRAUMA

ZYGOMATIC FR CTU E

Z

ygoma ic rac ures are he resul o signi can rauma ic orce o he zygoma ic area ( Fig. 15-4A–C). T e resul injury and symp om depend on he direc ion and amoun o displacemen o he bone. Repair should be done wi hin he rs week when needed. Epidemiology and Etiology Age: Young adul s Gender: Males mos common E iology: rauma wi h orce direc ed a he zygoma History rauma wi h signi can orce Pa ien s of en complain o pain and di cul y opening heir mou h and chewing. Examination Ini ial ndings may be minimal i here is signi can swelling and ecchymosis o he orbi and cheek. Depressed cheek, orbi al rim s ep o , and inabili y o open he mou h wide are common ndings.

Imaging C scanning shows rac ure o he zygoma ic arch a he ron al-zygoma ic su ure and a he maxillary–zygoma ic su ure. T e zygoma ic arch is displaced in various direc ions depending on he direc ion o rauma. T ere is usually an associa ed orbi al oor rac ure ( Fig. 15-4D–E). MRI does no image bone well and should no be used ini ially af er rauma. Treatment Repair is required or mos rac ures wi h any signi can displacemen . T is should be done as soon as he swelling has lessened. T is is done wi h open reduc ion and pla ing as needed. Nondisplaced rac ures do no require repair. Prognosis Excellen i repaired promp ly

Orbital Fractures

269

A

B FIGURE 15-4. Zygomatic racture. A. A 43-year-old man s ruck on he righ cheek and eye wi h a ba . T ere is f at ening o he cheek wi h rismus. B and C. C scans show a zygoma ic rac ure. ( continued)

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C

D FIGURE 15-4. (Continued) Zygomatic racture. D and E. C scans show a smaller minimally displaced zygoma ic rac ure wi h associa ed orbi al f oor rac ure. ( continued)

Orbital Fractures

E FIGURE 15-4. (Continued)

271

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MISCELLANEOUS TRAUMA O BITAL HEMO

O

HAGE

rbi al hemorrhage as he resul o orbi al rauma is common and rarely requires any speci c rea men ( Fig. 15-5A&B). Spon aneous orbi al hemorrhage is rare and requires evalua ion o he orbi or a source o bleeding, al hough none may be ound. Orbi al or eyelid surgery is also a cause. T e need o drain an orbi al bleed is very rare. Epidemiology and Etiology Age: Any Gender: Males more common because o he higher incidence o rauma E iology: rauma , surgery ( Fig. 15-5E) or orbi al vascular lesion such as lymphangioma or vascular mal orma ion

History His ory is ha o rauma. Wi h vascular mal orma ions, here is sudden onse o orbi al pain, pressure, prop osis, and some imes ecchymosis. Examination Examina ion reveals prop osis wi h variable symp oms depending on he severi y o he hemorrhage. T ere can be o her ocular and orbi al injuries i he cause is rauma. Mild hemorrhage may only reveal prop osis. Severe hemorrhage can resul in no ligh percep ion vision, wi h severe prop osis, corneal exposure, rozen globe, eleva ed in raocular pressure, and inabili y o close he eye because o he severe prop osis ( Fig. 15-5C–D). Imaging C scan: May show a discre e mass or more in l ra ive lesion. Mos commonly

he hemorrhage is di use wi hin he issues MRI: Acu e hemorrhage is hypoin ense on 1 and hyperin ense in 2. When blood is more han 7 days old, i will become hyperinense on 1 and variable on 2. Special Considerations In spon aneous hemorrhage, an orbi al vascular mal orma ion needs o be looked or. I no hing is seen on imaging af er he acu e hemorrhage, a ollow-up MRI wi h gadolinium may iden i y a lesion. Dif erential Diagnosis Spon aneous hemorrhage (no rauma) includes he ollowing. Lymphangioma Venous mal orma ion and varix Ar eriovenous mal orma ion Treatment Observa ion, unless here is visual loss Mild visual loss needs moni oring wi h in ravenous s eroids, ace azolamide, and possible la eral can ho omy. I visual loss is more severe, immedia e la eral can holysis is indica ed along wi h highdose in ravenous s eroids. Orbi al imaging is done o look or locula ed blood. T e blood is usually wi hin he orbi al issues and orbi al drainage or even decompression is rarely o value. T e excep ion would be a locula ed hemorrhage such as in a lymphangioma. Prognosis Chance o permanen visual loss is presen wi h severe hemorrhage. Less severe hemorrhages resolve wi hou sequelae.

Miscellaneous Trauma

273

A

B FIGURE 15-5. Orbital hemorrhage. A. A 17-year-old girl wi h orbi al hemorrhage a er being poked in he eye wi h a eld hockey s ick. T ere was no o her injury no ed on C scan. Vision was normal bu he orbi was modera ely igh . She was observed or progressive hemorrhage bu he ullness o he orbi resolved overnigh . B.T ere was a small corneal delle ha resolved wi h lubrica ion and resolu ion o he hemorrhage. ( continued)

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C

D FIGURE 15-5. (Continued) Orbital hemorrhage. C. Severe orbi al hemorrhage a er re robulbar injec ion in pa ien on Coumadin. D. C scan shows di use hemorrhage wi hin he issue and no locula ed blood. No e he s re ching and s raigh ening o he op ic nerve. ( continued)

Miscellaneous Trauma

E FIGURE 15-5. (Continued) Orbital hemorrhage. E. Bila eral orbi al hemorrhage a er blepharoplas y.

275

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O BITAL FO EIGN BODIES

O

rbi al oreign bodies mus always be suspec ed in any kind o orbi al rauma ( Fig. 15-6A–J). Mos oreign bodies are removed surgically wi h he excep ion o cer ain iner ma erials ha are deep in he orbi .

Epidemiology and Etiology Age: Any age Gender: More common in males E iology: Foreign bodies can en er he orbi be ween he globe and he orbi al wall or by double per ora ion o he globe. History T e his ory may be o a speci c oreign body en ering he orbi . he more di icul si ua ion is where here is rauma wi h a poor his ory bu wi h wounds ha could sugges a oreign body. Examination I he oreign body is an erior, i may be palpable or even visible. I i is deeper, here may very ew signs excep an en rance wound, or here may be signi can hemorrhage and swelling .

Imaging Imaging is key o iden i ying and localizing an orbi al oreign body and C scanning is he imaging modali y o choice. MRI should never be done af er rauma unless a me allic oreign body has been ruled ou . Glass, plas ic, and organic oreign bodies may no show up well wi h C scanning. T ese can be bet er visualized wi h MRI scanning, bu even MRI may no show hese oreign bodies. Treatment Orbi al oreign bodies should be removed i hey are organic, cause symp oms, or i hey have sharp edges so ha migra ion could cause damage. T e posi ion o he oreign body can a ec he decision o remove a oreign body. T e more pos erior i is, he more di cul i will be o remove i . Any oreign body lef in place requires counseling o he pa ien abou he po en ial or u ure ex rusion or in ec ion. I a oreign body is suspec ed, wound and orbi al explora ion is required even i imaging is nega ive. Prognosis Good. Organic oreign bodies can someimes be re ained and lead o chronic in amma ion or in ec ion.

Miscellaneous Trauma

277

A

B FIGURE 15-6. Orbital oreign body. A. A 12-year-old sho wi h a BB gun 2 weeks prior. T e child has a longs anding eso ropia. No e he mild ery hema o he la eral righ globe. B. C scan shows he BB in he an erior la eral orbi . BBs can be le in he orbi wi hou a problem. In his case, because o he an erior loca ion and rela ive ease o removal, he BB was removed. ( continued)

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C

D FIGURE 15-6. (Continued) Orbital oreign body. C and D. Mul iple eyelid lacera ions rom being s ruck wi h a wineglass ha broke. A re ained oreign body mus always be suspec ed wi h broken glass. On C scan, a oreign body is no ed. ( continued)

Miscellaneous Trauma

279

E

F FIGURE 15-6. ( Continued) Orbital oreign body. E.T e glass oreign body ha was removed. T e wineglass was leaded crys al, which is why i showed up so well on C scan. F.T e pa ien was s ruck in he eye wi h a pencil 4 mon hs prior. He presen ed wi h mild irri a ion o he le orbi . No e he lump in he le medial can hus. ( continued)

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G

H FIGURE 15-6. (Continued) Orbital oreign body. G. C scan shows a medial orbi al opaci y. H.T e medical orbi al opaci y urned ou o be par o a pencil. ( continued)

Miscellaneous Trauma

281

I

J FIGURE 15-6. ( Continued) Orbital oreign body. I and J.T e pa ien ran in o a bush and an eyelid lacera ion was revealed on examina ion. I. C scan showed no oreign body. J. Explora ion showed mul iple wood ragmen s.

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MUCOCELE

D

es ruc ion o he sinus os ium rom rauma or sinus disease can resul in a mucous- lled sinus ha can hen expand in o he orbi ( Fig. 15-7). Symp oms depend on he loca ion o he mucocele. rea men is usually aimed a excision o he cys and obli era ion o he sinus. Epidemiology and Etiology Age: Any age bu mos common age is 40 o 70 years Gender: More common in males E iology: Blockage o he sinus os ium resul s in a mucous- lled cys ha can expand wi h ime. History T ere is usually a his ory o rauma o he sinuses or a long his ory o sinus disease. T e orbi al process shows very slow insidious onse o prop osis or globe displacemen . More pos erior mucoceles can presen wi h slow visual loss. Rarely, i he mucocele becomes in ec ed, he symp oms can have a rapid progression.

Examination Findings will depend on he loca ion o he mucocele. T e mass expands slowly and will displace he globe, cause prop osis, and, i pos erior, may cause op ic nerve compression and/ or an orbi al apex syndrome. Special Considerations Fron al and e hmoidal sinus mucoceles are he mos common, wi h sphenoid sinus mucoceles being less common. Dif erential Diagnosis Orbi al abscess Primary sinus umor wi h orbi al ex ension Treatment Surgical excision wi h obli era ion o he a ec ed sinus Prognosis T ere can be recurrences. Mos mucoceles are easily rea ed surgically unless ex remely large.

Miscellaneous Trauma

283

A

B FIGURE 15-7. Orbital mucocele. A. A pa ien wi h a his ory o prior acial rac ures presen s wi h he complain ha his le eye is “ou o place.” T e dura ion o his condi ion is unknown. B. C scan shows a large ron al sinus mucocele displacing he globe downward.

Index NO E: Locators ollowed by ‘ ’ re er to f gures.

A Acid and alkali burns, 123 Acquired myogenic ptosis, 76–77 Acquired nasolacrimal duct obstruction, 130–131 Actinic keratosis, 32–33 , 34, 40 pigmented, 4 Acute spastic entropion, 56–57 Ankyloblepharon, 120–121 Anophthalmos, 170 Antibiotic ointment, 54 Apocrine hydrocystoma, 16–17 Aponeurotic ptosis, 78–79 , 90 Arteriovenous mal ormations (AVM), 200–203 , 272 Aspergillosis, 152–153 Atopic disease, 123 AVM. see Arteriovenous mal ormations (AVM)

B Balloon dacryoplasty, 130 Basal cell carcinoma, 18, 20, 24, 30, 36–39 , 40, 42 cystic, 16 pigmented, 4, 44 Bell’s palsy, 64 Benign adenoma, 14 Benign essential blepharospasm, 104–105 , 106 Benign eyelid lesions apocrine hydrocystoma, 16–17 cutaneous horn, 6–7 epidermal inclusion cyst, 8–9 hemangioma, 22–23 molluscum contagiosum, 10–11 nevocellular nevi, 20–21 papilloma, 2–3 seborrheic keratosis, 4–5 syringoma, 14–15 trichoepithelioma, 18–19 xanthelasma, 12–13 Birth trauma, 116 Blepharitis, 130, 132 Blepharophimosis, 108–109 , 110 Botulinum toxin injection, 104

Brow ptosis, 96–97 Burns, 54–55

C Canalicular eyelid laceration, 50–51 Canalicular obstruction, 132–133 Canaliculitis, 137–138, 139 Capillary hemangiomas, 180–183 , 218, 222, 226 Cavernous hemangiomas, 184–187 , 192, 208 Cavernous sinus thrombosis, 146, 150 Central eyelid laceration, 49 Chalazion, 8, 24–25 , 46 Cherry angioma. see Hemangioma Cholesteatoma, 236 Chronic blepharitis, 42 Chronic chalazion, 42 Chronic conjunctivitis, 126, 137 Chronic progressive external ophthalmoplegia (CPEO), 74, 82 Cicatricial ectropion, 62, 66–67 , 68 Coloboma, 116–117 Congenital distichiasis, 72 Congenital entropion, 112, 114–115 Congenital eyelid anomalies ankyloblepharon, 120–121 blepharophimosis, 108–109 coloboma, 116–117 distichiasis, 118–119 entropion, 114–115 epiblepharon, 112–113 epicanthus, 110–111 Congenital myogenic ptosis, 74–75 Congenital nasolacrimal duct obstruction, 126–127 Congenital orbital anomalies microphthalmos, 170–173 Congenital orbital tumors dermoid cysts, 174–177 lipodermoids, 178–179 Congenital ptosis, 76, 78 Conjunctival granulomas, 164 Conjunctival papillomas, 2 Contralateral ptosis, 102 Copious corneal lubrication, 54

Corneal abrasion, 57 CPEO, see chronic progressive external ophthalmoplegia (CPEO) Cryptophthalmos, 120 Cutaneous horn, 6–7

D Dacryoadenititis, 164 Dacryocystitis, 134–135, 136 , 140 Dacryocystocele, 128 Dacryocystorhinostomy, 130 Dermal nevus, 2 Dermatochalasis, 98–99 , 100 Dermatof broma, 20 Dermoid cysts, 174–177 Discoid lupus, 32 Distichiasis, 118–119 Dog bites, 48, 52 with eyelid lacerations, 53

E Eaton–Lambert syndrome, 82 Eccrine hydrocystoma, 16 Ectropion cicatricial, 66–67 involutional, 62–63 mechanical, 68–69 paralytic, 64–65 symblepharon, 70–71 trichiasis, 72–73 Electrical burn with necrosis, 55 Entropion, 58, 60, 114–115 acute spastic, 56–57 , 58 cicatricial, 56, 58, 60–61 , 72, 122 congenital, 112, 114–115 involutional, 56 spastic, 72 Epiblepharon, 112–113 , 114 Epicanthus, 110–111 Epidermal inclusion cyst, 8–9 , 10, 18 Epithelial tumors, 240–245 Eyelid abscess, 26 burns, 54–55 in ammation

285

286

INDEX

Eyelid (cont.) chalazion, 24–25 oppy eyelid syndrome, 28–29 hordeolum, 26–27 neoplasms actinic keratosis, 32–33 basal cell carcinoma, 36–39 Kaposi’s sarcoma, 46–47 keratoacanthoma, 30–31 lentigo maligna, 34–35 malignant melanoma, 44–45 sebaceous adenocarcinoma, 42–43 squamous cell carcinoma, 40–41 retraction, 102–103 trauma canalicular eyelid laceration, 50–51 dog bites, 52–53 eyelid burns, 54–55 marginal eyelid laceration, 48–49 Eyelid malpositions Brow ptosis, 96–97 dermatochalasis, 98–99 dyskinesis benign essential blepharospasm, 104–105 hemi acial spasm, 106–107 ectropion cicatricial, 66–67 involutional, 62–63 mechanical, 68–69 paralytic, 64–65 symblepharon, 70–71 trichiasis, 72–73 entropion acute spastic entropion, 56–57 cicatricial entropion, 60–61 involutional entropion, 58–59 mechanical ptosis, 90–91 neurogenic ptosis Horner’s syndrome, 88–89 marcus gunn jaw winking syndrome, 84–87 myasthenia gravis, 82–83 third nerve palsy, 80–81 pseudoptosis, 94–95 ptosis acquired myogenic, 76–77 aponeurotic, 78–79

congenital myogenic, 74–75 traumatic, 92–93 retraction, 102–103

F Fat prolapse, 178 Fibrous histiocytoma, 184, 192, 208, 218, 226–227 Floor racture, 258–261 Floppy eyelid syndrome, 28–29 Fractures, orbital trauma oor, 258–261 medial wall, 262–265 orbital roo , 266–267 zygomatic, 268–271

H Hemangioma, 22–23 , 46 Hemangiopericytoma, 184, 192– 195 , 218, 226 Hemi acial spasm, 104, 106–107 Histiocytic disorders, 232, 236–239 Hordeolum, 26–27 Horner’s syndrome, 88–89 Hyperkeratotic actinic keratosis, 30 Hypotropia, 102

I Idiopathic orbital in ammation. see Orbital pseudotumor Idiopathic orbital pseudotumor, 164 Intracranial meningiomas, 210 Involutional ectropion, 62–63 , 68 Involutional entropion, 56, 58–59 , 60, 72

K Kaposi’s sarcoma, 46–47 Keratitis sicca, 130, 132 Keratoacanthoma, 10, 30–31 , 40

L Lacrimal f stula, 129 Lacrimal gland tumors epithelial tumors, 240–245 Lacrimal in ections canaliculitis, 137–138, 139 dacryocystitis, 134–135, 136

Lacrimal obstructions acquired nasolacrimal duct, 130–131 canalicular, 132–133 congenital nasolacrimal duct, 126–127 dacryocystocele, 128 lacrimal f stula, 129 Lacrimal sac tumor, 134, 140–141 Lentigo maligna, 34–35 Lipodermoids, 178–179 Lymphangiomas, 188–189, 190 –191 , 196, 208, 210, 228, 272 Lymphoid hyperplasia and lymphomas, 228–231 Lymphoid inf ltration o lacrimal gland, 240 Lymphoma, 160, 178, 252 Lymphoproli erative tumors histiocytic disorders, 236–239 lymphoid hyperplasia and lymphomas, 228–231 plasmacytoma, 232–235

M Malignant melanoma, 20, 34, 44–45 survival in relation to tumor depth, 44t Marcus gunn jaw winking syndrome, 84–87 Marginal eyelid laceration, 48–49 Mechanical ectropion, 68–69 Mechanical ptosis, 90–91 Medial wall racture, 262–265 Melanocytic nevus, 46 Melanoma, 22 Meningiomas, 210–217 Mesenchymal tumors f brous histiocytoma, 226–227 rhabdomyosarcoma, 222–225 Metastatic breast carcinoma, 253 Metastatic disease, 160, 232 Metastatic orbital tumors, 143, 152, 228, 252–257 Metastatic tumor, 222 Microphthalmos, 120, 170–173 Migrated punctal plug, 137 Molluscum contagiosum, 2, 8, 10 eyelid margin, lesions o , 11 Mucocele, 282–283 rontal and ethmoidal sinus, 282 sinusitis with, 152

INDEX Mucormycosis. see Phycomycosis Multiple myeloma, 232 Myasthenia gravis, 74, 78, 82–83 Myokymia, 106

N Neural tumors meningiomas, 210–217 neurof bromas, 208–209 optic nerve gliomas, 204–207 schwannomas, 218–221 Neurof bromas, 208–209 Neurof bromatosis, 204 Neurogenic ptosis, 76 Nevi. see Nevocellular nevi Nevocellular nevi, 20–21 Nevus, 44

O OCP. see Ocular cicatricial pemphigoid (OCP) Ocular cicatricial pemphigoid (OCP), 122–123, 124 –125 Oculopharyngeal dystrophy, 74 Optic nerve gliomas, 204–207 , 210 Optic nerve meningioma, 204 Orbital abscess, 143, 146–149 , 282 Orbital cellulitis, 142–143, 143 –145 , 146, 150, 155, 160, 222 Orbital oreign bodies, 276–281 Orbital hemorrhage, 272–275 Orbital in ections abscess, 146–149 aspergillosis, 152–153 cellulitis, 142–143, 143 –145 phycomycosis, 150–151 Orbital in ammation pseudotumor, 160–163 sarcoidosis, 164–167 RO, 154–155, 156 –159 Wegener’s granulomatosis, 168–169 Orbital in ammatory disease, 100 Orbital lymphoma, 155 Orbital neoplasms congenital orbital tumors dermoid cysts, 174–177 lipodermoids, 178–179

lacrimal gland tumors epithelial tumors, 240–245 lymphoproli erative tumors histiocytic disorders, 236– 239 lymphoid hyperplasia and lymphomas, 228–231 plasmacytoma, 232–235 mesenchymal tumors f brous histiocytoma, 226–227 rhabdomyosarcoma, 222–225 metastatic orbital tumors, 252–257 neural tumors meningiomas, 210–217 neurof bromas, 208–209 optic nerve gliomas, 204–207 schwannomas, 218–221 secondary orbital tumors, 246–251 vascular orbital tumors AVM, 200–203 capillary hemangiomas, 180–183 cavernous hemangiomas, 184–187 hemangiopericytoma, 192–195 lymphangiomas, 188–189, 190 –191 orbital varices, 196–199 Orbital pseudotumor, 143, 146, 150, 155, 160–163 , 208, 222, 228, 252 Orbital roo racture, 266–267 Orbital trauma oreign bodies, 276–281 ractures oor, 258–261 medial wall, 262–265 orbital roo , 266–267 zygomatic, 268–271 hemorrhage, 272–275 mucocele, 282–283 Orbital varices, 196–199

P Papilloma, 2–3 Paralytic ectropion, 62, 64–65 , 68 Parinaud syndrome, 102

287

Phycomycosis, 143, 146, 150–151 , 152 Pigmented actinic keratosis, 4 Plasmacytoma, 232–235 Pleomorphic adenoma, 240 Polyclonal lymphocytic populations, 228 Preseptal cellulitis, 26, 143 Prolapsed lacrimal gland, 178 Pseudoptosis, 94–95 Pseudotumor, 160–163 Punctal abnormalities, 130 Punctal dysgenesis, 126 Pyogenic granuloma, 22, 46

R Rhabdomyosarcoma, 180, 222–225 Ruptured dermoid cyst, 160, 222

S Sarcoidosis, 164–167 , 240 Schwannomas, 184, 192, 218–221 , 226 Sebaceous adenocarcinoma, 24, 42–43 Seborrheic keratosis, 4–5 , 20, 34 pedunculated, 2 Secondary orbital tumors, 246–251 Sinus tumor, 282 Solitary neurof broma, 2 Spastic entropion, 72 SPK. see Superf cial punctate keratitis (SPK) Squamous cell carcinoma, 24, 32, 36, 40–41 , 42 Stevens-Johnson syndrome, 123 Superf cial punctate keratitis (SPK), 58 Symblepharon, 70–71 Syringoma, 8, 10, 14–15 , 18

T etanus immunization, 48, 52 T ird nerve palsy, 80–81 , 82 T yroid-related ophthalmopathy ( RO), 100, 154–155, 156 –159 , 160 rachoma, 123 raumatic ptosis, 90, 92–93

288

INDEX

richiasis, 72–73 , 122, 126 richoepithelioma, 18–19 , 36

V Vascular orbital tumors AVM, 200–203 capillary hemangiomas, 180–183 cavernous hemangiomas, 184–187 hemangiopericytoma, 192–195

lymphangiomas, 188–189, 190 –191 orbital varices, 196–199 Venous mal ormation and varix, 272 Verruca vulgaris, 4

W WEBOF. see White-eyed blowout racture (WEBOF) Wegener’s granulomatosis, 168–169 , 252

White-eyed blowout racture (WEBOF), 259

X Xanthelasma, 12–13

Z Zygomatic racture, 268–271