Cornea (Color Atlas & Synopsis of Clinical Ophthalmology Wills Eye Institute) [2nd Edition] 818473719X, 9781609133382, 1609133382

Citation preview

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

xiii

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.

14

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.

16

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.

18

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.

20

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.

22

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.)

24

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.)

26

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.

28

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.

30

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.

38

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.

40

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.

44

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.

46

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.

48

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)

50

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.

52

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)

54

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)

56

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.

58

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)

60

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)

62

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.

64

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)

66

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.

68

2 CO NJUNCTIVAL DEGENERATIO NS AND MASS LESIO NS

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.

72

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.

74

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.)

76

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

77

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.

78

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

79

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)

80

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

81

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.

82

3 ANTERIO R SEGMENT DEVELO PMENTAL ANO MALIES

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)

84

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.

86

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

5 CO RNEAL DYST RO PHIES

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)

118

5 CO RNEAL DYST RO PHIES

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.

120

5 CO RNEAL DYST RO PHIES

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)

122

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.

124

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.

126

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.

128

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)

130

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.

132

5 CO RNEAL DYST RO PHIES

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)

134

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.

136

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)

138

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.

140

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)

146

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.

148

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)

150

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)

152

6 CO RNEAL DEGENERATIO NS AND DEPO SITS

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.

154

6 CO RNEAL DEGENERATIO NS AND DEPO SITS

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.

156

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.

158

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.

160

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)

162

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.

164

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.

166

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.

170

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)

172

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.

174

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

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)

176

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

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.

178

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

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)

180

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

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.

182

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

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.

184

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

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)

186

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

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.”

188

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

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)

190

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

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.

192

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

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.

194

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

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.

196

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

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.

198

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.

202

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)

204

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.

206

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.

208

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.

210

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.

212

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 (