4 115153222871553228

EDITORS
Mitchell S. Fineman, MD
Associate Professor of Ophthalmology
Thomas Jefferson University
Attending Surgeon
Wills Eye Institute
Philadelphia, Pennsylvania
Allen C. Ho, MD
Professor of Ophthalmology
Thomas Jefferson University
Attending Surgeon
Wills Eye Institute
SECTION EDITORS
Gary C. Brown, MD
Franco M. Recchia, MD
Carl D. Regillo, MD
James F. Vander, MD
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
Senior Executive Editor: Jona han W. Pine, Jr.
Senior Product Managers: Emilie Moyer and Grace Capu o
Senior Manu acturing 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
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
Re ina / edi ors, Mi chell S. Fineman, Allen C. Ho. – 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 978-1-60913-336-8 (pbk. : alk. paper)
I. Fineman, Mi chell S. II. Ho, Allen C. III. Wills Eye Hospi al
(Philadelphia, Pa.) IV. Series: Color a las and synopsis o clinical
oph halmology series.
[DNLM: 1. Re inal Diseases–A lases. WW 17]
617.7 35–dc23
2011052818
Care has been aken o con rm he accuracy o he in orma ion presen ed and o describe generally ac-
cep 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
T is edition is dedicated in memory o our colleague and mentor,
J. Arch McNamara (1955–2010), whose clinical skills and passion
or teaching will be missed by all who knew him, were inspired by
him, and had the pleasure o his f iendship.
Edi ors
SERIES EDI OR SEC ION EDI ORS
Christopher J. Rapuano, MD Gary C. Brown, MD
Direc or and At ending Surgeon, Cornea Service Pro essor o Oph halmology
Co-Direc or, Re rac ive Surgery Depar men T omas Je erson Universi y
Wills Eye Ins i u e Direc or, Re ina Service
Pro essor o Oph halmology Wills Eye Ins i u e
Je erson Medical College o T omas Je erson Philadelphia, Pennsylvania
Universi y
Franco M. Recchia, MD
Associa e Pro essor o Oph halmology and
Visual Sciences
EDI ORS
Vanderbil Universi y School o Medicine
Mitchell S. Fineman, MD Nashville, ennessee
Associa e Pro essor o Oph halmology
Carl D. Regillo, MD
T omas Je erson Universi y
Pro essor o Oph halmology
At ending Surgeon
Wills Eye Ins i u e
Direc or, Clinical Re ina Research
Wills Eye Ins i u e
Allen C. Ho, MD Philadelphia, Pennsylvania
James F. Vander, MD
At ending Surgeon
Wills Eye Ins i u e
At ending Surgeon
Wills Eye Ins i u e
vi
Con ribu ors
J. Luigi Borrillo, MD Nikolas J.S. London, MD
Nor hern Cali ornia Re ina Vi reous Associa es Fellow, Vi reore inal Surgery
Medical Group Wills Eye Ins i u e
San Ma eo, Cali ornia Philadelphia, Pennsylvania
Richard S. Kaiser, MD Mithlesh C. Sharma, MD
Associa e Pro essor o Oph halmology At ending Vi reore inal Surgeon
T omas Je erson Universi y Kaiser Permanen e Medical Group
At ending Surgeon Roseville, Cali ornia
Wills Eye Ins i u e
vii
Abou he Series
he beau y o he a las/ synopsis concep volumes. T e goal o he series is o provide an
is he power ul combina ion o illus ra- up- o-da e clinical overview o he major areas
ive pho ographs and a summary approach o oph halmology or s uden s, residen s, and
o he ex . Oph halmology is a very visual prac i ioners in all o he heal h care pro es-
discipline ha lends i sel nicely o clinical sions. T e abundance o large, excellen qual-
pho ographs. Al hough he seven oph hal- i y pho ographs and concise, ou line- orm ex
mic subspecial ies in his series—Cornea, will help achieve ha objec ive.
Re ina, Glaucoma, Oculoplas ics, Neuro-
Oph halmology, Pedia rics, and Uvei is—use Chris opher J. Rapuano, MD
varying levels o visual recogni ion, a rela ively Series Editor
s andard orma or he ex is used or all
viii
Pre ace
V i reore inal disease is a privileged visual
discipline. T ere are signi can barri-
ers o i s s udy beyond he cons ric ed pupil.
manual bu realize ha i canno be an ency-
clopedic re erence.
T e images o his color a las and synopsis
Oph halmology rainees rs acquire he
include over 300 color images and over 100
observa ional skills and acili y wi h diagnos-
black and whi e images, ypically uorescein
ic ins rumen a ion such as he sli lamp bio-
angiographic images. Each was digi ized rom
microscope and he indirec oph halmoscope
an original pho ographic slide as a high resolu-
o begin o explore diseases ha a ec he
ion RGB image, a leas 1500 pixels by 1200
pos erior segmen o he eye. I akes clinical
pixels. Our goals were o presen he images in
experience o discern normal varia ion rom
heir highes quali y na ive colors and con ras s,
signi can pa hology. Un or una ely, mos
o limi pho ographic ar i ac , and o highligh
nonoph halmic physicians are limi ed o acil-
cer ain clinical ea ures o he images wi h anno-
i y wi h he direc oph halmoscope ha only
a ions or image inse s. Every e or was made
a ords a keyhole view o he back o he eye.
o main ain he in egri y o he original pho o-
We are privileged o be li elong s uden s,
graphs, wi h requen re erence o he original
prac i ioners, clinical researchers and each-
source. Image enhancemen was reserved only
ers o his aspec o he eye here a Wills Eye
or he selec ed image inse s o his work, in
Hospi al.
cases where we el par icular ea ures could be
When we were asked o crea e a concise color bet er illus ra ed wi h digi al manipula ion. We
a las and synopsis o vi reore inal disease we o en magni ed ( hough a no ime was in er-
knew our challenges would be o be concise pola ion used o crea e new pixels) he inse s,
and o be selec ive since here is grea rich- made hem grayscale, and increased he con-
ness o clinical de ail, bo h visually and wi h ras or he ease o he reader.
words. Our aim was o balance he bread h
Ul ima ely, our in en is o presen his color
o he subjec ma erial wi h enough ocused
a las and synopsis as an aid o he diagnosis
de ail o provide he ramework o our hink-
and managemen o vi reore inal diseases in
ing regarding impor an clinical signs, asso-
he care o pa ien s and as a resource or s u-
cia ed clinical signs, di eren ial diagnosis,
den s o hese condi ions.
diagnos ic evalua ion, and prognosis and
managemen o hundreds o vi reore inal Mi chell C. Fineman, MD
condi ions. We wan his o be a “go o” eld Allen C. Ho, MD
Editors
ix
Acknowledgmen s
G ra e ul acknowledgmen is given o hese consul an s or heir e or s and exper ise in
imaging and or heir con ribu ions o his publica ion:
Ms. S e anie Carey, BS Ms. Lisa Lave sky
Ms. Donna Galloway Henry C. Lee, MD
Ms. Saman ha Groch Ms. Elaine Liebenbaum, BS
Ms. MaryAnn Jay Ms. Michele Skibo
Jay Klancnik, MD Ms. Kris en Winkelspech
x
Con en s
Edi ors vi
Con ribu ors vii
Abou he Series viii
Pre ace ix
Acknowledgmen s x
Ch a pt er 1 Age-Related Macular Degeneration 1

Allen C. Ho
Dry or Nonexuda ive Age-Rela ed Macular Degenera ion 1
Exuda ive Age-Rela ed Macular Degenera ion 17
Ch a pt er 2 Macular Diseases 44
Nikolas J.S. London and Mitchell S. Fineman
Macular Epire inal Membrane 44
Idiopa hic Macular Hole 49
Vi reomacular rac ion Syndrome 58
Cys oid Macular Edema 60
Polypoidal Choroidal Vasculopa hy 64
Degenera ive Myopia 67
Angioid S reaks 72
Cen ral Serous Re inopa hy 78
Choroidal Folds 89
Hypo ony Maculopa hy 92
Ch a pt er 3 Diabetic Retinopathy 94
James F. Vander
Diabe ic Re inopa hy 94
Nonproli era ive Diabe ic Re inopa hy 95
Proli era ive Diabe ic Re inopa hy 112
Diabe ic Papillopa hy 132
Ch a pt er 4 Retinal Vascular Disease 133

Gary C. Brown
Cot on-Wool Spo s 133
Hyper ensive Re inopa hy 136
Ciliore inal Ar ery Obs ruc ion (Occlusion) 142
Branch Re inal Ar ery Obs ruc ion (Occlusion) 145
Cen ral Re inal Ar ery Obs ruc ion (Occlusion) 149
Acu e Oph halmic Ar ery Obs ruc ion (Occlusion) 153
Combined Cen ral Re inal Ar ery and Vein Obs ruc ion (Occlusion) 156
Ocular Ischemic Syndrome 158
Branch Re inal Vein Obs ruc ion (Occlusion) 163
Cen ral Re inal Vein Obs ruc ion (Occlusion) 166
Re inal Ar erial Macroaneurysm 172
xi
xii CO NTENTS
Para oveal elangiec asis 175

Sickle Cell Re inopa hy 178
Radia ion Re inopa hy 182
Lipemia Re inalis 187
Ch a pt er 5 Retinal Degenerations and Dystrophies 189

Mithlesh C. Sharma and Allen C. Ho
Bes ’s Disease 189
Cone Dys rophy 195
Pat ern Dys rophy 198
S argard ’s Disease 203
Choroideremia 210
Gyra e A rophy 215
Congeni al S a ionary Nigh Blindness 219
Albinism 223
Re ini is Pigmen osa 228
Sys emic Diseases Associa ed wi h Re ini is Pigmen osa 233
Carcinoma-Associa ed Re inopa hy Syndrome 236
Ch a pt er 6 Retinal and Choroidal umors 240

Franco M. Recchia
As rocy ic Hamar oma 240
Re inoblas oma 243
Re inal Capillary Hemangioma 247
Re inal Cavernous Hemangioma 250
Congeni al Hyper rophy o he Re inal Pigmen Epi helium 252
Combined Hamar oma o he Re ina and Re inal Pigmen Epi helium 255
Choroidal Nevus 257
Choroidal Melanoma 259
Choroidal Melanocy oma 262
Choroidal Me as asis 264
Choroidal Hemangioma 267
In raocular Lymphoma 269
Choroidal Os eoma 271
Ch a pt er 7 Congenital and Pediatric

Retinal Diseases 273
Nikolas J.S. London and Richard S. Kaiser
Re inopa hy o Prema uri y 273
Incon inen ia Pigmen i 284
Familial Exuda ive Vi reore inopa hy 289
Coa s’ Disease 294
Choriore inal Coloboma 300
Persis en Hyperplas ic Primary Vi reous/ Persis en Fe al Vascula ure 304
Juvenile X-Linked Re inoschisis 307
Leber’s Congeni al Amaurosis 312
CO NTENTS xiii
Ch a pt er 8 raumatic and oxic Retinopathies 314

J. Luigi Borrillo and Carl D. Regillo
Commo io Re inae 314
Choroidal Rup ure 316
Avulsed Vi reous Base 318
Solar Maculopa hy 319
Valsalva Re inopa hy 321
Shaken Baby Syndrome 323
erson’s Syndrome 325
Pur scher’s Re inopa hy 327
rauma ic Macular Hole 329
Choriore ini is Sclope aria 331
In raocular Foreign Body 333
Disloca ed Lens 336
alc Re inopa hy 338
Chloroquine or Hydroxychloroquine Re inopa hy 341
T ioridazine Re inopa hy 343
Ch a pt er 9 Peripheral Retinal Disease 346
James F. Vander
Re inal Break or ear 346
Rhegma ogenous Re inal De achmen 357
Proli era ive Vi reore inopa hy 366
Lat ice Degenera ion 372
Vi reore inal u and Meridional Fold 374
Cobbles one Degenera ion 376
Peripheral Grouped Pigmen a ion 378
Degenera ive Re inoschisis 380
Exuda ive Re inal De achmen 383
Choroidal De achmen 386
Index 389
C H AP ER
Age-Rela ed Macular
Degenera ion
Allen C. Ho
A ge-rela ed macular degenera ion (AMD)

describes a common degenera ive con-
di ion o he re ina ha may a ec cen ral
(63 µm or smaller); calcif ed drusen, which are
yellow and glis ening; and basal laminar dru-
sen, which are small round di use drusen ha
vision. By def ni ion, i occurs in individu- are more apparen on uorescein angiography
als 50 years and older and is more prevalen han on clinical undus examina ion (Fig. 1-3).
wi h increasing age. Popula ion-based surveys Mul iple large drusen are represen a ive o a
in he Wes ern world vary bu es ima e he di use hickening o Bruch’s membrane. Large
prevalence o AMD o be approxima ely 10% drusen, also known as so drusen, are a risk ac-
o 35% in individuals over he age o 50 years. or or more advanced AMD and vision loss.
AMD is divided in o “dry” or nonexuda ive Small (also known as hard) drusen alone do
AMD and “we ” or exuda ive AMD. no increase he risk or more advanced orms
o AMD (Fig. 1-4).
Re inal pigmen epi helial abnormali-
ies, including nongeographic a rophy, ocal
DRY
D RY O R N O N EX
EXUDA
XU DA
A IIVE
VE
E hyperpigmen a ion, and rank geographic
AGE-RELA
AGE-RR EL
L A ED M MACULAR
AC UL
L AR
R a rophy, are also common undus ea ures
DEGEN
D EGE
EN EERA
R A IIO
ON o dry AMD (Fig. 1-5). Granulari y o he
RPE may be an early ea ure o re inal pig-
D rusen are he clinical hallmark o dry

AMD. T ey are subre inal pigmen epi-
helial deposi s be ween he basemen mem-
men epi helial dis urbance due o AMD.
T is may progress o areas o nongeographic
a rophy (Fig. 1-6) in which here is loss o
brane o he re inal pigmen epi helium (RPE) pigmen o he RPE, bu his is no discre e,
and Bruch’s membrane (Figs. 1-1 and 1-2) or and underlying choroidal vessels are no
wi hin Bruch’s membrane i sel . Mul iple ypes apparen . Geographic a rophy comprises dis-
o drusen have been described, including large cre e loss o RPE in a so-called cookie-cut er
drusen (grea er han 64 µm); small drusen ashion wi h a minimal diame er o 250 µ m
1
2 1 AGE-RELA ED MACULAR DEGENERA IO N
associa ed wi h underlying loss o choroidal and associa ed re inal pigmen epi helial
s romal pigmen and clearly visible underly- abnormali ies including granulari y o he
ing larger choroidal vessels (Fig. 1-7). RPE, a rophy o he RPE, or ocal hyperpig-
men a ion will o en no e uc ua ing vision,
including cen ral blurring. T ey ypically will
EPIDEMIOLOGY AND
describe a need or increased ligh in ensi y
ETIOLO GY in order o read and have di cul y adap ing
be ween di eren ligh ing.
Drusen are seen increasingly wi h advanc-
Pa ien s wi h dry AMD and wi hou evi-
ing age and ypically are presen in he six h
dence o geographic a rophy o he RPE or
decade o li e or la er. Popula ion-based s ud-
exuda ive AMD ypically have good cen ral
ies es ima e approxima ely 10% prevalence
vision be ween 20/ 20 and 20/ 60.
o early AMD (drusen) in he f h decade o
li e, increasing o 35% in he seven h decade.
CLINICAL AND
Drusen may be seen in younger pa ien s and
may be heri able in hese cases. FLUORESCEIN
T e precise source o drusen ma erial is no ANGIO GRAPHIC SIGNS
comple ely unders ood, bu hey are hough
Fundus biomicroscopy shows subre inal
o represen degenera ive produc s o re inal
pale yellow deposi s ha may vary in size rom
pigmen epi helial cells; hey are composed o
grea er han 64 µm (large drusen) o small or
lipids and glycopro eins, and may be mineral-
hard drusen (63 µm or smaller) in diame er.
ized. Re inal pigmen epi helial al era ions are
Calcif c drusen have a glis ening appearance,
seen increasingly wi h age and are common in
and mos pa ien s wi h AMD have a mix ure o
he seven h, eigh h, and nin h decades o li e.
clinical drusen ypes. Large drusen will o en
become con uen in o larger drusenoid pig-
PATHOLO GY men epi helial de achmen s. Drusen should
be considered uid and dynamic s ruc ures
ransmission elec ron microscopy o eyes ha can appear or resolve over ime (Fig. 1-8).
wi h drusen and dry AMD shows wo ypes
An irregular granular appearance o he
o deposi s:
RPE is o en seen in associa ion wi h drusen.
Basal laminar deposi s consis o wide- Areas o nongeographic a rophy or rank geo-
spaced collagen localized be ween he re i- graphic a rophy are o en apprecia ed a er he
nal pigmen epi helial plasma membrane spon aneous resolu ion o drusen and, in par-
and he re inal pigmen epi helial base- icular, drusenoid pigmen epi helial de ach-
men membrane. men s. In rare inal pigmen clumps or ocal
Basal linear deposi s consis o lipid-rich hyperpigmen a ion represen s advanced re i-
ma erial ex ernal o he basemen mem- nal pigmen epi helial degenera ion as well.
brane o he RPE in he inner collagenous Fluorescein angiography ypically demon-
zone o Bruch’s membrane. s ra es a pa chy hyper- and hypo uorescence
wi hou leakage o dye. Drusen may show
HISTORY early or la e hyper uorescence, depending
on he in egri y o he overlying RPE and
Pa ien s wi h drusen may be visually he his ochemis ry o he drusen hem-
asymp oma ic. Pa ien s wi h mul iple drusen selves. Large so drusen ypically show early
Dry or Nonexudative Age Related Macular Degeneration 3
hypo uorescence and la e hyper uorescence be evalua ed wi h uorescein angiography o

(Fig. 1-9). T is angiographic pat ern is incon- rule ou exuda ive AMD.
sis en , however, because some drusen, even Care ul undus biomicroscopy is impor-
hose ha are large, will show earlier hyper- an o rule ou sub le signs o exuda ive
uorescence (Fig. 1-10). Geographic a rophy AMD.
shows discre e hyper uorescence wi h s able
Op ical coherence omography (OC ) is
boundaries hroughou he angiogram
an essen ial diagnos ic ool in he evalua ion
(Fig. 1-11).
o AMD pa ien s and can help es ablish he
presence o early exuda ive AMD and help
ASSO CIATED CLINICAL de ermine he response o herapy.
SIGNS
PROGNOSIS AND
When drusen are no ed in pa ien s over
he age o 50, o her ea ures o AMD are o en MANAGEMENT
observed, including granulari y and a rophy
o he RPE. Drusen ha are associa ed wi h Pa ien s wi h drusen are counseled ha
subre inal uid, hemorrhage, or lipid exuda- hey have he dry orm o AMD and ha
ion due o choroidal neovasculariza ion mos pa ien s wi h drusen will no develop
(CNV) are charac eris ics o exuda ive AMD. vision loss due o more advanced orms o
AMD (exuda ive AMD and CNV or geo-
graphic a rophy). Pa ien s wi h mul iple
DIFFERENTIAL DIAGNOSIS large drusen are a a higher risk o develop-
ing CNV, par icularly i he ellow eye has
Drusen are subre inal and should be dis- previously developed exuda ive AMD
inguished rom in rare inal processes such as ( able 1-1). T e 5-year risk o developing
in rare inal lipid, re inal emboli, and cot on- CNV in ellow eyes o pa ien s wi h exuda-
wool spo s. T e borders o drusen may be ive AMD ranges be ween 40% and 85%.
more dis inc in smaller hard drusen and less Managemen includes counseling regarding
dis inc wi h large drusen. he impor ance o moni oring cen ral vision
O her yellow macular lesions can be in each eye wi h a es objec such as he
included in he di eren ial diagnosis o dru- Amsler grid (Fig. 1-12).
sen, including he ollowing:
Pat ern dys rophy: Presen s in younger
TABLE 1-1. Risk o Choroidal
pa ien s; lesions show geographic shape.
Neovasculariza ion (CNV) or Eyes wi h
Bes ’s disease: Round or oval lesions Drusen (Fellow Eye wi h Exuda ive CNV)
may show di eren s ages.
Overall estimate is 10%of patients per year with
Adul oveomacular dys rophy: unilateral drusen will develop CNV. T e Macular
Yellowish green sub oveal lesion; may sim- Photocoagulation Study Group has established risk
ula e CNV on uorescein angiography. factors that increase the risk of CNV:
Multiple large drusen
DIAGNOSTIC EVALUATION Focal hyperpigmentation
Hypertension
Pa ien s wi h a sudden change in vision or
Smoking
new blur or dis or ion o cen ral vision may
In orma ion rom he age-rela ed eye believe ha his clinical ea ure may represen
disease s udy (AREDS) demons ra es ha a sign o early, ill-def ned CNV in many cases.
micronu rien and an ioxidan supplemen a- I here is a suspicion o early exuda ive AMD,
ion (vi amin C, 500 mg; vi amin E, 400 IU; hen uorescein angiography and OC imag-
be a caro ene, 15 mg; zinc, 80 mg as zinc ing may be per ormed. Since early re inal pig-
oxide; and copper, 2 mg as cupric oxide) men epi helial abnormali ies and granulari y
can e ec a modes bu def ni e reduc ion in o he RPE may lead o nongeographic a ro-
clinical progression o AMD and modera e phy and rank geographic a rophy, hey may
visual loss in pa ien s wi h dry AMD and be harbingers o vision loss.
a leas one large druse o 125 µ m or larger. T ere are numerous clinical rials inves-
Da a were no signif can or pa ien s wi h iga ing po en ial herapies or dry AMD
mild or borderline dry AMD (mul iple small including o her nu ri ional supplemen s such
drusen or nonex ensive in ermedia e drusen as omega 3 at y acids, lu ein and zeaxan hin
o 63 o 124 µ m, pigmen abnormali ies, or caro enoids (AREDS 2 S udy), visual cycle
any combina ion o hese). inhibi ors, an i-in amma ory or complemen
Pa ien s wi h ocal hyperpigmen a- inhibi ors and s em cell herapies. An in raoc-
ion have a higher risk o developing more ular implan able elescope has been approved
advanced orms o AMD associa ed wi h or pa ien s wi h geographic a rophy based on
vision loss and, in par icular, CNV. Some clinical rial evidence.
B C
FIGURE 1-1. Large drusen. A. Fundus pho ograph demons ra ing predominan ly large drusen, some o which
are conf uen (inse ) . Visual acui y was 20/ 25. B and C. Red ree undus image and OC image o drusen a he
level o Bruch’s membrane and he re inal pigmen epi helium. No e he irregular eleva ion o he re inal pigmen
epi helium caused by drusen.
FIGURE 1-2. Conf uent drusen. Fundus pho ograph demons ra ing mul iple large, predominan ly conf uen
drusen. Conf uence is grea es emporal o he ovea. Conf uen drusen are a risk ac or or exuda ive age rela ed
macular degenera ion (AMD) .
FIGURE 1-3. Basal laminar drusen. Fundus pho ograph demons ra ing mul iple small, round, di use drusen
(inse ) wi h large areas o conf uence in he pos erior pole and midperipheral re ina. Basal laminar drusen may be
more apparen wi h f uorescein angiography han clinically.
FIGURE 1-4. Hard drusen. Hard drusen (inse ) are small ( 63 µm or smaller) and are no a risk ac or or more
advanced orms o AMD.
FIGURE 1-5. Focal hyperpigmentation. Fundus pho ograph showing mul iple drusen wi h re inal pigmen
epi helial al era ions (inse ) . Focal hyperpigmen a ion is no ed in he ovea and jus nasal o he ovea. Focal
hyperpigmen a ion is a risk ac or or more advanced orms o AMD associa ed wi h vision loss.
FIGURE 1-6. Nongeographic atrophy. A. Mul iple large drusen are no ed and here are areas o re inal
pigmen epi helial al era ions. Surrounding he ovea superiorly and emporally are wo areas o nongeographic
a rophy. T ere is hinning o he re inal pigmen epi helium (RPE) , bu he borders are no discre e around he
en ire lesion and he underlying larger choroidal vessels are no visible a his ime. B. Fluorescein angiogram
demons ra ing ransmission hyperf uorescence in nongeographic a rophy. La er images do no demons ra e
leakage.
FIGURE 1-7. End stage geographic atrophy. Large geographic a rophy involving he ovea. No e he visibili y
o he underlying larger choroidal vessels. Visual acui y was coun ing ngers.
B
FIGURE 1-8. Drusenoid pigment epithelial detachment. A. Righ eye o a pa ien showing large conf uen
drusen in a drusenoid pigmen epi helial de achmen con gura ion. T ere is ocal hyperpigmen a ion cen ered
on he ovea. Visual acui y was 20/ 40. B. Le eye o he same pa ien showing spon aneous resolu ion o a
drusenoid pigmen epi helial de achmen wi h a residual rim o conf uen large drusen. Visual acui y was 20/ 30.
B
FIGURE 1-9. Multiple large drusen and conf uent drusen. A. Drusen may spon aneously regress and progress
o areas o righ pigmen epi helial a rophy ( arrow) . T ere is loss o oveal pigmen rom spon aneous resolu ion
o drusen. B. Early phase f uorescein angiogram demons ra ing mild rela ive hypof uorescence corresponding o
drusen ( arrow) .
( continued)
C
FIGURE 1-9. (Continued) Multiple large drusen and conf uent drusen. C. Recircula ion phase o f uorescein
angiogram showing s aining o drusen as discre e areas o hyperf uorescence (arrow) .
A
FIGURE 1-10. Multiple large drusen. A. Mul iple large conf uen drusen (inse s) . Visual acui y was 20/ 25.
( continued)
C
FIGURE 1-10. (Continued) Multiple large drusen. B. Fluorescein angiogram showing early hyperf uorescence
o he large drusen ( arrow) . C. La e f uorescein angiogram showing drusen s aining bu no evidence o choroidal
neovasculariza ion (CNV, arrow) .
B
FIGURE 1-11. Atrophic AMD. A. Color undus pho ograph demons ra ing a rophic AMD. Mul iple large
and medium sized drusen are no ed, and an area o geographic a rophy is no ed jus superior o he ovea (inse
upper righ ) . T e borders are discre e, and he larger underlying choroidal vessels are visible. Areas o ocal
hyperpigmen a ion are no ed as well (inse lower righ ) . B. Early phase f uorescein angiogram demons ra ing
ransmission hyperf uorescence in he area o geographic a rophy.
( continued)
D
FIGURE 1-11. (Continued) Atrophic AMD. C. Recircula ion phase pho ograph shows hyperf uorescence in
he area o geographic a rophy bu no evidence o leakage (inse ) . D. La e phase f uorescein angiogram showing
some ading o he choroidal f uorescence and s aining hyperf uorescence in he region o geographic a rophy.
FIGURE 1-12. Amsler grids. Pa ien s are ins ruc ed o moni or heir cen ral vision one eye a a ime.
Dis or ion, blurriness, or missing areas should promp evalua ion.
Exudative Age Related Macular Degeneration 17
EXUDA
E XUD
D A IVE
I VE AG
AGE-RELA
G E -R
R EL
L A ED
D PATHOLO GY
MACULAR
M ACU
U LARRDDEGEN
EG
G EN EERA
RA
A IO N CNV is ypically derived rom choroidal
venules and may invade above and benea h
E xuda ive AMD is charac erized by in ra-
re inal, subre inal or subre inal pigmen epi-
helial leakage, hemorrhage, or lipid exuda ion.
he RPE hrough breaks in Bruch’s membrane.
CNV is he abnormal grow h o new choroidal HISTORY

vessels in o he subre inal space hrough de ec s
in Bruch’s membrane. In AMD, CNV may be Pa ien s wi h exuda ive AMD may no e
inves ed wi hin Bruch’s membrane and he re i- loss o vision, dis or ion o vision, or blurring
nal pigmen epi helium. Younger pa ien s (non- o vision. Early changes may be more eviden
AMD) who develop CNV ypically develop wi h Amsler grid moni oring.
his vasculariza ion in he subre inal space alone. Pa ien s may be asymp oma ic, however, i
Pigmen epi helial de achmen describes a blis er- he ellow eye con inues o unc ion well.
like eleva ion o he re inal pigmen epi helium T ere may be a his ory o loss o vision in
and is ano her orm o exuda ive AMD. Pigmen he ellow eye due o exuda ive AMD.
epi helial de achmen s may be vascularized
(f brovascular pigmen epi helial de achmen )
CLINICAL O CT
or may be purely serous and no associa ed wi h
CNV. Disci orm scarring is a f nal common pa h- AND FLUORESCEIN
way o CNV and pigmen epi helial de achmen ANGIO GRAPHIC SIGNS
in which here is progressive f brosis and loss o
macular pho orecep or unc ion (Fig. 1-13). Care ul sli lamp undus biomicroscopy is
Al hough he prevalence o he exuda- impor an in he diagnosis o exuda ive AMD.
ive orm o AMD is low (approxima ely 10% Clinical signs o re inal edema, subre inal
o all pa ien s wi h AMD), his orm o la e uid, lipid exuda ion and in ra and subre inal
AMD accoun s or he majori y o legally blind hemorrhage can be sub le. In par icular, i is
pa ien s wi h AMD (90%). T e incidence o di cul o de ermine re inal uid when here
exuda ive AMD is on he rise, wi h an es ima ed are areas o RPE depigmen a ion or a rophy.
200,000 individuals experiencing severe cen ral OC imaging o exuda ive AMD may
loss o vision due o exuda ive AMD in he year reveal hickening o he macula or re inal pig-
2011. T is is expec ed o rise o approxima ely men epi helium. T ere is o en in rare inal
500,000 per year by he year 2030. cys ic change, subre inal uid or RPE de ach-
men . Quan i a ive imaging over ime and
wi h image regis ra ion a ords in orma ion o
EPIDEMIOLOGY AND de ermine response o herapy. No all cys ic
ETIOLO GY change may represen rue edema as chronic
re inal uid may crea e ubular changes ha
CNV is he leading cause o cen ral blind- mimic edema.
ness among he elderly in he Uni ed S a es Fluorescein angiographic classif ca ion o
and in mos o he Wes ern world. T e pa ho- exuda ive AMD was par icularly impor an
physiology o exuda ive AMD and he spe- prior o pharmacologic herapy, specif -
cif c s imuli required or he developmen o cally an i-vascular endo helial grow h ac or
CNV are no well unders ood. rea men .
Classic Choroidal Neovascularization la e leakage o unde ermined source, is char-

Fundus biomicroscopy may reveal a green- ac erized by leakage o uorescein dye in he
ish gray subre inal lesion o en wi h associa ed recircula ion phase o he uorescein angio-
submacular uid, hemorrhage, or lipid exuda- gram associa ed wi h pooling o uorescein
ion due o he incompe en vascula ure o dye in he subre inal space and areas o speck-
he CNV. Occasionally, a ring o dark pigmen led hyper uorescence (Fig. 1-16).
may surround he CNV. T e pigmen ring is Mos pa ien s wi h exuda ive AMD dem-
more commonly observed in pa ien s wi h ons ra e a combina ion o classic and occul
classic CNV. Fluorescein angiography is nec- CNV (Fig. 1-17). Charac eriza ion o he di -
essary o charac erize he na ure and loca ion eren ypes o CNV is impor an as manage-
o he CNV. CNV may be sub oveal i local- men recommenda ions are based no only
ized benea h he geome ric cen er o he ovea, on he loca ion o he lesion, bu also on he
jux a oveal i localized rom 1 o 199 µ m rom na ure o he CNV.
he oveal cen er, or ex ra oveal i 200 µ m or
ar her rom he cen er o he ovea.
Fibrovascular Pigment Epithelial
Classic CNV is charac erized by early
Detachment and Serous Pigment
appearance o uorescein leakage during he
Epithelial Detachment
choroidal and re inal vascular f lling phases
o he uorescein angiogram (Fig. 1-14). Fibrovascular pigmen epi helial de ach-
T e leakage may be charac erized by a lacy men is a orm o occul CNV. Purely serous
appearance o a ne work o blood vessels (bu pigmen epi helial de achmen is charac er-
his is no requisi e) and ypically is airly well ized by a blis er-like eleva ion o sub-RPE
delinea ed. More impor an ly, he lesion mus uid. Fundus biomicroscopy shows an orange
demons ra e con inued leakage hrough he ring ha illumina es wi h a sli -lamp ligh
recircula ion and la e phases o he uores- beam. A serous pigmen epi helial de ach-
cein angiogram. men is o en associa ed wi h a serous de ach-
men o he neurosensory macula as well.
Occult Choroidal Neovascularization Fluorescein angiography shows a uni orm
Fundus biomicroscopy may demons ra e rapid f lling o uorescein dye benea h he
irregular eleva ion o he RPE mos com- boundaries o he pigmen epi helial de ach-
monly in associa ion wi h submacular uid, men (Fig. 1-18).
hemorrhage, or lipid exuda ion due o he Fibrovascular pigmen epi helial de ach-
incompe en vascula ure o he CNV; less men s may have associa ed serous re inal
commonly, irregular eleva ion o he RPE pigmen epi helial de achmen s and are o en
alone may comprise he only clinical sign o deno ed by a no ch on he uorescein angio-
occul CNV. Occul CNV is charac erized by gram (Fig. 1-19A, B). T e no ch represen s
la er appearing and less in ense uorescein he CNV. Fibrovascular pigmen epi helial
angiographic leakage. de achmen s are more likely o have a sero-
One ype o occul CNV, f brovascular pig- sanguineous componen in he sub-RPE uid,
men epi helial de achmen , is charac erized and his may be visualized oph halmoscopi-
by irregular s ippled hyper uorescence wi h cally. Indocyanine green angiography may
mild leakage or s aining o uorescein dye be help ul in imaging presumed CNV in he
in poorly demarca ed boundaries o leakage set ing o occul CNV and pigmen epi helial
(Fig. 1-15). T e second ype o occul CNV, de achmen (Fig. 1-19C, D).
Retinal Pigment Epithelial ears Occasionally, re inochoroidal anas omoses

Re inal pigmen epi helial ears may be are seen connec ing he underlying pa ho-
observed in associa ion wi h pigmen epi he- logic CNV and f brosis wi h he re inal
lial de achmen s. A scrolled edge o RPE may circula ion. Fluorescein angiography shows
be apparen wi h undus biomicroscopy and s aining o he disci orm scar and leakage
some imes is associa ed wi h a at ening o he rom any residual ac ive CNV.
re inal pigmen epi helial de achmen . Vision
loss is ypically observed wi h a re inal pigmen
epi helial ear, al hough in some cases, good DIFFERENTIAL DIAGNOSIS
cen ral vision is preserved in he shor erm.
Fluorescein angiography shows early in ense Signs o exuda ive AMD such as re inal
hyper uorescence corresponding o he area hemorrhage, lipid exuda ion, macular edema,
o denuded RPE and rela ive hypo uorescence and submacular uid may be con used wi h
where here is scrolled and redundan re inal non-AMD condi ions. For example, serous
pigmen epi helial issue (Fig. 1-20). de achmen s o he macula may be associa ed
wi h cen ral serous re inopa hy, al hough his
Submacular Hemorrhage is ypically seen in younger pa ien s.
A large submacular hemorrhage may be In rare inal hemorrhage can be seen in
associa ed wi h apoplec ic loss o cen ral vision. re inal venous occlusive disease, hyper ensive
I may be he presen ing mani es a ion o exu- re inopa hy, or diabe ic re inopa hy, bu i
da ive AMD or may ollow he developmen o also may be he presen ing sign o early CNV.
classic or occul CNV, or bo h (Fig. 1-21). Cys oid macular edema may be a presen -
Fundus biomicroscopy shows subre inal ing sign o CNV. Cys oid macular edema due
and some imes prere inal or subre inal pig- o CNV may be con used wi h pseudophakic
men epi helial hemorrhage ha may have sig- cys oid macular edema. Macular edema
nif can eleva ion grea er han 1 mm in heigh . associa ed wi h re inal vascular condi ions
T e ex en o he hemorrhage may be limi ed such as venous occlusive disease, para oveal
or some imes can ex end o involve he en ire elangiec asia, or diabe ic re inopa hy may
pos erior pole or beyond. Vision loss ypically presen in similar ashion o exuda ive AMD.
correla es wi h he ex en o sub oveal involve- S ereoscopic uorescein angiography is
men and he wo-dimensional size o he cri ical o make he correc diagnosis.
submacular hemorrhage. Polypoidal choroidal vasculopa hy is an
Fluorescein angiography may or may no exuda ive maculopa hy ha may be seen in
show source CNV. T e submacular hemor- all races bu usually is observed in A rican
rhage will ypically obscure underlying nor- Americans or Asian Americans in whom
mal and pa hologic choroidal uorescence. exuda ive AMD is less common. T is condi-
ion is charac erized by choroidal aneurysms
Disciform Scar wi h associa ed serosanguineous pigmen
Disci orm scar is he f nal common pa h- epi helial de achmen s and submacular exu-
way o exuda ive AMD. T in or hick f bro ic da ion. T is par icular condi ion is imaged
submacular scarring may be observed, and well wi h indocyanine green angiography,
here may be associa ed chronic submacular whereby choroidal saccular aneurysms may
uid, lipid exuda ion, cys oid macular edema, be highligh ed in a peripapillary dis ribu ion
and submacular hemorrhage (see Fig. 1-13). or in a macular dis ribu ion.
Large submacular or in rare inal hemor- Group Classic es ablished ha hermal laser
rhages can be seen in he set ing o rauma, pho ocoagula ion was he rea men o choice
choroidal umor, or re inal ar erial macroan- or well-delinea ed ex ra oveal CNV. T e major
eurysm, and hese condi ions, in addi ion o problem wi h his herapy is ha up o 60%o
exuda ive AMD, should be considered in he eyes will develop recurren CNVs, he majori y
con ex o his clinical presen a ion. o which are sub oveal (Fig. 1-22). Curren ly,
many specialis s would choose an i-VEGF injec-
PROGNOSIS AND ion herapy or ex ra oveal CNV because i is
less des ruc ive and po en ially more e cacious.
MANAGEMENT
Verteporf n photodynamic therapy
T e cen ral visual prognosis o exuda ive (PD ): Prior o an i-VEGF injec ion herapy
AMD has drama ically improved wi h he he rea men o AMD wi h Pho odynamic
adven o an i-VEGF injec ion herapies; T erapy S udy Group es ablished ha ver e-
peripheral vision ypically remains una ec ed. porf n PD was he rea men o choice
Over 90% o pa ien s can experience main- or predominan ly classic sub oveal CNV.
enance o vision over ime wi h requen Al hough visual improvemen was achieved in
an i-VEGF injec ions. Up o a hird or higher only a minori y o eyes (approxima ely 15% a
o hese pa ien s can experience signif can 1 year), rea ed eyes show more visual s abil-
visual gains wi h his rea men . T ere are a i y han observed eyes over 2 years. rea men
varie y o clinical research rials inves iga ing benef was also es ablished or occul CNV
new rea men s or exuda ive AMD. bu no or mixed lesions ha are less han pre-
dominan ly classic CNV (Fig. 1-23). Curren ly,
ver eporf n PD is generally considered as
Evidence-based and Other T erapies
a po en ial adjunc ive rea men or exuda-
T e curren benchmark ounda ion herapy ive AMD and is o en per ormed a reduced
or exuda ive AMD is mon hly (ranibizumab, energy uence. PD may play a larger role in
Lucen is) o bimon hly (a ibercep , Eylea) he rea men o polypoidal choroidal vascu-
an i-VEGF injec ions as es ablished by ran- lopa hy compared wi h exuda ive AMD.
domized con rolled clinical rials ha requen
moni oring visi s and requen injec ions Surgical T erapies
a ord he highes chance o bes visual acui y.
T e submacular surgery rials did no
Ranibizumab, a ibercep and o -label demons ra e benef or surgical evacua ion
bevacizumab appear o have grea er po ency o large submacular hemorrhage and CNV.
compared wi h pegap anib (Macugen) Surgical displacemen o large submacular
al hough a compara ive rial has no been hemorrhages wi h or wi hou clo lysing
per ormed. Curren ly, here are mul iple clini- agen s such as issue plasminogen ac iva-
cal rials exploring he po en ial enhanced or may improve vision or some pa ien s
e cacy o combining an i-VEGF injec ion and may also reveal underlying CNV—a
herapy wi h o her pharmacologic agen s. his ime here are no surgical clinical rials
T ermal laser photocoagulation: In he exploring he displacemen o large submacu-
1980s he Macular Pho ocoagula ion S udy lar hemorrhage.
FIGURE 1-13. Disci orm scar. A large area o submacular brosis wi h chronic submacular f uid is no ed.
Disci orm scarring is he nal common pa hway or exuda ive AMD. Visual acui y is coun ing ngers eccen rically.
A
FIGURE 1-14. Exudative AMD, classic CNV. A. Shallow submacular f uid and in rare inal hemorrhage are no ed.
( continued)
C
FIGURE 1-14. (Continued) Exudative AMD, classic CNV. B. Ar erio venous phase f uorescein angiogram
showing a car wheel o ex ra oveal classic CNV ( arrow) and hypof uorescence corresponding o re inal
hemorrhage. C. Recircula ion phase pho ograph showing early leakage o dye rom he classic CNV.
( continued)
E
FIGURE 1-14. (Continued) Exudative AMD, classic CNV. D. La e image showing some pooling o dye benea h
he neurosensory re ina. E. T ermal laser pho ocoagula ion is per ormed and shows re inal whi ening.
( continued)
G
FIGURE 1-14. (Continued) Exudative AMD, classic CNV. F. T ree weeks la er here is evidence o a rophy
in he area o laser rea men and resolu ion o he submacular f uid. G. Fluorescein angiogram showing no
evidence o recurren CNV a 3 weeks. T e pa ien remains a risk or he subsequen developmen o recurren
CNV.
B
FIGURE 1-15. Occult CNV. A. Color undus pho ograph demons ra ing drusen and sligh ly urbid submacular
f uid cen ered in erior o he ovea (inse ). B. Recircula ion phase f uorescein angiogram demons ra ing ill
de ned s ippled hyperf uorescence in erior o he ovea. T ere is some drusen s aining as well (inse ).
(continued)
D E
FIGURE 1-15. (Continued) Occult CNV. C. Fluorescein angiogram demons ra ing some mild leakage o
f uorescein dye benea h he ovea. La e f uorescein angiographic images show ill de ned leakage in erior o he
ovea (inse ) . D. Red ree image o occul CNV. E. OC image demons ra ing in rare inal f uid, subre inal f uid
and re inal pigmen epi helial de achmen .
B
FIGURE 1-16. Occult CNV. A. Color undus pho ograph showing urbid submacular f uid and drusen ( arrow) .
B. Fluorescein angiogram showing ill de ned s ippled hyperf uorescence emporal o he ovea ( arrow).
( continued)
D
FIGURE 1-16. (Continued) Occult CNV. C. Fluorescein angiogram showing more di use ill de ned leakage
(arrow) . D. La e f uorescein angiogram showing poorly demarca ed, s ippled hyperf uorescence and leakage o
dye charac eris ic o occul CNV ( arrow) .
B
FIGURE 1-17. Classic and occult CNV. A. Fundus pho ograph demons ra ing macular edema as well as
shallow submacular f uid due o exuda ive AMD (arrow) . Some drusen are no ed peripheral o he neurosensory
de achmen . B. Ar erio venous phase f uorescein angiogram showing hyperf uorescence consis en wi h
combina ion o classic and occul CNV. Classic componen is superior and emporal.
( continued)
D
FIGURE 1-17. (Continued) Classic and occult CNV. C. Midphase f uorescein angiogram demons ra ing
leakage rom he sub oveal CNV. Classic componen shows brigh er hyperf uorescence han he occul CNV. D.
Pooling o f uorescein dye below he neurosensory de achmen rom classic and occul CNV (inse ) .
B
FIGURE 1-18. Retinal pigment epithelial detachment. A. Color undus pho ograph showing a re inal
pigmen epi helial de achmen wi h submacular f uid cen ered emporal o he ovea. Some drusen are no ed
emporal o he de achmen . B. Midphase f uorescein angiogram showing hyperf uorescence corresponding o a
large re inal pigmen epi helial de achmen .
( continued)
D
FIGURE 1-18. (Continued) Retinal pigment epithelial detachment. C. Recircula ion phase f uorescein
angiogram demons ra ing pooling o dye benea h he pigmen epi helial de achmen and a brovascular
componen cen ered on he ovea. D. Fluorescein angiogram showing he ex en o he serous and brovascular
pigmen epi helial de achmen . T e area o involvemen remains unchanged rom he recircula ion phase image.
( continued)
E F
FIGURE 1-18. (Continued) Retinal pigment epithelial detachment. E. Red ree undus image o re inal
pigmen epi helial de achmen . F. OC image demons ra es re inal pigmen epi helial de achmen and
associa ed macular edema.
B
FIGURE 1-19. Serous pigment epithelial detachment. A. Color undus pho ograph demons ra ing a blis er
like eleva ion o a serous pigmen epi helial de achmen (inse ). B. Fluorescein angiogram showing a serous
pigmen epi helium de achmen cen ered on he ovea wi h a no ch on he nasal border. T e no ch may represen
an area o CNV.
( continued)
D
FIGURE 1-19. (Continued) Serous pigment epithelial detachment. C. Ano her pa ien wi h pigmen epi helial
de achmen seen on f uorescein angiography; no no ch is observed. D. Indocyanine green angiogram showing
a ocal area o hyperf uorescence (arrow) wi hin he pigmen epi helial de achmen corresponding o presumed
CNV.
B
FIGURE 1-20. Retinal pigment epithelial tear. A. A re inal pigmen epi helial ear is no ed by he discre e
borders o he loss o pigmen in he emporal macula. A scrolled edge can be seen as a curved hyperpigmen ed
line ex ending hrough he ovea (inse ) . B. Early phase f uorescein angiogram showing a brigh , well delinea ed
area o hyperf uorescence corresponding o he re inal pigmen epi helial ear. T e redundan scrolled edge o he
ear shows as rela ive hypof uorescence in an arc hrough he ovea (inse ) .
( continued)
D
FIGURE 1-20. (Continued) Retinal pigment epithelial tear. C. La e phase f uorescein angiogram showing no
evidence o f uorescein dye leakage beyond he emporal border o he ear (inse ) . D. T ir y mon hs la er, he
color undus pho ograph shows some submacular brosis and a rophy in he re inal pigmen epi helial ear.
Underlying choroidal vessels are visible in he region o he ear (inse ).
FIGURE 1-21. Submacular hemorrhage. Submacular hemorrhage can cause drama ic sudden loss o cen ral
vision in pa ien s wi h exuda ive AMD. No e ha some overlying re inal vessels are visible, he clue ha he
hemorrhage is subre inal.
B
FIGURE 1-22. Recurrent CNV a er thermal laser treatment. A. Color undus pho ograph shows prior
area o hyperpigmen ed hermal laser pho ocoagula ion scar and new submacular hemorrhage and f uid
ex ending hrough he ovea. B. Fluorescein angiogram showing he hyperf uorescen , sub oveal, recurren CNV
surrounded by a rim o hypof uorescence. T e old hermal laser scar is predominan ly hypof uorescen in erior o
he CNV.
FIGURE 1-23. Anti VEGF therapy or exudative AMD. A. Color undus pho ograph demons ra ing serous
macular de achmen and re inal hemorrhage prior o rea men wi h mon hly in ravi real ranibizumab an i VEGF
herapy. Visual acui y was 20/ 100. B. Pre rea men f uorescein angiogram demons ra ing occul sub oveal CNV.
( continued)
D
FIGURE 1-23. (Continued) Anti VEGF therapy or exudative AMD. C. Pre rea men f uorescein angiogram
demons ra ing leakage wi hin he neurosensory de achmen . D. Pre rea men OC image demons ra es CNV
and hickening o he macula.
( continued)
FIGURE 1-23. (Continued) Anti VEGF therapy or exudative AMD. E. One year post rea men color undus
pho ograph reveals diminished submacular f uid and absence o re inal hemorrhage. Visual acui y was 20/ 40.
F and G. Post rea men f uorescein angiogram demons ra ing less leakage and s aining.
( continued)
H
FIGURE 1-23. (Continued) Anti VEGF therapy or exudative AMD. H. Post rea men OC image reveals
reduced macular hickening. Visual acui y was 20/ 40.
C H AP T ER
2
Macular
Macullar Diseases
D
Nikolas
N
Nikolas J.S. London
Lon
ondo
don and Mitchell S. Fineman
MACU
MACULAR
M
MAAC
CUUL
LAAR EPIRE
E IIR
EP R E INAL
I NA
N L o he membrane. A er PVD, a por ion o
MEMBRAN
M EM
MBBR
R AN
A E pos erior cor ical vi reous may be le behind
on he macula, or he in ernal limi ing mem-
M acular epire inal membrane is an acquired brane may be disrup ed, allowing glial cells o
orma ion o a semi ransparen f bro- proli era e on he sur ace o he re ina. T ese
cellular membrane in he macula. proli era ing glial cells orm he epire inal
membrane. A er re inal break orma ion and
rauma, re inal pigmen epi helial cells may
EPIDEMIOLOGY escape in o he vi reous cavi y and set le upon
AND ETIOLO GY he macula. T ese cells may hen undergo
me aplasia o glial cells, allowing he orma-
Macular epire inal membranes may be ion o macular epire inal membranes.
ei her idiopa hic or secondary o o her in ra-
ocular abnormali ies, including re inal breaks, HISTORY
re inal vascular disease, uvei is, blun and
pene ra ing rauma, and surgery. Idiopa hic I he macular epire inal membrane is hin
macular epire inal membranes are mos com- and no producing dis or ion o he re ina,
mon a er 50 years o age. hen he pa ien is usually asymp oma ic.
Males and emales are a ec ed equally. Wi h progressive hickening and con rac ion
Bila eral macular epire inal membranes o he membrane, pa ien s begin o no ice
occur in approxima ely 20% o pa ien s, decreased vision, me amorphopsia, and mac-
wi h he severi y o he membranes usually ropsia or micropsia.
asymme ric. Mos pa ien s main ain vision bet er han
Mos pa ien s have pos erior vi reous 20/ 50.
de achmen (PVD), and i is hough ha his Mos epire inal membranes progress slowly
phenomenon may con ribu e o orma ion and occasionally improve spon aneously.
44
IMPORTANT DIAGNOSTIC EVALUATION

CLINICAL SIGNS
Usually measuremen o visual acui y,
Macular epire inal membranes ini ially Amsler grid es ing ( o assess or me amor-
appear as ranslucen , glis ening membranes phopsia, macropsia, or micropsia) and oph-
over, or adjacen o, he cen ral macula halmoscopy su ce o make he diagnosis
(Fig. 2 1). T ere may be a “pseudohole” o macular epire inal membrane. Sli -lamp
appearance over he ovea. As he membrane biomicroscopy wi h ei her a noncon ac or a
ma ures, i becomes more opaque (Fig. 2 2A). con ac lens grea ly acili a es diagnosis.
T ere may be associa ed superf cial hem- Op ical coherence omography (OC ) has
orrhages or nerve f ber layer in arc s. become s andard in documen ing he presence
o an epire inal membrane. OC reveals a
T e membranes con rac cen rally and
hyperre ec ive band on he sur ace o he re ina,
cause wrinkling and dis or ion o he under-
associa ed wi h re inal dis or ion, hickening,
lying re ina. T is wrinkling and dis or ion
and/ or cys oid edema in advanced cases.
gives rise o o her names or his condi ion:
cellophane maculopa hy, sur ace wrinkling Fluorescein angiography will clearly delinea e
re inopa hy, and macular pucker. I he dis- re inal vascular dis or ion and leakage ha may
or ion is severe, cys oid macular edema and occur rom cys oid macular edema (Fig. 2 2B, C).
even shallow rac ional re inal de achmen
can occur. PROGNOSIS AND
MANAGEMENT
DIFFERENTIAL Mos pa ien s wi h uniocular macular
DIAGNOSIS epire inal membrane and vision bet er han
20/ 50 are usually no signif can ly symp om-
Cys oid macular edema a ic and do no require in erven ion. Pa ien s
Choroidal neovasculariza ion (CNV) wi h vision o 20/ 60 or worse and hose
Macular hole pa ien s wi h in olerable dis or ion may benef
rom pars plana vi rec omy wi h membrane
Choroidal olds
peeling and removal (Fig. 2 3).
Combined hamar oma o he re ina
Rarely, he membrane may spon aneously
Re inal pigmen epi helium release, leading o resolu ion o symp oms.
46 2 MACULAR DISEASES
FIGURE 2-1. Macular epiretinal membrane. Glis ening membrane over he macula causing dis or ion o
cen ral re ina.
A
FIGURE 2-2. Macular epiretinal membrane. A. T ick membrane over he macula causing dis or ion o
he cen ral re ina.
( continued)
C
FIGURE 2-2. ( Continued) Macular epiretinal membrane. B. Fluorescein angiogram showing dis or ion o he
macular re ina. C. La e-phase f uorescein angiogram showing in rare inal leakage o dye rom dis or ed re inal
vessels.
B
FIGURE 2-3. Macular epiretinal membrane. A. Preopera ive appearance o membrane (inse ) ; visual acui y
was 6/ 60. No e he re inal olds and re inal vascular compression and or uosi y. B. Pos opera ive appearance
o membrane (inse ) ; visual acui y was 6/ 12. T ere is less re inal vascular or uosi y.
he second eye. Pa ien s wi h a ull- hickness

IIDIO
DIOO PA
A H IC
C MACULAR
M ACUL
L AR
R
macular hole in one eye and a normal re ina
H O LE
LE wi h PVD in he ellow eye are a very lit le
risk o progressing o macular hole in he
I diopa hic macular hole is an acquired ull-
hickness de ec o re ina in he cen ral
macula.
second eye.
HISTORY
EPIDEMIOLOGY Pa ien s usually repor decreased visual
AND ETIOLO GY acui y wi h cen ral sco oma. T ere may be
me amorphopsia.
Idiopa hic macular holes ypically occur in
O en pa ien s no ice decreased vision in
he six h hrough eigh h decades o li e wi h a
he a ec ed eye when he ellow eye is inciden-
3:1 predominance in women.
ally covered as or a rou ine eye examina ion.
T e incidence o bila erali y is 5% o 10%.
angen ial vi reore inal rac ion is he
presumed cause o developmen o idiopa hic IMPORTANT CLINICAL SIGNS
macular hole.
T e Gass classif ca ion o he s ages o Depending on he s age and severi y o
idiopa hic macular hole developmen is he macular hole, he visual acui y may be
help ul in unders anding he progression o near normal or severely reduced o less han
he disease and he biomicroscopic f ndings 20/ 400.
(Table 2 1). Amsler grid es ing will o en reveal
Pa ien s wi h a ull- hickness macular hole me amorphopsia or a cen ral sco oma.
in one eye and an impending macular hole Oph halmoscopy and sli -lamp biomicros-
wi h no PVD in he ellow eye are a subs an ial copy reveal f ndings consis en wi h he s age
risk o progression o s age 2 macular hole in o he macular hole.
TABLE 2-1. S ages o Developmen o Idiopa hic Macular Hole

S age 1A Early con rac ion o ou er par o vi reous cor exwi h oveolar de achmen (impending macular
hole)
S age 1B Fur her vi reous con rac ion and condensa ion o he pre oveal vi reous cor exwi h oveal
de achmen (impending macular hole)
S age 2 Small (<400 µ m) peri oveal dehiscence
S age 3 Larger (>400 µ m) cen ral ull- hickness hole usually accompanied by a rim o re inal eleva ion;
he pos erior cor ical vi reous remains at ached; here may be a small operculum overlying he
macular hole
S age 4 Macular hole has an associa ed comple e pos erior vi reous de achmen ; hese holes are usually
large (>400 µ m).
Based on Gass JD. Reappraisal o biomicroscopic classifca ion o s ages o developmen o a macular hole. Am J Opthalmol.
1995;119:752–759.
S age 1 holes appear as a small yellow eyes wi h s age 2, 3, or 4 macular holes

cys or ring around he ovea wi h a loss o will reveal early cen ral hyper luorescence
he oveal depression (Fig. 2 4). in he ovea corresponding o loss o xan-
S age 2 holes appear as a small round or hophyll pigmen and re inal pigmen epi-
crescen -shaped de ec in he ovea (Fig. 2 5). helial depigmen a ion and a rophy a he
base o he hole. OC clearly dis inguishes
S age 3 holes have a dark round de ec
be ween par ial- and ull- hickness holes.
in he ovea, o en wi h a cu o subre inal
(Fig. 2 9).
uid accumula ion (Fig. 2 6).
S age 4 holes are o en larger han s age
3 holes and are associa ed wi h a PVD
(Fig. 2 7). PROGNOSIS AND
T ere are o en small yellow do s in he MANAGEMENT
cen er o he hole a he level o he re inal
No rea men is recommended or s age 1
pigmen epi helium (Fig. 2 8).
macular holes because hese resolve spon a-
neously in 50% o cases. Spon aneous resolu-
ASSO CIATED CLINICAL ion o more advanced s ages o macular hole
SIGNS can occur, bu i is rare.
Vi rec omy can be per ormed or more
T e Wa zke–Allen sign is he pa ien ’s advanced s ages o macular hole. T e surgery
descrip ion o discon inuance in he cen er o consis s o a s andard pars plana vi rec omy,
a hin sli beam shone over he ovea. peeling o he pos erior hyaloid, and injec-
ion o a long-ac ing gas such as per uoro-
DIFFERENTIAL DIAGNOSIS propane. Peeling o he in ernal membrane
may also be done. Pa ien s mus hen main-
Macular epire inal membrane wi h ain ace-down posi ioning or 1 o 2 weeks
pseudohole o allow he gas bubble o amponade he
Cys oid macular edema hole. Recen evidence sugges s ha ace-
Cen ral serous re inopa hy (CSR) down posi ioning is no as cri ical as once
believed, bu his needs o be s udied ur her.
Choroidal neovascular membrane T e success ra e or macular hole surgery
Solar re inopa hy approaches 80% o 90% wi h closure o he
Adul vi elli orm dys rophy hole and improvemen in visual acui y
(Fig. 2 10).
DIAGNOSTIC EVALUATION Nega ive prognos ic indica ors
include a long dura ion o hole presence
Clinical examina ion alone is o en (i.e. grea er han 1 year) and larger size o
diagnos ic. Fluorescein angiography in he hole.
B
FIGURE 2-4. Idiopathic macular hole, stage 1. A. S age 1 macular hole wi h yellow ring appearance around
he ovea. Visual acui y remains 6/ 7.5. B. Op ical coherence omography showing s age 1 macular hole.
B
FIGURE 2-5. Idiopathic macular hole, stage 2. A. S age 2 macular hole appears as a small round de ec in he
ovea (inse ) . B. Op ical coherence omography showing s age 2 macular hole wi h persis en rac ion on one
edge o he hole.
B
FIGURE 2-6. Idiopathic macular hole, stage 3. A. S age 3 macular hole wi h cu o subre inal f uid around
he hole. B. Op ical coherence omography showing s age 3 macular hole.
B
FIGURE 2-7. Idiopathic macular hole, stage 4. A. S age 4 macular hole; no e condensed vi reous o pos erior
vi reous de achmen overlying in ero emporal vascular arcade ( arrow) . B. Op ical coherence omography
showing s age 4 macular hole.
B
FIGURE 2-8. Idiopathic macular hole, chronic. A. Chronic s age 4 macular hole wi h subre inal precipi a es
(inse ) . B. Re inal pigmen epi helial ring around macular hole indica es chronici y.
FIGURE 2-9. Idiopathic macular hole. Op ical coherence omography (OC ) o s age 4 macular hole showing
comple e de ec in re ina.
B
FIGURE 2-10. Idiopathic macular hole. A. Preopera ive appearance o s age 3 macular hole ( yellow spo s are
inciden al drusen) . B. Pos opera ive appearance o s age 3 macular hole; no e closed appearance o hole. Vision
improved o 6/ 12 rom 6/ 30.
VI RREO
EO
OM MACULAR
AC
C U L AR DIFFERENTIAL DIAGNOSIS
RAC
RAAC IO
I O N SYN
SY
YN DRO
DR
R O ME
E
Macular epire inal membrane
V i reomacular rac ion syndrome (VM S) Combined hamar oma o he re ina and
is an acquired condi ion in which here is re inal pigmen epi helium
par ial separa ion o he pos erior hyaloid wi h
persis en at achmen o he macula and, occa-
sionally, he op ic nerve head.
DIAGNOSTIC EVALUATION
Fluorescein angiography may reveal re inal

EPIDEMIOLOGY vascular dis or ion and leakage. T ere may
AND ETIOLO GY be cys oid macular edema and op ic nerve
edema.
VM S occurs in he same age group as Spec ral-domain OC helps de ermine
hose who develop PVD. PVD is uncommon he presence o VM S as well as associa ed
be ore 50 years o age and is presen in over macular edema and rac ional de achmen .
50% o people aged 70 years and older.
PROGNOSIS AND
HISTORY MANAGEMENT
Pa ien s who have VM S experience Occasionally pa ien s may experience
progressive dis or ion and visual loss, which spon aneous improvemen i PVD occurs.
is o en more severe han ha occurring wi h Surgical in erven ion is indica ed i visual
macular epire inal membrane. acui y is reduced o 20/ 70 or worse. During
vi rec omy surgery, he pos erior hyaloid is
IMPORTANT removed, as are any epire inal membranes in
CLINICAL SIGNS he macular region. T e re inal archi ec ure
can be res ored o a normal appearance. T e
T e pos erior hyaloid is visibly hickened. vision can be improved bu usually comple e
T ere is macular dis or ion, o en wi h rac ional recovery does no occur due o residual
re inal de achmen in he macula. Re inal s riae macular edema.
may be presen . T ere may be rac ion in he Pharmacologic vi reolysis wi h microplasmin
peripapillary region. An epire inal membrane is curren ly under inves iga ion or he rea men
may be seen clinically (Fig. 2 11). o VM S, wi h promising ini ial resul s.
Vi reomacular Trac ion Syndrome 59
FIGURE 2-11. Vitreomacular traction syndrome. A. Adhesion o he vi reous o a premacular membrane

eleva es he ovea and crea es macular dis or ion. B. OC showing vi reomacular rac ion. C. Severe
vi reomacular rac ion may lead o secondary macular hole orma ion.
CY
YSS O IID
DMMACULAR
AC U LAR
R ASSO CIATED
EDEMA
ED
DEMA CLINICAL SIGNS
T ere may be no associa ed clinical signs

C ys oid macular edema (CME) is he resul
o accumula ion o in rare inal uid in
he peri oveal region. Fluid accumula es in
when CME occurs a er ca arac surgery.
However, CME is more common a er compli-
ca ed ca arac surgery in which here has been
cys ic spaces ha may be visible clinically and
rup ure o he pos erior capsule and vi reous
on uorescein angiography.
loss. Such f ndings as vi reous o he wound, iris
o he wound, iris a rophy, and an opening in
EPIDEMIOLOGY he pos erior capsule may here ore be presen .
AND ETIOLO GY When CME is presen in associa ion wi h
o her oph halmic diseases, hen he f ndings
CME is mos commonly seen a er ca arac o hose en i ies will be presen . For example,
surgery. O her ypes o ocular surgery, such as pigmen migra ion in o he re inal midpe-
rabeculec omy, laser pho ocoagula ion and riphery will be presen in pa ien s wi h CME
cryore inopexy, may also give rise o CME. in associa ion wi h re ini is pigmen osa, and
Less commonly, CME is seen in associa- di use in rare inal hemorrhages will be pres-
ion wi h diabe ic re inopa hy, CNV, uvei is, en in pa ien s wi h CME in associa ion wi h
re inal vein obs ruc ion, peri oveal elan- re inal venous occlusive disease.
giec asis, re ini is pigmen osa, and o her
en i ies. DIFFERENTIAL DIAGNOSIS
HISTORY CNV
CME ollowing ca arac surgery ypi- Diabe ic macular edema
cally has i s onse 6 o 10 weeks a er surgery.
Pa ien s experience an ini ial improvemen in DIAGNOSTIC EVALUATION
vision only o be ollowed by decreasing cen-
ral vision in he range o 6/ 40 o 6/ 100. Fluorescein angiography is help ul in es ab-
lishing he diagnosis o CME. Fluorescein
IMPORTANT angiography shows accumula ion o dye in he
CLINICAL SIGNS peri oveal region in a pe alloid pat ern
(Fig. 2 12B). T ere is o en leakage o dye
When CME is presen in he pos –ca arac rom he op ic nerve head (Fig. 2 12C),
surgery pa ien , here is o en no abnormali y o en called Irvine–Gass syndrome. T e oveal
no ed in he an erior segmen . avascular zone is no enlarged in uncompli-
On sli -lamp biomicroscopy, pa ien s will ca ed CME.
have cys ic spaces in he peri oveal area Angiographic CME may be presen in as
(Fig. 2 12A), bes seen by narrowing he sli many as 60% o pa ien s ollowing rou ine
beam adjacen o he ovea. T ere will also be ca arac surgery. Clinically signif can CME,
hickening o he cen ral macula and, occa- in which pa ien s are symp oma ic, occurs in
sionally, iny round in rare inal hemorrhages 2% o 10% o pa ien s ollowing uncompli-
a he edge o he oveal avascular zone. ca ed ca arac surgery.
PROGNOSIS AND requencies o adminis ra ion and combina ion

MANAGEMENT o agen s. A ypical s ar ing regimen is opical
prednisolone ace a e and a opical NSAID 4
Mos pa ien s who su er pos opera ive imes daily or several mon hs. For unrespon-
CME will undergo spon aneous resolu ion sive cases, periocular or in ravi real cor icos e-
wi hin 6 mon hs. T erapeu ic in erven ion roid injec ion can be considered. Al erna ively,
is indica ed i pa ien s are symp oma ic wi h in ravi real an i-VEGF or low-dose sys emic
decreased vision. ace azolamide can be considered.
T ere is no single accep ed regimen or Surgical in erven ion wi h Nd:YAG laser
managemen o pos opera ive CME. T e vi reolysis or hin s rands o vi reous rapped
mos common herapies are opical or peri- in he ca arac wound or vi rec omy or more
ocular cor icos eroids, opical nons eroidal ex ensive vi reous or iris incarcera ion or vi -
an i-in amma ory drugs (NSAIDs), and reomacular adhesion may resul in resolu ion
oral carbonic anhydrase inhibi ors in various o CME.
B
FIGURE 2-12. Cystoid macular edema. A. T e normal oveal ref ex is los and here are cys oid changes in
he cen ral macula. B. Early ar eriovenous ransi phase f uorescein angiogram showing leakage o dye in he
peri oveal area.
( continued)
D
FIGURE 2-12. ( Continued) Cystoid macular edema. C. La e-phase f uorescein angiogram showing “pe alloid”
pat ern o dye leakage in addi ion o leakage o dye rom he op ic nerve head. D. OC showing cys oid macular
edema.
he re ina and re inal pigmen epi helium. T e

PO
P O LY
LYPO
YP O IDAL
I D AL CH O RO
O IDAL
ID
D AL vascular lesions may be seen wi h sli -lamp bio-
VASCULO
V ASCC U LO
O PA
PA H Y microscopy as reddish orange spheroidal or pol-
ypoidal lesions. T e lesions have a predilec ion
P olypoidal choroidal vasculopa hy (PCV)

is an idiopa hic hemorrhagic disorder o
he macula.
or he peripapillary area bu may be seen else-
where in he macula and even in he periphery.
Rarely, bullous or o al serosanguineous
re inal de achmen wi h or wi hou vi reous
EPIDEMIOLOGY AND hemorrhage may occur.
ETIOLO GY
ASSO CIATED
PCV is a disorder o he inner choroidal CLINICAL SIGNS
vascula ure in which here is a ne work o
branching vessels deep o he choriocapil- Sys emic hyper ension is o en associa ed
laris in associa ion wi h erminal aneurysmal wi h severe PCV wi h visual loss.
dila ions. I is assumed ha PCV represen s
a orm o CNV. However, polypoidal CNV
DIFFERENTIAL DIAGNOSIS
behaves di eren ly rom o her orms o
CNV, and he visual prognosis is bet er
Age-rela ed macular degenera ion wi h CNV
compared wi h CNV.
Cen ral serous choroidopa hy
PCV was ini ially described in elderly
black women bu is now known o occur in Re inal pigmen epi helial de achmen
all races wi h preponderance in heavily pig-
men ed individuals. DIAGNOSTIC EVALUATION
Men are equally a ec ed as women.
T e average age o onse o polypoidal Serosanguineous de achmen s o he
CNV is much younger han ha o age-rela ed re ina and re inal pigmen epi helium may be
macular degenera ion, bu he range o age seen clinically (Fig. 2 13A).
a onse is wider (less han 25 o more han Fluorescein angiography is usually no an
85 years). Lesions are usually bila eral, bu e ec ive imaging echnique, because he uo-
pa ien s have been ollowed or years wi h rescence o he choriocapillaris o en masks
unila eral involvemen . he vascular lesions (Fig. 2 13B, C).
Indocyanine green angiography, which
HISTORY bet er images he choroid, o en provides he
bes visualiza ion o he ac ive lesions. OC
Pa ien s wi h PCV presen wi h decreased is use ul o ollow macular involvemen .
and dis or ed vision i serosanguineous com-
plica ions occur in he macula.
PROGNOSIS AND
IMPORTANT MANAGEMENT
CLINICAL SIGNS
T e serosanguineous lesions may resolve
Pa ien s wi h PCV may develop chronic spon aneously wi hou progressing o f brous
recurren acu e serosanguineous de achmen s o proli era ion. T e vascular lesions may
Polypoidal Choroidal Vasculopa hy 65
involu e during periods o disease inac ivi y, ou side he ovea o en leads o regression
making diagnosis di cul . In con ras , he o he en ire lesion. T is is unlike he experi-
vascular lesions may con inue o grow and ence wi h CNV due o age-rela ed macular
repea edly bleed. T ese lesions may hen degenera ion, in which he en ire lesion mus
develop f brovascular scarring. Pa ien s may be rea ed o preven ur her hemorrhagic
su er severe visual loss. rea men or sys- complica ions.
emic hyper ension, i associa ed wi h PCV, In ravi real an i-VEGF agen s have lim-
may be impor an in limi ing he severi y o i ed e ec iveness in PCV, al hough a rial
he disease. is o en u ilized wi h macular involvemen .
Laser pho ocoagula ion can be considered, An i-VEGF herapy may have more e ec
especially or serosanguineous complica ions as adjunc ive herapy o o her modali ies.
under he ovea. rea men o he ac ive pol- Ocular pho odynamic herapy may be consid-
ypoidal CNV or o he aneurysmal changes ered or sub oveal lesions.
A
FIGURE 2-13. Polypoidal choroidal vasculopathy (PCV). A. Areas o serosanguineous re inal de achmen in
he macula ( arrows) . (continued)
C
FIGURE 2-13. ( Continued) Polypoidal choroidal vasculopathy (PCV). B. Venous lling phase f uorescein
angiogram showing ne work o branching choroidal vessels (arrows) . C. La e-phase f uorescein angiogram
showing leakage o dye in he choroid and erminal aneurysmal dila ions.
T e disc i sel may be ver ically elonga ed or

DEGEN
D EG
G E N ERA
E R A IVE
IV
VE MYO
MY
YO PIA
A il ed, or bo h (Fig. 2 14B).
D egenera ive myopia describes a re inal Cen ral macular abnormali ies may lead
degenera ive condi ion ha consis s o o visual loss. Gyra e areas o a rophy in
hinning o he re inal pigmen epi helium and he pos erior pole may involve he oveal
choroid, re inal pigmen epi helial a rophy, region. Lacquer cracks, which are spon ane-
CNV, and subre inal hemorrhage in pa ien s ous linear breaks in Bruch’s membrane, may
wi h progressive elonga ion o he eye rom be loca ed in he ovea (see Fig. 2 14B).
myopia usually grea er han 6 diop ers. Lacquer cracks are presen in 4% o highly
myopic eyes. Spon aneous subre inal hemor-
rhage wi hou CNV may arise rom lacquer
EPIDEMIOLOGY cracks (Fig. 2 14C). Fuchs’ spo s are round
AND ETIOLO GY areas o subre inal hyperpigmen a ion,
occasionally wi h surrounding a rophy, ha
T e prevalence o degenera ive myopia are hough o represen areas o previous
varies among di eren races and e hnic groups. subre inal hemorrhage or CNV. Fuchs’ spo s
are seen in 10% o highly myopic eyes a er
Degenera ive myopia is more prevalen in
he age o 30.
women han in men.
HISTORY ASSO CIATED

CLINICAL SIGNS
Pa ien s wi h degenera ive myopia may
slowly lose cen ral vision due o progressive CNV develops in 5% o 10% o eyes
a rophy o he macular region. More abrup wi h an axial leng h grea er han 26.5 mm
vision loss may occur rom macular subre inal (Fig. 2 14D). CNV is o en seen in associa-
hemorrhage or CNV. ion wi h lacquer cracks. Pos erior pole s aph-
yloma, an excava ion in he pos erior pole
Spon aneous improvemen in vision may
associa ed wi h choriore inal a rophy, may be
occur i subre inal hemorrhage no associa ed
presen (Fig. 2 14E).
wi h CNV resorbs.
Di use pigmen ary al era ion and pa chy
or di use areas o choriore inal degenera ion
IMPORTANT may be presen in he re inal periphery
CLINICAL SIGNS (Fig. 2 14F, G). PVD is more common and
occurs a an earlier age in pa ien s wi h degen-
T e clinical f ndings o degenera ive era ive myopia. Al hough lat ice degenera ion
myopia are hough o be due o progressive is no more common in degenera ive myopia,
elonga ion o he globe. T e hallmark f nd- pa ien s are a an increased risk o re inal ear
ing is he so-called myopic crescen o re inal and re inal de achmen .
pigmen epi helial a rophy adjacen o he
op ic nerve (Fig. 2 14A). T is a rophic area
is usually a he emporal aspec o he disc. DIFFERENTIAL DIAGNOSIS
However, he a rophy may be loca ed any-
where around he circum erence o he disc il ed disc syndrome
and may ex end hrough he cen ral macula. Op ic disc coloboma
Presumed ocular his oplasmosis degenera ive e ec s upon he re ina. Scleral

Age-rela ed macular degenera ion rein orcemen and resec ion echniques have
been repor ed o limi he elonga ion o he
Gyra e a rophy
globe, bu s abiliza ion or improvemen in
vision has no been decisively demons ra ed.
DIAGNOSTIC EVALUATION An i-VEGF herapy is e ec ive in rea -
ing eyes wi h myopic CNV. Laser pho o-
His ory, re rac ive error, and axial leng h coagula ion is a second line herapy, and
measuremen in associa ion wi h he myriad should be considered care ully as he CNV
f ndings on oph halmoscopy all aid in he o en remains small wi hou rea men .
diagnosis o degenera ive myopia. Fur hermore, spreading o he a rophic
Fluorescein angiography and OC are pho ocoagula ion lesion may lead o ur her
help ul o assess or CNV. visual loss.
Ocular pho odynamic herapy wi h ver e-
PROGNOSIS AND porf n or sub oveal CNV may be use ul.
MANAGEMENT CNV lesions in degenera ive myopia, in
dis inc ion rom hose in age-rela ed macular
T ere are no proven herapies o pre- degenera ion, may remain s able wi hou signi -
ven he progression o myopia and i s ican visual loss when no rea men is applied.
A
FIGURE 2-14. Degenerative myopia. A. emporal myopic crescen . No e “ hinning” o re inal pigmen
epi helium (inse reveals rue borders o op ic nerve) .
( continued)
C
FIGURE 2-14. ( Continued) Degenerative myopia. B. Prominen il ed disc wi h emporal crescen and lacquer
crack above ovea ( arrow) . C. Spon aneous subre inal ( oveal) hemorrhage rom lacquer crack wi hou choroidal
neovasculariza ion (CNV).
( continued)
E
FIGURE 2-14. ( Continued) Degenerative myopia. D. Sub oveal CNV ( arrow) wi h pigmen a ion and shallow
subre inal f uid. E. A pos erior s aphyloma is presen around he op ic nerve.
( continued)
G
FIGURE 2-14. ( Continued) Degenerative myopia. F. Ex ensive choriore inal a rophy in pos erior pole and
periphery in he righ eye. G. Ex ensive choriore inal a rophy in pos erior pole and periphery in he le eye.
o vision loss due o rup ure o he CNV, sub-

AN
N GI
GIO
I O ID
D S RE
REAKS
E AKS
S re inal hemorrhage, and scarring (Fig. 2 17).
A ngioid s reaks are red or brown irregular Pa ien s wi h pseudoxan homa elas icum
lines ha radia e rom he op ic nerve may have an addi ional undus f nding. T ere
head. T ey represen breaks in hickened and may be a f ne s ippled appearance o he un-
calcif ed Bruch’s membrane. dus re erred o as peau d’orange (like skin o
an orange) mos commonly seen in he em-
poral midperiphery (Fig. 2 18).
EPIDEMIOLOGY
Pa ien s wi h his disease have abnormal
AND ETIOLO GY
dermal elas ic issue. T ey have loose skin
olds in he neck and on he exor aspec s
Angioid s reaks are idiopa hic 50% o
o join s. T ey may su er cardiovascular
he ime bu are also seen in associa ion
disease rom abnormal elas ic issue in
wi h cer ain sys emic diseases. T e sys emic
blood vessel walls. T ey may develop gas-
disease mos commonly associa ed wi h
roin es inal bleeding.
angioid s reaks is pseudoxan homa elas icum,
or Grönblad–S randberg syndrome. O her Pa ien s wi h Page ’s disease (os ei is
sys emic condi ions associa ed wi h angioid de ormans) have abnormal bone des ruc ion
s reaks are Page ’s disease o bone, sickle cell and orma ion. T ey ypically su er rom
anemia, and Ehlers–Danlos syndrome. headache, enlarged skull, enlarged digi s,
bone rac ures, and cardiovascular complica-
HISTORY ions. Approxima ely 10% o pa ien s wi h
Page ’s disease develop angioid s reaks la e in
Pa ien s are asymp oma ic unless hey he course o heir disease. T ese pa ien s may
develop CNV in associa ion wi h heir angioid also su er visual loss rom op ic nerve com-
s reaks. When CNV develops, pa ien s compression by enlarging bone.
plain o decreased and dis or ed cen ral vision. Angioid s reaks develop in 1% o 2% o
pa ien s wi h sickle cell hemoglobinopa hy.
IMPORTANT Pa ien s wi h Ehlers–Danlos syndrome have
CLINICAL SIGNS hyperelas ici y o he skin and hyper ex-
ibili y o he join s due o abnormal collagen
Angioid s reaks may appear as ligh red- organiza ion.
orange o dark red-brown. T e s reaks may
orm a concen ric ring around he op ic nerve
(Fig. 2 15). T ey may ex end hrough he
macula and in o he periphery. T ey may be
rauma ic choroidal rup ure
hin or our imes he wid h o re inal ves-
sels. T ey are usually bila eral. Over ime he
s reaks may become more a rophic. DIAGNOSTIC EVALUATION
ASSO CIATED In he early phase o uorescein angiog-
CLINICAL SIGNS raphy angioid s reaks appear as hyper uo-
rescen lines due o a rophy o he overlying
CNV can be associa ed wi h angioid re inal pigmen epi helium. As in any condi-
s reaks (Fig. 2 16) and is he leading cause ion associa ed wi h disrup ion o Bruch’s
Angioid S reaks 73
membrane, CNV may occur. ypical f ndings herapy may become use ul or pa ien s
o early hyper uorescence o CNV wi h leak- wi h sub oveal CNV in associa ion wi h
age may be seen on uorescein angiography. angioid s reaks ha is resis an o an i-VEGF
herapy.
Pa ien s wi h angioid s reaks should be
PROGNOSIS AND par icularly cau ious regarding ocular rauma
MANAGEMENT (Fig. 2 19). Sa e y glasses should be worn
because hese pa ien s are more suscep ible o
When pa ien s have angioid s reaks, choroidal rup ure and hemorrhage rom direc
hey remain a risk or CNV. T ere are no blows o he eye. Pa ien s wi h angioid s reaks
measures available o preven he develop- should have a general medical evalua ion o
men o CNV. I pa ien s develop ex ra oveal assess or sys emic associa ions, especially
or jux a oveal CNV, in ravi real an i-VEGF because some o he mani es a ions, such as
and/ or s andard laser pho ocoagula ion cardiovascular disease and gas roin es inal
can be considered. Ocular pho odynamic bleeding, are po en ially li e hrea ening.
FIGURE 2-15. Angioid streaks, orange streaks. Orange lines around he op ic nerve wi h ex ensions
hroughou he pos erior pole.
B
FIGURE 2-16. Angioid streaks. A. Subre inal hemorrhage and re inal eleva ion adjacen o angioid
s reak is highly sugges ive o choroidal neovasculariza ion. B. Fluorescein angiogram con rms choroidal
neovasculariza ion.
Angioid S reaks 75
FIGURE 2-17. Angioid streaks, CNV. Severe macular scarring a er rup ure o CNV and hemorrhage (no e
peau d’orange appearance emporally) .
A
FIGURE 2-18. Angioid streaks. A. Pigmen ed s reaks wi h subre inal hemorrhage.
( continued)
B
FIGURE 2-18. ( Continued) Angioid streaks. B. Peau d’orange appearance in he emporal periphery.
A
FIGURE 2-19. Angioid streaks, traumatic subretinal hemorrhage. Pa ien wi h bila eral angioid s reaks
( A, righ eye; B, le eye) was punched in he le eye and su ered ex ensive subre inal hemorrhage. T e
subre inal hemorrhage even ually resolved, bu le severe scarring (C) and visual loss.
(continued)
Angioid S reaks 77
C
FIGURE 2-19. ( Continued)
serous de achmen (Fig. 2 20). Pa ien s

CE
CEN
E N RAL
R AL SERO
SE
E R O US
S wi h chronic or recurren episodes may
RE
E IN
I N O PA
PA H Y have pa ches o re inal pigmen epi helial
a rophy.
C SR is a disease in which a circumscribed

serous de achmen o he neurosensory
re ina develops, usually conf ned o he pos- ASSO CIATED
erior pole. T ere may be an associa ed serous CLINICAL SIGNS
de achmen o he re inal pigmen epi helium.
CSR is usually an idiopa hic condi ion bu may T ere may be yellow spo s; subre inal pre-
be seen in he set ing o cor icos eroid use. cipi a es o f brin deep o he de ached re ina
(Fig. 2 21).
EPIDEMIOLOGY O en pa ien s will have pigmen epi helial
clumping rom prior episodes in ei her he
AND ETIOLO GY involved or he ellow eye, or bo h (Fig. 2 22).
CSR usually occurs in heal hy young o Occasionally pa ien s will have an associ-
middle-aged men, al hough women may also be a ed serous de achmen o he re inal pigmen
a ec ed. T e exac e iology o CSR is unknown. epi helium (Fig. 2 23).
A di use abnormali y o he re inal pigmen Rarely pa ien s will have di use de ach-
epi helium and choroid is likely because uid men o he pos erior pole wi h gravi y-depen-
resorp ion is impaired. Recen evidence indi- den pooling o uid in eriorly (Fig. 2 24).
ca es a possible link o ac ive gas roin es inal T is may lead o “gut ers” o re inal pigmen
Helicobacter pylori in ec ion, al hough his asso- epi helial al era ions crea ed by subre inal
cia ion needs o be subs an ia ed. uid ha gravi a es in eriorly.
CSR is repor edly more common in
pa ien s wi h a so-called ype A personali y. DIFFERENTIAL DIAGNOSIS
Pa ien s being rea ed wi h cor icos eroids
can have par icularly severe CSR. CNV, especially in older pa ien s
Op ic nerve pi wi h neurosensory macular
HISTORY re inal de achmen
Pos erior scleri is
Pa ien s may be asymp oma ic unless
he cen ral macula is involved. Symp oma ic Harada’s disease
pa ien s experience sudden onse o Rhegma ogenous re inal de achmen
decreased cen ral vision wi h me amorphop- Circumscribed choroidal hemangioma
sia. T ere may be macropsia or micropsia. Amelano ic choroidal melanoma
Color vision is o en a ec ed, and pa ien s
may no ice a rela ive sco oma.
IMPORTANT CLINICAL
SIGNS A varie y o uorescein angiographic
al era ions may be seen in CSR. An expand-
On undus examina ion pa ien s will ing do o hyper uorescence is he mos com-
have an eleva ion in he macula due o mon al era ion (Fig. 2 25). As he angiogram
progresses, here is a spo o increasing hyper- reduced con ras sensi ivi y, decreased color
uorescence a he level o he re inal pigmen vision, and me amorphopsia. Rarely, pa ien s
epi helium. In he la e phase o he s udy, have severe visual loss. Recurrences happen in
here is pooling o dye in he neurosensory 20% o 40% o pa ien s.
de achmen . I pa ien s have persis en decreased
Ano her less common pat ern o hyper uo- vision wi h persis en uid beyond 3 o
rescence is a “smokes ack” appearance in which 4 mon hs, pho ocoagula ion can be o ered o
dye spreads ver ically rom he re inal pigmen he leak spo seen on uorescein angiography
epi helium (Fig. 2 26A D). Occasionally, mul- (Fig. 2 26E).
iple leakage spo s will be seen. Al erna ively, here has been recen success
wi h he use o in ravi real an i-VEGF agen s
PROGNOSIS AND or he rea men o persis en CSR. For unre-
MANAGEMENT sponsive sub oveal lesions, pho odynamic
herapy can be considered. Pa ien s wi h occu-
Implica ed cor icos eroids should be pa ional needs or improved vision or re urn
immedia ely discon inued. Mos pa ien s o s ereoacui y can be considered or earlier
undergo spon aneous resolu ion in 1 o 3 rea men . Care ul ollow-up a er laser pho o-
mon hs. However, here may be mild residual coagula ion is necessary because pa ien s may
symp oms, including decreased cen ral acui y, develop CNV a he rea men si e.
FIGURE 2-20. Central serous retinopathy, serous macular detachment. Serous re inal de achmen in he
macula.
FIGURE 2-21. Central serous retinopathy, f brin ormation. Subre inal brin precipi a ion in serous
de achmen o he macula ( arrow) .
FIGURE 2-22. Central serous retinopathy, retinal pigment epithelial alterations. Re inal pigmen epi helial
clumping in he macula ollowing resolu ion o serous de achmen (inse ) .
B
FIGURE 2-23. Central serous retinopathy. A. Earlier phase o disease in same pa ien as in Fig. 2-22. No e
serous de achmen o he re inal pigmen epi helial supero emporal o he op ic nerve and brin accumula ion
in he serous de achmen o he macula. B. Fluorescein angiogram con rms serous de achmen o re inal
pigmen epi helium adjacen o op ic nerve.
B
FIGURE 2-24. Central serous retinopathy. A and B. Pa ien wi h recurren CSR (no e small serous de achmen
o macula) who had prior episode(s) o serous de achmen ha led o gravi y-dependen pooling o f uid in eriorly
as evidenced by re inal pigmen epi helial al era ions ex ending in o he in erior periphery.
( continued)
D
FIGURE 2-24. ( Continued) Central serous retinopathy. C and D. Fluorescein angiogram showing
hyperf uorescence ex ending rom he macula o he in erior periphery due o re inal pigmen epi helial
al era ions rom f uid pooling.
B
FIGURE 2-25. Central serous retinopathy. A. Large serous de achmen o he macula wi h brin under re ina.
B, C and D. Progressive enlargemen o spo o hyperf uorescence on f uorescein angiography ( arrow) .
( continued)
D
FIGURE 2-25. ( Continued)
B
FIGURE 2-26. Central serous retinopathy, “smokestack” leakage. A. Small serous de achmen o he macula
wi h re inal pigmen epi helial al era ions. B and C. “Smokes ack” appearance o dye leakage on f uorescein
angiography.
(continued)
D
FIGURE 2-26. ( Continued) Central serous retinopathy, “smokestack” leakage. D. Fluorescein angiogram
showing mul iple leakage spo s o bo h he expanding do and “smokes ack” ype o leakage.
( continued)
E
FIGURE 2-26. ( Continued) Central serous retinopathy, post–laser treatment. E. en weeks a er laser
pho ocoagula ion. T ere is comple e resolu ion o subre inal f uid bu residual re inal pigmen epi helial
al era ions. Vision improved rom 6/ 30 o 6/ 9.
Choroidal Folds 89
CH
H O RO
R O IDAL
ID
D AL FO
OLLDS
DS
S IMPORTANT
CLINICAL SIGNS
F olding or wrinkling o he inner choroid,

Bruch’s membrane, re inal pigmen epi-
helium, and inner re ina is known as choroi-
Al erna ing ligh and dark s reaks are
seen in he pos erior pole in pa ien s wi h
dal or choriore inal olds. choroidal olds (Fig. 2 27A). T e olds may
be horizon al, ver ical, or oblique in orien a-
ion (Fig. 2 27B).
EPIDEMIOLOGY
AND ETIOLO GY
ASSO CIATED
Choroidal olds are usually idiopa hic, bu CLINICAL SIGNS
hey can be seen in associa ion wi h o her
ocular abnormali ies, including: T ere may be no associa ed f ndings i he
olds are idiopa hic or associa ed wi h hyper-
Hyperopia
opia. I here is ano her ocular condi ion, hen
Orbi al umors here may be addi ional signs rela ed o he
Pos erior scleri is cause o he olds, such as prop osis in asso-
Scleral buckling surgery cia ion wi h an orbi al umor.
Choroidal umors
Hypo ony DIFFERENTIAL DIAGNOSIS
CNV
Re inal olds (e.g., rom macular epire inal
Choriore inal scarring membrane or re inal de achmen )
T ey may be unila eral or bila eral.
HISTORY
On uorescein angiography, al erna ing
Pa ien s wi h long-s anding choroidal olds hyper uorescen and hypo uorescen bands
are usually en irely asymp oma ic. T ose wi h are seen. T e cres s o he olds are hyper-
acu e onse o olds are usually symp oma ic, uorescen , and he roughs hypo uorescen
wi h decreased vision and me amorphopsia. (Fig. 2 27C).
B
FIGURE 2-27. Choroidal olds. A. Al erna ing ligh and dark s reaks hrough he macula. B. Obliquely
orien ed al erna ing ligh and dark s reaks above he macula.
( continued)
Choroidal Folds 91
C
FIGURE 2-27. ( Continued) Choroidal olds. C. Fluorescein angiogram showing al erna ing hyperf uorescen
and hypof uorescen bands.
segmen signs consis en wi h surgery or

H YP
YPO
PO ONY rauma.
MACULO
M AC
C U L O PA
PA H Y
Hypo ony maculopa hy is a condi ion DIFFERENTIAL DIAGNOSIS

in which choriore inal olds develop in he
pos erior pole o pa ien s wi h chronically low Choriore inal olds rom o her causes:
in raocular pressure. Idiopa hic
Hyperopia
EPIDEMIOLOGY Orbi al umors
AND ETIOLO GY Pos erior scleri is
Scleral buckling surgery
Pa ien s wi h chronically low in raocular Choroidal umors
pressure rom a wound leak, cyclodialysis
Hypo ony
cle , or excessive f l ering a er glaucoma sur-
gery may develop secondary re inal changes. CNV
Choriore inal scarring
HISTORY
Pa ien s will experience loss o cen ral
vision rom he choriore inal olds. Fluorescein angiography will demons ra e
al erna ing hypo uorescen and hyper uores-
cen lines corresponding o he olds. Op ic
IMPORTANT disc hyper uorescence and macular leakage
CLINICAL SIGNS may be observed.
OC is help ul in diagnosing pa ien s
Broad choriore inal olds radia e ou em-
wi h reduced vision and an o herwise normal
porally rom he op ic nerve in a branching
exam. OC may reveal he re inal olds or
ashion (Fig. 2 28). Nasally he olds are usu-
macular edema.
ally arranged concen ric o he op ic nerve or
have an irregular arrangemen .
T e macular re ina may be hrown in o
radia ing olds around he ovea dis inc rom
PROGNOSIS AND
he choroidal olds. MANAGEMENT
T e peripapillary choroid is o en swollen,
Surgical correc ion o he underlying cause
mimicking op ic disc edema.
o hypo ony maculopa hy will usually resul
in resolu ion o he olds and visual improve-
ASSO CIATED men . Long-s anding olds may resolve wi h
CLINICAL SIGNS rea men , bu here may be residual linear
re inal pigmen epi helial al era ions rom
T e in raocular pressure will be low, usu- he chronic olding o he re inal pigmen
ally less han 5 mm Hg. T ere may be an erior epi helium.
Hypo ony Maculopa hy 93
FIGURE 2-28. Hypotony maculopathy. Horizon al and oblique macular olds in a pa ien wi h hypo ony
maculopa hy a er glaucoma surgery.
C H AP ER
3
Diabe ic Re inopa hy
James F. Vander
DIABE
D
DIIA
ABBE IC
IC R
REE IN
N O PA
PA H Y pa ien s show some re inopa hy. Proli era ive
re inopa hy is very uncommon wi h less han
D iabe ic re inopa hy encompasses a broad

range o undus mani es a ions o dia-
be es melli us. T is is a clinical erm ha
10 years’ dura ion o disease. For y percen o
pa ien s have proli era ive disease by 25 years.
T e rend or ype 2 diabe ic pa ien s is
includes exuda ive, hemorrhagic, ischemic, very similar. Many pa ien s will have asymp-
proli era ive, and rac ional mani es a ions o oma ic, occul diabe es or many years prior
his re inal vascular disease. I can be arbi rarily o diagnosis, however, and here ore may
divided in o a nonproli era ive and a proli - presen wi h re inopa hy even a he ime o
era ive orm. diagnosis o diabe es melli us.
Age is ano her impor an risk ac or in he
EPIDEMIOLOGY prevalence o diabe ic re inopa hy. Diabe ic
re inopa hy is very rare prior o puber y.
AND ETIOLO GY I s prevalence increases drama ically a er
puber y, however, and over 50% o pa ien s
Diabe ic re inopa hy is he leading cause
will develop re inopa hy by heir early
o blindness in he Uni ed S a es and Wes ern
wen ies.
Europe among adul s less han age 55 years.
I af ec s bo h genders and all races, al hough
A rican Americans are more requen ly and PATHOPHYSIOLOGY
more severely af ec ed han Caucasians.
Hyperglycemia is a key ac or in he
T e bes predic or o diabe ic re inopa hy
developmen o diabe ic re inopa hy. T e
is he dura ion o disease. For ype 1 diabe ic
mechanism o re inopa hy developmen may
pa ien s here is no risk o re inopa hy or
be rela ed o:
roughly 5 years a er ini ial diagnosis. Some
re inopa hy is presen in up o 50% o pa ien s Rela ive hypercoagulabili y
10 years a er diagnosis. A er 15 years, 95% o Red blood cell abnormali ies
94
Excessive glycosyla ion o pro eins Macular edema— he mos common

Enzyma ic conversion o excessive reason or legal blindness resul ing rom
glucose by aldose reduc ase diabe ic re inopa hy. I is bes apprecia ed
as macular hickening by he use o a high
His opa hologically, hickening o re inal
magni ca ion sli -lamp examina ion using
capillary basemen membranes and loss o
a hand held or con ac lens providing a
pericy es have been shown consis en ly.
good s ereoscopic view (Fig. 3-4).
Moderate Nonproliferative Diabetic
HISTORY Retinopathy
T is degree o re inopa hy is charac erized
Pa ien s are o en asymp oma ic bu may by increased number and size o in rare inal
have blurry vision or oa ers. More ex ensive hemorrhaging wi h grea er evidence or exu-
visual loss occurs wi h large vi reous hemor- da ion as mani es ed by more HYE (Fig. 3-5)
rhages or re inal de achmen . and macular edema han is presen in mild
nonproli era ive re inopa hy. (S andardized
NONP PRO
RO
OLLIFERA
IFE
E R A IV
IVE
VE pho ographs exis o es ablish he ransi ion
poin s be ween he various s ages o non-
DIABE
D IAB
BE IIC
C REE INNOPPA
A HY
proli era ive re inopa hy, bu i should be
remembered ha his classi ca ion re ec s a
IMPORTANT con inuum o disease severi y.) In modera e
CLINICAL SIGNS nonproli era ive re inopa hy one also begins
o see evidence o capillary occlusive disease.
Nonproliferative Diabetic retinopathy T is is re ec ed by he developmen o :
(NPDR) is he pre erred erm or his less
Cot on-wool spo s (Fig. 3-6)
severe mani es a ion o diabe ic re inopa hy.
I may be arbi rarily subdivided in o mild, Venous dila ion and beading (Fig. 3-7)
modera e, and severe ca egories. In rare inal microvascular abnormali y
Mild Nonproliferative Diabetic Retinopathy (IRMA)— a , in rare inal, irregular blood
vessel. (I is some imes di cul o dis in-
Earlies undus mani es a ions o diabe ic
guish be ween IRMA and neovasculariza-
re inopa hy. Fea ures re ec re inal capillary
ion o he re ina. Fluorescein angiography
hyperpermeabili y. May be mani es ed as:
can be help ul in making his dis inc ion;
In rare inal hemorrhage—do hemor- Fig. 3-8B.)
rhages are small mid-level re inal hemor-
Vision loss in modera e NPDR may be he
rhages (Fig. 3-1).
resul o macular edema or, less requen ly,
Blo hemorrhages—larger, wi h uzzier loss o some o he normal peri oveolar capil-
borders. lary bed. Frequen ly, bo h problems may be
Flame-shaped hemorrhages—super - presen .
cial in he nerve ber layer. Severe Nonproliferative Retinopathy
Microaneurysms—saccular enlarge- In severe NPDR here is worsening o he
men o re inal capillaries. exuda ive aspec o diabe ic re inopa hy and,
Lipopro ein exuda ion—also known especially, evidence or capillary occlusive
as hard yellow exuda e (HYE; Figs. 3-2 changes. More ex ensive in rare inal hem-
and 3-3). orrhaging, venous beading, IRMA, as well
96 3 DIABE IC RE INO PA H Y
TABLE 3-1. 4-2-1 Rule Ocular ischemic syndrome

T e presence o : Radia ion re inopa hy (Fig. 3-12)
Severe re inal hemorrhages in 4 quadran s Idiopa hic jux a oveal elangiec asis
or Coa s’ disease
Venous beading in 2 quadran s Vasculi is (e.g., sarcoidosis, lupus)
or
IRMAin 1 quadran
Indica es a 50%risk o developing proli era ive
T e mos impor an aspec o he evalu-
re inopa hy wi hin 1 year
a ion o NPDR is a magni ed, s ereoscopic,
sli -lamp biomicroscopic examina ion o
he pos erior pole and midperipheral re ina
as edema and exuda e are he ea ures ha using a handheld indirec lens or con ac
de ne severe nonproli era ive re inopa hy lens. A cri ical de ermina ion is he presence
(Figs. 3-8 to 3-10). T e presence o cer ain or absence o clinically signi can macular
undus ea ures predic s he progression edema (CSME; Table 3-2).
oward proli era ive re inopa hy (Table 3-1). Fluorescein angiography is a valu-
able ancillary es in evalua ion o NPDR.
ASSO CIATED Indica ions include:
CLINICAL SIGNS De ermina ion o loca ion o ocal
and dif use leakage o guide rea men
Cornea—decrease o corneal sensi ivi y; (Fig. 3-13)
increased risk abrasion Rule ou loss o peri oveal capillaries
Ca arac — ypically nuclear and cor ical Mechanism or unexplained vision loss
ca arac orma ion is chronic and progressive; Risk ac or or vision loss a er ocal
acu e cor ical ca arac orma ion wi h pro- laser
ound eleva ions in blood glucose
Rule ou vasculi is or o her diagnos ic
Glaucoma—grea er incidence o primary possibili ies
open-angle glaucoma
Cranial nerve palsy—isola ed palsy, mos TABLE 3-2. Clinically Signif can
o en six h Macular Edema (CSME)
Re inal hickening wi hin 500 µ m cen er o ovea
or
O her causes o re inal capillary leakage Exuda e wi hin 500 µ m cen er o ovea wi h adjacen
and occlusion include: hickening
Hyper ensive re inopa hy or

T ickening o a leas one disc area any par wi hin
Re inal vein occlusion (branch or
one disc diame er o cen er o ovea
cen ral) (Fig. 3-11)
Note: CSME is a diagnosis based on s ereoscopic macular
Hemoglobinopa hies viewing independen o visual acui y or fuorescein
Anemia or leukemia angiography.
Op ical coherence omography (OC ) TABLE 3-4. Early rea men Diabe ic
is ano her ancillary es ha is impor an in Re inopa hy S udy (E DRS) Fac s
managing diabe ic macular edema. OC pro-
E DRS rea men o macular edema:
vides a noncon ras , pho ographic me hod or
de ermining he presence o uid wi hin and Generally s abilizes visual acui y bu o en does
no improve i
under he re ina, quan i ying he ex en o
ha uid and moni or he response o herapy Consis s o direc ly rea ing ocal areas o leakage
(Fig. 3-14). and placing a grid in areas o di use capillary
leakage; de ermina ion o rea men placemen
is generally guided by he use o a fuorescein
PROGNOSIS AND angiogram
MANAGEMENT Should be avoided in he presence o signi can
loss o peri oveal capillaries
NPDR ends o progress gradually over May ake mon hs o show resolu ion o hickening
mon hs o years. T e risk o vision loss and longer or exuda es
increases wi h increasing severi y o re inopa-
hy. rea men o sys emic disease reduces
bu does no elimina e he risk o progression 2. Wha is he role o ini ia ing early laser
and vision loss (Table 3-3). Newer medica- (as compared o DRS high-risk cri eria;
ions under developmen may ac ually reverse Table 3-5) in he managemen o severe
re inopa hy. nonproli era ive and early proli era ive
Ocular rea men consis s o macular re inopa hy? Answer: Inconclusive. No
laser pho ocoagula ion or macular edema s rong bene o early scat er panre inal
(Fig. 3-15). Early rea men diabe ic re inop- pho ocoagula ion (PRP) was ound.
a hy s udy (E DRS) guidelines are widely Cer ain clinical circums ances (e.g.,
applied (Table 3-4). T e u ili y and iming o poor compliance wi h ollow-up exami-
re rea men , he role o early rea men be ore na ions, rapid progression in ellow eye)
E DRS hreshold is reached, and he applica- may jus i y early ini ia ion o PRP.
ion o al erna ive rea men s ra egies are less 3. Wha is he role o laser (PRP or ocal
uni ormly accep ed. macular laser, or bo h) in he manage-
T e E DRS addressed hree ques ions: men o macular edema? Answer: T ere
is no role or PRP in rea men o macu-
1. Wha is he role o aspirin in dia- lar edema. Macular laser is o bene ,
be ic re inopa hy? Answer: I nei her reducing he risk o modera e visual loss
improves nor worsens re inopa hy. by 50%. Pa ien s wi h CSME should be
rea ed.
TABLE 3-3. Diabe es Con rol and
Complica ions rial (DCC ) TABLE 3-5. Diabe ic Re inopa hy
Clinical Research (DRCR)
DCC showed ha igh ened blood glucose con rol
reduces: Macular laser pho ocoagula ion is superior o
Developmen o re inopa hy by 76% repea ed in ravi real injec ion o riamcinolone
ace onide or primary rea men o macular edema
Progression o re inopa hy by 80%
in general.
Risk o nephropa hy by abou 60% Ranibizumab injec ions are as good (? bet er) as
Risk o neuropa hy by abou 60% laser or rea men o CSME a 1 year
More recen ly, he use o in ravi real injec- resul s sugges ha s eroid injec ion is no as
ion o pharmacologic agen s has provided ef ec ive as macular laser or primary rea -
an al erna ive me hod or rea ing macular men o edema.
edema. T e diabe ic re inopa hy clinical More recen ly, injec ion o an i-VEGF
research (DRCR) s udies have assessed he agen s bevacizumab and ranibizumab have
u ili y o some o hese agen s (Table 3-5). shown a s rong posi ive herapeu ic ef ec
In ravi real riamcinolone ace onide causes wi h repea ed injec ions (Fig. 3-16). T e
a rapid reduc ion o macular edema and DRCR preliminary resul s sugges ha ranibi-
subre inal uid in mos cases. T e ef ec is zumab may be superior o ocal laser as pri-
generally emporary and side ef ec s include mary rea men , a leas or 12 mon hs. T ese
ca arac progression and eleva ion o in ra- indica ions are evolving.
ocular pressure, some imes markedly. DRCR
B
FIGURE 3-1. Minimal nonproli era ive diabe ic re inopa hy (NPDR) .
FIGURE 3-2. NPDR wi h re inal hemorrhages and hard yellow exuda es (HYEs) .
FIGURE 3-3. Sub le HYE near ovea in NPDR.

B
FIGURE 3-4. A. Re inal hemorrhages, HYE, and edema in NPDR. B. Venous phase in ravenous uorescein
angiogram (IVFA) showing numerous microaneurysms seen as pinpoin do s o hyper uorescence.
(continued)
D
FIGURE 3-4. ( Continued) C. Leakage rom microaneurysms wi h obscura ion o hyper uorescen do s.
D. La er phase showing more ex ensive leakage.
FIGURE 3-5. Macular edema and HYE wi h blun ing o oveal re ex.
FIGURE 3-6. Cot on-wool spo s as well as hemorrhage and HYE.

FIGURE 3-7. Venous beading ( arrow) indica ing more severe NPDR.
A
FIGURE 3-8. A. Severe NPDR.
(continued)
C
FIGURE 3-8. ( Continued) B. IVFA shows numerous microaneurysms and pa ches o capillary nonper usion
(arrowhead). No e abnormal vessels (in rare inal microvascular abnormali y, IRMA) along supero emporal
arcade ( arrowhead). C. High-powered view o IRMA seen in B. Absence o leakage dis inguishes IRMA rom
neovasculariza ion.
FIGURE 3-9. Sausaging o re inal venules ( arrow) seen in severe NPDR.
FIGURE 3-10. Venous loop.

FIGURE 3-11. Hemorrhages and cot on-wool spo s in branch re inal vein obs ruc ion. No e he segmen al
dis ribu ion o he undus abnormali ies.
FIGURE 3-12. Numerous cot on-wool spo s wi h a ew hemorrhages in a nondiabe ic pa ien wi h a his ory o
prior radia ion or rea men o a brain umor.
B
FIGURE 3-13. A. Re inal hemorrhages wi h mild macular edema. B. Enlargemen o he oveal avascular zone
wi h microaneurysms near he cen er o he macula (inse ).
( continued)
C
FIGURE 3-13. ( Continued) C. La e leakage rom he microaneurysms.
FIGURE 3-14. OC shows cys ic edema in cen ral macula.

B
FIGURE 3-15. A. Macular edema and HYE in NPDR. B. Several mon hs a er laser rea men , resolu ion o
edema and HYE is seen.
FIGURE 3-16. A. OC shows cys ic macular edema. B. One week a er in ravi real bevacizumab here is
marked reduc ion in macular hickening.
(Fig. 3-22). Involvemen o he an erior

PR
PRO
R O LIF
LIFERA
F E RA
A IVE
IV
VE DI
DIABE
I AB
BE IC
C chamber angle can produce neovascular glau-
RE
E IN O PA A HY coma (NVG), leading o a blind, pain ul eye.
Vitreous hemorrhage: Bleeding rom
P roli era ive diabe ic re inopa hy (PDR)
represen s he mos severe mani es a ion
o diabe es in he eye. I is he resul o he
NVD or NVE may occur and produce prere i-
nal or vi reous hemorrhage. Vi reous hemor-
rhage is more likely when NVD and NVE
loss o normal re inal per usion and he sub-
are more ex ensive. Hemorrhages are usually
sequen developmen o neovascular proli era-
spon aneous and produce a sudden devel-
ive issue in he undus. T e developmen o
opmen o oa ers. Prere inal hemorrhage
his neovascular issue re ec s an al era ion
re ec s seques ra ion o blood be ween he
in he balance be ween angiogenesis inhibi-
inner re inal sur ace and an in ac pos erior
ors and s imula ors in he re ina and vi reous.
hyaloid ace (Fig. 3-23). T ere ore, his will
Mul iple local chemical media ors (cy okines)
generally occur in younger pa ien s. T is may
are believed o be a work.
produce a dense, well-circumscribed sco oma
IMPORTANT (Figs. 3-24 and 3-25). O en prere inal hem-
orrhage will subsequen ly break apar and
CLINICAL SIGNS produce more dif use oa ers charac eris ic o
vi reous hemorrhage (Figs. 3-26 and 3-27).
Neovascularization of the disc (NVD):
Vi reous hemorrhage is o en recurren and
Neovasculariza ion ha develops on he
can produce pro ound visual loss.
sur ace o he op ic nerve or wi hin one disc
diame er o he op ic nerve is de ned as NVD
(Figs. 3-17 to 3-19; see also Fig. 3-21). MANAGEMENT
Shun vessels ha may develop on he op ic
disc (e.g., a er a re inal venous obs ruc- T e rea men o proli era ive re inopa hy
ion) may be easily con used wi h NVD. is guided by he diabe ic re inopa hy s udy
Neovasculariza ion o he disc ypically has a (DRS; Tables 3-6 and 3-7). Laser PRP as
lacy irregular appearance and may be eleva ed
above he op ic nerve sur ace. rue NVD
should be dis inguished rom he hyperemic TABLE 3-6. Diabe ic Re inopa hy
disc swelling o diabe ic papillopa hy. S udy (DRS)
Neovascularization elsewhere (NVE): DRS showed:
T is erm re ers o re inal neovasculariza-
Risk ea ures or severe visual loss (de ned as
ion anywhere in he undus ha is no NVD
visual acui y o 5/ 200 or worse) are:
(Figs. 3-20 and 3-21). Neovasculariza ion
elsewhere in PDR ends o occur in he NVD > ⅓ o ¼ o disc area
pos erior pole or midperiphery, al hough Any NVD wi h associa ed VH
ex reme peripheral NVE can also develop. NVE wi h associa ed VH
Neovasculariza ion elsewhere ends o T ese ea ures are known as high-risk charac-
orm a he junc ion be ween per used and teristics (HRC). Pa ien s wi h HRC rea ed wi h
nonper used re ina, and his can be readily PRP have a 50%reduc ion in risk o severe visual loss.
apprecia ed wi h uorescein angiography.
NVD, neovasculariza ion o he disc; NVE, neovasculariza ion
Neovascularization of the iris (NVI): elsewhere; PRP, panre inal pho ocoagula ion; VH, vi reous
Developmen o NVI is an ominous sign hemorrhage.
TABLE 3-7. Panre inal Pho ocoagula ion be ween he vi reous and re ina. Wi h
(PRP) Fac s con rac ion o he vi reous as well as he
brovascular proli era ive issue, increasing
PRP:
rac ion on he re ina will develop. Su cien
Does no improve visual acui y rac ion may ul ima ely lead o a re inal
May cause worsening macular edema, and loss o de achmen . A rac ion re inal de achmen
peripheral vision and nigh vision ypically has a concave, immobile appearance
Indica ions or supplemen a ion are uncer ain wi h re inal s riae radia ing rom he areas
Does no always cause regression o NVD/ NVE o grea es rac ion. When rac ion re inal
Is also indica ed in pa ien s wi h NVI rom PDR de achmen af ec s he macula, severe visual
even in he absence o NVD/ NVE loss is no ed.
NVD, neovasculariza ion o he disc; NVE, neovasculariza ion Combined rac ion and rhegma ogenous
elsewhere; NVI, neovasculariza ion o he iris; PDR, re inal de achmen may develop i vi reous
proli era ive diabe ic re inopa hy. rac ion is severe enough o produce a ull-
hickness re inal break. Combined re inal
es ablished by he DRS is he rea men o de achmen s end o develop more rapidly
choice (Figs. 3-28 to 3-30). han purely rac ional re inal de achmen s.
For eyes wi h more advanced nonclearing T e re ina appears more mobile wi h cor-
vi reous hemorrhage or brovascular scar- ruga ions and undula ions no ed wi h eye
ring, or bo h, vi rec omy may be indica ed. movemen .
In ravi real injec ion o an i-VEGF agen s will Indications for Vitrectomy in Proliferative
induce rapid regression o neovasculariza ion. Diabetic Retinopathy
T is approach may be u ilized as primary De ni e
rea men , in conjunc ion wi h laser pho o-
Persis en or recurren vi reous hemor-
coagula ion or as a pre-opera ive adjunc o
rhage (see Figs. 3-25 and 3-26)
an icipa ed vi rec omy surgery.
rac ion macular de achmen (Figs. 3-32
Macular Ischemia
and 3-33)
T ere is no ef ec ive rea men or diabe ic
Combined rac ion and rhegma og-
macular ischemia. T is condi ion more com-
enous re inal de achmen (Fig. 3-34)
monly occurs in eyes wi h PDR bu may be
observed in associa ion wi h nonproli era ive Possible
disease as well. Irregular enlargemen o he Severe proli era ion unresponsive o
oveal avascular zone on uorescein angiogra- PRP (Fig. 3-35)
phy is observed (Fig. 3-31). rac ion de achmen hrea ening he
Retinal Detachment macula
T e developmen o neovascular is- Persis en macular edema wi h au pos-
sue produces an unusually s rong adhesion erior hyaloid ace
FIGURE 3-17. Neovasculariza ion o he disc (NVD) . Modera ely severe NVD as def ned in he Diabe ic
Re inopa hy S udy. (S andard Pho o 10A, cour esy o he Diabe ic Re inopa hy S udy Group.)
FIGURE 3-18. Severe eleva ed NVD.

B
FIGURE 3-19. A. Proli era ive diabe ic re inopa hy (PDR) wi h macular edema, HYE, and NVD. B. IVFA
conf rming NVD and enlarged irregular oveal avascular zone.
( continued)
FIGURE 3-19. ( Continued) C. La e phase showing marked leakage rom NVD and severe macular edema.
A
FIGURE 3-20. A. Pa ches o neovasculariza ion elsewhere (NVE) . No e he bland appearance o he undus
peripheral o he NVE.
( continued)
C
FIGURE 3-20. ( Continued) B. Macular view on IVFA demons ra es microaneurysms bu minimal ischemia.
C. Hyper uorescence o he NVE.
( continued)
D
FIGURE 3-20. ( Continued) D. No e he marked capillary nonper usion peripheral o he NVE.
A
FIGURE 3-21. A. PDR wi h NVD and NVE ( arrows) .
(continued)
C
FIGURE 3-21. ( Continued) B. IVFA showing hyper uorescence o NVD and NVE. No e he irregular capillary
bed in he cen ral macula (inse ) . C. Marked hyper uorescence o NVE wi h peripheral nonper usion.
(continued)
D
FIGURE 3-21. ( Continued) D. Marked la e hyper uorescence rom leaking NVD and NVE wi h macular
edema.
FIGURE 3-22. Neovasculariza ion o he iris in PDR seen hrough a gonioscopic mirror.
FIGURE 3-23. Mild vi reous hemorrhage in PDR.
FIGURE 3-24. Large prere inal hemorrhage in PDR resul ing in a large, dense sco oma.
FIGURE 3-25. Boa -shaped prere inal hemorrhage in PDR.
FIGURE 3-26. More dense vi reous hemorrhage.

B
FIGURE 3-27. A. Early-phase IVFA showing enlargemen o oveal avascular zone in diabe ic re inopa hy.
No e he hyper uorescen do in erior o he ovea (arrow) . B. Hyper uorescence and linear horizon al
hypo uorescence develop suddenly during he IVFA as spon aneous prere inal hemorrhage begins o occur
rom a iny area o NVE.
( continued)
D
FIGURE 3-27. ( Continued) C. T e area o hyper- and hypo uorescence enlarges as hemorrhage expands during
he IVFA. D. Red- ree pho ograph showing resh prere inal hemorrhage. Pho o was aken shor ly a er (C) .
FIGURE 3-28. Laser pho ocoagula ion scars spaced abou one burn wid h apar in panre inal pho ocoagula ion
(PRP) .
FIGURE 3-29. PRP scars in PDR. No e sparing o he macula.

FIGURE 3-30. Equa or-plus pho o a er ull PRP.

B
FIGURE 3-31. A. Pos erior pole a er PRP. T e pa ien had no appreciable macular hickening bu vision was
reduced o 20/ 80. B. IVFA showing enlarged, irregular oveal avascular zone (inse ) iden i ying ischemia as he
mechanism o vision loss.
B
FIGURE 3-32. A. rac ion re inal de achmen involving he macula. B. Pos opera ive appearance a er
vi rec omy, membrane peeling, and PRP.
B
FIGURE 3-33. A. Marked rac ion on he macula in a pa ien ound o have a ull- hickness re inal break as well
during vi rec omy. B. Pos opera ive appearance shows he re ina o be at ached, PRP and a residual vi reous
cavi y gas bubble ( arrow) slowly resolving.
FIGURE 3-34. Wide angle pho ograph showing combined rac ion and rhegma ogenous re inal disease in PDR.
No e he ull- hickness re inal hole nasally and adjacen whi e f brovascular rac ion.
FIGURE 3-35. A. Preopera ive appearance o highly eleva ed and vascularized NVD. B. wo days a er
in ravi real injec ion o bevacizumab he NVD looks whi e and issue manipula ion during vi rec omy is grea ly
acili a ed.
DIABE
D I ABE
E IC
CPPAPILLO
AP
P IL
L L O PA
PA H Y DIFFERENTIAL DIAGNOSIS
D
Op ic disc neovasculariza ion rom PDR
iabe ic papillopa hy describes op ic disc
edema, ei her unila eral or bila eral, in a AION
diabe ic pa ien wi h evidence or minimal or Op ic neuri is
mild op ic nerve dys unc ion and no evidence Op ic disc drusen
or ocular in amma ion or eleva ed in racra-
Papilledema
nial pressure.
EPIDEMIOLOGY DIAGNOSTIC EVALUATION

AND ETIOLO GY
In general, no workup is needed in he
Roughly 70% o pa ien s have ype 1 appropria e clinical set ing. I he case is
diabe es, and 60% o cases are unila eral. a ypical, hen imaging such as a magne ic
resonance scan is indica ed.
E iology is uncer ain, al hough some
sugges a mild nonar eri ic an erior ischemic Visual eld examina ion may be use ul o
op ic neuropa hy (AION). documen and ollow visual loss.
Fluorescein angiography can dis inguish
HISTORY diabe ic papillopa hy rom NVD bu is
rarely needed. Diabe ic papillopa hy ypi-
Pa ien s may have blurry vision or be cally s ains wi hou signi can leakage on
asymp oma ic. uorescein angiography whereas NVD
Rarely here will be ransien visual shows leakage o dye in o he vi reous
obscura ion. cavi y.
Neurologic symp oms are absen .
PROGNOSIS AND
CLINICAL SIGNS
No rea men is indica ed. Subs an ial
Disc swelling occurs wi h prominen sur- spon aneous recovery occurs, usually over
ace vessels and ne hemorrhages on he disc. weeks o mon hs, wi h many pa ien s show-
ing sub le op ic a rophy or visual eld de ec s
An af eren papillary de ec is presen bu
permanen ly.
generally no severe.
Pa ien s should be moni ored o rule ou
Visual loss is o en mild wi h 20/ 40 or be -
rapid progression o PDR, which occurs in a
er in 75% o cases.
minori y o cases.
ASSOCIATED
CLINICAL SIGNS
A crowded op ic disc is o en presen ,

similar o AION. T is does no correla e wi h
he degree o diabe ic re inopa hy.
C H AP T ER
e inal Vascular Disease

Gary C. Brown
CO
O ON
N-WO
-W
-WO
WO O L SP
SPO
PO S he sudden appearance o corresponding
blind spo s.
C ot on-wool spo s describe he re inal

change resul ing rom acu e blockage o
blood ow wi hin a erminal re inal ar eriole.
Pupillary changes: An af eren pupillary
de ec is usually absen .
Fundus changes: Cot on-wool spo s
appear in he pos erior pole o he undus
EPIDEMIOLOGY (no he peripheral re ina) as super cial areas
AND ETIOLO GY o re inal opaci ca ion ha charac eris ically
measure less han one quar er disc area in size
T e prevalence is uncer ain. Cot on-wool (Fig. 4-1). When associa ed wi h diabe es
spo s are seen in over 40% o cases o diabe ic melli us, sys emic ar erial hyper ension, and
re inopa hy and also wi h acu e sys emic re inal venous obs ruc ion, hey are generally
ar erial hyper ension. seen concomi an ly wi h re inal hemorrhages.
No heredi ary pat ern is known. Cot on-wool spo s usually resolve over 5 o
7 weeks bu may remain longer when presen
PATHOPHYSIOLOGY in conjunc ion wi h diabe ic re inopa hy.
Embolic
Hyper ensive ar eriolar necrosis In amma ory re ini is may occur
In amma ory rom en i ies such as oxoplasmosis or
See Diagnos ic Evalua ion below cy omegalovirus.
e inal hemorrhages are ypically presen
CLINICAL SIGNS wi h he lat er.
T ere are also usually vi reous cells pres-
Visual acui y: Cen ral visual acui y is usu- en wi h in amma ory condi ions, bu no
ally unaf ec ed, al hough pa ien s may no e wi h cot on-wool spo s alone.
133
134 4 RETINAL VASCULAR DISEASE
DIAGNOSTIC EVALUATION Miscellaneous: Migraine, Lyme disease,

hypo ension, acquired immunode ciency
In ravenous uorescein angiography is syndrome (AIDS), in er eron herapy, me -
minimally help ul. I reveals areas o rela- as a ic carcinoma, in ravenous drug abuse
ive hypo uorescence corresponding o he (chronic), papilledema, acu e pancrea i is,
cot on-wool spo s. severe anemia, radia ion re inopa hy, lep o-
Sys emic workup (similar o ha o acu e spirosis, Pur scher’s re inopa hy.
cen ral re inal ar ery obs ruc ion, unless
he obvious causes o diabe ic re inopa hy, PROGNOSIS AND
sys emic ar erial hyper ension, and re inal MANAGEMENT
venous obs ruc ion are presen ):
Diabe ic re inopa hy: Cot on-wool T e visual prognosis or cen ral vision is
spo s are presen in 44% o cases. good unless here are innumerable cot on-
Sys emic ar erial hyper ension: Dias olic wool spo s as wi h en i ies such as sys emic
blood pressure o 105 o 110 mm Hg or lupus ery hema osus, pancrea i is, Pur scher’s
more is usually necessary o induce cot on- re inopa hy, or in ravenous drug abuse.
wool spo orma ion in adul s. Associa ed damage rom en i ies ha cot on-
wool spo s accompany (e.g., diabe ic re inop-
e inal vein obs ruc ion: Cen ral, branch.
a hy or re inal venous obs ruc ion) can lead
Embolic: Caro id and cardiac. o severe visual loss.
In amma ory: Gian cell ar eri is, T ere is no consis en ly proven rea -
Wegener’s granuloma osis, polyar eri is men o ameliora e he visual acui y. When
nodosa, sys emic lupus ery hema osus, known diabe ic re inopa hy and re inal vein
scleroderma, orbi al mucormycosis, oxo- obs ruc ion are excluded as causes, a serious
plasmosis re ini is. associa ed sys emic disease can be ound in
Coagulopa hies: Sickle cell disease, 95% o cases. T us, i is cri ical o under ake
homocys einuria, lupus an icoagulan a sys emic workup i here is no appreciable
syndrome, pro ein S de ciency, pro ein C underlying cause, even i only one cot on-
de ciency, an i hrombin III de ciency, and wool spo is presen .
he ac or V Leiden mu a ion.
Cot on-Wool Spo s 135
FIGURE 4-1. Cot on wool spo s. Mul iple cot on wool spo s inse in he undus o a pa ien wi h human
immunodef ciency virus HIV in ec ion.
re inopa hy. Ar eriovenous nicking o a re inal

H YP
YPER
P E R ENN SIVE
SIV
VE vein does no occur a an ar eriovenous cross-
RE
R E INN O PA
A HY ing unless he re inal ar ery is loca ed an erior
o he re inal vein, a phenomenon which
H yper ensive re inopa hy re ers o he

re inal vascular changes associa ed wi h
sys emic ar erial hyper ension. Hyper ensive
occurs in approxima ely wo- hirds o he ar e-
riovenous crossings in he pos erior pole.
Grade 3: T e sys emic dias olic blood
choroidopa hy may also accompany he acu e
pressure in an adul wi h grade 3 hyper en-
phases o hyper ensive re inopa hy. Blood
sive re inopa hy is ypically a leas 105 o
pressure can vary hroughou he day, as
110 mm Hg. A his poin , he re inal ar eries
can coverage wi h hyper ensive medica ions.
lose heir abili y o au oregula e he blood
T us, having a pa ien measure blood pres-
ow, and he high pressure is passed dis ally
sure a home hree imes a day or 3 o 4 days
o he re inal ar erioles and capillary bed. T is
gives he clinician and he pa ien grea er con-
same clinical signs can be seen in children
dence ha he blood pressure is ei her s able
when he sys olic blood is in he nine ies.
or no .
Grade 4: T e sys emic dias olic blood
pressure in an adul wi h grade 4 hyper ensive
EPIDEMIOLOGY re inopa hy is usually a leas 130 o 140 mm
Hg. Wi h bo h grades 3 and 4 hyper ensive
Hyper ensive re inopa hy can be divided re inopa hy, he increased blood pressure can
in o chronic and acu e phases. T e mos damage he blood vessel wall, leading o bri-
commonly used classi ca ion is he Kei h– noid necrosis ( he presence o brin hrombi
Wagener–Barker classi ca ion. T e grades o wi hin he vascular lumina). A similar process
hyper ensive re inopa hy are as ollows: occurs wi h hyper ensive choroidopa hy,
Grade 1: e inal ar erial narrowing leading o necrosis o he overlying re inal
(Fig. 4-2) pigmen epi helium (Elschnig spo ).
Grade 2: e inal ar eriovenous nicking
(Fig. 4-3) CLINICAL SIGNS
Grade 3: e inal hemorrhages, cot on-
Grade 1 and 2 changes are chronic,
wool spo s, hard exuda es (Fig. 4-4)
whereas grade 3 and 4 changes indica e acu e
Grade 4: Grade 3 changes plus op ic re inal vascular decompensa ion.
disc swelling (Fig. 4-5)
Hyper ensive choroidopa hy (Elschnig
Grades 1 and 2 are commonly seen in spo s) may accompany grade 3 and 4 changes.
prac ice. Grade 3 and 4 changes are much less Elschnig spo s are round and yellow acu ely,
requen ly seen. even ually changing o pigmen ed lesions.
PATHOPHYSIOLOGY DIFFERENTIAL DIAGNOSIS
Grades 1 and 2: Hyaliniza ion and hicken- Diabe ic re inopa hy, radia ion re inopa hy,
ing o he re inal ar erial walls is seen, leading venous occlusive disease, caro id ar ery occlu-
o he s raigh ened vessels in grade 1 and he sive disease (ocular ischemic syndrome), and
inden a ion (ar eriovenous nicking) o he re i- collagen vascular diseases can all mimic he
nal veins by he ar eries in grade 2 hyper ensive changes o hyper ensive re inopa hy.
DIAGNOSTIC EVALUATION resolu ion o he undus abnormali ies over a

period o weeks o mon hs in eyes wi h grade
Measuremen o he sys emic blood 3 and 4 changes, bu o en does no af ec he
pressure is cri ical when he diagnosis is sus- changes seen wi h grades 1 and 2 hyper en-
pec ed. I he acu e changes (grades 3 and sive re inopa hy.
4) are classic and he blood pressure is no Laser herapy has no been shown o be o
eleva ed a he ime o measuremen , consid- bene in rea ing he visual loss associa ed
era ion should be given o he possibili y ha wi h grade 3 and 4 hyper ensive re inopa hy.
he blood pressure has been uncon rolled Correc ing he blood pressure a er grade
recen ly or is uncon rolled a o her imes dur- 4 re inopa hy can lead o visual improvemen ,
ing he day. al hough here may be residual, permanen
visual loss due o vascular shu down in
hyper ensive choroidopa hy, hyper ensive
PROGNOSIS AND re inopa hy, hyper ensive op ic neuropa hy.
MANAGEMENT Oral ni edipine has been sugges ed or he
rea men o acu e grade 4 hyper ensive
Vision is ypically unaf ec ed wi h grades re inopa hy.
1 and 2 hyper ensive re inopa hy and may be
T eore ically, in ravi real VEGF-A inhibi-
mildly decreased wi h grade 3 re inopa hy.
or herapy wi h ranibizumab and/ or beva-
Wi h grade 4 re inopa hy, vision can be cizumab could s abilize he walls o re inal
markedly decreased due o re inal edema, o vessels wi h grade 3 or 4 sys emic ar erial
hard exuda es in he cen ral macula (macular hyper ension changes. As o his wri ing, da a
s ar) and/ or he presence o a serous re inal on he rea men o hyper ensive re inopa hy
de achmen . wi h hese in erven ions are lacking.
T e rea men or hyper ensive re inopa- I he blood pressure associa ed wi h grade
hy is o correc he underlying condi ion by 4 hyper ensive re inopa hy is un rea ed, he
normalizing he blood pressure. T is causes 18-mon h mor ali y ra e is 94%.
FIGURE 4-2. Hyper ensive re inopa hy, grade 1. T e re inal ar eries are markedly narrowed and s raigh ened.
Small re inal hemorrhages are presen , no due o hyper ension bu due o background diabe ic re inopa hy.
FIGURE 4-3. Hyper ensive re inopa hy, grade 2. Prominen ar eriovenous nicking arrows is seen. Small
re inal hemorrhages are presen , no due o hyper ension, bu due o diabe ic re inopa hy.
FIGURE 4-4. Hyper ensive re inopa hy, grade 4. Cot on wool spo s, re inal hemorrhages, a macular s ar
composed o in rare inal lipid exuda es, and a serous de achmen o he macula are all presen . T e op ic nerve
head is swollen, he ea ure ha separa es grade 3 and grade 4 hyper ensive re inopa hy.
B
FIGURE 4-5. Hyper ensive re inopa hy, grade 4. A. Re inal hemorrhages are presen , he op ic disc is
swollen, and an exuda ive re inal de achmen arrows is presen in eriorly. Yellow Elschnig spo s as erisk are
presen in he macula. B. Fluorescein angiogram corresponding o A. Pro ound re inal capillary nonper usion
is presen in he macula macular ischemia , and oci o hyper uorescence corresponding o he Elschnig spo s
as erisk are also seen in he macula.
(continued)
C
FIGURE 4-5. Continued Hyper ensive re inopa hy, grade 4. C. Fluorescein angiogram o he superior
undus o he eye shown in A. Numerous oci o hyper uorescence corresponding o Elschnig spo s as erisk
can be seen.
Fundus changes: T ree varian s occur:

CILIO
C ILII O RE
R E INAL
IN
N AL AR
AR ERY
E RY
Ciliore inal ar ery obs ruc ion alone
O BSS RURUC
U C IIO
ON (Fig. 4-6): Super cial re inal whi ening,
( O CCLUSIO
C C LUU SIO N) usually loca ed wi hin he papillomacu-
lar bundle, bu which may ake hours o
C iliore inal ar ery obs ruc ion is he acu e

blockage o blood ow wi hin a ciliore i-
nal ar ery.
develop
Ciliore inal ar ery obs ruc ion associ-
a ed wi h cen ral re inal vein obs ruc ion
Ciliore inal ar ery obs ruc ion associ-
EPIDEMIOLOGY a ed wi h acu e an erior ischemic op ic
AND ETIOLO GY neuropa hy (Fig. 4-7): Mus be par icularly
concerned abou underlying gian cell
Ciliore inal ar ery obs ruc ion ypically ar eri is
occurs in pa ien s aged 65 years and older bu e inal in ra-ar erial emboli: Prevalence is
can be seen a any age. uncer ain.
I is seen in approxima ely 1:100,000 ou - Choles erol (Hollenhors plaque named
pa ien oph halmology visi s. a er ober Hollenhors a he Mayo
T e abnormali y is unila eral in over 99% Clinic): Glis ening yellow and ypically
o cases. No heredi ary pat ern is known. arises rom he caro id ar eries. T e mos
likely scenario ollows:
PATHOPHYSIOLOGY 1. A herosclerosis causes urbulen ow.
2. urbulence causes damage o he
Embolic caro id endo helium.
Hyper ensive ar erial necrosis 3. Plaque is dislodged rom he underly-
In amma ory (e.g., gian cell ar eri is) ing, exposed caro id a herosclerosis.
T e plaque ravels o lodge in he
Hemorrhage under an a herosclero ic
cen ral re inal ar ery or branch re inal
plaque
ar ery.
Associa ed wi h cen ral re inal vein
4. A brin–pla ele hrombus may or
obs ruc ion
may no be he nidus or he develop-
men o a pla ele – hrombin. T us,
CLINICAL SIGNS a larger embolus may cause a more
severe occlusion.
Visual acui y: Generally, here is a his-
5. Even ually, he endo helium repairs
ory o acu e, unila eral, painless visual
he damage and he plaque is no
eld loss occurring over several seconds.
exposed o blood.
Approxima ely 10% o hose af ec ed have
a his ory o ransien visual loss (amaurosis 6. urbulence
ugax) in he af ec ed eye prior o he cen ral Calci c: Large, whi e plaque generally
re inal ar erial obs ruc ion. origina ing rom he cardiac valves
Pupillary changes: An af eren pupillary Fibrin–pla ele : Longer and dull whi e;
de ec may presen immedia ely, depending may origina e rom he caro ids or cardiac
on he area o dis ribu ion o he obs ruc ion. valves
Ciliore inal Ar ery Obs ruc ion (Occlusion) 143
DIFFERENTIAL DIAGNOSIS syndrome, pro ein S de ciency, pro ein C

de ciency, an i hrombin III de ciency
In amma ory re ini is rom en i ies such Miscellaneous: Fibromuscular hyper-
as oxoplasmosis or cy omegalovirus. e inal plasia, Sydenham’s chorea, Fabry’s disease,
hemorrhages are ypically presen wi h he migraine, Lyme disease, hypo ension
lat er.
T ere are also usually vi reous cells pres- PROGNOSIS AND
en wi h in amma ory condi ions bu no MANAGEMENT
wi h acu e ciliore inal ar ery obs ruc ion.
Wi h isola ed ciliore inal ar ery obs ruc-
DIAGNOSTIC EVALUATION ion, 90% o eyes re urn o 20/ 40 vision or
bet er. Wi h cen ral re inal vein occlusion,
In ravenous uorescein angiography: 70% o eyes re urn o 20/ 40 vision or bet er.
Ciliore inal ar eries normally ll wi h uo- Wi h an erior ischemic op ic neuropa hy,
rescein dye during he early choroidal lling vision o en remains coun ing ngers o hand
phase o a uorescein angiogram. A ciliore i- mo ions.
nal ar ery obs ruc ion ypically shows non- T ere is no consis en ly proven rea men
per usion o dye in he af ec ed vessel hrough o ameliora e he visual acui y. Because o he
he re inal ar eriovenous phase. rela ively good prognosis or cen ral vision,
Sys emic workup: T is is similar o ha o digi al massage and an erior chamber para-
acu e cen ral re inal ar ery obs ruc ion. cen esis are no ypically under aken.
Emboli: Caro id and cardiac Despi e he lack o an ef ec ive ocular
In amma ory: Gian cell ar eri is, rea men , a sys emic workup should be
Wegener’s granuloma osis, polyar eri is under aken. Al hough gian cell ar eri is
nodosa, sys emic lupus ery hema osus, likely only accoun s or 1% o 2% o cases,
orbi al mucormycosis, oxoplasmosis he possibili y should be ac ively inves iga ed
re ini is because he ellow eye can be involved by
re inal ar erial obs ruc ion wi hin hours o
Coagulopa hies: Sickle cell disease,
days.
homocys inuria, lupus an icoagulan
FIGURE 4-6. Ciliore inal ar er y obs ruc ion. Isola ed ciliore inal ar ery obs ruc ion. No e he re inal
whi ening indica ing ischemic re inal edema inse .
FIGURE 4-7. Ciliore inal ar er y obs ruc ion and ischemic op ic neuropa hy. Ciliore inal ar ery occlusion
associa ed wi h an erior ischemic op ic neuropa hy. No e he associa ed disc edema and pallor.
Branch Re inal Ar eryObs ruc ion (Occlusion) 145
Choles erol (Hollenhors plaque):

BRAN
B RAAN CHH RE E INN AL
AL Glis ening yellow and ypically rom he
AR
A R ERY
E RY
Y O BSS RUC
RUU C IIO
ON caro id ar eries
( O CCLUSIO
C C LU
U SIO N)
N) Calci c: Large, whi e plaque generally
origina ing rom he cardiac valves (Fig. 4-9)
B ranch re inal ar ery obs ruc ion is he

acu e blockage o blood ow wi hin a
branch re inal ar ery.
Fibrin–pla ele : Longer and dull whi e;
may origina e rom he caro ids or cardiac
valves (see Fig. 4-8A)
EPIDEMIOLOGY
AND ETIOLO GY DIFFERENTIAL DIAGNOSIS
Branch re inal ar ery obs ruc ion ypically In amma ory re ini is may occur rom
occurs in pa ien s aged 65 years and older bu en i ies such as oxoplasmosis or cy omegalo-
can be seen a any age. virus. e inal hemorrhages are ypically pres-
en wi h he lat er.
I is seen in approxima ely 1:15,000 o
20,000 ou pa ien oph halmology visi s. T ere are also usually vi reous cells pres-
en wi h in amma ory condi ions bu no
T e abnormali y is unila eral in 99% o wi h acu e branch re inal ar ery obs ruc ion.
cases. No heredi ary pat ern is known.
PATHOPHYSIOLOGY
In ravenous uorescein angiography:
Embolic eveals a delay in re inal ar erial and venous
Hyper ensive ar erial necrosis lling in he area o obs ruc ion versus he
In amma ory (e.g., gian cell ar eri is) normal remaining undus (see Fig. 4-8A).
T ere may be s aining o he ischemic re inal
Hemorrhage under an a herosclero ic plaque vascula ure (see Fig. 4-8C).
Sys emic workup: T is is similar o ha o
CLINICAL SIGNS acu e cen ral re inal ar ery obs ruc ion.
Visual acui y: Generally, here is a his- Embolic: Caro id and cardiac
ory o acu e, unila eral, painless visual In amma ory: Gian cell ar eri is,
eld loss occurring over several seconds. Wegener’s granuloma osis, polyar eri is
Approxima ely 10% o hose af ec ed have nodosa, sys emic lupus ery hema osus,
a his ory o ransien visual loss (amaurosis orbi al mucormycosis, oxoplasmosis
ugax) in he af ec ed eye. re ini is
Pupillary changes: An af eren pupillary Coagulopa hies: Sickle cell disease,
de ec may presen immedia ely, depending homocys einuria, lupus an icoagulan
on he area o dis ribu ion o he obs ruc ion. syndrome, pro ein S de ciency, pro ein C
Fundus changes: Super cial re inal whi - de ciency, an i hrombin III de ciency
ening (Fig. 4-8A) can ake hours o develop. Miscellaneous: Fibromuscular hyper-
e inal in ra-ar erial emboli (prevalence plasia, Sydenham’s chorea, Fabry’s disease,
uncer ain): migraine, Lyme disease, hypo ension
PROGNOSIS AND digi al massage and an erior chamber para-

MANAGEMENT cen esis are no ypically under aken.
Despi e he lack o an ef ec ive ocular
Mos pa ien s improve o 20/ 40 or bet er rea men , a sys emic workup should be
vision wi hou rea men , al hough a eld under aken. Al hough gian cell ar eri is
de ec corresponding o he area o obs ruc- likely only accoun s or 1% o 2% o cases,
ion usually persis s. he possibili y should be ac ively inves iga ed
T ere is no consis en ly proven rea men because he ellow eye can be involved wi hin
o ameliora e he visual acui y. Because o he hours o days.
rela ively good prognosis or cen ral vision,
A
FIGURE 4-8. Branch re inal ar er y obs ruc ion. A. Re inal whi ening inse due o branch re inal ar ery
obs ruc ion. No e he proximal in ra ar erial pla ele f brin hrombus arrow .
continued
Branch Re inal Ar ery Obs ruc ion (Occlusion) 147
C
FIGURE 4-8. (Continued) Branch re inal ar er y obs ruc ion. B. Fluorescein angiogram corresponding o
A reveals re inal ar eriolar and capillary nonper usion in he dis ribu ion o he occluded vessel. C. S aining o
he in ero emporal branch re inal ar ery is presen in he area o occlusion.
FIGURE 4-9. Branch re inal ar er y obs ruc ion, calcif c plaque. In ra ar erial calcif c plaque arrow
associa ed wi h branch re inal ar ery occlusion.
Fundus changes
CEN
C ENN RAL
RA
AL RE INAL
IN
N AL
Super cial re inal whi ening: Can ake
AR
A R ERY
E RY
Y O BS
S RUC
RUU C IIO
ON hours o develop
( O CCLUSIO
C C LU
U SIO N)
N)
Cherry red spo in he oveola (Fig. 4-10)
C en ral re inal ar ery obs ruc ion is he

acu e blockage o blood ow wi hin he
cen ral re inal ar ery.
Ciliore inal ar erial sparing o cen ral
ovea (Fig. 4-11): Presen in 10% o cases
e inal in ra-ar erial emboli: Presen in
20% o cases
EPIDEMIOLOGY Choles erol (Hollenhors plaque):
Glis ening yellow (Fig. 4-12) and ypically
AND ETIOLO GY origina es rom he caro id ar eries
Cen ral re inal ar ery obs ruc ion ypically Calci c: Large, whi e plaque generally
occurs in pa ien s aged 65 years and older bu origina ing rom he cardiac valves
can be seen a any age. Fibrin–pla ele : Longer and dull whi e;
I is seen in approxima ely 1:10,000 ou pa- may origina e rom he caro ids or cardiac
ien oph halmology visi s. valves (see Fig. 4-8A)
T e abnormali y is unila eral in 99% o
cases. DIFFERENTIAL DIAGNOSIS
No heredi ary pat ern is known.
Acu e oph halmic ar ery obs ruc ion
(cherry red spo absen )
PATHOPHYSIOLOGY ay–Sach’s disease (cherry red spo pres-
en , bu in in an s less han 1 year o age and
Embolic
wi h severe neurologic dys unc ion)
Hyper ensive ar erial necrosis
Dissec ing aneurysm wi hin he cen ral DIAGNOSTIC EVALUATION
re inal ar ery
In amma ory (e.g., gian cell ar eri is) In ravenous uorescein angiography:
Hemorrhage under an a herosclero ic eveals delay in re inal ar erial and venous
plaque lling (normally, he vein o he emporal
Vasospasm vascular arcade should comple ely ll wi hin
11 seconds a er dye en ers he corresponding
re inal ar eries.
CLINICAL SIGNS Elec rore inography: Normal a-wave
ampli ude, bu diminished b-wave ampli ude.
Visual acui y: Generally, here is a his ory o
acu e, unila eral, painless visual loss occurring Sys emic workup
over several seconds. Approxima ely 10% o Embolic: Caro id and cardiac
hose af ec ed have a his ory o ransien visual In amma ory: Gian cell ar eri is,
loss (amaurosis ugax) in he af ec ed eye. Wegener’s granuloma osis, polyar eri is
Pupillary changes: An af eren pupillary nodosa, sys emic lupus ery hema osus,
de ec is usually presen immedia ely. orbi al mucormycosis
Coagulopa hies: Sickle cell disease, massage o he globe and an erior chamber
homocys einuria, lupus an icoagulan paracen esis has been advoca ed bu has
syndrome, pro ein S de ciency, pro ein C minimal bene . rea men wi h brinoly ic
de ciency, an i hrombin III de ciency agen s is s ill considered inves iga ional.
Miscellaneous: Fibromuscular hyper- Despi e he lack o a consis en ly ef ec ive
plasia, Sydenham’s chorea, Fabry’s disease, ocular rea men , a sys emic workup should
migraine, Lyme disease, hypo ension be under aken. Al hough gian cell ar eri is
Vasospas ic: Migraine likely only accoun s or 1% o 2% o cases,
he possibili y should be ac ively inves iga ed
because he ellow eye can be involved wi hin
PROGNOSIS AND hours o days. In regard o sys emic workup, i
MANAGEMENT should be no ed ha pa ien s wi h acu e cen-
ral ar ery obs ruc ion have a high dea h ra e
T e visual prognosis is ypically poor, rom cardiac vascular disease.
wi h mos pa ien s re aining coun ing nger I iris neovasculariza ion develops, laser
o hand mo ions vision and a small emporal panre inal pho ocoagula ion (P P) should
island o vision remaining. I a ciliore inal be considered o help preven neovascular
ar ery spares he cen ral ovea, 80% o eyes glaucoma. I causes resolu ion o he new iris
will re urn o 20/ 20 o 20/ 50 vision over a vessels in approxima ely wo- hirds o he
period o 2 weeks. Never heless, in he lat er rea ed cases.
ins ance here is ypically severe visual eld
loss. Approxima ely 18% o eyes will progress Embolec omy has been per ormed wi h
o develop iris neovasculariza ion wi hin 4 o he yt rium-aluminium-garne (YAG) by
6 weeks a er he acu e obs ruc ion. crea ing a hole in he ar ery over he laser,
hough he incidence o vi reous hemorrhage
T ere is no consis en ly proven rea - is high and he resul s variable.
men o ameliora e he visual acui y. Digi al
FIGURE 4-10. Acu e cen ral re inal ar er y occlusion. Superf cial re inal opacif ca ion is presen , and a cherry
red spo can be seen in he oveola. No e he segmen ed columns o blood in re inal ar erioles boxcarring .
FIGURE 4-11. Cen ral re inal ar er y occlusion wi h ciliore inal ar er y sparing. Acu e cen ral re inal ar ery
occlusion inse wi h ciliore inal ar erial sparing o he oveola. Compare wi h Figure 4-6.
FIGURE 4-12. Hollenhors plaque. Glis ening choles erol embolus Hollenhors plaque wi hin a re inal
ar eriole arrow . T ese emboli ypically lodge a re inal ar eriolar bi urca ions.
occurring over a period ranging rom seconds.

ACU
A CUU E O PH H HA ALMIC
LM
MI C T e visual acui y is no ligh percep ion in 90%
AR
A R ERY
E RY
Y O BS
S RUC
RU
U C IIO
ON o cases.
( O CCLUSIO
C C LU
U SIO N)
N) Pupillary changes: An af eren pupillary
de ec is presen .
A cu e oph halmic ar ery obs ruc ion is he

acu e blockage o he oph halmic ar ery.
Fundus changes: Super cial re inal whi -
ening in he pos erior pole occurs, o en more
pronounced han wi h acu e cen ral re inal
EPIDEMIOLOGY ar ery obs ruc ion because he re inal pig-
AND ETIOLO GY men epi helium may be opaci ed as well
wi h acu e oph halmic ar ery obs ruc ion
Acu e oph halmic ar ery obs ruc ion (Fig. 4-13A). T e presence o a cherry red
occurs in approxima ely 1:100,000 oph hal- spo in he oveola is variable; one- hird o
mologic visi s. he pa ien s have none, one- hird have a mild
cherry red spo , and one- hird have a promi-
T e mean age o onse is in he six ies.
nen cherry red spo .
T e presence o re inal ar erial emboli is
variable. “Sal and pepper” re inal pigmen
PATHOPHYSIOLOGY epi helial changes occur in he pos erior pole
and elsewhere wi hin weeks a er he acu e
Embolic obs ruc ion. T e pigmen epi helial changes
rauma do no occur secondary o cen ral re inal
In ec ious (e.g., mucormycosis o he ar ery obs ruc ion alone.
orbi )
In amma ory (e.g., collagen vascular DIFFERENTIAL DIAGNOSIS
disease, gian cell ar eri is)
e er o Table 4-1.
O her (see causes o acu e cen ral re inal
ar ery obs ruc ion)
CLINICAL FEATURES
In ravenous uorescein angiography
Visual acui y: Generally, here is a his- Delay in choroidal lling: T e choroid
ory o acu e, unila eral, painless visual loss should be comple ely lled wi hin
TABLE 4-1. Di eren ial Diagnosis o Acu e Oph halmic Ar ery Obs ruc ion
Central Retinal Artery Obstruction Ophthalmic Artery Obstruction
Vision Finger coun ing—hand mo ions No ligh percep ion
Fundus Re inal opacifca ion wi h cherry Marked opacifca ion ± cherry
red spo red spo
Fluorescein angiography Delayed re inal vascular f lling Delayed choroidal and re inal
vascular f lling
Elec rore inography Decreased b-wave Decreased a- and b-waves
5 seconds a er he rs appearance o uo- Miscellaneous: Fibromuscular

rescein dye wi hin i (Fig. 4-13B). hyperplasia, Sydenham’s chorea,
Delayed re inal ar erial and venous Fabry’s disease, migraine, Lyme disease,
lling. hypo ension
La e ocal or dif use s aining o he re i- T e mos common e iology is as an ia ro-
nal pigmen epi helium due o choroidal genic sequela o re robulbar injec ion.
ischemia.
Elec rore inography: Decreased or PROGNOSIS AND
absen a-wave (ou er layer re inal ischemia) MANAGEMENT
and b-wave (inner layer re inal ischemia)
ampli udes. Al hough spon aneous reversal can rarely
occur, he long- erm vision in mos cases is
Sys emic workup: T is is similar o ha o
usually ligh percep ion o no ligh percep-
acu e cen ral re inal ar ery obs ruc ion.
ion. T ere is no proven rea men o amelio-
Embolic: Caro id and cardiac ra e he visual acui y. Despi e he lack o an
In amma ory: Gian cell ar eri is, ef ec ive ocular rea men , a sys emic workup
Wegener’s granuloma osis, polyar eri is should be under aken.
nodosa, sys emic lupus ery hema osus, T e pa ien should be observed regu-
orbi al mucormycosis, oxoplasmosis larly or he rs several mon hs or he
re ini is. developmen o iris neovasculariza ion.
Coagulopa hies: Sickle cell disease, Laser P P should be considered i iris neo-
homocys einuria, lupus an icoagulan vasculariza ion develops ( he incidence o
syndrome, pro ein S de ciency, pro ein developmen o iris neovasculariza ion is
C de ciency, an i hrombin III de ciency unknown).
B
FIGURE 4-13. Acu e oph halmic ar er y obs ruc ion. A. Marked re inal whi ening is presen , and a cherry red
spo is absen . T e visual acui y was no ligh percep ion. B. Fluorescein angiogram corresponding o A. A 116
seconds a er injec ion, here is no dye wi hin he re inal vessels and he majori y o he choroid. Peripapillary
s aining is presen , presumably due o colla erals be ween episcleral vessels and he choroidal circula ion.
e inal hemorrhages
CO MBIN
M BII N ED
DCCEN
E N RAL
R AL
L
Macular edema
RE INAL
I NA
AL ARAR ERY
E RY
Y AN
AN D
VEIN
VEI I N O BS R RUC
UC C IO
ON
( O CCLUSIO
C C LU SIOO N)
N)
In amma ory re ini is rom
C ombined cen ral re inal ar ery and vein
obs ruc ion is he acu e blockage o bo h
he cen ral re inal ar ery and he cen ral re inal
cy omegalovirus
Cen ral re inal vein obs ruc ion (no cherry
vein. red spo is presen )

AND ETIOLO GY
In ravenous luorescein angiography:
T e prevalence is uncer ain. eveals a delay in re inal ar erial and
No heredi ary pat ern is known. venous illing in he area o obs ruc ion
versus he normal remaining undus. Severe
re inal capillary nonper usion is o en
PATHOPHYSIOLOGY
presen .
T e disease process is uncer ain; blockage Sys emic workup: T is is similar o ha o
o bo h he cen ral re inal ar ery and he cen- acu e cen ral re inal ar ery obs ruc ion.
ral re inal vein has been shown in one case. Embolic: Caro id and cardiac
In amma ory: Gian cell ar eri is,
CLINICAL SIGNS Wegener’s granuloma osis, polyar eri is
nodosa, sys emic lupus ery hema osus,
Visual acui y: Generally, here is a his ory orbi al mucormycosis, oxoplasmosis
o acu e or subacu e, unila eral, painless visual re ini is
eld loss occurring over a period ranging Coagulopa hies: Sickle cell disease,
rom seconds o days. homocys einuria, lupus an icoagulan
Pupillary changes: An af eren pupillary syndrome, pro ein S de ciency, pro ein C
de ec is ypically presen . de ciency, an i hrombin III de ciency
Fundus changes (Fig. 4-14) Miscellaneous: Fibromuscular hyper-
Super cial re inal whi ening in he pos- plasia, Sydenham’s chorea, Fabry’s disease,
erior pole migraine, Lyme disease, hypo ension
Cherry red spo in he oveola T e mos common e iology is as a sequela
Dila ed, or uous re inal veins o re robulbar injec ion.
Combined Cen ral Re inal Ar ery and Vein Obs ruc ion (Occlusion) 157
PROGNOSIS AND o an ef ec ive ocular rea men , a sys emic

MANAGEMENT workup should be under aken. T e pa ien
should be ollowed regularly or he rs
Al hough here are excep ions, he vision several mon hs or he developmen o iris
mos o en remains in he coun ing ngers o neovasculariza ion.
ligh percep ion range. Approxima ely 80% o Laser P P should be considered i iris
eyes will progress o iris neovasculariza ion a neovasculariza ion develops.
a mean ime o approxima ely 6 weeks a er I he visual acui y is coun ing ngers or
he obs ruc ion. worse, P P can be considered prior o he
T ere is no proven rea men o ame- developmen o iris neovasculariza ion.
liora e he visual acui y. Despi e he lack
FIGURE 4-14. Combined cen ral re inal ar er y and cen ral re inal vein occlusion. T e re inal veins are
dila ed and or uous, re inal hemorrhages are presen , and a cherry red spo due o superf cial re inal opacif ca ion
can be seen.
Periorbi al pain: “Ocular angina” is pres-

OC
CULAR
U L AR ISCH
IS
SC H E
EMIC
M IC
C en in abou 40% o cases and is described
SYN
SY
YN DRO
D R O ME
ME as a dull ache.
Prolonged visual recovery ime a er
O cular ischemic syndrome describes ocu-
lar symp oms and signs at ribu able o
marked caro id or oph halmic ar ery obs ruc-
exposure o brigh ligh .
Pupillary changes: An af eren pupillary
ion. Al erna ive nomencla ures include venous de ec is ypically presen .
s asis re inopa hy, ischemic ocular in amma- An erior segmen
ion, and ischemic oculopa hy. Iris neovasculariza ion (67%)
An erior chamber cells (20%)
EPIDEMIOLOGY Pos erior segmen
AND ETIOLO GY Narrowed re inal ar eries in mos cases
Approxima ely 2000 cases occur in he Dila ed, bu no or uous, re inal veins
Uni ed S a es per year. (Fig. 4-15A) in mos cases
T e en i y is unila eral in 80% o cases and Microaneurysms (see Fig. 4-15C) in
bila eral in 20%. mos cases (pos erior pole or peripheral,
or bo h)
I occurs in approxima ely 5% o pa ien s
wi h caro id ar ery obs ruc ion and is no usu- e inal do and blo hemorrhages (80%
ally seen in hose under he age o 50 years. o eyes)
T e mean age is 65 years. Neovasculariza ion o he op ic disc
and/ or re ina (35%)
Super cial re inal whi ening in he pos-
PATHOPHYSIOLOGY erior pole (12%)
Macular edema (see Fig. 4-15B) (11%)
Disease involves blockage o he caro id Spon aneous re inal ar erial pulsa ions
or oph halmic ar ery, or bo h. No ow dis ur- (4%)
bance occurs un il here is 70% obs ruc ion.
Associa ed sys emic abnormali ies
Wi h 90% obs ruc ion, per usion pressure in
he cen ral re inal ar ery decreases by 50%. Sys emic ar erial hyper ension (65%)
Hal o he cases have a 100% ipsila eral com- Cardiac disease (50%)
mon or in ernal caro id ar ery obs ruc ion. Diabe es melli us (50%)
Causes include:
Previous s roke (20%)
A herosclerosis (over 90% o cases)
Severe peripheral vascular disease (20%)
Gian cell ar eri is
CLINICAL FEATURES
Cen ral re inal vein obs ruc ion ypically
Symp oms and signs has or uous re inal veins, as well as more
Vision: Decreases over a period o weeks re inal hemorrhages and macular edema han
o mon hs, al hough in 12% here is acu e he ocular ischemic syndrome. Ligh digi al
visual loss associa ed wi h a cherry red spo . pressure on he lid, or minimal pressure wi h
oph halmodynamome ry, will induce re inal Sys emic: T ere is a 40% 5-year mor ali y,
ar erial pulsa ions wi h he ocular ischemic wi h cardiac disease as he mos common
syndrome, whereas subs an ial pressure is cause o dea h.
required wi h cen ral re inal vein occlusion.
Also consider: MANAGEMENT
Diabe ic re inopa hy
adia ion re inopa hy Laser P P is per ormed i here is iris
neovasculariza ion and he an erior cham-
DIAGNOSTIC EVALUATION ber angle is open. P P induces regression
o iris new vessels in 36% o cases. T e
In ravenous uorescein angiography: pa ien should be evalua ed or possible
Delay in choroidal lling (Fig. 4-16) occurs caro id endar erec omy. In surgical candi-
in 60% o cases. Delayed re inal ar erial and da es, 33% demons ra e improved vision,
venous lling (see Fig. 4-16) occurs in 95% 33% demons ra e s abilized vision, and
o cases. La e re inal vascular s aining, more 33% progress o lose vision despi e endar-
pronounced o he re inal ar eries (Fig. 4-17), erec omy surgery.
occurs in 85% o cases. I he caro id ar ery is 100% obs ruc ed,
Elec rore inography: Decreased or absen endar erec omy is no o bene ; nei her
a-wave (ou er layer re inal ischemia) and is ex racranial o in racranial (e.g., super -
b-wave (inner layer re inal ischemia) ampli- cial emporal o middle cerebral) bypass.
udes are seen. emember no o ignore he cardiac s a us,
Sys emic workup: Caro id noninvasive because cardiac disease is he leading cause
s udies have approxima ely 90% chance o o dea h.
de ec ing caro id s enosis o 50% or more. Endar erec omy is indica ed or symp-
Caro id ar eriography or magne ic resonance oma ic pa ien s ( hose wi h amaurosis
angiography (MR ) is per ormed i caro id ugax, ransien ischemic at ack, or nondis-
noninvasive s udies are ambiguous or i abling s roke), and hose wi h 70% o 99%
caro id ar ery surgery is being considered. ipsila eral caro id s enosis (Table 4-2).
An ipla ele herapy is indica ed or hose
PROGNOSIS who are symp oma ic and have less han
70% s enosis.
Ocular: Seven y- ve percen o eyes will Caro id s en ing can also be considered
progress o coun ing ngers o worse vision in selec cases.
wi hin 1 year a er diagnosis.
TABLE 4-2. Ou comes A er rea men o Symp oma ic Pa ien s wi h High grade
Caro id Ar ery S enosis
Endarterectomy Antiplatelet Agent
Periopera ive mor ali y 2% 1%
Severe s roke by 2 years 9% 26%
Da a rom he Nor h American Symp oma ic Caro id Endar erec omy Trial (NASCET).
B
FIGURE 4-15. Ocular ischemic syndrome. A. T e re inal veins are sligh ly dila ed, bu no or uous, and
he re inal ar eries are narrowed. A ew re inal hemorrhages are no ed in he macula. B. Fluorescein angiogram
corresponding o A. Hyper uorescence o he op ic disc and macular edema are prominen .
continued
FIGURE 4-15. (Continued) Ocular ischemic syndrome. C. Fluorescein angiogram in an eye wi h ocular
ischemic syndrome demons ra ing pinpoin oci o hyper uorescence due o microaneurysms in he
midperipheral undus.
FIGURE 4-16. Ocular ischemic syndrome. Fluorescein angiogram revealing delayed re inal ar erial and
choroidal vascular f lling in an ocular ischemic syndrome eye. No e he abnormal leading edge o uorescein dye
in he re inal ar eriole arrow .
FIGURE 4-17. Ocular ischemic syndrome. La e phase uorescein angiogram demons ra ing re inal vascular
s aining in an ocular ischemic syndrome eye.
Branch Re inalVein Obs ruc ion (Occlusion) 163
5% o 10% o hemispheric re inal vein occlu-

BR
BRAN
R AN CH
C H RE
R E INI N AL
L sions and 1% o 2% o branch re inal vein
VEIN N OBBS
S R RUC
U C IO N obs ruc ions.
( O CCLUSIO
C CL
LU SII O N)) Pos erior segmen changes: e inal venous
engorgemen and or uosi y, as well as re inal
B ranch re inal vein obs ruc ion is he acu e

blockage o blood ow wi hin a branch
re inal vein.
hemorrhages and edema, are ypically presen
wi hin he dis ribu ion o he occluded vessel
(Fig. 4-18A). Macular branch ( wig) vein
occlusions may be clinically sub le wi h mini-
EPIDEMIOLOGY mal hemorrhage, elangiec asia, or macular
AND ETIOLO GY edema.
Neovasculariza ion o he op ic disc or
Branch re inal vein obs ruc ion ypically re ina, or bo h, can develop mon hs o years
occurs in pa ien s aged 65 years and older bu a er he occlusion. Vi reous rac ion on re i-
can be seen a any age. T e Beaver Dam Eye nal or op ic disc neovasculariza ion may lead
S udy no ed a prevalence o 0.6% and a 5-year o vi reous hemorrhage wi h or wi hou rac-
incidence o 0.6% as well. No heredi ary pa - ion re inal de achmen .
ern is known.
PATHOPHYSIOLOGY DIFFERENTIAL DIAGNOSIS
Branch re inal vein occlusion ypically e inal cavernous hemangioma can occa-
occurs a a re inal ar eriovenous crossing. sionally mimic he appearance o a branch
Impingemen o he branch re inal ar ery on re inal vein occlusion.
he branch re inal vein is believed o cause
urbulen ow, leading o endo helial cell
damage and predisposing o hrombus orma-
ion wi hin he branch re inal vein.
In ravenous uorescein angiography:
When he branch re inal vein occlusion eveals a delay in re inal ar erial and venous
does no occur a an ar eriovenous crossing, lling in he dis ribu ion o he obs ruc ed
an in amma ory cause, such as rom sarcoid- vessel. e inal capillary nonper usion may be
osis, should be considered. presen (Fig. 4-18B).
Sys emic workup: Includes an evalua ion or
CLINICAL SIGNS sys emic ar erial hyper ension and increased
body mass. A his ory o glaucoma has also been
Visual acui y: Generally, here is a his ory
associa ed wi h branch re inal vein occlusion.
o unila eral, painless visual loss occurring
over a period o days.
Pupillary changes: An af eren pupillary PROGNOSIS AND
de ec may be presen , depending on he size MANAGEMENT
o he venous occlusion and he degree o
re inal ischemia. Laser Photocoagulation for Macular Edema
An erior segmen changes: Iris neovascu- T e mean resul an visual acui y in eyes
lariza ion has been observed o develop in wi h un rea ed branch re inal vein occlusion
in he Branch Vein Occlusion S udy is 20/ 70. ranibizumab herapy does no leave visual
In eyes ha are candida es or grid laser pho- eld de ec s (which end o enlarge consider-
ocoagula ion or macular edema, he mean ably over years) wi hin he macular region.
visual resul is 20/ 40 o 20/ 50. anibizumab should be considered he pri-
According o he Branch Vein Occlusion mary rea men herapy or he rea men o
S udy, laser grid pho ocoagula ion or visual macular edema associa ed wi h branch re inal
loss due o macular edema can be considered vein occlusion.
or eyes wi h branch re inal occlusion ha Sector Laser PRP
mee he ollowing cri eria: I pos erior segmen neovasculariza ion
Visual acui y o 20/ 40 o 20/ 200 develops, sec or laser P P in he dis ribu ion
In ac peri oveal capillaries wi h uores- o he obs ruc ed branch re inal vein should
cein angiography be considered. T is herapy reduces he inci-
dence o subsequen vi reous hemorrhage
esolu ion o he majori y o in rare i-
rom approxima ely 60% o 30%.
nal blood
I iris neovasculariza ion develops, sec or
Ranibizumab T erapy for Macular Edema, laser P P should be considered o help pre-
the Primary reatment ven neovascular glaucoma.
T e BR VO Clinical rial demons ra ed T e BR VO s udy, in which in ravi real
ha he mean vision in a cohor o un rea ed 0.5 mg ranibizumab injec ions were given
pa ien s wi h branch re inal vein occlusion mon hly or 6 mon hs, pa ien s gained
was approxima ely 20/ 70, while he cohor almos hree lines o vision, meaning hey
rea ed wi h in ravi real ranibizumab once a saw wice as well as sham herapy. T e near
mon h or 6 mon hs resul ed in a mean visual vision wi hou ranibizumab a 6 mon hs was
acui y o approxima ely 20/ 30. Unlike he 20/ 70 a baseline improving o 20/ 32 wi h
case wi h laser pho ocoagula ion herapy, ranibizumab.
Branch Re inal Vein Obs ruc ion (Occlusion) 165
B
FIGURE 4-18. Branch re inal vein occlusion. A. Re inal hemorrhages and cot on wool spo s are presen in
he dis ribu ion o he occluded vessel inse . B. Fluorescein angiogram corresponding o A. Re inal capillary
nonper usion is presen in he dis ribu ion o he occluded vessel. C. Op ical coherence omography in eye wi h
a branch re inal vein occlusion demons ra ing macular edema.
cen ral re inal vein occlusions (Fig. 4-19)

CEN N RAL
R AL RER E INAL
IN
N AL ypically are associa ed wi h vision o 20/ 200
VEIN N OBBS
S R RUC
U C IO N or bet er, whereas ischemic cen ral re inal
( O CCLUSIO
C CL
LU SII O N)) vein obs ruc ions (Fig. 4-20A–E) are associ-
a ed wi h vision o coun ing ngers or worse.
C
vein.
en ral re inal vein obs ruc ion is blockage
o blood ow wi hin he cen ral re inal
Approxima ely 20% o eyes wi h
nonischemic cen ral re inal vein occlusion
will even ually progress o he ischemic
varian .
EPIDEMIOLOGY Pupillary changes: An af eren pupillary
AND ETIOLO GY de ec may be presen , increasing in severi y
as he visual acui y decreases and he degree
Cen ral re inal vein obs ruc ion ypically o ischemia increases.
occurs in pa ien s aged 65 years and older bu
An erior segmen changes: Iris neovas-
can be seen a any age.
culariza ion (rubeosis iridis) develops in
In he Beaver Dam Eye S udy, he preva- approxima ely 20% o cases a a mean ime o
lence was 0.1% and he 5-year incidence was 3 o 5 mon hs a er he obs ruc ion.
0.2%.
Pos erior segmen changes
Bila erali y even ually occurs in approxi-
Dila ed, or uous re inal veins
ma ely 10% o cases, more commonly in
hose wi h underlying sys emic abnormali ies. e inal hemorrhages, mos pronounced
in he pos erior pole
e inal edema, mos pronounced in he
macula
PATHOPHYSIOLOGY
Neovasculariza ion o he op ic disc
In ravenous hrombus a or near he or re ina, or bo h, or op ic disc colla erals
lamina cribrosa is seen in eyes wi h cen ral (Fig. 4-20F); may develop mon hs a er
re inal vein obs ruc ion s udied his opa ho- he obs ruc ion
logically. Impingemen o he cen ral re inal
ar ery on he cen ral re inal vein is believed DIFFERENTIAL DIAGNOSIS
o cause urbulence and subsequen endo he-
lial damage, which predisposes o hrombus Dif eren ial diagnosis includes ocular
orma ion. ischemic syndrome (Table 4-3). Diabe ic
Increased in raocular pressure may also re inopa hy can also mimic cen ral re inal
predispose o cen ral re inal vein obs ruc ion vein obs ruc ion, bu he ormer is ypically
by heore ically bowing he lamina cribrosa bila eral, has prominen hard exuda es (rare
pos eriorly, leading o urbulence, endo helial in cen ral re inal vein obs ruc ion) and many
damage, and hrombus orma ion. more microaneurysms han wi h cen ral re i-
nal vein obs ruc ion.
CLINICAL SIGNS
Visual acui y: Generally, here is a his ory
o unila eral, painless visual loss occurring In ravenous uorescein angiography
over hours o days or weeks. Nonischemic reveals delay in re inal venous lling and
TABLE 4-3. Di eren ial Diagnosis o Cen ral Re inal Vein Obs ruc ion
Central Retinal Vein Obstruction Ocular Ischemic Syndrome
Vision 20/ 20—hand mo ions 20/ 20—no ligh percep ion
Iris neovasculariza ion 20% 67%
Re inal hemorrhages Mild o severe Mild
Re inal venous or uosi y Usually presen Absen
Macular edema Mild o severe Mild
Fluorescein angiography
Choroidal f lling Normal Delayed
Re inal AV ransi Delayed Delayed
La e ar erial s aining Absen Presen
AV, ar eriovenous.
in rare inal leakage o dye, mos prominen Hypercoagulabili y s a es (e.g., lupus

in he macula, as he s udy progresses. an icoagulan syndrome, pro ein S de -
Increasing areas o re inal capillary nonper- ciency, pro ein C de ciency, an i hrombin
usion can be seen in more ischemic cases. III de ciency, hyperhomocys einemia)
When he re inal capillary nonper usion (in
seven undus pho ographic elds) exceeds PROGNOSIS AND
75 disc areas, he incidence o developmen
o iris neovasculariza ion rises o more han MANAGEMENT
50%.
Un il he use o in ravi real ranibizumab
Elec rore inography: Normal a-wave injec ions or he rea men o he macular
ampli ude, bu diminished b-wave ampli ude, edema associa ed wi h cen ral re inal vein
occurs as ischemia increases. occlusion, here was no ef ec ive in erven ion.
Sys emic workup T e C UISE Clinical rial demons ra ed ha
Sys emic ar erial hyper ension 6-mon hly in ravi real injec ions o 0.5mg
ranibizumab resul ed in a visual acui y o
Diabe es melli us
20/ 32, while he un rea ed cohor had a mean
Hyperviscosi y syndromes (e.g., poly- visual acui y o 20/ 63.
cy hemia vera, Waldens rom’s macroglobu-
According o he guidelines o he
linemia, plasma cell dyscrasias such as
Cen ral e inal Vein Occlusion S udy, i any
mul iple myeloma)
an erior chamber angle neovasculariza ion
Hyperlipidemias or 2 clock hours or more o iris neovascu-
In amma ory or in ec ious (e.g., sar- lariza ion develops, laser P P should be
coidosis, sys emic lupus ery hema osus, considered o help preven neovascular
syphilis) glaucoma.
FIGURE 4-19. Nonischemic cen ral re inal vein occlusion. No e he re inal hemorrhages in all our quadran s
around he op ic disc. T e visual acui y in he eye was 20/ 50.
B
FIGURE 4-20. Ischemic cen ral re inal occlusion. A. Re inal hemorrhage, di use re inal edema, and
numerous cot on wool spo s are presen . T e visual acui y was hand mo ions. B. Fluorescein angiogram
corresponding o A. Areas o marked re inal capillary nonper usion and macular edema are presen . Some
o he areas o hypo uorescence correspond o re inal hemorrhages.
continued
FIGURE 4-20. Continued Ischemic cen ral re inal occlusion. C. Re inal hemorrhage, re inal venous
or uosi y, di use macular edema, and markedly narrowed re inal ar erioles are presen . D. Fluorescein
angiogram corresponding o C. Delayed re inal venous f lling and re inal elangiec asia are no ed. T ere is
marked hypo uorescence rom re inal ischemia and blockage rom re inal hemorrhages.
continued
F
FIGURE 4-20. Continued Ischemic cen ral re inal occlusion. E. Fluorescein angiogram corresponding o
C. T ere is widespread macular ischemia. F. Op ic disc colla erals shun ing re inal venous blood o he choroidal
circula ion may be no ed on he op ic disc.
e inal edema may be he presen -

RE
E INAL
IN
N AL AR
AR ERIAL
E RIA
AL ing sign when chronic leakage o plasma
MACROAN
MA
ACRR O AN
N EU
EURYSM
U RYSSM encroaches upon he ovea. In his ins ance,
lipid exuda ion is also o en presen .
F irs described in 1973, re inal ar erial mac-

roaneurysm is charac erized by he pres-
ence o vascular dila ion or ou pouching o a
In approxima ely 4% o cases, a re inal
ar erial obs ruc ion dis al o he macroaneu-
rysm is seen a he ime o presen a ion.
re inal ar ery or ar eriole.
EPIDEMIOLOGY
T e abnormali y is rela ively unique in
e inal ar erial macroaneurysms occur
appearance. Aneurysmal abnormali ies asso-
as isola ed phenomena in wo- hirds o he
cia ed wi h Coa s’ disease can occasionally
cases. Abou one- hird o he cases are seen in
cause bleeding seen wi h re inal ar erial mac-
conjunc ion wi h re inal venous obs ruc ions.
roaneurysms, bu wi h Coa s’ disease here
en percen o cases are bila eral. are ypically mul iple aneurysmal ar erial or
venous dila ions, or bo h.
PATHOPHYSIOLOGY T e presence o mul iple, bila eral ar e-
rial aneurysmal abnormali ies occurring
T e aneurysms are believed o be clinically principally a ar erial bi urca ions has been
similar in size (300 µ m) o he in racerebral described wi h he disorder, ermed idio-
varian , al hough no associa ion wi h in ra- pa hic re inal vasculi is, aneurysms, and neu-
cerebral aneurysms has been convincingly rore ini is (I VAN).
demons ra ed.
T ey can occur as saccular dila ions wi hin DIAGNOSTIC EVALUATION
he vessel or as ou pouchings rom he vessel.
Abou hree quar ers o cases are associ- In ravenous uorescein angiography
a ed wi h sys emic ar erial hyper ension. reveals hyper uorescence corresponding o
he macroaneurysm (Fig. 4-21B). Vascular
CLINICAL SIGNS elangiec asias surrounding he aneurysmal
abnormali y may be presen .
T e en i y is ypically unila eral and iso-
la ed. Pulsa ions o he macroaneurysm are PROGNOSIS AND
occasionally seen. T e wo mos common MANAGEMENT
varian s o presen a ion are as ollows:
Acu e hemorrhage may develop in he T e visual prognosis depends on whe her
subre inal space, re inal or prere inal region bleeding involves he cen ral macular region.
i he macroaneurysms rup ures. A mul i- In such ins ances, vision can be reduced
level hemorrhage should arouse suspicion o coun ing ngers or worse. Spon aneous
o he presence o a macroaneurysm. A improvemen can occur, par icularly when
whi e or yellow spo ( he aneurysm) is he blood is loca ed super cially wi hin he
o en presen cen rally wi hin he hemor- re ina. Involvemen o he macula by edema
rhage (Fig. 4-21). ecurren bleeding is can lead o visual loss, ypically ranging rom
ex remely unusual. 20/ 25 o 20/ 200.
Re inal Ar erial Macroaneurysm 173
T e bleeding associa ed wi h macroaneu- he aneurysm and he re ina surrounding he

rysms is no ypically rea ed. I is usually a abnormali y.
one- ime even , and visual improvemen can In approxima ely 16% o cases, rea -
occur when he hemorrhage is loca ed primar- men leads o a re inal ar erial obs ruc ion
ily wi hin he super cial layers o he re ina. dis al o he aneurysm. T us, rea men o
Al hough here are no randomized clini- aneurysms ha could lead o an ar erial
cal s udies addressing he issue, mos re inal obs ruc ion involving he cen ral macula
exper s recommend rea ing he macroan- should be under aken wi h cau ion.
eurysms when here is involvemen o he In a cross-sec ional s udy, 4% o eyes
cen ral ovea by re inal edema or hard exuda- presen ed wi h a branch re inal ar ery
ion, or bo h. rea men is given using 200- o occlusion dis al o he macroaneurysm.
500-µ m spo size, ligh argon laser burns o
B
FIGURE 4-21. Re inal ar erial macroaneur ysm associa ed wi h yellowed blood. A. T e blood superiorly
is superf cial o he re inal vessels prere inal , whereas in eriorly i is loca ed in he subre inal space. T e
yellow macroaneurysm arrow is presen along he course o he re inal ar ery. B. Fluorescein angiogram
corresponding o A. T e aneurysm is hyper uorescen and loca ed along he in ero emporal re inal ar ery.
Para oveal Telangiec asis 175
and highligh ed wi h red- ree pho ography.

PA
PARAFOVEAL
ARAAF OV
VEAAL e inal pigmen epi helial hyperplasia, mos
ELAN
E LA
AN G
GIEC
IEC
C ASIS
AS
SIS
S prominen in he emporal ovea, can be seen
in he la er s ages, as can righ -angle venules
P ara oveal ( jux a oveal) elangiec asis is

a re inal vascular en i y charac erized by
he presence o incompe en re inal capillaries
diving in o he ou er re ina. Yellow in ra-
re inal crys als in he ovea are also seen in
some cases (Fig. 4-22A). Despi e he re inal
in he oveal region o one or bo h eyes. hickening, he re inal cys oid changes o en
seen wi h diabe ic re inopa hy or re inal vein
EPIDEMIOLOGY obs ruc ion are no usually presen wi h para-
AND ETIOLO GY oveal elangiec asis.
In approxima ely 5% o pa ien s, choroidal
Group 1 para oveal elangiec asis has neovasculariza ion can develop in he region
been associa ed wi h an abnormal glucose o he elangiec a ic re inal vessels.
olerance in more han 30% o cases. Group
2 para oveal elangiec asis has been associ-
a ed wi h an abnormal glucose olerance es
in more han 60% o cases. T e incidence o
Diabe ic re inopa hy
para oveal elangiec asis is uncer ain. T ere is
no known heredi ary pat ern. adia ion re inopa hy
Caro id obs ruc ive disease (ocular isch-
PATHOPHYSIOLOGY emic syndrome)
wig re inal vein obs ruc ion
His opa hology has shown hickening o Coa s’ disease
he walls o he re inal capillaries by a deposi- Macular edema associa ed wi h he
ion o basemen membrane. T e changes Irvine–Gass syndrome
are similar o hose seen wi h diabe ic
re inopa hy. Macular edema associa ed wi h uvei is
T e appearance o emporal oveal
leakage wi h uorescein angiography is
CLINICAL SIGNS very help ul or making he diagnosis o
para oveal elangiec asis. In con ras o he
T e en i y is s ra i ed in o hree varian s:
Irvine–Gass syndrome (macular edema a er
Group 1: Unila eral para oveal ca arac surgery) or uvei is associa ed wi h
elangiec asis macular edema, he op ic disc is no usually
Group 2: Bila eral para oveal hyper uorescen in eyes wi h para oveal
elangiec asis elangiec asis.
Group 3: Bila eral occlusive para oveal
elangiec asis alone or associa ed wi h cen- DIAGNOSTIC EVALUATION
ral nervous sys em occlusive vasculi is
T ere is ypically a blun ed oveal re ex T e oph halmoscopic diagnosis is o en
wi h localized re inal hickening mos pro- di cul . In ravenous uorescein angiogra-
nounced in he emporal ovea. A grayish phy is o en required o make he diagnosis.
macular re ex may be observed clinically T ere is charac eris ic in rare inal leakage o
dye loca ed primarily in he emporal macula can decrease drama ically o legal blindness.
(Fig. 4-22B). When abrup loss o vision is presen , he
In he group 3 varian , areas o re inal possibili y o an associa ed choroidal neovas-
capillary dropou can be seen in he oveal cular membrane should be considered.
region. Laser pho ocoagula ion has no been
shown o be o bene or he rea men o
para oveal elangiec asis. Laser herapy may
PROGNOSIS AND be o bene in rea ing he choroidal neovas-
MANAGEMENT culariza ion associa ed wi h para oveal elan-
giec asis. Pa ien s should be made aware o
When pa ien s rs presen , he visual acu- he s rong associa ion be ween an abnormal
i y is o en only mildly decreased o he 20/ 20 glucose olerance es and para oveal elangi-
o 20/ 30 range. Over he years, he vision ec asis, especially he group 2 varian .
A
FIGURE 4-22. Group 2 para oveal elangiec asis. A. T e emporal oveal re ina is hickened, and crys alline
deposi s are presen in his area as well. T e visual acui y in he eye was 20/ 100. Inse highligh s elangiec a ic
changes.
continued
Para oveal Telangiec asis 177
C
FIGURE 4-22. Continued Group 2 para oveal elangiec asis. B. Early phase uorescein angiogram
corresponding o A. elangiec a ic re inal vascular changes are presen surrounding he oveal avascular zone.
C. La e phase uorescein angiogram corresponding o A. In rare inal leakage o dye is presen in he vicini y o
he elangiec a ic changes in he emporal ovea.
epi helial cells ha are believed o develop

SICKLE
S I C KLEECCELL
E LL rom a salmon pa ch hemorrhage ha has
RE
R E INI N O PA
PA H Y dissec ed in o he subre inal space or rom a
ocal choroidal occlusion.
S ickle cell re inopa hy describes he un-

dus changes associa ed wi h sickle cell
hemoglobinopa hies.
Proliferative Changes
Five s ages have been described.
S age I: Peripheral re inal ar eriolar
occlusions
EPIDEMIOLOGY
S age II: Peripheral re inal ar eriove-
Approxima ely 10% o he U.S. popula ion nous anas omoses
have any orm o sickle hemoglobin; 0.4% S age III: Peripheral re inal neovascu-
have hemoglobin SS, 0.2% have hemoglobin lariza ion (“sea ans”) (Fig. 4-24A)
SC, and 0.03% have sickle cell halassemia S age IV: Vi reous hemorrhage
(ST al).
S age V: hegma ogenous or rac ion
Proli era ive sickle re inopa hy has been re inal de achmen , or bo h
no ed o occur in Jamaican individuals wi h
sickle hemoglobinopa hy in he ollowing per-
cen ages: SS, 3%; SC, 33%; and ST al, 14%. DIFFERENTIAL DIAGNOSIS
Eales’ disease
PATHOPHYSIOLOGY Proli era ive diabe ic re inopa hy
Sickled red blood cells cause obs ruc ion adia ion re inopa hy
wi hin he re inal vascula ure. Mul iple hemo- e inal vein occlusion
globin varian s have been described, along Sarcoidosis
wi h heir gene ic changes. Al hough SS dis-
ease is associa ed wi h more severe sys emic
disease, SC disease causes more advanced
ocular disease.
A his ory o sickle cell disease may be elic-
i ed, and hus a sickle cell prep or hemoglobin
CLINICAL SIGNS elec rophoresis should be considered when
charac eris ic ndings are no ed.
Nonproliferative Manifestations T e disease is diagnosed by i s clinical
Salmon pa ch hemorrhage: An oval- appearance. In ravenous uorescein angiog-
shaped area o in rare inal or prere inal blood raphy reveals re inal capillary nonper usion
believed o occur secondary o an obs ruc ed adjacen and peripheral o areas o peripheral
re inal ar eriole, which subsequen ly rup ures. re inal neovasculariza ion (Fig. 4-24).
Iridescen spo : A small re inoschisis cav-
i y wi hin he super cial re ina ha can occur PROGNOSIS AND
as a salmon pa ch resolves. I is lled wi h MANAGEMENT
hemosiderin-laden macrophages.
Black sunburs lesion (Fig. 4-23): An T e visual prognosis is o en rela ively
oval or round collec ion o re inal pigmen good unless he sequelae o proli era ive
sickle disease (vi reous hemorrhage or re inal reduce he incidence o subsequen vi reous
de achmen , or bo h) develop. hemorrhage. Many have advoca ed rea ing
rea men is no indica ed or he he peripheral re ina or 360 degrees.
nonproli era ive changes o sickling hemo- Para plana vi rec omy can be o ben-
globinopa hies. When peripheral re inal e or chronic vi reous hemorrhage.
neovasculariza ion is presen , ull scat er laser Vi rec omy, wi h or wi hou scleral buckling,
pho ocoagula ion o he re ina peripheral o may be o bene or he repair o re inal
he neovasculariza ion has been shown o de achmen .
FIGURE 4-23. Sickle cell re inopa hy, “black sunburs lesion.” Small, black sunburs lesion inse in an eye
wi h sickle cell re inopa hy. T ere is a sclero ic re inal vessel leading o ischemic peripheral re ina in eriorly.
B
FIGURE 4-24. S age 3 proli era ive sickle cell re inopa hy. A. Orange “sea ans,” or areas o peripheral
re inal neovasculariza ion, are presen a he junc ure o per used and ischemic peripheral re ina. B. Fluorescein
angiogram corresponding o A a 29 seconds a er injec ion. T e sea ans are hyper uorescen , and re inal
capillary nonper usion is visible adjacen o hem on he le side o he pho o.
continued
C
FIGURE 4-24. Continued S age 3 proli era ive sickle cell re inopa hy. C. La e phase uorescein angiogram
showing marked leakage o uorescein dye in o he vi reous rom he re inal sea an neovasculariza ion.
T e re inal changes (Fig. 4-25) are charac-

RADIA
R AD
D I A IOON erized by he presence o cot on-wool spo s,
RE
R E IN
I N O PA
PA H Y re inal hemorrhages, and hard exuda ion.
Neovasculariza ion o he op ic disc or re ina,
R adia ion re inopa hy re ers o damage

induced o he re ina or op ic nerve, or
bo h, by ex ernal beam irradia ion ( ele her-
or bo h, can develop as well. Op ic nerve
changes are charac erized by disc edema, a
imes in conjunc ion wi h peripapillary sub-
apy) or by localized irradia ion (brachy her- re inal uid and lipid exuda ion (Fig. 4-26).
apy). T e op ic nerve changes are re erred o e inopa hy and op ic neuropa hy can occur
as radia ion op ic neuropa hy. concomi an ly or separa ely.
T e undus ndings wi h brachy herapy
EPIDEMIOLOGY are similar o hose wi h ele herapy
AND ETIOLO GY (Fig. 4-27), al hough hard exuda ion
ends o be a more prominen ea ure
Damage rom ex ernal beam irradia ion wi h brachy herapy (Fig. 4-28).
o en occurs rom irradia ion o s ruc ures
adjacen o he eyes (brain, oropharynx, e c.).
ypically, a minimum dose o 1500 cGy (cen- DIFFERENTIAL DIAGNOSIS
igray) o ex ernal beam irradia ion is required
o induce re inopa hy. T e mean dose is abou adia ion re inopa hy can closely mimic
5000 cGy. A a dose o 7000 o 8000 cGy, diabe ic re inopa hy. T ere are usually more
85% o eyes will develop radia ion re inopa- microaneurysms presen wi h diabe ic re i-
hy. Frac ion sizes o more han 200 per day nopa hy han wi h radia ion re inopa hy. Key
appear o ampli y he chances o radia ion o he diagnosis o radia ion is he elici a ion
damage. For 60Co brachy herapy, a minimum o a his ory o radia ion o or around he
o 20,000 cGy o he umor base is necessary eyes.
o induce radia ion changes, wi h he average
dose over 30,000 cGy. DIAGNOSTIC EVALUATION
PATHOPHYSIOLOGY In ravenous uorescein angiography
reveals re inal capillary dropou , re inal vascu-
adia ion re inopa hy and op ic neuropa- lar elangiec ases, and la e leakage o dye rom
hy are caused primarily by damage o he he damaged vessels.
vascula ure o he respec ive s ruc ures.
CLINICAL SIGNS PROGNOSIS

AND MANAGEMENT
adia ion changes usually occur a a mean
ime o 12 o 18 mon hs a er he ermina- T e visual prognosis depends on he dose
ion o radia ion, wi h a range rom 1 mon h o radia ion received. T e presence o che-
o 7 years. T e condi ion can be unila eral or mo herapy, sys emic ar erial hyper ension,
bila eral, depending on he elds included in diabe ic re inopa hy, and o her diseases ha
he irradia ion. can damage he re inal vascula ure appear o
be addi ive o he radia ion damage.
Visual acui y is variable, depending on he
degree o damage o he re inal or op ic nerve Wi h brachy herapy or choroidal mela-
vascula ure. noma, abou wo- hirds o he eyes have
20/ 200 vision or bet er a 2 o 3 years a er rea men o clinically signi can macular
rea men . edema in he Early rea men Diabe ic
Approxima ely 25% un rea ed eyes wi h e inopa hy S udy. Laser P P is indica ed
radia ion re inopa hy rom ex ernal beam when neovasculariza ion o he iris or pos e-
irradia ion progress o develop iris neo- rior segmen neovasculariza ion develops.
vasculariza ion o he iris and neovascular T ere is no known ef ec ive rea men or
glaucoma. radia ion op ic neuropa hy, bu spon aneous
Macular edema can be rea ed wi h ocal improvemen o vision occurs in abou 20%
laser herapy in a ashion similar o he o cases.
B
FIGURE 4-25. Radia ion re inopa hy a er ele herapy. A. Cot on wool spo s and small re inal hemorrhage
are presen in he pos erior pole. B. Fluorescein angiogram corresponding o A a 25 seconds a er injec ion. T e
cot on wool spo s are hypo uorescen . No e he radia ion induced capillary elangiec asia a he ovea and below
he in erior re inal vascular arcade where capillary nonper usion is no ed.
continued
C
FIGURE 4-25. Continued Radia ion re inopa hy a er ele herapy. C. Fluorescein angiogram corresponding
o A a 375 seconds a er injec ions. T e cot on wool spo s are now more hyper uorescen due o leakage o dye
rom he re ina a heir border.
FIGURE 4-26. Radia ion op ic neuropa hy a er ele herapy. T e op ic disc is swollen and surrounded by
peripapillary lipid exuda es and subre inal uid.
FIGURE 4-27. Radia ion re inopa hy ollowing brachy herapy or a choroidal melanoma. Cot on wool
spo s are presen in he peripapillary region.
FIGURE 4-28. Radia ion re inopa hy surrounding a choroidal melanoma rea ed wi h brachy herapy.
Marked lipid exuda ion is presen a he necro ic umor base.
Lipemia Re inalis 187
sys ems and ypically have skip segmen s

LIPEMIA
L IPE
E MII A R
REE IINALIS
NA
AL IS
S where he re inal vascula ure is unaf ec ed.
L ipemia re inalis re ers o salmon-colored Lipemia re inalis shows dif use re inal
re inal ar eries and re inal veins ar erial and venous involvemen .
due o eleva ed lipids, mos commonly
hyper riglyceridemia. DIAGNOSTIC EVALUATION
PATHOPHYSIOLOGY Fundus biomicroscopic examina ion

reveals he charac eris ic re inal appear-
Eleva ed serum lipid levels may cause ance described earlier and usually su ces o
re inal vascular obs ruc ion. T e lipid abnor- promp sys emic lipid evalua ion.
mali ies are ypically heri able lipid me abolic Fluorescein angiography may be ob ained
abnormali ies. o evalua e re inal per usion; areas o re inal
elangiec asia may be observed.
CLINICAL SIGNS Family members should be evalua ed, and
sys emic evalua ion is indica ed because o
Pale conjunc iva may resul rom lipemic po en ial problems such as pancrea i is.
conjunc ival blood vessels. Fundus exami-
na ion reveals salmon-colored re inal ar er-
ies and veins wi hou skip areas (Fig. 4-29). PROGNOSIS AND
MANAGEMENT
T e visual prognosis can be good or
e inal vascular whi ening can be pa ien s who do no develop re inal vascular
observed in prior re inal vascular occlusion occlusive disease.
or in re inal vasculi is. T ese are ypically rea men is direc ed oward decreasing
con ned o he re inal ar erial or venous he causa ive lipemic ac or.
B
FIGURE 4-29. Lipemia re inalis. A. A pa ien wi h markedly eleva ed riglyceride lipid levels who presen ed
wi h a branch re inal vein occlusion and lipemic yellow re inal vessels and microaneurysms. B. A er rea men
wi h oral lipid lowering agen s, he re inal vascula ure assumes normal coloring.
C H AP T ER
Re inal Degenera ions

and Dys rophies
Mithlesh C. Sharma and Allen C. Ho
BES
B E S ’S
SDDISEASE
I E
IS EA
ASE
E CLINICAL SIGNS
B es ’s disease, also known as vi elli orm

macular dys rophy, is a macular dys ro-
phy ha is clinically charac erized by an egg
Five s ages are delinea ed.
1. Previ elli orm s age: Pa ien s are
asymp oma ic wi h no undus abnor-
yolk-like lesion a he level o he re inal pig- mali y, bu elec rooculography shows a
men epi helium (RPE), usually loca ed in he reduced ligh -peak o dark- rough ra io.
pos erior pole. T e disease progresses hrough
2. Vi elli orm s age: T is s age is char-
various s ages o culmina e in macular a rophy
ac erized by an egg yolk-like lesion in
or scarring wi h loss o cen ral vision.
he macular area. I is usually de ec ed
during he f rs or second decade o li e.
Al hough usually single and bila erally
EPIDEMIOLOGY symme ric, mul iple egg yolk lesions
AND ETIOLO GY may be observed in he pos erior pole.
T e egg yolk lesions are loca ed a he
Symp oms develop in in ancy or early level o he RPE, are rounded or oval in
childhood. shape wi h dis inc borders, and range
An excessive amoun o lipo uscin-like rom one-hal o wo disc diame ers in
ma erial wi hin he re inal pigmen epi helial size. Vision may be normal or sligh ly
cells, par icularly in he ovea, is observed. decreased a his s age (Fig. 5-1).
T ere appears o be a secondary loss o he 3. Pseudohypopyon s age: By he second
pho orecep or cells. or hird decade o li e, lesions break
I is an au osomal-dominan disease whose hrough he RPE, and he yellow ma e-
gene is mapped o chromosome 11q13. rial accumula es in eriorly in he macula
189
190 5 RETINAL DEGENERATIO NS AND DYSTRO PHIES
wi hin he subre inal space o orm Color vision: Mild dyschroma opsia may
pseudohypopyon (Fig. 5-2). be no iced.
4. Vi ellirup ive s age: T e egg yolk breaks Dark adap ome ry is normal.
up o produce a scrambled egg appear- Elec rore inography is normal.
ance. Pa ien s usually no ice some visual
Elec rooculography: Bes ’s disease is one
impairmen a his s age (Fig. 5-3).
o he ew condi ions ha resul s in an abnor-
5. End s age: Subre inal f brosis, or a vas- mal elec rooculogram (EOG) in he set ing o
cularized scar wi h choroidal neovascu- a normal elec rore inogram (ERG). During
lariza ion, con ribu es o he visual loss an EOG, he ligh -peak:dark- rough ra io
a his s age. (Arden ra io) is ypically below 1.5.
T e vi elli orm degenera ion presen ing Fluorescein angiography: In he vi elli orm
a er childhood is called adul Bes ’s disease. s age, comple e blockage o background cho-
In he lat er varian , he yellow oveal deposi s roidal uorescence by he lesion is observed.
are symme ric and similar o childhood Bes ’s Areas o hyper uorescence due o a rophic
disease excep ha he lesions are smaller and RPE are no iced as he egg yolk lesions show
have a cen ral pigmen ed spo . T e mos com- disrup ion.
mon lesion mis aken or Bes ’s disease is a yel-
low premacular hemorrhage (Fig. 5-4).
PROGNOSIS AND
DIAGNOSTIC EVALUATION MANAGEMENT
Visual acui y: Vision remains good while In general, he overall prognosis is good as
he egg yolk lesions are in ac . Disrup ion or mos pa ien s re ain reading level o vision in
scarring o he lesions may reduce visual acu- a leas one eye hroughou li e.
i y o he level o 20/ 200. When severe vision loss does ake place
Visual f elds: Cen ral visual f elds are in an eye, i occurs slowly and usually begins
normal ini ially, bu a rela ive sco oma may a er he age o 40 years.
develop wi h ime. No rea men is available or Bes ’s disease.
B
FIGURE 5-1. Bes ’s disease, vi elliform s age. A. Charac eris ic egg yolk like lesion in he ovea.
B. Corresponding f uorescein angiogram showing blocked f uorescence due o egg yolk lesion during ransi
phase. (Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia, Pennsylvania, compiled by
Dr. amara Vrabec and Dr. Gordon Byrnes.)
B
FIGURE 5-2. Bes ’s disease, pseudohypopyon s age. A. Collec ion o yellow ma erial wi hin he subre inal
space simula ing hypopyon ( arrow) . B. Blocked f uorescence (arrow) due o deposi ion o yellow ma erial
in eriorly and peri oveal hyperf uorescence in he cen er o he lesion is seen in f uorescein angiogram
pho ographs. Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia, Pennsylvania, compiled
by Dr. amara Vrabec and Dr. Gordon Byrnes.)
B
FIGURE 5-3. Bes ’s disease, vi ellirup ive s age. A. Irregular areas o re inal pigmen epi helial loss secondary
o breakup o he egg yolk lesion ( arrow) . B. In ense peri oveal hyperf uorescence surrounded by mul iple areas
o hyperf uorescence is shown in he corresponding f uorescein angiogram. (Cour esy o Re ina Slide Collec ion,
Wills Eye Hospi al, Philadelphia, Pennsylvania, compiled by Dr. amara Vrabec and Dr. Gordon Byrnes.)
B
FIGURE 5-4. Pseudo–Bes ’s disease. A. Old premacular hemorrhage simula ing he egg yolk lesion o Bes ’s
disease. No e ha he re inal vessels are obscured by he prere inal lesion ( arrow) . Sligh ly irregular borders
o he lesion and presence o neighboring re inal hemorrhages are clues o he yellow lesion being an old
hemorrhage. B. Fluorescein angiogram demons ra ing microaneurysms and re inal elangiec asia emporal
o he lesion, consis en wi h diabe ic re inopa hy and a premacular hemorrhage.
ConeDys rophy 195
CO
O N E DYS
D YS RO
R O PH Y DIFFERENTIAL DIAGNOSIS
Reduced vision wi h normal undus in chil-
C one dys rophy is an inheri ed de ec ha
primarily a ec s he cone pho orecep or
sys em.
dren: In his ca egory, cone dys rophy should
be di eren ia ed rom S argard ’s disease.
“Bull’s-eye” maculopa hy: T e ollowing
causes o “bull’s-eye” maculopa hy need o be
EPIDEMIOLOGY considered in he di eren ial diagnosis:
AND ETIOLO GY S argard ’s disease
Chloroquine oxici y
Symp oms begin in early childhood o Bat en’s disease
middle adul hood.
Benign concen ric annular macular
Cone dys rophy is primarily au osomal dys rophy
dominan , al hough au osomal-recessive and Leber’s congeni al amaurosis
X-linked orms have also been repor ed.
HISTORY
Visual f elds: Cen ral sco oma is usually seen.
T e ra e o progression and he sever- Color vision: Reduced.
i y o signs and symp oms are variable. T e Dark adap ome ry: T e cone componen
symp oms consis o progressive visual loss, o he dark adap a ion curve is abnormal.
hemeralopia (decreased vision in brigh ly illu-
mina ed environmen ), color vision di cul- Elec rore inography: T e single- ash
ies, and cen ral visual f eld de ec s. pho opic ERG and he pho opic icker ERG
are low or unrecordable. T e sco opic ERG is
Macular changes ypically ollow he visual usually normal.
dis urbances; here ore, early in he disease
process he undus may appear en irely Fluorescein angiography: T e re inal pig-
normal. men epi helial changes in he macular area
may be visible by uorescein angiography
be ore hey can be visualized clinically. Early
CLINICAL SIGNS in he disease process, a mot led hyper uo-
rescence is seen. As he “bull’s-eye” pat ern
T ere is gradual, ypically symme ric o re inal pigmen epi helial loss develops,
loss o visual acui y o he level o 20/ 200. hyper uorescence surrounding a cen ral area
Occasionally, his may be reduced o he level o hypo uorescence is observed.
o coun ing f ngers o hand mo ion acui y.
Fundus changes are variable (Fig. 5-5). PROGNOSIS AND
Early in he disease process, pigmen ary MANAGEMENT
s ippling wi h di use pigmen granulari y
in he pos erior pole is he mos common T e visual loss is gradual and symme ric o
abnormali y observed. T e classic “bull’s-eye” he level o 20/ 200. However, i may occasion-
pat ern o re inal pigmen epi helial a rophy is ally be severe enough o cause a visual acui y o
a la e f nding. In advanced cases, a round, dis- coun ing f ngers o hand mo ion. T e visual loss
cre e area o cen ral a rophy is seen. emporal is more severe in early-onse cases.
pallor o he op ic disc may be observed. No rea men is available or cone dys rophy.
B
FIGURE 5-5. Cone dys rophy. A. Early “bull’s eye” maculopa hy in a pa ien wi h cone dys rophy (inse ) .
No e he emporal op ic disc a rophy ( arrow) . B. Corresponding f uorescein angiogram showing cen ral
hypof uorescence surrounded by a ring o hyperf uorescence seen during he ransi phase ( box) .
(continued)
Cone Dys rophy 197
D
FIGURE 5-5. ( Continued) Cone dys rophy. C. T e hyperf uorescence ades away in he la er rame indica ing
he presence o window de ec s due o re inal pigmen epi helial a rophy. D. Advanced cone dys rophy wi h a
classic “bull’s eye” maculopa hy. No e he emporal op ic disc pallor. (Cour esy o Dr. Joseph Maguire and he
Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia, Pennsylvania, compiled by Dr. amara Vrabec and
Dr. Gordon Byrnes.)
macular region. Arim o re inal pigmen

PA ER
ERN
RN D
DYS
YS RO
O PH
PH Y epi helial a rophy around he pigmen f gure,
which is more apparen on uorescein angiog-
P at ern dys rophy describes a group o
rela ed condi ions ha are inheri ed in
an au osomal-dominan ashion and are clini-
raphy, may also be seen (Figs. 5-6 and 5-7).
A single, round, vi elli orm lesion in
cally charac erized by variably shaped yellow he ovea (adul -onse oveomacular vi el-
or gray deposi s in he macula. li orm dys rophy).
Ex ensive macular f shne arrangemen
(re icular dys rophy).
EPIDEMIOLOGY
Coarse pigmen mot ling o he macula
AND ETIOLO GY ( undus pulverulen us) (Fig. 5-8).
Symp oms begin in middle age. T e a ec ed members o a given pedigree
may have di eren pat erns, and he pat erns
T e disease has an au osomal-dominan may di er in he eyes o an a ec ed indi-
pat ern. Gene ic analysis o pa ien s wi h bu - vidual. Even a change rom one o ano her
er y dys rophy has shown mu a ion in he pat ern over ime may be observed.
peripherin/ RDS gene, loca ed on he shor
arm o chromosome 6. T e peripherin gene
produc plays an impor an role in he s ruc- DIFFERENTIAL DIAGNOSIS
ural in egri y o pho orecep or ou er seg-
men discs. However, his mu a ion does no Large drusen: Oph halmoscopically, he
correspond o a par icular pheno ype. T a is, yellow pigmen f gures o pat ern dys rophy
o her orms o re inal degenera ion have been may be con used wi h he large drusen o age-
linked o peripherin/ RDS gene mu a ions. rela ed macular degenera ion.
HISTORY DIAGNOSTIC EVALUATION
Mos pa ien s are ei her asymp oma ic or Visual f elds: Normal, excep or minimally
have minimal visual dis urbances. ypically, reduced sensi ivi y in he macular area.
he diagnosis is made on rou ine undus Color vision, dark adap ome ry, elec ro-
examina ion o a middle-aged adul . re inography: Normal.
Elec rooculography: Mildly abnormal,
CLINICAL SIGNS which is consis en wi h he dis urbed re inal
pigmen epi helial unc ion.
Visual acui y: Pa ien s may have normal Fluorescein angiography: Pigmen f gures
visual acui y up o he f h or six h decade are hypo uorescen hroughou he s udy.
o li e. Reduced vision and me amorphopsia T e re inal pigmen epi helial a rophy around
may be he presen ing symp oms. he lesions produces hyper uorescence.
Oph halmoscopically: T e ollowing pa -
erns o pigmen deposi s in he macular area PROGNOSIS
may be observed:
Mos commonly, a bila eral, riradia e T e prognosis or re en ion o good cen-
(“but er y”) pat ern o yellow or gray pig- ral vision in a leas one eye hroughou li e
men a he level o he RPE in he cen ral is excellen .
B
FIGURE 5-6. Pat ern dys rophy. A and B. Bila eral, mul iple, discre e, yellow areas a he level o he re inal
pigmen epi helium (RPE) in he cen ral macular region (inse ) .
( continued)
C
FIGURE 5-6. ( Continued) Pat ern dys rophy. C. T e lesions are more pronounced in he corresponding
f uorescein angiogram image o he righ eye, where here is pa chy hyperf uorescence.
B
FIGURE 5-7. Pat ern dys rophy. A. Classic riradia e ( “but erf y”) pat ern o pigmen deposi s a he ovea
(inse ) surrounded by mul iple areas o re inal pigmen epi helial a rophy. B. Fluorescein angiogram showing
pa chy hyperf uorescence in he ar eriovenous phase. T e hyperf uorescen areas correspond o re inal pigmen
epi helial a rophy in he macular region. (Cour esy o Dr. Eric Shakin and he Re ina Slide Collec ion, Wills Eye
Hospi al, Philadelphia, Pennsylvania, compiled by Dr. amara Vrabec and Dr. Gordon Byrnes.)
B
FIGURE 5-8. Pat ern dys rophy, fundus pulverulen us. Fluorescein angiogram pho ographs o bo h eyes
showing radia ing pat ern o hypof uorescence due o coarse pigmen deposi s a he level o RPE—re icular
dys rophy. (Cour esy o Dr. William Annesley, and he Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia,
Pennsylvania, compiled by Dr. amara Vrabec and Dr. Gordon Byrnes.)
he la er s age o disease. Loss o he oveal

S ARGARD ’S DISEASE re ex may be he only ini ial clinical f nding.
Discre e, yellowish, “pisci orm” ecks loca ed
S argard ’s disease is a macular dys rophy
ha is charac erized by he presence o dis-
cre e, yellow, pisci orm ecks a he level o he
a he level o he RPE are o en no iced a
some poin in he course o disease. T e
ecks may or may no involve he macula
RPE. Curren ly, S argard ’s disease and undus (Fig. 5-9). Peri oveal re inal pigmen epi he-
avimacula us are regarded as varian s o he lial mot ling may become eviden wi h he
same disorder. T e erm undus f avimaculatus is progression o disease.
generally applied when he charac eris ic ecks
are scat ered hroughou he undus. When he A “bull’s-eye” pat ern o re inal pigmen
ecks are conf ned o he pos erior pole and are epi helial loss may become apparen , par-
associa ed wi h macular a rophy, he condi ion icularly by uorescein angiography. T e
is described as S argard ’s disease. macula classically develops a “bea en
bronze” appearance corresponding o a ro-
phy o he cen ral RPE in he advanced
EPIDEMIOLOGY s age o disease (Figs. 5-10 and 5-11).
His opa hology shows an accumula ion o
AND ETIOLO GY
an abnormal lipo uscin-like ma erial in he
RPE (Fig. 5-12).
T e disease usually presen s in he f rs or
second decade o li e.
Bo h sexes are a ec ed equally.
S argard ’s disease is usually au osomal
recessive, al hough dominan ly inheri ed cases Cone dys rophy: reduced vision and nor-
have been described. T e gene or au osomal- mal undus in a child
recessive S argard ’s disease is loca ed on
“Bull’s-eye” maculopa hy: chloroquine
chromosome 1. T is gene codes or an A P-
oxici y, Bat en’s disease, benign concen ric
binding ranspor pro ein (ABCR) ha is
annular macular dys rophy
expressed in he rod inner segmen s, bu no
he RPE. A homozygous mu a ion in he
ABCR gene causes undus avimacula us.
HISTORY
Visual f elds: Usually a cen ral sco oma
Children wi h S argard ’s disease are usu- is no ed, bu a paracen ral sco oma, cen ral
ally brough o he at en ion o an oph hal- cons ric ion, and a ring sco oma may also be
mologis as a resul o a gradual impairmen seen, especially early in he disease.
o vision no iced by he paren s or a er ailing Color vision: Mild dyschroma opsia o red
a school vision screening. and green may be no ed.
Dark adap ome ry: Dark adap a ion may
CLINICAL SIGNS be delayed.
Fluorescein angiography: Fea ures ha
Visual acui y is sligh ly a ec ed in he may help conf rm he diagnosis o S argard ’s
beginning bu may be severely reduced in disease include dark or silen choroid;
mul iple, irregular hyper uorescen spo s ha PROGNOSIS AND

do no precisely correspond o he ecks; and MANAGEMENT
a “bull’s-eye” window-de ec pat ern o hyper-
uorescence in he macula. T e majori y o pa ien s preserve moder-
Elec rore inography: Usually normal bu a e visual acui y (20/ 70 o 20/ 200), a leas
may be reduced wi h increasing amoun s o in one eye.
peripheral ecks and a rophy. No e ec ive rea men is available or
Elec rooculography: Usually subnormal. S argard ’s disease.
B
FIGURE 5-9. S argard ’s disease. A. Mul iple, discre e, yellow, “pisci orm” f ecks (inse shows one f eck)
loca ed a he level o he RPE wi h corresponding hyperf uorescen areas in he f uorescein angiogram
pho ograph are dis ribu ed hroughou he pos erior pole o he le eye. B. T e background choroid is dark on
he f uorescein angiogram pho ograph, and here is ransmission hyperf uorescence associa ed wi h macular
f ecks and re inal pigmen epi helial al era ions. (Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al,
Philadelphia, Pennsylvania, compiled by Dr. amara Vrabec and Dr. Gordon Byrnes.)
B
FIGURE 5-10. S argard ’s disease. A. Advanced S argard ’s disease wi h “bea en bronze” macula.
B. Corresponding f uorescein angiogram showing a cen ral area o hypof uorescence (re inal pigmen epi helial
clumping) surrounded by a ring o hyperf uorescence (re inal pigmen epi helial a rophy) . No e he dark or
silen choroid ( blocked f uorescence) .
( continued)
C
FIGURE 5-10. ( Continued) S argard ’s disease. C. S argard ’s disease wi h “bull’s eye” macula. Compare wi h
Figure 5-5D. No e he “bea en bronze” appearance o he macula (inse ) . (Cour esy o Dr. Eric Shakin and he
Dr. Gordon Byrnes.)
B
FIGURE 5-11. S argard ’s disease. A. Severe loss o RPE in a geographic ashion in he cen ral macular region
in a pa ien wi h advanced S argard ’s disease. T e visual acui y was reduced o 20/ 200. B. Corresponding
f uorescein angiogram showing irregularly dis ribu ed areas o hypof uorescence and hyperf uorescence, wi h a
discre e rim o hyperf uorescence wi hin he area o geographic pat ern o re inal pigmen epi helial loss. Dark
choroid is apparen beyond he macula.
FIGURE 5-12. S argard ’s disease, elec ron micropho ograph. Elec ron micropho ograph showing enlarged
re inal pigmen epi helial cells due o in racellular accumula ion o lipo uscin like ma erial. (Cour esy o
Dr. Ralph Eagle, Wills Eye Hospi al, Philadelphia, Pennsylvania.)
he di use yellow–whi e re ex o he underly-

CH
H O RO
R O IDEREMIA
ID
D ER
R E M IA
A ing sclera.
C horoideremia is a generalized heredi ary Female carriers show unique and pa hog-
re inal degenera ion ha primarily a ec s nomonic undus ea ures. T e generalized
he choriocapillaris and he RPE–pho orecep or re inal pigmen epi helial mot ling, espe-
complex. cially in he midperiphery, resembles he
changes seen in males in he early s age o
disease. T e undus changes in emale car-
EPIDEMIOLOGY riers remain s a ionary, and he uorescein
AND ETIOLO GY angiography reveals an in ac choroidal
vascula ure.
Symp oms are usually no ed in he f rs or
second decade o li e. DIFFERENTIAL DIAGNOSIS
Only males are a ec ed, and emales are
carriers. Re ini is pigmen osa: Unlike re ini is
pigmen osa (RP), in choroideremia a “bone-
Choroideremia is an X-linked recessive
spicule” pat ern o pigmen ary change is
disorder.
usually no seen, and he re inal blood vessels
remain rela ively normal.
HISTORY Gyra e a rophy: In he f nal s ages, undus
appearance in choroideremia may resemble
Pa ien s usually presen in he f rs or sec- gyra e a rophy, bu he di eren mode o
ond decade o li e wi h a chie complain o gene ic ransmission is an impor an dis in-
di cul y wi h nigh vision. guishing ea ure.
CLINICAL SIGNS
Early in he disease process, undus
appearance in he a ec ed males is a “sal -and- Visual Fields: T ere is a loss o peripheral
pepper” re inal pigmen epi helial mot ling visual f eld.
a he equa or and he pos erior pole. Below Elec rore inography: T is may be normal
he re inal pigmen epi helial mot ling, he during he early s age, bu by he end o he
underlying choroid may appear clinically nor- f rs decade he sco opic ERG becomes non-
mal, bu uorescein angiography may show a recordable and he pho opic ERG is severely
pa chy loss o choroidal vascula ure. reduced.
La er in he disease process, small areas o Fluorescein angiography: In he early
he RPE drop ou in he midperiphery. T ese s age, despi e he clinically normal-appearing
areas o drop-ou even ually coalesce and choroid, uorescein angiography may reveal
progress cen rally. T e macula is involved las a pa chy loss o choriocapillaris. La er in he
(Fig. 5-13). In he f nal s ages, he en ire un- disease, an ex ensive loss o choroidal vascula-
dus, wi h an excep ion o he macula, shows ure is observed (Figs. 5-14 and 5-15).
Choroideremia 211
PROGNOSIS AND pa ien s usually have severely reduced vision

MANAGEMENT by he age o 35 years.
Female carriers usually re ain normal
Rela ively good cen ral vision is preserved visual unc ion hroughou heir li e.
un il la e in he disease because he macula is No rea men is available or
a ec ed la e. Al hough he ra e o progression choroideremia.
may vary wi hin a pedigree, he majori y o
B
FIGURE 5-13. Choroideremia. Mul iple areas o re inal pigmen epi helial loss, some o which have coalesced
o orm larger pa ches. No e ha he ovea is spared and he pa ien had 20/ 40 vision in his eye. (Cour esy o
Dr. Gordon Byrnes.)
Choroideremia 213
B
FIGURE 5-14. Early choroideremia. Peripapillary loss o he RPE (A) and sub le pigmen ary mot ling in
he midperipheral undus (B) can be seen (inse ). Similar undus ndings can be observed in emale carriers o
choroideremia. (Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia, Pennsylvania, compiled
by Dr. amara Vrabec and Dr. Gordon Byrnes.)
B
FIGURE 5-15. La e choroideremia. A. Ex ensive loss o choriocapillaris is eviden in he corresponding la e
phase f uorescein angiogram pho ograph (same pa ien as in Figure 5-13A) . B. Equa or plus red ree undus
pho ograph showing mul iple pa ches o he re inal pigmen epi helial loss ex ending rom he pos erior pole o
he midperiphery (same pa ien as in Figure 5-13B) . (Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al,
Philadelphia, Pennsylvania, compiled by Dr. amara Vrabec and Dr. Gordon Byrnes.)
Gyra e A rophy 215
normal early in he disease, as ime passes

GYRA
GY
YRA
A EA R
ROO PH Y blood vessels show a gradual narrowing.
G yra e a rophy is a rare choroidal disor- In he la er s ages, marked choroidal a ro-

der, which is usually ransmit ed in an phy rom he periphery o he pos erior pole
au osomal-recessive ashion and is caused by is eviden . However, he macula is usually
an absence or near-absence o he pyridoxine- spared. Macular involvemen may be in he
dependen mi ochondrial ma rix enzyme orm o macular edema or rela ed o progres-
orni hine amino rans erase. sion o a rophic changes.
EPIDEMIOLOGY ASSOCIATED CLINICAL SIGNS

AND ETIOLO GY
Ocular
Symp oms begin in he f rs decade o li e. Myopia: Almos invariably presen
T e disorder is au osomal recessive. Ca arac : Seen in a high propor ion o
pa ien s wi h gyra e a rophy
Eleva ed levels o orni hine in he plasma
and urine in pa ien s wi h gyra e a rophy are Sys emic
caused by an absence or near-absence o he Charac eris ically hin, sparse, s raigh
pyridoxine-dependen mi ochondrial ma rix hair
enzyme orni hine amino rans erase. T e la - Less consis en f ndings: Abnormal
er is necessary or he breakdown o excess elec roencephalogram, muscle weakness,
orni hine in humans. abnormal elec rocardiogram, and seizures
HISTORY
T e usual presen ing symp oms are poor
nigh vision and cons ric ed visual f elds. Choroideremia: In la e s ages, undus
appearance o choroideremia and gyra e
a rophy can be remarkably similar. However,
CLINICAL SIGNS di eren modes o gene ic ransmission and
he dis inc ive undus ea ures in he emale
Early in he disease, a hinning and rans- carriers o choroideremia are impor an dis-
parency o he RPE, beginning in he midpe- inguishing ea ures.
riphery, is observed. T e underlying choroid
may appear ei her normal or sclero ic. T e
a ec ed areas are separa ed rom he normal- DIAGNOSTIC EVALUATION
appearing re ina by scalloped borders. T e
involved areas begin as isola ed pa ches bu Visual f elds: Cons ric ion o he periph-
la er coalesce (Fig. 5-16). eral visual f elds corresponds o he expansion
o he undus changes.
Progression o he disease is accompanied
by pigmen clumping and choroidal a rophy. Fluorescein angiography: A loss o chorio-
Even ually he en ire choroidal vascula ure capillaris is demons ra ed in he a ec ed areas.
disappears, exposing he whi e sclera. T e Elec rore inography: Severely diminished
op ic disc and he re inal vessels may be or abolished ampli udes.
Elec rooculography: Abnormal. Pyridoxine (vi amin B6) may help nor-
O her: Eleva ed serum orni hine levels and malize he plasma and urinary orni hine
markedly decreased orni hine amino rans erase levels and preserve visual unc ion. On he
ac ivi y in cul ured f broblas s or leukocy es. basis o a response o vi amin B6, gyra e
a rophy may have wo clinically di eren
sub ypes. Pa ien s responsive o vi amin B6
PROGNOSIS AND
usually have a less severe and more slowly
MANAGEMENT progressive clinical course han pa ien s who
Pa ien s are usually legally blind by he are no responsive.
our h o seven h decade o li e. However, ear- A low-pro ein die , par icularly a low-
lier visual loss may resul rom ca arac . arginine die , may also be o benef .
Gyra e A rophy 217
B
FIGURE 5-16. Gyra e a rophy. A. Bila eral, mul iple, geographic pa ches o re inal pigmen epi helial loss wi h
scalloped borders (inse ) are presen in he pos erior pole and he peripapillary area. B. Scat ered pigmen ary
clumping is also visible.
( continued)
D
FIGURE 5-16. ( Continued) Gyra e a rophy. C and D. Ex ensive a rophy o he RPE and he choroidal
vascula ure is eviden in he corresponding f uorescein angiogram pho ographs. T e ovea is spared in bo h
eyes, and he pa ien ’s visual acui y was 20/ 40 OD, 20/ 30 OS. (Cour esy o Re ina Slide Collec ion, Wills Eye
Hospi al, Philadelphia, Pennsylvania, compiled by Dr. amara Vrabec and Dr. Gordon Byrnes.)
CONGENI
C ONG
G EN
N I AL S A IO IONARY
O N AR
RY CLINICAL SIGNS
N IGH
H BBLIN
L IN DN
D N ESS
ES
SS Visual acui y: Usually una ec ed.
C ongeni al s a ionary nigh blindness Fundus: Examina ion shows a mul i ude o
(CSNB) is a group o disorders ha is yellow–whi e, iny do s in he pos erior pole
charac erized by de ec ive nigh vision, which ha radia e ou oward he periphery. T e mac-
remains s able hroughou li e. ula is almos invariably spared (Fig. 5-17).
CLASSIFICATION
Re ini is punc a a albescens: T is is a vari-
CSNB wi h normal undus appearance. an o RP in which he undus shows
CSNB wi h abnormal undus appearance: yellow–whi e do s bu has narrowed vessels
T is group includes undus albipunc a us and and a severely reduced ERG ha does no
Oguchi’s disease. recover wi h dark adap a ion.
Fleck re ina o Kandori: A disorder wi h
larger pa ch-like ecks and a less severe
FUNDUS ALBIPUNCTATUS impairmen o nigh vision.
F undus albipunc a us (FA) is a disorder o

he visual pigmen regenera ion process
ha is charac erized by an abnormally pro-
longed recovery o normal rhodopsin levels Visual f elds: Normal.
ollowing an in ense ligh exposure.
Dark adap ome ry: Bo h he cone and rod
componen s o he dark adap a ion curve are
HISTORY very slow in reaching he f nal hreshold.
Elec rore inography: I is impor an o
T e presen ing symp om is nonprogres- know ha unless given enough ime o dark
sive impaired nigh vision, bu given enough adap , bo h he a- and b-waves o he ERG are
ime o adap in he dark, pa ien s will achieve severely reduced. However, wi h prolonged
a normal sensi ivi y. dark adap a ion, ERG re urns o normal.
Elec rooculography: T ere is a slow recov-
EPIDEMIOLOGY ery o he ligh rise wi h dark adap a ion.
AND ETIOLO GY
PROGNOSIS AND
T e proposed de ec in pa ien s wi h FA MANAGEMENT
appears o be an abnormal regenera ion ra e
o he visual pho orecep or pigmen s. T e de ec ive nigh vision is nonprogres-
Au osomal recessive. sive, and he vision is usually una ec ed.
B
FIGURE 5-17. Fundus albipunc a us. Mul iple iny yellow–whi e do s radia ing ou rom he pos erior pole
oward he undus periphery.
OGUCHI’S DISEASE DIAGNOSTIC
O guchi’s disease is a varian o CSNB

charac erized by a nonprogressive nigh
vision impairmen ha is hough o be due o
EVALUATION
Visual f elds: Normal.

a de ec ive pho o ransduc ion process. Dark adap ome ry: T ere is a normal cone
adap a ion bu a markedly delayed rod adap-
EPIDEMIOLOGY a ion ha reaches a normal hreshold level
AND ETIOLO GY over a prolonged period o ime ( rom 3 o
24 hours).
T e visual pho orecep or pigmen s are Elec rore inography: Normal-ampli ude
normal, and he proposed de ec is hough o a-wave and reduced or absen b-wave are seen
be impaired pho o ransduc ion, giving rise o under pho opic and sco opic condi ions. I
abnormal ERG f ndings. is o no e ha even a er he dark-adap ed
hresholds have re urned o normal, he ERG
CLINICAL SIGNS b-wave may s ill be absen .
Elec rooculography: Normal ligh rise.
T e re ina has a peculiar silvery me allic
sheen where re inal vessels s and ou agains
he background undus appearance. T is PROGNOSIS AND
unusual appearance may be seen in he en ire MANAGEMENT
re ina or may be presen only in he pos erior
pole or he periphery. T e nigh vision de ec is nonprogressive.
Mizuo–Nakamura phenomenon: T e T ere is no rea men available or
re ina has a me allic sheen ollowing ligh Oguchi’s disease.
adap a ion, which disappears a er ew hours
in he dark (Fig. 5-18).
FIGURE 5-18. Mizuo–Nakamura phenomenon. Me allic sheen o he re ina a er ligh exposure (righ hand
pho ographs) has disappeared a er a ew hours o dark adap a ion ( le ). (Cour esy o Re ina Slide Collec ion,
Albinism 223
def ci is propor ional o he degree o un-

ALBIN
A L BI N IS
ISM
SM dus hypopigmen a ion (Fig. 5-19).
A lbinism is a group o condi ions ha Nys agmus, ligh sensi ivi y, and a high
involves he melanin sys em o he eye or degree o re rac ive errors.
skin, or bo h. Iris ransillumina ion rom decreased
pigmen a ion.
CLASSIFICATION Foveal aplasia or hypoplasia. In he
presence o signif can oveal hypoplasia,
Oph halmically, wo clinical pat erns are nys agmus begins wi hin 2 o 3 mon hs o
no ed: li e (Fig. 5-20).
rue albinism: Congeni ally subnormal Hypopigmen ed re ina rom he periph-
vision and nys agmus are presen . ery o he pos erior pole.
Albinoidism: T ere is normal or mini- Abnormal re inogeniculos ria e projec-
mally reduced vision and no nys agmus. ions where many emporal nerve f bers
decussa e ra her han projec o he ipsila eral
However, bo h clinical pat erns share
genicula e body. T is phenomenon accoun s
many clinical charac eris ics. rue albinism is
or he abnormal s ereopsis in hese pa ien s.
divided in o he ollowing wo ypes:
T e emale carriers o ocular albinism may
Oculocu aneous albinism: Bo h he eye
reveal par ial iris ransillumina ion and un-
and he skin are a ec ed.
dus hypopigmen a ion (Fig. 5-21).
Ocular albinism: Only he eyes appear
o be a ec ed.
ASSOCIATED CLINICAL SIGNS
EPIDEMIOLOGY Skin hypopigmen a ion is seen in
AND ETIOLO GY pa ien s wi h oculocu aneous albinism.
wo orms o po en ially le hal albinisms
Oculocu aneous albinism resul s rom a
are observed:
reduc ion in he amoun o primary melanin
deposi ed in each melanosome, whereas ocu- Chédiak–Higashi syndrome:
lar albinism is caused by a reduc ion in he Oculocu aneous albinism is combined
o al number o melanosomes. wi h ex reme suscep ibili y o in ec ion
ha can o en lead o dea h in childhood
Gene ics
or you h.
Oculocu aneous albinism: au osomal
Hermansky–Pudlak syndrome:
recessive
Oculocu aneous albinism wi h a pla ele
Ocular albinism: X-linked de ec causing easy bruising and bleed-
ing. Mos pa ien s wi h his disorder in he
IMPORTANT CLINICAL SIGNS Uni ed S a es are o Puer o Rican origin.
Bo h ocular and oculocu aneous albinism HISTOLO GY

share he ollowing ocular clinical ea ures:
Reduced visual acui y in he range o Macromelanosomes in he eye or skin,
20/ 40 o 20/ 400. T e severi y o visual or bo h
Increased number o decussa ing f bers a yrosinase-posi ive albinos have some
he chiasm degree o pigmen a ion.
Hema ologic consul a ion is necessary i
DIAGNOSTIC EVALUATION Chédiak–Higashi or Hermansky–Pudlak syn-
dromes are suspec ed (Fig. 5-22).
T e ypical cons ella ion o symp oms
and signs sugges s he diagnosis. Asymme ric PROGNOSIS AND
visually evoked cor ical po en ials occur due MANAGEMENT
o abnormal decussa ion o he nerve f bers a
he chiasm. Since all orms o albinism are inheri ed,
T e yrosinase hair bulb es indica es gene ic counseling is impor an . A majori y o
he presence or absence o he yrosinase children wi h albinism are able o at end regu-
enzyme (i is required in he biosyn hesis lar schools wi h some degree o assis ance,
o melanin) and divides he oculocu aneous bu only a ew see well enough o drive.
albinism in o wo ypes, yrosinase-posi ive Re rac ion, in ed glasses, and visual aids
and yrosinase-nega ive. yrosinase-nega ive are help ul or older pa ien s.
albinos show a comple e lack o pigmen a- Hema ologic consul a ion in an appropri-
ion in he skin, hair, and eyes, whereas a e set ing is manda ory.
FIGURE 5-19. Albinism. Markedly hypopigmen ed undus. No e he haphazard underlying large choroidal
vessels. (Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia, Pennsylvania, compiled by
Albinism 225
FIGURE 5-20. Albinism, foveal hypoplasia. Color undus pho ograph concen ra ing on he oveal region
o a pa ien wi h albinism. No e a comple e absence o oveal archi ec ure and oveal ref ex— oveal hypoplasia
( box) . (Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia, Pennsylvania, compiled by
B
FIGURE 5-21. Albinism, female carrier. Par ially hypopigmen ed undus in a emale carrier o ocular
albinism. (Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia, Pennsylvania, compiled by
Albinism 227
FIGURE 5-22. Oculocu aneous albinism, Hermansky–Pudlak Syndrome. Purpuric pa ches on he cheeks,
orehead, and le upper lid o a young boy wi h oculocu aneous albinism and Hermansky–Pudlak syndrome.
(Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia, Pennsylvania, compiled by Dr. amara Vrabec
and Dr. Gordon Byrnes.)
RE
R E IN
I N I IS
S PI
PIGMEN
I G M E N OSA
O SA
A CLINICAL SIGNS
R
T e classic clinical riad o RP includes
P is a group o heredi ary disorders asso-
(Fig. 5-23):
cia ed wi h a primary abnormali y o he
pho orecep or–RPE complex, and is charac- Re inal “bone-spicule” pigmen a ion
erized subjec ively by nigh blindness and a Ar eriolar at enua ion
loss o peripheral vision, and objec ively by Waxy op ic disc pallor (Fig. 5-24)
a grossly reduced or ex inguished ull-f eld
Addi ionally, vi reous debris is requen ly
ERG. However, he heredi ary basis can be
encoun ered.
es ablished in only 50% o cases.
Important Clinical Signs
EPIDEMIOLOGY
Nigh blindness: T e mos common pre-
AND ETIOLO GY sen ing clinical symp om. Peripheral vision
Onse : Variable and depends on he inher- problems are ini ially no iced only in he
i ance pat ern. dim ligh , bu la er hese problems are no ed
under all condi ions. Even ually, only a small
Gene ics: T e ollowing modes o inheri-
zone o cen ral vision remains.
ance have been described:
Visual acui y: T e cen ral vision may be
Sporadic: T ere is no amily his ory
preserved or many years in he au osomal-
o RP. T ese are he mos common cases.
dominan orm o RP. An early loss o cen ral
Some o hese cases are au osomal reces-
vision is o en observed in he X-linked or
sive, and o hers may represen au osomal-
au osomal-recessive orms. Addi ionally,
dominan mu a ions.
ca arac , cys oid macular edema, and sur ace
Au osomal dominan : T e nex mos wrinkling o he in ernal limi ing membrane
common mode o inheri ance and has he may con ribu e o early vision loss.
bes prognosis.
Fundus f ndings: Fundus f ndings may
Au osomal recessive. di er according o he s age o he disease.
X-linked recessive: Leas common Very early: Ar eriolar narrowing; f ne
group wi h wors prognosis. dus -like in rare inal pigmen a ion.
La er ea ures: Perivascular “bone-
HISTORY
spicule” pigmen clumping. Pigmen ary
Pa ien s wi h ypical RP presen wi h a changes begin in he midre inal periphery
his ory o nigh blindness or nyc alopia (a and hen ex end an eriorly as well as pos e-
comple e or par ially reduced ra e o visual riorly, giving rise o a ring sco oma. Waxy
adap a ion a nigh or in dim illumina ion). pallor o he op ic disc is he leas reliable
Pa ien s may also have di cul y wi h periph- sign o he RP riad.
eral vision in dim ligh . Advanced ea ures: Unmasking o he
T e di cul y wi h nigh vision may begin large choroidal vessels, prominen ar e-
in early childhood, or pa ien s may no ice riolar at enua ion, and marked op ic disc
i in he second or hird decade o li e. By pallor.
he age o 30 years, over 75% o pa ien s are Macula: Macular involvemen may occur
symp oma ic. in he ollowing ways:
Cys oid macular edema (may respond elec rore inography. T e b-wave implici
o sys emic ace azolamide) ime on he ERG may be prolonged in
Sur ace wrinkling pa ien s wi h predominan loss o rods ra her
han cones. Recen ly, dis inc ive ERG pa -
A rophic changes
erns in he ype I and he ype II au osomal-
Associated Ocular Features dominan orms o RP have been observed.
T e rod ERG is more severely a ec ed han
Op ic nerve head drusen
he cone ERG in ype I RP, whereas rod and
Open-angle glaucoma (in 3% o pa ien s cone ERGs are equally abnormal in ype
wi h RP) II RP.
Pos erior subcapsular ca arac (common Elec rooculography: Almos invariably
in all orms o RP) reduced.
Kera oconus A ypical RP
Myopia ( requen ly encoun ered) Re ini is punc a a albescens: Scat ered
whi e do s are loca ed mos ly be ween he
DIAGNOSTIC EVALUATION pos erior pole and he equa or.
Sec or RP: Only one or wo quadran s
A cons ella ion o charac eris ic signs and o he undus are involved (Fig. 5-25).
symp oms helps es ablish he diagnosis. Pericen ric RP: Pigmen ary changes are
Visual f elds: Ini ially, here is a ull or par- conf ned o he area around he pos erior
ial ring sco oma in he midperiphery, which pole.
ex ends an eriorly as well as pos eriorly, leav- RP sine pigmen o: Pigmen ary changes
ing only a cen ral island o vision in he la e in he undus are ei her minimal or absen .
s ages.
RP wi h exuda ive vasculopa hy: Coa s’
Dark adap ome ry: An eleva ion o he rod disease-like appearance in he undus.
as well as he cone segmen o he dark adap-
a ion curve occurs. On he basis o he dark
adap ome ry resul s, au osomal-dominan RP PROGNOSIS AND
can be divided in o wo ypes: MANAGEMENT
ype I RP: Early-onse nyc alopia wi h
an early di use loss o he rod sensi ivi y Abou one quar er o pa ien s main ain
rela ive o cone sensi ivi y good vision and are able o read hroughou
ype II RP: Adul -onse nyc alopia heir lives. T e au osomal-dominan orm
wi h an equal loss o he rod and cone o he disease has he bes prognosis, and
sensi ivi y he X-linked orm has he wors
prognosis.
Elec rore inography: T is may be sig-
nif can ly subnormal even when he undus Only a ew pa ien s younger han 20 years
shows minimal changes. Severely reduced or o age have a visual acui y o 20/ 400 or less.
almos ex inguished sco opic ERG responses By he age o 50 years, however, a signif can
are observed, whereas he pho opic ERG propor ion o pa ien s will have a visual acu-
is rela ively una ec ed. T e cone b-wave i y o approxima ely 20/ 400.
implici imes, as elici ed by icker s imuli, Elec rore inography is help ul in iden i y-
are almos always prolonged in all orms o ing he emale carriers o X-linked RP (who
may have a normal undus), which may have in a dose o 125 mg wice a day or 2 mon hs
implica ions or gene ic counseling. o conf rm i s benef cial e ec . I e ec ive,
Appropria e inves iga ions should be per- ace azolamide may need o be con inued or
ormed o rule ou a ypical orms o RP ha many mon hs in some pa ien s.
may have an associa ed rea able sys emic Serial Goldmann perime ry should be
condi ion. Explain o pa ien s ha no all per ormed a regular in ervals o assess visual
people wi h RP go comple ely blind. f eld changes.
Yearly ollow-up is recommended o de ec T e role o oral vi amin A herapy in
a precipi ous all in vision ha may raise a slowing he ra e o progression o ypical RP
possibili y o a rea able cause, such as cys oid remains con roversial.
macular edema or ca arac . Cys oid macular
A varie y o low-vision aids may be
edema associa ed wi h RP responds avorably
help ul.
o oral ace azolamide, which is ini ially ried
FIGURE 5-23. Re ini is pigmen osa (RP). T e classic clinical riad o waxy op ic disc pallor, ar eriolar
at enua ion, and “bone spicule” pigmen a ion (inse ) in a pa ien wi h RP. (Cour esy o Re ina Slide Collec ion,
B
FIGURE 5-24. RP. Ar eriolar at enua ion (inse , A) , op ic disc pallor (arrow, B) , and pigmen ary changes in
he undus are he charac eris ic ndings in pa ien s wi h RP. Visual acui y was reduced o nger coun ing vision
due o oveal changes. (Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia, Pennsylvania,
compiled by Dr. amara Vrabec and Dr. Gordon Byrnes.)
FIGURE 5-25. RP, sec or varian . Equa or plus color undus pho ograph showing in erior sec orial re inal
pigmen ary changes. (Cour esy o Re ina Slide Collec ion, Wills Eye Hospi al, Philadelphia, Pennsylvania,
compiled by Dr. amara Vrabec and Dr. Gordon Byrnes.)
Sys emicDiseases Associa ed wi h Re ini is Pigmen osa 233
rea men : Supplemen a ion o vi amins

SYS
S YS EEMIC
MIC DI DISEASES
I SE
E ASES A and E may be benef cial. Oral supplemen-
ASSO
A SSO
OCCIA
IA
A ED DW WII H a ion o vi amin A o reverse he psycho-
RE
R E IN
I N I IIS
S PI
PIGMEN
I G MEE N OSA
O SA
A physical and elec rophysiologic abnormali ies
remains con roversial.
USHER’S SYNDROME
REFSUM’S DISEASE
U sher’s syndrome is def ned as congeni al
or progressive hearing loss associa ed
wi h RP and is he mos common syndrome
( HEREDOPATHIA ATACTICA
POLYNEURITIFORMIS)
associa ed wi h RP. Approxima ely 4% o
children at ending schools or he dea have
RP, and 50% o blind–dea individuals have
Usher’s syndrome.
R e sum’s disease is an au osomal-recessive
disorder caused by a me abolic abnor-
mali y ha resul s in a deposi ion o phy anic
acid in many body issues, including he eyes.
Pigmen ary re inopa hy: I is progressive
and develops be ore puber y. Pigmen ary re inopa hy: Nyc alopia is
almos always presen . Fundus pigmen a ion
Sys emic ea ures: Usher’s syndrome is
is generally o a “sal -and-pepper” ra her han
divided in o our sub ypes, and ypes I and II
“bone-spicule” ype.
cons i u e he majori y o cases.
Sys emic ea ures: Hyper rophic periph-
ype I: Pro ound congeni al dea ness,
eral neuropa hy, cerebellar a axia, dea ness,
abnormal ves ibular unc ions, and early-
ich hyosis, cardiac arrhy hmias, and eleva ed
onse RP
cerebrospinal uid pro ein.
ype II: Par ial dea ness, in ac ves ibu-
rea men : Normaliza ion o he
lar unc ions, and a milder orm o RP
eleva ed serum phy anic acid levels by a
low phy anic acid die may improve re inal
BASSEN–KORNZWEIG unc ion, and s op or slow he progression
SYNDROME o disease.
( ABETALIPOPROTEINEMIA)
CO CKAYNE’S SYNDROME
T his disorder is hough o be caused by an
inabili y o he body o syn hesize apoli-
popro ein B, which helps in he absorp ion o
Pigmen ary re inopa hy: “Sal -and-pepper
ype” wi h ar eriolar at enua ion and op ic
a s in he small in es ine.
nerve pallor.
Pigmen ary re inopa hy: I develops a he Sys emic ea ures: Childhood dwarf sm
end o he f rs decade o li e. P osis and ocu- wi h cachec ic and prema urely aged appear-
lar mo ili y dis urbances may also be seen. ance. A ec ed children have a small head wi h
Sys emic ea ures: Spinocerebellar a axia, charac eris ic bird-like acies, and dispro-
acan hocy osis, and abe alipopro einemia por iona ely large hands and ee . Dea ness,
are no ed. Fa malabsorp ion and associa ed pho oderma i is, nys agmus, a axia, and pro-
def ciency o a -soluble vi amins, par icu- gressive men al re arda ion are associa ed ea-
larly A and E, is presen , and jejunal biopsy is ures. Dea h may occur in he hird or our h
diagnos ic. decade o li e.
KEARNS–SAYRE SYNDROME MUCOPOLYSACCHARIDOSES
T his syndrome is a mi ochondrial myopa-

hy in which an increased number o
dis or ed mi ochondria are accumula ed in
T his group o me abolic disorders is
caused by a def ciency o lysosomal
enzymes involved in he degrada ion o many
he skele al muscles (including he ex raocu- mucopolysaccharides, such as derma an sul-
lar muscles), RPE, and hear . His ologically, a e, heparan sul a e, and kera an sul a e. An
a ec ed muscles show “ragged-red” f bers, abnormal deposi ion o he nondegraded
caused by he accumula ion o mi ochondria mucopolysaccharides leads o mul iple organ
benea h he plasma membrane and be ween sys em dys unc ion.
myof brils.
Gene ics: Au osomal recessive, excep
Pigmen ary re inopa hy: Variable appear- Hun er’s disease, which is X-linked recessive.
ance. Di use re inal pigmen epi helial mo - Ocular ea ures: Re inal pigmen ary
ling may be observed. degenera ion— ound in Hurler’s, Hun er’s,
Sys emic ea ures: Combina ion o chronic and Sanf lippo’s diseases; corneal s romal
progressive ex ernal oph halmoplegia (mos inf l ra ion; op ic nerve head swelling; op ic
common f nding), pigmen ary re inopa hy, a rophy; and glaucoma (rare).
and comple e hear block (may cause dea h). Sys emic ea ures: Facial coarseness,
T is condi ion usually becomes mani es skele al anomalies, and hear disease.
be ore he age o 20 years.
NEURONAL CEROID
BARDET–BIEDL LIPOFUSCINOSIS
SYNDROME ( BATTEN DISEASE)
T
Pigmen ary re inopa hy: “Bull’s-eye” mac- his group o disorders is charac erized
ulopa hy occurs in mos cases. A ew cases by an accumula ion o au o uorescen
are similar o ypical RP wi h “bone-spicule” lipopigmen s in he neurons as well as in non-
pigmen a ion. neural issues. Four ypes are di eren ia ed, as
Sys emic ea ures: Men al handicap, poly- ollows:
dac yly, obesi y, and hypogeni alism; renal
abnormali ies (in mos cases). In an ile ype (Hagberg–San avuori)
Generalized re inal degenera ion
LAURENCE–MO ON Brownish discolora ion o he macula
SYNDROME Early visual loss
Op ic a rophy
Re inopa hy: Ei her ypical RP ype or Men al re arda ion and mo or abnor-
choroidal a rophy. mali ies be ween he ages o 1 and
Sys emic ea ures: Spas ic paraplegia, men- 1½ years
al handicap, and hypogeni alism. La e in an ile ype ( Jansky–Bielschowsky)
Sys emic Diseases Associa ed wi h Re ini is Pigmen osa 235
Generalized re inal degenera ion and FRIEDREICH’S ATAXIA

op ic a rophy
Seizures and psychomo or problems
wi h an onse be ween 2 and 4 years
o age
F riedreich’s a axia is an au osomal-
recessive inheri ed disorder caused by a
dis urbance o pyruva e me abolism. Onse o
Juvenile ype (Spielmeyer–Vog ) symp oms is no ed be ween 10 and 20 years
o age.
Mos common
“Bull’s-eye” maculopa hy wi h even- Ocular ea ures
ual developmen o RP-like clinical pic- Pigmen ary re inopa hy
ure: May need o be di eren ia ed rom Ves ibular nys agmus
S argard ’s disease
Op ic a rophy
Reduced vision
Ver ical gaze paresis
Progressive men al de eriora ion
Sys emic ea ures
Seizures (occasionally)
Cerebellar a axia
Average age o dea h: 17 years
Absence o endon re exes
Adul -onse (KUFS)
Scoliosis
No ocular abnormali ies
Cardiomyopa hy: O en he cause o
Benign; no a al dea h in he hird o our h decade o li e
Nyc alopia
CARCIN
C ARC
CINO MMA-ASSO
A-A
ASSO O CI
CIA
I A EDD
Prolonged dark adap a ion
RE
R E IN
N O PA
PA H Y S
SYN
YN
N DRO
D ROO ME
ME
Ring sco oma
C arcinoma-associa ed re inopa hy (CAR)

syndrome, also known as paraneoplas ic
re inopa hy, is a visual paraneoplas ic disorder
Fundus ea ures
Early s ages may be normal (Fig. 5-26)
La e f nding: Narrow re inal ar erioles
in which au oan ibodies agains umor an igen
cross-reac wi h re inal pro eins, resul ing in Re inal pigmen epi helial mot ling
rod and cone pho orecep or dys unc ion. Op ic nerve pallor
EPIDEMIOLOGY ASSO CIATED

AND ETIOLO GY CLINICAL FEATURES
Age a presen a ion is variable. Sys emic ea ures o he underlying malig-

nancy may or may no be presen .
T ere is no gender pre erence.
Associa ed malignancies include:
Small cell carcinoma o he lung (mos
common)
Decreased vision rom re inal cause and
Gynecologic, breas , endocrine, or normal-appearing undus
o her visceral malignancies
S argard ’s disease
In CAR syndrome, au oan ibodies cross-
Cone dys rophy
reac wi h he pro ein recoverin, loca ed
wi hin he pho orecep ors. wig branch re inal vein occlusion
wig branch re inal ar ery occlusion
HISTORY oxic re inopa hy
Reper used cen ral re inal ar ery occlusion
T e ypical presen a ion is severe, pro- Age, amily and medica ion his ory, bila -
gressive, bila eral visual loss over a period o erali y, uorescein angiography, and he elec-
mon hs, wi h rela ively unremarkable undus. rophysiologic es s help o di eren ia e CAR
Visual symp oms may precede diagnosis o syndrome rom he preceding disorders.
he underlying malignancy.
CLINICAL SIGNS
ypical se o symp oms and signs
Cone dys unc ion: As evidenced by: Color vision: Reduced
Decreased visual acui y Visual f elds: ypically, ring sco oma
Pho osensi ivi y Dark adap ome ry: Prolonged dark
Reduced color vision adap a ion
Cen ral sco oma Elec rore inography: Reduced ampli udes
Rod dys unc ion: As evidenced by: o bo h rod and cone responses
Serum an i-recoverin an ibodies Various rea men measures have been

Sys emic oncologic evalua ion ried, bu heir role in improving he visual
prognosis remains o be proven. T ese mea-
sures include rea men o he underlying
PROGNOSIS AND malignancy, s eroids, plasmapheresis, and
MANAGEMENT in ravenous immunoglobulins.
I pho orecep ors are su cien ly damaged,
visual unc ions are usually permanen ly al ered.
FIGURE 5-26. Carcinoma associa ed re inopa hy (CAR) syndrome. A. Normal undus bu decreased vision.
B. Severe and generalized visual eld depression.
( continued)
FIGURE 5-26. ( Continued) Carcinoma associa ed re inopa hy (CAR) syndrome. C. Near comple ely
ex inguished elec rore inogram. D. Mass lesion in he righ lower lobe o he lung diagnosed as small cell
carcinoma causing CAR syndrome. (Cour esy o Dr. William asman and he Re ina Slide Collec ion, Wills
Eye Hospi al, Philadelphia, Pennsylvania, compiled by Dr. amara and Dr. Gordon Byrnes.)
C H AP T ER
6
e inal and Choroidal umors
Franco M. Recchia
AS
A S RO
R O C Y IC
I HISTORY
HAAMAR
M AR O M MAA Pa ien s wi h as rocy ic hamar omas are
usually asymp oma ic. Visual f eld es ing may
A s rocy ic hamar oma is a congeni al,
minimally progressive, benign umor
arising rom he glial cells o he re ina and
reveal a sco oma in he area corresponding o
he umor.
usually loca ed around he op ic disc. I is o en
associa ed wi h he sys emic condi ion uber-
ous sclerosis (Bourneville’s disease), occasion- IMPORTANT
ally wi h neurof broma osis, bu also occurs CLINICAL SIGNS
sporadically in o herwise normal individuals.
T e appearance o a re inal as rocy ic ham-
ar oma is principally o wo ypes:
Smaller, noncalcif ed, a , smoo h
EPIDEMIOLOGY umor ha appears as mild hickening o
AND ETIOLO GY he nerve f ber layer
Larger, calcif ed, whi ish-yellow nodular
Mos as rocy ic hamar omas occur con-
mass (“mulberry lesion”)
geni ally in associa ion wi h uberous sclerosis
( S), a amilial phakoma osis charac erized Aspec s o bo h may be seen in he
by he riad o seizures, men al re arda ion, and same lesion, as i is likely ha calcif ca ion
skin lesions. S has an es ima ed incidence progresses slowly over many years
o 1 in 15,000 o 1 in 100,000 and exhibi s (Fig. 6-1).
au osomal-dominan inheri ance. oughly hal Every pa ien wi h an as rocy ic hamar-
o he pa ien s wi h S have as rocy ic ham- oma o he re ina or op ic nerve must be
ar omas. Causa ive genes have been iden if ed evalua ed or S. ypical mani es a ions o
on chromosomes 9q34 and 16p13. S include:
240
Astrocytic Hamartoma 241
Skin lesions ( 95% incidence) e inal granuloma

Hypomelano ic macules: Oval (“ash- Drusen o he op ic disc
lea ”), polygonal, or punc a e (“con- Papilli is
et i”) in shape. Usually presen a bir h
and o en he f rs presen ing sign.
eddish-brown papular rash over
he ace ( ermed adenoma sebaceum, Fluorescein angiography: T e umor
bu ac ually angiof bromas), o en mis- appears rela ively hypo uorescen in he
aken or acne. arely presen be ore a ar erial phase. Superf cial f ne blood vessels
ew years o age. are seen during he venous phase. T e umor
Seizures ( 90% incidence): Myoclonic s ains in ensely and homogeneously in he
spasms las ing 10 o 50 seconds; end o la e phases.
become grand mal ype la er in li e. B-scan Ul rasonography: A larger, calcif ed
Men al re arda ion ( 60% incidence) lesion o en appears as a discre e, oval, solid
mass wi h a sharp an erior border.
ASSO CIATED Neuroimaging: Subependymal hamar o-
CLINICAL SIGNS mas, charac eris ic o S, may be seen wi h
compu ed omography (C ) or magne ic
Ocular: Occasionally, may produce vi re- resonance imaging (M I).
ous hemorrhage, vi reous seeding, subre inal
hemorrhage, or re inal de achmen . PROGNOSIS AND
Sys emic (addi ional mani es a ions o MANAGEMENT
S): Ungual f bromas, pleural cys s (lead-
ing o spon aneous pneumo horax), renal T e vas majori y o re inal as rocy ic
angiomyolipoma, cardiac rhabdomyoma, and hamar omas are asymp oma ic and do no
hamar omas o he liver, hyroid, pancreas, or require rea men .
es is may occur. Associa ed re inal de achmen can
o en be rea ed wi h demarca ing laser
DIFFERENTIAL DIAGNOSIS pho ocoagula ion.
Pa ien s and amily members should
e inoblas oma ( b) be examined regularly or mani es a ions
Myelina ed nerve f bers o S.
242 6 RETINAL AND CHO RO IDAL TUMORS
FIGURE 6-1. Astrocytic hamartoma. A peripapillary astrocytic hamartoma can be seen with classic mulberry
calcif cation overlying an underlying smooth tumor. Patients and amily members should be examined or
tuberous sclerosis. Courtesy o Drs. Jerry and Carol Shields, Oncology Service, Wills Eye Hospital, Philadelphia,
Pennsylvania.
Retinoblastoma 243
RE
E IN
N O BLAS
BLAS
S OM
MAA HISTORY
A whi e pupillary re ex (leukokoria;

R b is he mos common in raocular malig-
nancy o children. I may be amilial bu
mos o en is sporadic in occurrence. T e
Fig. 6-2) and s rabismus are he wo mos
common f ndings repor ed by paren s. In
ewer han 10% o cases is a amily his ory
umor arises rom cells in he developing re ina
known a he ime o diagnosis.
o one or bo h eyes as a resul o mu a ions in
he b) umor-suppressor gene. b presen s
mos o en as a whi e in raocular mass wi h IMPORTANT
propensi y or direc ex ension in o he brain CLINICAL SIGNS
and almos cer ain mor ali y i un rea ed.
Mos pa ien s presen wi h leukokoria and
EPIDEMIOLOGY s rabismus. Small re inoblas omas appear as
a , ranslucen , whi e re inal lesions
AND ETIOLO GY
(Fig. 6-3A). Wi h grow h, he umor appears
more solid, eleva ed, and chalky-whi e, wi h
b occurs in approxima ely 1 in 15,000
overlying dila ed or uous blood vessels.
live bir hs. Mos children are diagnosed a a
mean o 18 mon hs. T ree grow h pat erns have been
described.
No predilec ion or race or gender has
been shown. Endophy ic: T e umor grows rom he
re ina inward o seed he vi reous cavi y or
oughly wo- hirds o he cases are unila -
an erior chamber.
eral, and one- hird is bila eral. Unila eral cases
are more likely o be diagnosed a an older Exophy ic: T e umor grows rom he
age (mean o 24 mon hs) and are mos o en re ina ou ward o occupy he subre inal
non amilial (sporadic). Bila eral cases are space, o en causing an exuda ive re inal
diagnosed a a mean o 12 mon hs, are usually de achmen (Fig. 6-3B).
amilial, and are nearly always mul i ocal. Di use inf l ra ing: T e leas com-
b resul s rom mu a ions or loss o bo h mon orm; his is charac erized by shal-
alleles o he b gene, loca ed on chromo- low spread o umor along he en ire
some 13q14. T e b gene produc appears re ina and in o he vi reous and an erior
o unc ion as regula or o cellular proli era- chamber.
ion hrough inhibi ory e ec s on gene ran- O her impor an f ndings are iris neovas-
scrip ion a specif c s ages o he cell cycle. culariza ion, which occurs in nearly one-f h
T e iming o allelic inac iva ion de ermines o all cases, and “pseudohypopyon” (set ling
whe her he mu a ion is germinal (i.e., heri- o umor and in amma ory cells in he an e-
able by o spring o he a ec ed child) or rior chamber).
somatic (nonheri able). In germinal cases, a
mu an allele is presen be ore er iliza ion, ASSO CIATED
mos commonly as a resul o inheri ance
CLINICAL SIGNS
rom ei her paren . In soma ic cases,
bo h alleles are presen and ac ive a er il-
Clear lens
iza ion, and spon aneous mu a ions in each
allele arise subsequen ly. He erochromia iridis
Spon aneous hyphema re inoblas omas), assessing ex raocular ex en-

Ex rascleral ex ension sion, and iden i ying he presence o pineal
umors.
Pinealoblas oma (“ rila eral
re inoblas oma”) Fluorescein angiography reveals early
ar erial f lling o he vessel eeding he umor,
leakage o dye rom in rinsic umor vessels,
DIFFERENTIAL DIAGNOSIS and la e hyper uorescence o he umor.
Leukokoria
PROGNOSIS
Coa s’ disease
Persis en e al vascula ure syndrome Spon aneous regression is rare and leads
( ormerly ermed persis en hyperplas ic o ph hisis bulbi. ypically, i un rea ed, chil-
primary vi reous, or PHPV) dren die wi hin 2 years o diagnosis. Early
oxocariasis de ec ion, coupled wi h improvemen s and
e inopa hy o prema uri y promp ness o rea men , has reduced he
mor ali y ra e o less han 10%. T e main
Familial exuda ive vi reore inopa hy
de erminan or mor ali y is op ic nerve inva-
e inal as rocy oma sion. For his reason, i is impera ive o ob ain
Ca arac as long a sec ion o op ic nerve as possible
Norrie’s disease during enuclea ion.
Incon inen ia pigmen i Prognos ic ac ors or ailure o preserve
vision or o preserve he eye are larger
Vi reous seeding
umor size, vi reous seeding, and macular
In raocular in amma ion involvemen .
Endoph halmi is Children wi h germinal b have an
Vi reous hemorrhage increased risk o developing o her primary
Leukemic inf l ra ion malignancies over he course o heir li e-
imes. T ese umors include principally
in racranial b, os eogenic sarcoma o he
DIAGNOSTIC EVALUATION long bones, and sarcoma o so issues. T e
risk is es ima ed o be 20% wi hin 25 years o
De ailed sys emic evalua ion and examina- rea men .
ion is required, as well as amily his ory and
ocular examina ion o paren s, and comple e MANAGEMENT
examina ion o bo h eyes (o en requiring
anes hesia or comple e visualiza ion o he Children diagnosed wi h b should
undi wi h scleral depression). undergo evalua ion or sys emic involvemen ,
Ul rasonography: An eleva ed, rounded, including comple e blood coun , lumbar
in raocular mass is seen, wi h high in ernal punc ure, neuroimaging, and bone marrow
re ec ivi y (calcif ca ion) and shadowing o biopsy. Gene ic es ing o he child and amily
sclera and so issue pos erior o he lesion. members should be per ormed.
C is help ul in de ec ing in raocular Individual rea men varies according
calcif ca ion (presen in roughly 80% o o number, size, and loca ion o umors, as
Retinoblastoma 245
well as sys emic s a us. T erapeu ic op ions In amilial cases, gene ic counseling pro-
include cryo herapy, laser pho ocoagula ion, vides paren s wi h impor an in orma ion
enuclea ion, ex ernal-beam irradia ion, plaque regarding he probabili y o ur her occur-
radio herapy, chemo herapy, hermo herapy, rences. Pa ien s wi h germinal b mus be
and chemo hermo herapy. warned o he possibili y o ransmission o
Chemoreduc ion prior o def ni ive ocular heir o spring.
rea men may be help ul in reducing he
need or enuclea ion and reducing he ra e o
occurrence o pinealoblas oma.
FIGURE 6-2. Retinoblastoma. T is young boy has leukokoria and strabismus, the most common clinical
presentation o retinoblastoma. Courtesy o Drs. Jerry and Carol Shields, Oncology Service, Wills Eye Hospital,
Philadelphia, Pennsylvania.
B
FIGURE 6-3. Retinoblastoma. A. Focal retinoblastoma presenting as a macular intraretinal amelanotic tumor.
B. Massive exophytic retinoblastoma with tumor behind the clear lens. Courtesy o Drs. Jerry and Carol Shields,
Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
Retinal Capillary Hemangioma 247
RE INAL
IN
N AL C
CAPILLARY
APII LL
L ARY
Y IMPORTANT
H EM
EMAN
M AN G
GIO
IO
O MA
MA CLINICAL SIGNS
e inal capillary hemangioma: Usually

O riginally ermed angiomatosis retinae,
re inal capillary hemangioma is a benign
vascular umor o variable size loca ed in he
loca ed peripherally and well circumscribed.
Ini ially appears as a yellow-red do wi h a
minimally dila ed “ eeding” ar eriole or drain-
re ina or adjacen o he op ic disc. Usually
ing venule (Fig. 6-4). Wi h grow h, appears
diagnosed by he our h decade, i may be
orange-red wi h more prominen ly dila ed
he f rs mani es a ion o von Hippel–Lindau
a eren and e eren vessels. May have associ-
(VHL) disease, a amilial cancer syndrome wi h
a ed exuda ion, subre inal uid, or prere inal
which i is commonly associa ed.
f brosis.
Jux apapillary capillary hemangioma:
EPIDEMIOLOGY Orange-red in color, bu less well circum-
AND ETIOLO GY scribed. I o en lacks eeder vessels.
VHL syndrome: Hemangio-blas omas o
T is umor may occur in a sporadic or
he cerebellum and spinal cord, renal cell car-
heredi ary ashion. e inal capillary heman-
cinoma, or pheochromocy oma.
gioma occurs in up o 80% o pa ien s wi h
VHL syndrome and is o en he f rs mani es-
a ion, diagnosed a a mean age o 25 years. ASSO CIATED
T e VHL syndrome has an es ima ed CLINICAL SIGNS
prevalence o 1 in 40,000 and is possibly
more common in whi es. I exhibi s dominan e inal de achmen and, rarely, neovas-
inheri ance and has variable pheno ypes cular glaucoma may complica e capillary
wi hin amilies. hemangioma.
Mean survival o pa ien s wi h VHL is Hemangiomas o he adrenal glands, lungs,
41 years o age. and liver, and mul iple cys s o he pancreas
and kidneys have been observed in some
VHL syndrome is caused by mu a ions
pa ien s wi h VHL syndrome.
in he VHL gene, a umor-suppressor gene
loca ed on chromosome 3p25. T e VHL gene
produc regula es he expression and unc ion DIFFERENTIAL DIAGNOSIS
o hypoxia-responsive angiogenic ac ors
[e.g., vascular endo helial grow h ac or e inal capillary hemangioma
(VEGF)].
O her umors: e inal cavernous hem-
angioma, racemose hemangioma, choroi-
HISTORY
dal melanoma, and as rocy ic hamar oma
Capillary hemangiomas may be diagnosed Vascular diseases: Coa s’ disease, re inal
inciden ally or in pa ien s suspec ed o hav- ar erial macroaneurysm, amilial exuda ive
ing VHL syndrome. T e umors may be vi reore inopa hy, and exuda ive macular
asymp oma ic or may produce painless visual degenera ion
impairmen rom vi reous hemorrhage, macu- N.B.: A dis inc ion has been made
lar pucker, or re inal de achmen . be ween he re inal angioma o VHL
syndrome and an acquired, nonheredi ary PROGNOSIS AND

en i y occurring in older pa ien s and MANAGEMENT
ermed vasoproliferative retinal tumor. T is
lat er condi ion is usually loca ed in he T e na ural his ory is variable, wi h bo h
in ero emporal peripheral undus and lacks progressive enlargemen and spon aneous
markedly dila ed eeder vessels. regression having been repor ed. Visual prog-
Op ic disc hemangioma nosis is highly variable and depends on umor
Papilli is loca ion, size, and associa ed complica ions,
especially hose ha involve he macula.
Op ic disc granuloma
In pa ien s wi h VHL syndrome, risk o
Op ic disc glioma
developing associa ed umors increases wi h
age. Morbidi y and mor ali y are rela ed o
hese associa ed umors. Median survival is
DIAGNOSTIC EVALUATION less han 50 years, wi h renal carcinoma as he
leading cause o dea h.
Ocular: Fluorescein angiography is he rea men is recommended or umors wi h
mos help ul ancillary s udy. In he ar erial documen ed grow h or e ec s on visual unc-
phase, a prominen , dila ed eeder ar eriole ion. T e goal o rea men is o induce resolu-
may be seen. T e umor appears hyper uo- ion o exuda ion or subre inal uid. Laser
rescen early and remains so hrough he pho ocoagula ion is reserved or smaller umors
la e phases, some imes leaking dye in o he (less han 2 mm in diame er). Larger umors
vi reous. Ul rasonography may be help ul in are bes rea ed wi h cryo herapy or plaque
diagnosing lesions o grea er han 1 mm and radio herapy. Vi reore inal surgery, and, rarely,
demons ra es acous ic solidi y hroughou enuclea ion may be necessary or pa ien s wi h
he lesion. advanced or uncon rollable pa hology.
Sys emic: All pa ien s wi h re inal capil- Pa ien s wi h VHL syndrome and heir
lary hemangioma, as well as rela ives, should rela ives should be examined regularly or
be evalua ed or VHL syndrome. Gene ic li e. T is includes regular es ing or urine ca -
es ing or mu a ions in he VHL gene is echolamines, C and ul rasonography o he
available. kidneys, and M I o he brain.
Retinal Capillary Hemangioma 249
FIGURE 6-4. Retinal capillar y hemangioma. Note the dilated eeding retinal arteriole (arrow) and the
segmented draining retinal vein ( arrowhead) with associated macular edema, premacular f brosis, and lipid
exudation. T ese lesions may be the f rst mani estation o von Hippel–Lindau disease. Courtesy o Drs. Jerry
and Carol Shields, Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
T ere may also be cu aneous vascular

RE INAL
I NA
AL CAVERN
C AVE RN
N O US mal orma ions.
H EM
EMAN
M ANNG
GIO
IO
O MA
MA Cavernous hemangiomas o he midbrain
or cerebellum may produce seizures or sub-
R e inal cavernous hemangioma is a benign,
rarely progressive, vascular umor o he
re ina or op ic disc charac erized by a collec-
arachnoid hemorrhage.
ion o venous aneurysms. I may be associa ed DIFFERENTIAL DIAGNOSIS

wi h similar vascular anomalies o he skin and
cen ral nervous sys em (CNS). Capillary hemangioma
Acquired vasoproli era ive umor
EPIDEMIOLOGY acemose hemangioma
AND ETIOLO GY
Coa s’ disease
umor occurrence is mos ly sporadic.
Small pedigrees o pa ien s wi h cavernous DIAGNOSTIC EVALUATION
hemangioma as par o a dominan ly inher-
i ed oculoneurocu aneous syndrome have Fluorescein angiography produces a ypi-
been repor ed. cal pat ern: T e lesion is hypo uorescen
in he early ar erial phase and exhibi s slow
HISTORY hyper uorescence during he la e venous
phase as dye en ers he venous channels.
Pa ien s are usually asymp oma ic, bu he A uorescein–blood in er ace may be seen
umor may produce painless visual loss. wi hin he aneurysms in he la e phases o he
A heredi ary componen may be no ed. angiogram.
IMPORTANT
PROGNOSIS AND
CLINICAL SIGNS
MANAGEMENT
A clus er o dark-red, in rare inal aneu-
Mos cavernous hemangiomas do no
rysms is loca ed along a re inal venule,
enlarge and can be managed wi h periodic
appearing as a “clus er o grapes” arising
observa ion.
rom he inner re inal sur ace (Fig. 6-5).
O en here is overlying gray f broglial issue. T e main complica ion is vi reous hemor-
Usually he umor does not have associa ed rhage, al hough his rarely causes permanen
exuda ion or a eeding ar eriole. visual loss.
umors causing recurren vi reous
ASSO CIATED hemorrhage may be rea ed wi h cryo-
CLINICAL SIGNS herapy, laser pho ocoagula ion, or plaque
radio herapy.
Vi reous hemorrhage occurs in up o 10%
o cases.
Retinal Cavernous Hemangioma 251
FIGURE 6-5. Retinal cavernous hemangioma. Note the “cluster o grapes” appearance o these intraretinal
aneurysms (inset) . T ere is a lack o exudation, and sur ace gray retinal f brous tissue is noted. Courtesy o
Drs. Jerry and Carol Shields, Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
Mul i ocal: Also ermed congeni al

CO
C O N GEN
GE E N I AL
AL grouped pigmen a ion or “bear racks.”
H YP
YPER
P E R RO
R O PH
PH Y OF HE Groups o 3 o 30 small (0.1 o 2 mm)
RE
R E INAL
I NAAL PIGMEN
PIGME EN lesions ypically appear in one sec or o he
EPI
E PI H E ELIUM
L I UM
M midperipheral re ina. Usually hey lack he
in ernal lacunae and hypopigmen ed halo
DEFINITION o he soli ary orm.
Mul i ocal undus lesions resembling
C ongeni al hyper rophy o he re inal pig-

men epi helium (CH PE) is a benign,
asymp oma ic condi ion, consis ing o one or
CH PE have been repor ed in close associa-
ion wi h Gardner’s syndrome (Fig. 6-7),
a amilial condi ion o colonic polyps and
more well-demarca ed, pigmen ed, a , nonex rain es inal os eomas and f bromas wi h
progressive lesions, usually ound in he equa- invariable progression o colonic cancer. T e
orial or peripheral undus. In rare ins ances, lesions associa ed wi h Gardner’s syndrome,
mul i ocal lesions may be associa ed wi h however, are bila eral, have irregular borders,
amilial colonic polyposis. and are o en scat ered in he undus.
EPIDEMIOLOGY DIFFERENTIAL DIAGNOSIS

AND ETIOLO GY
Malignan choroidal melanoma
T e condi ion is probably congeni al. Choroidal nevus
I occurs wi h equal requency in blacks Combined hamar oma o he re ina and
and whi es. re inal pigmen epi helium ( PE)
HISTORY DIAGNOSTIC EVALUATION

Pa ien s are usually asymp oma ic. O en Diagnosis is based on ypical oph halmo-
he disorder is no ed as an inciden al f nding scopic ea ures. By uorescein angiography,
during oph halmoscopy. he umor exhibi s persis en hypo uores-
cence. Lacunar areas are seen as hyper uores-
IMPORTANT cen , consis en wi h depigmen a ion o he
CLINICAL SIGNS PE.
wo orms have been described. PROGNOSIS AND

Soli ary: Unila eral, deeply pigmen- MANAGEMENT
ed, a , circular lesion measuring 1 o
6 mm in diame er. Usually sharply Mos CH PE lesions are nonprogressive
demarca ed, he lesion may be solid and require only periodic examina ion. In rare
black, or ringed wi h a small border o ins ances, mild enlargemen may occur.
hypopigmen a ion. Lacunar areas o Pa ien s wi h bila eral lesions sugges ive o
depigmen a ion wi hin he lesion may hose seen in Gardner’s syndrome should be
be seen (Fig. 6-6). re erred or colonoscopy.
Congenital Hypertrophy of the Retinal Pigment Epithelium 253
FIGURE 6-6. Congenital hypertrophy of the retinal pigment epithelium. Note the sharp, discrete borders
o the lesion as well as the lacunar areas o depigmentation ( arrow) within the lesion. Courtesy o Drs. Jerry and
Carol Shields, Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
B
FIGURE 6-7. Pigmented fundus lesion, Gardner ’s syndrome. Pigmented lesion associated with Gardner’s
syndrome and amilial gastrointestinal cancer. A depigmented “tail” ( arrowheads) is noted adjacent to the
pigmented undus lesion (insets) . Courtesy o Drs. Jerry and Carol Shields, Oncology Service, Wills Eye
Hospital, Philadelphia, Pennsylvania.
Combined Hamartoma of the Retina and Retinal Pigment Epithelium 255
vessels oward he lesion by he overlying

CO
C O MBIN
M BINN ED
E D H AMAR
AMA AR O MA
A membrane.
O F H E RER E INA
IN
N A AN
AN D
RE
R E INAL
I N AL
L PIGMEN
P IGMM ENN ASSO CIATED
EPI
E PI H ELELIUM
LI U M CLINICAL SIGNS
C ombined hamar oma o he re ina and

PE is a benign, sligh ly eleva ed, par ially
pigmen ed umor loca ed around he op ic
Choroidal neovasculariza ion
Vi reous hemorrhage
Mani es a ions o neurof broma osis ype
nerve or in he peripheral undus. I is composed
2 (bila eral acous ic neuromas, brain menin-
his ologically o proli era ed glial cells, f brovas-
giomas, spinal cord schwannomas, pos erior
cular issue, and pigmen epi helial cells.
subcapsular ca arac s)

AND ETIOLO GY
Choroidal melanoma
T e umor is o en diagnosed by early
Choroidal nevus
adul hood. T e preponderance o cases in
in an s and young children sugges s ha he eac ive hyperplasia o he PE
lesion may be congeni al. Melanocy oma
An associa ion o combined hamar omas, When ligh ly pigmen ed and occur-
usually macular and some imes bila eral, ring in children, may be mis aken or b or
has been seen wi h neurof broma osis (mos oxocariasis
commonly ype 2).
HISTORY
Diagnosis is based on oph halmoscopic
In cases o jux a oveal lesions, here is ea ures. Fluorescein angiography reveals
painless visual loss rom epimacular mem- mul iple, dila ed, f ne blood vessels wi hin he
brane rac ion or subre inal exuda ion. umor, which may become hyper uorescen
as he angiogram progresses.
IMPORTANT
CLINICAL SIGNS PROGNOSIS AND
MANAGEMENT
Jux apapillary varian : Ill-def ned, eleva ed,
charcoal-gray mass adjacen o, or overlying, Since lesions are usually no progressive,
he op ic disc. A gray-whi e membrane over- regular observa ion is appropria e. However,
lying he umor causes s re ching o re inal con rac ion o overlying f broglial issue leads
blood vessels and re inal s riae, o en involv- o macular dis or ion, secondary re inoschi-
ing he macula (Fig. 6-8). sis, and re inal holes.
Peripheral varian : Sligh ly eleva ed, In cases o visual loss, vi rec omy and
pigmen ed ridge concen ric o he op ic membrane s ripping may be per ormed, bu
disc. T ere is dragging o dila ed re inal visual recovery is limi ed.
FIGURE 6-8. Combined hamartoma of the retina and retinal pigment epithelium. T is is an example o the
juxtapapillary variant o the lesion. T e edge o an apparent membrane is noted most prominently on the nasal
aspect o the lesion. T ere is marked distortion and tortuosity o the involved retinal vasculature. Courtesy o
Drs. Jerry and Carol Shields, Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
Choroidal Nevus 257
CH
H O RO
R O IDAL
ID
D AL N EVUS
E VUS
S ASSO CIATED
CLINICAL SIGNS
C horoidal nevus is a common, benign

umor o he pos erior undus. Al hough
usually a or minimally eleva ed, and gray or
Serous de achmen o he neurosensory
re ina or PE
brown in appearance, i may show variable Choroidal neovasculariza ion
degrees o pigmen a ion.
EPIDEMIOLOGY
Pigmen ed lesion
Prevalence in he general popula ion is Choroidal melanoma
es ima ed o be 1% o 6%. T e umor occurs Congeni al hyper rophy o he PE
much more commonly in whi es.
Combined hamar oma o he re ina and
PE
HISTORY Subre inal hemorrhage
Amelano ic lesion
Pa ien s are usually asymp oma ic, and
he umor is generally discovered inciden ally Circumscribed choroidal hemangioma
during rou ine oph halmoscopy. Choroidal os eoma
Vision may be reduced rom ex ension o Choroidal me as asis
associa ed subre inal uid in o he macula, or In amma ory lesion
rom an associa ed serous re inal de achmen .

CLINICAL SIGNS
Diagnosis is based on charac eris ic oph-
T e umor is mos o en sla e gray or halmoscopic ea ures. Fluorescein angiogra-
brown in color, bu i may be he erogeneously phy, al hough no specif c or choroidal nevus,
pigmen ed or even amelano ic (pale yellow). may provide conf rma ory evidence. Areas
o deep pigmen a ion are hypo uorescen ,
Choroidal nevi are usually 1 o 5 mm in
whereas areas o overlying PE al era ion will
diame er, a or minimally eleva ed (less han
appear hyper uorescen .
2 mm in an eropos erior dimension), wi h ill-
def ned margins. Ul rasonography may be help ul or es ab-
lishing baseline hickness.
Drusen overlying he umor are common
and signi y chronici y o he lesion (Fig. 6-9).
Al era ions o he overlying PE include PROGNOSIS AND
pigmen clumping and f brous me aplasia MANAGEMENT
(yellow-whi e plaques). “Orange pigmen ” a
he level o he PE represen s aggrega es o All small choroidal melanocy ic
macrophages con aining lipo uscin granules umors have he po en ial or malignan
and may sugges grow h or malignan rans- rans orma ion and me as asis. isk ac ors
orma ion o he nevus. or grow h include grea er hickness (larger
han 2 mm), proximi y o he op ic disc, Managemen consis s o baseline pho o-

orange pigmen on he sur ace o he umor, graphs and regular examina ion o es ablish
and presence o subre inal uid. T e chance quiescence or grow h o he nevus. Serial
o grow h o small, a (“nonsuspicious”) pho ographs, ul rasonography, and more re-
lesions lacking hese clinical ea ures is less quen examina ions are indica ed in cases o
han 5%. suspec ed grow h.
FIGURE 6-9. Choroidal nevus. T is is a minimally elevated choroidal pigmented lesion with overlying
drusen ( arrow) . No orange pigment or submacular uid suggestive o potential trans ormation o a malignant
choroidal melanoma is noted. Courtesy o Drs. Jerry and Carol Shields, Oncology Service, Wills Eye Hospital,
CH
C H O RO
R O IDAL
I D AL MELAN
M EL
L AN
N O MA
A IMPORTANT
CLINICAL SIGNS
C horoidal melanoma is he mos com-

mon primary in raocular malignancy,
occurring mos o en in whi e adul s. Dome-
T e umor appears as a dome-shaped,
eleva ed choroidal mass, ypically conf ned
or mushroom-shaped, his variably pig- o he subre inal space. Abou 20% o choroi-
men ed mass arises rom he choroid o one dal melanomas break hrough Bruch’s mem-
eye. I has a propensi y or me as asis o he brane and ake on a charac eris ic mushroom
liver. shape.
Color varies rom brown o gray o pale
EPIDEMIOLOGY yellow (amelano ic), wi h overlying clumps
AND ETIOLO GY o orange pigmen , represen ing collec ions o
lipo uscin (Fig. 6-10).
T e incidence is 1 in 2000 o 1 in 2500 in Associa ed subre inal uid, usually sur-
Caucasians. In he Uni ed S a es, choroidal rounding he base o he lesion, may be no ed,
melanoma is nearly en imes more common and he umor may lead o o al serous re inal
in whi es han in A rican Americans. de achmen .
T ere is no gender predilec ion.
T e umor is uncommon in people ASSO CIATED
younger han 30, bu incidence increases wi h CLINICAL SIGNS
age and he average age a diagnosis is in he
six h decade. Vi reous hemorrhage
isk ac ors may include prolonged expo- Subre inal hemorrhage
sure o ul raviole ligh , congeni al oculoder- Choroidal neovasculariza ion
mal melanocy osis (nevus o O a), and amily
Ex rascleral ex ension wi h orbi al
his ory.
invasion
T e umor arises rom dendri ic mela-
nocy es o he choroid. His opa hologically,
here are wo major ca egories o cells: spin- DIFFERENTIAL DIAGNOSIS
dle cells and epi helioid cells.
Choroidal nevus
are associa ed karyo ypic abnormali ies
have been repor ed, mos commonly al era- Choroidal me as asis
ions o chromosome 3. Combined hamar oma o he re ina and
PE
HISTORY Congeni al hyper rophy o he PE
(CH PE)
T e malignancy is usually asymp oma ic Circumscribed choroidal hemangioma,
and painless. reac ive hyperplasia o he PE
Visual e ec s include blurred vision, Bila eral di use uveal melanocy ic
oa ers, pho opsia, and visual f eld proli era ion
de ec s. Choroidal os eoma
DIAGNOSTIC EVALUATION Higher risk or me as asis and mor ali y

appears o be associa ed wi h specif c pat erns
Diagnosis is based on oph halmoscopic o gene expression, which can be de ermined
ea ures. Ul rasonography reveals an acous i- hrough analysis o umor issue.
cally hollow, dome-shaped or mushroom-
shaped mass wi h low in ernal re ec ivi y.
Ul rasonographic measuremen s o umor MANAGEMENT
hickness are valuable in de ermina ion o
doses or radio herapy, as well as or serial Ocular
evalua ion ollowing rea men . T e primary goal o rea men o choroidal
Fluorescein angiography o small choroi- melanoma is preven ion o me as asis. Choice
dal umors shows early mot led hyper uores- o specif c modali y depends on umor size,
cence o he umor wi h progressive s aining loca ion, and he pa ien ’s sys emic and psy-
in he la e phases. Large umors display f lling chological s a us.
o large in rinsic vessels in he venous phase, Enuclea ion remains he s andard rea -
wi h di use la e s aining. men or large melanomas (grea er han
12 mm in L D and 8 mm in hickness).
PROGNOSIS O her op ions or smaller umors include
ranspupillary hermo herapy ( ),
Overall, 5-year survival or pa ien s wi h radio herapy (plaque brachy herapy or
choroidal melanoma is abou 80%. Median pro on-beam irradia ion), and local resec ion
survival is abou 7 years. Prognosis or sur- (reserved or an erior lesions). Observa ion
vival depends on a varie y o ac ors, includ- is appropria e or small umors wi h lit le
ing age a diagnosis, in raocular loca ion, evidence o grow h.
ex en o me as asis, umor size, and umor Pa ien s should be evalua ed regularly
cell ype. T e 5-year mor ali y or pa ien s wi h care ul unduscopy, serial undus pho o-
wi h epi helioid melanomas is 42%; or hose graphs, and ul rasonography.
wi h spindle cell umors, 10%. T e 5-year Systemic
mor ali y or pa ien s wi h umors wi h larg-
T e vas majori y ( 98%) o pa ien s have
es basal umor diame er (L D) grea er han
no discernible me as asis a he ime o diag-
15 mm is nearly 50%; or hose wi h L D less
nosis o heir choroidal melanoma.
han 10 mm, under 20%.
Baseline sys emic evalua ion should be per-
isk ac ors or me as asis include docu-
ormed in every pa ien , under he direc ion
men ed grow h, proximi y o he op ic disc,
o a medical oncologis . Assessmen ypically
and grea er umor hickness. Nearly one-hal
includes comple e blood coun , liver enzyme
o he pa ien s wi h me as a ic choroidal
panel, ches roen genogram, and imaging (i.e.,
melanoma die wi hin 1 year o rea men .
C or ul rasound) o he abdomen.
T ere appears o be no di erence in mor ali y
in pa ien s rea ed ini ially wi h enuclea ion Measuremen o liver enzymes
when compared wi h hose rea ed wi h should be per ormed regularly along
iodine-125 plaque radio herapy. wi h oph halmoscopy.
B
FIGURE 6-10. Choroidal melanoma. A. A large, elevated, pigmented choroidal mass surrounding the temporal
aspect o the optic disc is appreciated with overlying orange pigment. T e primary goal o treatment o choroidal
melanoma is prevention o systemic metastases. B. A large, elevated, pigmented choroidal mass with subretinal
uid and orange pigment. Orange pigment may represent macrophage ingestion o lipo uscin pigment and re ect
tumor activity. Courtesy o Drs. Jerry and Carol Shields, Oncology Service, Wills Eye Hospital, Philadelphia,
Pennsylvania.
CH
H O RO
R O IDAL
ID
D AL
L ASSO CIATED
MELAN
M E LA
AN O C Y O MA
MA CLINICAL SIGNS
T e uninvolved area o he op ic disc may

C horoidal melanocy oma is a benign,
minimally progressive, deeply pig-
men ed umor occurring a or around he
become acu ely edema ous wi h an associa ed
decline in vision.
op ic nerve. I is probably a varian o choroi- arely, neovascular glaucoma secondary o
dal nevus. vascular obs ruc ion may occur.
Blacks and whi es are a ec ed equally. Jux apapillary choroidal melanoma

Malignan melanomas, by con ras , occur Choroidal nevus
much less requen ly in black pa ien s. Hyperplasia o he PE
Combined hamar oma o he re ina and
HISTORY PE
Pa ien s are usually asymp oma ic.

Visual loss may occur in rare ins ances
o umor grow h or re inal vascular Diagnosis is based on charac eris ic oph-
obs ruc ion. halmoscopic ea ures.
No inheri ance pat ern has been egular examina ions and serial undus
recognized. pho ographs are essen ial or documen a ion
o grow h or malignan rans orma ion.
IMPORTANT
MANAGEMENT
Charac eris ically, his charcoal gray or
gray-black umor is loca ed on or adjacen T e pa ien is ollowed by observa ion.
o he op ic disc, wi h bo h a deep choroidal
componen and superf cial ex ension in o he oughly 15% o melanocy omas exhibi
nerve f ber layer (Fig. 6-11). small degrees o enlargemen . However,
malignan rans orma ion is possible and is
I is usually unila eral. o en heralded by more rapid grow h and
A rela ive a eren papillary de ec (R PD) visual changes. In such cases, enuclea ion o
may be seen in up o 30% o cases. he a ec ed eye should be considered.
Choroidal Melanocytoma 263
FIGURE 6-11. Choroidal melanocytoma. Note the dark brown lesion involving the optic disc. T ere are
eathery edges to the lesion. A small percentage ( 15%) o melanocytomas will demonstrate some growth;
malignant trans ormation is rare but has been reported. Courtesy o Drs. Jerry and Carol Shields, Oncology
Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
CH
C H O RO
R O IDAL
I D AL ME
ME A
ASS ASIS
ASII S HISTORY
Blurred vision (usually painless) is

C horoidal me as asis is he mos common
in raocular malignancy, visible oph hal-
moscopically in up o 1% o pa ien s wi h
repor ed in 80% o pa ien s. Floa ers and
visual f eld de ec s are o her symp oms,
along wi h eye pain (5% o 15%). A his ory
sys emic malignancy (mos commonly aris-
o malignancy is no ed in 65% o 75% o
ing rom he lungs or breas s). Clinically i is
pa ien s.
apparen as an amelano ic, shallow, round or
oval choroidal mass pos erior o he equa or.
Choroidal me as asis is usually unila eral bu IMPORTANT
may be mul i ocal and bila eral. I is he pre- CLINICAL SIGNS
sen ing sign o me as a ic malignancy in up o
one- hird o he pa ien s. T e umor appears as a round or oval,
placoid, minimally eleva ed choroidal mass
EPIDEMIOLOGY ha is variable in color. I is uni ocal and
AND ETIOLO GY unila eral in roughly wo- hirds o he cases.
Me as ases rom breas and lung are ypically
T ere is a cumula ive li e ime incidence o pale yellow, whereas hose rom cu aneous
1 in 400 o 1 in 1000 Americans. melanoma are dark gray or brown
(Fig. 6-12). O her umors wi h charac er-
A au opsy, microscopic in raocular is ic colora ion include me as a ic renal cell
lesions are de ec able in 5% o 10% o and hyroid carcinoma ( ypically orange-red)
pa ien s wi h sys emic malignancy; o and me as a ic carcinoid umors ( ypically
hese, 10% have clinically apparen lesions. pink or yellow-orange).
he increasing incidence is likely a ribu -
able o increased survival o pa ien s wi h ypically he umor is loca ed pos erior o
cancer, improved de ec ion, and grea er he equa or (grea er han 90%); i is predomi-
awareness. nan ly macular in roughly 10%.
T e mos common primary malignancy umor grow h may resul in disc edema
is breas carcinoma in women and lung car- and serous re inal de achmen .
cinoma in men. In pa ien s wi h no known
primary si e a he ime o diagnosis, sys- ASSO CIATED
emic evalua ion leads o diagnosis o lung
carcinoma in one- hird o he pa ien s and
CLINICAL SIGNS
breas carcinoma in one- en h o he pa ien s.
Conjunc ival injec ion
Known primary si es o her han breas s
and lungs comprise less han 10% o cases Hyperopic shi
wi h choroidal me as asis. In one-hal o Neovascular glaucoma
he pa ien s, however, a primary si e is no
discovered.
umors are overwhelmingly carcinomas, DIFFERENTIAL DIAGNOSIS
rarely sarcomas. Spread is hema ogenous.
Cy ologic f ndings are consis en wi h he Amelano ic choroidal melanoma
umor o origin. Circumscribed choroidal hemangioma
Choroidal Metastasis 265
e inal as rocy oma blindness and secondary angle-closure

Choroidal os eoma glaucoma.
In raocular lymphoma Op ic disc edema causes pro ound and
o en irreversible visual loss. Fac ors rela ed o
Sclerochoroidal calcif ca ion
he preserva ion o vision and eye s ruc ures
Cen ral serous choriore inopa hy include number and size o umors, proximi y
Choroidal granuloma o he op ic nerve and ovea, and responsive-
Pos erior scleri is ness o herapy.
Vi elli orm dys rophy Survival is rela ed o e ec ive rea men
and remission o sys emic disease. Pa ien s
wi h choroidal me as asis rom breas car-
DIAGNOSTIC EVALUATION cinoma end o survive longer han pa ien s
wi h choroidal me as ases rom o her si es.
Fluorescein angiography o carcinoma
me as a ic o he choroid shows early hypo-
uorescence o he lesion wi h a rela ive MANAGEMENT
pauci y o in rinsic vessels, and di use hyper-
uorescence o he umor in he la e phases Ocular: Choroidal me as ases are mos
o he s udy. Indocyanine green angiography o en rea ed wi h ex ernal-beam radio her-
may demons ra e addi ional, smaller choroi- apy, chemo herapy, hormonal herapy, or a
dal umors. combina ion. Single, smaller umors may be
Ul rasonography is mos help ul or rea ed e ec ively wi h plaque radio herapy.
eleva ed umors; hey appear hyperechoic Sys emic: Pa ien s wi h sys emic disease
(brigh ) wi h high in ernal re ec ivi y. Fine- should be re erred o a medical oncologis .
needle aspira ion biopsy may be help ul, espe- Managemen o pa ien s wi h a known pri-
cially in pa ien s wi h no known malignancy. mary ex raocular malignancy requires a s ag-
ing evalua ion appropria e or he primary
PROGNOSIS umor. Pa ien s wi h no known primary si e
a ime o diagnosis are evalua ed wi h a hor-
Mos me as a ic umors are relen lessly ough physical examina ion (wi h at en ion o
progressive and end o grow as er han pri- breas s and lymph nodes), mammography,
mary choroidal malignancies. I un rea ed, ches roen genogram, C o he ches , abdo-
bullous re inal de achmen can lead o men, and pelvis, and bone and PE scans.
B
FIGURE 6-12. Choroidal metastasis. A. Metastatic lung cancer presents as an amelanotic choroidal lesion
( arrow) superior to the optic disc. Most choroidal metastases occur posterior to the equator. T ey may be
uni ocal or multi ocal. (Courtesy o Drs. Jerry and Carol Shields, Oncology Service, Wills Eye Hospital,
Philadelphia, Pennsylvania.) B. Choroidal metastasis to iris. Metastatic breast cancer presents initially to the
patient as an amelanotic iris mass.
Choroidal Hemangioma 267
Serous re inal de achmen in wo- hirds

CH
C H O RO
O IDAL
I D AL o he cases
H EM
EMAN
M ANN GI
GIOI O MA
MA Overlying PE me aplasia or re inos-
chisis (Fig. 6-13)
C horoidal hemangioma is a benign vas-
cular umor ha occurs in wo orms:
circumscribed (discre e) and di use. T e cir-
Di use ype
Brigh red color involving a large sec-
cumscribed varian is a well-def ned, orange- ion (more han 50%) o he undus
red, dome-shaped mass, usually occurring or uosi y o overlying re inal vessels
in he pos erior undus and diagnosed by
Shi ing subre inal uid
he our h decade o li e. T e di use varian
is charac eris ically associa ed wi h S urge– Mani es a ions o S urge–Weber syn-
Weber syndrome (encephalo rigeminal angio- drome include acial nevus ammeus (100%
ma osis) and is diagnosed a a younger age. I o pa ien s), cerebral abnormali ies, and in ra-
gives a markedly asymme ric, brigh red re ex cranial calcif ca ions. Asymme ric cupping o
o he undus o he involved eye. he op ic nerve (due o congeni al glaucoma)
occurs, par icularly in cases o nevus am-
meus involving he ipsila eral upper eyelid.
EPIDEMIOLOGY
ASSOCIATED
Circumscribed ype: Sporadic, usually CLINICAL SIGNS
diagnosed in hird or our h decade. May be
worsened by pregnancy. Usually unila eral. Choroidal neovasculariza ion (circum-
Di use ype: Diagnosed in childhood. scribed ype)
Occurs in roughly one- hird o he pa ien s e ini is pigmen osa-like changes (di use
wi h S urge–Weber syndrome. Usually unila - ype)
eral and ipsila eral o he charac eris ic acial
hemangioma (nevus ammeus, or “por -wine DIFFERENTIAL DIAGNOSIS
s ain”), bu may be bila eral and associa ed
wi h bila eral acial hemangiomas. Cen ral serous re inopa hy
Amelano ic choroidal melanoma
HISTORY Choroidal me as asis
Pa ien s may be asymp oma ic. Choroidal in amma ion
Visual impairmen is usually painless and
resul s rom hyperopic shi (in cases o sub-
macular umor) or subre inal uid.
Fluorescein angiography and indocyanine
green angiography reveal early hyper uores-
IMPORTANT cence corresponding o f lling o choroidal
CLINICAL SIGNS vessels eeding he umor.
B-scan ul rasonography demons ra es a
Circumscribed ype dome-shaped eleva ion in he case o a cir-
Discre e, sligh ly eleva ed, orange-red cumscribed hemangioma and marked choroi-
mass arising in he pos erior choroid dal hickening in cases o di use umors.
PROGNOSIS AND rea men consis s o radia ion herapy

MANAGEMENT (ex ernal-beam, cobal -60 brachy herapy,
or pro on-beam irradia ion). Delimi ing
Managemen consis s o observa ion as laser pho ocoagula ion, ex ernal drainage,
long as he pa ien s are asymp oma ic. In or scleral buckling is indica ed or re inal
cases o macular involvemen or ex ensive de achmen .
re inal de achmen , visual impairmen is
inevi able, and rea men is indica ed.
FIGURE 6-13. Choroidal hemangioma, circumscribed type. A macular choroidal hemangioma demonstrates
submacular uid and overlying retinal pigment epithelial metaplasia ( arrow) . T is lesion is o en con used with
central serous retinopathy. Courtesy o Drs. Jerry and Carol Shields, Oncology Service, Wills Eye Hospital,
Intraocular Lymphoma 269
IIN
N RRAO
AOO CULAR
C U L AR IMPORTANT
LYMPH
L YM P H O MA
MA CLINICAL SIGNS
Vi reous in amma ion

I n raocular lymphoma is a malignan , indo-
len , o en bila eral, lymphocy ic proli era-
ion wi h di use inf l ra ion o he pos erior
Mul iple, yellow-whi e lesions deep o he
re ina ha progressively enlarge and coalesce
segmen ha occurs in wo orms: (1) arising Overlying pigmen ary al era ions
primarily rom he eye or CNS; or (2) sys-
emic, usually visceral, lymphoma, me as a ic
ASSO CIATED
o he uvea. Mos o en i a ec s older pa ien s
and is associa ed wi h CNS lymphoma. CLINICAL SIGNS
In raocular lymphoma was ormerly ermed
reticulum cell sarcoma. An erior uvei is
e inal vasculi is, leading o vascular
EPIDEMIOLOGY obs ruc ion (Fig. 6-14)
AND ETIOLO GY Op ic disc edema
Usually in raocular lymphoma a ec s

immunocompe en pa ien s in heir six h o DIFFERENTIAL DIAGNOSIS
seven h decade. I is bila eral in up o 90%
o cases. A more aggressive orm is seen in Vi ri is
pa ien s who are severely immunocompro- Amyloidosis
mised, mos commonly rom he acquired Old vi reous hemorrhage
immunodef ciency syndrome (AIDS).
Senile vi ri is
In raocular lymphoma is he presen ing
e inal inf l ra es and vasculi is
sign o CNS lymphoma in 80% o pa ien s,
bu may precede CNS involvemen by up o oxoplasmosis
10 years (mean o 2 years). T e incidence Cy omegalovirus re ini is
appears o be increasing, possibly as a resul Acu e re inal necrosis
o more widespread immunosuppression or
Sarcoidosis
improved diagnosis.
Subre inal inf l ra es
Mos o en he lymphoma is a large-cell B
lymphocy ic (i.e., non-Hodgkin) umor. I is Mul i ocal choroidi is
hough o arise rom cells o he re ina, PE, Acu e pos erior mul i ocal placoid
brain, meninges, and spinal cord. pigmen epi heliopa hy
Mul iple evanescen whi e do syndrome
HISTORY Fundus avimacula us
Choroidal granuloma
Pa ien s have a his ory o oa ers and pain-
less visual loss. Amelano ic choroidal melanoma
Chronic, unremit ing vi ri is ha is unre- Choroidal me as asis
sponsive o cor icos eroids occurs, along wi h Bila eral di use uveal melanocy ic
neurologic impairmen . proli era ion
DIAGNOSTIC EVALUATION neuroimaging ( hin-sec ion magne ic reso-

nance imaging), lumbar punc ure, and bone
In he absence o known CNS disease, marrow biopsy.
vi reous biopsy ( hrough ei her f ne-needle Un rea ed, mos pa ien s die wi hin a
aspira ion or pars plana vi rec omy) has he ew years o diagnosis. Pa ien s wi h disease
grea es diagnos ic yield. Mul iple vi reous limi ed o he eye may be rea ed wi h
specimens may be needed. ex ernal-beam irradia ion only. Adjunc
chemo herapy and cor icos eroids pro-
PROGNOSIS AND long survival o a median o 41 mon hs
MANAGEMENT (wi h 22% disease- ree a 5 years), bu do
no decrease he risk o subsequen CNS
All pa ien s wi h in raocular lymphoma involvemen .
should be evalua ed or CNS and sys emic Ocular relapse, especially wi hin he f rs
involvemen , by neurologic examina ion, year a er rea men , is common.
FIGURE 6-14. Intraocular lymphoma. Hazy photograph demonstrating vitritis and intraretinal invasion o
lymphoma with associated hemorrhagic retinal vasculitis. Courtesy o Drs. Jerry and Carol Shields, Oncology
Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
Choroidal Osteoma 271
CH
C H O RO
O IDAL
I D AL
L OS
S EO
E O MA
A DIFFERENTIAL DIAGNOSIS
Amelano ic choroidal melanoma

C horoidal os eoma is a rare, benign cho-
roidal umor composed o ma ure bone,
occurring mos commonly in he pos erior
Carcinoma me as a ic o he choroid
Circumscribed choroidal hemangioma
pole o one eye o heal hy young women. Disci orm scar o age-rela ed macular
degenera ion
EPIDEMIOLOGY Idiopa hic sclerochoroidal calcif ca ion
AND ETIOLO GY
T e umor mos o en a ec s women
younger han 30 years o age. Diagnosis is based on ypical oph hal-
I is unila eral in 75% o cases and usually moscopic ea ures. Fluorescein angiography
sporadic, bu rare amilial cases have been reveals early mot led hyper uorescence and
repor ed. la e di use s aining. Leakage rom associ-
a ed choroidal neovasculariza ion may be
seen.
HISTORY
B-scan ul rasonography demons ra es a
Pa ien s are usually asymp oma ic. mildly eleva ed, highly re ec ive choroidal
mass wi h acous ic shadowing.
Diminished visual acui y or me amor-
phopsia are caused by macular involvemen C reveals ocal hyperin ensi y similar o
by he umor or by associa ed choroidal bone in he a ec ed choroid.
neovasculariza ion.
PROGNOSIS AND
CLINICAL SIGNS
Prognosis is variable. Visual loss may
T e umor is mos o en adjacen o, or resul rom degenera ion o he overlying
surrounding, he op ic disc. I appears pale PE, choroidal neovasculariza ion, or re inal
yellow o orange in color and is minimally de achmen .
eleva ed (usually less han 2 mm in hick- Managemen consis s o observa ion
ness). Overlying clumping o brown, gray, and, as appropria e, rea men o choroidal
or orange pigmen may be no ed (Fig. 6-15). neovasculariza ion.
ASSO CIATED
CLINICAL SIGNS
Choroidal neovasculariza ion

Serous re inal de achmen
FIGURE 6-15. Choroidal osteoma. Peripapillary choroidal osteoma is yellow-white in color and there are
some overlying areas o clumping o brown pigment. B-scan ultrasonography o choroidal osteoma shows
a highly re ective choroidal mass with acoustic shadowing behind the lesion; this is due to intrinsic bone.
Courtesy o Drs. Jerry and Carol Shields, Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
C H AP T ER
7
Congeni al and
Pedia ric Re inal Diseases
Nikolas J.S. London and Richard S. Kaiser
o he re ina. Con inued grow h and con rac-

RE
E IN
I N O PA
PA H Y O F
ion o f brovascular issue may lead o re inal
PREMA
PR
R EM
M A URI
U RI Y de achmen .
R e inopa hy o prema uri y (ROP) is a

proli era ive re inopa hy o prema ure,
low-bir h-weigh in an s.
HISTORY
T e cri ical componen s o he his ory are

he bir h weigh , he ges a ional age a bir h,
EPIDEMIOLOGY he curren pos mens rual age, and he curren
AND ETIOLO GY chronological age. One should also inquire
regarding he use o mechanical ven ila ion and/
Despi e recen advances, ROP remains a or supplemen al oxygen, he leng h o s ay in he
leading cause o visual loss in children. In an s neona al ICU, blood rans usions, he presence
born a less han 30 weeks ges a ion and/ o any bronchodysplasia or hyaline membrane
or hose wi h a bir h weigh o 1500 g or less disease. Pregnancy his ory, ma ernal heal h, and
should be examined by indirec oph halmos- amily his ory are also o en ob ained.
copy. In an s who are born weighing 1000 g or
less are a par icularly high risk o developing DIAGNOSTIC EVALUATION
severe ROP.
T e re inal vascula ure o in an s born Care ul examina ion o neona es wi h
prema urely has no comple ed i s grow h o indirec oph halmoscopy in he in ensive care
he ora serra a. T e developing vascula ure is nursery or a discharge is essen ial o de ec
suscep ible o capillary endo helial cy o ox- ROP a a s age where rea men can be applied.
ici y. Pre-exis ing vessels are damaged and Examina ions should s ar a 31 weeks
neovasculariza ion develops o he sur ace pos mens rual age or by 4 weeks chronological
273
274 7 CO NGENITAL AND PEDIATRIC RETINAL DISEASES
age, based on ges a ional age, and should TABLE 7-1. In erna ional Classif ca ion
con inue every 1 o 3 weeks, un il one o he o Re inopa hy o Prema uri y (ICROP)
ollowing occurs:
Location
T ere is vasculariza ion o zone III
Zone I Posterior circle o retina centered
wi hou prior zone I or II disease. on the optic nerve with a radius o
Pos mens rual age 45 weeks is achieved twice the disc- ovea distance
wi h no pre hreshold ROP. Zone II Circular area o retina rom the
T e re ina is comple ely vascularized. edge o zone I to the nasal ora
serrata
T ere is o al regression o rea ed ROP.
Zone III Remaining temporal crescent o
retina
IMPORTANT Extent
CLINICAL SIGNS Number o clock hours or 30-degree sectors involved
Severity
T e pos erior segmen signs o ROP ol-
low a progression o increasing severi y ha Stage 1 Demarcation line between
posterior vascularized retina and
were classif ed according o he In erna ional
anterior avascular retina
Classif ca ion o ROP (ICROP).
Stage 2 Demarcation line with height,
Firs he loca ion o he disease is de er- width, and volume (ridge)
mined, hen he s age o severi y, and f nally
Stage 3 Ridge with extraretinal
he ex en o ha s age is no ed (Table 7-1,
f brovascular proli eration
Figs. 7-1 to 7-5). (ERFP); may be mild, moderate,
or severe
ASSO CIATED Stage 4 Subtotal retinal detachment
CLINICAL SIGNS 4A Extra oveal
4B Involving the ovea
“Plus” disease deno es increased severi y. Stage 5 Total retinal detachment; always
When an eye has dila ion and or uosi y o unnel-shaped
he re inal vessels in he pos erior pole, hen Open anteriorly, open posteriorly
i is said o have plus disease and has a poorer
Open anteriorly, narrow
prognosis (Fig. 7-6). When vasculariza ion is
posteriorly
only presen in zone I and here is plus disease,
Narrow anteriorly, open
hen a very poor prognosis exis s because o
posteriorly
he risk o rapid progression (“rush” disease).
Narrow anteriorly, narrow
“T reshold” disease is a level o ROP posteriorly
a which i is predic ed ha here is a 50%
chance o progression o re inal de ach-
men wi hou rea men . I was def ned
in he CRYO-ROP s udy as 5 con iguous is reached, rea men is recommended. In
clock hours o ex rare inal f brovascular addi ion, curren guidelines indica e rea -
proli era ion (ERFP) wi h plus disease or 8 men wi hin 72 hours or he ollowing clini-
accumula ed clock hours o ERFP wi h plus cal si ua ions:
disease (Fig. 7-7). When hreshold disease Zone I, any s age, wi h plus disease
Retinopathy o Prematurity 275
Zone I, s age III, wi h or wi hou plus Di eren ial diagnosis depends on he

disease s age o he disease. In less severe ROP,
Zone II, s age II or III, wi h plus condi ions ha cause peripheral re inal vas-
disease cular changes and re inal dragging should be
considered.
Occasionally neovascular issue may orm
buds o vascular proli era ion behind he Familial exuda ive vi reore inopa hy
ridge or demarca ion line. T ese lesions are (FEVR)
called “popcorn” because o heir similar- Incon inen ia pigmen i (Bloch–
i y o popped corn (see Fig. 7-3A). T ese Sulzberger syndrome)
lesions do no carry any prognos ic signif - X-linked re inoschisis
cance unless hey are a par o ERFP a he
Norrie’s disease
advancing border o vasculariza ion. Vi reous
hemorrhage and prere inal hemorrhage can In advanced cica ricial ROP, he di eren-
occasionally be seen in eyes wi h severe ial diagnosis is ha o re inal de achmen or a
ERFP. whi e pupillary re ex, or bo h.
In early cica ricial ROP (a er resolu ion Ca arac
o he acu e phase) here may be a varie y o FEVR
rac ional complica ions. A hallmark f nding Persis en e al vascula ure (PFV)
o regressed cica ricial ROP is emporal re i-
Ocular oxocariasis
nal dragging (Fig. 7-8).
In ermedia e uvei is
A er regression o ROP, ei her spon ane-
ously or wi h rea men , several associa ed Coa s’ disease
abnormali ies may occur. Re inoblas oma
Myopia Vi reous hemorrhage
As igma ism Re inal de achmen
S rabismus Endoph halmi is
Amblyopia
Ca arac PROGNOSIS AND
Glaucoma MANAGEMENT
Macular ec opia
T e majori y o in an s who develop
Re inal old
ROP undergo spon aneous regression (85%).
Re inal de achmen —rhegma ogenous However, 7% o in an s weighing less han
or exuda ive 1251 g a bir h will develop hreshold ROP.
Cryo herapy o he an erior avascular zone
DIFFERENTIAL DIAGNOSIS (Fig. 7-9) has been shown o reduce he likeli-
hood o an un avorable ou come (re inal old
T e clinical set ing usually aids in he diag- hrough zone I, re inal de achmen , or re ro-
nosis o ROP. In an s are born prema urely len al f broplasia) by 50% in he cryo herapy
and are o low bir h weigh wi h a his ory o or ROP S udy. Visual resul s are commensu-
oxygen exposure. ra ely improved.
Laser pho ocoagula ion delivered wi h an early repor s o success. Bevacizumab

indirec oph halmoscope o he an erior avascu- elimina es he angiogenic hrea o ROP
lar zone has vir ually replaced cryo herapy or is a randomized rial comparing in ravi -
hreshold ROP (Fig. 7-10). Laser burns should real bevacizumab o conven ional laser
be spaced evenly, approxima ely one-hal burn rea men .
wid h apar , wi h par icular at en ion o he mos More advanced s ages o ROP (s ages
pos erior avascular re ina. Bet er visual ou comes 4 and 5 re inal de achmen ) can be rea ed
have been achieved wi h laser rea men . wi h scleral buckling or vi rec omy, or bo h.
An iangiogenic injec ion herapy T e prognosis or visual recovery a er re inal
or ROP is being inves iga ed, wi h de achmen remains poor.
12 12
CLOCK HOURS
ZONE III
ZONE III
ZONE II
ZONE II
ZONE I ZONE I
OPTIC 3
9 MACULA 3
N. 9
ORA SERRATA
RE LE
6 6
FIGURE 7-1. Retinopathy o prematurity (ROP). Schema ic diagram o division o undus in o zones or
def ning loca ion o involvemen wi h ROP.
FIGURE 7-2. ROP, stage 2. T e demarca ion line ( arrow) be ween pos erior vascularized and an erior
avascular re ina is eleva ed as a ridge.
B
FIGURE 7-3. ROP, stage 3. A. T e ridge has f brovascular issue and is eleva ed o he sur ace o he re ina
(ex rare inal f brovascular proli era ion, or ERFP) . T is is an example o severe s age 3 ROP. Buds o vascular
proli era ion behind he ridge are “popcorn” lesions ( arrow) . B. Fluorescein angiogram rom same pa ien as in
A showing in ense hyper uorescence rom vascular proli era ive issue in region o ridge.
FIGURE 7-4. ROP, stage 5. Eye wi h shallow o al re inal de achmen . No e loss o choroidal de ail due o
shallow subre inal uid accumula ion in pos erior pole.
FIGURE 7-5. ROP, stage 5. T is eye has a o al rac ional re inal de achmen due o severe f brous proli era ion
wi h con rac ion o he f brous issue. No e he vascularized issue behind he clear lens.
FIGURE 7-6. ROP, plus disease. Dila ion and or uosi y o vessels in he pos erior pole deno es progressive
disease and is known as “plus” disease.
12 12
CLOCK HOURS
ZONE III
ZONE III
ZONE II
ZONE II
ZONE I ZONE I
MACULA 3
9 3
9
ORA SERRATA
RE LE
6 6
FIGURE 7-7. ROP, threshold disease. Schema ic diagram o hreshold disease according o he Cryo herapy
or Re inopa hy o Prema uri y S udy Group. Reprin ed by permission o American Medical Associa ion rom
Cryo herapy or Re inopa hy o Prema uri y Coopera ive Group. Mul icen er rial o cryo herapy o re inopa hy
o prema uri y: Preliminary resul s. Arch Ophthalmol 1988;106:474.
FIGURE 7-8. ROP. Severe dragging o he emporal re ina wi h vascular s raigh ening (arrow) is a la e
cica ricial complica ion o ROP. Compare wi h amilial exuda ive vi reore inopa hy, in Figure 7-18A.
B
FIGURE 7-9. ROP, cr yotherapy. A. T e arrow poin s o he an erior avascular zone where cryo herapy will
be placed or hreshold ROP. B. La e pos opera ive appearance o cryo herapy. No e regression o ex rare inal
f brovascular proli era ion.
B
FIGURE 7-10. ROP. A. Preopera ive appearance o an eye wi h pos erior hreshold ROP. B. La e pos opera ive
appearance a er laser pho ocoagula ion. No e regression o ex rare inal f brovascular proli era ion.
T e ocular abnormali ies are o en

IN
N COO N IN EN
E N IA
A ex remely asymme ric.
PIGMEN
PI
I G MEN I
I ncon inen ia pigmen i, or Bloch–

Sulzberger syndrome, is a rare X-linked
dominan condi ion ha includes ocular, skin,
ASSO CIATED
CLINICAL SIGNS
cen ral nervous sys em, skele al, den al, and O her ocular f ndings include ca arac ,
o her sys emic abnormali ies. conjunc ival pigmen a ion, and s rabismus.
Skin f ndings, or which he disease is named,
EPIDEMIOLOGY include vesicular skin erup ions (Fig. 7-13)
AND ETIOLO GY ha la er urn in o depigmen ed lesions
(Fig. 7-14). T ese changes s ar days a er
T e pa hogenesis o incon inen ia pigmen i bir h.
is unknown. I is an X-linked dominan condi- Den al abnormali ies consis ing o miss-
ion ha is usually le hal in males and hus ing or cone-shaped ee h are presen in
usually only seen in emale in an s. However, approxima ely wo- hirds o a ec ed individ-
an a ec ed male wi h Kline el er syndrome uals (Fig. 7-15). Associa ed cen ral nervous
(XXY) or a gene ic mosaicism may survive. sys em abnormali ies include seizures, spas-
ic paralysis, and men al re arda ion.
HISTORY
Charac eris ic skin changes usually s ar DIFFERENTIAL DIAGNOSIS

days a er bir h, wi h oph halmic f ndings
developing in in ancy or even la er in li e. As or early s ages o ROP:
FEVR
IMPORTANT Incon inen ia pigmen i (Bloch–
CLINICAL SIGNS Sulzberger syndrome)
X-linked re inoschisis
Approxima ely one- hird o in an s wi h Norrie’s disease
incon inen ia pigmen i have ocular f ndings. I re inal de achmen is a presen ing f nd-
Fundus abnormali ies include dila ed, or u- ing, o her causes o in an ile re inal de ach-
ous re inal vessels, peripheral re inal capillary men should be considered.
nonper usion wi h ar eriovenous anas omo-
ses and neovasculariza ion (Figs. 7-11 and
7-12), oveal hypoplasia, branch ar ery occlu- DIAGNOSTIC EVALUATION
sions, neovasculariza ion o he disc, re inal
dragging, rac ional and rhegma ogenous Clinical evalua ion including examina ion
re inal de achmen s, re inal olds, vi reous o he skin may lead o a diagnosis o incon i-
hemorrhage, and op ic disc pallor. nen ia pigmen i.
Incontinentia Pigmenti 285
PROGNOSIS AND pho ocoagula ion or cryo herapy. In ravi real

MANAGEMENT an i-VEGF agen s have been used wi h lim-
i ed success.
Mos pa ien s wi h incon inen ia pigmen i Re inal de achmen s may be repaired wi h
require no rea men . scleral buckling or vi rec omy, or bo h.
Progressive peripheral re inal neo-
vasculariza ion may respond o laser
FIGURE 7-11. Incontinentia pigmenti. Peripheral undus o a pa ien wi h incon inen ia pigmen i showing
peripheral re inal capillary nonper usion and ar eriovenous anas omoses ( arrow) .
FIGURE 7-12. Incontinentia pigmenti. Fluorescein angiogram o he peripheral undus o a pa ien wi h

incon inen ia pigmen i showing peripheral re inal capillary nonper usion and ar eriovenous anas omoses ( arrow).
FIGURE 7-13. Incontinentia pigmenti, dermatologic f ndings. Vesicular skin lesions in an in an wi h

incon inen ia pigmen i.
Incontinentia Pigmenti 287
FIGURE 7-14. Incontinentia pigmenti, dermatologic f ndings. Pigmen ary al era ion o he skin in a pa ien
wi h resolved vesicular erup ions o incon inen ia pigmen i.
FIGURE 7-15. Incontinentia pigmenti, dental f ndings. Den al x ray o cone shaped oo h in a pa ien wi h
incon inen ia pigmen i.
Familial ExudativeVitreoretinopathy 289
Progression o more severe changes

FAM
FAMILIAL
M ILII AL E
EXUDA
XUDD A IIVE
VE
E such as rac ional, exuda ive, and even rheg-
VII R
V REO
EO
OR RE
E IN N O PA
A HY ma ogenous re inal de achmen may occur
(Fig. 7-19). In rare inal and subre inal lipid
FEVR is a group o au osomal- exuda ion may some imes occur.
dominan undus disorders charac erized
T e f ndings are o en asymme ric.
by peripheral re inal nonper usion and
neovasculariza ion.
ASSO CIATED
EPIDEMIOLOGY CLINICAL SIGNS
AND ETIOLO GY
In severe cases wi h re inal de achmen ,
here may be ca arac , band kera opa hy, neo-
T e exac e iology o FEVR is unknown. I
vascular glaucoma, ph hisis, or a combina ion
is an au osomal-dominan heredi ary disorder
o hese f ndings.
(rare cases o X-linked inheri ance have been
repor ed) ha is asymp oma ic in 50% o
cases. DIFFERENTIAL DIAGNOSIS
HISTORY As or early s ages o ROP:

FEVR
In an s born wi h FEVR are o herwise Incon inen ia pigmen i (Bloch–
heal hy. T ere is usually no his ory o pre- Sulzberger syndrome)
ma uri y, oxygen exposure, or respira ory
X-linked re inoschisis
di cul ies.
Norrie’s disease
T e clinical ea ures vary consider-
ably and, al hough bila eral, here is o en I re inal de achmen is a presen ing f nd-
asymme ry. ing, o her causes o in an ile re inal de ach-
men should be considered.
In an s may presen wi h s rabismus or a
whi e pupillary re ex i he f ndings are severe.
However, symp oms o decreased vision may DIAGNOSTIC EVALUATION
occur a any age.
ypical undus f ndings are no ed on
oph halmoscopy.
IMPORTANT
I FEVR is suspec ed, examina ion o he
CLINICAL SIGNS peripheral re ina o asymp oma ic amily
members may reveal f ndings consis en wi h
T e classic f nding in FEVR is peripheral he diagnosis.
re inal capillary nonper usion (Fig. 7-16).
Peripheral neovasculariza ion may orm a he
border o pos erior vascularized and an erior PROGNOSIS AND
avascular re ina (Fig. 7-17). Re inal dragging MANAGEMENT
rom con rac ion o f brovascular issue may
occur (Fig. 7-18). Vi reous hemorrhage rarely Pa ien s who are a ec ed a a young age
occurs. usually have more severe pa hology. Laser
pho ocoagula ion or cryo herapy o he All amily members o an a ec ed individ-

peripheral avascular re ina may preven pro- ual should be examined, because progressive
gression o f brovascular complica ions. changes may occur hroughou li e.
Scleral buckling and vi rec omy surgery
have been at emp ed or more severe cases
wi h re inal de achmen .
FIGURE 7-16. Familial exudative vitreoretinopathy (FEVR). Peripheral re inal capillary nonper usion
(arrow) in FEVR appears ea ureless.
Familial Exudative Vitreoretinopathy 291
B
FIGURE 7-17. FEVR. A. Clinical pho ograph showing peripheral re inal capillary nonper usion wi h
some in rare inal lipid exuda e (arrow) . B. Corresponding uorescein angiogram documen ing peripheral
nonper usion and areas o neovasculariza ion.
B
FIGURE 7-18. FEVR. A. emporal dragging o he re ina wi h vascular s raigh ening (arrow) . Compare wi h
ROP, Figure 7-8. B. emporal periphery wi h in rare inal lipid, peripheral re inal capillary nonper usion, and
peripheral neovasculariza ion ( arrow) .
Familial Exudative Vitreoretinopathy 293
FIGURE 7-19. FEVR. Severe rac ional re inal de achmen wi h dragging.

abnormali ies. T ese can occur on he re inal

COA
C O A S’ DISEASE
D IS
SE ASE
E ar erial or venous sides, or bo h.
C oa s’ disease is a unila eral, idiopa hic re - Marked hard exuda ion is requen ly pres-
inal vascular abnormali y f rs described en , ex ending rom he re ina in o he sub-
by George Coa s in 1908. I is charac erized re inal space when i is massive.
by elangiec a ic re inal vascular abnormali-
ies in associa ion wi h lipid exuda ion. Coa s’ IMPORTANT
disease should be di eren ia ed rom a Coats’ CLINICAL SIGNS
response, or a large degree o lipid exuda ion,
which can occur wi h abnormali ies such as elangiec a ic vessels, venous dila ion,
re ini is pigmen osa, diabe ic re inopa hy, re i- microaneurysms, and usi orm capillary dila-
nal venous obs ruc ion, re inal capillary hem- ion are he hallmark f ndings o Coa s’ dis-
angioma, and he la e sequelae o ROP. ease (Fig. 7-22).
Progressive exuda ion rom hese re inal
EPIDEMIOLOGY vascular abnormali ies may lead o exuda ive
AND ETIOLO GY re inal de achmen .
Coa s’ disease is idiopa hic. Mos cases are ASSO CIATED

diagnosed be ore age 20 wi h he peak inci-
CLINICAL SIGNS
dence a he end o he f rs decade.
I occurs predomina ely in males (85%) Pos erior segmen neovasculariza ion is
and is almos always uniocular. rare even hough re inal capillary nonper u-
T e severi y o he disease varies widely sion is o en presen . Re inal elangiec asis
rom asymp oma ic pa ches o elangiec a ic has been repor ed in associa ion wi h many
vessels in he re inal periphery (Fig. 7-20) o her ocular and sys emic diseases. Re ini is
o o al exuda ive re inal de achmen pigmen osa wi h a “Coa s’-like response” has
(Fig. 7-21). been documen ed.
O her en i ies associa ed wi h re inal el-
HISTORY angiec asia are Alpor ’s syndrome, uberous
sclerosis, urner’s syndrome, Senior–Loken
In an s may presen wi h s rabismus, leu- syndrome, he ich hyosis hys rix varian
kokoria, or a red, pain ul eye ( rom neovascu- o epidermal nevus syndrome, muscular
lar glaucoma). dys rophy, and ascio-scaspulohumeral
dys rophy.
T e severi y and ra e o progression are
grea es in younger pa ien s (less han 4 years
o age). DIFFERENTIAL DIAGNOSIS
Older children and, rarely, adul s may
complain o reduced vision in one eye. T e di eren ial diagnosis o Coa s’ disease
depends on he severi y o he disease being
considered.
PATHOPHYSIOLOGY
Childhood disease (leukokoria or exuda-
ive re inal de achmen )
T e re inal vessels become elangiec-
a ic and develop mul iple aneurysmal Re inoblas oma
Coats’Disease 295
Persis en hyperplas ic primary vi reous angiography may be help ul in de ec ing

(PHPV)/ PFV elangiec a ic re inal vascular abnormali ies.
ROP Charac eris ic “ligh bulb” aneurysmal
dila ions o larger re inal vessels are par icu-
FEVR
larly obvious on uorescein angiography
Norrie’s disease (Fig. 7-23). Re inal capillary nonper u-
Ocular oxocariasis sion may also be seen wi h uorescein
Von Hippel–Lindau disease angiography.
Peripheral exuda ive vi reore inopa hy In cases wi h a poor view o he undus,
ul rasonography is help ul and may exhibi a
Incon inen ia pigmen i (Bloch–
hyperechoic mass in he pos erior vi reous,
Sulzberger syndrome)
occasionally wi h evidence o vi reous and/ or
Pars plani is subre inal hemorrhage.
Re ini is pigmen osa wi h Coa s’-like
response PRO GNOSIS AND
Vi reous hemorrhage MANAGEMENT
Para oveal elangiec asia wi h or wi hou
lipid exuda ion Managemen consis s o observa ion;
Diabe ic re inopa hy cryo herapy or laser pho ocoagula ion may be
applied o areas o re inal vascular abnormali-
Radia ion re inopa hy
ies wi h progressive exuda ion (Fig. 7-24).
Jux a oveal re inal elangiec asia Mul iple rea men sessions are o en neces-
Branch re inal vein occlusion sary and close ollow-up is warran ed because
Localized elangiec asia wi h ar erial or recurrences have been repor ed up o 5 years
venous aneurysms a er comple e resolu ion.
Re inal cavernous hemangioma Vi rec omy and scleral buckling can be
considered or re inal de achmen , o al
Acquired re inal ar erial
re inal de achmen ypically resul s in
macroaneurysm
permanen , pro ound vision loss despi e
Idiopa hic re inal vasculi is, aneurysms, in erven ion.
and neurore ini is (IRVAN)
Enuclea ion should be considered or
eyes wi h no ligh percep ion and persis en
DIAGNOSTIC EVALUATION pain.
T e diagnosis is usually made hrough

oph halmoscopy; however, uorescein
B
FIGURE 7-20. Coats’ disease. A. Peripheral undus o an asymp oma ic pa ien wi h microaneurysms,
in rare inal hemorrhages, and lipid exuda es. B. Pos erior pole o he same pa ien wi h a normal macula.
Coats’Disease 297
FIGURE 7-21. Coats’ disease. o al exuda ive re inal de achmen .
FIGURE 7-22. Coats’ disease. Peripheral undus demons ra ing venous dila ion, microaneurysms, subre inal
lipid exuda ion ( arrow), and larger re inal vessels wi h “ligh bulb” aneurysmal dila ions (inse ) .
FIGURE 7-23. Coats’ disease. Fluorescein angiogram o he same pa ien as in Figure 7 22 clearly showing
dila ed re inal vessels wi h aneurysmal dila ions.
Coats’Disease 299
B
FIGURE 7-24. Coats’ disease. A. Peripheral undus showing aneurysmal dila ion and hemorrhage wi h some
exuda ion rom Coa s’ disease. B. Same area 4 mon hs a er cryo herapy showing involu ion o aneurysms and
resolu ion o hemorrhage and exuda es.
T ere is o en a pigmen ed border marking

CH
H O RIO
R IO
O RE
R E INAL
I NAL
L he ransi ion rom he coloboma o he nor-
CO
O LO
OBBOOMMA A mal re ina and choroid.
A choriore inal coloboma is a develop-

men al abnormali y caused by ailure o
comple e closure o he embryonic f ssure.
ASSO CIATED
CLINICAL SIGNS
T e coloboma may ex end back o he

EPIDEMIOLOGY op ic nerve (Fig. 7-26) and orward o
AND ETIOLO GY involve he ciliary body, zonules, and iris.
T e lens may be at ened a he pole cor-
Choriore inal coloboma is a congeni al
responding o he coloboma owing o he
developmen al abnormali y. T ere are rare
missing zonule (Fig. 7-27).
cases o au osomal-recessive inheri ance.
T e re ina and choroid are absen in areas
a ec ed. A hin in ercalary membrane cov- DIFFERENTIAL DIAGNOSIS
ers he sclera. T is membrane consis s o
rudimen ary re ina wi h blood vessels. T e Choriore inal scar (e.g., ocular
coloboma is loca ed in eriorly, because he oxoplasmosis)
embryonic f ssure is loca ed in eronasally in Degenera ive myopia
he developing eye.
HISTORY
Oph halmoscopy usually su ces o make
Pa ien s are usually asymp oma ic unless he diagnosis o choriore inal coloboma.
he op ic nerve or macula are involved or i
here are secondary e ec s. PROGNOSIS AND
Re inal breaks may occur in he in ercalary MANAGEMENT
membrane. Re inal de achmen may hen
occur and cause symp oms. No rea men is necessary unless re inal
de achmen occurs (Fig. 7-28).
Vi rec omy surgery is necessary o man-
IMPORTANT age he re inal de achmen associa ed wi h
CLINICAL SIGNS choriore inal coloboma. In ernal drainage
hrough he hole in he in ercalary membrane
A whi e area (sclera) is visible in he in e- is per ormed (i he hole can be ound) and
rior undus o a variable ex en depending on laser pho ocoagula ion is placed around he
he size o he de ec (Fig. 7-25). margins o he coloboma.
T e margins o he coloboma are well
def ned.
Chorioretinal Coloboma 301
FIGURE 7-25. Chorioretinal coloboma. Choriore inal de ec in he in erior undus.
FIGURE 7-26. Chorioretinal coloboma. Coloboma ous de ec involving he in erior undus and op ic nerve.
FIGURE 7-27. Chorioretinal coloboma. Ex ension o coloboma an eriorly. No e at ening o he in erior pole
o he lens (arrow) due o he missing por ion o he zonule.
Chorioretinal Coloboma 303
B
FIGURE 7-28. Chorioretinal coloboma. Pos erior pole (A) and in eronasal area ( B) o a pa ien wi h
choriore inal coloboma and associa ed rhegma ogenous re inal de achmen .
re rolen al membrane. T ere is a s alk o is-

PERSIS
P ERS
SI S E
ENN sue emana ing rom he op ic disc o he re -
H YPE
YPERPLAS
E RPLL AS IC C PRIMARY
P RII MA
AR Y rolen al area (Fig. 7-30).
VII R
V REO
EO U
US/
S/ PE
PERSIS
E R SII S ENN O en, an associa ed re inal old is loca ed
FE
F E AL
AL VVASCULA
ASCU U L A UREURRE in an in erior quadran .
P HPV was hough o be caused by ailure

o he primary vi reous o regress. Now
he erm PFV has been proposed o in egra e
ASSO CIATED
CLINICAL SIGNS
he f ndings ha occur when here is ailure o
regression o componen s o he e al vessels. Ca arac and narrow-angle glaucoma may
occur in he an erior orm o PFV i a dehis-
cence in he lens capsule occurs.
EPIDEMIOLOGY Severe cases may progress o re inal
AND ETIOLO GY de achmen and ph hisis bulbi.
PFV is a rare developmen al abnormali y.

T e condi ion is almos always unila eral
and is more common in males han emales. T e di eren ial diagnosis o he an erior
An erior and pos erior orms exis , and PFV is ha o leukokoria.
bo h may be presen oge her. T e di eren ial diagnosis o he pos erior
PFV is ROP, ocular oxocariasis, and FEVR.
HISTORY
In he an erior orm, in an s presen
wi h leukokoria due o a whi e, vascularized Clinical examina ion o he an erior seg-
f brous membrane behind he lens. men is impor an in es ablishing he diag-
In he pos erior orm, he eye may be nosis o PFV. I he an erior segmen is no
microph halmic bu he an erior segmen is a ec ed, he diagnosis o he pos erior PFV
o herwise normal. T e in an may presen wi h can be es ablished by oph halmoscopy.
leukokoria due o a persis en s alk o issue Ul rasonography and compu ed omo-
rom he op ic nerve o he re rolen al region. graphic (C ) scanning may help di eren ia e
PFV rom re inoblas oma, especially when
IMPORTANT he an erior segmen is involved.
CLINICAL SIGNS
PROGNOSIS AND
In he an erior orm, microph halmia, a MANAGEMENT
shallow an erior chamber and long ciliary
processes visible hrough he pupil are seen Pars plana vi rec omy, lensec omy removal
in associa ion wi h he whi e pupillary re ex o he f brovascular re rolen al membrane,
(Fig. 7-29). and an erior vi rec omy in he an erior PFV
In he pos erior orm, he eye may be help preven narrow-angle glaucoma, bu
microph halmic wi h a clear lens and no visual resul s are poor.
Persistent Hyperplastic Primary Vitreous/ Persistent Fetal Vasculature 305
FIGURE 7-29. Persistent hyperplastic primar y vitreous (PHP V)/ persistent etal vasculature (PFV).
An erior PHPV/ PFV in a microph halmic eye. No e he whi e re ex due o vascularized membrane behind he
lens and ciliary processes dragged in oward he cen er.
FIGURE 7-30. PHPV/ PFV. Ex ernal view o pos erior PHPV/ PFV. No e ermina ion o he s alk o issue on
he pos erior sur ace o he lens. Ciliary processes are no dragged in oward he cen er.
JuvenileX-Linked Retinoschisis 307
re inal pigmen epi helial al era ions in older

JUV
JUVEN
VEN
N ILE
E X-LIN
X-LL IN
N KED
KED
D pa ien s.
RE
E IN
NOOSCH
SC
C H IISIS
SIS
S
Peripheral re inoschisis is presen in
50% o pa ien s (Fig. 7-32). I is mos
J uvenile X-linked re inoschisis is a heredi-
ary disorder charac erized by di use
re inal dys unc ion wi h a s ella e macula in
common in eriorly and is a he level o he
nerve iber layer. O en, breaks occur in he
eleva ed nerve iber layer leaving unsup-
all cases and split ing o he nerve f ber layer
por ed re inal vessels ha can rup ure,
in 50% o cases.
causing vi reous hemorrhage (Fig. 7-33).
Breaks can occur in he inner layer causing
rhegma ogenous re inal de achmen
EPIDEMIOLOGY (Fig. 7-34).
AND ETIOLO GY
Juvenile X-linked re inoschisis is a bila - ASSO CIATED

eral inheri ed disorder occurring in males. CLINICAL SIGNS
Linkage s udies have localized he re inoschi-
sis gene o he dis al shor arm o he X chro- Less common indings include rac ion
mosome (Xp22.1–p22.3). or exuda ive re inal de achmen , ex en-
T e elec rore inographic abnormali ies sion o he re inoschisis in o he macula,
implica e Müller cell dys unc ion. macular ec opia wi h nasal or emporal
dragging, hyperme ropia, ca arac , and
s rabismus.
HISTORY
Pa ien s may presen wi h reduced vision DIFFERENTIAL DIAGNOSIS

rom he macular changes.
Earlier presen a ion occurs due o rou ine ROP
examina ion o amily members wi h a amily Goldmann–Favre disease
his ory o he disease. Re ini is pigmen osa
In pa ien s wi h severe peripheral involve- FEVR
men , vi reous hemorrhage resul ing rom
rup ure o unsuppor ed re inal vessels in he
eleva ed nerve f ber layer may cause abrup DIAGNOSTIC EVALUATION
loss o vision.
T e s ella e macula is o en bes seen wi h
red- ree illumina ion. Al hough i has he
IMPORTANT appearance o cys oid macular edema, here is
no leakage o dye on uorescein angiography.
CLINICAL SIGNS
Elec rore inography is use ul because pa ien s
Foveal schisis is presen in all cases and ypically have selec ive loss o he b-wave.
appears as a s ella e maculopa hy on oph hal- Examina ion o amily members, some
moscopic examina ion (Fig. 7-31). T e s el- o whom may have he disease, may aid in
la e appearance o en gives way o ill-def ned diagnosis.
PROGNOSIS AND Re inal de achmen repair can be

MANAGEMENT at emp ed wi h scleral buckling or vi rec omy
echniques.
T ere is no rea men or he macular Gene ic counseling should be o ered.
aspec s o he disease.
Vi rec omy can be per ormed or non-
clearing vi reous hemorrhage.
Juvenile X-Linked Retinoschisis 309
B
FIGURE 7-31. Juvenile X linked retinoschisis. A. Foveal schisis. B. High power image o oveal schisis; no e
radia ing re inal s riae.
FIGURE 7-32. Juvenile X linked retinoschisis. Highly bullous peripheral re inoschisis.

Juvenile X-Linked Retinoschisis 311
FIGURE 7-33. Juvenile X linked retinoschisis. In erior re inoschisis wi h large breaks ( arrow) and
unsuppor ed re inal vessels ( *).
FIGURE 7-34. Juvenile X linked retinoschisis. Rhegma ogenous re inal de achmen in a pa ien wi h juvenile
X linked re inoschisis. No e he s ella e appearance in he macula due o oveal schisis.
LEB
LEBER’S
BE R’’S CO
O N GEN
G E N I AL
L ASSO CIATED
AMAUROSIS
A MAAURR O SIS CLINICAL SIGNS
Many children are highly hyperopic.

L eber’s congeni al amaurosis is a group
o diseases charac erized by severe loss
o vision rom bir h associa ed wi h nys ag-
Older children may develop ca arac s and
kera oconus.
mus and severely impaired elec rore inogram
(ERG) responses or bo h rods and cones. DIFFERENTIAL DIAGNOSIS
EPIDEMIOLOGY Albinism
AND ETIOLO GY Congeni al s a ionary nigh blindness
Achroma opsia
Leber’s congeni al amaurosis is a heredi-
ary disease mos commonly inheri ed as an
au osomal-recessive rai .
Several gene ic de ec s have been Fundus examina ion is o en no
iden if ed. help ul because in an s may have a normal-
appearing undus. Ar eriolar a enua ion,
HISTORY op ic nerve pallor, and pigmen ary degen-
era ion may occur la er in childhood
Pa ien s usually presen because o nys- (Fig. 7-35).
agmus or s rabismus; however, paren s may Elec rophysiologic es ing is necessary o
be concerned earlier ha he in an does no es ablish a diagnosis. T e ERG is ypically
recognize aces. minimal or ex inguished.
IMPORTANT PROGNOSIS AND

CLINICAL SIGNS MANAGEMENT
T e undi are usually normal. Horizon al T ere is no rea men available.
nys agmus is presen .
Leber’s Congenital Amaurosis 313
FIGURE 7-35. Leber’s congenital amaurosis. Fundus o an older child demons ra ing one o he la e
pat erns o Leber’s congeni al amaurosis. No e pigmen ary re inopa hy and “macular coloboma,” choriore inal
degenera ion in he macula.
C H AP ER
rauma ic and oxic Re inopa hies

J. Luigi Borrillo and Carl D. Regillo
CO
O MMOT
MM
M M OT
O T IO
O RET
RE
ETT IN
INAE
A
AE Be mind ul o o her f ndings seen in
rauma such as hyphema or microhyphema,
C ommo io re inae is re inal whi ening ha

occurs a er blun ocular rauma. T is
ransien condi ion a ec s he ou er re ina.
choroidal rup ure, re inal hemorrhage, re inal
dialysis, avulsed vi reous base, and vi reous
hemorrhage.
O en he damage incurred o he pho orecep-
ors is reversible. DIFFERENTIAL DIAGNOSIS
EPIDEMIOLOGY O her en i ies ha may mimic commo io
re inae include branch re inal ar ery occlu-
S a is ically he condi ion is mos com- sion, whi e-wi hou -pressure, and shallow
mon in young males. re inal de achmen .
HISTORY
Pa ien s have a his ory o blun ocular
Diagnosis is based on clinical examina ion.
rauma.
Op ical coherence omography (OC )
CLINICAL SIGNS may demons ra e increased re ec ivi y
in areas o damaged pho orecep or ou er
T e condi ion is usually asymp oma ic. segmen s.
Decreased vision may occur wi h macular
involvemen . T e re ina appears whi ened, PROGNOSIS AND
while he re inal vascula ure remains una - MANAGEMENT
ec ed. T e re ina regains i s normal appear-
ance wi hin weeks (Fig. 8-1). Occasionally, Re inal whi ening resolves wi hou visual
changes o he re inal pigmen epi helium compromise. However, permanen visual acu-
(RPE), such as s ippling or clumping, may be i y loss can some imes occur i macular re inal
seen a er he re inal whi ening resolves. pigmen epi helial disrup ion is presen .
314
Commotio Retinae 315
FIGURE 8-1. Commotio retinae. A. Commo io re inae: Ou er re inal whi ening in he pos erior pole af er
blun rauma. B. Ou er re inal whi ening in he peripheral re ina.
316 8 RAUMA IC AND OXIC RE INO PA HIES
rup ures, commo io re inae, vi reous hemor-

CH O RO
O IDAL
ID
D AL RUPT
RU
U PT
T URE
U RE
E rhage, re inal ears, re inal dialysis, and orbi al
rac ures.
I n his injury, disrup ion o he choriocapil-
laris and Bruch’s membrane occurs second-
ary o rauma ic ocular compression. Rup ures
La er, choroidal neovasculariza ion
(CNV) may occur a he edge o he rup ure
loca ed in he pos erior pole are concen ric o si e (Fig. 8-2B).
he op ic disc. T ese breaks are o en associ-
a ed wi h subre inal and subre inal pigmen DIFFERENTIAL DIAGNOSIS
epi helial hemorrhage.
Myopic lacquer cracks and angioid s reaks
may have a similar appearance.
EPIDEMIOLOGY
AND ETIOLO GY O her causes o subre inal hemorrhage
include CNV, re inal ar erial macroaneurysm,
Choroidal rup ure occurs as a resul o Valsalva re inopa hy, and anemia.
rauma, mos commonly in young males.
HISTORY
rauma. PROGNOSIS AND
MANAGEMENT
CLINICAL SIGNS
Visual prognosis depends on he loca ion
T e charac eris ic f nding is subre inal o he choroidal rup ure wi h respec o he
hemorrhage, wi h whi ish, crescen -shaped ovea and any associa ed subre inal or subre i-
lesions around he op ic disc (Fig. 8-2A). nal pigmen epi helial hemorrhage.
In he acu e set ing, choroidal rup ures may Secondary CNV can occur a any ime dur-
be associa ed wi h o her f ndings rom blun ing he ollow-up period and can cause visual
ocular rauma such as hyphema, iris sphinc er acui y loss.
Choroidal Rupture 317
B
FIGURE 8-2. Choroidal rupture. A. Choroidal rup ure: Crescen -shaped lesion in he macula wi h subre inal
hemorrhage (inse ). B. Chronic choroidal rup ure: Yellow crescen -shaped rup ure si e wi h pigmen ed
choroidal neovasculariza ion ( arrow) and associa ed subre inal uid in he ovea.
An avulsed vi reous base may be associ-

AVULSED
AVUL
L SED
D VIT
VIT
T REO
O US
S BASE
E a ed wi h hyphema, iris sphinc er ears, com-
mo io re inae, vi reous hemorrhage, re inal
T his en i y describes separa ion o he vi -
reous base a he ora serra a ha occurs
secondary o rauma. Some imes he avulsed vi -
ears, re inal dialysis, or orbi al rac ures.
reous base can be seen oa ing in he periphery.

Avulsed vi reous base should be dis in-

EPIDEMIOLOGY guished rom re inal dialysis.
AND ETIOLO GY O her en i ies ha may mimic his condi-
ion include old vi reous hemorrhage.
T e injury occurs as a resul o rauma,
usually in young males.
HISTORY Diagnosis is based on binocular indirec
oph halmoscopy wi h scleral depression.
rauma.
PROGNOSIS AND
CLINICAL SIGNS MANAGEMENT
A semi ransparen , some imes pigmen ed, No rea men is necessary. Pa ien s
curvilinear ribbon-like s ruc ure may or may should be observed or he subsequen
no be comple ely separa ed rom he re inal developmen o rauma-rela ed ocular
periphery (Fig. 8-3). problems such as re inal breaks and angle-
recession glaucoma.
T e presence o an avulsed vi reous base is
pa hognomonic or ocular rauma.
FIGURE 8-3. Avulsed vitreous base. Nasal avulsed vi reous base ( arrow) is pa hognomonic or prior rauma.
Solar Maculopathy 319
Re inal pigmen epi helial changes ( ocal

SO
S OL
LAR
AR M
MACULO
AC
C U LO
O PAT
PA
AT H Y hyperpigmen a ion) may be eviden in he
oveal or para oveal area a la er s ages.
S olar maculopa hy is he visual loss resul ing
rom oveal pho orecep or and re inal pig-
men epi helial damage as a consequence o sun-
gazing or direc observa ion o a solar eclipse.
Pseudomacular hole, pho ic maculopa hy,
and macular dys rophy or degenera ion may
EPIDEMIOLOGY have a similar appearance.
T ere is a rend or higher incidence
in areas where a solar eclipse was direc ly
observable. Solar maculopa hy is more com-
mon among individuals involved in sun-
worshiping religious groups. Loss o he ozone Fluorescein angiography will reveal non-
layer has been associa ed wi h solar re inopa- specif c re inal pigmen epi helial window
hy among sunba hers in he Uni ed S a es. de ec s cen ered on he ovea.
OC may demons ra e disrup ion o oveal
HISTORY inner and ou er pho orecep or segmen s.
Pa ien s have a his ory o sungazing. PROGNOSIS AND

MANAGEMENT
CLINICAL SIGNS
Good visual recovery occurs in mos
T ere is an abnormal oveal re ex. pa ien s, bu i may ake weeks or mon hs or
O en, a sharply demarca ed yellow or red- vision o improve.
dish spo is visible in he ovea (Fig. 8-4). No rea men is needed.
FIGURE 8-4. Solar retinopathy. A. Color undus pho ograph demons ra ing deep yellow oveal lesion (inse ) .
( continued)
FIGURE 8-4. ( Continued) Solar retinopathy. B. Corresponding uorescein angiogram pho ograph
demons ra ing oveal window de ec . C. Acu e solar re inopa hy wi h yellow oveal lesion and hemorrhage.
Visual acui y is 20/ 30. ( B, Cour esy o Dr. Alexander J. Brucker.)
Valsalva Retinopathy 321
VALSALVA
VALS
SALV
VA RE
RET
ET IN
NOP
PAT
AT
THY DIFFERENTIAL DIAGNOSIS
Re inal macroaneurysm, diabe ic re i-

I n Valsalva re inopa hy, unila eral or bila eral
re inal or prere inal hemorrhage occurs as a
resul o an acu e episode o increased in ra ho-
nopa hy, venous occlusion, anemia, an i-
coagulan herapy, re inal ear, or pos erior
vi reous de achmen wi h associa ed hemor-
racic pressure. Superf cial capillaries rup ure
rhage may mimic he f ndings o Valsalva
secondary o a sharp rise in ocular in ravenous
re inopa hy.
pressure.
T e condi ion can occur in persons o any Diagnosis is based on clinical examina ion.
age. B-scan ul rasonography is per ormed o
evalua e or underlying re inal de achmen
HISTORY or re inal ear in he set ing o dense vi reous
hemorrhage.
Pa ien s usually have a his ory o recen OC may demons ra e hemorrhage
s renuous physical exer ion, coughing, vomi - undernea h re ec ive signal o he in ernal
ing, or s raining (e.g., wi h cons ipa ion). limi ing membrane.

Single or mul iple in rare inal hemor-
MANAGEMENT
rhages (o en undernea h he in ernal limi ing
T e visual prognosis is good.
membrane) are no ed in he pos erior pole
(Fig. 8-5). T ere may be a decrease in visual Managemen consis s o observa ion,
acui y when he hemorrhage is localized in or only, in mos cases. Hemorrhages will resolve
over he oveal region. spon aneously.
Subconjunc ival hemorrhage may be asso- Vi rec omy may be considered or non-
cia ed wi h his condi ion. Signif can vi reous clearing vi reous hemorrhage (bu is rarely
hemorrhage is rare. indica ed).
FIGURE 8-5. Valsalva retinopathy. Mul iple super cial re inal hemorrhages in he macula.
Shaken Baby Syndrome 323
hemorrhages resolve over he course o weeks

SH
S H AK
AKEN
KE N B
BABY
ABY
YSSYN
YN
N DRO
D RO
O ME
ME and are o en no associa ed wi h neurologic
sequelae.
T his condi ion re ers o in raocular hem-
orrhages in in an s or young children
secondary o child abuse. T e f ndings are
Leukemia and in raocular in ec ions may
also mimic he ocular f ndings o shaken baby
associa ed wi h decreased visual acui y and syndrome.
increased mor ali y.
EPIDEMIOLOGY
Clinical examina ion: Evalua ion or
T e condi ion is mos common in in an s delayed neurologic developmen and sys-
and oddlers. emic signs o child abuse
Head C o evalua e or in racranial
hemorrhage
HISTORY
Bone scan is more sensi ive in de ec ing
Recen ly, o en mul iple episodes o vio- rac ures and exposes he child o less radia-
len shaking o he in an precede f ndings, ion while providing a whole body skele al
al hough i is o en di cul o ob ain a his ory survey. Bone scan f ndings may ailor subse-
o abuse rom he care aker. Care akers may quen x-ray s udies.
re use diagnos ic evalua ion. X-ray s udies: Frac ures a di eren s ages
o healing, leg rac ures prior o bipedal
CLINICAL SIGNS ambula ion (prior o 12 mon hs o age), and
pos erior rib rac ures are highly suspicious
Subre inal, in rare inal, or prere inal or child abuse.
hemorrhages are no ed in one or (more com- Op ical coherence omography: Prere inal
monly) bo h eyes (Fig. 8-6A). T e prere inal hemorrhage, vi reore inal rac ion, and
hemorrhages are ypically globular as opposed hemorrhagic macular re inoschisis may be
o a (Fig. 8-6B). O her signs include poor observed.
visual or pupillary response. No single ocular
f nding is pa hognomonic. PROGNOSIS AND
Ecchymosis, long bone and rib rac ures, MANAGEMENT
le hargy, or developmen al delay are common
associa ed f ndings. O en he child has physi- A pedia ric consul a ion should be con-
cal f ndings ha do no ma ch he repor ed sidered o evalua e or child abuse. T e
mechanism o he injuries. prognosis is largely dependen on associa ed
brain injury. Poor pupillary response, poor
DIFFERENTIAL DIAGNOSIS visual acui y, and re inal hemorrhages have
been associa ed wi h high in an mor ali y.
Bir h rauma: In rare inal hemorrhages Conversely, he presence o good visual acui y
may be seen in newborns, especially wi h and normal pupillary re exes are associa ed
he use o orceps in he delivery. T ese wi h a bet er prognosis.
B
FIGURE 8-6. Shaken baby syndrome. A. Mul iple in rare inal and prere inal hemorrhages in he pos erior
pole. B. No e he globular na ure ( arrows) o he prere inal hemorrhage in shaken baby syndrome. ( A, Cour esy
o Dr. Richard Spaide.)
erson’s Syndrome 325
Spon aneous hemorrhages are a resul o vas-

T ERSO
ER
R SO N ’S S
SYN
YN D
DRO
R O ME
E cular abnormali ies such as aneurysms, ar e-
riovenous mal orma ions, or f s ulas.
T erson’s syndrome encompasses any in ra-
ocular (usually prere inal or vi reous)
hemorrhage associa ed wi h ei her rauma- DIFFERENTIAL DIAGNOSIS
induced or spon aneous acu e in racranial
bleeding. Pos erior vi reous de achmen wi h vi -
reous hemorrhage, re inal vein occlusion,
re inal ear, proli era ive diabe ic re inopa hy,
EPIDEMIOLOGY Valsalva re inopa hy, or re inal ar erial mac-
AND ETIOLO GY roaneurysm may mimic erson’s syndrome.
T e syndrome may a ec individuals o DIAGNOSTIC EVALUATION

any age.
T e sudden increase in in racranial pres- Neuroimaging is per ormed, using
sure direc ly or indirec ly rup ures he peri- compu ed omography (C ) or magne ic
papillary capillaries. resonance imaging (MRI). B-scan ul ra-
sonography is used o evalua e or re inal
HISTORY de achmen or re inal ear in cases where
vi reous hemorrhage precludes a view o he
T e presen a ion may include severe head- pos erior segmen . Op ical coherence omog-
ache or known acu e neurologic even . raphy f ndings may be similar o Valsalva
re inopa hy.
CLINICAL SIGNS
PROGNOSIS AND
Pa ien s may have varying degrees o MANAGEMENT
decreased visual acui y and mul iple, usu-
ally bila eral, re inal hemorrhages (Fig 8-7). T e visual prognosis is o en good.
Vi reous hemorrhage can also occur and may T ere can be a high mor ali y ra e depend-
be dense. ing on he loca ion and severi y o he in ra-
O her ocular signs associa ed wi h erson’s cranial hemorrhage.
syndrome include cranial nerve palsies, la e- Neurosurgical consul a ion is
appearing epire inal membrane, or rac ional recommended.
re inal de achmen . In cases o bila eral vi reous hemorrhage
T e in racranial hemorrhages are usu- or dense nonclearing vi reous hemorrhage,
ally loca ed in he subarachnoid space. vi rec omy may be considered.
B
FIGURE 8-7. erson’s syndrome. A. Mul iple re inal and prere inal hemorrhages in he pos erior pole o a
pa ien who has su ered an acu e subarachnoid hemorrhage. B. More severe re inal hemorrhaging and vi reous
hemorrhage in Terson’s syndrome.
Purtscher’s Retinopathy 327
PURTSCH
P UR
RT SC H ER’S
ER
R’S DIFFERENTIAL DIAGNOSIS
RET
R ET
T IN
I N O PAT
PAT H Y T is condi ion should be dis inguished
rom Pur scher’s-like re inopa hy, which has
P ur scher’s re inopa hy describes decreased
vision associa ed wi h in rare inal hemor-
rhages and pa ches o re inal whi ening sec-
a similar undus presen a ion associa ed wi h
microemboli o various composi ions rom a
wide spec rum o sys emic condi ions such
ondary o severe crushing injuries o he orso as pancrea i is, amnio ic uid embolism, col-
or head. T e undus f ndings are concen ra ed lagen vascular disease, hrombo ic hrombo-
in he peripapillary area and may be unila eral cy openic purpura, and long bone rac ures
or bila eral. (Fig. 8.8B).
Cen ral re inal ar ery and vein occlusion
EPIDEMIOLOGY may also mimic he f ndings o Pur scher’s
AND ETIOLO GY re inopa hy.
Persons o any age may be a ec ed.

Endo helial damage leads o in ravascular
coagulopa hy and granulocy ic aggrega ion
C imaging o ches and long bones is per-
wi h microemboli orma ion.
ormed, when indica ed.
Fluorescein angiography ypically reveals
HISTORY
areas o re inal ischemia.
T ere is a his ory o compressive rauma. Op ical coherence omography reveals
edema o he nerve f ber layer and subre inal
CLINICAL SIGNS uid.
Acu ely, severe vision loss is no ed in one PROGNOSIS AND

or bo h eyes. MANAGEMENT
Cot on-wool spo s cen ered on he op ic
disc, hemorrhages, exuda es, and re inal Permanen visual loss may occur in hal
edema are o en seen. o he a ec ed pa ien s. No rea men is
Fundus f ndings resolve over several weeks available.
(Fig. 8-8A).
Op ic a rophy may be a la e f nding.
B
FIGURE 8-8. Purtscher ’s retinopathy. A. Mul iple peripapillary cot on-wool spo s cen ered around he op ic
disc in a pa ien wi h massive ches rauma. B. Mul iple cot on-wool spo s, hemorrhage, and macular in arc ion
in a man wi h S ill’s disease.
raumatic Macular Hole 329
T e presence o a pos erior vi reous

TR
RAUMAT
AU MAAT IC
IC de achmen is unlikely.
MACULAR
M AC U LA
AR H O LE
E
T his en i y describes a ull- hickness mac-
ular hole occurring a er blun ocular
rauma. Solar re inopa hy
Non rauma ic macular hole
EPIDEMIOLOGY Pseudohole secondary o epire inal
AND ETIOLO GY membrane
Persons o any age may be a ec ed. DIAGNOSTIC EVALUATION

T e oveal de ec develops as a resul o
vi reous rac ion or con usion necrosis o he Care ul con ac lens biomicroscopy is
re ina and may be a direc consequence o indica ed.
globe de orma ion. Fluorescein angiography may reveal a
hyper uorescen spo a he ovea correspond-
ing o a re inal pigmen epi helial de ec .
HISTORY
Op ical coherence omography will reveal
T ere is a his ory o recen blun rauma or ull- hickness neurosensory re inal loss in he
whiplash injury. ovea.

MANAGEMENT
A ull- hickness macular hole wi h
irregular border is seen on clinical examina- T e visual prognosis is variable.
ion. Macular pigmen ary changes are o en Spon aneous closure o rauma ic macular
observed (Fig. 8-9), as well as a posi ive holes in young pa ien s has been repor ed in
Wa zke–Allen sign. he li era ure bu is rare.
O her associa ed signs may include sub- A recen series sugges s ha he macu-
macular hemorrhage, choroidal rup ure, lar hole surgery may be benef cial, wi h a
commo io re inae, or vi reous repor ed visual acui y o 20/ 50 or bet er in
hemorrhage. 64% o pa ien s.
B
FIGURE 8-9. raumatic macular hole. A. Small, irregular ull- hickness macular hole (inse ) and associa ed
macular re inal pigmen epi helial clumping. B. Larger, irregular rauma ic macular hole (inse ) wi h marked
re inal pigmen epi helial al era ions and a choroidal rup ure (arrow) in erior o he op ic disc.
Chorioretinitis Sclopetaria 331
pa h o he projec ile. O en hese areas are

CH
H O RIO
R IO RET
R ET IN
N IT
T IS
S covered by hemorrhage.
SCLO
SC
C L O PETARIA
P ET ARII A Fibrous proli era ion a he rup ure si es
even ually ensues and becomes visible as any
C horiore ini is sclope aria is ull- hickness
disrup ion o he choroid and re ina sec-
ondary o he concussive orces o a nonpen-
associa ed hemorrhage clears. In raorbi al or-
eign body is o en presen .
e ra ing high-veloci y projec ile.
EPIDEMIOLOGY
A rup ured globe should be ruled ou .
AND ETIOLO GY
Choroidal rup ures may demons ra e a
T e injury is mos common in young similar appearance as sclope aria, bu hey are
males. usually loca ed in he pos erior pole.
raveling a high speeds, he projec ile cre-
a es shock waves ha can rup ure choroid and DIAGNOSTIC EVALUATION
re ina bu leave sclera in ac . T e de ec s are
subsequen ly replaced by f brous issue. C imaging is impor an o evalua e he
in egri y o he globe, de ec any associa ed
orbi al or cen ral nervous sys em injuries, and
HISTORY
iden i y any orbi al oreign bodies.
Pa ien s have a his ory o a high-veloci y
projec ile, such as a BB pelle , o he orbi . PROGNOSIS AND
MANAGEMENT
CLINICAL SIGNS
Visual prognosis is dependen on he
T e visual acui y is variable. Acu ely, here loca ion o sclope aria. Lesions involving he
may be prere inal, in rare inal, or subre inal macular region have poorer visual acui y.
hemorrhage as well as vi reous hemorrhage Ini ial managemen is nonsurgical.
(Fig. 8-10A). Subsequen ly, lesions have a T e subsequen cica ricial process o en
“claw-like” branching pat ern (Fig. 8-10B). uses issues, making re inal de achmen s
Bare sclera may be visible hrough he ull- unlikely. Surgery is indica ed in he subse-
hickness de ec s o he choroid and overly- quen developmen o re inal de achmen
ing re ina. Sclope aria is usually loca ed in or or he removal o nonclearing vi reous
he peripheral undus, corresponding o he hemorrhage.
B
FIGURE 8-10. Chorioretinitis sclopetaria. A. Re inal whi ening and prere inal hemorrhage in he pos erior
pole o a pa ien sus aining an orbi al oreign body injury. B. Claw-like choriore inal scarring af er a high-veloci y
bulle passed hrough he orbi adjacen o he eye.
Intraocular Foreign Body 333
end o produce mild in amma ion. Glass,

IIN
NTRRAO
AOO CULAR
CUU L AR carbon, porcelain, silver, and pla inum are
FO
F OR
REIGN
E IG
G N BOODDY
Y iner .
I n raocular oreign body (IOFB) re ers o

he presence o a oreign body in he eye
rom a pene ra ing injury.
Endoph halmi is and uvei is may mimic

he f ndings o IOFB.
EPIDEMIOLOGY
AND ETIOLO GY
Young males are mos commonly a ec ed.
Orbi al C is he imaging modali y o
T e oreign body may serve as a nidus choice in de ec ing nonorganic IOFBs. Small
or endoph halmi is or severe in amma ory organic oreign bodies such as wood or veg-
reac ion. e able mat er may escape de ec ion.
An IOFB may also cause delayed e ec s B-scan ul rasonography may de ec
secondary o proli era ive vi reore inopa hy IOFBs, re inal ears, or re inal de achmen s
or oxici y. when he pos erior pole canno be visualized
(Fig. 8-11B).
HISTORY Serial elec rore inograms should be con-
sidered or re ained me allic oreign bodies o
Mos o en here is a his ory o pene ra ing evalua e or oxic me allosis.
ocular injury.
A oreign body sensa ion associa ed wi h
hammering, grinding, or o her mechanism
PROGNOSIS AND
o injury may also be elici ed. Injury in a MANAGEMENT
rural set ing increases he risk o secondary
endoph halmi is. Prognosis is largely dependen on he
loca ion and na ure o he IOFB. Repair
o he rup ured globe should be he f rs
CLINICAL SIGNS priori y. Promp removal o he IOFB via
pars plana vi rec omy be ore 24 hours has
Visual acui y is variable. An obvious or
been ound o decrease he incidence o
sub le poin o en ry o he IOFB may be seen
endoph halmi is.
(Fig. 8-11A). O her signs associa ed wi h
IOFBs include sec orial microcys ic corneal Considera ions in he managemen o
edema, irregular pupil, ransillumina ion iris IOFBs include he ollowing:
de ec , low in raocular pressure, in raocular Always consider an IOFB masquerade
in amma ion, or vi reous hemorrhages. La er syndrome in he set ing o unila eral unex-
f ndings include endoph halmi is, he ero- plained uvei is.
chromia, ca arac , or corneal deposi s. A C scan wi h 1-mm sec ions hrough
Subs ances such as vege able, iron, copper, he globe and orbi is he bes single imag-
and s eel may lead o in ense in amma ion ing scan. MRI may be superior or imaging
or in ec ion. Nickel, aluminum, and mercury wood and organic ma erial.
Copper, iron, and s eel produce he Promp surgical removal is recommended

mos in ense in raocular in amma ory or vege able, copper, iron, s eel, or iner sub-
response. s ances wi h oxic chemical coa ings. Surgical
Surgical removal o in raocular or- ex rac ion o cer ain me allic IOFBs may be
eign bodies is considered emergen and acili a ed by a magne .
should be per ormed as soon as possible. Sys emic and in ravi real an ibio ics
Endoph halmi is associa ed wi h IOFBs is should be adminis ered when here is a high
es ima ed up o 20%. index o suspicion or endoph halmi is.
Intraocular Foreign Body 335
B
FIGURE 8-11. Intraocular foreign body. A. Me allic oreign body in he re inal periphery. B. B-scan
ul rasonography demons ra ing acous ic shadowing ( arrow) behind he in raocular oreign body.
Cardiac evalua ion is impor an in

DISLO
DI
I SLO
O CAT
C AT ED
D LEN
LE N S evalua ion or aor ic aneurysm or
insu ciency.
T his erm describes he o al displacemen
o he na ive lens in o he an erior cham-
ber or he vi reous cavi y as a resul o lens
Weill–Marchesani syndrome: A ec ed
individuals have shor s a ure, seizures,
zonular disrup ion. microspherophakia, brachydac yly, and
may have decreased hearing.
Homocys inuria: Pa ien s may have
EPIDEMIOLOGY mar anoid ea ures, hrombosis (especially
wi h general anes hesia), and below-
T e condi ion is mos common in young average IQ.
males in he set ing o rauma. Individuals
O her: Syphilis, sulf e oxidase def -
wi h a amilial or me abolic predisposi ion
ciency, high myopia, Ehlers–Danlos
o zonular weakness are also prone o lens
syndrome, and aniridia are o her possible
disloca ion.
e iologies o lens disloca ion.
HISTORY
T ere is usually a his ory o blun ocular
rauma. O en, he pa ien no es a drama ic B-scan ul rasonography is used in
decrease in visual acui y because o lens he set ing o vi reous hemorrhage o
malposi ion. evalua e or lens posi ion and re inal
de achmen .
CLINICAL SIGNS Medical consul a ion is appropria e
i Mar an’s syndrome, homocys inuria,
T ere is decreased vision, and he na ural syphilis, or ano her predisposing condi ion is
lens is seen in he an erior chamber or pos erior suspec ed.
pole (Fig. 8-12). O her signs associa ed wi h
lens disloca ion may include iridodonesis, irreg-
ular an erior chamber dep h, eyelid ecchymosis,
PROGNOSIS AND
vi reous hemorrhage, or orbi al rac ures. MANAGEMENT
T e pa ien may have a physical habi us
Conserva ive managemen such as
compa ible wi h predisposing condi ions
observa ion and con ac lens wear may be
such as Mar an’s syndrome or Weill–
considered. Managemen o disloca ion o
Marchesani syndrome, among o hers.
he na ural lens in o he an erior chamber
causing glaucoma may include a rial o
DIFFERENTIAL DIAGNOSIS pupillary dila ion and supine posi ioning
in an at emp o disloca e he lens in o he
Condi ions o her han severe blun rauma vi reous cavi y.
ha predispose pa ien s o lens disloca ion A lens ha canno be reposi ioned in o
include: he vi reous cavi y or ha has a disrup ed
Mar an’s syndrome: Pa ien s are lens capsule may require vi rec omy and
o en all and may have arachnodac yly. lensec omy.
Dislocated Lens 337
FIGURE 8-12. Dislocated lens. In ac na ive lens in he vi reous over a glaucoma ous op ic disc. (Cour esy o
he Wills Eye Hospi al collec ion, Philadelphia, Pennsylvania.)
Can haxan hin oxici y: Pa ien s may

TALC
TAL
T LC R
RET
ET IN
N O PA
PAT
AT H Y volun eer a his ory o using oral anning
agen s. T e o al cumula ive dosage usually
T alc re inopa hy describes he presence
o an in rare inal, yellow, re rac ile sub-
s ance in pa ien s who abuse in ravenous
exceeds 19 g.
amoxi en (Nolvadex) use: O en here
drugs, especially hose using produc s made is a his ory o breas cancer and use o he
rom crushed able s or powder. an ies rogen agen or a cumula ive dosage
o a leas 7.7 g.
EPIDEMIOLOGY Re inal emboli associa ed wi h caro id
AND ETIOLO GY obs ruc ive disease or cardiac valve
disease. Caro id ul rasonography or
T e condi ion can occur in persons o any cardiac echography may reveal he embolic
age. source.
T e alc componen o he injec ed medi- In rare inal crys als
ca ion migra es hrough microvascular venu- alc par icles (in ravenous drug abuse)
lar o ar eriolar shun s in he lungs and in o Choles erol (Hollenhors plaque) or
he re inal ar erioles. o her emboli
Can haxan hin inges ion (oral anning
HISTORY agen )
amoxi en
Pa ien s have a his ory o chronic in ra-
venous drug abuse. A his ory o in rave- Me hoxy urane anes hesia
nous injec ion o crushed me hylphenida e Cys inosis
(Ri alin) able s may be elici ed. Para oveal elangec asia (Singerman’s
do s)
CLINICAL SIGNS In rare inal lipid (hard exuda es)
Irregular re rac ile elemen s are seen Subre inal crys als (o en associa ed wi h
hroughou he re ina (in ravascular space), RPE al era ions)
especially wi hin he macula (Fig. 8-13). Calcif ed drusen
O her in raocular signs ha may be seen Biet i’s corneal and macular crys alline
include neovasculariza ion o he re inal dys rophy
periphery (or op ic nerve), macular pucker or
f brosis, or vi reous hemorrhage.
Inspec ion o he skin may reveal evidence
o in ravenous drug abuse.
Fluorescein angiography may reveal areas
o nonper usion in he re inal periphery.
Op ical coherence omography
T e di eren ial diagnosis or alc re inopa- may reveal hyperre ec ive bodies cor-
hy includes o her causes o crys alline re i- responding o inner re inal crys alline
nopa hy such as: deposi s.
alc Retinopathy 339
PROGNOSIS AND Decreased visual acui y may also occur

MANAGEMENT secondary o macular f brosis associa ed
wi h in ravenous me hylphenida e abuse.
T e visual prognosis is variable. In he se - Peripheral laser rea men can cause regres-
ing o chronic in ravenous drug abuse, visual sion o re inal neovasculariza ion.
acui y may be decreased as a resul o macular
re inal capillary nonper usion or vi reous
hemorrhage.
B
FIGURE 8-13. alc retinopathy. A. Color undus pho ograph demons ra ing yellow re rac ile par icles wi hin
he re ina. B. Red- ree undus pho ograph highligh ing in rare inal macular re rac ile deposi s.
Chloroquine or Hydroxychloroquine Retinopathy 341
may display sys emic f ndings o rheuma oid

CH
C H LO
O RO O Q UIN E O R ar hri is or lupus ery hema osus.
H YDR
YDROXYCH
R OXXYC H LOOR
ROO Q UIN
U IN E
RET
R ET IN
I N O PAT
PAT H Y DIFFERENTIAL DIAGNOSIS
T his en i y describes degenera ion o he

RPE and neurosensory re inal dam-
age resul ing rom chronic daily inges ion o
Cone dys rophy
Chloroquine or hydroxychloroquine oxici y
chloroquine (Aralen) or hydroxychloroquine Benign concen ric annular dys rophy
(Plaquenil). Spielmeyer–Vog –Bat en disease
S argard ’s maculopa hy
EPIDEMIOLOGY Age-rela ed macular degenera ion
Fenes ra ed sheen macular dys rophy
T e condi ion occurs in individuals receiv-
ing chloroquine or an imalarial rea men DIAGNOSTIC EVALUATION
and in pa ien s wi h rheuma oid ar hri is or
lupus ery hema osus who ake hydroxychlo- Amsler grid evalua ion may allow he
roquine. Hydroxychloroquine is less likely pa ien o de ec early sco oma orma ion.
han chloroquine o cause re inopa hy.
Humphrey visual f eld es ing using red
ligh may be he mos sensi ive means o
HISTORY de ec ing a paracen ral sco oma.
T ere is a his ory o daily dosages exceed- Color vision es ing may reveal
ing 250 mg o chloroquine or 400 mg o dyschroma opsia.
hydroxychloroquine. Fluorescein angiography will show re inal
pigmen epi helial window de ec s in macular
CLINICAL SIGNS region.
Elec rore inography and elec rooculogra-
Visual acui y is variable. phy may be abnormal la e f ndings.
An abnormal oveal re ex and sub le Op ical coherence omography may reveal
para oveal re inal pigmen epi helial s ippling hinning o macular and para oveal re ina
precedes he developmen o a ring o re inal wi h loss o ganglion cell layer.
pigmen epi helial a rophy surrounding he
oveal region, known as he classic “bull’s-eye” PROGNOSIS AND
maculopa hy (Fig. 8-14). Re inal pigmen MANAGEMENT
epi helial dis urbance undernea h he ovea is
associa ed wi h decreased visual acui y. Promp discon inua ion o medica ion on
A paracen ral sco oma is he earlies sign de ec ion o oxici y usually preven s ur her
o chloroquine or hydroxychloroquine oxic- damage o he RPE and re ina.
i y and may precede he developmen o un- Pa ien s wi h mild re inal pigmen epi helial
dus f ndings. changes may rever o normal and re ain good
O her signs o chloroquine or hydroxy- visual acui y. In advanced cases, however, he
chloroquine re inopa hy include re inal vessel condi ion may worsen despi e cessa ion o he
at enua ion and corneal ver icilla a. Pa ien s medica ion, and visual loss may ensue.
B
FIGURE 8-14. Chloroquine retinopathy. A. Sub le para oveal re inal pigmen epi helial al era ions ( arrow)
in a pa ien wi h a his ory o long- erm chloroquine use. B. Bull’s-eye maculopa hy (inse ) associa ed wi h
chloroquine re inopa hy. ( A, Cour esy o Dr. Alexander J. Brucker; B, cour esy o he Wills Eye Hospi al
collec ion, Philadelphia, Pennsylvania.)
T ioridazine Retinopathy 343
Nummular re inal pigmen epi helial

T H IIO
O RIDAZIN
RID
D AZ IN
NE loss may be seen in in ermedia e s ages
RET
R ET INN O PAT
PA
AT H Y (Fig. 8-15).
La e ea ures o hioridazine oxici y
T hioridazine re inopa hy describes visual
dis urbance and pigmen ary re inopa hy
ha resul s rom chronic high-dose hiorida-
include op ic a rophy, re inal vessel at enu-
a ion, di use RPE, and choriocapillaris
a rophy.
zine use. Daily doses are more predic ive o
re inal oxici y han he o al cumula ive dose.
EPIDEMIOLOGY Gyra e a rophy, re ini is pigmen osa,
AND ETIOLO GY cancer-associa ed re inopa hy, choroideremia,
syphilis, viral choriore ini is, and rauma may
Individuals wi h psychia ric disorders demons ra e f ndings similar o hioridazine
requiring hioridazine. oxici y.
T e exac mechanism o re inal damage
is unclear al hough he medica ion is el o DIAGNOSTIC EVALUATION
accumula e in re inal pigmen epi helial cells.
Humphrey visual f eld es ing may reveal
HISTORY paracen ral sco omas or cons ric ion.
Fluorescein angiography may reveal a
T e use o hioridazine (Mellaril) in excess spec rum o re inal pigmen epi helial win-
o 800 mg per day has been associa ed wi h dow de ec s and disrup ion o he choriocap-
re inopa hy. illaris. T e elec rore inogram may be normal
in early s ages, bu demons ra e at enua ion in
CLINICAL SIGNS la er s ages.
Acu e oxici y is mani es ed by he sud- PROGNOSIS AND

den onse o visual dis urbance, nyc alopia, MANAGEMENT
or dyschroma opsia (red or brown colora ion
o vision). Re inal pigmen epi helial changes Cessa ion o he drug early in he course
can progress despi e drug cessa ion. o he oxici y may lead o reversal o visual
Early changes include granular re inal dis urbance. However, prolonged use o he
pigmen epi helial s ippling pos erior o he medica ion may lead o progressive visual loss
equa or. despi e drug cessa ion.
B
FIGURE 8-15. T ioridazine retinopathy. A. Widespread re inal pigmen epi helial and choriocapillaris
a rophy. B. Less widespread re inal pigmen epi helial and choriocapillaris a rophy may be mis aken or
geographic a rophy due o age-rela ed macular degenera ion. Visual acui y is 20/ 200.
( continued)
T ioridazine Retinopathy 345
C
FIGURE 8-15. ( Continued) T ioridazine retinopathy. C. Fluorescein angiogram showing re inal pigmen
epi helial a rophy as well as nummular areas o choroidal vascular loss ( arrows) .
C H AP T ER
9
Peripheral Re inal Disease
James F. Vander
RE
R E INAL
IN
NAAL BREAK
BR EA
AK O R EAR
EA
AR Many breaks are asymp oma ic.
I associa ed wi h re inal de achmen
R e inal break or ear describes a ull-

hickness de ec in he re ina, generally in
he re inal periphery, al hough he break may
(RD), hen progressive visual f eld loss occurs.
occur anywhere. Breaks occurring as he resul IMPORTANT

o vi reous rac ion are known as re inal ears. CLINICAL SIGNS
ypes o re inal breaks

EPIDEMIOLOGY
AND ETIOLO GY Horseshoe ( ap) ears: T e horseshoe
is open an eriorly. Vi reore inal rac ion
Re inal breaks are common. T ey are o en persis s (Fig. 9-2).
more requen in myopia, pseudophakia, and Opercula ed ears: A ragmen o re ina
a er rauma. is orn comple ely ree o he re ina and
Many cases are bila eral and mul iple. oa s above i . T e vi reous rac ion is
relieved (Fig. 9-3).
Re inal ears are caused by vi reous rac-
ion, mos commonly ound wi h degenera ive A rophic re inal break: Usually round,
vi reous lique ac ion and pos erior vi reous o en small, holes; no associa ed wi h rac-
de achmen (Fig. 9-1). O her re inal breaks ion (Fig. 9-4).
(see la er discussion) resul rom developmen- Re inal dialysis: Disinser ion o he re ina
al or degenera ive abnormali ies or rauma. rom he pars plana a he ora serra a; mos
o en in ero emporal; superonasal is vir ually
HISTORY pa hognomonic or rauma (Fig. 9-5).
Gian ear: A ear grea er han 90
Re inal ears are o en associa ed wi h degrees, spon aneous or post rauma ic
oa ers and ashing ligh s (pho opsia). (Fig. 9-6).
346
Re inal Break or Tear 347
S re ch/ necro ic ears: rauma ic ears Con ac lens examina ion o he periphery
o variable size, o en irregular in orien a- may help conf rm he presence and na ure o
ion wi h jagged edges; hemorrhage or he break.
o her signs o rauma (Fig. 9-7).
PRO GNOSIS AND
ASSO CIATED MANAGEMENT
CLINICAL SIGNS
T e risk o developing RD (and, here-
Look or predisposing condi ions (see ear- ore, indica ion or prophylac ic rea men )
lier discussion) depends on he ype o break presen .
Pigmen cells in he vi reous (“ obacco Symp oma ic ears wi h persis en rac ion
dus ”) (horseshoe ears, gian ears) have a high
risk o subsequen RD and are rea ed when
70% wi h hemorrhagic pos erior vi reous recognized. Asymp oma ic ap ears have
de achmen (PVD) a lower risk bu are o en prophylac ically
Vi reous hemorrhage [ ears are ound in rea ed. Symp oma ic opercula ed ears also
70% o pa ien s wi h hemorrhagic proli era- have a much lower risk o subsequen RD.
ive vi reore inopa hy (PVR)] rea men is more con roversial. Dialysis
Subre inal uid accumula ing around he ear and o her post rauma ic ears are generally
Pigmen around he base o he re inal rea ed when recognized. rea men or
break (indica es chronici y) a rophic breaks, asymp oma ic opercula ed
ears, and breaks wi h pigmen around hem
almos never require prophylac ic rea men .
DIFFERENTIAL DIAGNOSIS Excep ions migh include pa ien s wi h a
his ory o RD o he ellow eye, an icipa ed
Vi reore inal u ca arac surgery, or a s rong amily his ory o
Meridional old or complex re inal ears or RD.
Ou er wall hole in re inoschisis rea men op ions include cryo herapy
Cobbles one degenera ion or laser pho ocoagula ion (Figs. 9-8–9-10).
Lat ice degenera ion Developmen o he indirec oph halmo-
scopic laser delivery sys em has acili a ed he
use o laser rea men and reduced he need
DIAGNOSTIC EVALUATION or cryo herapy, which generally produces
more pain during rea men . Pa ien s wi h
Indirec oph halmoscopy wi h scleral cloudy media or signif can subre inal uid
depression is cri ical. may be bet er rea ed wi h cryo herapy.
348 9 PERIPHERAL RET INAL DISEASE
B
FIGURE 9-1. Pos erior vi reous de achmen (P VD). A. Annulus o condensed vi reous ( Weiss ring) ( arrow)
f oa ing in ron o he op ic disc a er PVD. B. Pho ograph ocused on Weiss ring.
B
FIGURE 9-2. Horseshoe re inal ear. A. Horseshoe ear wi h a ew f ecks o hemorrhage and a small amoun o
subre inal f uid surrounding i . B. Large horseshoe ear wi h a bridging re inal vessel.
( continued)
C
FIGURE 9-2. ( Continued) Horseshoe re inal ear. C. Horseshoe ear wi h associa ed re inal hemorrhages
(arrow) . Vi reous eleva es he f ap ear.
FIGURE 9-3. Opercula ed re inal ear.

FIGURE 9-4. Peripheral cys oid degenera ion. Peripheral cys oid degenera ion wi h associa ed a rophic
re inal holes.
FIGURE 9-5. Re inal dialysis. Wide-angle view o in ero emporal dialysis wi h associa ed re inal de achmen .
FIGURE 9-6. Gian re inal ear. Rolled edge o a gian re inal ear.
A
FIGURE 9-7. Post rauma ic re inal ear. A. S re ch ear a er blun rauma (arrows o re inal de achmen ) .
( continued)
C
FIGURE 9-7. ( Continued) Post rauma ic re inal ear. B. wo s re ch ears a er blun rauma. C. rauma ic
macular hole.
FIGURE 9-8. Re inal ear, pre rea men and post rea men . A. Small horseshoe ear ( arrow) . B. Immedia ely
a er laser pho ocoagula ion.
B
FIGURE 9-9. Re inal ear, pre rea men and post rea men . A. Horseshoe ear wi h bridging vessel.
B. Immedia ely a er laser pho ocoagula ion.
B
FIGURE 9-10. Re inal ear, post rea men . A. Horseshoe re inal ear immedia ely a er laser pho o-
coagula ion. B. Several weeks a er laser pho ocoagula ion or re inal ear in ano her pa ien . Laser marks have
become pigmen ed.
Iden i ying he re inal break (o en small

RH
R H EGMA
E G M A O GEN
G E N O US
US and di cul o f nd) is he key. Pseudophakic
RE
R E INAL
IN
N AL DEE ACH
AC H MMEN
EN RDs are o en caused by small, pinpoin re i-
nal holes a he vi reous base and may be di -
R hegma ogenous RD is a separa ion o

he re ina rom he underlying re inal
pigmen epi helium (RPE) by uid ha gains
f cul o de ec .
access o he subre inal space via one or more

ASSO CIATED
ull- hickness re inal breaks. CLINICAL SIGNS
Pigmen : Granules in he vi reous are

EPIDEMIOLOGY almos always seen. Hyperpigmen a ion or
AND ETIOLO GY loss o pigmen a he RPE is common, espe-
cially in chronic RD. Linear pigmen (“demar-
Epidemiology is he same as ha or re i- ca ion line”) sugges s chronici y and may be
nal breaks since, by def ni ion, a re inal break mul iple (Fig. 9-12).
is presen and he cause o he RD. Hypo ony: Rela ive o he ellow eye. T is
Re inal breaks develop in o rhegma og- is no invariably presen . In chronic rheg-
enous RD by a combina ion o vi reore inal ma ogenous RD, pressure may be normal or
rac ion and uid curren s ha cause vi reous even high.
uid o move hrough he re inal break(s) O her ea ures o chronic RD: Re inal
and overcome he normal at rac ive orces neovasculariza ion, ca arac , an erior uvei is,
be ween he pho orecep ors and he RPE. rubeosis iridis, and re inal cys s (Fig. 9-13).
HISTORY DIFFERENTIAL DIAGNOSIS
T ere is a progressive loss o he visual Re inoschisis

f eld (o en described as a cur ain or shadow Exuda ive RD
blocking he vision), requen ly accompanied
rac ional RD
or preceded by oa ers and ashing ligh s.
Choroidal de achmen
Pa ien s wi h peripheral RDs may be asymp-
oma ic or simply have ashes and oa ers.
IMPORTANT Indirec oph halmoscopy wi h scleral
CLINICAL SIGNS depression is he key. Con ac lens examina-
ion may help f nd small peripheral re inal
In addi ion o iden i ying he re inal breaks.
break(s), he re ina is seen o be eleva ed by
Sli -lamp examina ion o he an erior vi re-
subre inal uid (Fig. 9-11). T e re ina loses
ous conf rms vi reous pigmen .
i s ransparency o a variable degree, o en
becoming ranslucen wi h a corruga ed B-scan ul rasonography conf rms re inal
appearance. T ere is undula ion wi h eye eleva ion in cases wi h media opaci ies.
movemen . Chronic rhegma ogenous RD Examine he ellow eye o look or
may appear ransparen and no undula e. re inoschisis.
PROGNOSIS phakic RDs wi h a single, superior re inal

break wi hou vi reous hemorrhage, ex en-
Chronic, asymp oma ic RD may remain sive lat ice degenera ion, or early PVR do
s a ionary and no require rea men . bes . T is rela ively noninvasive, low-cos ,
Spon aneous regression o RD can occur quick-recovery o ce procedure is gaining in
bu is rare (Fig. 9-14). accep ance.
Mos RDs and vir ually all symp oma ic emporary balloon: T is rea men con-
de achmen s will progress, causing severe sis s o an ex ernal device applied via small
permanen visual loss i un rea ed. Visual conjunc ival incisions. T e balloon emporar-
po en ial is direc ly rela ed o he presence ily inden s he sclera o allow cryo herapy or a
and dura ion o macular involvemen laser-induced choriore inal adhesion o orm.
(Fig. 9-15). T is rea men op ion is especially use ul or
in erior RD when pneuma ic re inopexy is
RDs no involving he macula o en
no an op ion; however, i is no a widely used
recover vision ully.
echnique.
“Macula-o ” RDs usually lead o per-
Scleral buckle: T is widely applied ech-
manen reduc ion o cen ral vision even
nique consis s o inden a ion o he sclera
when repaired properly. Recovery o en
using a exible silicone sponge or s rip ha is
akes mon hs, and he degree o recovery
permanen ly su ured on or wi hin he sclera
diminishes wi h longer periods o macular
o relieve vi reore inal rac ion on he re inal
de achmen .
break(s). Cryo herapy is generally used o
crea e permanen adhesion al hough pos op-
Management era ive laser herapy can be applied. Drainage
Laser pho ocoagula ion: Used alone i has o subre inal uid or injec ion o in ravi real
a limi ed role in managemen o RD. Usually gas, or bo h, are also some imes per ormed o
i canno seal a re inal break closed in he assis in reat achmen . Success ra es o over
presence o subre inal uid. Laser rea men 95% have been repor ed or repair o primary
may be used o crea e a barrier (“wall o he RD. Side e ec s and complica ions o he
de achmen ”) o preven progression o he scleral buckle include:
de achmen . I is especially use ul in chronic Pain
in erior RD or in cases where sys emic illness In ec ion
preven s more def ni ive repair.
Hemorrhage (especially wi h drainage
Cryo herapy (see preceding discussion o o subre inal uid; Fig. 9-16)
Laser Pho ocoagula ion): Occasionally RDs
wi h very shallow uid around he re inal Re inal incarcera ion a drainage si e
break can be cured by rea ing he break wi h (Fig. 9-17)
cryo herapy alone. Induced myopia
Pneuma ic re inopexy: An in ravi real gas Diplopia
bubble is used o amponade he re inal break Ex rusion or in rusion (Fig. 9-18)
closed emporarily. T e subre inal uid will An erior segmen ischemia
resolve and ei her laser pho ocoagula ion or
cryo herapy is used o permanen ly close he P osis
re inal break(s). T e success ra e is high and Vi rec omy: T is echnique is increas-
varies wi h pa ien selec ion. Pa ien s wi h ingly used in managing primary RD wi h or
wi hou a scleral buckle. I allows or direc Eleva ed in raocular pressure

release o vi reore inal rac ion. In ravi real Ca arac
gas or silicone oil is used o amponade he
Disloca ion o in raocular lens
re ina while re inopexy wi h laser or cryo-
herapy akes e ec . Vi rec omy is especially In ec ion
use ul or RDs wi h pos erior breaks, PVR, Hemorrhage
vi reous hemorrhage, or a igh orbi preven - Pos opera ive posi ioning (e.g.,
ing scleral buckle. Side e ec s and complica- ace-down) may be needed
ions o vi rec omy include:
A
FIGURE 9-11. Rhegma ogenous re inal de achmen (RD). A. Rhegma ogenous RD wi h a small horseshoe
ear ( arrow) .
( continued)
C
FIGURE 9-11. ( Continued) Rhegma ogenous re inal de achmen (RD). B. RD wi h bullous eleva ion and
corruga ed appearance o he de ached re ina. C. RD wi h a small horseshoe ear ( arrow).
B
FIGURE 9-12. Rhegma ogenous RD, chronic. A. Chronic RD ( right of arrows) wi h pigmen ed demarca ion
line ( arrows) . B. Chronic RD ( black arrow) wi h mul iple demarca ion lines and some subre inal brous bands
(white arrows) .
B
FIGURE 9-13. Rhegma ogenous RD, chronic. A. Re inal cys s associa ed wi h chronic RD. B. B-scan
ul rasound o re inal cys ( short arrow) and chronic RD ( long arrow) .
FIGURE 9-14. Rhegma ogenous RD, regressed. Pigmen a ion rom spon aneously regressed RD.
FIGURE 9-15. Rhegma ogenous RD, pigmen ar y dis urbances. Subre inal dispersion o pigmen a er repair
o “macula-o ” RD.
B
FIGURE 9-16. Rhegma ogenous RD, subre inal hemorrhage. A. Subre inal hemorrhage benea h he at ached
re ina associa ed wi h drainage o subre inal f uid during scleral buckling surgery. B. Macular ex ension o
subre inal blood complica ing drainage o subre inal f uid.
FIGURE 9-17. Rhegma ogenous RD, re inal incarcera ion. Incarcera ion o he re ina in o he drainage si e
during scleral buckle.
FIGURE 9-18. Scleral buckle. Ex ruding scleral buckle.

PVR may be primary or develop

PRO
OLLIFERA
IF
F ERA
A IVE
IV
VE a er at emp ed repair o RD. I ends o
VI R
REO
EO
ORRE
E IN O PA
A HY occur 3 weeks o 3 mon hs a er ini ial
repair.
PVR re ers o he developmen o prere i-
nal, subre inal, and even in rare inal f brous
proli era ion ha induces rac ion and dis- HISTORY
or ion o he re ina in he presence o RD
(Table 9-1). T e same as or RD.
EPIDEMIOLOGY IMPORTANT
AND ETIOLO GY CLINICAL SIGNS
In he presence o RD, ac iva ion o T e re ina is rela ively immobile wi h f xed

vi reous glial cells and me aplasia o re inal olds.
pigmen epi helial cells produces f brous Rolled edges o he re inal break(s) and
issue ha proli era es on and under he ex ensive pigmen in vi reous, on and under
re ina. re ina are no ed (Fig. 9-19).
Al hough he mechanism is no o ally Vi reous bands are prominen .
unders ood, risk ac ors include RDs wi h
mul iple re inal breaks, large re inal breaks,
chronici y, vi reous hemorrhage, and ASSO CIATED
rauma. CLINICAL SIGNS
TABLE 9 1. Classi ca ion o Proli era ive Hypo ony

Vi reore inopa hy An erior are or uvei is
Grade A Vi reous haze, vi reous pigmen clumps, Rubeosis iridis
and pigmen clus ers in erior o re ina
Grade B Inner re inal wrinkling, re inal s if ness, DIFFERENTIAL DIAGNOSIS
vessel or uosi y, rolled edge o break,
and decreased vi reous mobili y
Purely rac ional RD (e.g., diabe ic re i-
Grade C P (pos erior)—expressed in he nopa hy; Fig. 9-20)
number o clock hours involved (1–12)
Exuda ive RD
Focal, dif use, or circum eren ial ull-
hickness olds, subre inal s rands
A(an erior)—expressed in he number DIAGNOSTIC EVALUATION
o clock hours involved (1–12)
Focal, dif use, or circum eren ial ull- Diagnosis is based on indirec
hickness olds; subre inal s rands; oph halmoscopy.
condensed vi reous; and an erior Ul rasound may show he rigid na ure
displacemen o vi reous base wi h o RD i de achmen canno be direc ly
an erior rough.
visualized.
PROGNOSIS AND Repair almos always includes vi rec omy,

MANAGEMENT o en wi h a high encircling scleral buckle as
well as a long-las ing vi reous gas or silicone
PVR almos always progresses, causing oil amponade.
severe visual loss. Repair is di cul , Recurrence ra e is high, and recovery o
especially or more advanced degrees o excellen visual acui y is uncommon in severe
PVR (see Table 9-1). cases (Figs. 9-21 and 9-22).
B
FIGURE 9-19. Proli era ive vi reore inopa hy (P VR). A. High magni ca ion view o PVR wi h radia ing
re inal olds. B. Wide eld view showing re inal olds radia ing rom op ic disc.
FIGURE 9-20. Trac ional RD associa ed wi h proli era ive diabe ic re inopa hy. Wide- eld view o rac ion
re inal de achmen wi h regressed neovasculariza ion producing a ring o bro ic rac ion.
FIGURE 9-21. PVR, recurren RD. Recurren RD wi h severe PVR. No e severe brous proli era ion on he
re inal sur ace and associa ed re inal breaks.
B
FIGURE 9-22. PVR, f xed re inal olds. A. PVR wi h macular pucker ( long arrow) a er vi rec omy and scleral
buckle or RD. No e he edge o he slowly clearing vi reous gas bubble ( short arrow) used during he ini ial
repair. B. In erior proli era ion causing rac ional eleva ion o he re ina (arrow) .
inheri ance, consis s o vi reore inopa hy wi h

LA ICE DEGEN ERA IO N ar hropa hy and o her sys emic ea ures.
L at ice degenera ion is a peripheral undus Re inal breaks: A rophic round holes
abnormali y consis ing o re inal hinning are o en presen wi hin he lat ice
wi h loss o inner re inal issue and unusually degenera ion, some imes wi h associa ed
s rong vi reore inal adhesion a he edges o subre inal uid. rac ional ap ears may
he re inal excava ion. occur, usually a he edges o he lat ice
(Fig. 9-24).
EPIDEMIOLOGY
AND ETIOLO GY DIFFERENTIAL DIAGNOSIS
T e condi ion is common, occurring in Cobbles one degenera ion

abou 8%o he popula ion. I is more common Cys oid degenera ion
and more ex ensive in pa ien s wi h myopia. Re inoschisis
Vi reore inal u
HISTORY
Pigmen ed or a rophic choriore inal
scars
Pa ien s are asymp oma ic excep when
he condi ion is associa ed wi h re inal ear Grouped pigmen a ion
and de achmen orma ion.
IMPORTANT
CLINICAL SIGNS Indirec oph halmoscopy is per ormed
wi h scleral depression.
Lat ice degenera ion can be variable in Peripheral con ac lens examina ion may
pigmen a ion, circum eren ial ex en , and help conf rm he presence o associa ed
orien a ion (usually concen ric wi h ora ears.
serra a bu may be radial or perivascular)
(Fig. 9-23).
PROGNOSIS AND
Cross-ha ched whi e lines, which give he
MANAGEMENT
appearance o a lat ice, are o en no presen .
Indirec oph halmoscopy is usually necessary
Usually his condi ion is o no clinical
o see lat ice degenera ion.
signif cance.
Dynamic scleral depression is he key o
T e risk o RD increases wi h he ex en
apprecia ing he inner re inal excava ion.
o lat ice degenera ion. Prophylac ic rea -
men (laser or cryo herapy) generally is no
ASSO CIATED indica ed excep i he ellow eye had lat ice-
CLINICAL SIGNS rela ed RD.
A s rong amily his ory o re inal ear
Myopia or RD or an icipa ed in raocular surgery
S ickler’s syndrome: T is syndrome, are o her possible considera ions or
which has an au osomal-dominan rea men .
Lat ice Degenera ion 373
FIGURE 9-23. Pigmen ed lat ice degenera ion. No e he lat ice-like ne work wi hin he area o increased
pigmen a ion.
FIGURE 9-24. Lat ice degenera ion wi h associa ed horseshoe ear and RD (arrow). A horseshoe ear a he
edge o lat ice ref ec s rm vi reore inal adhesion a he edge o he lat ice degenera ion.
discre e re inal eleva ion (Fig. 9-25). T ere

VII R
V REO
E O RE
R E INAL
I N ALL UF F may be pigmen around he u . T ey o en
AN
A ND MMERIDIO
E R I DII O N
NAAL
AL FO
O LD
D are mis aken or small ap ears, bu no ull-
hickness break is presen .
V i reore inal u and meridional old are

common s ruc ural abnormali ies o
he ex reme re inal periphery wi h unusually
Meridional olds are olds o redundan
re ina (Fig. 9-26). Mos o en superonasal,
hey may s raddle and are perpendicular
s rong vi reore inal adhesion. T ey are in re-
o he ora serra a. Small re inal breaks may
quen ly he cause o rhegma ogenous RD.
develop a heir pos erior end.

AND ETIOLO GY
Re inal ear
Vi reore inal u s are very common in he
“Snowball” or o her in amma ory
general popula ion and are he source o RD
precipi a e
in less han 1% o all a ec ed pa ien s.
Meridional olds are very common in he
general popula ion and are he source o RD PROGNOSIS AND
in less han 1% o all a ec ed pa ien s. MANAGEMENT
T ey are a developmen al abnormali y.
Bo h abnormali ies are almos
always inciden al f ndings o no clinical
HISTORY impor ance.
Rarely, hey may be he only abnormal
T e abnormali ies are asymp oma ic
f nding in cases o RD and are presumed o
excep when hey precipi a e RD.
be he cause o he de achmen .
T ey are generally rea ed wi h cryo her-
IMPORTANT apy or laser pho ocoagula ion a he ime o
CLINICAL SIGNS RD repair, bu prophylac ic rea men is no
warran ed.
Vi reore inal u s are small areas o sub-
s an ial ocal vi reous rac ion producing a
Vi reore inal Tu and Meridional Fold 375
FIGURE 9-25. Vi reore inal u . Focal opaci ca ion and eleva ion o he re ina occurs a wo gray-whi e
vi reore inal u s.
FIGURE 9-26. Schema ic o meridional old. T e old represen s a plea o re ina be ween he ora bays.
O en a pigmen ed halo is no ed.

CO
C O BBLES
BBLEES O N E
T e degenera ion is mos common in eri-
DEGEN
D EG E N E
ERA
R A IO
ON orly and is usually bila eral.
C obbles one degenera ion, also called pav-

ing s one degenera ion, describes dis-
cre e circular areas o peripheral a rophy o he
re ina, RPE, and choriocapillaris. Re inal breaks

Lat ice degenera ion
EPIDEMIOLOGY Congeni al hyper rophy o RPE
AND ETIOLO GY
T is is a degenera ive process o unknown
e iology. Diagnosis is based on indirec
T e condi ion is more common in he oph halmoscopy.
elderly.
PROGNOSIS AND
HISTORY MANAGEMENT
Pa ien s are usually asymp oma ic. Cobbles one degenera ion is o no clinical
impor ance. I is no a predisposing condi ion
or RD and, in ac , may be pro ec ive agains
IMPORTANT a progressive RD.
CLINICAL SIGNS
Circular areas o hinning o he re ina

wi h depigmen a ion are seen (Fig. 9-27).
Cobbles one Degenera ion 377
FIGURE 9-27. Cobbles one ( paving s one) degenera ion. Areas o pigmen a ion are observed wi hin he
discre e areas o depigmen a ion.
Lat ice degenera ion

PERIPH
P ER
R IPH
H ERAL
E R AL GR
GRO
R O UPED
UP
P ED
D
Choriore inal scar
PIGMEN
P IGM
M ENN A IO ON
Impor an : Peripheral grouped
P eripheral grouped pigmen a ion describes pigmen a ion mus be dis inguished rom
a clus er o a , discre e pigmen ed spo s he pigmen ed spo s in he undus ha
deep o re ina. are seen in amilial polyposis (Gardner’s
syndrome). T is au osomal-dominan
condi ion, which is usually asymp oma ic,
EPIDEMIOLOGY o en includes a , variably pigmen ed
AND ETIOLO GY spo s in he undus. Lesions o Gardner’s
syndrome end o be more oval, wi h
I may occur a any age, in bo h genders. an irregularly pigmen ed “come ’s ail”
T is is a congeni al abnormali y. (Fig. 9-29). A ec ed pa ien s have a very
high risk o colonic carcinoma. Fundus
lesions in Gardner’s syndrome are usually
HISTORY seen as early as in ancy. For pa ien s wi h a
posi ive amily his ory, he presence o undus
Pa ien s are usually asymp oma ic.
lesions is vir ually diagnos ic o he sys emic
syndrome.
IMPORTANT
CLINICAL SIGNS DIAGNOSTIC EVALUATION
A clus er o a , uni ormly pigmen ed spo s Diagnosis is based on indirec
o variable size are o en no ed (Fig. 9-28). oph halmoscopy.
T ese are also known as “bear racks” because
For suspicious lesions (see preced-
o heir paw-prin appearance. Rarely, he pig-
ing discussion), ob ain a amily his ory o
men a ion is bila eral.
gas roin es inal malignancy and consider
colonoscopy.
ASSO CIATED
T ere are no signs o in amma ion, uid,
MANAGEMENT
or eleva ion.
Peripheral grouped pigmen a ion is o no
clinical impor ance wi h no po en ial or RD
DIFFERENTIAL DIAGNOSIS or malignan rans orma ion.
Cobbles one degenera ion

Choroidal nevus or melanoma
Peripheral Grouped Pigmen a ion 379
FIGURE 9-28. Grouped pigmen a ion. Small clumps o grouped pigmen a ion (“bear racks”; inse ) have
lit le risk o malignan rans orma ion.
FIGURE 9-29. Pigmen ed undus lesions associa ed wi h Gardner’s syndrome. No e he depigmen ed halo
(arrowhead) and oblong shape o he lesion.
De ec ion o an in ac , overlying, inner

DEGEN
D E G E N ERAA IVE
IV
VE layer is acili a ed by no ing re inal vessels
RE
R E IN
INO OSCH
SC H IS
ISIS
SIS
S coursing over he hole.
An absolu e visual f eld de ec corresponds
Degenera ive re inoschisis is a split ing o
o he area o re inoschisis.
he re ina ha produces eleva ion o he inner
re ina, mimicking a de achmen .
EPIDEMIOLOGY
AND ETIOLO GY Rhegma ogenous RD: T e presence o a re -
inal break, corruga ions, undula ions, pigmen
T e condi ion is a degenera ive process in vi reous or demarca ion lines, symp oms,
ha begins wi h peripheral cys oid degenera- hypo ony, and a ellow eye ha is normal help
ion. Fur her split ing in he ou er plexi orm dis inguish his condi ion rom re inoschisis.
layer leads o he eleva ion no ed clinically. Exuda ive RD: Associa ed umor or
Degenera ive re inoschisis a ec s bo h in amma ory signs, shi ing uid, and symp-
genders and all races. oms sugges his e iology.
Juvenile re inoschisis (X-linked): T is
heri able condi ion produces a s ella e oveal
HISTORY
appearance wi h split ing neurosensory re ina
wi hin he nerve f ber layer. I may presen as
Pa ien s are asymp oma ic excep in rare
vi reous hemorrhage.
cases o progressive RD.

CLINICAL SIGNS Evalua ion consis s o indirec oph hal-
moscopy wi h scleral depression and con ac
A ransparen dome-shaped eleva ion o
lens examina ion.
peripheral re ina may be easily overlooked
(Fig. 9-30). T e ellow eye should also be examined.
A hin, cys ic appearance is no ed.
PROGNOSIS AND
T e in ero emporal undus is mos o en
a ec ed. In he majori y o cases he condi-
MANAGEMENT
ion is bila eral.
Degenera ive re inoschisis rarely produces
T e re ina is immobile, and here is no asso- vision loss. Progression o pos erior re ina or
cia ed pigmen a ion o he RPE or vi reous. developmen o rue rhegma ogenous de ach-
men is uncommon.
ASSO CIATED Ou er wall de ec s usually are easily
CLINICAL SIGNS de ec ed, bu inner wall breaks are di cul o
iden i y. In cases o very pos erior re inoschi-
Whi e “snow akes” on he underside sis wi h progression, ou er wall breaks may
o he inner re inal layer or a pockmarked be surrounded wi h laser rea men
appearance may be no ed. (Fig. 9-32).
Ou er wall holes are circular, and here are I he ou er layer is de ached as well, hen
some imes mul iple de ec s (Fig. 9-31). surgery, usually vi rec omy, is needed.
Degenera ive Re inoschisis 381
B
FIGURE 9-30. Peripheral re inoschisis. A. No e he smoo h, dome-shaped eleva ion in he in ero emporal
quadran ( arrows) . Degenera ive re inoschisis B. Wide-angle pho ograph o in ero emporal re inoschisis
( lower le ) . T e ex ernal ransillumina ion is no ed (direc ly le ) .
FIGURE 9-31. Degenera ive re inoschisis, ou er wall breaks. Ou er wall holes associa ed wi h re inoschisis
(inse ) are required o cause a re inoschisis-associa ed re inal de achmen . No e ha he inner-layer re inal
vessels course over he ou er-layer re inal holes.
FIGURE 9-32. Degenera ive re inoschisis, ou er wall breaks. Laser rea men ( arrows) surrounding ou er
wall holes (as erisks) in re inoschisis.
Choroidal me as asis
EXU
EXUDA
U DA
A IVE
IV
VE RE
E INAL
IN
N AL
L
Re inoblas oma
DE AC
ACH
C H MEN
ME
EN
Miscellaneous
E xuda ive RD describes eleva ion o he

re ina by uid ha leaks rom wi hin or
under he re ina.
Bullous cen ral serous choroidopa hy
Uveal e usion syndrome
Nanoph halmos
Coloboma
EPIDEMIOLOGY
AND ETIOLO GY HISTORY
By def ni ion, he re inal eleva ion is no Pa ien s experience a progressive, o en
due o a re inal break. uc ua ing loss o peripheral vision, ha is
Breakdown o he normal inner (re inal similar o rhegma ogenous RD, bu o en
vascular endo helial cells) or ou er blood– more variable in course.
re inal barrier (RPE) produces exuda ion o Visual changes may be posi ional, caused
uid, eleva ing he re ina. by shi ing subre inal uid wi h changes in
Cases can be divided in o one o our ca - head posi ion.
egories, as ollows: I in amma ory in na ure, he condi ion may
Inf ammatory cause pain; however, i is o en asymp oma ic.
Scleri is
Harada’s disease IMPORTANT
Sympa he ic oph halmia CLINICAL SIGNS
Orbi al pseudo umor and o her
T ere is a dome-shaped eleva ion o
orbi al in amma ion
he re ina, which re ains i s ransparency
In ec ious re inochoroidi is (e.g., (Fig. 9-35).
oxoplasmosis, syphilis, Lyme disease,
T e subre inal uid generally shi s o
bar onellosis)
he mos gravi y-dependen posi ion wi h
Vasculi is or au oimmune (e.g., changes in he pa ien ’s head posi ion.
lupus, polyar eri is nodosa)
Vascular ASSO CIATED
Coa s’ disease (Fig. 9-33) CLINICAL SIGNS
Re inal capillary hemangioma
Acu e sys emic hyper ension Observe or ea ures o he underlying
cause, such as in amma ion, vascular changes,
Eclampsia
or solid umor (see Fig. 9-34).
Dissemina ed in ravascular coagula-
ion (DIC)
Renal ailure
Neoplastic Rhegma ogenous RD (especially chronic
Choroidal melanoma (Fig. 9-34) wi h small re inal break) di eren ial diagnosis
Re inoschisis Calcif ca ion sugges s re inoblas oma.

Choroidal de achmen Iden i y ea ures o choroidal umor or
hickening.
A-scan ul rasound: Shor axial leng h is
ypical o nanoph halmos.
Fluorescein angiography: O en
shows source(s) o subre inal uid and he PROGNOSIS AND
na ure o he de ec causing i (see discus- MANAGEMENT
sion under “Epidemiology and E iology,”
earlier). T e prognosis varies markedly, depend-
B-scan ul rasound: T ickening o sclera ing on he underlying e iology. rea men
is seen in scleri is or orbi al in amma ion. depends on he underlying cause.
FIGURE 9-33. Exuda ive RD associa ed wi h Coa s’ disease (arrow). No e he subre inal lipid precipi a es
where he exuda ive RD is shallower.
FIGURE 9-34. Exuda ive RD overlying choroidal melanoma wi h re inal hemorrhages. T is smoo h, dome-
shaped eleva ion ex ends beyond he choroidal melanoma.
nerve and macula. I eleva ions are very large,

CH
C H O ROO IDAL
ID
D AL hey may con ac each o her in vi reous cavi y
DE
D E AACH
C H MEN
MEN (“kissing choroidals”).
C horoidal de achmen re ers o eleva ion

o he re ina and choroid by ei her accu-
mula ion o serous uid or blood in he supra-
ASSO CIATED
CLINICAL SIGNS
choroidal space.
Hypo ony
EPIDEMIOLOGY Marked eleva ion o in raocular pressure
(hemorrhagic choroidals)
AND ETIOLO GY
Vi reous hemorrhage
Serous choroidal de achmen is gener- Re inal olds or de achmen
ally a secondary e ec o ano her underlying Shallow an erior chamber
problem.
Causes include:
Hypo ony
Wound leak Choroidal melanoma
Pos glaucoma surgery Choroidal me as asis
Cyclodialysis cle RD
In amma ion Re inoschisis
High scleral buckle Scleral buckle
Hemorrhagic choroidal de achmen may
occur spon aneously, in raopera ively a er DIAGNOSTIC EVALUATION
rauma, or as he resul o vascular abnormali-
ies such as choroidal neovasculariza ion. I choroidal de achmen is associa ed wi h
hypo ony, look or he cause (e.g., gonioscopy
HISTORY or cyclodialysis cle ).
B-scan ul rasonography conf rms
Occurrence o a visual f eld de ec or
he diagnosis and helps dis inguish
shadow may be gradual or sudden in onse .
be ween serous and hemorrhagic de ach-
T ere may be associa ed severe pain, i men . I also will show evidence o
hemorrhagic. in amma ion o he sclera or orbi , and
can help dis inguish de achmen rom
IMPORTANT neoplasm.
CLINICAL SIGNS
A dome-shaped eleva ion o he re ina and PROGNOSIS AND

choroid is no ed. T e re ina and choroid may MANAGEMENT
appear normal in every way excep or heir
rela ive posi ion wi hin he eye. T e prognosis or pa ien s wi h serous
I large enough, he de achmen may choroidal de achmen is generally avorable
obs ruc he examiner’s view o he op ic i he underlying e iology can be reversed.
Choroidal De achmen 387
Res ora ion o normal in raocular pressure I he vi reous or re ina is adheren o

and eradica ion o in amma ion are gener- an erior s ruc ures such as a ca arac wound,
ally adequa e o reverse his process. vi rec omy is indica ed or else RD will occur
Hemorrhagic de achmen can produce as he choroidal de achmen resolves.
severe pain or marked eleva ion o in raocular Surgical drainage is usually recommended
pressure requiring promp surgical drainage. or “kissing choroidals,” al hough he neces-
O herwise, he condi ion can generally be si y and op imal iming o his procedure are
observed and will slowly resolve. con roversial.
FIGURE 9-35. Exuda ive RD. T is de achmen exhibi ed shi ing subre inal f uid wi h change o posi ion.
Index
Note: Page numbers ollowed by f and t indicate f gures and tables, respectively.
A prognosis or, 3–4 Antithrombin III def ciency, 134, 143,

ABCR. See ATP-binding transport wet or exudative, 1, 17–43, 21f–24f, 145, 150, 167
protein 40f–43f Anti-VEGF injection therapy, 20,
Abetalipoproteinemia. See Bassen– AIDS. See Acquired immunodef ciency 40f–43f
Kornzweig syndrome syndrome AREDS. See Age-related eye disease
Acetazolamide, or retinitis Albinism, 223–227, 224f study
pigmentosa, 229 causes o , 223 Arteriolar a enuation, 230f–231f,
Achromatopsia, 312 classif cation o , 223 233, 312
Acquired immunodef ciency syndrome clinical signs o , 223 Astrocytic hamartoma, 240–242, 242f
(AIDS), 134 diagnostic evaluation o , 224 causes o , 240
and intraocular lymphoma, 269 epidemiology o , 223 clinical signs o , 240–241
Acute ophthalmic artery obstruction, emale carrier, 226f diagnostic evaluation o , 241
153–154, 155f oveal hypoplasia, 225f di erential diagnosis o , 241
diagnostic evaluation o Hermansky–Pudlak syndrome, 227f epidemiology o , 240
electroretinography, 154 histology o , 223–224 history o , 240
uorescein angiography, 153–154 management o , 224 prognosis or, 241
di erential diagnosis o , 153t ocular, 223 management o , 241
symptoms o , 153 oculocutaneous, 223, 227f ATP-binding transport protein
Acute pancreatitis, 134 prognosis or, 224 (ABCR), 203
Adult oveomacular dystrophy, 3 true, 223 Atrophic AMD, 14f–15f
Adult vitelli orm dystrophy, 50 Albinoidism, 223 Atrophic retinal break, 346, 351f
Aneurysms, 172, 295, 299 Allelic inactivation, 243 Atrophy o RPE, 2, 218f
chroidal, 19 Alport’s syndrome, 294 Avulsed vitreous base, 318, 318f
intraretinal, 251f AMD. See Age-related macular causes o , 318
macro, 172, 173 degeneration clinical signs o , 318
micro, 101f–102f, 105, 108f–109f, Amelanotic choroidal melanoma, 78, diagnostic evaluation o , 318
117f, 158, 161f, 166, 172, 182, 264, 267, 269, 271 di erential diagnosis o , 318
188f, 194f, 294, 297f Amsler grids, 3, 16f, 17 epidemiology o , 318
Age-related eye disease study (AREDS), 4 o macular epiretinal membranes, 45 history o , 318
Age-related macular degeneration scotoma ormation and, 341 nasal, 318f
(AMD), 68 Anemia, 72, 96, 316, 321 prognosis or, 318
atrophic, 14f–15f severe, 134 management o , 318
basal laminar deposits, 2 Angiographic CME, 60
basal linear deposits, 2 Angioid streaks, 72–77, 73f–77f, 316 B
with calcif c drusen, 2 causes o , 72 Bardet–Biedl syndrome, 234
causes o , 2 clinical signs o , 72 Basal laminar deposits, in dry age-
clinical signs o , 3 diagnostic evaluation o , 72–73 related macular degeneration, 2
with CNV, 64, 65 di erential diagnosis o , 72 Basal laminar drusen, 1, 6f
diagnosis o , 3 epidemiology o , 72 Basal linear deposits, in dry age-related
di erential diagnosis o , 3 uorescein angiography or, 72 macular degeneration, 2
disci orm scar o , 271 history o , 72 Bassen–Kornzweig syndrome, 233
drusen, 2, 5f idiopathic, 72 Ba en disease. See Neuronal ceroid
dry/ nonexudative, 1–16 laser photocoagulation or, 73 lipo uscinosis
epidemiology o , 1, 2 management o , 73 BB pellet, 331
exudative, 17–43, 21f–24f mani estations in, 73 Beaten bronze appearance, in Stargardt’s
uorescein angiographic pa ern, 2–3 ocular photodynamic therapy or, 73 disease, 203, 206f–207f
with ocal hyperpigmentation, 4 prognosis or, 73 Best’s disease, 3, 189–194
undus biomicroscopy images, 2, 3 sa ety glasses or, 73 causes o , 189
history o , 2 Angiomatosis retinae. See Retinal clinical signs o
irregular granular appearance in, 2 capillary hemangioma end stage, 190
management o , 3–4 Anterior ischemic optic neuropathy, previtelli orm stage, 189
with multiple drusen, 2 142–143, 144f pseudohypopyon stage, 189–190
OCT o , 3 Antiplatelet therapy, or ocular ischemic vitelli orm stage, 189
pathology o , 2 syndrome, 159 vitelliruptive stage, 190
389
3 9 0 In d e x
Best’s disease (continued) di erential diagnosis o , 203 prognosis or, 150

diagnostic evaluation o , 190 in Stargardt’s disease, 203, 207f pupillary changes in, 149
epidemiology o , 189 superf cial retinal whitening in, 149
management o , 190 C visual acuity in, 149
prognosis or, 190 Calcif ed drusen, 1 Central retinal vein obstruction, 142,
pseudo, 194f Canthaxanthin toxicity, 338 156, 158, 166–171
pseudohypopyon stage, 192f CAR. See Carcinoma-associated causes o , 166
vitelli orm stage, 189, 191f retinopathy syndrome clinical signs o , 166
vitelliruptive stage, 193f Carcinoma-associated retinopathy diagnostic evaluation o , 166–167
Bevacizumab, 20, 98, 111f, 131f, (CAR) syndrome, 236–239, di erential diagnosis o , 166, 167t
137, 276 238f, 239f epidemiology o , 166
Black sunburst lesion, in sickle cell causes o , 236 ischemic, 166–167, 169f–171f
retinopathy, 178, 179f clinical signs o , 236 nonischemic, 166, 168f
Bloch–Sulzberger syndrome. See cone dys unction, 236 pathophysiology o , 166
Incontinentia pigmenti undus eatures, 236 management o , 167
Blot hemorrhages, in nonproli erative rod dys unction, 236 prognosis or, 167
diabetic retinopathy, 95 diagnostic evaluation o , 236–237 Central serous choroidopathy, 64
Blurred vision, 259, 264 di erential diagnosis o , 236 bullous, 383
Bourneville’s disease. See Astrocytic epidemiology o , 236 Central serous retinopathy (CSR), 19,
hamartoma history o , 236 50, 78–88, 79f–88f, 267, 268f
Brachytherapy, and radiation management o , 237 anti-VEGF therapy, 79
retinopathy, 182, 186f prognosis or, 237 causes o , 78
Branch retinal artery obstruction, Carotid arteriography, 159 clinical signs o , 78
146f–148f Carotid artery stenosis, 159t diagnostic evaluation o , 78–79
causes o , 145 Carotid, blockage o , 158 di erential diagnosis o , 78
cholesterol (Hollenhorst plaque) CAR syndrome. See Carcinoma- di use abnormality o retinal
in, 145 associated retinopathy pigment epithelium in, 78
clacif c, 145 syndrome epidemiology o , 78
clinical signs o , 145 Cataract surgery uorescein angiography or, 78–79
diagnostic evaluation o , 145 choroidal detachment a er, 387 gu ers o retinal pigment epithelial
di erential diagnosis o , 145 cystoid macular edema a er, alterations, 78
epidemiology o , 145 60–61 history o , 78
f brin–platelet in, 145 Cataracts, in nonproli erative diabetic laser photocoagulation or, 79
undus changes in, 145 retinopathy, 96 management o , 79
management o , 146 Cellophane maculopathy. See Macular prognosis or, 79
pathophysiology o , 145 epiretinal membrane retinal pigment epithelium
prognosis or, 146 Central retinal artery obstruction, 134, detachment in, 78
pupillary changes in, 145 143, 145, 151f, 153–154, 156 smokestack appearance in, 79
visual acuity in, 145 acute, 150, 151f type A personality and, 78
Branch retinal vein obstruction, calcif c, 149 yellow spots, 78
163–165, 165f causes o , 149 Chédiak–Higashi syndrome, and
causes o , 163 cholesterol (Hollenhorst plaque) albinism, 223
clinical signs o , 163 in, 149 Cherry red spot
diagnostic evaluation o , 163 clinical signs o , 149 in acute ophthalmic artery
di erential diagnosis o , 163 with cilioretinal arterial sparing, obstruction, 153
epidemiology o , 163 149, 151f in central retinal artery obstruction,
management o , 163–164 diagnostic evaluation o , 149 149, 151f
laser photocoagulation, or di erential diagnosis o , 149 in combined retinal artery and vein
macular edema, 163–164 digital massage o globe and anterior obstruction, 157f
ranibizumab therapy, or macular chamber or, 150 Chloroquine retinopathy, 341–342,
edema, 164 electroretinography or, 149 342f
sector laser PRP, 164 epidemiology o , 149 clinical signs o , 341
pathophysiology o , 163 f brin–platelet in, 149 diagnostic evaluation o , 341
prognosis or, 163–164 undus changes in, 149 di erential diagnosis o , 341
Branch Vein Occlusion Study, 164 intravenous uorescein angiography epidemiology o , 341
Bruch’s membrane, 1, 2, 17, 259, 316 o , 149 history o , 341
Bull’s-eye maculopathy, 195, laser panretinal photocoagulation management o , 341
196f–197f, 203, 234–235, (PRP) or, 150 prognosis or, 341
341, 342f management o , 150 subtle para oveal retinal pigment
in cone dystrophy, 195, 196f–197f pathophysiology o , 149 epithelial alterations, 342
In d e x 391
Chorioretinal coloboma, 300–303, di erential diagnosis o , 262 Choroidal osteoma, 271–272, 272f
301f, 302f epidemiology o , 262 causes o , 271
causes o , 300 history o , 262 clinical signs o , 271
clinical signs o , 300 management o , 262 diagnostic evaluation o , 271
diagnostic evaluation o , 300 prognosis or, 262 di erential diagnosis o , 271
di erential diagnosis o , 300 Choroidal melanoma, 259–261, 261f epidemiology o , 271
epidemiology o , 300 amelanotic, 78 history o , 271
history o , 300 branchytherapy or, 182, 186f, 247, management o , 271
management o , 300 255 prognosis or, 271
prognosis or, 300 causes o , 259 Choroidal rupture, 316–317
and rhegmatogenous retinal clinical signs o , 259 causes o , 316
detachment, 303f diagnostic evaluation o , 260 clinical signs o , 316
Chorioretinal scarring, 89, 92, 332f di erential diagnosis o , 259 crescent-shaped lesion, 317f
Chorioretinitis sclopetaria, 331–332 epidemiology o , 259 diagnostic evaluation o , 316
causes o , 331 history o , 259 di erential diagnosis o , 316
clinical signs o , 331 malignant, 252, 258f epidemiology o , 316
diagnostic evaluation o , 331 management o history o , 316
di erential diagnosis o , 331 ocular, 260 management o , 316
epidemiology o , 331 systemic, 260 prognosis or, 316
history o , 331 prognosis or, 260 traumatic, 72
management o , 331 Choroidal metastasis, 259, 264–266, Choroidal tumors, 20, 89, 92, 260, 265,
prognosis or, 331 266f, 269, 383, 386 271, 384
retinal whitening and preretinal causes o , 264 Choroideremia, 210–214
hemorrhage, 332f clinical signs o , 264 causes o , 210
Choroidal detachment, 384, diagnostic evaluation o , 265 clinical signs o , 210
386–388 di erential diagnosis o , 264–265 diagnostic evaluation o , 210
causes o , 386 epidemiologyo , 264 di erential diagnosis o , 210
clinical signs o , 386 history o , 264 early, 213f
diagnostic evaluation o , 386 management o , 265 epidemiology o , 210
di erential diagnosis o , 386 prognosis or, 265 history o , 210
epidemiology o , 386 Choroidal neovascularization (CNV), late, 214f
history o , 386 13f, 45, 60, 69f, 74f, 175–176, prognosis and management o , 211
management o , 386–387 190, 255, 259, 267, 271, 316, retinal pigment epithelial loss, 212f
prognosis or, 386–387 317f, 386 Choroiditis, multi ocal, 269
Choroidal olds, 45, 89–91, 90f–91f and angioid streaks, 72, 74f–75f Choroidopathy, hypertensive, 136
causes o , 89 choroidal olds, 89 Chronic submacular uid, 19, 21f
clinical signs o , 89 classic, 18, 21f CHRPE. See Congenital hypertrophy o
crests o olds, 89 CSR in, 78 the retinal pigment epithelium
diagnostic evaluation o , 89 in degenerative myopia, 67, Cicatricial retinopathy o prematurity,
di erential diagnosis o , 89 68f–71f 275, 281f
epidemiology o , 89 and dry age-related macular Cilioretinal artery obstruction,
uorescein angiography or, 89 degeneration, 3, 3t 142–144, 144f
history o , 89 and exudative age-related macular causes o , 142
Choroidal granuloma, 265 degeneration, 17 clinical signs o , 142
Choroidal hemangioma, 267–268 f brovascular pigment epithelial diagnostic evaluation o , 143
circumscribed type, 259, 264, 268 , detachment, 18 di erential diagnosis o , 143
271f occult, 18, 25f–28f epidemiology o , 142
clinical signs o , 267 polypoidal, 64 management o , 143
diagnostic evaluation o , 267 recurrent, a er laser therapy, 39f pathophysiology o , 142
di erential diagnosis o , 267 in wet (exudative) AMD, 17–18 prognosis or, 143
di use type, 267 Choroidal neovascular membrane, Cilioretinal artery sparing, in central
epidemiology o , 267 50, 176 retinal artery obstruction,
history o , 267 Choroidal nevus, 257–258, 258f 149, 151f
management o , 268 clinical signs o , 257 Circumscribed choroidal hemangioma,
prognosis or, 268 diagnostic evaluation o , 257 78, 259, 264, 268f, 271
Choroidal mass, di erential diagnosis di erential diagnosis o , 257 Classic choroidal neovascularization,
o , 271 epidemiology o , 257 18, 21f–24f, 29f–30f
Choroidal melanocytoma, 262, 263f history o , 257 Clinically signif cant macular edema
clinical signs o , 262 management o , 257–258 (CSME), 96t
diagnostic evaluation o , 262 prognosis or, 257–258 Cluster o grapes appearance, 250, 251f
3 9 2 In d e x
CME. See Cystoid macular edema diagnostic evaluation o , 273–274 Cryotherapy, 275, 347, 358
CNS lymphoma, 269 epidemiology o , 273 or retinal tears and breaks, 347
CNV. See Choroidal neovascularization history o , 273 or retinopathy o prematurity, 275,
Coats’ disease, 96, 172, 229, 244, Congenital grouped pigmentation, 280f
294–299, 297f 252 or rhegmatogenous retinal
aneurysmal dilation and hemorrhage, Congenital hypertrophy o the retinal detachment, 358
298f, 299f pigment epithelium (CHRPE), CSME. See Clinically signif cant
causes o , 294 252, 253f macular edema
clinical signs o , 294 causes o , 252 CSNB. See Congenital stationary night
diagnostic evaluation o , 295 clinical signs o , 252 blindness
di erential diagnosis o , 294–295 def nition o , 252 CSR. See Central serous retinopathy
epidemiology o , 294 diagnostic evaluation o , 252 Cystoid macular edema (CME), 19, 45,
and exudative retinal detachment, di erential diagnosis o , 252 50, 60–63, 62f–63f
297f epidemiology o , 252 angiographic, 60
uorescein angiogram o , 298f history o , 252 anti-VEGF therapy or, 61
history o , 294 multi ocal, 252 causes o , 60
intraretinal hemorrhages, 296f management o , 252 clinical signs o , 60
pathophysiology o , 294 prognosis or, 252 diagnostic evaluation o , 60
management o , 295 solitary, 252 di erential diagnosis o , 60
prognosis or, 295 Congenital oculodermal melanocytosis, epidemiology o , 60
Cobblestone degeneration, 376, 377f 259 uorescein angiography or, 60
causes o , 376 Congenital stationary night blindness history o , 60
clinical signs o , 376 (CSNB), 219–222, 220f management o , 61
diagnostic evaluation o , 376 causes o , 219 Nd:YAG laser vitreolysis or, 61
di erential diagnosis o , 376 classif cation o , 219 NSAIDs or, 61
epidemiology o , 376 clinical signs o , 219 prognosis or, 61
history o , 376 diagnostic evaluation o , 219 slit-lamp biomicroscopy or, 60
management o , 376 di erential diagnosis o , 219 surgery or, 61
prognosis or, 376 epidemiology o , 219 Cytomegalovirus retinitis (CMV),
Cockayne’s syndrome, 233 undus albipunctatus, 219 269
Color vision, 78–79, 190, 195, 198, 203, history o , 219 Cytologic f ndings, 264
236, 341 management o , 219
Combined central retinal artery and vein prognosis or, 219 D
obstruction, 156–157, 157f Contact lens, 45, 95, 96, 329, 336, 347, DCCT. See Diabetes Control and
Combined hamartoma o retina and 357, 372, 380 Complications Trial (DCCT)
retinal pigment epithelium, 45, Cornea, in nonproli erative diabetic Degenerative myopia, 67–71, 68f–71f
252, 255–256, 256f, 259, 262 retinopathy, 96 anti-VEGF therapy or, 68
Commotio retinae, 314–315, 315f Co on-wool spots, 133–135, 135f causes o , 67
clinical signs o , 314 causes o , 133 clinical signs o , 67
diagnostic evaluation o , 314 clinical signs o , 133 CNV lesions in, 68
di erential diagnosis o , 314 in coagulopathies, 134 diagnostic evaluation o , 68
epidemiology o , 314 in diabetic retinopathy, 134 di erential diagnosis o , 67–68
history o , 314 diagnostic evaluation o , 134 epidemiology o , 67
management o , 314 di erential diagnosis o , 133 uorescein angiography or, 68
prognosis or, 314 in embolic disorders, 134 history o , 67
Cone dys unction, 236 epidemiology o , 133 laser photocoagulation or, 68
Cone dystrophy, 195–197, 196f–197f undus changes, 133 management o , 68
causes o , 195 management o , 134 ocular photodynamic therapy
clinical signs o , 195 in nonproli erative diabetic or, 68
diagnostic evaluation o , 195 retinopathy, 95, 103f ophthalmoscopy or, 68
di erential diagnosis o , 195 pathophysiology o , 133 prognosis or, 68
epidemiology o , 195 prognosis or, 134 scleral rein orcement and resection
history o , 195 pupillary changes, 133 techniques, 68
management o , 195 in retinal vein obstruction, 107f, 134 Degenerative retinoschisis, 380–382
prognosis or, 195 in systemic arterial hypertension, causes o , 380
Con uent drusen, 6f, 11f–12f 134 clinical signs o , 380
Congenital and pediatric retinal visual acuity, 133 diagnostic evaluation o , 380
diseases Cranial nerve palsy, and di erential diagnosis o , 380
causes o , 273 nonproli erative diabetic epidemiology o , 380
clinical signs o , 274 retinopathy, 96 history o , 380
In d e x 393
management o , 380 Drusen Extraretinal f brovascular proli eration

outer wall breaks, 382f basal laminar, 6f (ERFP), 274
peripheral retinoschisis, 381f choroidal neovascularization and, Exudative AMD, 1–4, 17–20, 21f, 22f,
prognosis or, 380 3, 3t 23f, 24f, 40f, 41f, 42f, 43f
Dental abnormalities, 284 con uent, 6f Exudative maculopathy, 19
Diabetes Control and Complications di erential diagnosis o , 3, 198 Exudative retinal detachment, 383
Trial (DCCT), 97t in dry age-related macular categories, 383
Diabetic macular edema, 60 degeneration, 1–4, 5f–7f, causes o , 383
Diabetic papillopathy 10f–13f choroidal melanoma, 385f
causes o , 132 hard, 7f clinical signs o , 383
clinical signs o , 132 large, 5f with Coats’ disease, 385f
diagnostic evaluation o , 132 multiple large, 11f–13f diagnostic evaluation o , 384
di erential diagnosis o , 132 Drusenoid pigment epithelial di erential diagnosis o , 383–384
disc swelling, 132 detachments, 2, 10f epidemiology o , 383
epidemiology o , 132 Dry (nonexudative) AMD, 1–16 history o , 383
uorescein angiography or, 132 central vision, 2 prognosis and management o , 384
history o , 132 geographic atrophy, 1
management o , 132 retinal pigment epithelial F
papillary de ect, 132 abnormalities, 1 FA. See Fundus albipunctatus
prognosis or, 132 subretinal pigment epithelial Fabry’s disease, 143, 145, 150, 154,
visual loss, 132 deposits, 1 156
Diabetic retinopathy. See also therapies or, 4 Factor V Leiden mutation, 134
Proli erative diabetic Dystrophy(ies) Familial exudative vitreoretinopathy
retinopathy (PDR) adult oveomacular, 3 (FEVR), 275, 289–293, 290f,
causes o , 94 cone, 195–197, 196f–197f 291f
and co on-wool spots, 95 pa ern, 3, 198–202, 199f–201f causes o , 289
epidemiology o , 94 retinal, 189–239 clinical signs o , 289
history o , 95 diagnostic evaluation o , 289
hyperglycemia and, 94 E di erential diagnosis o , 289
loss o pericytes in, 95 Early Treatment Diabetic Retinopathy epidemiology o , 289
mechanism o development, Study (ETDRS) guidelines, history o , 289
94–95 97, 97t management o , 289–290
nonproli erative, 95–98 Ecchymosis, 323, 336 prognosis or, 289–290
pathophysiology o , 94–95 Edema retinal dragging, 292f
thickening o retinal capillary macular rhegmatogenous retinal detachment,
basement membranes, 95 clinically signif cant, 96, 96t 293f
Diabetic Retinopathy Clinical Research cystoid, 19 Fascio-scaspulohumeral dystrophy,
(DRCR), 97t in nonproli erative diabetic 294
Diabetic retinopathy study (DRS), retinopathy, 95, 97t, 103f Female carriers, 210, 211, 223, 226f,
112t, 114f retinal, in retinal artery 229f
DIC. See Disseminated intravascular macroaneurysm, 172–173 FEVR. See Familial exudative
coagulation Ehlers–Danlos syndrome, and angioid vitreoretinopathy
Di use choroidal hemangioma, 267 streaks, 72, 336 Fibromuscular hyperplasia, 143, 145,
Disci orm scar, with exudative age-related Electroretinography, 154, 159, 167, 150, 154, 156
macular degeneration, 17, 19, 229, 307 Fibrosis, 19
21f, 271 Elsching spots. See Hypertensive Fibrotic submacular scarring, 19
Dislocated lens, 336–337, 337f choroidopathy Fibrovascular pigment epithelial
blunt ocular trauma, history o , Emboli detachment, 18
336 retinal, 338 Flame-shaped hemorrhages, in
clinical signs o , 336 retinal intra-arterial, 142, 145 nonproli erative diabetic
diagnostic evaluation o , 336 End-stage retinopathy, 95
di erential diagnosis o , 336 o Best’s disease, 190 Fleck retina o Kandori, 219
epidemiology o , 336 geographic atrophy, 9f Fluorescein angiography, 18, 187, 190,
management o , 336 Endarterectomy, or ocular ischemic 195, 241
prognosis or, 336 syndrome, 159, 159t age-related macular degeneration
Disseminated intravascular coagulation Enucleation, 244–245, 248, 260, (AMD), 2–3
(DIC), 383 262, 295 angioid streaks, 72
DRCR. See Diabetic Retinopathy Epidermal nevus syndrome, 294 o carcinoma metastatic, 265
Clinical Research ETDRS. See Early Treatment Diabetic central serous retinopathy (CSR),
DRS. See Diabetic retinopathy study Retinopathy Study guidelines 78–79
3 9 4 In d e x
Fluorescein angiography (continued) Gyrate atrophy, 68, 210, 215–218, Homocysteinuria, and dislocated lens,
choroidal olds, 89 217f–218f 336
cystoid macular edema (CME), 60 causes o , 215 Horseshoe retinal tear, 346, 349f, 350f
degenerative myopia, 68 clinical signs o , 215 Hunter’s disease, 234
hypotony maculopathy, 92 diagnostic evaluation o , 215–216 Hurler’s disease, 234
intravenous, 172 di erential diagnosis o , 215 Hydroxychloroquine retinopathy,
macular epiretinal membrane, 45 epidemiology o , 215 341–342
nonproli erative diabetic retinopathy history o , 215 HYE. See Hard yellow exudates
(NPDR), 96 management o , 216 Hyperopia, 89, 92
polypoidal choroidal vasculopathy prognosis or, 216 Hyperplasia, reactive, o retinal pigment
(PCV), 64 epithelium, 255
vitreomacular traction syndrome H Hypertension, and co on-wool spots,
(VMTS), 58 Hagberg–Santavuori disease, 234 133
Focal hyperpigmentation, in dry age- Hamartoma Hypertensive choroidopathy, 136
related macular degeneration astrocytic, 240–242, 242f Hypertensive retinopathy, 96, 136–141,
(AMD), 1, 2, 7f o retina and retinal pigment 138f–141f
4-2-1 Rule, 96t epithelium, 58 clinical signs o , 136
Foveal aplasia/ hypoplasia, 223 Harada’s disease, 78, 383 diagnostic evaluation o , 137
Foveal hypoplasia, 223, 225f, 284 Hard drusen. See Small drusen di erential diagnosis o , 136
Foveal schisis, and juvenile X-linked Hard yellow exudates (HYE), in epidemiology o , 136
retinoschisis, 307, 309f, 311f nonproli erative diabetic grades o , 136
Frank geographic atrophy, 1, 2, 4 retinopathy, 100f–103f intravitreal VEGF-A inhibitor
Friedreich’s ataxia, 235 Helicobacter pylori in ection, 78 therapy or, 137
Fuchs’ spots, in degenerative myopia, Hemangioma Keith–Wagener–Barker classif cation
67 choroidal, 267–268, 268f o , 136
Fundus albipunctatus (FA), 219, 220f circumscribed choroidal, 267, laser therapy or, 137
Fundus biomicroscopy, 2, 3, 17, 18, 268f management o , 137
19, 187 optic disc, 248 pathophysiology o , 136
Fundus avimaculatus, 203 retinal capillary, 247–249, 249f prognosis or, 137
Fundus pulverulentus, 198, 202f retinal cavernous, 250–251, 251f Hypertrophy, congenital, o retinal
Hemoglobinopathies, 96 pigment epithelium, 252–254,
G Hemorrhage 253f–254f
Gardner’s syndrome, 252, 254f, 378, blot, in nonproli erative diabetic Hyperviscosity syndrome, 167
379f retinopathy, 95 Hypomelanotic macules, 241
Gass classif cation o idiopathic macular ame-shaped, in nonproli erative Hypoplasia, oveal, 225f
hole, 49, 49t diabetic retinopathy, 95 Hypotension, 134, 143, 145, 150
Genetic analysis, o peripherin/ RDS intraretinal, in nonproli erative Hypotony, 89, 92
gene, 198 diabetic retinopathy, 95 Hypotony maculopathy, 92, 93f
Genetic counseling, retinoblastoma premacular, 190, 194f causes o , 92
and, 245 in retinal artery macroaneurysm, 172 chorioretinal olds in, 92
Genetic de ects, retinal diseases salmon patch, in sickle cell clinical signs o , 92
associated with, 228 retinopathy, 178 diagnostic evaluation o , 92
Genetic testing, o children, 244 submacular, 19, 38f epidemiology o , 92
Geographic atrophy, in dry age-related subretinal, and angioid streaks, 72, uorescein angiography or, 92
macular degeneration, 1, 2, 74f–76f history o , 92
14f–15f vitreous, in proli erative diabetic intraocular pressure in, 92
Germinal, children with, 241 retinopathy, 112, 121f, 122f macular leakage in, 92
Giant cell arteritis, 134, 142, 143, 145, Hemorrhagic choroidal detachment, macular retina in, 92
146, 149, 150, 153, 156, 158 386 management o , 92
Giant retinal tear, 346, 352f Heredopathia atactica optic disc hyper uorescence, 92
Glaucoma, and nonproli erative polyneuriti ormis. See Re sum’s peripapillary choroid in, 92
diabetic retinopathy, 96 disease prognosis or, 92
Goldmann perimetry, or retinitis Hermansky–Pudlak syndrome, 223, surgery or, 92
pigmentosa, 230 224, 227f
Granularity o the RPE, 2 Hollenhorst plaque I
Granuloma, choroidal, 265, 269 in branch retinal artery obstruction, ICROP. See International Classif cation
Grid laser photocoagulation, or branch 145 o ROP
retinal vein obstruction, 164 in central retinal artery obstruction, Idiopathic juxta oveal telangiectasis, 96
Grönblad–Strandberg syndrome, and 149, 152f Idiopathic macular hole, 49–50, 51f–57f
angioid streaks, 72 in cilioretinal artery obstruction, 142 Amsler grid testing o , 49
In d e x 395
causes o , 49 Intraretinal microvascular abnormality Laser panretinal photocoagulation

clinical signs o , 49–50 (IRMA), in nonproli erative (PRP), 150
diagnostic evaluation o , 50 diabetic retinopathy, 95, 105f Laser photocoagulation. See also
di erential diagnosis o , 50 Intraretinal pigment clumps, 2 Panretinal photocoagulation
epidemiology o , 49 Intravenous drug abuse (chronic), 134 or branch retinal vein obstruction,
Gass classif cation o stages, 49 Intravenous uorescein angiography, 163–164
history, 49 156, 159, 182 or central retinal artery obstruction,
management o , 50 Intravitreal VEGF-A inhibitor therapy 150
negative prognostic indicators, 50 hypertensive retinopathy, 137 or central retinal vein obstruction,
OCT and, 50 IOFB. See Intraocular oreign body 167
prognosis or, 50 Iridescent spot, in sickle cell or central serous retinopathy, 79
stages o development, 49t retinopathy, 178 or combined retinal artery and vein
surgery or, 50 Iris neovascularization, in proli erative obstruction, 157
tangential vitreoretinal traction diabetic retinopathy, 112, 120f or degenerative myopia, 68
and, 49 IRMA. See Intraretinal microvascular or exudative age-related macular
vitrectomy or, 50 abnormality degeneration, 20, 39f
Watzke–Allen sign in, 50 IRVAN. See Idiopathic retinal vasculitis, guidelines or use, 97t
Idiopathic retinal vasculitis, aneurysms, aneurysms, and neuroretinitis or nonproli erative diabetic
and neuroretinitis (IRVAN), Irvine–Gass syndrome, 60, 175 retinopathy, 97, 97t
172 Ischemia, macular, in proli erative or proli erative diabetic retinopathy,
Incontinentia pigmenti, 284–288, 285f diabetic retinopathy, 113 112–113, 125f
causes o , 284 or retinal tears and breaks, 347,
clinical signs o , 284 J 354f–356f
dental f ndings, 288f Jansky–Bielschowsky disease, 234 or retinopathy o prematurity, 276
dermatologic f ndings, 286f, 287f Juvenile retinoschisis, 380 or rhegmatogenous retinal
diagnostic evaluation o , 284 Juvenile X-linked retinoschisis, detachment, 358
di erential diagnosis o , 284 307–308 Laser therapy, 175
epidemiology o , 284 causes o , 307 La ice degeneration, 372–373
uorescein angiogram o , 286f clinical signs o , 307 causes o , 372
history o , 284 diagnostic evaluation o , 307 clinical signs o , 372
management o , 285 di erential diagnosis o , 307 diagnostic evaluation o , 372
prognosis or, 285 epidemiology o , 307 di erential diagnosis o , 372
Indocyanine green angiography, 18, 19, oveal schisis, 309f epidemiology o , 372
35f, 64, 265, 267 history, 307 history, 372
Inter eron therapy, 134 in erior retinoschisis, 311f horseshoe tear and RD, 373f
International Classif cation o ROP management o , 308 pigmented, 373f
(ICROP), 274t peripheral retinoschisis, 310f prognosis and management o ,
Intraocular oreign body (IOFB), prognosis or, 308 372
333–335, 335f rhegmatogenous retinal detachment, Laurence–Moon syndrome, 234
causes o , 333 311f Leber’s congenital amaurosis, 312–313,
clinical signs o , 333 Juxta oveal lesions, 255 313f
diagnostic evaluation o , 333 Juxta oveal telangiectasis. See Para oveal causes o , 312
di erential diagnosis o , 333 telangiectasis clinical signs o , 312
epidemiology o , 333 Juxtapapillary capillary hemangioma, diagnostic evaluation o , 312
history o , 333 247 di erential diagnosis o , 312
management o , 333–334 epidemiology o , 312
prognosis or, 333–334 history o , 312
Intraocular lymphoma, 269–270, K management o , 312
270f Kandori, eck retina o , 219 prognosis or, 312
causes o , 269 Kearns–Sayre syndrome, 234 Lens
clinical signs o , 269 Kissing choroidals, 386, 387 contact, 347, 357, 372
diagnostic evaluation o , 270 Kline elter syndrome, 284 dislocated, 336–337, 337f
di erential diagnosis o , 269 KUFS disease, 235 Leptospirosis, 134
epidemiology o , 269 Leukemia, 96
history o , 269 L Leukokoria, 245f
management o , 270 Lacquer cracks, in degenerative di erential diagnosis o , 244
prognosis or, 270 myopia, 67 and retinoblastoma, 243–244, 245f
Intraretinal hemorrhages, in Large drusen, 1–3, 5f, 198 Lipemia retinalis, 187–188, 188f
nonproli erative diabetic Largest basal tumor diameter (LTD), clinical signs o , 187
retinopathy, 95 260 diagnostic evaluation o , 187
3 9 6 In d e x
Lipemia retinalis (continued) Maculopathy Nolvadex. See Tamoxi en

di erential diagnosis o , 187 bull’s eye, 195, 196f–197f, 203, 207f, Nonarteritic ischemic optic neuropathy
management o , 187 341, 342f (AION), 132
pathophysiology o , 187 hypotony, 92–93, 93f Nonexudative age-related macular
prognosis or, 187 solar, 319, 319f degeneration, 1–16
Lipid exudation, 19 Magnetic resonance angiography Nongeographic atrophy, in dry age-
Lipoprotein exudation, in (M ), 159 related macular degeneration,
nonproli erative diabetic Mar an’s syndrome, 336 1, 2, 8f
retinopathy, 95 Melanocytoma, 255 Non-Hodgkin tumor, 269
Liver enzymes, measurement o , 260 choroidal, 262–263, 263f Nonproli erative diabetic retinopathy
LTD. See Largest basal tumor diameter Melanoma, choroidal, 259–261, 261f (NPDR), 95–98, 99f–111f
Lupus anticoagulant syndrome, 134, and exudative retinal detachment, anti-VEGF therapy or, 98
143, 145, 150 385f blot hemorrhages in, 95
Lyme disease, 134, 143, 145, 150 Meridional old, 374–375, 375f clinical signs o , 96
Lymphoma, intraocular, 269–270, 270f Metastasis, choroidal, 264–266, 266f co on-wool spots, 95
Metastatic carcinoma, 134 diagnostic evaluation o , 96–97
M Methylphenidate, 338 di erential diagnosis o , 96
Macroaneurysm, retinal artery, Microaneurysms, in nonproli erative ETDRS guidelines or, 97
172–174, 174f diabetic retinopathy, 95, 101f ame-shaped hemorrhages in, 95
Macula-o RDs, 358 Migraine, 134, 143, 145, 150 uorescein angiography or, 96
Macular branch vein, 163 Mizuo–Nakamura phenomenon, 221, 4-2-1 rule, 96t
Macular degeneration, age-related. 222f intraretinal hemorrhage in, 95
See Age-related macular M . See Magnetic resonance intraretinal microvascular
degeneration angiography abnormality (IRMA), 95
Macular diseases, 44–93 Mucopolysaccharidoses, 234 lipoprotein exudation in, 95
Macular edema, 19, 183 Muscular dystrophy, 294 macular edema in, 95
clinically signif cant, 96, 96t Myopia, degenerative, 67–71, 68f–71f management o , 97–98
cystoid, 60–63, 62f–63f microaneurysm in, 95
in nonproli erative diabetic N mild, 95, 99f–102f
retinopathy, 95, 96t, 97t Necrotic tears o retina, 347 moderate, 95, 103f–104f
Macular epiretinal membrane, 44–48, Neovascularization elsewhere (NVE), OCT o , 97
46f–48f, 58 112 prognosis or, 97–98
Amsler grid testing o , 45 in proli erative diabetic retinopathy, role o laser, 97
causes o , 44 112, 116f–119f severe, 95–96, 105f–106f
clinical signs o , 45 and vitreous hemorrhage, 112 vision loss in, 95
diagnosis o , 45 Neovascularization o the disc (NVD), NPDR. See Nonproli erative diabetic
di erential diagnosis o , 45 112 retinopathy
epidemiology o , 44 and diabetic papillopathy, 132 NVD. See Neovascularization o the
in emales, 44 in proli erative diabetic retinopathy, disc
uorescein angiography or, 45 112, 114f–115f, 118f NVE. See Neovascularization
history o , 44 and vitreous hemorrhage, 112 elsewhere
idiopathic, 44 Neovascularization o the iris (NVI) NVI. See Neovascularization o the iris
in males, 44 in branch retinal vein obstruction, Nystagmus, 223, 235
management o , 45 163
OCT or, 45 in central retinal artery obstruction, O
posterior vitreous detachment 150 Occlusion
(PVD) in, 44 in central retinal vein obstruction, 166 acute ophthalmic artery obstruction,
prognosis or, 45 in combined retinal artery and vein 153–155, 153t, 155f
retinal break ormation and trauma obstruction, 157 branch artery, 284
in, 44 in ocular ischemic syndrome, 158 branch retinal vein, 163–165, 165f
slit-lamp biomicroscopy o , 45 in proli erative diabetic retinopathy, central retinal artery, 149–152, 151f,
Macular hole, 45 112, 113t 153t
idiopathic, 49–57, 49t, 51f–57f Neuroimaging, 325 central retinal vein, 166–171, 167t,
traumatic, 329–330, 330f Neuronal ceroid lipo uscinosis, 168f–171f
Macular ischemia, in proli erative 234–235 cilioretinal artery, 142–144, 144f
diabetic retinopathy, 113 Nevus, choroidal, 257–258, 258f combined retinal artery and vein
Macular pucker. See Macular epiretinal Ni edipine, 137 obstruction, 156–157, 157f
membrane Night blindness Occult choroidal neovascularization,
Macular scarring, and angioid streaks, congenital stationary, 219–220, 220f 18, 25f–30f
75f in retinitis pigmentosa, 228 Ocular albinism, 223
In d e x 397
Ocular ischemic syndrome, 96, 158–162, or central retinal vein obstruction, clinical signs o , 304
160f–162f, 167t 167 diagnostic evaluation o , 304
carotid artery stenosis treatment, or combined retinal artery and vein di erential diagnosis o , 304
outcomes, 159t obstruction, 157 epidemiology o , 304
causes o , 158 or ocular ischemic syndrome, 159 history o , 304
clinical eatures o , 158 Papilledema, 132, 134 management o , 304
diagnostic evaluation o , 159 Para oveal telangiectasis, 175–177 posterior orm, 306f
di erential diagnosis o , 158–159 causes o , 175 prognosis or, 304
epidemiology o , 158 clinical signs o , 175 PFV. See Persistent etal vasculature
management o , 159 diagnostic evaluation o , 175–176 Photodynamic therapy
pathophysiology o , 158 di erential diagnosis o , 175 or degenerative myopia, 68
prognosis or, 159 epidemiology o , 175 or exudative age-related macular
Ocular toxoplasmosis, 300 group 2, 176f–177f degeneration, 20
Ocular trauma, angioid streaks and, management o , 176 PHPV. See Persistent hyperplastic
73, 76f pathophysiology o , 175 primary vitreous
Oculocutaneous albinism, 223, 227f prognosis or, 176 Pigment epithelial detachment
Oguchi’s disease, 221–222 Pars planitis, 295 in dry age-related macular
causes o , 221 Pa ern dystrophy, 3, 198–202, degeneration, 2, 10f
clinical signs o , 221 199f–202f and exudative age-related macular
diagnostic evaluation o , 221 causes o , 198 degeneration, 17
epidemiology o , 221 clinical signs o , 198 f brovascular, 18
management o , 221 diagnostic evaluation o , 198 serous, 18
Mizuo–Nakamura phenomenon o , di erential diagnosis o , 198 Pigmented undus lesion, 254f
221, 222f epidemiology o , 198 Pisci orm ecks, in Stargardt’s disease,
prognosis or, 221 undus pulverulentus, 202f 203, 205f
Operculated retinal tear, 346, 350f history o , 198 Plus disease, o retinopathy o
Ophthalmia, sympathetic, 383 prognosis or, 198 prematurity, 274, 280f
Ophthalmic artery obstruction, acute, Paving stone degeneration, 376–377, Pneumatic retinopexy, or
153–155, 155f 377f rhegmatogenous retinal
causes o , 153 PCV. See Polypoidal choroidal detachment, 358
clinical eatures o , 153 vasculopathy Polyarteritis nodosa, 149
diagnostic evaluation o , 153–154 PDR. See Proli erative diabetic Polypoidal choroidal vasculopathy
di erential diagnosis o , 153t retinopathy (PDR) (PCV), 19, 64–66, 65f–66f
epidemiology o , 153 Peau d’orange, 72, 75f–76f anti-VEGF therapy or, 65
management o , 154 Pediatric retinal diseases, 273 causes o , 64
pathophysiology o , 153 Pegaptanib, 20 clinical signs o , 64
prognosis or, 154 Peripheral cystoid degeneration, diagnostic evaluation o , 64
Optical coherence tomography (OCT), 351f di erential diagnosis o , 64
97, 314, 323, 325 Peripheral grouped pigmentation, 378, epidemiology o , 64
Optic disc hemangioma, 248 379f uorescein angiography o , 64
Optic disc/ retina, neovascularization causes o , 378 history o , 64
o , 182 clinical signs o , 378 laser photocoagulation or, 65
Optic nerve pit with neurosensory diagnostic evaluation o , 378 management o , 64–65
macular retinal detachment, 78 di erential diagnosis o , 378 ocular photodynamic therapy or,
Optic neuropathy, 182 epidemiology o , 378 65
Orbital mucormycosis, 149 Gardner’s syndrome, 379f prognosis or, 64–65
Orbital tumors, 89, 92 history o , 378 serosanguineous detachments in, 64
Osteitis de ormans, and angioid management o , 378 slit-lamp biomicroscopy, 64
streaks, 72 prognosis or, 378 Popcorn lesions, o retinopathy o
Osteoma, choroidal, 271–272, 272f Peripheral retinal disease, 346–388 prematurity, 275, 278f
Peripheral retinoschisis, and juvenile Posterior scleritis, 78, 89, 92
P X-linked retinoschisis, 307, Posterior vitreous detachment (PVD),
Paget’s disease, and angioid streaks, 72 310f 346, 348f
Panretinal photocoagulation (PRP), Peripheral vision problems, 228 and macular epiretinal membrane, 44
112, 113t, 125f–129f. See also Persistent etal vasculature (PFV), 275, Pos raumatic retinal tear, 352f–353f
Laser photocoagulation 304–306, 305f Prednisolone acetate, 61
or branch retinal vein obstruction, Persistent hyperplastic primary vitreous Premacular hemorrhage, 190, 194f
164 (PHPV), 304–306 Presumed ocular histoplasmosis, 68
or central retinal artery obstruction, anterior orm, 305f Previtelli orm stage, o Best’s disease,
150 causes o , 304 189
3 9 8 In d e x
Proli erative diabetic retinopathy brachytherapy or choroidal types o

(PDR), 112–113, 114f–131f melanoma, 186f atrophic retinal break, 346
chemical mediators, 112 causes o , 182 giant tear, 346
clinical signs o , 112 clinical signs o , 182 horseshoe ( ap) tears, 346
macular ischemia, 113 diagnostic evaluation o , 182 operculated tears, 346
neovascularization elsewhere di erential diagnosis o , 182 retinal dialysis, 346
(NVE), 112 epidemiology o , 182 stretch/ necrotic tears, 347
neovascularization o the disc management o , 182–183 Retinal capillary hemangioma,
(NVD), 112 pathophysiology o , 182 247–249, 249f
panretinal photocoagulation (PRP) prognosis or, 182–183 causes o , 247
acts, 113t Radiation therapy, 268 clinical signs, 247
preretinal hemorrhage in, 112 Ranibizumab, 20, 98, 137, 164 diagnostic evaluation o , 248
retinal detachment, treatment or, PD. See Relative a erent papillary di erential diagnosis o , 247–248
113 de ect epidemiology o , 247
treatment or, 112–113 Rb. See Retinoblastoma history o , 247
vitrectomy in, indications or, 113 Rb gene, 243 management o , 248
vitreous hemorrhage and, 112 RD. See Rhegmatogenous retinal prognosis or, 248
Proli erative vitreoretinopathy (PVR), detachment von Hippel–Lindau (VHL) disease,
347, 366–367 Reactive hyperplasia, o retinal pigment 247
causes o , 366 epithelium, 255 Retinal capillary nonper usion, 156,
classif cation o , 366t Reddish-brown papular rash, 241 163, 165f, 170, 178, 284, 289,
clinical signs o , 366 Re sum’s disease, 233 294, 295, 339
diagnostic evaluation o , 366 Relative a erent papillary de ect Retinal cavernous hemangioma, 163,
epidemiology o , 366 ( PD), 262 250–251, 251f
f xed retinal olds, 371f Reticulum cell sarcoma. See Intraocular causes o , 250
history, 366 lymphoma central nervous system (CNS) and,
management o , 367 Retina, hamartoma o , combined with 250
prognosis or, 367 hamartoma o retinal pigment clinical signs o , 250
recurrent RD, 370f epithelium, 255–256, 256f diagnostic evaluation o , 250
retinal tear, 368f Retinal arterial macroaneurysm, di erential diagnosis o , 250
tractional RD, 369f 172–174, 174f epidemiology o , 250
Protein C def ciency, 134, 143, 145, clinical signs o , 172 history o , 250
150 diagnostic evaluation o , 172 management o , 250
Protein S def ciency, 134, 143, 145, 150 di erential diagnosis o , 172 prognosis or, 250
PRP. See Panretinal photocoagulation epidemiology o , 172 Retinal degenerations, 189–239
Pseudo–Best’s disease, 194f management o , 172–173 Retinal detachment
Pseudohypopyon stage, o Best’s pathophysiology o , 172 exudative, 383–385, 385f
disease, 189–190, 192f prognosis or, 172–173 di erential diagnosis o , 383–384
Pseudoxanthoma elasticum, and Retinal artery, central in proli erative diabetic retinopathy,
angioid streaks, 72 and vein obstruction, combination, 113
Purtscher’s retinopathy, 134, 327–328, 156–157, 157f rhegmatogenous, 357–365,
328f causes o , 156 359f–365f
causes o , 327 clinical signs o , 156 Retinal dialysis, 346, 351f
clinical signs o , 327 diagnostic evaluation o , 156 Retinal dragging, 289
diagnostic evaluation o , 327 di erential diagnosis o , 156 Retinal dystrophies, 189–239
di erential diagnosis o , 327 epidemiology o , 156 Retinal edema, in retinal artery
epidemiology o , 327 management o , 157 macro-aneurysm, 172
history o , 327 pathophysiology o , 156 Retinal emboli, 338
management o , 327 prognosis or, 157 Retinal olds, 89
prognosis or, 327 Retinal artery macroaneurysm, Retinal hemorrhage, 19, 169f, 170f
PVD. See Posterior vitreous detachment 172–174, 174f Retinal intra-arterial emboli, 142
PVR. See Proli erative vitreoretinopathy Retinal break, 346–347, 357, 372 in central retinal artery obstruction,
Pyridoxine, or gyrate atrophy, 216 causes o , 346 149, 152f
clinical signs o , 346–347 Retinal ischemia, 154
R diagnostic evaluation o , 347 Retinal macroaneurysm, 321
Radiation optic neuropathy, a er di erential diagnosis o , 347 Retinal necrosis, acute, 269
teletherapy, 185f epidemiology o , 346 Retinal neovascularization, 178
Radiation retinopathy, 96, 134, history o , 346 Retinal pigment epithelium (RPE), 45,
182–183 management o , 347, 354f, 355f, 356f 58, 187, 189, 314, 357
a er teletherapy, 184f–185f prognosis or, 347 abnormalities, 1
In d e x 399
in Best’s disease, 189 pericentric, 229 Retinoschisis, 267

bull’s-eye pa ern o , 203 prognosis or, 229–230 degenerative, 380–382, 381f–382f
causes o , 255 Re sum’s disease, 233 juvenile X-linked, 307–311, 309f–311f
changes, 319 Sanf lippo’s diseases, 234 peripheral, 307, 310f
clinical signs o , 255 sector, 229, 232f Rhegmatogenous retinal detachment,
congenital hypertrophy o , 252–254, systemic diseases associated with, 78, 357–359, 359f, 360f
253f–254f 233–235 causes o , 357
cystoid macular edema, 230 Usher’s syndrome, 233 chronic, 361f, 362f
detachment o , 17, 19, 31f–33f Retinitis pigmentosa sine pigmento, clinical signs o , 357
diagnostic evaluation o , 255 229 diagnostic evaluation o , 357
di erential diagnosis o , 255 Retinitis punctata albescens, 219, 229 di erential diagnosis o , 357
epidemiology o , 255 Retinoblastoma (Rb), 241, 243–246, epidemiology o , 357
hamartoma o , combined with 245f, 246f, 384 history, 357
hamartoma o retina, 255–256, causes o , 243 pigmentary disturbances, 363f
256f children with germinal, 244 prognosis or, 358–359
history o , 255 clinical signs, 243–244 regressed, 363f
hyperplasia, 175 diagnostic evaluation o , 244 retinal incarceration, 365f
management o , 255 di erential diagnosis o , 244 scleral buckle, 365f
prognosis or, 255 epidemiology o , 243 subretinal hemorrhage, 364f
reactive hyperplasia o , 255 history o , 243 Ritalin, 338
retina, hamartoma o , 255, 256f management o , 244–245 Rod dys unction, 236
tears, 19, 36f–37f prognosis or, 244 ROP. See Retinopathy o prematurity
Retinal tear. See Retinal break Retinochoroidal anastomoses, 19 RP. See Retinitis pigmentosa
Retinal telangiectasis. See Coats’ Retinopathy, 182 RPE. See Retinal pigment epithelium
disease central serous, 78–79, 79f–88f Rubeosis iridis, 166, 357, 366
Retinal tumors, 240–272 chloroquine, 341–342, 342f Rush disease, o retinopathy o
Retinal vascular disease, 133–188 diabetic, 94–98 prematurity, 274
Retinal vascular whitening, 187 and co on-wool spots, 95
Retinal vasculature, o in ants, 273 nonproli erative, 95–98, S
Retinal vasculitis, 270f 99f–103f Salmon patch hemorrhage, in sickle cell
Retinal vein obstruction, 158 hydroxychloroquine, 341–342 retinopathy, 178
branch, 163–165, 165f hypertensive, 136–137, 138f–141f Sanf lippo’s diseases, 234
central, 156–157, 157f proli erative diabetic, 112–113, Scarring
Retinal vein occlusion, 96 99f–111f disci orm, exudative age-related
Retinal whitening Purtscher’s, 327–328, 328f macular degeneration and, 17,
in acute ophthalmic artery radiation, 182–183, 184f–186f 19, 21f
obstruction, 153, 155f sickle cell, 178–179, 179f–181f macular, angioid streaks and, 72, 75f
in central retinal artery obstruction, talc, 338–340, 340f Scleral buckle, or rhegmatogenous
149 thioridazine, 343–345, 344f–345f retinal detachment, 358–359,
with traumatic injury, 314, 315f Valsalva, 321–322, 322f 365f
Retinitis pigmentosa (RP), 210, Retinopathy o prematurity (ROP), Scleral buckling surgery, 89, 92
228–232, 230f, 231f 273, 277f Scleritis, posterior, 78, 89, 92, 265
atypical, 229 antiangiogenic injection therapy, 276 Sea ans, in sickle cell retinopathy, 178,
Bardet–Biedl syndrome, 234 causes o , 273 180f
Bassen–Kornzweig syndrome, clinical signs o , 274–275 Sector laser panretinal
233 complication o , 281f photocoagulation, or branch
causes o , 288 diagnostic evaluation o , 273–274 retinal vein obstruction, 164
clinical signs o , 228–229 di erential diagnosis o , 275 Senior–Loken syndrome, 294
Cockayne’s syndrome, 233 epidemiology o , 273 Serous pigment epithelial detachment,
diagnostic evaluation o , 229 history, 273 18, 34f–35f
epidemiology o , 228 laser photocoagulation or, 283f Shaken baby syndrome, 323–324
with exudative vasculopathy, 229 management o , 275–276 clinical signs o , 323
Friedreich’s ataxia, 235 plus disease, 280f diagnostic evaluation o , 323
history o , 228 prognosis or, 275–276 di erential diagnosis o , 323
Kearns–Sayre syndrome, 234 retinal dragging, 281f epidemiology o , 323
Laurence–Moon syndrome, 234 stages o severity, 277f–279f history o , 323
management o , 229–230 threshold disease, 274, 280f intraretinal and preretinal
mucopolysaccharidoses, 234 Retinopexy, pneumatic, or hemorrhages, 324f
neuronal ceroid lipo uscinosis, rhegmatogenous retinal management o , 323
234–235 detachment, 358 prognosis or, 323
4 0 0 In d e x
Sickle cell anemia, and angioid streaks, 72 idiopathic macular hole, 50 yroid carcinoma, 264
Sickle cell disease, 134, 143, 145, 150, scleral buckling, 89, 92 Tilted disc syndrome, 68
154 vitreomacular traction syndrome Total serosanguineous retinal
Sickle cell retinopathy, 178–179 (VMTS), 58 detachment, 64
black sunburst lesion, 179f wet (exudative) AMD, 20 Toxic retinopathies, 314–345
clinical signs o Sydenham’s chorea, 143, 145, 150 Toxocariasis, ocular, 275, 295, 304
nonproli erative mani estations, Sympathetic ophthalmia, 383 Toxoplasmosis, ocular, 300
178 Systemic lupus erythematosus, 149 Transpupillary thermotherapy ( T),
proli erative changes, 178 260
diagnostic evaluation o , 178 T Trauma, angioid streaks and, 73, 76f–77f
di erential diagnosis o , 178 Talc retinopathy, 338–339 Traumatic choroidal rupture, 72
epidemiology o , 178 causes o , 338 Traumatic macular hole, 329–330, 330f
management o , 178–179 clinical signs o , 338 causes o , 329
pathophysiology o , 178 diagnostic evaluation o , 338 clinical signs o , 329
peripheral retinal neovascularization, di erential diagnosis o , 338 diagnostic evaluation o , 329
180f–181f epidemiology o , 338 di erential diagnosis o , 329
prognosis or, 178–179 history o , 338 epidemiology o , 329
Sickled red blood cells, 178 management o , 339 history o , 329
Skin hypopigmentation, 223 prognosis or, 339 management o , 329
Small drusen, 1, 7f yellow re ractile particles, 340f prognosis or, 329
Smokestack appearance, in central Tamoxi en (Nolvadex), and talc Traumatic retinopathies, 314–345
serous retinopathy, 79, 86f retinopathy, 338 True albinism, 223
So drusen. See Large drusen Telangiectasia TS. See Tuberous sclerosis
Solar maculopathy, 319–320 localized, 295 T. See Transpupillary thermotherapy
clinical signs o , 319 para oveal, 175–177, 176f–177f Tuberous sclerosis (TS), and astrocytic
diagnostic evaluation o , 319 retinal. See Coats’ disease hamartomas, 240–241
di erential diagnosis o , 319 Telangiectatic retinal vascular changes, Tumors
epidemiology o , 319 177f choroidal, 240–272
management o , 319 Teletherapy, and radiation retinopathy, retinal, 240–272
prognosis or, 319 182, 184f–185f Tumor-suppressor gene, 241
sungazing and, 319 Temporary balloon, or Turner’s syndrome, 294
yellow oveal lesion, 319f–320f rhegmatogenous retinal Type A personality and CSR, 78
Solar retinopathy, 50, 329 detachment, 358 Tyrosinase-negative albinos, 224
Spielmeyer–Vogt disease, 235 Terson’s syndrome, 325–326, 326f
Stargardt’s disease, 203–209, 208f, 209f causes o , 325 U
with beaten bronze macula, 206f clinical signs o , 325 Ultrasonography, 260
“bull’s-eye” macula, 207f diagnostic evaluation o , 325 Usher’s syndrome, 233
bull’s-eye pa ern, 204 di erential diagnosis o , 325
causes o , 203 epidemiology o , 325 V
clinical signs o , 203 history o , 325 Valsalva retinopathy, 321, 322f
diagnostic evaluation o , 203–204 management o , 325 clinical signs o , 321
di erential diagnosis o , 203 prognosis or, 325 diagnostic evaluation o , 321
epidemiology o , 203 retinal and preretinal hemorrhages, di erential diagnosis o , 321
history o , 203 326 epidemiology o , 321
management o , 204 ermal laser photocoagulation, or history o , 321
pisci orm ecks, 205f exudative age-related macular management o , 321
prognosis or, 204 degeneration, 20, 39f prognosis or, 321
RPE, loss o , 208f ioridazine retinopathy, 343 retinal hemorrhages, 322f
Stickler’s syndrome, 372 causes o , 343 Vascular endothelial growth actor
Strabismus, and retinoblastoma, 243, clinical signs o , 343 (VEGF), 247
245f diagnostic evaluation o , 343 Vasculitis, retinal, 270f
Stretch tears o retina, 347, 352f di erential diagnosis o , 343 Vasculopathy, polypoidal choroidal,
Sturge–Weber syndrome, 267 epidemiology o , 343 64–66, 65f–66f
Submacular hemorrhage, and angioid history o , 343 Vasoproli erative retinal tumor, 248
streaks, 72, 72f, 76f management o , 343 VEGF. See Vascular endothelial growth
Sur ace wrinkling retinopathy. See nummular retinal pigment epithelial actor
Macular epiretinal membrane loss, 344f–345f VEGF injections, 20
Surgery prognosis or, 343 Vein obstruction, central
cystoid macular edema (CME), 61 reshold disease, o retinopathy o and retinal artery, combination,
hypotony maculopathy, 92 prematurity, 274, 280f 156–157, 157f
In d e x 401
Venous beading, in nonproli erative prognosis or, 58 in A rican Americans, 19

diabetic retinopathy, 95–96, retinal striae in, 58 Amsler grid monitoring, 17
96t, 104f spectral-domain OCT, 58 anti-VEGF injection therapy, 20
Verteporf n photodynamic therapy vitrectomy or, 58 in Asian Americans, 19
(PDT) Vitreoretinal tu s, 374–375, 375f causes o , 17
or degenerative myopia, 68 causes o , 374 clinical signs o , 17–19
or exudative age-related macular clinical signs o , 374 CNV and, 17, 18
degeneration, 20, 40f–43f di erential diagnosis o , 374 comparative AMD treatment trial
VHL disease. See von Hippel-Lindau epidemiology o , 374 (CA Study), 20
disease history o , 374 di erential diagnosis o , 19–20
Vitamin A management o , 374 disci orm scarring, 17
or Bassen–Kornzweig syndrome, meridional old, 375f early changes, 17
233 prognosis or, 374 in elderly, 17
or retinitis pigmentosa, 230 Vitreoretinopathy epidemiology o , 17
Vitamin B6, or gyrate atrophy, 216 amilial exudative, 289–293, evidence-based therapy, 20
Vitamin E, or Bassen–Kornzweig 290f–293f uorescein angiographic
syndrome, 233 proli erative, 366–371, 366t, classif cation, 17
Vitelli orm stage, o Best’s disease, 189, 368f–371f uorescein leakage, 18
191f Vitreous base, avulsed, 318, 318f history o , 17
Vitelliruptive stage, o Best’s disease, Vitreous hemorrhage, in proli erative incidence o , 17
190, 193f diabetic retinopathy, 112, late, 17
Vitrectomy, 50 121f–122f loss o vision in, 17
or proli erative diabetic retinopathy, chronic, 179 management o , 20
113, 128f–129f VMTS. See Vitreomacular traction OCT imaging, 17
or rhegmatogenous retinal syndrome PDT or, 20
detachment, 358–359 von Hippel–Lindau (VHL) disease, pigment epithelial detachment,
Vitreomacular traction syndrome 247, 249f 17
(VMTS), 58–59, 59f prognosis or, 20
causes o , 58 W retinal pigment epithelial tears
clinical signs o , 58 Watzke–Allen sign, 50, 329 in, 19
diagnostic evaluation o , 58 Waxy pallor, o optic disc, 228 submacular hemorrhage in, 19
di erential diagnosis o , 58 Wegener’s granulomatosis, 134, 143, surgery or, 20
epidemiology o , 58 145, 149, 154, 156 in United States, 17
uorescein angiography o , 58 Weill–Marchesani syndrome, and in Western world, 17
history o , 58 dislocated lens, 336
macular distortion in, 58 Wet (exudative) AMD Y
management o , 58 adjunctive treatment or, 20 Yellow intraretinal crystals, 175

4 115153222871553228

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

4 115153222871553228

Uploaded by

Copyright:

Available Formats

EDITORS

Ch a pt er 1 Age-Related Macular Degeneration 1

Ch a pt er 4 Retinal Vascular Disease 133

Para oveal elangiec asis 175

Ch a pt er 5 Retinal Degenerations and Dystrophies 189

Ch a pt er 6 Retinal and Choroidal umors 240

Ch a pt er 7 Congenital and Pediatric

Ch a pt er 8 raumatic and oxic Retinopathies 314

A ge-rela ed macular degenera ion (AMD)

D rusen are he clinical hallmark o dry

hypo uorescence and la e hyper uorescence be evalua ed wi h uorescein angiography o

CNV is he abnormal grow h o new choroidal HISTORY

Classic Choroidal Neovascularization la e leakage o unde ermined source, is char-

Retinal Pigment Epithelial ears Occasionally, re inochoroidal anas omoses

IMPORTANT DIAGNOSTIC EVALUATION

he second eye. Pa ien s wi h a ull- hickness

TABLE 2-1. S ages o Developmen o Idiopa hic Macular Hole

S age 1 holes appear as a small yellow eyes wi h s age 2, 3, or 4 macular holes

Fluorescein angiography may reveal re inal

FIGURE 2-11. Vitreomacular traction syndrome. A. Adhesion o he vi reous o a premacular membrane

T ere may be no associa ed clinical signs

PROGNOSIS AND requencies o adminis ra ion and combina ion

he re ina and re inal pigmen epi helium. T e

P olypoidal choroidal vasculopa hy (PCV)

T e disc i sel may be ver ically elonga ed or

HISTORY ASSO CIATED

Presumed ocular his oplasmosis degenera ive e ec s upon he re ina. Scleral

o vision loss due o rup ure o he CNV, sub-

serous de achmen (Fig. 2 20). Pa ien s

C SR is a disease in which a circumscribed

F olding or wrinkling o he inner choroid,

segmen signs consis en wi h surgery or

Hypo ony maculopa hy is a condi ion DIFFERENTIAL DIAGNOSIS

D iabe ic re inopa hy encompasses a broad

Excessive glycosyla ion o pro eins Macular edema— he mos common

TABLE 3-1. 4-2-1 Rule Ocular ischemic syndrome

Hyper ensive re inopa hy or

FIGURE 3-3. Sub le HYE near ovea in NPDR.

FIGURE 3-6. Cot on-wool spo s as well as hemorrhage and HYE.

FIGURE 3-9. Sausaging o re inal venules ( arrow) seen in severe NPDR.

FIGURE 3-10. Venous loop.

FIGURE 3-14. OC shows cys ic edema in cen ral macula.

(Fig. 3-22). Involvemen o he an erior

FIGURE 3-18. Severe eleva ed NVD.

FIGURE 3-23. Mild vi reous hemorrhage in PDR.

FIGURE 3-25. Boa -shaped prere inal hemorrhage in PDR.

FIGURE 3-26. More dense vi reous hemorrhage.

FIGURE 3-29. PRP scars in PDR. No e sparing o he macula.

FIGURE 3-30. Equa or-plus pho o a er ull PRP.

EPIDEMIOLOGY DIAGNOSTIC EVALUATION

A crowded op ic disc is o en presen ,

e inal Vascular Disease

C ot on-wool spo s describe he re inal

DIAGNOSTIC EVALUATION Miscellaneous: Migraine, Lyme disease,

re inopa hy. Ar eriovenous nicking o a re inal

H yper ensive re inopa hy re ers o he

PATHOPHYSIOLOGY DIFFERENTIAL DIAGNOSIS

DIAGNOSTIC EVALUATION resolu ion o he undus abnormali ies over a

Fundus changes: T ree varian s occur:

C iliore inal ar ery obs ruc ion is he acu e

DIFFERENTIAL DIAGNOSIS syndrome, pro ein S de ciency, pro ein C

Choles erol (Hollenhors plaque):

B ranch re inal ar ery obs ruc ion is he