Color Atlas of Ophthalmology

Color Atlas of Ophthalmology
The Quick-Reference Manual

for Diagnosis and Treatm ent
Second Edit ion
Color Atlas of Ophthalmology
The Quick-Reference Manual
for Diagnosis and Treatm ent
Second Edit ion
Amar Agarw al, MS, FRCS, FRCOphth

Professor of Ophthalmology
Ram achandra Medical College
Private Practice
Chennai, India
Soosan Jacob, MS, FRCS, DNB, MNAMS

Private Practice
Chennai, India
Th iem e
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Library of Congress Cataloging -in-Publication Data
Color atlas of ophthalm ology : the quick-reference m anual for diagnosis and treatm ent / [edited
by] Am ar Agarwal, Soosan Jacob.—2nd ed.
p. ; cm.
Includes index.
Prev. ed. has subtitle: Manhat tan Eye, Ear & Throat Hospital pocket guide.
ISBN 978-1-60406-211-3 (alk. paper)
1. Eye—Diseases—Atlases. 2. Eye—Diseases—Handbooks, manuals, etc.
3. Ophthalm ology—Atlases. 4. Ophthalm ology—Handbooks, m anuals, etc.
I. Agarwal, Am ar. II. Jacob, Soosan.
[DNLM: 1. Eye Diseases—diagnosis—Atlases. 2. Eye Diseases—diagnosis—Handbooks.
3. Eye Diseases—pathology—Atlases. 4. Eye Diseases—pathology—Handbooks. 5. Eye—
pathology—Atlases. 6. Eye—pathology—Handbooks. 7. Opthalmologic Surgical Procedures—
Atlases. 8. Ophthalmologic Surgical Procedures—Handbooks. WW 17 C7195 2009]
RE71.C65 2009
617.70022'3—dc22
2009020093
Copyright © 2010 by Thiem e Medical Publishers, Inc. This book, including all part s thereof, is le-
gally protected by copyright. Any use, exploitation, or com m ercialization out side the narrow lim -
its set by copyright legislation without the publisher’s consent is illegal and liable to prosecution.
This applies in particular to photostat reproduction, copying, m im eographing or duplication of
any kind, translating, preparation of m icrofilm s, and electronic data processing and storage.
Important note : Medical knowledge is ever-changing. As new research and clinical experience
broaden our knowledge, changes in treatm ent and drug therapy m ay be required. The authors
and editors of the m aterial herein have consulted sources believed to be reliable in their effort s
to provide information that is complete and in accord with the standards accepted at the time
of publication. However, in view of the possibilit y of hum an error by the authors, editors, or
publisher of the work herein or changes in m edical knowledge, neither the authors, editors, nor
publisher, nor any other part y who has been involved in the preparation of this work, warrants
that the inform ation contained herein is in every respect accurate or complete, and they are not
responsible for any errors or omissions or for the results obtained from use of such inform ation.
Readers are encouraged to confirm the information contained herein with other sources. For
example, readers are advised to check the product inform ation sheet included in the package of
each drug they plan to adm inister to be certain that the inform ation contained in this publication
is accurate and that changes have not been m ade in the recom m ended dose or in the contraindi-
cations for adm inistration. This recom m endation is of particular importance in connection with
new or infrequently used drugs.
Som e of the product names, patents, and registered designs referred to in this book are in
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always m ade in the text. Therefore, the appearance of a nam e without designation as proprietary
is not to be construed as a representation by the publisher that it is in the public domain.
Printed in India
54321
ISBN 978-1-60406-211-3
Noth ing in th is w orld m oves w ith out Him ,
an d so also th is book w as on ly w rit ten by Him .
Th is book is dedicated to
a great frien d,
Brian S. Boxer Wach ler, MD

Contents
Fo rew o rd . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .ix
Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .xi
Abo ut the Edito rs. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiii
Co ntributo rs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xv
Chapter 1 Ocu lar Traum a . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
Daniele Veritt i, Carlo Sborgia, Francesco Boscia,
Giuseppe Sm aldone, and Paolo Lanzetta
Chapter 2 Eyelids an d Lacrim al System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
Christopher I. Zoum alan, Andrea Olm os, and
Kim berly P. Cockerham
Chapter 3 Orbit al In fect ion s, In am m at ion , an d Neoplasm s . . . . . . . . . . . . . . . . 69
Praveen Saluja, Sw at i Ravani, Soosan Jacob, and Am ar Agarw al
Chapter 4 Extern al Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100
Guillerm o Sim ón-Castellví, Pablo Gili-Manzanaro,
Sarabel Sim ón-Castellví, José María Sim ón-Castellví,
Crist ina Sim ón-Castellví, and José María Sim ón-Tor
Chapter 5 Corn ea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141
W . Barry Lee and Ivan R. Schw ab
Chapter 6 In t raocu lar In am m at ion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194
Soosan Jacob, Dhivya Ashok Kum ar, Athiya Agarw al, and
Am ar Agarw al
Chapter 7 Len s an d Cataract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 218
Athiya Agarw al, Soosan Jacob, and Am ar Agarw al
Chapter 8 Glaucom a . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 244
Soosan Jacob and Am ar Agarw al
Chapter 9 Medical Ret in a . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 260
Mandeep Lam ba, Soosan Jacob, and Am ar Agarw al
Chapter 10 Su rgical Ret in a . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 288
Clem ent K. Chan and Dariusz G. Tarasew icz
Chapter 11 Ocular Neoplasm s. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 322
Soosan Jacob, Santosh Hanovar, and Am ar Agarw al
Chapter 12 St rabism u s . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 339
Federico G. Velez, Noa Ela-Dalm an, and Arthur L. Rosenbaum
Chapter 13 Neuro- Op h th alm ology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359
vii
viii Content s
Chapter 14 Oph th alm ic Ph arm acology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 405

Jam es M. Hill, Lori Vidal Denham , Blake A. Booth,
Duncan A. Friedm an, Je ery A. Hobden, Andrea T. Murina,
Marie D. Acierno, Jean T. Jacob, Herbert E. Kaufm an, and
Donald R. Bergsm a
Chapter 15 Ocu lar Man ifest at ion s of System ic Disease . . . . . . . . . . . . . . . . . . . . . 430
Chapter 16 Con tact Len ses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 447
Kenneth M. Daniels
Chapter 17 Oph th alm ic In st r um en t s an d Diagn ost ic Tests . . . . . . . . . . . . . . . . . . 486
Sam uel Boyd and Am ar Agarw al
Chapter 18 Oph th alm ic O ce Procedures. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 507
Sam uel Boyd
Chapter 19 Refract ive Su rger y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 518
David R. Hardten, Natalia Kram arevsk y, Louis E. Probst, and
Richard L. Lindst rom
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 539
Forew ord
For som e au th ors of oph th alm ology, w rit ing or edit ing 50 book ch apters is a size-
able accom plish m en t over a lifet im e, let alon e a decade. Th is is n ot t ru e for Professor
Agar w al. Sin ce 1998, Am ar Agar w al h as
edited on average m ore th an fou r books
a year, w h ich is an u npreceden ted an d
virt u ally u n im agin able yearly n u m ber
for any on e person . His books sp an top-
ics from diagn ost ics an d im aging (su ch
as aberrom et r y, angiography, topogra-
phy, dr y eyes, and neuro-ophthalm ology)
to therapeutics and surger y (including
contact len ses, lasers, LASIK, strabism us,
oculoplastics, cataracts, phako, an d m ore),
an d m any h ave been bestsellers. He h as
au th ored several com prehensive hand-
books and textbooks of oph th alm ology,
an d Color Atlas of Ophthalm ology stan ds
out as on e of h is best . In it s 19 su bspe-
cialt y ch apters, th is secon d edit ion illus-
t rates over 500 diagn ost ics an d th erapeu-
t ic con dit ion s. Th e com preh en sive an d
system at ic form at h elp s th e reader fin d
an d view th e facts abou t any con dit ion in
oph th alm ology in st an tly, m aking it an essen t ial resou rce for both th e st u den t an d
th e pract it ion er in th e field.
Anyon e w h o kn ow s Professor Agar w al can tell you th at h e is a con sum m ate
in n ovator an d teach er. As a professor of oph th alm ology an d in tern at ion ally re-
n ow n ed lect urer, h is exp erien ce an d dedicat ion to teach ing speak for th em selves.
Over th e years, I h ave p ar t icip ated in several Am erican Academ y of Oph th alm olo-
gy an d Am erican Societ y of Cataract an d Refract ive Su rger y cou rses w ith Professor
Agar w al an d h ave learn ed a great deal about m icroin cision cataract su rger y an d
th e m an agem en t of ch allenging LASIK cases from th em . Person ally, I look for w ard
to review ing an d referring to th e con ten t of th is book on a regu lar basis. I recom -
m en d th at you do so as w ell!
Ronald R. Krueger, MD, MSE

Medical Director,
Depar t m en t of Refract ive Su rger y
Cole Eye In st it ute
Clevelan d Clin ic
Clevelan d, Oh io
ix
Preface
Every day you m ay m ake progress. Every step m ay be fruitful. Yet there w ill stretch
out before you an ever-lengthening, ever-ascending, ever-im proving path. You k now
you w ill never get to the end of the journey. But this, so far from discouraging, only
adds to the joy and glory of the clim b.
Win ston Ch u rch ill (1874–1965)
W h at is an oph th alm ologist? On e w h o h as gradu ated from m edical sch ool an d is

in terested in th e eye. On e w h o h as com pleted th ree years of sp ecialized oph th al-
m ic t rain ing. On e w h o h as sp en t a fur th er t w o years in sub-specializat ion . On e
w h o h as m en tored th e n ext gen erat ion of oph th alm ologists. On e w h o h as in n o-
vated an d ch anged preferred pract ice p at tern s in oph th alm ology. All th ese classes
of oph th alm ologists n eed solid kn ow ledge com bin ed w ith pat ien t em pathy to best
ser ve p at ien t s.
Th e purpose of th is fu lly revised secon d edit ion of th e su ccessful Color Atlas of
Ophthalm ology: The Quick-Reference Manual for Diagnosis and Treatm ent is to give
you th e kn ow ledge n eeded to face w ith con fiden ce th e fu ll range of pat ien ts’ op h -
th alm ic disorders. In ou r field, w h ere w e n ecessarily learn , diagn ose, an d teach
visu ally, top -fligh t clin ical p h otograph s, like th e h u n dreds in th is book (all n ew to
th is edit ion ), are essen t ial. Th e n in eteen ch apters, w rit ten by expert con t ribu tors
from North Am erica, Eu rope, an d In dia, are w ell-organ ized an d con cise, an d th e
form at of “Presen tat ion -Differen t ial Diagn osis-Man agem en t” is design ed for easy
an d speedy referen ce, so you don’t h ave to w ade th rough ext ran eou s m aterial to
fin d w h at you n eed. Th is pocket atlas is in ten ded for th e residen t on rou n ds or
st udying for an exam , th e pract icing oph th alm ologist w h o n eeds con firm at ion of
an un usual fin ding, or th e prim ar y physician w h o requ ires a reliable guide to eye
disorders.
We are gratefu l to th e people at Th iem e Pu blish ers, especially to J. Ow en Zurh el-
len an d Dom in ik Pu cek. We an d Th iem e h ope an d expect th at ou r pocket atlas aids
you in p roviding pat ien t s w ith th e best care possible.
Am ar Agarw al, MS, FRCS, FRCOphth

Ch en n ai, In dia
xi
About the Editors
Amar Agarw al, MS, FRCS, FRCOphth

Dr. Agar w al’s Grou p of Eye Hospitals an d Eye Research Cen t re
Ch en n ai, In dia
Prof. Am ar Agar w al is th e p ion eer of ph akon it , w h ich is ph ako w ith n eedle in ci-
sion tech n ology. Th is tech n ique becam e
popularized as bim an u al ph aco, m icroin -
cision cataract surger y (MICS), or m icro-
ph aco. He is th e first to rem ove cat aracts
th rough a 0.7 m m t ip w ith th e tech n ique
called m icroph akon it . He is also th e
first to h ave discovered an d developed
cataract su rger y w ith out an esth esia. An -
oth er of h is in n ovat ion s is FAVIT (fallen
vit reous), a n ew tech n ique for rem oving
dropped n uclei. Th e air pu m p, based on
th e sim ple idea of using an aqu ariu m
pum p to in crease th e flu id in fusion in to
th e eye in bim an u al ph aco an d coaxial
ph aco, h as h elped preven t u n st able an -
terior ch am ber an d su rge du ring cata-
ract su rger y. Th is provided th e basis for variou s tech n iqu es of forced in fu sion for
sm all in cision cat aract surger y. Dr. Agar w al w as also th e first to use t r ypan blu e for
stain ing ep iret in al m em bran es an d p ublish ed th e det ails in h is four-volu m e text-
book of op h th alm ology. He h as also discovered a n ew refract ive error called aber-
ropia. He h as been th e first to do a com bin ed su rger y of m icroph akon it (700 µm
cataract surger y) w ith a 25-gauge vit rectom y in th e sam e pat ien t , th u s u sing th e
sm allest in cision s possible for cat aract an d vit rectom y. An d h e is th e first surgeon
to im plan t a n ew m irror telescopic in t raocu lar len s (IOL) Lipsh it z Macu lar Im plan t
(LMI) for pat ien ts suffering from age-related m acular degen erat ion . Th e Malyugin
ring for sm all pu pil cat aract surger y w as also m odified by h im an d is kn ow n as
th e Agar w al m odificat ion of th e Malyugin ring for m iot ic pu pil cat aract surgeries
w ith posterior capsular defect s. He h as been th e w orld’s first to im plan t a glu ed
IOL. In th is procedure a posterior ch am ber (PC) IOL is fixed in th e eye w ith ou t any
capsules, using fibrin glu e.
An terior segm en t opt ical coh eren ce tom ography w as u sed for th e first t im e in
Dr. Agar w al’s eye h ospital to qu an t ify ch anges in an terior ch am ber (AC) m orp h ol-
ogy in in flam m at ion , assessing all th e param eters. Th e hyperreflect ive spots de-
tected in th e AC w ere cou n ted m an u ally an d w ith an au tom ated com pu ter algo-
rith m . Also for th e first t im e, pseudoph akic bu llou s keratopathy w as m an aged by
a com bin at ion of In t raLase (Abbot t Medical Opt ics, San t a An a, CA) keratoplast y,
explan t at ion of th e AC IOL, vit rectom y, an d im plan t at ion of a glu ed PC IOL. A n ew
su rgical tech n ique, Jacob-Agar w al sling surger y, w as devised for acquired an d con -
gen it al ptosis w ith poor levator fu n ct ion in w h ich th e Seiff silicon e su spen sion set
is u sed in a fron t alis sling procedu re. Th e en t ire length of th e silicon e set is gu ided
in th e m uscle plan e th rough a single st ab in cision .
xiii
xiv About the Editors
Prof. Agar w al h as received m any aw ards for h is w ork in oph th alm ology, th e m ost
sign ifican t being th e Barraquer Aw ard an d th e Kelm an Aw ard. His videos h ave w on
m any aw ards at film fest ivals of th e Am erican Societ y of Cat aract an d Refract ive
Surger y, Am erican Academ y of Oph th alm ology, an d Eu ropean Societ y of Cataract
an d Refract ive Su rger y. He h as also w rit ten m ore th an 40 books, w h ich h ave been
p ublish ed in variou s langu ages, in cluding English , Span ish , an d Polish . He t rain s
doctors from all over th e w orld in h is cen ter on ph aco, bim an ual ph aco, LASIK, an d
vitreo-ret in al su rger y. He is a professor of oph th alm ology at Ram a ch an dra Medical
College in Ch en n ai, In dia, an d can be con t acted th rough th e h ospital’s Web site at
h t tp://w w w.dragar w al.com .

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

Professor of Oph th alm ology
Ram ach an dra Medical College
Dr. Agar w al’s Grou p of Eye Hospitals & Eye Research Cen t re
Ch en n ai, In dia
Athiya Agarw al, MD, FRSH, DO

Director
Ch en n ai, In dia
Do nald R. Bergsm a, MD
Herbert E. Kau fm an Professor an d Ch airm an
Director
LSU Eye Cen ter of Excellen ce
New Orlean s, LA
Blake A. Bo oth, MD
Residen t
Un iversit y of Alabam a at Birm ingh am
Birm ingh am , AL
Francesco Bo scia, MD
Dirigen te Medico I Livello
Depart m en t of Oph th alm ology an d Otolar yngology
Un iversit y Bari
Bari, It aly
Sam uel Boyd, MD

Ch ief
Vit reoret in al Depart m en t
Boyd Eye Cen ter
Pan am a Cit y, Republic of Pan am a
xv
xvi Contributors
Clem ent K. Chan, MD

Presiden t an d Medical Director
South ern Californ ia Desert Ret in a Con su ltan t s
Associate Clin ical Professor
Lom a Lin da Un iversit y
Lom a Lin da, CA
Kim berly P. Co ckerham , MD, FACS

Adju n ct Clin ical Professor
St an ford Un iversit y Sch ool of Medicin e
Private Pract ice
Cockerh am Eye Con su lt an ts
Los Altos, CA
Kenneth M. Daniels, OD, FAAO

Adju n ct Assist an t Clin ical Professor
Cen ter for In tern at ion al St u dies
Pen n sylvan ia College of Optom et r y at Salus Un iversit y
Ph iladelph ia, PA
Private Pract ice
Hopew ell-Lam bert ville Eye Associates
Hopew ell, NJ
Lo ri Vidal Denham , MS
Research Assistan t
Depart m en t of Bioch em ical Engin eering
Tu lan e Un iversit y
New Orlean s, LA
No a Ela-Dalm an, MD
Clin ical In st r uctor
Jules Stein Eye In st it u te
UCLA Sch ool of Medicin e
Los Angeles, CA
Duncan A. Friedm an, MD, MPH

Residen t Physician
Un iversit y of Alabam a at Birm ingh am
Birm ingh am , AL
Pablo Gili-Manzanaro
Fu n dación Hospital de Alcorcón
Barcelon a, Spain
Santo sh Hanovar, MD
LV Prasad Eye In st it u te,
Hyderabad, In dia
Contributors xvii
David R. Hardte n, MD, FACS

Adju n ct Associate Professor
Un iversit y of Min n esot a
Min n esot a Eye Con sult an t s
Min n eap olis, MN
Jam es M. Hill, PhD

Assistan t Professor
Depart m en t of Microbiology, Im m u n ology, an d Parasitology
New Orlean s, LA
Jeffery A. Ho bde n, PhD

Assistan t Professor
Depart m en t of Microbiology, Im m u n ology, an d Parasitology
New Orlean s, LA
Jean T. Jaco b, PhD

Professor of Oph th alm ology an d Neu roscien ce
LSH Health Scien ces Cen ter
Director of Research
LSU Eye Cen ter
New Orlean s, LA
So o san Jaco b, MS, FRCS, DNB, MNAMS

Ch en n ai, In dia
Herbert E. Kaufm an, MD

Em erit us Boyd Professor of Oph th alm ology, Ph arm acology, an d Microbiology
New Orlean s, LA
Natalia Kram arevsk y, MD

Virgin ia Beach Eye Cen ter, P.C.
Sen tara Virgin ia Beach Gen eral Hosp ital
Virgin ia Beach , VA
Dhivya Asho k Kum ar, MD

Con su ltan t
Ch en n ai, In dia
Man de ep Lam ba, MBBS, DNB, FERC

Con su ltan t , Ret in a Fou n dat ion
Ch en n ai, In dia
xviii Contributors
Pao lo Lan zetta, MD

Associate Professor
Un iversit y of Udin e
Udin e, Italy
W. Barry Lee, MD
Private Pract ice
Corn ea, Extern al Disease, an d Refract ive Surger y
Eye Con sult an ts of Atlan t a, Piedm on t Hospit al
Atlan ta, GA
Richard L. Lindstro m , MD
Adju n ct Professor Em erit u s
Un iversit y of Min n esot a
Fou n der an d At ten ding Surgeon
Min n esot a Eye Con sult an t s
Min n eapolis, MN
Andrea T. Murina, MD
Residen t
Depart m en t of In tern al Medicin e
New Orlean s, LA
Andrea Olm o s, BS
Un iversit y of Californ ia, San Fran cisco
San Fran cisco, CA
Lo uis E. Pro bst, MD

Nat ion al Medical Director
TLC Th e Laser Eye Cen ters
Ch icago, IL
Sw ati Ravani, MS
Associate Professor
BMJ Region al In st it ute of Oph th alm ology
Ah m edabad, In dia
Arthur L. Ro se nbaum , MD
Brin dell an d Milton Got tlieb Professor of Oph th alm ology
Ch ief, Division of Pediat ric Oph th alm ology an d St rabism us
Vice Ch airm an
Los Angeles, CA
Pravee n Saluja, MS
Ch en n ai, In dia
Contributors xix
Carlo Sbo rgia, MD

Ch air, In st it u te of Oph th alm ology
Un iversit y of Bari
Bari, It aly
Ivan R. Schw ab, MD, FACS

Professor
Un iversit y of Californ ia, Davis Health System Eye Cen ter
Sacram en to, CA
Cristina Sim ó n-Castellví

Sim on Eye Clin ic
Barcelon a, Spain
Guillerm o Sim ó n-Castellví

Ch ief, An terior Segm en t Su rgeon
Sim on Eye Clin ic
Un iversit y of Barcelon a
Barcelon a, Spain
Jo sé María Sim ó n-Castellví

An terior Segm en t Con su lt an t
Em ergen cy Room
Sim on Eye Clin ic
Presiden t
World Federat ion of Cath olic Medical Associat ion s
Barcelon a, Spain
Sarabel Sim ó n-Castellví

Ch ief, Posterior Segm en t Su rgeon
Sim on Eye Clin ic
Barcelon a, Spain
Jo sé María Sim ó n-To r

Ch airm an
Glaucom a Sen ior Con su ltan t
Sim on Eye Clin ic
Barcelon a, Spain
Giuseppe Sm aldo ne, MD

Residen t
Un iversit y of Bari
Bari, It aly
Dariusz G. Tarasew icz, MD, PhD

Ret in a Sect ion Ch ief
Kaiser Perm an en te
Sacram en to, CA
xx Contributors
Fede rico G. Velez, MD

Assistan t Clin ical Professor
Los Angeles, CA
Director
Division of Pediat ric Op h th alm ology an d St rabism u s
Olive View -UCLA Medical Cen ter
Sylm ar, CA
Daniele Ve ritti, MD
Regist rar
Un iversit y of Udin e
Udin e, Italy
Christo pher I. Zo um alan, MD

Clin ical In st r uctor
Division of Oph th alm ic Plast ic an d Recon st ruct ive Su rger y
New York Un iversit y
Langon e Medical Cen ter
Man h at tan Eye, Ear, an d Th roat Hospital
New York, NY
1 Ocular Trauma
Daniele Verit t i, Carlo Sborgia, Francesco Boscia, Giuseppe Sm aldone, and
Paolo Lanzet ta
Anterior Segment Trauma
Eyelid Laceration
Blun t an d pen et rat ing facial t raum a m ay result in eyelid lacerat ion . Th e lacera-
t ion m ay be ext ram argin al, m ay involve th e eyelid m argin , or m ay cau se t issue
loss. Eyelid t rau m a is often associated w ith veh icle acciden ts, falls, sport-related
t raum as, an d assau lts. Eyelid lacerat ion is m ore com m on in young m ales du e to
occup at ion al an d recreat ion al preferen ces. Proper m an agem en t is n ecessar y to
preser ve correct lid dyn am ics an d cosm et ic appearan ce.
Presentation
Pat ien ts u sually com plain of m ild pain an d epiph ora. Displacem en t or abn orm ali-
t ies of th e can th al angles m ay in dicate can th al ligam en t injur y. Lacerat ion s of th e
deep h ead of th e m edial can th al ligam en t m ay cau se telecan th u s. Hyph em a, oth er
ocu lar adn exa t raum as, an d orbit al fract ures m ay be presen t (Fig. 1.1 ).
Management
Th e m ech an ism of inju r y sh ould be invest igated first , follow ed by a com plete ocu -
lar exam in at ion to ru le out injuries to th e globe. If n o globe rupt u re is presen t ,
lids sh ou ld be everted, palpated, an d exam in ed for foreign bodies. Th e lacerat ion
sh ould be carefu lly exam in ed to determ in e depth , exten sion , an d m argin involve-
m en t . Ph otography of th e lesion s is recom m en ded. Can alicular involvem en t an d
inju r y to th e levator an d th e supraorbit al n er ve sh ould be exclu ded. A com pu ted
tom ograph ic scan sh ou ld be obt ain ed w h en globe rupt ure an d foreign bodies are
Fig. 1.1 Eyelid laceration involving the eyelid margin with

loss of tissue.
1
2 Color Atlas of Ophthalm ology
su spected. Tetan u s prophylaxis an d baselin e serology for h u m an im m un odefi-

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

Th e lacrim al drain age apparat u s con sist s of th e lacrim al pu n cta on th e upp er lid
an d th e low er lid, th e can aliculi, th e com m on can aliculu s, th e lacrim al sac, an d
th e n asolacrim al du ct . From th eir origin at th e pun ct a, th e can alicu li run m edi-
ally tow ard th e in tern al angulu s of th e eye, w h ere th ey join to form th e com m on
lacrim al can alicu lus th at open s in th e lacrim al sac. Can alicu lar lacerat ion s are th e
m ost frequen t cau se of injur y to th e lacrim al system an d occu r in up to 16% of all
eyelid injuries. Com m on cau ses of can alicu lar lacerat ion in clu de veh icle acciden t s,
falls, assau lts, sh arp t rau m a, an d an im al bites. Su ccessfu l m an agem en t of th ese
inju ries depen ds on prom pt in ter ven t ion an d good surgical tech n ique to m in im ize
th e in ciden ce of post t rau m at ic epiph ora due to scarring an d sten osis in any t ract
of th e lacrim al drain age system .
Presentation
Pat ien ts u su ally presen t w ith a h istor y of t raum a an d m ild pain . Th e lacrim al
drain age system lesion m ay be obvious or occult . Th e u se of m ethylen e blu e or flu-
orescein -t inged w ater irrigat ion th rough th e pu n cta an d subsequ en t visu alizat ion
of th e dye in th e w oun d m ay be h elpful in iden t ifying th e cut en d (Fig. 1.2A,B).
Differential Diagnosis
Lid lacerat ion n ot involving th e lacrim al drain age system , p reexist ing epiph ora
Management
Th e m ech an ism of inju r y sh ou ld be invest igated, an d a com plete oph th alm ic ex-
am in at ion sh ould be perform ed. Th e injur y to th e lacrim al drain age system can
be proven w ith Bow m an probe in sert ion in th e pun ct a or by irrigat ion w ith fluo-
rescein -stain ed salin e solu t ion . Tet an us p rophylaxis sh ou ld be con sidered. Surgi-
cal repair sh ould p rovide accu rate app roxim at ion of th e severed en ds to prom ote
m u cosal h ealing. Most su rgeon s use silicon e in t u bat ion s of th e system , follow ed
by apposit ion of th e perican alicu lar t issues w ith m icroscopically assisted 7–0 su -
t u re. Th e m edial can th al ligam en t is often injured from th e t raum a an d m u st be
repaired to restore lid fun ct ion an d an atom y. Th e success rate w ith silicon e in t uba-
t ion an d m icroscopic rean astom osis ranges from 86 to 95%.
1 Ocular Traum a 3
Fig. 1.2 (A) Lacrimal system trauma

with laceration of the inferior cana-
liculus. (B) Canalicular injury with
eyelid laceration.
Subconjuctival Hemorrhage
Su bconju n ct ival h em or rh age follow s t h e bleed in g of conju n ct ival an d ep iscleral
blood vessels in to t h e su bconju n ct ival sp ace. It is u su ally associated w it h m in or
t rau m a or ar ises sp on t an eou sly w it h in creased ven ou s p ressu re d u e to violen t
Valsalva m an eu vers. Less frequ en t ly su bconju n ct ival h em or rh age can be associ-
ated w it h severe hyp er ten sion an d coagu lop at h ies. Var iou s d r ugs, su ch as w ar fa-
r in , n on steroidal an t iin flam m ator y d r ugs (NSAIDs), an d steroid s can m ake con -
ju n ct ival vessels m ore su scept ible. It is also a n or m al sequ ela of ocu lar su rger y.
Presentation
A brigh t red an d flat collection of blood is seen underneath the conjunctiva; it is usu-
ally sharply dem arcated at the lim bus and surrounded by n orm al conjunctiva. This
condition is usually asym ptom atic. If pain, ph otophobia, or dim inished visual acuit y
occurs, a m ore serious pathological condition should be considered (Fig. 1.3 ).
Th e differen t ial diagn osis of subconju n ct ival h em orrh age in clu des oth er cau ses of
red eye, such as conju n ct ivit is, episclerit is, irit is, acu te glaucom a, an d den drit ic ul-
cer. Kaposi sarcom a, or oth er conju n ct ival n eoplasm s w ith secon dar y h em orrh age
sh ould be t aken in to con siderat ion .
Fig. 1.3 Subconjunctival hemorrhage. A bright red

and flat collection of blood is seen underneath the con-
junctiva; it is sharply dem arcated and surrounded by
normal conjunctiva.
Management
Blood pressure sh ould be ch ecked in all pat ien t s, an d if th ere is a h istor y of re-
curren t , u nprovoked subconju n ct ival h em orrh ages, a bleeding diath esis sh ou ld be
invest igated. Th e u n com plicated h em orrh age, n ot associated w ith any sign ifican t
t raum a or bleeding diath esis, is t ypically a self-lim it ing con dit ion th at requ ires
on ly reassu ran ce. Cold com presses for 24 h ou rs an d ar t ificial tears can be used for
m ild irrit at ion . Hem orrh age clears spon tan eously in 1 to 2 w eeks. Elect ive use of
NSAIDs is t ypically discou raged.
Conjunctival Laceration
Th e conju n ct iva is a st rong an d resilien t t issue, but it m ay be lacerated in cases
of ocu lar t raum a w ith poin ted an d sh arp objects, such as broken glass. It m ay be
isolated or par t of m ore severe in t raocular injuries.
Presentation
Pat ien ts u su ally presen t w ith a h istor y of ocu lar t rau m a an d com plain of red eye,
m ild pain , an d foreign body sen sat ion . Slit-lam p exam in at ion reveals a conju n c-
t ival surface defect . Th e edges are u su ally ret racted an d rolled up, disclosing th e
un derlying w h ite sclera. Subconjun ct ival h em orrh ages an d ch em osis are often
presen t . Flu orescein stain ing u n der th e cobalt filter w ill en h an ce th e visualizat ion
of th e defect ( Fig. 1.4 ).
Management
An accu rate h istor y of ocular t raum a an d a com plete oph th alm ic exam in at ion are
n ecessar y: topical an esth esia m ay be used to accurately invest igate th e u n derlying
sclera in search of inju ries an d su bconjun ct ival foreign bodies. How ever, pat ien t s
un der topical an esth esia m ay lose sym ptom s associated w ith th e presen ce of a
foreign body. A Seidel test sh ould be perform ed to ru le ou t a ru pt ured globe.
B-scan ult rason ography an d a com puted tom ograp h ic scan of th e orbit m ay be
usefu l to exclu de in t raocular or in t raorbit al foreign bodies.
1 Ocular Traum a 5
A B
Fig. 1.4 Conjunctival laceration and foreign body. (Courtesy Pablo Gili M.D.)
In th e absen ce of a rupt ured globe or perforat ing inju ries, sm all conjun ct ival
lacerat ion s h eal w ith out su rgical repair. Large lacerat ion s (e.g., greater th an 1.0
to 1.5 cm ) m ay be su t ured (e.g., Vicr yl 8–0). Pressu re patch ing for 24 h ou rs an d
p rophylact ic an t ibiot ic oin t m en t (e.g., gen tam icin ) th ree t im es a day for 4 to 7 days
sh ould su ffice.
Chemical Exposure
Chem ical burns constit ute a true ocular em ergency and should be treated prom ptly.
Ch em ical burn s m ay be caused by eith er acidic or alkalin e agen ts. Acid bu rn s cau se
coagulat ive n ecrosis of th e corn eal epith elium . Th e form at ion of a coagulu m lim -
its pen et rat ion an d corn eal dam age. Hydroflu oric acid is an except ion becau se it
cau ses liquefact ive n ecrosis. Com m on acids causing ocu lar burn s in clude su lfu -
rous acid (presen t in som e bleach es), sulfuric acid (presen t in car bat teries), hydro-
ch loric acid (u sed in sw im m ing pools), n it ric acid, ch rom ic acid, an d acet ic acid.
Alkali burn s are t ypically m ore severe becau se alkalin e agen t s are lipoph ilic an d
pen et rate m ore rapidly th an acids. Th ey com bin e w ith cell m em bran e lipids an d
cau se sapon ificat ion of cell m em bran es, cell death , an d disr upt ion of th e ext racel-
lular m at rix. Th e release of collagen ases an d p roteases after th e inju r y leads to cor-
n eoscleral m elt ing. Alkali su bstan ces th at com m on ly cause ocular burn s con tain
sodiu m hydroxide (caust ic soda), am m on ium hydroxide (fert ilizer produ ct ion ),
potassium hydroxide, an d calciu m hydroxide. Ch em ical burn s are often bilateral
an d are frequ en tly du e to in du st rial an d occupat ion al exposu res.
Presentation
Th e diagn osis of ocular ch em ical burn is t ypically based on h istor y of con t act w ith
alkalin e or acid agen t s. Th e sym ptom s u su ally in clude pain , ph otoph obia, bleph a-
rospasm , reduced vision , an d excessive tearing. If th e bu rn is m ild or m oderate,
th e conju n ct iva is hyperem ic. Focal conju n ct ival ch em osis, hyperem ia, or h em or-
rh ages can be presen t . Eyelid edem a an d first- to secon d-degree periocular skin
burn s can be seen . Corn eal fin dings m ay range from superficial pu n ctate kerat it is
(SPK) to focal epith elial defect s. In severe con dit ion s w h ite areas of conju n ct ival
an d lim bal isch em ia can be seen . Corn eal fin dings u sually con sist of tot al epith e-
lial loss, st rom al h azing, an d, in sam e cases, com plete opacificat ion . Oth er sign s
in clu de an terior ch am ber react ion an d secon d- or th ird-degree periocu lar bu rn s.
( Fig. 1.5A,B).
Fig. 1.5 (A) A moderate chem i-

cal injury with 6 hours of limbal
blanching, a large epithelial defect,
and strom al haze. (B) The sequelae
of a severe chemical injury demon-
strating a scarred and vascularized
cornea. This eye underwent a per-
m anent keratoprosthesis. (Courtesy
of Christopher Rapuano)
Th erm al bu rn s, u lt raviolet (UV) kerat it is, ulcerat ive kerat it is
Management
Ch em ical bu rn s are con sidered a t ru e oph th alm ologic em ergen cy an d requ ire im -
m ediate care. Th e first p riorit y is im m ediate an d copious irrigat ion w ith sterile
irrigat ing solut ion or salin e solu t ion . If th ese solu t ion s are n ot available, t ap w ater
can be used. Irrigat ion sh ould be con t in u ed u n t il n eut ral pH is reach ed. In sert ion
of a lid specu lum an d topical an esth et ic p rior to irrigat ion facilit ates th e proce-
du re. After irrigat ion a good h istor y w ith an exact iden t ificat ion of th e ch em ical
agen t sh ou ld be obtain ed. Slit-lam p exam in at ion w ith flu orescein stain ing sh ould
be perform ed. Eyelids sh ould be everted to search for residual ch em icals an d for-
eign bodies. Th e goal of th erapy is to reduce pain , in flam m at ion , an d risk of in fec-
t ion . Th u s cyclop legic agen ts (avoid ph enyleph rin e becau se it is a vasocon st rictor),
oral an algesics (avoid repeated ap plicat ion s of topical an esth et ics becau se th ey can
delay epith elial h ealing), an d oph th alm ic an t ibiot ics (avoid am in oglycoside an t i-
biot ics because th ey im pair ep ith elial h ealing) sh ould be adm in istered. Th e u se
of topical steroids rem ain s con t roversial. Th ey can lim it in flam m at ion -m ediated
ocu lar dam age, bu t th ey ret ard w ou n d h ealing an d predispose to in fect ion . Severe
burn s can be m an aged w ith adju n ct ive th erapy: ocular hypoten sive m edicat ion s
if th e in t raocu lar pressure is elevated, collagen ase in h ibitors if any m elt ing of th e
1 Ocular Traum a 7
corn ea occu rs, lysis of conju n ct ival adh esion s if presen t , an d act ive surgical re-
m oval of n ecrot ic t issu e. Long-term com plicat ion s of ch em ical burn s in clu de per-
forat ion , scarring, corn eal n eovascularizat ion , sym bleph aron , glaucom a, cat aract s,
an d ret in al dam age. Ult im ate progn osis is related to th e degree of lim bus isch em ia,
th e depth of th e corn eal inju r y, an d th e presen ce of sym blep h aron .
Corneal Abrasion
Corneal abrasions represent one of the m ost com m on ophthalm ic problem s seen in
em ergency departm ents. A corneal abrasion is the disruption of the protective epi-
thelium covering the cornea; it m ay be caused by direct or tangential im pact. Com -
m on causes are scratches from fingernails, anim al paw s, tree branches, or a paper
cut . An oth er com m on cause is con t act len s over w ear. A large n u m ber of corn eal
abrasion s are preven table. High -risk w orkers (e.g., w oodw orkers, m etal w orkers)
an d players of cert ain spor ts (e.g., h ockey, racquetball, cross-coun t r y skiing, m oun -
tain biking) sh ould w ear ap propriate eye protect ion .
Presentation
Th e pat ien t’s h istor y t ypically in clu des eye t raum a an d subsequ en t acute pain .
Presen t ing sym ptom s u sually in clu de severe pain , excessive tearing, ph otoph obia,
foreign body sen sat ion , bleph arospasm , an d blu rred vision . At slit-lam p exam in a-
t ion diffuse corn eal edem a, ep ith elial disru pt ion , an d circu m corn eal inject ion can
be seen ( Fig. 1.6 ).
Acute angle glau com a, h erpes ulcers an d oth er corn eal u lcers, corn eal foreign
body, an d corn eal perforat ion
Management
Visual acuit y should be assessed because it m ay be significantly reduced if th e abra-
sion is on the optic axis. Upper and low er tarsal conjunctiva should be inspected
carefully for foreign bodies. If exam ination is lim ited by excessive pain, one drop of
topical anesthetic could be adm inistered for diagnostic purposes. At slit-lam p ex-
Fig. 1.6 Corneal defect stained

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

A corn eal foreign body is a com m on cau se of visits for oph th alm ic em ergen cies. It
frequ en tly occurs w h en on e is grin ding an d drilling steel w ith ou t w earing p rotec-
t ive goggles.
Presentation
Th e pat ien t’s h istor y u sually in clu des an ocu lar t rau m a. Th e m ore frequen t sym p-
tom s are m ild or m oderate pain , foreign body sen sat ion , excessive tearing, ph oto-
ph obia, an d blu rred vision . At slit-lam p exam in at ion on e or m ore object s can be
seen lodged superficially or em bedded w ith in th e corn ea. Metallic foreign bodies
m ay leave rust rings in th e su rrou n ding corn ea. Oth er sign s in clu de a circu m lim -
bal conju n ct ival inject ion , eyelid edem a, an d a sterile in filt rate su rroun ding th e
foreign body ( Fig. 1.7 ).
Fig. 1.7 Corneal foreign body.

1 Ocular Traum a 9
Corn eal abrasion , in t raocu lar foreign body, bacterial or fungal kerat it is
Management
After h aving assessed visual acuit y, it is im por tan t to ru le ou t a p ossible perfo-
rat ing inju r y. Th is can be don e using a Seidel test (in st ill flu orescein to in spect
for aqu eous leakage), m easu ring in t raocu lar pressu re, an d paying at ten t ion to an -
terior ch am ber react ion . Con sider a b-scan ult rasou n d an d an orbit al com pu ted
tom ograph ic scan to exclu de in t raocular an d in t raorbital foreign bodies. If th ere
is n o perforat ion , th e object can be rem oved u n der topical an esth esia (e.g., pro-
paracain e 0.5%) u sing a foreign body spud or a 25-gauge n eedle. Th is operat ion can
be facilit ated by sterile irrigat ion . Th e rust ring can be rem oved u sing an oph th al-
m ic drill. Th ese procedures sh ou ld be perform ed at slit lam p by w ell-t rain ed an d
experien ced physician s. Before an d after th e rem oval, an t ibiot ic drops sh ou ld be
applied u n t il h ealing. A top ical cycloplegic can be used to reduce ph otoph obia an d
pain . Pat ien t s sh ou ld be reevalu ated ever y 2 to 3 days u n t il th e w ou n d is h ealed
an d th e in filt rate resolved.
Corneal Laceration
Th e lacerat ion can be par t ial th ickn ess or fu ll th ickn ess.
Presentation
In part ial-th ickn ess lacerat ion , th e an terior ch am ber is n ot en tered, an d, th erefore,
th e corn ea is n ot perforated. If th e Seidel test is posit ive, a full-th ickn ess lacera-
t ion is p resen t . In full-th ickn ess lacerat ion th e pat ien t presen ts w ith tearing, pain ,
an d loss of vision . Associated fin dings in clude: sh allow an terior ch am ber, an terior
syn ech iae, corn eal opacit y w ith en doth elial dysfun ct ion , or cataract . In t raocular
pressure m ay be ver y low ( Fig. 1.8 ).
Management
A h istor y an d com plete oph th alm ic exam in at ion are required to ascer tain th e di-
agn osis. W h ile m an aging a part ial-th ickn ess lacerat ion , a cycloplegic (e.g., scopol-
Fig. 1.8 Penetrating corneal laceration with iris prolapse.

(Courtesy Pablo Gili M.D.)
am in e 0.25%) an d an an t ibiot ic (e.g., frequ en t polym yxin B/bacit racin oin t m en t

su ch as polysporin ) or flu oroquin olon e drops, depen ding on th e n at u re of th e
w ou n d, are st arted im m ediately.
W h en a m oderate to deep corn eal lacerat ion is accom pan ied by w oun d gape, it
is often best to su t u re th e w ou n d closed in th e operat ing room to avoid excessive
scarring an d corn eal irregu larit y, especially in th e visu al axis. Tet an us toxoid for
dir t y w ou n ds is a m ust .
Note th at sm all, self-sealing, or slow -leaking lacerat ion s m ay be t reated w ith
aqu eou s suppressan ts, ban dage soft con tact len ses, flu oroquin olon e drop s four
t im es a day. Altern at ively, a pressu re patch an d t w ice-daily an t ibiot ics m ay be
used. Avoid top ical steroids.
Traumatic Iritis
A blun t t raum a to th e eye can cau se t rau m at ic in flam m at ion of th e iris or, m ore
accu rately, of th e an terior uveal t ract . Th is leads to th e presen ce of in flam m ator y
cells in th e an terior ch am ber of th e eye. Traum at ic irit is gen erally develops qu ickly
after th e t rau m a an d u su ally affect s on ly th e inju red eye.
Presentation
Pat ien ts usu ally presen t w ith a h istor y of ocu lar t rau m a. Sym ptom s in clude pain ,
ph otoph obia, an d possibly h eadach e. Pain t ypically w orsen s w h en eith er th e
inju red eye or th e u n involved eye is exposed to brigh t ligh t (du e to con sen su al
pupillar y con st rict ion ). Sign s in clude cells an d flare in th e an terior ch am ber an d
perilim bar inject ion ( Fig. 1.9 ). Th e iris pu pillar y m argin of th e involved eye m ay be
differen t in sh ape com pared w ith th e con t ralateral.
Trau m at ic corn eal abrasion , t raum at ic m icrohyph em a, oth er causes of an terior
uveit is
Management
Post t raum at ic pain w ith out corn eal abrasion or ulcer sh ould suggest th e diagn osis
of t raum at ic irit is. Th is diagn osis can be con firm ed by th e presen ce of cells an d
Fig. 1.9 Post traumatic hyphema

and iritis. (Courtesy of Amar Agarwal, Dr.
Agarwal’s Eye Hospital, Chennai, India)
1 Ocular Traum a 11
flare in th e an terior ch am ber at slit-lam p exam in at ion . A com plete oph th alm ic
evalu at ion sh ou ld be perform ed, in cluding ton om et r y an d fu n dus exam in at ion .
Treat m en t t ypically con sists of cycloplegic agen t s. In refractor y cases an d if n o cor-
n eal epith elial defect is detected, a steroid drop could be given . Pat ien ts sh ou ld be
re-evalu ated w ith in a w eek; if irit is is resolved, m edicat ion can be discon t in u ed.
Iris Sphincter Tear

Blun t inju r y often causes tears in th e sph in cter p upillae of th e iris.
Presentation
Th e pat ien t m ay be asym ptom at ic or m ay h ave glare an d ph otoph obia. Th e tears in
th e pupillar y m argin can be visu alized on slit lam p exam in at ion (Fig. 1.10 )
Oth er causes of a dilated p upil (e.g., ph arm acological m ydriasis)
Management
A th orough ocu lar exam in at ion is don e to ru le ou t any oth er coexist ing dam age. It
m ay be left alon e un t reated. If cau sing sym ptom s, an d if cat aract ext ract ion is also
being plan n ed, on e m ay perform a pupilloplast y or use an iridia segm en t s.
Traumatic Cataract
Trau m at ic cataract can develop after various t ypes of in sult: blun t or perforat ing
t raum a, elect ric sh ock, in frared, UV, an d ion izing radiat ion . Blun t t rau m a is th e
m ost com m on cause, an d cou p an d con t recoup inju ries, along w ith equ atorial
expan sion , are th e path ophysiological m ech an ism respon sible for ocu lar dam age.
As regards len s inju r y, “coup” is th e cau se for Vossius ring (iris pigm en t rem ain s
im prin ted on th e an terior capsule), an d “con t recou p” is respon sible for th e sh ock
w aves th at m ay lead to an terior or posterior capsular ru pt u re an d su bsequ en t len s
opacificat ion . Th e equatorial st retch ing can disr upt th e zon ules an d capsu le. Th e
Fig. 1.10 Sphincter tear. (Courtesy of Amar Agarwal, Dr.

release of len s protein s du e to capsu le rupt u re can lead to ph acoan aphylact ic uve-
it is, ch aracterized by th e presen ce of polym orph on uclear leukocyte (eosin oph ils)
an d gian t cell in filt rat ion su rroun ding len s m aterials. Th e occlusion of th e t rabecu-
lar m esh w ork du e to len s protein s an d m acroph ages can lead to an acu te rise in
in t raocular p ressure. Glaucom a can also be secon dar y to relat ive pup illar y block
du e to posterior syn ech iae or len s sw elling (ph acom orph ic glaucom a).
Presentation
If no perforating traum a or traum a-related sym ptom atic iritis occurs, the patient
could w ait for days, weeks, or m onths before searching for m edical care. The patient
usually presents w ith a history of traum a and m ay com plain of decreased vision and
m onocular diplopia. At slit lam p exam ination, cataract associated w ith blunt traum a
usually appears as stellate or roset te-shaped “visual axis” opacification located in-
axis and involving the posterior capsule. Perforating traum a leads to cortical opaci-
fication at the site of injur y. This opacification usually rem ains localized, but if the
capsular tear is large enough, the entire lens m ay opacify. Hyphem a, signs of iritis,
and lens dislocation or sublu xation m ay be present (Fig. 1.11A,B).
Fig. 1.11 (A) Cataract and iridodialysis secondary to severe

penetrating trauma. (B) Post traum atic endophthalmitis.
1 Ocular Traum a 13
Sen ile cat aract , ectopia len t is, angle recession glaucom a, an d hyph em a
Management
Mech an ism of injur y an d past ocular h istor y sh ould be invest igated first . Th en a
rupt ured globe an d in t raocular foreign body sh ou ld be ru led ou t an d a com plete
oph th alm ic exam in at ion sh ou ld be perform ed. Type an d exten t of len s opacifi-
cat ion an d th e presen ce of ocu lar in flam m at ion , hyph em a, ph acodon esis, irido-
don esis, angle recession , len s sw elling, len s dislocat ion , or sublu xat ion sh ould be
docum en ted. Zon u lar disru pt ion m ay be detected gon ioscopically th rough a di-
lated pu pil. Posterior segm en t t rau m a-related p ath ology sh ould be invest igated
by fu n du scopy. If opacificat ion obst ruct s th e view of th e posterior segm en t , u l-
t rason ography m ay be h elpfu l. Medical t reat m en t sh ou ld be directed to focal an d
off-a xis opacit ies, in flam m at ion , an d in t raocular pressu re rise. Miot ics can h elp
to obt ain a clear visual axis, in flam m at ion can be con t rolled w ith cor t icosteroids,
in creased in t raocu lar pressu re can be t reated w ith st an dard ocu lar hypoten sive
m edicat ion s. How ever, su rgical rem oval of th e len s u sually resolves th ese com pli-
cat ion s. Decreased visual acu it y, len s-in duced in flam m at ion or glau com a, capsular
rupt ure w ith len s sw elling, an d poor visualizat ion of posterior segm en t path ology
are in dicat ion s for su rger y. St an dard ph acoem u lsificat ion is preferred if th e len s
capsule is in tact an d th ere is su fficien t zon u lar su ppor t; in t racapsu lar ext ract ion
is in dicated for zon ular in st abilit y or an terior dislocat ion . In cases of posterior dis-
locat ion or posterior capsular rupt u re, pars plan a len sectom y an d vit rectom y m ay
be preferred. As regards len s im plan tat ion , capsu lar fixat ion is in dicated if zon u lar
su ppor t an d th e len s capsu le are in tact; capsu lar ten sion rings m ay h elp in cases
of lim ited zon ular dialysis. If th e posterior capsule is com prom ised but sufficien t
zon ular su ppor t rem ain s, su lcus fixat ion sh ould be ch osen . A sut u re fixat ion ap-
proach w ou ld be th e best if both zon u lar an d capsu lar su ppor t are in adequ ate.
If n o posterior supp or t is m ain t ain ed, an terior ch am ber posit ion ing sh ou ld be
con sidered. Com plicat ion s associated w ith t raum at ic cat aract in clude glau com a
(pup illar y block glau com a, p h acolyt ic glaucom a, ph acom orph ic glau com a, angle
recession glaucom a), ph acoan aphylact ic uveit is, hyph em a, ret in al det ach m en t ,
ch oroidal ru pt u re, t raum at ic opt ic n eu ropathy, an d globe rupt u re.
Lens Dislocation/Subluxation
Su blu xat ion is par t ial disrupt ion of th e zon u lar fibers; th e len s is decen tered bu t
rem ain s par t ially in th e pu pillar y apert u re. Dislocat ion is com plete disru pt ion
of th e zon ular fibers; th e len s is displaced ou t of th e pu pillar y apert u re. Trau m a
(m ost com m on cau se), Marfan syn drom e, h om ocyst in u ria, Weill–March esan i syn -
drom e, acqu ired syph ilis, congen it al ectop ia len t is, an iridia, Eh lers–Dan los syn -
drom e, Crou zon disease, hyperlysin em ia, sulfite oxidase deficien cy, h igh m yopia,
ch ron ic in flam m at ion s, an d hyperm at ure cat aract are som e of th e cau ses of len s
su blu xat ion .
Presentation
Decreased vision an d dou ble vision th at persist w h en covering on e eye (m on ocu lar
diplopia) are th e m ain sym ptom s. Crit ical sign s are decen tered or displaced len s,
iridodon esis (qu ivering of th e iris), an d p h acodon esis (qu ivering of th e len s). Oth er
sign s in clu de m arked ast igm at ism , cataract , angle closure glaucom a as a resu lt
of pupillar y block, acqu ired h igh m yopia, vit reou s in th e an terior ch am ber, an d
asym m et r y of th e an terior ch am ber depth ( Fig. 1.12 )
Fig. 1.12 Lens subluxation and zonular disruption.
Management
Fam ily, person al, m edical, an d t rau m a h istor y is ver y im port an t . System ic exam i-
n at ion sh ould evalu ate st at u re, ext rem it ies, h an ds, an d fingers as n ecessar y. Rapid
plasm a reagin test an d fluorescen t t rep on em al an t ibody absorpt ion test , sodiu m
n it ropru sside test , ech ocardiography, an d urin e ch rom atography to rule out h o-
m ocyst in uria are n eeded.
Lens dislocated into the vit reous: Surgically rem ove th e len s.
Lens capsule intact, pat ient asym ptom at ic no signs of inflam m at ion : Obser ve.
Lens capsule broken eye inflam ed : Len sectom y is don e eith er th rough th e pars
plan a or by using a lim bal approach
Sublu xat ion
Asym ptom at ic: Obser ve.
High uncorrectable ast igm at ism or m onocular diplopia : Su rgical rem oval of
th e len s .
Sym ptom at ic cataract : Opt ion s in clu de surgical rem oval of th e len s.
Pupillary block : Treat m en t is iden t ical to th at for aph akic pup illar y block .
Marfan syndrom e is present : Refer th e pat ien t to a cardiologist for an an n u al
ech ocardiogram an d m an agem en t of any cardiac-related abn orm alit ies. Pro-
phylact ic system ic an t ibiot ics are required if th e p at ien t un dergoes su rger y
(or a den tal procedu re) to preven t en docardit is.
Hom ocyst inuria is present :
Adm in ister pyridoxin e, 50 to 1000 m g by m outh fou r t im es a day.
Redu ce diet ar y m eth ion in e.
Avoid su rger y if possible becau se of th e risk of th rom boem bolic com pli-
cat ion s. If surgical in ter ven t ion is n ecessar y, an t icoagu lan t th erapy is in -
dicated.
Microhyphema/ Hyphema
Trau m at ic hyph em a is defin ed by post inju r y accu m ulat ion of blood w ith in th e an -
terior ch am ber. Microhyph em a con sist s of su spen ded er yth rocytes in th e an terior
ch am ber, gen erally visible at slit lam p. Equatorial expan sion after blu n t t raum a
in duces st ress to angle st ru ct u res, w h ich can lead to ru pt u re of iris an d ciliar y body
1 Ocular Traum a 15
vessels w ith su bsequen t h em orrh age. Lacerat ing injur y can be associated w ith di-
rect dam age of blood vessels an d hypotony. Som e con dit ion s such as ru beosis iri-
dis, juven ile xan th ogran u lom a, h em oph ilia, leu kem ia, an d th e use of dr ugs th at al-
ter platelet or th rom bin fu n ct ion m ay facilit ate th e on set of hyph em a. A sign ifican t
n um ber of sigh t-th reaten ing com plicat ion s m ay develop, w h ich requires careful
follow -u p for hyph em a pat ien ts.
Presentation
Pat ien ts usu ally presen t w ith a h istor y of blun t t rau m a. Pain an d blu rred vision
are com m on sym ptom s. Hyph em as are graded on th e am ou n t of blood w ith in th e
an terior ch am ber: grade I is less th an on e th ird filling of th e an terior ch am ber,
grade II hyph em as h ave m ore th an on e th ird but less th an on e h alf of th e an terior
ch am ber filled w ith blood, grade III is m ore th an on e h alf bu t less th an total filling,
grade IV is a total hyph em a, also kn ow n as eigh t-ball hyph em a ( Fig. 1.13 ).
Uveit ic glau com a an d cau ses of spon tan eou s hyph em a such as juven ile xan th o-
gran ulom a, iris cavern ous h em angiom a, hyper ten sion , an d bleeding disorders
Management
Mech an ism an d t im e of inju r y sh ou ld be invest igated carefully. A h istor y of sickle
cell t rait or disease sh ou ld be sough t ou t . In spect ion for gross ocu lar injur y an d eval-
uat ion of th e adn exa sh ou ld be perform ed. A ru pt u red globe sh ould be ruled ou t . A
com plete ocu lar exam in at ion is im perat ive an d m ust in clu de in t raocu lar pressure
m easu rem en t an d dilated fun du scopic evalu at ion . Gon ioscopy sh ould be deferred
un t il hyph em a resolves to detect poten t ial rebleeding sites an d angle recession .
A draw ing of th e hyph em a docum en t ing sh ape an d size sh ou ld be recorded at
ever y oph th alm ic evalu at ion . Depen ding on th e pat ien t’s h istor y, h em oglobin opa-
th ies an d bleeding disorders sh ou ld be invest igated. B-scan u lt rason ography m ay
be u sefu l in pat ien t s w ith large hyph em as, w h en oph th alm oscopy is n ot feasible.
Non com plian t pat ien t s or th ose w ith in creased risk of rebleeding, u n con t rolled
Fig. 1.13 Post traum atic hyphema. Grade II hyphemas have

more than one third but less than one half of the anterior
chamber filled with blood.
glaucom a, posit ive sickle cell t rait , or an em ia sh ou ld be con sidered for inpat ien t
h ospitalizat ion . Th e elevat ion of th e pat ien t’s h ead to 30 to 45 degrees w h ile
lying su pin e m ay facilit ate th e set tling an d layering of th e hyp h em a in th e in fe-
rior an terior ch am ber, allow ing an easier classificat ion of th e hyph em a, an earlier
evalu at ion of th e posterior pole, an d a m ore rap id im provem en t in visual acu it y. A
t ran sparen t plast ic sh ield sh ould be u sed to protect th e involved eye from fur th er
inju r y. It s t ran sparen cy allow s recogn izing rebleeding or su dden visu al loss.
Medical t reatm ent in cludes topical cycloplegics (1 drop of 1% at ropin e three
tim es a day for up to 5 days) to in crease the pat ient’s com fort (con sider th e risk of
precipitating acute glaucom a in pat ien ts w ith a n arrow ch am ber angle) and topical
steroids (0.1%dexam eth ason e) to decrease inflam m at ion, reduce anterior ch am ber
reaction , an d prevent the incidence of secon dar y hem orrh age (caution sh ould be
exerted if steroids are used for a prolonged period because th ey can in crease the
risk of cataract an d glaucom a). Topical and system ic an tifibrin olytics, such as am i-
nocaproic acid, could be used to prevent rebleeding an d retarding clot lysis. Th e
m ore com m on side effects of am in ocaproic acid in clude vom iting, diarrhea, and
post ural hypoten sion . Its system ic use sh ould be avoided in patien ts w ith hepatic
or ren al disease. Persistent in creased in traocular pressure should be treated in i-
tially w ith topical -blockers. If th is treatm en t is unsuccessful, topical -agon ist or
carbonic an hydrase in hibitor m ay be added in patients w ith out sickle cell t rait or
disease. Aspirin an d other NSAIDS should be discont in ued. Un con trolled elevated
int raocular pressure (at least 45 m m Hg for 5 days) could be surgically t reated w ith
paracentesis and an terior ch am ber w ashout . Other indicat ions to surger y are early
corneal staining or rebleeding hyph em as. Sm aller hyphem as are usually self-lim it-
ing an d clear w ith in 5 days. Large hyphem as are associated w ith com plication s an d
th e w orst progn osis. Such com plicat ions are secon dar y h em orrhage, corneal blood
stain ing, glaucom a, an terior an d posterior syn ech iae, cataract, an d opt ic atrophy.
Ruptured Globe
A rupt ured globe is a devastating injur y w ith significant long-term con sequen ces
for the pat ien t. It represen ts a discont in uit y of the eye’s outer m em branes caused
by blunt or pen et rating t raum a. Rupt ures resulting from blunt traum a usually occur
at th e sites w here the sclera is w eakest, such as at th e in sertion of th e extraocular
m uscles, around the opt ic ner ve, an d at the lim bus. Sh arp objects w ith sufficient
m om en t um m ay directly perforate th e globe. Globe rupt ure is m ore com m on in
young m ales ow ing to their occupation al and recreation al preferen ces. High m yopia
and previous eye surger y can m ake t issues m ore vulnerable to rupture. A rupt ured
globe is an ophth alm ic em ergen cy and requires surgical repair as soon as possible.
Th e visual outcom e depends largely on early recogn ition and prom pt in ter vent ion.
Presentation
Th e pat ien t usu ally p resen t s w ith a h istor y of ocu lar t rau m a. Sym ptom s in clude
pain , w h ich can be n ot ext rem ely severe in th e case of sh arp injur y, an d decreased
vision . Diplopia m ay be presen t du e to ext raocu lar m u scle en t rapm en t or dysfun c-
t ion an d t raum a-associated cran ial n er ve palsy. At physical exam in at ion th e globe
rupt ure m ay be obviou s or occu lt . A fu ll-th ickn ess corn eal or scleral lacerat ion is a
sign of globe perforat ion . Prolapse of th e iris or ext rusion of ocular con ten t s m ay be
presen t . Severe conju n ct ival h em orrh age, usually involving 360 degrees of bu lbar
conju n ct iva, t ypically in dicates globe ru pt ure. Oth er accom panying sign s in clu de
irregu lar pupil, hyph em a, len s injur y, com m ot io ret in ae, vit reou s h em orrh age,
ch oroidal rupt ure, ret in al tears an d detach m en ts, an d t raum at ic opt ic n eu ropathy.
A ru pt u red globe m ay presen t w ith both en oph th alm os an d exoph th alm os, de-
pen ding on th e presen ce of an associated ret robu lbar h em orrh age ( Fig. 1.14 ).
1 Ocular Traum a 17
Fig. 1.14 Perforating ocular trauma.
Management
Th e m ech an ism an d th e circu m st an ces of injur y an d th e n at ure of th e t raum at iz-
ing object sh ou ld be invest igated. Visu al acuit y sh ould be docu m en ted an d ext ra-
ocu lar m u scle fu n ct ion sh ou ld be evaluated. Pup ils sh ould be exam in ed for size,
sh ape, an d ligh t reflex. Th e diagn osis of a rupt u red globe sh ould be m ade by slit
lam p or pen ligh t . Th e orbit an d adn exa sh ou ld be exam in ed for inju ries, foreign
bodies, bon e deform it y, an d eyeball displacem en t . In t raocu lar pressu re m easu re-
m en t is con t rain dicated to avoid pressure to th e globe. Th e eye sh ou ld be p rotected
w ith a sh ield. System ic prophylact ic an t ibiot ics an d an algesics, if advisable, sh ou ld
be adm in istered. Th e pat ien t sh ou ld receive tet an u s im m un izat ion if in dicated an d
be kept n oth ing per os. Th e im aging st u dy of ch oice is com pu ted tom ography; if it
is n ot available a p lain x-ray film sh ou ld be obt ain ed. Magn et ic reson an ce im aging
m ay be usefu l to iden t ify soft t issue an d globe inju ries, bu t it is con t rain dicated if
a m et allic foreign body is suspected. Careful B-scan ult rason ography m ay be u sefu l
to iden t ify th e site of ru pt u re an d in t raocular foreign bodies. Surgical repair sh ou ld
be prom pt . If th ere is n o expect at ion to restore vision , en ucleat ion sh ou ld be con -
sidered. En dop h th alm it is an d sym path et ic oph th alm ia are possible sigh t-t reat ing
com plicat ion s th at sh ould be born e in m in d.
Posterior Segment Trauma
Posttraumatic Vitreous Hemorrhage

Vit reous h em orrh age resu lt s from bleeding in to on e of th e several poten t ial spaces
form ed aroun d an d w ith in th e vit reou s body. Th is con dit ion can follow injuries to
th e ret in a an d uveal t ract an d th eir associated vascu lar st ru ct u res. Neovascu lariza-
t ion occu rring in diseases like proliferat ive diabet ic ret in opathy m ay predispose to
bleeding, even if th e t raum a is m ild. Oth er disorders th at p rom ote th e release of
angiogen ic vasoact ive factors an d subsequ en t form at ion of n eovascu lar an d fragile
vessels th at can easily bleed are isch em ic ret in op athy secon dar y to ret in al vein oc-
clusion , ret in opathy of prem at u rit y, an d proliferat ive sickle cell ret in opathy. Trau -
m at ic vit reou s h em orrh age in ch ildren m ay be a sign of ch ild abu se (sh aken baby
syn drom e).
Presentation
Pat ien ts w ith t raum at ic vit reou s h em orrh age usually presen t w ith a com plain t of
decreased visu al acu it y, floaters, cloudy vision , percept ion of sh adow s, visual h aze,
an d ph otoph obia. Pat ien ts m ay n ot rem em ber th e t raum at ic in su lt . Direct oph th al-
m oscopy reveals a dim in ish ed red reflex th at can be black in severe cases. In direct
oph th alm oscopic exam in at ion discloses th e presen ce of blood in th e an terohyaloid
or ret rohyaloid spaces or w ith in th e vit reou s gel. Usu ally a subhyaloid h em orrh age
suggest s a source of bleeding an terior to th e ret in a, w h ereas a h em orrh age pos-
terior to th e in tern al lim it ing m em bran e im plies a source of bleeding w ith in th e
ret in a. Long-stan ding h em orrh ages can evolve in w h ite m asses ( Fig. 1.15 ).
Differen t ial diagn osis of t raum at ic vit reous h em orrh age in clu des oth er vit reous
h em orrh ages n ot related to t rau m a. Spon t an eou s vit reou s h em orrh age m ay occu r
in con dit ion s like proliferat ive ret in opath ies, ch oroidal or ciliar y body m elan om a,
ret in oblastom a, uveit is, sarcoidosis, ocu lar m an ifestat ion of syph ilis, or h istoplas-
m osis.
Management
A detailed h istor y is ver y im port an t . Un derlying path ologies an d m ech an ism of
t raum a sh ou ld be docu m en ted. A com plete eye exam in at ion sh ould be perform ed,
in clu ding slit lam p exam in at ion , in t raocu lar p ressure m easu rem en t , an d dilated
fun du s evalu at ion . Globe perforat ion an d in t raocu lar foreign body sh ou ld be ruled
out . B-scan ult rason ography can be u sed w h en th e fu n dus is difficu lt to visu al-
ize, disclosing th e presen ce of ret in al det ach m en t , ret in al tears, in t raocu lar for-
eign body, or in t raocular t um or. In it ial th erapy con sist s of bed rest w ith 30- to
45-degree h ead elevat ion (allow s th e blood to set tle in feriorly) an d avoidan ce of
an t icoagu lat ive drugs an d in ten se Valsalva m an euvers. Con clu sive th erapy is fired
at th e u n derlying cau se: ret in al breaks can be closed w ith laser ph otocoagulat ion ,
an d surger y can resolve ret in al det ach m en t s. Vit rectom y is also in dicated in long-
stan ding vit reou s h em orrh age ( 2 to 3 m on th s) an d w h en vit reous h em orrh age is
associated w ith ru beosis an d gh ost-cell glaucom a. Com plicat ion s of vit reou s h em -
Fig. 1.15 Post traum atic vitreous

hemorrhage.
1 Ocular Traum a 19
orrh age usu ally develop w h en large am oun ts of blood rem ain for long periods in
th e vit reou s cavit y an d in clude en h an ced proliferat ive ret in opathy, h em osiderosis
bulbi an d con sequen t iron toxicit y, gh ost-cell glaucom a, am blyop ia (resu lt ing from
visu al deprivat ion ), an d m yopic sh ift in in fan t s.
Commotio Retinae
Com m ot io ret in ae is a clin ical en t it y first described in 1873 by Berlin an d is ch ar-
acterized by a t ran sien t w h iten ing at th e deep sen sor y ret in a. Th is con dit ion is
com m on ; it h as been sh ow n to be resp on sible for 9.4%of all p ost t raum at ic fu n dus
ch anges. Th e m ech an ism of inju r y is th e con t recoup force follow ing blun t ocu lar
t raum a th at causes degen erat ion of th e ph otoreceptors’ ou ter segm en t s an d sub-
sequ en t ph agocytosis by ret in al p igm en t epith eliu m cells. Th e presen ce of edem a
in th e ou ter plexiform layers, n uclear layers, an d su bret in al space h as been dem on -
st rated. Angiograph ic eviden ce h as sup por ted th e belief th at ret in al an d ch oroidal
vessels do n ot play a sign ifican t role in th e path ogen esis of th is con dit ion .
Presentation
Pat ien ts m ay be asym ptom at ic if com m ot io ret in ae is lim ited to th e periph eral
ret in a, or th ey m ay com plain of decreased vision if th e w h iten ing occurs in th e
foveal region . Visu al acuit y m ay be variably affected an d does n ot alw ays relate
to th e degree of opacificat ion . Oph th alm oscopic exam in at ion reveals a cloudy
opacificat ion of th e ret in a, u su ally w ith poorly defin ed m argin s. It can be located
anyw h ere w ith in th e posterior segm en t . In som e cases th e en t ire posterior pole
can be involved, an d it m ay appear as a pseudoch err y red spot . Ret in al vessels are
clearly visible an d ap pear un dist u rbed. Oth er associated t raum at ic path ology m ay
be presen t , such as su bret in al, in t raret in al, an d preret in al h em orrh ages; m acu lar
h oles; m acu lar detach m en ts, an d ch oroidal ru pt ures ( Fig. 1.16 ).
Differen tial diagnosis of com m otio ret in ae in cludes retinal detach m ent , cen tral ar-
ter y occlusion, bran ch ret in al arter y occlusion , and ret in al w h ite w ith out pressure.
Fig. 1.16 Peripheral comm otio

retinae with undefined posterior
borders.
Management
Th e m ech an ism of t raum a sh ou ld be docu m en ted. A com plete op h th alm ic exam i-
n at ion sh ould be perform ed, in clu ding dilated fu n dus evaluat ion an d scleral de-
pression if th ere is n o eviden ce of hyph em a, m icrohyph em a, or irit is. Th e ret in al
w h iten ing u su ally fades w ith in som e w eeks, an d n o t reat m en t is available, on ly
obser vat ion . Abou t 60% of pat ien ts fu lly recover vision , an d 40% su stain perm a-
n en t visu al loss. Com plicat ion s of com m ot io ret in ae in clu de cystoid areas th at m ay
degen erate in to m acular h oles, ph otoreceptor loss, ret in al p igm en t epith elium
(RPE) m igrat ion , degen erat ion , at rop hy, or hyperplasia.
Choroidal Rupture
Traum atic choroidal rupt ure is a com m on occurrence after a blunt ocular traum a
(5 to 10%). It is a defect in th e Bru ch m em bran e, th e ch oroid, an d th e ret in al pig-
m en t epith eliu m . W h en su dden an teroposterior com pression an d equ atorial
expan sion subsequ en t to ocu lar blu n t t raum a t ake p lace, th e sclera h as en ough
ten sile st rength an d th e ret in a h as en ough elast icit y to be relat ively protected. Be-
cau se th e Bru ch m em bran e does n ot h ave th ese proper t ies, it is pron e to break .
Th e dam age at th e ch oriocapillaris vessels m ay lead to su bret in al, sub-ret in al p ig-
m en t epith eliu m , or in t rach oroidal h em orrh age. In th e acute ph ase th e overlying
h em orrh age an d th e ret in al edem a m ay obscure th e ch oroidal ru pt ure itself. Typi-
cally, during th e h ealing p h ase, ch oroidal n eovascu larizat ion occu rs an d in m ost
cases resolves spon t an eou sly. Con dit ion s associated w ith an in creased fragilit y of
th e Bru ch m em bran e, su ch as angioid st reaks, are risk factors for t rau m at ic ch o-
roidal ru pt u re.
Presentation
Pat ien ts u su ally p resen t w ith a h istor y of ocu lar blu n t t raum a, decreased vision ,
an d a variet y of visu al field defects (paracen t ral, cen t ral, sector scotom as). At oph -
th alm oscopic exam in at ion th e ch oroidal lesion appears as a yellow -w h ite, cres-
cen t-sh aped, su bret in al st reak, con cen t ric to th e opt ic disc. Th e border of th e rup-
t u re m ay be hyperpigm en ted or hypopigm en ted. Often th e overlying h em orrh age
m ay obscu re th e ch oroidal ru pt u re ( Fig. 1.17A,B).
A B
Fig. 1.17 (A) Traumatic choroidal rupture. (B) Traumatic choroidal rupture.
1 Ocular Traum a 21
Angioid st reaks, h igh m yopia, subret in al n eovascu lar m em bran es, ocular h isto-
plasm osis syn drom e, ch oroidal n eovascularizat ion , pseudoxan th om a elast icum
Management
A com p lete ocular exam in at ion is m an dator y. Fluorescein angiography m ay be
con sidered to con firm th e presen ce of ch oroidal ru pt ure an d to detect ch oroidal
n eovascularizat ion . In docyan in e green angiography m ay be u sefu l w h en su bret i-
n al h em orrh age obscures ch oroidal n eovascu larizat ion recogn it ion . Con ser vat ive
t reat m en t is advised for m ost t raum at ic ch oroidal rupt u res. Ext rafoveal ch oroidal
n eovascularizat ion m ay be t reated w ith laser ph otocoagu lat ion . Pars plan a vit rec-
tom y an d m em bran e ext ract ion m ay be con sidered for subfoveal an d ju xtafoveal
ch oroidal n eovascu larizat ion . Good visu al ou tcom es are expected if th e rupt u re
does n ot involve th e fovea. Possible com p licat ion s are h em orrh agic or serou s m ac-
ular detach m en t .
Posttraumatic Retinal Tears and Detachment

Ocu lar t rau m a is respon sible for 10% of ret in al det ach m en t s. Usu ally th e t rau -
m at ic inju r y causes an an terior-posterior com pression of th e globe an d a lateral
expan sion of th e equator. Th is resu lts in a t ract ion al force on th e vit reou s base,
w h ere th e vit reous body is physiologically adh eren t to th e p eriph eral ret in a. Ret i-
n al breaks are th e resu lt of vit reou s t ract ion at th e ora serrat a or in sites of fo-
cal vit reoret in al adh esion (such as corioret in al scars an d lat t ice degen erat ion ). In
th e presen ce of vit reou s syn eresis, fluid dissect s th e ret in a, giving rise to ret in al
detach m en t . Com m on abn orm alit ies cau sing post t raum at ic ret in al det ach m en t s
are ret in al dialysis an d gian t ret in al tears. An oth er m ech an ism of inju r y is ret in al
n ecrosis as a result of direct t raum a to th e sclera. It is often associated w ith ret in al
h em orrh ages an d edem a an d leads to large an d irregularly sh aped ret in al tears.
High m yopia an d sites of focal vit reoret in al adh eren ce are risk factors for t raum at ic
ret in al detach m en t .
Presentation
Ret in al detach m en ts an d ret in al tears can be diagn osed m on th s or years after th e
traum a, so th e causal n exu s is n ot alw ays easy to iden t ify. Pat ien t s can presen t
com plain ing of m ild blu rring of vision , floaters, ph otopsia, an d visual-field defects.
Oph th alm oscopic fin dings th at suggest a vitreoret in al in terface involvem en t after
a t raum a in clude vit reous base avulsion , retin al dialysis, ret in al tears of various
sh apes an d dim en sion s (gian t, roun d, h orsesh oe), an d ret in al detach m en t . On ce
th e ret in a becom es detach ed, it appears as an elevated, sligh tly opaqu e, corrugated
surface th at un du lates freely w ith eye m ovem en ts. In th e cases of retin al detach -
m en t in t raocular pressu re is u sually low er th an th at of th e fellow eye (Fig. 1.18 ).
Pen et rat ing t raum a, ret in al det ach m en t s cau sed by oth er con dit ion s (proliferat ive,
t ract ion al, postoperat ive, exu dat ive), acute ret in al n ecrosis, sen ile ret in osch isis
Management
A com plete oph th alm ic evaluation should be perform ed, in cluding int raocular pres-
sure m easurem en t an d accurate retinal exam inat ion. Retinal abnorm alities, vitreo-
Fig. 1.18 Retinal detachment sec-

ondary to a retinal dialysis.
retin al tractions, tears, an d detachm en ts m ust be recorded. B-scan ultrason ography

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

A m acular h ole is a full-th ickn ess defect of th e ret in a involving th e foveal region .
Trau m at ic m acu lar h ole w as first described in 1869 by Kn ap p. Sin ce th en a large
n um ber of cases h ave been reported an d, despite several publicat ion s, th e exact
m ech an ism of t rau m at ic m acular h ole form at ion rem ain s con t roversial. Som e
th eories h ave been proposed to explain developm en t of t rau m at ic m acu lar h oles:
h istorical hypoth eses claim ed t raum at ic, cyst ic degen erat ion , an d vit reous an d
vascu lar et iologies. In m ore recen t t im es, Joh n son et al advan ced th at equ atorial
expan sion cau ses ret in al flat ten ing an d tangen t ial t ract ion . Yam ada et al obser ved
th at vit reous t ract ion m ay play a role in th e form at ion of som e t rau m at ic m acular
h oles. Torn am be proposed th e experim en tal hydrat ion th eor y, st at ing th at th e al-
tered h om eostasis du e to a break in th e in tern al ret in al layer leads to in t raret in al
sw elling an d h ole form at ion . Th e in ciden ce of t rau m at ic m acular h oles varies from
1 to 9%. Pat ien ts are u su ally young an d m ale. Most t rau m at ic m acu lar h oles derive
from closed-globe con t u sion inju ries from variou s in sult s, th e m ost com m on being
blun t ocular t rau m a caused by a variet y of t ypes of balls . Trau m at ic m acu lar h oles
can also be cau sed by acciden t al yt t rium -alu m in u m -garn et (i.e., YAG) laser burn s.
Presentation
Pat ien ts u sually presen t w ith a h istor y of ocu lar t rau m a an d subsequ en t reduc-
t ion of cen t ral visual acuit y, w h ich is u su ally 20/80 to 20/400. Oph th alm oscopic
1 Ocular Traum a 23
exam in at ion n orm ally discloses a fu ll-th ickn ess an d w ell-defin ed h ole in th e cen -
ter of th e m acu la. It is usually roun d or ellipt ical an d m easures 300 to 500 m .
Oth er com m on fin dings are th e presen ce of sm all yellow deposits at th e level of
th e ret in al pigm en t epith eliu m (RPE) an d a ring of subret in al flu id su rroun ding
th e h ole. Associated epiret in al m em bran e an d operculu m are t ypically m issing.
Er yth rocytes an d in flam m ator y cells m ay be presen t in th e vit reous, an d associ-
ated ocu lar inju ries are com m on ( Fig. 1.19 ).
Idiopath ic m acu lar h ole, epiret in al m em bran e
Management
A com plete oph th alm ic exam in at ion sh ou ld be perform ed, in cluding in t raocu lar
pressure m easurem en t an d carefu l posterior segm en t evalu at ion . Useful im ag-
ing st u dies in clu de flu orescein angiography, opt ical coh eren ce tom ography, an d
B-scan u lt rason ography. Microperim et r y m ay docum en t th e pat tern of visual acu -
it y loss. Vit rectom y h as been sh ow n to close t raum at ic m acular h oles effect ively
an d im p rove vision . Curren t tech n ique in cludes rem oval of th e posterior hyaloid
an d all epiret in al m em bran es from th e m acu lar area an d prolonged postopera-
t ive m acular gas tam pon ade. Spon tan eous closure of t rau m at ic m acular h oles is
relat ively frequen t . Th erefore, a period of obser vat ion before deciding on su rgical
in ter ven t ion is recom m en ded. Associated m acular RPE at rophy an d ch oroidal in -
jur y m ay lim it visual outcom es.
Intraocular Foreign Body

Th e oph th alm ic pathologies caused by an in traocular foreign body arise from t w o
m ech anism s: th e direct dam age caused by the penet rating injur y an d its associated
com plicat ion s, depen ding on th e size, sh ap e, an d m om en t um of th e object; an d
th e dam age caused by th e existen ce of an in t raocular foreign body, su ch as m etal
toxicit y an d m icrobial en doph th alm it is. Met allosis bu lbi is an exten sive ocular
dam age cau sed by th e ch ron ic presen ce of a react ive m et allic foreign body, m ost
com m on ly m ade of iron or copper. Siderosis is ch aracterized by a ru st y brow n
deposit an d discolorat ion involving th e len s an d th e iris, an d ret in al degen erat ive
Fig. 1.19 Traumatic macular hole.

pigm en t ar y ch anges. Ch alcosis is m ade dist in ct ive by th e presen ce of a green ish

blue ring in th e periph eral corn ea (Kayser-Fleisch er ring), green ish colorat ion of
th e iris, an terior subcapsular cat aract , an d refract ive deposit s on th e surface of
th e ret in a. Com m on ly, in t raocu lar foreign bodies arise from h am m ering an d u sing
pow er tools. Protect ive eyew ear can preven t m ost inju ries.
Presentation
Pat ien ts usu ally presen t w ith a suggest ive h istor y, but oph th alm ologist s sh ould
take in to accou n t th at a pat ien t m ay be u n aw are of any object pen et rat ing th e
eye. Pat ien ts m ay be asym ptom at ic or com plain of decreased vision an d eye p ain .
Th e foreign body m ay be visible at slit-lam p exam in at ion of th e an terior segm en t;
oth er sign s in clude corn eal en t r y w ou n d, iris t ran sillu m in at ion defect , irregular
pupil, len s dam age, an d an terior ch am ber react ion . Dilated in direct oph th alm os-
copy m ay reveal a posterior segm en t foreign body an d associated inju ries, su ch as
vit reous h em orrh age, ret in al tears, an d detach m en t ( Fig. 1.20 ).
Oth er causes of sudden visu al loss
Management
Histor y sh ou ld be carefu lly invest igated, in clu ding m ech an ism of inju r y an d for-
eign body com p osit ion . Ocular exam in at ion sh ould be perform ed, w ith at ten t ion
to possible sites of ocu lar perforat ion . Th e an terior ch am ber an d posterior seg-
m en t sh ou ld be evalu ated carefu lly. Th e direct visualizat ion of th e foreign body
is u su ally ver y in form at ive for th e surgeon . Com pu ted tom ography is th e im aging
st udy of ch oice; if it is u n available a plain x-ray m ay be con sidered in th e case of a
m etallic foreign body. A carefu l u se of B-scan ult rason ography m ay be conven ien t
to localize th e foreign body even if th e globe is open . If a ch ron ic in t raocu lar for-
eign body is fou n d, elect roret in ography is a u seful for evaluat ing ret in al fu n ct ion
in th e m et allosis bu lbi. Top ical an d system ic an t ibiot ic th erapy, topical steroids,
an d tet an us prophylaxis (if n eeded) are requ ired p rior to th e surgical in ter ven -
t ion . Th e t im ing of su rger y depen ds on th e n at u re an d locat ion of th e foreign body
Fig. 1.20 Intraocular foreign body.

1 Ocular Traum a 25
an d on th e risk of en doph th alm it is. Foreign bodies in th e an terior ch am ber sh ou ld

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

Trau m a-associated lesion of th e opt ic n er ve can occur anyw h ere in th e course of
th e n er ve. Th e injur y can be du e to lacerat ion of th e n er ve by a foreign body or a
bon e fragm en t , com pression of th e n er ve, an d h em orrh age or perin eural edem a. It
is u sually associated w ith h ead t rau m a or m idfacial fract ure. Opt ic n er ve t raum a
is often du e to veh icle acciden ts, falls, recreat ion al spor ts, assau lt s, or pen et rat ing
orbit al t rau m a. Th e frequ en cy of opt ic n er ve injur y in th e Un ited States occu rring
in closed h ead t raum a varies from 0.5 to 5.0% ( Fig. 1.21 )
Presentation
Typically, pat ien ts presen t w ith a h istor y of h ead injur y an d repor t a classic se-
qu en ce of even ts: th e pat ien t recovers con sciou sn ess after h ead inju r y an d experi-
en ces a post t rau m at ic loss of visu al fun ct ion in on e eye. Visual acu it y an d color
vision m ay be altered, an d visual field defects m ay be p resen t . Th e crit ical sign is a
n ew ipsilateral afferen t pupillar y defect . Opt ic at rophy u su ally occu rs w eeks after
ret robu lbar t rau m a. Inju ries to th e opt ic n er ve m ay be eith er direct or in direct .
Direct inju ries in clude th e follow ing:
Fig. 1.21 Traumatic optic neuropathy. (Courtesy of Athi-

ya Agarwal, Dr. Agarwal’s Eye Hospital, Chennai, India)
Opt ic nerve avulsion : It u sually follow s severe orbit al t rau m a w ith an acute an d
seriou s visu al loss. Oph th alm oscopy sh ow s th e absen ce of th e opt ic disc an d
peripapillar y h em orrh age.
Opt ic nerve t ransect ion : Th e vision loss is im m ediate an d com plete, an d com -
puted tom ograph ic scan n ing reveals th e bon e fragm en t or th e foreign body
t ran sect ing th e opt ic n er ve.
Opt ic nerve sheath hem orrhage : Visu al fu n ct ion abn orm alit ies m ay var y an d
proptosis m ay n ot be presen t . Magn et ic reson an ce im aging m ay be h elpful in
con firm ing th e diagn osis. Th e visu al loss associated w ith th is con dit ion m ay be
reversible via sh eath fen est rat ion .
Orbital hem orrhage : It is associated w ith proptosis an d oph th alm oplegia. Raised
in t raocular pressu re m ay be in it ially con t rolled w ith topical ocular hypoten sive
agen ts. If con ser vat ive m easu res fail, lateral can th otom y an d h em orrh age drain -
age sh ou ld be con sidered.
Orbital em physem a : Injuries to th e th in bon es lim it ing th e paran asal sin us m ay
produ ce a on e-w ay valve th at results in an air accum ulat ion in th e orbit w ith
subsequent com pression of th e opt ic n er ve, proptosis, and elevat ion in th e in t ra-
ocular pressure. Drainage of th e in traorbital air usually resolves th is con dition .
In direct opt ic n er ve inju r y usually resu lts from a blu n t t raum a to th e superior
orbit al rim or th e fron t al area. Th e com pression forces are th en t ran sm it ted via
orbit al bon es to th e orbital ap ex an d opt ic can al. Com pression an d con t u sion of
th e n er ve produce a com par t m en t syn drom e th at result s in localized opt ic n er ve
isch em ia an d edem a.
Post t raum at ic in t raocu lar lesion s, preexist ing n europath ies, fact iou s am blyopia
Management
Th e m an agem ent of in direct optic n er ve injur y sh ould in clude com plete ocular
exam in ation , color vision testing, visual field testing, com puted tom ographic scan -
ning of the head an d orbit , an d B-scan ultrasonography. Oth er tests that m ay be
useful are visual evoked potent ial an d electroretin ography. Th e t reatm en t of optic
ner ve in direct injur y is som ew h at con troversial. Ver y h igh-dose cor ticosteroids
have been proposed to lim it free-radical am plificat ion of th e injur y respon se. Sur-
ger y m ay be reser ved, w h en in dicated, for the cases of direct injur y or to decom -
press th e opt ic can al in indirect injuries. Nevertheless, th e serious com plicat ion s of
surger y, such as iatrogenic dam age of th e optic n er ve or of th e adjacen t struct ures,
should be carefully considered.
Orbital Trauma
Orbital Fractures
Blow -out fract u re of th e in ferior w all of th e orbit is th e m ost com m on of th e orbit al
fract u res. Th e m edial w all of th e orbit is th e th in n est of all an d is com m on ly associ-
ated w ith m u lt iple w all fract u res of th e orbit .
1 Ocular Traum a 27
Presentation
Pat ien ts presen t w ith pain (especially on at tem pted ver t ical eye m ovem en t), local
ten dern ess, bin ocular double vision , eyelid sw elling an d crepit u s after n ose blow -
ing, an d recen t h istor y of t rau m a. Exam in at ion reveals rest ricted eye m ovem en t
(especially in upw ard or lateral gaze), subcut an eou s or conjun ct ival em physem a,
hypoesth esia in th e dist ribut ion of th e in fraorbital n er ve (ipsilateral ch eek an d up-
per lip), an d en oph th alm os (m ay in it ially be m asked by orbital edem a). Associated
sign s in clu de n osebleed, eyelid edem a, an d ecchym osis. Superior rim an d orbital
roof fract ures m ay sh ow hypoesth esia in th e dist ribut ion of th e sup rat roch lear or
su praorbital n er ve (ipsilateral foreh ead) an d ptosis. Trism u s, m alar flat ten ing, an d
a p alpable step-off deform it y of th e in ferior orbital rim are ch aracterist ic of t ripod
fract u res ( Fig. 1.22A,B).
Orbit al edem a an d h em orrh age w ith ou t a blow -ou t , cran ial n er ve palsy
Fig. 1.22 (A) Blow-out fracture inferior wall. (B) Orbital blow-
out fracture of the lateral wall. (Image (A) Courtesy of Soosan Jacob,
Dr. Agarwal’s Eye Hospital, Chennai, India)
Management
Com plete oph th alm ologic exam in at ion , in cluding m easurem en t of ext raocu lar
m ovem en t s an d globe displacem en t . Ch eck p upils an d color vision carefu lly to
rule out a t raum at ic opt ic n eu ropathy. Forced-duct ion test ing is perform ed. Com -
puted tom ograp h ic scan of th e orbit s is obt ain ed in all cases of su spected orbit al
fract u res.
Treat m en t in cludes n asal decongest an ts (e.g., pseudoeph edrin e n asal spray,
t w ice a day); broad-spect ru m oral an t ibiot ics (e.g., ceph alexin 250 to 500 m g by
m outh four t im es a day, or er yth rom ycin 250 to 500 m g by m ou th fou r t im es a day)
for 7 days m ay be used bu t are n ot m an dator y. Apply ice packs to th e orbit for th e
first 24 to 48 h ours. Su rgical repair sh ould be con sidered based on th e follow ing
criteria.
Im m ediate repair (u su ally w ith in 24 h ou rs) is requ ired if th ere is eviden ce by
com pu ted tom ograph ic scan of en t rapped m u scle or periorbit al t issu e in com bi-
n at ion w ith diplopia an d n on resolving bradycardia, h ear t block, n ausea, vom it ing,
or syn cope.
Rep air in 1 to 2 w eeks is don e if th ere is eviden ce of p ersisten t , sym ptom at ic
diplopia in prim ar y or dow ngaze th at h as im proved at 1 w eek, w ith posit ive forced
du ct ion s an d eviden ce of en t rapm en t on com puted tom ography or large floor frac-
t u res (m ore th an on e h alf of th e orbital floor) th at h ave caused or are likely to
cau se cosm et ically un acceptable en oph th alm os.
Intraorbital Foreign Body

In t raorbital foreign bodies can occu r eith er from h igh -velocit y injuries or from
relat ively m in or t raum as. Th e n at u re of th e object is fun dam en tal in determ in ing
th e severit y of ocu lar an d orbit al com plicat ion s. Organ ic foreign bodies are p oorly
tolerated an d often lead to in flam m at ion . Most m et als, ston e, glass, an d plast ic
are u su ally in ert an d w ell tolerated. Th u s in organ ic foreign bodies t ypically cause
decreased vision or orbital com plicat ion s due to direct t rau m a, w h ereas organ ic
foreign bodies can easily develop orbit al in fect ion s.
Presentation
Pat ien ts m ay presen t w ith a recen t h istor y of t rau m a an d severe pain . How ever,
th ey can also be asym ptom at ic an d do n ot recall th e t rau m a at all. Pain , decreased
vision , an d dip lopia are com m on presen t ing sym ptom s. In t raorbit al foreign bodies
can be su btle an d n ot easily iden t ifiable on exam in at ion . Clin ical sign s in clude pal-
pable ocu lar m ass, proptosis, afferen t pu pillar y defect , edem a an d ecchym osis of
th e eyelids, lacerat ion of th e conju n ct iva or th e periocu lar t issu es, an d lim itat ion of
th e ext raocu lar m ovem en t s. Organ ic foreign bodies m ay in duce a m arked in flam -
m ator y respon se w ith elevat ion of th e serum w h ite cell cou n t ( Fig. 1.23 ).
Management
A detailed h istor y is n ecessar y to determ in e th e m ech an ism of injur y an d th e n a-
t u re of th e foreign body. A com plete op h th alm ologic exam in at ion sh ould be per-
form ed, w ith par t icular at ten t ion to fu n duscopic exam in at ion , in t raocu lar pres-
su re, an d pu pillar y react ion . Ocu lar an d periocular in spect ion sh ould be addressed
to discover an en t r y w ou n d. Neu rological test ing an d at ten t ion to th e pat ien t’s
m en tal stat us are requ ired to evalu ate a possible n eurological inju r y. Th e im aging
st u dy of ch oice is com pu ted tom ograph ic scan . It can reveal m ost foreign bodies,
an d it is safe in case of m et allic foreign bodies. How ever, w ooden or plast ic foreign
bodies can be m issed on com puted tom ograp h ic scan or can be m isiden t ified as
in t raorbital air. On ce a m et allic foreign body h as been exclu ded, m agn et ic reso-
1 Ocular Traum a 29
A B
C D
Fig. 1.23 Orbital foreign body removal. (A) Computed tomographic scan showing ra-
diopaque foreign body within the orbit. (B) Foreign body approached through wound
of entry. (C) Foreign body located and removed. (D) Final appearance after rem oval of
both foreign bodies and closure of wound. (All images courtesy of Soosan Jacob, Dr. Agarwal’s
Eye Hospital, Chennai, India; courtesy, Pablo Gili)
n an ce im aging can be u sefu l in diagn osing w ooden an d plast ic foreign bodies. Ul-
t rason ography represen t s a com plem en t ar y test . Th e m edical t reat m en t con sists
of tetan u s prophylaxis an d broad-spect rum system ic an t ibiot ic th erapy. Surgical
rem oval of th e foreign body dep en ds on th e n at u re an d th e locat ion of th e object .
Surgical in ter ven t ion is in dicated if sign s of in fect ion or opt ic n er ve com pression
are eviden t . Moreover, all organ ic an d poorly tolerated foreign bodies sh ou ld be
su rgically rem oved. Asym ptom at ic pat ien ts w ith sm all, n on organ ic in t raorbital
foreign bodies do n ot requ ire any su rgical in ter ven t ion .
Retrobulbar Hemorrhage
Orbit al h em orrh age in th e poten t ial sp ace su rrou n ding th e globe m ay occur after
blun t t rau m a an d subsequen t inju r y to th e orbit al vessels. Th e orbit is an en closed
space w ith lim ited capacit y for expan sion . Th e globe an d sept u m can be disp laced
an teriorly to som e exten t , giving rise to p roptosis. How ever, th is for w ard m ove-
m en t is lim ited, an d th e in creased volum e resu lts in in creased in t raorbital pres-
su re an d com pression of th e st ru ct u res con t ain ed in th e orbit . Trau m at ic h em or-

rh age in th e ret robu lbar space m ay lead to acute loss of vision du e to cen t ral ret in al
ar ter y occlusion , direct opt ic n er ve com pression , or com pression of th e opt ic n er ve
vascu lat u re. Acu te ret robulbar h em orrh age is a rare an d sigh t-th reaten ing com pli-
cat ion of blu n t eye t raum a, bu t it can be reversible w h en diagn osed an d t reated
prom ptly.
Presentation
Pat ien ts u su ally presen t w ith a recen t h istor y of t rau m a or orbital surger y, pain ,
an d decreased vision . Acute ret robulbar h em orrh age gives rise to m arked clin ical
sign s: pain fu l exoph th alm os or proptosis w ith resistan ce to ret ropu lsion , rest ric-
t ion of ext raocular m ovem en ts, diffu se subconjun ct ival h em orrh age, periorbit al
edem a, an d ecchym osis. In t raocular pressu re is t ypically raised. Congested con -
jun ct ival vessels, par t ial or com p lete oph th alm oplegia, afferen t p upillar y defect ,
an d color vision dist urban ces m ay also be presen t . An orbit al com pu ted tom o-
grap h ic scan dem on st rates a ret robu lbar h em atom a ( Fig. 1.24 ).
Orbit al cellu lit is, isolated orbit al fract ure, globe ru pt u re, carot id cavern ous fist ula,
an d varix
Management
Com puted tom ography is th e im aging st u dy of ch oice to determ in e ret robulbar
h em orrh age an d associated orbit al inju ries. How ever, it sh ou ld be delayed in sigh t-
th reaten ing cases. Medical th erapy con sist s of ocu lar hypoten sive m edicat ion s,
but it is con sidered an an cillar y procedu re for pat ien t s presen t ing w ith in creased
orbit al pressu re an d decreased vision . Th ese pat ien ts sh ou ld u n dergo em ergen t
decom pression of th e orbit al sp ace via su rgical drain age. Su rgical procedu re con -
sists of lateral can th otom y an d can th olysis. Early recogn it ion an d prom pt surgical
in ter ven t ion preser ve an d restore vision in m ost cases.
Fig. 1.24 Retrobulbar hemorrhage.

1 Ocular Traum a 31
Posttraumatic Pulsating Exophthalmos

Th e classic clin ical pict ure of p ulsat ing exoph th alm os, w h ich is a rare con dit ion ,
can be produ ced by post t raum at ic carot id-cavern ou s fist u las. Cerebral t raum as
accou n t for 75% of carot id-cavern ous fist ulas, w h ich are in it iated by tears in th e
w alls of th e in t racavern ou s in tern al carot id arter y or it s bran ch es. Th us ar terial
blood m ay sh ort-circuit in th e ven ou s com plex of th e cavern ous sin uses. Oth er
cau ses of pu lsat ing exoph th alm os are congen ital arterioven ous m alform at ion s, ar-
teriosclerosis-related ret robulbar an eu r ysm s, an d n eu rofibrom atosis.
Presentation
Pat ien ts t yp ically com plain , days or w eeks after t raum a, of a severe an d su dden
ceph alic an d orbital pain , a roaring sou n d in th e h ead syn ch ron ous w ith th e pulse,
decreased vision , diplopia, an d op h th alm oplegia. Th e pulsat ing exoph th alm os is
usually reducible. In sp ect ion reveals engorged an d ch em ot ic conju n ct iva. Palpa-
t ion of th e eye discloses a th rill, an d auscu ltat ion reveals an ocu lar or ceph alic
bruit syn ch ron ou s w ith th e pulse. Oth er ocular sign s in clu de dilated ret in al vein s,
disk edem a, ret in al vein occlusion s, ven ou s st asis ret in opathy, an d in creased in t ra-
ocu lar pressure due to altered outflow in th e vor tex vein s ( Fig. 1.25A,B).
Fig. 1.25 (A) Post traumatic ca-

rotid cavernous fistula. (B) Engorged
vessels and chem otic conjunctiva in
a patient with a traum atic carotid
B cavernous fistula.
Pu lsat ing exop h th alm os th at is n ot t raum a related
Management
A com plete oph th alm ological exam in at ion sh ou ld be perform ed, in cluding dilated
fun du scopic exam in at ion an d in t raocular pressu re m easu rem en t . Th e fun ct ion of
cran ial n er ves III, IV, an d VI sh ou ld be tested. Th e diagn osis can be con firm ed by
ech ography, digit al angiography, an d com pu ted tom ography. Th erapy is directed
to th rom bosis of th e fist u la an d n orm alizat ion of orbital h em odyn am ics via t ran s-
orbit al or t ran sven ou s em bolizat ion . Th e in crease in in t raocular pressu re can be
in it ially t reated m edically.
2 Eyelids and Lacrimal System
Christopher I. Zoum alan , Andrea Olm os, and Kim berly P. Cockerham
Ectropion
Ect rop ion is a frequ en tly seen con dit ion in elderly people; it is an eyelid m alposi-
t ion in w h ich th e eyelid (usu ally low er) is ever ted aw ay from th e globe.
Presentation
Many sym ptom s are a resu lt of ch ron ic irritat ion an d exposu re to th e eye an d eye-
lids. Pat ien ts can com plain of excessive tearing, conju n ct ivit is, corn eal epith eli-
opathy, an d kerat it is, all of w h ich can resu lt in decreased vision . Th e tarsal con -
jun ct iva can be ch ron ically in flam ed, w ith secon dar y ch anges in cluding th icken ing
an d kerat in izat ion . Th ere are five gen eral classificat ion s of ect ropion : involut ion al,
cicat ricial, paralyt ic, congen ital, an d m ech an ical:
Involut ional: At t ributed to age-related ch anges th at affect th e low er lids. Th ere
is loss of elast icit y of th e lid com par t m en t s, an d often th ere is m edial an d lateral
can th al ten don laxit y. Th e dist ract ion test an d th e sn ap-back test can determ in e
an abn orm alit y in h orizon tal lid laxit y. An terior lid dist ract ion greater th an 6 to
8 m m (w h ere th e cen t ral par t of th e eyelid can be pu lled aw ay from th e globe)
suggest s a posit ive lid dist ract ion test . If th e low er lid is pu lled in feriorly, th e lid
sh ould qu ickly ret u rn to it s previou s posit ion . If n ot , th is m ay be in terpreted as
an abn orm al sn ap-back test result ( Fig. 2.1A).
Paralyt ic: Because of ipsilateral facial n er ve palsy, often associated w ith lid re-
t ract ion an d subsequ en t lagoph th alm os. Exposu re keratopathy an d epiph ora
are com m on com p licat ion s ( Fig. 2.1B).
Cicat ricial: Often , scarring from ch ron ic in flam m at ion such as t rich iasis or sun
exposure can lead to a con t ract u re of th e anterior lam ellae (skin an d orbicularis
m uscle). Th ere is a sh or tage of an terior lam ellae skin such th at th e low er lid can -
not be superiorly lifted past th e in ferior lim bus in excess of 2 m m ( Fig. 2.1C).
Congenital ect ropion : May be seen in th e low er lid an d is gen erally seen in as-
sociat ion w ith con dit ion s such as bleph aroph im osis syn drom e an d ich thyosis.
Mechanical: Discrete eversion of th e eyelid du e to a lid lesion .
Make sure to rule out oth er cau ses of ect ropion such as floppy eyelid syn drom e
described earlier.
Management
Depen ds on exten t of ect ropion an d sym ptom s. Con ser vat ive m easures in clu de
aggressive lu bricat ion of th e ocu lar surface. Surger y is often n eeded for a defin ite
solut ion .
Involut ion ect ropion : A variet y of su rgical opt ion s are available depen ding on
th e exten t of ect ropion , h orizon tal lid laxit y, an d degree of can th al ten don lax-
it y. Gen eralized ect ropion can be corrected by h orizon t al lid sh or ten ing via fu ll-
th ickn ess excision or reat tach ing it to th e lateral can th u s th rough a lateral t arsal
st rip su spen sion tech n ique.
33
Fig. 2.1 (A) Involutional ectropion (with punctal ectropion and stenosis). (B) Paralytic
ectropion secondary to seventh nerve palsy (brow ptosis and lagophthalmos are also
present). (C) Cicatricial ectropion caused by a lower eyelid scar. (Courtesy of Dr. Soosan
Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)
2 Eyelids and Lacrim al System 35
Paralyt ic ect ropion : Tem porar y t reat m en t w ith a t arsorrh aphy can h elp redu ce
th e exten t of exposu re keratopathy. Perm an en t cases m ay require th e use of
m edial can th oplast y, m edial w edge resect ion , an d/or lateral can th al su spen sion
to redu ce th e h orizon t al an d ver t ical dim en sion s of th e palpebral aper t ure.
Cicat ricial ect ropion : Use of skin grafts or t ran sposit ion al flaps to restore th e
n orm al an terior lam ellae
Congenital ect ropion : Recon st ru ct ing th e an terior lam ellae w ith th e u se of skin
graft s or t ran sposit ion al flaps
Entropion
Inw ard rot at ion of th e eyelid
Presentation
Th e m argin of th e eyelid an d lash es m akes con tact w ith th e globe an d, in cert ain
cases, can lead to corn eal epith eliopathy an d subsequ en t u lcerat ion or pan n u s for-
m at ion . Irrit at ion an d epiph ora are com m on presen t ing sign s. Th ey can be involu -
t ion al, congen it al, an d cicat ricial:
Involut ional: Age-related ch anges involving degen erat ion of th e elast ic an d fi-
brous t issu es, u sually affect ing th e low er lid. Th e lateral an d/or m edial h orizon -
tal laxit y is associated w ith in creased orbicu laris ton e causing inw ard rot at ion
of th e eyelid ( Fig. 2.2A).
Cicat ricial: Scarring from t raum a, Steven s-Joh n son syn drom e an d oth er cica-
t rizing con dit ion s, ch em ical bu rn s, an d t rach om a can lead to sh or ten ing of th e
posterior lam ellae ( Fig. 2.2B).
Congenital: Often seen in low er lids, u sually related to th e lack of n orm al devel-
opm en t of th e ret ractor apon eu rosis.
Epibleph aron , dist ich iasis, t rich iasis, bleph arospasm , an d r uling ou t oth er cau ses
of en t ropion already m en t ion ed
Management
Tem porar y t reat m en t can be ach ieved by ocular lu bricat ion an d lid taping. Bot u li-
n um toxin h as been used w ith su ccess in involu t ion al or congen it al cases. Surgical
correct ion is often used in severe cases.
Involut ional: If th ere is lit tle h orizon tal lid laxit y, t ran sverse evert ing su t ures
can provide a tem porar y solu t ion . Horizon t al lid split t ing w ith in ser t ion of
ever t ing sut ures provides a last ing correct ion . In cases w ith associated h ori-
zon tal lid laxit y, h orizon tal lid sh orten ing can provide ben efit in addit ion to th e
aforem en t ion ed procedures.
Cicat ricial: Mild cases can be corrected w ith a t ran sverse t arsotom y (tarsal frac-
t u re) w ith an terior rotat ion of th e lid m argin . More exten sive cases w ill often
em ploy th e use of com posite grafts (m ucou s m em bran e or palate) to recon -
st ru ct th e dam aged posterior lam ellae.
Congenital: Can be corrected w ith excision of a st rip of skin an d u n derlying orbi-
cularis m uscle an d w ith possible fixat ion of th e skin crease to th e tarsal plate.
Fig. 2.2 (A) Involutional entropion

with trichiasis. (B) Cicatricial entro-
pion with associated trichiasis.
Ptosis
An abn orm ally low posit ion of th e upper eyelid, w h ich m ay be congen it al or
acquired
Presentation
Pat ients com plain of a tired appearan ce and deficits in their superior visual field.
To overcom e this, patien ts m ay elevate th eir ch in posit ion or subsequently contract
their fron talis m uscle to raise th eir brow s. Certain m easurem en ts are key in the
evaluation. Margin to lid reflex (MRD) is the distance from the m argin of th e upper
lid to the central corn eal reflex (norm al is 4.0 to 4.5 m m ). Levator function m ea-
sures the distan ce of excursion of th e upper eyelid m argin from far dow ngaze to
upgaze w h ile the fron talis m uscle is held still w ith th e exam in er’s th um b (n orm al
is 14 m m or m ore). Th e palpebral fissure is th e distance from th e upper to th e low er
eyelid m argin w h en the patient is in prim ar y gaze (norm al range can var y from 7
to 12 m m and is greater in w om en th an in m en ). Superior lid crease is th e vertical
distan ce of th e superior lid m argin from th e lid crease in dow ngaze (norm al 8 to
10 m m ).
Th ere are variou s cau ses of ptosis, in clu ding th e follow ing:
Congenital: Failu re of n eu ron al m igrat ion w ith in th e levator com plex. Can be
un ilateral or bilateral w ith variable severit y. Poor levator fu n ct ion an d absen t
lid crease. Ptosis im proves on dow ngaze. Need to be evalu ated for am blyopia
( Fig. 2.3A).
Fig. 2.3 (A) Congenital ptosis, right eye. (B) Bilateral ptosis, compensating with fron-
talis overaction. (C) Suprabrow and lid scars following conventional sling surgery (D)
Presurgical and postsurgical pictures following Jacob Agarwal guided sling surgery. Note
only a single scar over the forehead. ([A] Courtesy of Deborah Alcorn, MD; [C–D] Courtesy of
Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)
Aponeurot ic: Most com m on t ype of ptosis, u su ally seen in elderly pat ien ts. Can
be bilateral or u n ilateral. Du e to a deh iscen ce or disin ser t ion of th e levator apo-
n eu rosis, u su ally th e result of involu t ion al ch anges. Norm al levator fun ct ion bu t
h igh superior lid crease (> 12 m m ) ( Fig. 2.3B).
Neurogenic: In n er vat ion al defect du e to an ocu lom otor n er ve palsy
Myogenic: Seen in defects in th e n eurom u scular jun ct ion it self or w ith in th e
levator com plex; can be du e to m yasth en ia gravis or m uscu lar dyst rophy
Mechanical: Secon dar y to a gravit y m ass effect or con t ract ion from a scar
It is im port an t to differen t iate t ru e ptosis from pseudoptosis, w h ich can be caused
by con t ralateral lid ret ract ion , ipsilateral hypot ropia, brow ptosis, an d derm atoch a-
lasis. Oth er differen t ial diagn oses in clu de Marcu s-Gun n jaw w in king syn drom e,
aberran t th ird or seven th n er ve regen erat ion , an d bleph arop h im osis syn drom e .
Management
Depen ds on th e severit y of th e ptosis an d its et iology. Usu ally severe ptosis w ith
poor levator fu n ct ion w ill n eed to be addressed by a fron t alis-sling procedure. Le-
vator resect ion is in dicated in cases w ith fair to good levator fun ct ion (at least 5
m m ). Cases w ith reason ably good or excellen t levator fu n ct ion can be addressed by
eith er a posterior approach or an an terior apon eu rosis repair.
Congenital ptosis: Usu ally n eeds to be addressed early if am blyopia is a con cern ,
especially in u n ilateral cases. Depen ding on th e levator fu n ct ion , differen t pro-
cedu res can effect ively correct th e ptosis. Poor levator fun ct ion (< 4 m m ) w ill
requ ire a fron talis-sling procedu re, w h ereas fair levator fu n ct ion (> 4 m m ) m ay
be corrected w ith a levator resect ion .
Aponeurot ic ptosis: Several opt ion s are available, depen ding on th e severit y of
th e ptosis. For in stan ce, a posterior approach (e.g., Fasan ella-Ser vat procedu re
or Mü ller-conjun ct ival resect ion ) can correct m ild cases. Altern at ively, an an te-
rior app roach w ith rein ser t ion or advan cem en t of th e apon eurosis can correct
cases w ith excellen t levator fu n ct ion .
Ja cob-Aga rwa l Technique of Guided Sling Surgery with Single Sta b Incision Fron t alis
m u scle su spen sion procedu re is th e gold st an dard for th e t reat m en t of congen ital
ptosis w ith poor levator fun ct ion . It creates a lin k bet w een th e fron t alis m u scle an d
th e tarsus of th e up per eyelid, w h ich allow s for a bet ter eyelid posit ion in prim ar y
gaze. Th e Jacob-Agar w al tech n iqu e differs from th e conven t ion al p rocedu res by
th e use of a single-stab in cision in m aking th e pen t agon an d guiding th e silicon e
sling in th e su rgical plan e w ith on e extern al in cision w h ile suspen ding th e fron ta-
lis m uscle ( Fig. 2.3C,D).
Surgica l Technique A pen t agon sh ape is m arked over th e skin w ith a m arker. A
single su praeyebrow st ab in cision of ~2.5 to 3.0 m m is pu t on th e su perior m ark
of th e pen t agon ~5 m m from th e eyebrow, an d a subp eriosteal pocket is dissected
upw ard ( Fig. 2.4 ). A sterile fron t alis su spen sion set (Seiff) dipped in an t ibiot ic is
used as th e sling m aterial. Th is h as a long, solid silicon e rod/t u be w ith a length of
40cm (15¾ in ) an d a diam eter if .80m m (.032 in ), w ith a stain less steel n eedle on
eith er en d (20G × 2½ in ). Th e silicon e t ube is provided w ith a silicon e sleeve, w h ich
is rem oved from th e t ube before su rger y begin s ( Fig. 2.5 ).
Th e n eedle is first passed th rough th e ep i-t arsal t issu e bet w een th e m arks m ade
on th e u p per eyelid. Th e lid is ever ted an d ch ecked to ascer t ain th at it h as n ot
gon e th rough th e fu ll th ickn ess of th e t arsu s. W ith a lid gu ard beh in d th e lid, th e
m edial en d of th e n eedle is th en in ser ted th rough th e m edial n eedle exit p oin t on
A B
C D
Fig. 2.4 (A) Upper eyelid elevation and contour checked preoperatively. (B) Pentago-
nal shape as in conventional sling surgery is m arked over the skin. (C) Single supraeye-
brow stab incision of ~ 2.5 to 3.0 mm is put on the superior mark of the pentagon ~ 5
mm from the eyebrow. (D) Subperiosteal pocket dissected upward. (All images courtesy of
Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)
Fig. 2.5 Seiff Silicone frontalis suspension set (BD Ophthal-

mic Systems, Bidford on Avon, UK). (Courtesy of Soosan Jacob, Dr.
th e eyelid an d advan ced u pw ard, dipp ing beh in d th e sept u m , to th e m ark m ade
on th e m edial eyebrow. W h en th e u p per m edial corn er of th e p en t agon is reach ed,
th e n eedle is th en t u rn ed an d gu ided in th e sam e su rgical plan e (w ith ou t exte-
riorizing), u sing a com bin at ion of visu alizat ion an d p alpat ion an d is brough t ou t
th rough th e cen t ral su p rabrow in cision ( Fig. 2.6 ). Th e sam e p rocedu re is repeated
w ith th e n eedle on th e lateral side so th at it t races th e path of th e lateral lim b of
th e pen t agon , dip s beh in d th e orbit al sept u m , t u rn s in direct ion above th e su -
praorbit al rim , an d th en exter iorizes th rough th e cen t ral sup rabrow in cision . Th e
su rgeon’s n on dom in an t in dex finger can be u sed to p alp ate th e n eedle as it is be-
ing advan ced. Th e t w o en ds of th e silicon e rod are th readed th rough th e silicon e
sleeve, an d th e lid m argin an d con tou r are adju sted according to th e am ou n t of
correct ion requ ired an d for m axim al cosm esis. Th e t w o en ds of th e silicon e rod s
are also kn ot ted togeth er, an d a 6-0 silk su t u re is t ied bet w een th e kn ot s to p re-
ven t late slip page. Th e sleeve w ith th e kn ot s is th en bu ried in to th e su bp eriosteal
pocket . If n eed ed, on e m ay also h itch th e silicon e t u be kn ot to th e u n derlying
periosteu m . Th e single su prabrow st ab in cision is closed w ith silk su t u re or fibrin
A B
C D
Fig. 2.6 (A) The needle is passed through the superficial tarsal tissue bet ween the
marks made on the upper eyelid. (B) The medial end of the tube is advanced through
the medial needle exit point on the eyelid and advanced upward, dipping behind the
septum, to the mark m ade on the m edial eyebrow. (C) When the upper medial corner
of the pentagon is reached, the needle is then turned and guided in the same surgical
plane (without exteriorizing) using a combination of visualization and palpation. (D) The
needle is brought out through the central suprabrow incision. The same is repeated on
the other side. (All images courtesy of Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)
glu e to m in im ize scar form at ion . Th e Oth er n eed le pu n ct u re sites n eed n ot be

su t u red ( Fig. 2.7 ).
Th e advan tage of th e tech n ique is th at w ith m in im al skin in cision s an d less
su rgical t im e, th e clin ical ou tcom e of a conven t ion al fron talis sling procedu re is
obt ain ed. Postoperat ive lid an d brow edem a, ecchym osis, pain , su t u re-related
com plicat ion s, an d scarring du e to m u lt iple in cision s an d su t u res can be avoided.
Th e tech n iqu e can be perform ed in all eyes w ith ptosis an d poor levator fu n ct ion
th at n ecessit ate a fron talis sling. Th e stab in cision used is on ly ~2.5 to 3.0 m m .
It is th us advan tageou s over th e conven t ion al procedure th at involves five stab
in cision s, w h ich creates m ore bleeding an d edem a in th e im m ediate postopera-
t ive period an d m ore scarring in th e late postoperat ive period. Mild im m ediate
postoperat ive edem a m ay occu r, but it gen erally resolves spon tan eously w ith in 24
h ou rs. Th ough silicon e m aterial for fron t alis sling su spen sion h as been t ried suc-
cessfully, th e gu ided sling procedure w ith a silicon e sling h as n ot been rep or ted.
Th ere h ave been report s in th e past of sling procedures u sing a m in im u m or n o in -
cision . Th e Jacob-Agar w al tech n iqu e differs from th ese procedures in th at th e en ds
of th e sling m aterial are un ited in th e cen t ral foreh ead in cision (rath er th an in th e
eyelid in cision as previou sly described by oth er au th ors), w h ich provides an up -
w ard direct ion of t ract ion for bet ter lid h eigh t an d con tour. Also, th e m aterial used
for th e sling is th e com m on ly available Seiff silicon e su spen sion set , w h ich ptosis
su rgeon s are m ore fam iliar an d com for t able w ith th an n on absorbable sut u re. Lid
closure is also bet ter w ith silicon e sling m aterial rath er th an w ith n on absorbable
slings. Th is tech n iqu e th erefore provides bet ter aesth et ic an d fun ct ion al resu lts
in pat ien t s w ith poor levator fu n ct ion an d congen ital ptosis. It is also opt im al for
pat ien ts w ith m yopath ies, m yasth en ia, th ird n er ve palsy, an d sim ilar con dit ion s
w h ere silicon e suspen sion is conven t ion ally preferred over oth er sling m aterials
becau se of its in h eren t elast icit y. Th is tech n ique is un ique in its sim ple learn ing
cur ve, good cosm esis, sm aller n um ber of su t u res w ith bet ter fun ct ion al result s
w h ile ret ain ing th e u sual advan tages of stan dard sling procedu res.
A B
Fig. 2.7 (A) The t wo ends of the rod are passed through the silicone sleeve, and the
lid margin and contour are adjusted according to the amount of correction required
and for maximal cosm esis. (B) Measurement of the incision site is shown. (Both images
courtesy of Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)
Dermatochalasis
Th is is a ver y com m on con dit ion seen in elderly pat ien ts, u su ally p resen t s bilater-
ally. It m ay be asym ptom at ic.
Presentation
Pat ien ts presen t w ith redu n dan t upper lid skin th at can be associated w ith fat h er-
n iat ion th rough a w eak sept um . In m oderate to severe cases, th e excess lid can
obst ruct th e sup erior visu al field ( Fig. 2.8A,B,C).
Acqu ired or congen ital ptosis, eyebrow ptosis, floppy eyelid syn drom e, prolapsed
lacrim al glan d an d eyelid edem a.
Fig. 2.8 (A) Bilateral dermatocha-

lasis. (B) Dermatochalasis and brow
ptosis. (C) Dermatochalasis with
C lash ptosis.
Management
In cludes bleph aroplast y to rem ove excess skin . Pat ien ts often h ave a com bin ed
ptosis an d n eed to be evaluated for th is com pon en t . If so, th ey m ay ben efit from
ptosis su rger y as w ell.
Eyelid Retraction
Eyelid ret ract ion occurs w h en th e upper lids rest above or at th e level of th e supe-
rior lim bus.
Presentation
Pat ien t s often h ave oth er sign s, in clu ding proptosis, in ferior scleral sh ow, lagoph -
th alm os w ith resu lt ing exp osure keratopathy, an d oth er sign s of acu te in flam m a-
t ion (conju n ct ivit is, ch em osis). Th is is m ost com m on ly seen in thyroid eye disease
( Fig. 2.9 ).
Thyroid eye disease, as already m en t ion ed. Oth er causes in clude con t ralateral pto-
sis w ith ipsilateral lid ret ract ion secon dar y to Herring’s law, aberran t th ird n er ve
regen erat ion , cicat ricial ch anges or scarring of th e upper lid, sym path etom im et ic
oph th alm ic drops, overcorrect ion of ptosis su rger y, ch ron ic con tact len s u se, Parin -
au d syn drom e, an d Miller Fish er varian t of Guillain -Barré syn drom e.
Management
W h en sym ptom s are m ild, art ificial lubricat ion of th e ocular surface can often
am eliorate th e sym ptom s. Severe cases w ith lit tle p roptosis m ay requ ire eyelid
su rger y. Th ere h ave been a variet y of tech n iqu es described to su rgically correct
eyelid ret ract ion . Both posterior (t ran sconju n ct ival) an d an terior (cut an eou s) ap-
proach es h ave been described. Both approach es involve a variable degree of leva-
tor m u scle com plex recession an d/or m u llerectom y to correct th e ret ract ion .
Fig. 2.9 Eyelid retraction and proptosis from thyroid eye

disease.
Floppy Eyelid Syndrome
Th is syn drom e is a frequ en tly m isdiagn osed con dit ion th at can presen t un ilater-
ally or bilaterally. It t ypically affect s obese m en w h o m ay also su ffer from obst ru c-
t ive sleep apn ea an d is also associated w ith tobacco sm oke.
Presentation
Th e upper eyelids are loose an d rubber y an d ten d to evert during sleep. Th e lax
upper eyelid is ever ted easily w h en pulled superiorly tow ard th e eyebrow du ring
physical exam in at ion . Th e soft an d ru bber y tarsal plate can also be folded up on
itself. Th is resu lts in t raum a to th e exposed tarsal conju n ct iva w ith su bsequen t
ch ron ic papillar y conjun ct ivit is ( Fig. 2.10A,B,C).
Fig. 2.10 (A) Floppy eyelid syndrome.

(B) Floppy eyelid syndrome with papillary
tarsal conjunctival changes. (C) Floppy
eyelid syndrome. (Image [A] courtesy of Soo-
san Jacob, Dr. Agarwal’s Eye Hospital, Chennai,
India; Image [C] courtesy of Pablo Gili)
C
Allergic conjun ct ivit is, gian t p apillar y conjun ct ivit is, atop ic keratoconju n ct ivit is,
su perior lim bic kerat it is, ect ropion , derm atoch alasis
Management
In m ild cases, taping of th e eyelids or n oct u rn al eye sh ields can am eliorate sym p-
tom s. Severe cases m ay requ ire h orizon t al lid sh orten ing using fu ll-th ickn ess pen -
tagon al w edge resect ion in th e u pper an d low er eyelids.
Chalazion
Ch ron ic, lipogran u lom atous in flam m ator y lesion located w ith in th e tarsus. Th ese
occur frequ en tly an d are cau sed by a bu ildup or blockage of secret ion from m ei-
bom ian glan d orifices.
Presentation
Can be seen in all ages, and presents w ith a gradually enlarging painless nodule. If
large enough, upper eyelid lesions can induce astigm atism . The lesions can be m ul-
tiple and bilateral. Make sure to evaluate the patient for blepharitis, ocular rosacea,
and seborrheic derm atitis, w hich can predispose patients to chalazia (Fig. 2.11A,B).
Fig. 2.11 (A) Upper eyelid chalazion. (B)

Pyogenic granulom a. (Courtesy of Soosan Ja-
A cob, Dr. Agarwal’s Eye Hospital, Chennai, India)
B
Hordeolu m (ten der to palpat ion ) an d sebaceou s cell carcin om a
Management
Lesion s th at are n ew or sm all m ay resolve spon t an eou sly. Warm com presses of 30
m in utes, fou r t im es per day, m ay h elp relieve or reduce th e in flam m at ion . Topical
an t ibiot ics can also be u sed. Persisten t lesion s m ay be t reated eith er by excision
(posterior approach w ith th e u se of a ch alazia clam p) or th rough in t ralesion al ste-
roid inject ion (0.5 to 2.0 m L t riam cin olon e aceton ide, 5 m g/m L). Biopsy of recu r-
ren t lesion s sh ou ld be don e to ru le out a sebaceou s cell carcin om a .
Hordeolum
Painful nodules as a result of an acute bacterial infection, m ost com m only from Staph-
ylococcus aureus. In contrast, chalazia are chronic lesions and t ypically not painful.
Presentation
Th ese can eith er presen t as an extern al h ordeolum (m argin of eyelid) or in tern al
h ordeolum (seen by ever t ing eyelid). In tern al h ordeolu m s can cause severe con -
jun ct ivit is or ch em osis. Usu ally associated w ith m eibom ian glan d dysfun ct ion an d
bleph arit is ( Fig. 2.12 ).
Ch alazion (n ot ten der to palpat ion ), blep h arit is
Management
Most cases are self-lim ited an d w ill resolve w ith in 5 to 7 days. Lid hygien e an d
w arm com p resses can h elp in th eir resolut ion . Som e m ay persist an d becom e cyst-
like, w h ich m ay requ ire su rgical in cision an d cu ret t age (see above t reat m en t for
Fig. 2.12 External hordeolum.

ch alazion ). Treat m en t of accom panying bleph arit is an d m eibom ian glan d dysfun c-
t ion w ill h elp to p reven t recu rren ces.
Eyelid Edema
Sw elling of eyelids due to flu id collect ion w ith in th e su bcu tan eous t issu es
Presentation
The thin skin and loose subcutaneous tissue are susceptible to water accum ulation
and edem a, especially in the upper lids. Sym ptom s can be either inflam m atory or non-
inflam m atory. Inflam m atory signs can include redness, sensation of heat and pain,
and m arked unilaterality. This can be secondary to infections such as cellulitis (presep-
tal or orbital), eczem a, and abscess. In contrast, noninflam m atory signs include pale
skin color, cool skin, absence of pain, and bilaterality. This can be seen m ore com m only
w ith system ic disorders (heart, kidneys, thyroid eye disease) and secondary to an al-
lergic response. Recurrent episodes of persistent eyelid edem a can result in stretching
of the overlying thin skin and m ay result in blepharochalasis (Fig. 2.13A,B).
Allergic respon se, h ordeolu m , abscess, eczem a, dacr yocyst it is, cellu lit is (preseptal
or orbit al), thyroid eye disease, derm atoch alasis
Management
Treat th e u n derlying system ic or in flam m ator y disorder.
Fig. 2.13 (A) Left eyelid edema in a female with thyroid eye
disease. (B) Left eyelid edema in a male with acute thyroid eye
disease.
Blepharoptosis and Blepharophimosis Syndrome
A rare, autosom al dom in an t , congen ital syn drom e
Presentation
Moderate to severe ptosis (sym m et rical) w ith poor levator fu n ct ion , sh or ten ed
h orizon t al palpebral fissures, lateral ect ropion of th e low er lids, telecan th us (ab-
n orm ally in creased dist an ce bet w een th e m edial can th i of both eyelids), an d epi-
can th al inversus (see th e sect ion on Epican th us) ( Fig. 2.14 ).
Fig. 2.14 Blepharophimosis syndrome. (Courtesy of Soosan Ja-

cob, Dr. Agarwal’s Eye Hospital, Chennai, India)
Congen it al or acquired ptosis, congen it al fibrosis syn drom e
Management
In it ially involves t reat m en t of epican th al folds an d telecan th u s follow ed later
(u sually a few m on th s) by bilateral fron t alis su spen sion . Th e p at ien ts n eed to be
screen ed an d follow ed up for am blyopia because it can develop in u p to 50% of
cases.
Epiblepharon
An ext ra h orizon t al fold of skin exten ding across th e an terior lid m argin resu lt ing
in ver t ically orien ted lash es
Presentation
A com m on fin ding am ong eastern Asian s, th is sh ou ld n ot be m ist aken for congen i-
tal or acqu ired en t ropion . Un like en t ropion , th e lash es do n ot m ake con t act w ith
th e corn ea in prim ar y gaze, bu t th ey often can in dow ngaze. Th e n orm al locat ion
of th e lid becom es apparen t w h en th e fold of skin is pulled dow n (Fig. 2.15 ).
Fig. 2.15 Epiblepharon. (Courtesy of

Soosan Jacob, Dr. Agarwal’s Eye Hospital,
Chennai, India)
Congen it al or acqu ired en t ropion
Management
Man agem en t is usually n ot requ ired because m ost cases spon tan eously resolve
w ith age. How ever, persisten t cases or on es th at cau se sym ptom s (corn eal epith e-
liopathy) are su rgically m an aged by excising a st rip of skin an d orbicularis m u scle
(an terior lam ellar resect ion ) w ith or w ith out fixat ion of th e skin to th e tarsus.
Epicanthus
Th ese are bilateral w ebs of skin th at exten d from th e up per to low er eyelids tow ard
th e m edial can th u s. If large en ough , th ey cau se pseudoesot ropia. Th ey can be seen
in syn drom es su ch as bleph aroph im osis syn drom e an d t risom y 21.
Presentation
Epican th al folds can presen t in fou r variou s su bt ypes:
Epicanthus tarsalis: Th e folds origin ate in th e upp er m edial eyelid an d exten d
in to th e m edial can th us. Th is is th e m ost com m on t ype seen in eastern Asian s
( Fig. 2.16 ).
Epicanthus inversus: Th is t ype origin ates in th e low er eyelid an d exten ds in to
th e m edial can th u s. Th is is associated w ith blep h aroph im osis syn drom e.
Epicanthus palpebralis: Th e folds exten d from th e u pper to low er eyelids in a
sym m et ric dist ribu t ion .
Epicanthus superciliaris: Th ese are broad folds th at exten d from th e eyebrow
dow n to th e low er orbital rim .
Epibleph aron . Rule ou t syn drom es associated w ith epican th al folds su ch as bleph -
aroph im osis syn drom e an d t risom y 21.
Fig. 2.16 Epicanthus tarsalis.
Management
Epican th u s u su ally resolves by th e age of 4. Several su rgical procedu res such as
Z- or Y-V-plast ies can be em ployed to repair persisten t cases.
Eyelid Coloboma
Th is is a par t ial- or fu ll-th ickn ess eyelid defect due to an em br yon ic cleft , u sually
t riangular in sh ape, w ith it s base at th e m argin .
Presentation
Th ese are rare defect s, u su ally congen ital an d result ing from a lack of closu re of th e
opt ic cu p. Th ey are m ost com m on ly seen in th e u pper lids (at th e ju n ct ion of th e
m iddle an d outer th irds), eith er in isolat ion or w ith oth er syn drom es. Kerat in iza-
t ion or exposure keratopathy can occu r depen ding on th e severit y of th e colobom a.
Colobom as can be accom pan ied by addit ion al deform it ies such as m icroph th alm os
or derm oid cysts ( Fig. 2.17 ).
Fig. 2.17 Upper eyelid coloboma. (Courtesy of Soosan Jacob, Dr.

Ableph aron (congen it al absen ce of eyelid), t rau m a. Ru le out oth er syn drom es as-
sociated w ith colobom as such as Golden h ar syn drom e (ocu loau riculover tebral
dysplasia) an d Fran cesch et t i syn drom e (m an dibulofacial dystosis).
Management
Con ser vat ive t reat m en t w ith th e u se of lubricat ing drops an d oin t m en ts if sm all
an d th ere is n o risk of exposure keratopathy. How ever, defects u sually n eed to be
closed by prim ar y, direct closure or, if large en ough , w ill requ ire th e use of skin
graft s or rotat ion flaps or both .
Eyelid Tumors
Papilloma
Th e m ost com m on ben ign t u m or of th e eyelids.
Presentation
Th ese presen t as a pedu n culated or sessile (broad-based) lesion . Th ey are a ben ign
t u m or of epith elial origin (Fig. 2.18A,B).
Molluscu m con tagiosum , ch alazion , squ am ous cell carcin om a, basal cell carci-
n om a
Management
Man agem en t can con sist of obser vat ion or elect ive rem oval by excision . Sh ave bi-
opsies can be perform ed if th e diagn osis is u n cer tain .
A B
Fig. 2.18 (A) Papilloma. (B) Upper eyelid margin papilloma.

Seborrheic Keratosis
Slow -grow ing, discrete, greasy lesion w ith a friable surface
Presentation
Th ese often ap pear to be “st u ck on ” th e skin . Th e lesion is usually brow n an d flat
but can often be pedu n cu lated ( Fig. 2.19A,B).
Nevus, m elanom a, squam ous cell carcinom a, acrochordon (skin tag), actinic keratosis
Management
Cu ret t age an d excision are cu rat ive.
Actinic Keratosis
Also term ed solar keratosis, th is is a “prem align an t” lesion seen in fair-skin n ed
in dividuals w h o h ave been exposed to excessive su n ligh t .
Presentation
Lesion s can be scaly, flat , w ith hyperkeratot ic feat u res. Th ey often begin as sm all,
rough m acu les or papules ( Fig. 2.20 ).
Fig. 2.19 (A) Seborrheic keratosis.

(B) Seborrheic keratosis, lower eyelid.
B
Fig. 2.20 Actinic keratosis (arrow s

depict lesions).
Basal cell carcinom a, cutaneous horn, squam ous cell carcinom a, seborrheic keratosis
Management
Th ese lesion s sh ou ld be biopsied for diagn osis an d t reated w ith com plete excision
or cr yoth erapy.
Keratoacanthoma
Th is is an un com m on but rapidly grow ing lesion w ith a cen t ral kerat in m ass.
Presentation
Th ey are often seen in fair-skin n ed in dividuals w ith excessive su n ligh t exp osure.
Th ey can often regress spon tan eously, leaving a cen t ral, su n ken scar. Th ey are h is-
topath ologically in clu ded in th e spect rum of squ am ou s cell carcin om as an d clin i-
cally appear sim ilar to squ am ous cell carcin om as ( Fig. 2.21 ).
Fig. 2.21 Keratoacanthoma.

Basal cell carcin om a, cu tan eous h orn , act in ic keratosis, squ am ous cell carcin om a,
an d seborrh eic keratosis
Management
Biopsy su spicious lesion s. Com plete su rgical excision w ith free m argin s is recom -
m en ded. Laser or cr yoth erapy can be applied to sm all lesion s.
Molluscum Contagiosum
Th ese are virally t ran sm it ted lesion s u sually seen in you nger pat ien t s (teen agers
an d ch ildren ) or in im m u n ocom prom ised pat ien ts.
Presentation
Th ey are n on in flam m ator y, sm ooth , p early, dom e-sh ap ed papules w ith cen t ral
depression s often fou n d n ear th e upper an d low er eyelids. Th ey are t ran sm it ted
by direct con t act an d are u su ally asym ptom at ic but can be associated w ith ch ron ic
conju n ct ivit is (Fig. 2.22 ).
Fig. 2.22 Molluscum contagiosum.
Basal cell carcin om a, squam ou s cell carcin om a, papillom a
Management
Lesion s can be excised by a curet or cr yoth erapy.
Nevi
Nevi are ben ign lesion s th at occu r w ith in th e epith elium an d derm is.
Presentation
Th e lesion s are derived from m elan ocyt ic cells an d can be eith er pigm en ted or
n onpigm en ted. Nevi can be h istologically classified as ju n ct ion al, com pou n d, an d
in t raderm al. In t raderm al n evi are con fin ed w ith in th e derm al layer. Th ey are usu-
ally n onpigm en ted an d elevated. Th ey h ave n o m align an t poten t ial (Fig. 2.23A).
Jun ct ion al n evi are w ell circum scribed, flat , an d u n iform ly brow n . Th ey are lo-
cated at th e ju n ct ion of th e epiderm is an d derm al layers an d h ave a low poten t ial
for m align an cy ( Fig. 2.23B). Com poun d n evi h ave both ju n ct ion al an d in t raderm al
involvem en t ( Fig. 2.23C).
Fig. 2.23 (A) Intraderm al nevi. (B) Junctional nevus. (C) Ca-
runcular compound nevus.
Malign an t m elan om a, basal cell carcin om a, ben ign lesion s
Management
Carefu lly docu m en t th e size of th e lesion w ith ph otograph s. Su rgically rem ove le-
sion s th at in crease in size.
Nevus of Ota
Congen it al oculoderm al m elan ocytosis th at involves both skin (derm is) an d eye
(ep isclera, sclera, an d uveal t issu es)
Presentation
Pat ien ts presen t w ith deep, u n ilateral hyp erpigm en tat ion of th e eyelid skin an d
ocu lar st ru ct u res. Th ese n evi are associated w ith iris hyperch rom ia an d fu n du s
hyperpigm en t at ion . Pat ien t s are at an in creased risk of glaucom a an d, th ough rare,
m elan om a ( Fig. 2.24 ).
Malign an t m elan om a
Management
Follow regularly for m align an t ch ange an d glaucom a screen ing.
Fig. 2.24 Nevus of Ota. Note the

relative sparing of dermal involve -
ment.
Xanthelasma
Xan th elasm a is a com m on ly seen con dit ion th at is frequen tly bilateral an d often
seen in elderly pat ien ts or th ose w ith hyperlip idem ia. How ever, m ost pat ien t s
w ith xan th elasm a are n orm olipoprotein em ic. Th ese can rarely be th e presen t ing
sign of xan th ogran ulom atou s disease.
Presentation
Yellow ish su bcu t an eou s plaques are often foun d aroun d th e eyelids, especially
arou n d th e m edial can th al areas.
Am yloidosis, eccrin e hydrocystom a, at ypical lym ph oid in filt rate sarcoid
Management
Th e lesion s can be su rgically rem oved elect ively; h ow ever, recurren ces are com -
m on . Altern at ively, excision or dest r uct ion by carbon dioxide, argon laser, cr yo-
th erapy, an d ch em ical cau terizat ion (ch lorin ated acet ic acids) can be perform ed,
th ough scarring an d hyperpigm en tat ion can occu r.
Cysts—Moll/Zeis/Sebaceous
Cysts of th e glan ds can resu lt in roun d, clear, an d t ran sillum in at ing lesion s .
Presentation
Th ere are various t yp es of du ctal cysts. Cysts of Moll (ap ocrin e sw eat glan d hydro-
cystom a) are usually foun d on th e an terior lid m argin an d t ran sillu m in ate w ell.
Th ey can be foun d in th e m edial can th al angle, an d gravit y can often result in
ect ropion . Eccrin e sw eat glan ds, th ough n ot con fin ed to th e lid m argin , appear
like apocrin e cyst s. Cysts of Zeis an d sebaceous cyst s con tain oily secret ion s an d
th erefore do n ot t ran sillum in ate ( Fig. 2.25A,B,C).
Ben ign an d m align an t lesion s, ch alazion , extern al h ordeolum
Management
Warm com presses an d topical an t ibiot ics are h elpfu l. Marsupializat ion of th e cyst s
is usu ally curat ive. Suspiciou s lesion s sh ou ld be sen t for biopsy.
Syringoma
Syringom as are ben ign skin t u m ors of eccrin e differen t iat ion , m ore often fou n d in
w om en .
Presentation
Skin -color papu les are u su ally located on th e eyelids an d can in crease in size an d
qu an t it y.
Fig. 2.25 (A) Apocrine hydrocys-

toma. (B) Eccrine hydrocystoma. (C)
Sebaceous hydrocystoma.
Verr uca, xan th elasm a, cylin drom a
Management
Pap ules can be elect ively rem oved by su rgical excision or elect rodissect ion an d
curet tage. Th e lesion s can recur.
Neurofibroma
Neu rofibrom as are in filt rat ive n er ve cell t u m ors th at are largely com p osed of
Sch w an n cells.
Fig. 2.26 Infiltrative neurofibroma of left orbit. (Courtesy of

Deborah Alcorn, MD).
Presentation
Th e t um ors u sually occur early in life an d can be eith er n odu lar or plexiform . Th ey
can involve th e upper lid (classic-sh aped appearan ce) an d frequen tly cau se a m e-
ch an ical ptosis ( Fig. 2.26 ).
Cap illar y h em angiom a, lym ph om a, rh abdom yosarcom a
Management
Su rgical excision can be at tem pted, bu t th ese lesion s are ver y difficu lt to rem ove
su ccessfully.
Tumors
Basal Cell Carcinoma

Th is is th e m ost com m on eyelid m align an cy. Th ere is an in creased risk in people
w ith fair skin an d in in dividuals w ith in creased exposure to ult raviolet radiat ion
(ch ron ic skin dam age).
Presentation
Th ese t ypically presen t as a firm lesion w ith raised m argin s. A cen t ral crater w ith
su perficial vascularizat ion or u lcerat ion can often be seen . Loss of eyelash es (m ad-
arosis) alm ost alw ays suggest s m align an cy. Most com m on ly seen (in decreasing
order of relat ive frequ en cy) in th e low er eyelids, m edial can th u s, up per eyelid, an d
lateral can th us ( Fig. 2.27A,B,C,D).
Nevu s, papillom a, keratoacon th om a, m align an t m elan om a, squ am ous cell carci-
n om a
Fig. 2.27 (A) Basal cell carcinom a.

(B) Ulcerating basal cell carcinoma. (C)
Ulcerating basal cell carcinoma with
necrosis of surrounding skin. (D) Ul-
cerating basal cell carcinoma with
umbilicated, central crater.
D
Management
Biopsy su spicious lesion s. Su rgical excision w ith free m argin s (recom m en ded 3 to
5 m m ) of h ealthy t issu e sh ould be p erform ed. Altern at ive t reat m en t m odalit ies
in clu de cr yoth erapy, elect rodissect ion , cu ret t age, Moh s m icrograph ic surger y, an d
radioth erapy. Th ese lesion s h ave rare m etast at ic poten t ial.
Squamous Cell Carcinoma

Squ am ous cell carcin om as com prise less th an 5%of eyelid m align an cies. Sim ilar to
basal cell carcin om a, th e prim ar y cau se of m ost squam ou s cell carcin om a is cu m u-
lat ive lifet im e su n exposu re, especially in fair-skin n ed in dividu als.
Presentation
Lesion s can presen t as clin ically sim ilar to basal cell carcin om as, bu t th ey com -
m on ly grow rapidly w ith spread to region al lym ph n odes. Th ey can also exten d
in to th e in t racran ial cavit y via perin eural spread ( Fig. 2.28A,B,C). Clin ically th ese
can presen t as th ree su bt ypes:
Fig. 2.28 (A) Squamous cell carci-

noma. (B) Squamous cell carcinom a
of upper eyelid. (C) Cystic squam ous
cell carcinoma. C
Plaquelike : Scaly, hyperkeratot ic lesion s at site of preexist ing act in ic keratosis

Nodular: Hyperkeratot ic n odu le w ith cru st ing fissu res
Ulcerat ing: Well-defin ed evert ing borders w ith an er yth em atous an d ulcerated
base
Basal cell carcin om a, keratoacan th om a, act in ic keratosis
Management
Su rgical excision w ith free m argin s (recom m en ded 3 to 5 m m ) of h ealthy t issu e or
Moh s m icrograph ic surger y sh ou ld be p erform ed. Lesion s n ot com pletely resect-
able can be t reated w ith adjun ct ive radiat ion or cr yoth erapy or both .
Sebaceous Cell Carcinoma

Th ese are older-grow ing lesion s frequ en tly arising from th e m eibom ian glan ds an d
usually seen in th e upper eyelids. Th ey are m ore com m on ly seen in older w h ite
w om en . Th ey com prise approxim ately 5% of eyelid m align an cies. Th ere is often a
delay in diagn osis given its in sidiou s clin ical appearan ce.
Presentation
Can presen t eith er as a n odu lar or a pagetoid spreading m eibom ian glan d carci-
n om a. Th e n odu lar t ype presen ts as a discrete n odu le th at often is m istaken for a
ch alazion . Th e pagetoid sp reading su bt ype spreads in to th e derm is an d epith elium
in a diffu se pat tern , often m im icking ch ron ic conju n ct ivit is ( Fig. 2.29A,B).
Fig. 2.29 (A) Sebaceous cell car-

cinoma of eyelid margin. (B) Seba-
ceous cell carcinoma involving m ost
of the upper tarsal conjunctiva.
B
Bleph arit is, ch alazion , superior lim bic keratoconju n ct ivit is, ch ron ic conju n ct ivit is,
cicat ricial pem ph igoid
Management
Su rgical excision w ith w ide su rgical m argin s w ith frozen -sect ion con t rols is often
n ecessar y. Conju n ct ival m apping h elps evaluate pagetoid spreading. Evalu ate local
lym ph n odes (preauricular an d cer vical), an d perform a system ic evaluat ion for
m etast at ic spread.
Cutaneous Malignant Melanoma

Th is is rarely seen on th e eyelids but can m anifest as potent ially lethal skin lesion s.
Presentation
Th ese often present as a slow ly grow ing pigm en ted lesion, but alm ost h alf of lesions
can be n onpigm ented ( Fig. 2.30A,B,C). Th ey are clinically seen in three t ypes:
Fig. 2.30 (A) Lentigo maligna.

(B) Melanoma of medial canthus.
(C) Melanoma of upper eyelid. C
Lent igo m aligna (pre-m elanom a lesion) : A slow -grow ing pigm en ted lesion , often
affect ing elderly pat ien t s, w h ich can develop in to a m elan om a
Superficial spreading: Su perficially spreading lesion w ith an irregu lar ou tlin e,
variable pigm en tat ion , an d delay in pen et rat ion in to deeper layers
Nodular: A m ore aggressive lesion th at h as a ten den cy to invade deeper layers
early in it s grow th
Nevus, basal cell carcin om a, keratoacon th om a, seborrh eic keratosis, squ am ous cell
carcin om a
Management
Excision al biopsy w ith 3- to 5-m m free m argin s is recom m en ded for th in cut a-
n eou s periocular m elan om as. Melan om as th icker (u sually greater th an 2m m in
th ickn ess) an d located elsew h ere m ay require 1 to 3 cm free m argin s depen ding
on th eir th ickn ess. Th e exten t of surgical an d adju n ct ive th erapy is determ in ed by
t u m or t ype, level, an d clin ical stage.
Distichiasis and Trichiasis
Dist ich iasis can be eith er a congen it al or an acquired con dit ion of th e eyelids an d
involves th e abn orm al grow th of lash es from th e orifices of th e m eibom ian glan ds.
Trich iasis is an acqu ired con dit ion of eyelash es th at are m isdirected tow ard th e
globe.
Presentation
Dist ich iasis can presen t in a variet y of w ays. Th e dist ich iat ic lash es can be th in
or of n orm al th ickn ess, pigm en ted or n onpigm en ted, h ave n orm al orien tat ion ,
or m ay be m isdirected. Acqu ired cases of dist ich iasis can be seen in longst an d-
ing cicat rizat ion associated w ith t rach om a, ch em ical inju r y, Steven s-Joh n son syn -
drom e, an d ocular pem p h igoid. Trich iasis can be th e result of scarring of th e lid
m argin secon dar y to ch ron ic t rach om a, bleph arit is, Steven s-Joh n son syn drom e,
an d h erpes zoster oph th alm icu s. In all cases, th e lash es ru b again st th e eye an d can
cau se irrit at ion , tearing, an d corn eal epith eliopathy. Longst an ding cases can resu lt
in corn eal u lcerat ion an d pan n us ( Fig. 2.31A,B).
En t ropion , ep ibleph aron , bleph arit is, an d topical prostaglan din an alogue m edica-
t ion s for glaucom a
Management
Num erou s approach es h ave been reported for t reat m en t . Ep ilat ion is accom plish ed
by lash rem oval w ith forceps (n ot a p erm an en t solut ion ) or m ore effect ively w ith
elect rocau ter y, cr yoth erapy, or argon laser to in dividu al lash es. Altern at ively, th ey
can be surgically approach ed in a variet y of w ays, in cluding a com bin at ion of la-
m ellar eyelid division w ith cr yoth erapy to th e aberran t lash es or direct surgical
excision by w edge resect ion .
Fig. 2.31 (A) Distichiasis. (B) Tri-

chiasis (of upper lid). Note the cica-
tricial changes of both upper and
lower lids.
Lacrimal System Disorders
Canaliculitis
Can alicu lit is con sist s of eith er or both in flam m at ion an d in fect ion of th e upp er or
low er can alicu lus.
Presentation
Th e can alicu lar region is er yth em atou s, in du rated, an d ten der to p alpat ion . Pa-
t ien t s com plain of ep iph ora w ith ch ron ic m u copuru len t disch arge. Th e m ost com -
m on bacterial path ogen is Act inom yces, w h ich produces gran ular-like con cret ion s
th at are difficu lt to express from th e pun ct i ( Fig. 2.32A, B).
Ch ron ic dacr yocyst it is, eth m oidal m u cocele
Management
Local an t ibiot ics sh ould be u sed according to th e path ogen s iden t ified on cult u res
an d sen sit ivit y (gen erally su scept ible to pen icillin s an d ceph alosporin s). Surgical
in cision (can alicu lotom y) w ith drain age an d curet tage of th e con cret ion s is often
used for su ccessful t reat m en t .
Fig. 2.32 (A) Chronic canaliculitis.

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

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

Th is can be congen it al or acquired. In congen it al cases, th ere is a delay in th e can a-
licu lizat ion of th e low er port ion of th e n asolacrim al du ct , w h ich is seen in up to
20% of in fan t s du ring th e first year of life bu t is sym ptom at ic in less th an 4% of
th ese ch ildren . Acquired cases can be secon dar y to t rau m a or in fect ion .
Presentation
Con st an t ep iph ora an d w et t ing of th e eyelash es. Mu copuru len t m aterial is often
expressed from th e low er pu n ct i after lacrim al m assage. Sym ptom s can w orsen
du ring an u pper resp irator y in fect ion ( Fig. 2.34 ).
Fig. 2.34 Left nasolacrimal duct obstruction.

Congen it al glau com a in in fan t s, eth m oidal m u coceles, lacrim al sac t u m ors (ben ign
or m align an t), dacr yocyst it is, can alicu lit is
Management
Treat m en t sh ou ld be delayed in in fan t s un t il about 1 year of age becau se m ost (up
to 95%) of cases self-resolve. Nasolacrim al du ct probing an d irrigat ion are usually
curat ive in over 90% of in fan t s. Recu rren t failures often im ply an an atom ical prob-
lem an d m ay require silicon e in t ubat ion or balloon dilat ion . Persisten t failu res m ay
n eed a dacr yocystorh in ostom y.
3 Orbital Infections, Inflammation, and
Neoplasms
Praveen Saluja, Sw at i Ravani, Soosan Jacob, and Am ar Agarw al
Preseptal Cellulitis
Preseptal cellu lit is is defin ed as a soft t issue in fect ion an terior to th e orbit al sep -
t u m . In fect ion p osterior to th is sept um , anyw h ere in th e orbit , is orbit al cellulit is.
Orbit al cellu lit is is a dangerous con dit ion ow ing to th e close proxim it y to th e or-
bital apex, cavern ous sin us, m en inges, an d brain . Bacterial in fect ion of th e eyelid
an terior to th e orbital sept um t ypically affects ch ildren , usu ally secon dar y to lid
in fect ion su ch as severe acu te h ordeolu m , skin lacerat ion , an in sect bite, or th e
spread of in fect ion from th e su rroun ding st ruct ures (paran asal sin uses, lacrim al
sac, u pper respirator y t ract , in cluding th e m iddle ear). Th e in fect ion does n ot pen -
et rate th e orbital sept um , w h ich separates th e an terior st ru ct u res from th e orbit .
Presentation
Sym ptom s in clu de eyelid edem a (w h ich m ay lead to in abilit y to op en th e eye),
periorbit al sw elling, rubor, color, ten dern ess, w ith out proptosis. Un like orbit al cel-
lulit is, th ere is n o pain w ith eye m ovem en t s. Ocu lar m ot ilit y, visu al acuit y, an d
pupillar y react ion s are all n orm al (Fig. 3.1A,B,C).
Orbital cellulit is: Decreased visu al acu it y, decreased sen sat ion along th e first di-
vision of th e t rigem in al n er ve, eyelid edem a, proptosis, ch em osed conjun ct iva,
pain w ith eye m ovem en t s, rest ricted eye m ovem en ts, sign s of ocu lar m ot ilit y
disorders
Cavernous sinus throm bosis: Bilateral, decreased visual acu it y, decreased sen sa-
t ion along th e first an d secon d division of th e t rigem in al n er ve, p roptosis an d
paresis of cran ial n er ves III, IV, an d VI, ch em osed conju n ct iva
Chalazion: Focal, usu ally w ith ou t ten dern ess, gradu ally progressive ch ron ic in -
flam m at ion of th e m eibom ian glan d
Allergic edem a of the eyelid
Contact derm at it is
Viral conjunct ivit is associated w ith lid edem a: Watering, itch ing, st ickin ess of
th e eyelash es, conjun ct ival follicu lar react ion , w ith or w ith ou t disch arge an d
palp able preauricular lym ph n ode
Erysipelas: Acu te st reptococcal cellu lit is (m ostly h as a clear-cut dem arcat ion
lin e) w ith sign s of toxem ia, in clu ding h igh -grade fever an d ch ills
Others: In sect bite, angioedem a, t raum a, osteom yelit is of paran asal sin u ses, es-
pecially m axillar y sin us
Management
Ch eck for a h istor y of t rau m a, rapidit y of on set , pain , fever, ch ills, can cer, diabe-
tes, pulm on ar y diseases, an d ren al diseases. Ch ar t th e vit als (pu lse, respirat ion ,
tem perat u re, blood pressure). Exam in e for exoph th alm om et r y, globe displace-
m en t , an d resistan ce to ret ropulsion an d exam in e th e orbital rim . Record ocular
m ovem en t an d m easure deviat ion w ith a prism bar. Pup ils m u st be evalu ated for
ligh t reflexes, in cluding relat ive afferen t pupillar y defect (RAPD). Color vision , in -
69
Fig. 3.1 (A) Preseptal cellulitis, allergic reaction. (B) Presep-

tal cellulitis, bacterial infection final. (C) Preseptal cellulitis,
fungal infection.
t raocu lar pressu re (in clu ding th e pressu re in variou s gazes), an d ret in al evalu at ion
sh ould be recorded. Evaluate th e cran ial n er ves (especially III, IV, V1 , V2 , VI,). Exam -
in e th e h ead an d n eck for lym p h aden it is. Gram stain ing an d cu lt u re of any open
w ou n d an d disch arge sh ould be perform ed at th e earliest opport u n it y. A com plete
an d differen t ial blood cou n t is perform ed if sign s of toxem ia exist .
3 Orbital Infections, In am m ation, and Neoplasm s 71
Oral an t ibiot ics are in dicated in cases of m ild in flam m at ion , afebrile pat ien t , age
m ore th an 5 years, good pat ien t com p lian ce. Drugs of ch oice in clu de am oxicillin -
clavu lan ate, or cefaclor, or cot rim oxazole, or er yth rom ycin , clin dam ycin , am oxi-
cillin -cloxacillin for a durat ion of 10 days. In t raven ou s an t ibiot ics are in dicated
in m oderate to severe in flam m at ion s. In cases of pat ien t age less th an 5 years,
p oor pat ien t com plian ce, im m un ocom prom ised pat ien ts, n o im provem en t w ith or
w orsen ing w ith oral an t ibiot ics, dr ugs of ch oice in clu de ceft riaxon e w ith van com y-
cin . Supp or t ive t reat m en t in clu des h ot fom en t at ion s, local an t ibiot ics (p olym yxin
w ith bacit racin oin t m en t), an d n on steroidal an t i-in flam m ator y drugs (NSAIDs).
Explorat ion of th e w oun d is perform ed if n eeded.
Orbital Inflammation
Orbital Cellulitis/Subperiosteal Abscess/Cavernous Sinus Syndrome

In orbit al cellu lites, in fect ion occu rs posterior to th e orbit al sept um , usually secon d-
ar y to th e sp read of in fect ion from th e su rroun ding st ruct ures (paran asal sin u ses,
lacrim al sac, upper respirator y t ract in clu ding th e m iddle ear) or lid in fect ion , su ch
as severe acu te h ordeolu m , skin lacerat ion , or an in sect bite (Fig. 3.2A,B,C).
Presentation
Orbital cellulit is: Eyelid edem a (usually leading to in abilit y to open th e eye),
periorbit al sw elling, rubor, ten dern ess, proptosis, pain w ith eye m ovem en t s, re-
st ricted ocular m ot ilit y, decreased vision , ret in al ven ou s congest ion , opt ic disk
edem a, pu rulen t disch arge, an d decreased periorbital sen sat ion . Th e follow ing
are m ain t ypes:
Sin u s-related is th e m ost com m on an d is secon dar y to eth m oidal sin u sit is; it
affect s ch ildren an d young adu lts.
Caused by adjacen t st ru ct u res like dacr yocyst it is, m idfacial in fect ion , or den -
tal in fect ion .
Post t raum at ic m ost com m on ly develops w ith in 48 to 72 h ou rs of an inju r y
th at pen et rates th e orbit al sept u m .
Subperiosteal abscess: Most frequ en tly located along th e m edial w all of th e or-
bit . Orbital abscess is relat ively less com m on w ith sin usit is but is m ore com m on
in post t rau m at ic or postoperat ive cases. Usually presen t s w ith m edial m ass,
n on axial proptosis, local ten dern ess, in creased in t raocu lar pressure, abscess
(in t racon al/ext racon al).
Cavernous sinus throm bosis: Bilateral, decreased visu al acuit y; decreased sen sa-
t ion along th e first an d secon d division of th e t rigem in al n er ve; rapidly pro-
gressive proptosis; paresis of cran ial n er ves III, IV, an d VI; congest ion of th e
conju n ct ival vein s; ch em osed conjun ct iva; dilated an d sluggish pu pil; sign s of
toxem ia in clu ding h igh -grade fever, decreased level of con sciou sn ess, n au sea,
an d vom it ing.
B C
Fig. 3.2 (A) Ten-year-old boy with orbital abscess. (B) Magnetic resonance imaging
(MRI) of an orbital abscess. (C) MRI, coronal section.
Differential Diagnosis (Table 3.1)
Carot id cavernous fist ula: Spon tan eous or post t rau m at ic, bru it on au scult at ion
of globe; ar terialized conju n ct ival vessels an d conjun ct ival ch em osis are n ot un -
com m on on com puted tom ograph ic scan . En larged su perior oph th alm ic vein
(SOV), en larged ext raocu lar m u scles, orbit al color Doppler u lt rasou n d sh ow s
reversed ar terialized blood in SOV.
Erysipelas: Acute st reptococcal cellulit is. Mostly h as a clear-cu t dem arcat ion
lin e w ith sign s of toxem ia in clu ding h igh -grade fever, ch ills.
Others: In sect bite, angioedem a, t raum a, osteom yelit is of paran asal sin uses (es-
pecially m axillar y sin us), ch alazion , allergic edem a of th e eyelid, con t act der-
m at it is, viral conju n ct ivit is associated w ith lid edem a.
Table 3.1 Di erential Diagnosis of Orbital In ammatory Conditions
Thyroid
Feature Pseudotumor Exophthalmos Orbital Cellulitis
1. Lateralit y Unilateral Bilateral Unilateral

2. Age 20 to 50 years Fourth to fth Children and
decade young adults
3. Onset Acute, subacute, Chronic Acute
chronic
4. Clinical Proptosis, ptosis, Proptosis with Periorbital
presentation chemosis with pain lid signs swelling and
tenderness
5. Laboratory Increased ESR Abnormal Increased WBC
ndings thyroid
function
tests
6. Systemic Malaise Thyroid Fever
symptoms symptom s
7. Response to Small doses Higher doses Responds to
Steroids antibiotics
ESR, erythrocyte sedim entation rate; WBC, white blood cell count
Management
Th e pat ien t m u st be adm it ted to th e h ospit al. Gram st ain ing an d cult u re of any
open w ou n d an d disch arge sh ou ld be don e at th e earliest oppor t u n it y along w ith
com plete an d differen t ial blood cou n t an d blood cu lt ures. Mu corm ycosis m u st
be kept in m in d, especially in diabet ic an d im m u n osu ppressed p at ien ts. Lum bar
pun ct u re for suspected m en ingit is is perform ed un der a physician’s su per vision .
Neu rologic opin ion sh ould be un der t aken if th e gen eral con dit ion of th e pat ien t
dict ates th e sam e. In t raven ous an t ibiot ics su ch as ceft riaxon e plu s van com ycin ,
or van com ycin plus gen tam icin , or van com ycin plu s clin dam ycin w ith or w ith ou t
m et ron idazole are given in it ially follow ed by oral an t ibodies for 7 to 14 days. Hot
fom en t at ion is applied fou r to five t im es a day. Local an t ibiot ics in clude polym yxin
w ith bacit racin oin t m en t . For corn eal exposu re, NSAIDs h elp com bat pain an d in -
flam m at ion .
Mon itor for th e follow ing w arn ing sign s:
Dilated pu pils
Marked oph th alm oplegia
Loss of vision
Relat ive afferen t p upillar y defect
Papilledem a
Perivasculit is
Violaceou s lids
Explorat ion of th e w ou n d is in dicated if th e pat ien t is un respon sive to an t ibiot ics,
vision is decreasing, orbital abscess is presen t , an d a diagn ost ic biopsy is n eeded.
It is im port an t to drain th e orbit al abscess as w ell as th e in fected sin u ses. Th e fol-
low ing oral an t ibiot ics are given on ly after th e con dit ion im proves sign ifican tly:
am oxicillin -clavulan ic acid, or cefaclor, or cot rim oxazole, or er yth rom ycin , clin da-
m ycin , am oxicillin -cloxacillin w ith or w ith out m et ron idazole.
Thyroid-Related Ophthalmopathy
Graves’ disease or diffuse toxic goiter, is an au toim m u n e process th at in clu des on e
or m ore of th e follow ing: hyper thyroidism , oph th alm opathy, an d in filt rat ive der-
m opathy.
Presentation
Graves’ disease u sually occu rs w ith hyper thyroidism , but n orm al thyroid fu n ct ion
can also be n ot iced. It is five t im es m ore com m on in fem ales (Fig. 3.3A,B). Th ere
are t w o t ypes according to level of severit y:
1. Noninfilt rat ive (m ild): Min im al in flam m ator y react ion leading to m ild sym p-
tom s an d sign s.
2. Infilt rat ive (severe): Th is t ype h as a m ore fu lm in an t course w ith in flam m a-
t ion , in filt rat ion , an d scarring. Th ese pat ien t s h ave ch em osis, proptosis, corn eal
exposu re, m yosit is, an d en largem en t of m uscles. It ult im ately leads to corn eal
exposu re, rest ricted m ovem en t s, an d diplopia.
Werner classification reflects the severit y of the ophthalm opathy and is well know n
by the acronym of NO SPECS (as described below ). Each grade is further subdivided
as 0 to 4 and a to c:
Grade 0: No sign s or sym ptom s
Grade 1: On ly sign s (lid ret ract ion )
Grade 2: Soft t issu e involvem en t (e.g., ch em osis)
Grade 3: Proptosis (m in im um )
Grade 4: Ext raocu lar m u scle involvem en t
Grade 5: Corn eal involvem en t
Grade 6: Sigh t loss
Th ere are variou s sign s in thyroid eye disease, w h ich go by th e discoverers’ n am es
(Fig. 3.3C,D,E) (Table 3.2).
Fig. 3.3 (A) Thirt y-five-year-old woman with dysthyroid or-

bitopathy with lid retraction. (B) Lid lags behind when patient
looks down.
Fig. 3.3 (Continued) (C) Dysthyroid orbitopathy. (D) Com -

puted tomographic (CT) scan, extraocular muscle enlarge -
ment sparing muscle tendons. (E) Coronal section CT scan.
Table 3.2 Ophthalmic Manifestations of Graves Disease
Most important signs

Von Graefe: Upper lid lag on downgaze
Dalrymple: Upper eye lid retraction
Upper eyelid signs
Von Graefe: Upper lid lag on downgaze
Dalrymple: Upper eyelid retraction
Boston: Uneven jerky movement of upper lid on inferior movement
Jellinek: Abnormal pigmentation of upper lid
Kocher: Retraction of upper lid during xation
Gi ord: Di cult eversion of upper eyelid
Pupillary signs
Cowen: Extensive hippos of consensual papillary light re ex
Lowey: Dilatation of pupil with 1:1000 epinephrine
Knies’ sign: Uneven di cult dilatation in dim light
Bruit signs
Riesman: Bruit over eyelid
Snellen sign: Bruit over the eye
Eye movement signs
Ballet: Paralysis of one or more extraocular muscles (EOM)
Möbius: De cient convergence
Suker: Inabilit y to maintain xation at extrem e lateral gaze
Wilder: Jerking of eyes on movement from abduction to adduction
Blinking signs
Pochin: Reduced amplitude of blinking
Stellwag: Incomplete or infrequent blinking
Lag signs
Von Graefe: Upper eyelids lag on downgaze
Gri th sign: Lower eyelid lag on upgaze
Conjunctival signs
Goldzieher: Deep injection of temporal conjunctival vessels
Orbital pseudotum or, cavernous sinus throm bosis, orbital cellulitis (see Table 3.1)
Management
Man agem ent opt ion s for thyroid eye disease in clude obser vat ion , con ser vat ive in -
ter ven tion s, oral cort icosteroids, injected cor ticosteroids, extern al-beam radioth er-
apy, an d surger y. Obser vat ion an d con ser vative m easures are appropriate for m ild
conjun ctival inject ion an d ch em osis w ith n orm al corn ea and opt ic n er ve fu nction .
Advice to th e pat ien t in cludes sleeping w ith th e h ead of the bed elevated an d avoid-
ing salt or m on osodium glutam ate to m in im ize fluid reten tion . Sunglasses are rec-
om m en ded to m in im ize ph otoph obia. Nonpreser ved art ificial tears h elp decrease
th e ocular irritat ion . Glaucom a m edicat ion s sh ould be used on ly in patien ts w ith
ver y h igh in t raocular pressures and a fam ily h istor y of glaucom a. Surgical in ter-
ven t ion sh ould be perform ed in a stepped fash ion : decom pression first, strabism us
surger y second, an d eyelid surger y last . Pat ien ts should stop sm oking an d avoid
secon dh an d sm oke to lim it th e autoim m un e exacerbat ion of thyroid eye disease.
Man agem en t of t hyroid orbitop at hy m u st t ake in to accou n t w h eth er th e dis-
ease is act ive or ch ron ic an d t h e d egree to w h ich th e m an ifest at ion s im p act t h e
p at ien t ’s daily life or t h reaten sigh t . For exam p le, m ild eyelid ret ract ion w ith
m in im al or n o d r y eye sym ptom s m igh t be m an aged w it h con ser vat ive lu brica-
t ion p r ior to elect ive eyelid su rger y on ce th e p at ien t h as st abilized . At t h e op -
p osite en d of t h e sp ect r u m is t h e p at ien t p resen t ing w it h acu te opt ic n eu rop a-
thy resu lt in g from ap ical crow d in g th at requ ires u rgen t m ed ical an d /or su rgical
m an agem en t .
Pat ien ts sh ould be m an aged in close correspon den ce w ith an en docrin ologist ,
w h o m ay elect to t reat system ic hyp erthyroidism via ph arm acological supp res-
sion , surgical resect ion , or radioact ive iodin e. Recen t st udies suggest a ben efit to
steroid th erapy in th e peri-in ter ven t ion al period in m in im izing th e progression of
orbitopathy.
Gen eral t reat m en t opt ion s for th e m an agem en t of sequ elae of thyroid orbitopa-
thy in clude ph arm acological th erapy, radiat ion th erapy, an d surgical in ter ven t ion .
Mild cases w ith m in im al ocular irritat ion or sym ptom at ic diplopia m ay in it ially be
m an aged con ser vat ively w ith lu bricat ing eyedrops an d oin t m en t , n oct u rn al tap-
ing, an d Fresn el prism s.
Radioth erapy, gen erally 20 Gy delivered in 10 fract ion s over 2 w eeks, h as long
been used as t reat m en t for th e orbit al m an ifestat ion s of thyroid disease as h as
been sh ow n to be of ben efit in im provem en t of m ot ilit y. How ever, a recen t pro-
spect ive, ran dom ized st u dy by Gorm an et al dem on st rated n o ben eficial th erapeu-
t ic effect of radioth erapy in m oderate, sym ptom at ic thyroid orbitopathy.
Surgical m an agem en t can be divided in to elect ive an d u rgen t in ter ven t ion s.
Elect ive su rger y sh ould proceed in th e order of orbit al decom pression , st rabism us
su rger y, an d fin ally lid surger y, becau se each in ter ven t ion as listed can in fluen ce
th e ou tcom e of th e su bsequen t in ter ven t ion s. Th ere are a m u lt it u de of ap proach es
to orbit al decom pression en com passing on e to all w alls, w ith or w ith ou t en do-
scopic an d t ran sn asal exposure an d w ith or w ith ou t orbit al fat decom pression .
St rabism u s su rger y sh ould be u t ilized to m axim ize th e field of bin ocu lar vision
an d gen erally in cludes recession of m uscles on adjust able sut u res. Eyelid proce-
du res in clu de bleph arop last y w ith or w ith ou t rem oval of orbital fat , release of u p-
per lid ret ract ion via levator an d or Mu ller m u scle recession , an d repair of low er
lid ret ract ion w ith spacer grafts.
In dicat ion s for u rgen t su rgical in ter ven t ion in clude opt ic n europathy from api-
cal crow ding an d corn eal u lcerat ion secon dar y to exposu re. Th e m an agem en t of
th ese con dit ion s can in clude m any of th e procedures outlin ed earlier in addit ion
to urgen t m edical m an agem en t w ith pu lse cor t icosteroids, ph arm acoth erapy, an d
orbit al irradiat ion .
Cor t icosteroids can provide sh ort-term relief for sym ptom s an d sign s of thyroid
eye disease, but hyper thyroid pat ien t s can suffer sign ifican t m ood sw ings. In addi-
t ion , tapering th e steroids often result s in rebou n d in flam m at ion at least as severe
as th e origin al presen tat ion . Oth er im m u n osu ppressive agen ts h ave been used in
th e t reat m en t of thyroid orbitopathy as steroid sparing agen ts, in cluding cyclospo-
rin e, cytoxan , m eth ot rexate, an d azath ioprin e. Som e favor com bin at ion th erapy
w ith cyclosporin e an d cor t icosteroids. Oth er t reat m en t m odalit ies in clude som a-
tost at in an alogu es (oct reot ide), plasm aph eresis, an d in t raven ou s im m u n oglobulin
th erapy.
Orbital Inflammatory Pseudotumor

Orbit al in flam m ator y p seu d ot u m or (OIP) con sist s of a sp ect r u m of n ongran u lo-
m atou s in flam m ator y con d it ion s of th e orbit , w ith n o kn ow n et iology or system ic
associat ion s, th at p rodu ce p roptosis du e to a n on n eop last ic in flam m ator y m ass
in th e orbit . (Note: Som e au th ors classify Tolosa-Hu n t syn drom e as a su bt yp e of
OIP.)
Fig. 3.4 Middle-aged woman with

left eye pseudotum or.
Presentation
OIP presents w ith abrupt onset of pain, proptosis (unilateral), conjunctival chem osis,
epibulbar injection, visual loss, diplopia, and restricted ocular m ovem ents (Fig. 3.4).
According to the different tissues involved, it is classified as one of the follow ing:
Myosit is
Dacr yoaden it is
Periopt ic n eurit is
Posterior sclerit is or ten on it is
It s clin ical cou rse is variable, an d it m ay be regressed spon tan eously w ith out any
t reat m en t , or it m ay h ave prolonged in flam m at ion or in term it ten t act ivit y. A pro-
longed course m ay result in a frozen orbit secon dar y to fibrosis. Bilateral involve-
m en t is rare.
Graves oph th alm opathy, orbital cellulit is, leu kem ia, cavern ou s sin us th rom bosis,
rh abdom yosarcom a (see Table 3.1)
Management
Com puted tom ograph ic (CT) scan s an d m agn et ic reson an ce im aging (MRI) are es-
sen t ial in th e w orkup of susp ected OIP. In flam m ator y sign s predom in ate an d m ay
in clu de involvem en t of th e ext raocu lar m u scles (EOMs), orbit al fat , lacrim al glan d,
ch oroid, an d sclera. Orbit al ult rasoun d m ay be of use in dem on st rat ing th icken ing
of th e posterior Ten on capsu le an d in dist ingu ish ing th e m yosit is of OIP from EOM
involvem en t in thyroid orbitopathy becau se th e m uscu lar ten don s w ill classically
be involved in OIP an d spared in thyroid disease.
Th e CT orbit sh ow s ext raocu lar m uscle th icken ing involving ten din ous in ser-
t ion . In flam m at ion of th e ret robu lbar fat pad an d con t rast en h an cem en t of th e
sclera du e to ten don it is m ay produ ce a T sign or ring sign . Orbit al ult rasou n d
sh ow s th icken ing of th e posterior Ten on capsule along w ith m uscle belly th icken -
ing (un like thyroid related orbitop athy, w h ich t ypically spares th e ten don s).
System ic steroids (60 to 80 m g/day) are given . Rapid respon se is p ath ogn om on -
ic. Taper slow ly over m on th s to avoid recu rren ce. Pu lsed in t raven ou s steroids are
given in severe vision -th reaten ing cases. Radioth erapy is recom m en ded in steroid-
resist an t cases. Ch em oth erapeut ic agen ts su ch as cycloph osph am ide, m eth ot rex-
ate, an d cyclosporin e are used for cases resist an t to steroids an d radioth erapy an d
in pat ien t s in toleran t to steroids.
Orbital Lymphoma
Orbit al lym ph om a is a low -grade m align an cy ch aracterized by proliferat ion of
m on oclon al B cells (n on -Hodgkin disease), w h ich arises in lym ph n odes or in ex-
t ran odal sites su ch as th e orbit .
Presentation
Th e disease presen t s bet w een th e ages of 50 an d 80 years w ith involvem en t of any
par t of th e orbit . Bilateral involvem en t is rare. It occu rs rarely in ch ildren . Most
orbit al lym ph om as are low grade. Malign an t lym ph om as can produ ce a palpable
m ass th at m ay be presen t in th e an terior orbit . On e can h ave pain less progressive
proptosis, accom pan ied by vision loss, occasion al diplopia, lid edem a, ptosis, an d
lacrim al glan d involvem en t . A salm on -colored conju n ct ival t um or is ch aracterist ic
(Fig. 3.5A,B,C,D).
B C
Fig. 3.5 (A) Fift y-five -year-old m an with lymphoma. (B) Patient with lymphoma.
(C) Salmon patch in conjunctiva.
Metastasis, react ive lym ph oid hyperplasia, pseudot um or, sarcoidosis
Management
Com pu ted tom ograph ic scan sh ow s a w ell-defin ed m ass, located m ostly in th e an -
terior-su perior lateral orbit , w h ich m olds to en com pass adjacen t st ru ct u res. Th e
lacrim al glan d is frequen tly involved. Ult rason ography sh ow s variable sh ape an d
borders of th e lesion , w h ich h as low to m edium in tern al reflect ivit y.
Radioth erapy (2500 to 3000 cGy) is th e t reat m en t of ch oice for less w ell-
differen t iated lesion s. Ch em oth erapy can also be t ried. A w ell-differen t iated lesion
w ith ou t system ic involvem en t can be obser ved .Visu al progn osis is excellen t if th e
disease is con fin ed to th e orbit .
Other Orbital Neoplasms
Dermoid Cyst
Derm oid cyst is a developm en t al, slow -grow ing ch oristom a (t u m ors w ith h isto-
logically n orm al cells in an abn orm al locat ion ), lin ed w ith st rat ified squam ou s
epith eliu m an d filled w ith kerat in ized m aterial an d/or lipid. Most of th ese cyst s
are located in th e eyelid an d orbit , represen t ing th e single m ost com m on cause
of periorbital n eoplasm in ch ildren . Th ey develop because of sequ est rat ion of th e
su rface ectoderm pin ch ed off at th e bon e su t u re lin es or along th e lin es of em br y-
on ic closure. Th e cyst s are lin ed w ith epiderm is w ith derm al appen dages su ch as
h air follicles an d sebaceous glan ds in th e w all.
Presentation
Derm oids are classified according to th e an atom ical site of presen tat ion :
Superficial derm oids (Fig. 3.6A)
In fron t of th e orbital sept um an d su perotem poral or su peron asal quadran ts
Presen t at ion in in fan cy an d ch ildh ood
Palpable, firm , un ilateral, localized m ass, u sually asym ptom at ic, m ay be m o-
bile or fixed to th e un derlying st ruct ures an d free from th e overlying skin
Deep derm oids (Fig. 3.6B)
Posterior to th e orbital sept um , associated w ith bony sut u res in th e orbit
but m ay exten d across th e bon es in th e fron t al sin us, tem poral fossa, or cra-
n iu m
Presen t in adolescen ce, m ay be seen in ch ildren an d adu lt s
Proptosis, ocu lar displacem en t an d bony defect , m ot ilit y rest rict ion , de-
creased vision . Spon t an eou s rupt u re produces severe orbit al in flam m at ion .
Cavern ou s h em angiom a, m u cocele, opt ic n er ve gliom a, m en ingiom a, n eurilem -
m om a
Management
Superficial derm oids
CT scan of the orbit : Rou n d, w ell-defin ed lesion s w ith en h an cing rim an d a
lucen t a cen ter, w h ich m ay con t ain calcium . Th ere m ay be a w ell-cort icated
bon e defect .
Echography : A w ell-defin ed lesion w ith m edium to h igh in tern al reflect ivit y,
w ith an irregular acoust ic st r uct ure; usu ally sh ow s som e com pressibilit y
Com plete surgical excision : In on e piece
Incom plete excision or capsular rupt ure : May lead to recurren ce w ith in filt ra-
t ion
Deep derm oids
CT scan of the orbit : Well-defin ed lesion s w ith an en h an cing rim . Th ey m ay
con tain areas of calcificat ion . Th e cen t ral lu m en is n on en h an cing, of variable
den sit y, an d m ay sh ow a fluid–fat in terface. Th ere m ay be a bon e defect .
Echography : A cyst ic m ass w ith low to m edium in tern al reflect ivit y is seen .
High er ech oes occu r on ly w h en th e cyst is filled w ith kerat in debris an d fat .
Com plete surgical excision : In on e piece, w ith out ru pt ure of cap su le ( Fig.
3.6C– F).
Capillary Hemangioma
Capillar y h em angiom a is a p rim ar y, u n ilateral, ben ign h am ar tom a of t igh tly
packed cap illaries, ap paren t at birth or w ith in th e first 8 w eeks of life, st raw berr y
red to pu rp le. Most regress com pletely w ith in 7 years of age. Th ey are visible on
th e surface bu t m ay lie deep in th e orbit . It is m ore com m on ly seen in th e supero-
n asal qu adran t of th e u pper eyelid.
Presentation
More com m on in girls. Involvem en t of sup erficial st ru ct u res (derm is) results in
a st raw berr y m ark (st raw berr y n evus), single or m u lt iple, u sually elevated. Su ch
pat ien t s m ay presen t w ith ptosis, som et im es associated w ith ast igm at ism an d
am blyopia. Involvem en t of th e deep par ts (an d an terior orbit) appears as a blu ish
m ass w ith a spongy text u re. W h en th e ch ild cries or st rain s, th e m ass becom es
m ore p rom in en t an d deep en s in color. On exam in at ion , it is a circu m scribed, soft
red m ass w ith a m u lt in odular surface. Large feeding vessels are seen an d can be
th e source of bleeding ( Fig. 3.7 ).
Fig. 3.6 (A) Young girl with a superficial derm oid. (B) A
4-year-old child with a dermoid involving the lateral canthal
area. (C) A 9-year-old girl with a deep dermoid in the lateral
orbit pushing the left eyeball medially.
Fig. 3.6 (Continued) (D) Sagit tal

plane computed tomographic (CT)
scan of a dermoid. (E) Coronal sec-
tion CT scan. (F) Specimen of der-
moid cyst excised, same patient
F specimen.
Fig. 3.7 Eight-month-old boy with a capillary hemangioma.
Nevus flam m eus (darker, does n ot blan ch w ith pressu re), derm oid cyst , en cep h a-
locele, lym ph angiom a, in fect ion , n euroblastom a ( Table 3.3 )
Management
Pat ien t m u st be referred to a pediat rician for w orkup of system ic associat ion (s)
like h igh -out put cardiac failu re, Kasabach -Merrit t syn drom e (an em ia, th rom bo-
cytopen ia, low levels of coagulat ing factors due to th eir sequ est rat ion in th e le-
sion ), or Maffucci syn drom e (en doch ordom atas an d skin h em angiom as). Com -
plete oph th alm ologic exam in at ion m u st be don e to rule ou t poten t ial secon dar y
even t s, n am ely, am blyopia, com pressive opt ic n eu ropathy, an d corn eal exposu re.
Orbit al ult rasoun d suggest s a poorly ou tlin ed lesion , irregu lar in sh ape, h igh in -
tern al reflect ivit y, an d an irregu lar acoust ic st ruct ure w ith variable sou n d at ten u-
at ion . CT scan sh ow s con t rast en h an cem en t an d defin es th e exten t of th e lesion .
Deeper lesion s are w ell defin ed w ith m oderate to in ten se en h an cem en t . On MRI,
th e lesion sh ow s h om ogen eous an d h eterogen eou s sign als, being hypoin ten se on
T1 an d hyperin ten se on T2 w edging. Flow voids appear as hyperin ten se region s.
Gadolin iu m en h an ces th e lesion m oderately.
Obser vat ion is pract iced in m ost of th e cases becau se involu t ion usu ally occu rs
in th e follow ing con dit ion s:
Superficial condit ions: Severe cosm et ic deform it y an d deprivat ion am blyopia
are th e m ajor in dicat ion s of in ter ven t ion . Th e t reat m en t opt ion s in clu de in t ra-
lesion al (40 m g/m L t riam cin olon e plu s 6 m g/m L betam eth ason e) or system ic
(predn isolon e 1 to 2 m g/kg/day) steroid. Oth er opt ion s in clude radioth erapy,
yellow -dye laser, an d topical cort icosteroids. Su rger y sh ou ld be reser ved for
sm all, circum scribed lesion s.
Deep condit ions: Large lesion s or am blyopia u sually w arran t t reat m en t w ith lo-
cal radioth erapy (500 cGy) or system ic or local cor t icosteroids. Surger y m ay be
at tem pted in sm all, circu m scribed lesion s. Progn osis is good for vision an d for
life.
Table 3.3 Vascular Lesions
Capillary Cavernous Orbital

Features Hemangioma Lymphangioma Hemangioma Varices
Disease Benign lid and Benign lid and Benign orbital Ectatic
orbital orbital tum or tumor vascular
hamartoma channels
Onset Soon after Children Adults Young
birth adults
Clinical
features Strawberry Chocolate cysts Axial proptosis Exoph-
nevus and thalm os
thrombo- or
cytopenia enoph-
(Kasabach- thalmos
Merrit t
syndrome)
Ultrasonography High internal Cystic pat tern Well-de ned Calci ca-
x-ray re ectivit y round tion
tumor with
high internal
echoes
Computed Irregular, In ltrative, Late enhance- Enlarged
tomographic poorly multilobulated ment with vessels
scan circum - lesions contrast
scribed
mass
Treatment Observation, Observation, Observation, Conserva-
intralesional steroids, steroids tive
steroids, surgery
system ic
steroids, la-
ser, radiation
Lymphangioma
Lym ph angiom a is a rare vascu lar h am ar tom a of lym ph at ic ch an n els th at is h e-
m odyn am ically isolated from th e vascular system . Occu rring predom in an tly in
ch ildren an d teen agers (m ost frequ en tly in th e first decade of life), th e size of th e
lesion fluct uates w ith post ure an d Valsalva m an euver an d w ith u pper respirator y
t ract in fect ion s.
Presentation
Su perficial lesion s occu r in th e conjun ct iva or lid an d are visible as cyst ic spaces
w ith clear flu id par t ially filled w ith blood. Deep lym ph angiom atous lesion s clas-
sically presen t w ith acute ou t set of pain ful proptosis result ing from spon t an eou s
h em orrh age w ith in th e orbit . Su ch lesion s are called ch ocolate cysts. Th e t u m or
m ass m ay com press th e globe or opt ic n er ve, causing visu al loss, refract ive errors,
secon dar y glau com a, congest ion of th e opt ic n er ve, an d visu al field defects ( Fig.
3.8A,B).
Fig. 3.8 (A) Ten-year-old boy with lymphangioma

involving the lower nasal orbit. (B) Coronal section,
computed tom ographic scan.
Opt ic n er ve gliom a, plexiform n eurofibrom a, capillar y h em angiom a, pseudot u -
m or
Management
Th e orbital lesion is seen as low -den sit y cyst ic, in t racon al an d ext racon al m asses,
w ith variable en h an cem en t on m agn et ic reson an ce im aging. With T1 w edging
th ey are hypoin ten se, w h ereas T2 w edging respon se is variable, dep en ding on th e
state of h em oglobin degen erat ion . Angiography sh ow s n o vascular com pon en t .
Man agem en t of lym ph angiom as is ch allenging. Radiat ion an d system ic steroids
sh ow lim ited sen sit ivit y. Com p lete su rgical excision is ver y difficu lt because of th e
in filt rat ive n at u re of th e t u m or. If acu te h em orrh age cau ses sym ptom s, CO2 laser or
con tact n eodym ium :yt t rium -alu m in um -garn et can be t ried for h om eostasis an d
obliterat ion of t um or as an altern at ive to evacu at ion , p ar t ial resect ion , or ligat ion .
Am blyopia is com m on an d is m ostly from recu rren t h em orrh age or globe com -
pression .
Rhabdomyosarcoma
Rh abdom yosarcom a is th e m ost com m on prim ar y m align an t orbit al t um or in ch il-
dren (70%arise w ith in th e first decade of life). Th is soft t issue m esen chym al t um or
accou n ts for u p to 4% of all ch ildh ood m align an cies. It arises from pleuripoten t
m esen chym al precu rsors th at n orm ally differen t iate in to st riated m uscle cells.
Presentation
Presen tat ion is u sually in th e first decade of life w ith a rapidly progressive pro-
ptosis, m ore com m on ly in boys. It frequ en tly sh ow s a m ass in th e u pper par t of
th e orbit , ptosis, an d eyelid edem a. Th e diagn osis is con firm ed by biopsy. It can
be grouped in to four categories: em br yon al, alveolar, pleom orph ic, an d bot yroid
in th e orbit . Th e m ost com m on h istological varian t is em br yon al follow ed by th e
alveolar t ype ( Fig. 3.9A,B,C,D).
Orbit al cellu lit is, pseu dot u m or, lym ph angiom a, m etastat ic n eu roblastom a, ru p-
t u red derm oid cyst
Management
In th e past , p at ien ts w ith orbit al rh abdom yosarcom as u n der w en t orbital exen -
terat ion . Because of th e m align an t n at ure of th e t um or, it w as th ough t th at radical
resect ion p rovided th e best ch an ce for su r vival. Despite th ese m easures, m ort alit y
rem ain ed as h igh as 70%. Over th e past 30 years, w ith a com bin at ion of surger y,
radiat ion , an d ch em oth erapy, sur vival h as approach ed 90%.
A staging classificat ion w as proposed by th e In tergroup Rh abdom yosarcom a
St u dy grou p in 1972. Com plete resect ion of localized disease is categorized as
group I. In gen eral, m icroscopic residual disease or lym ph n ode involvem en t is cat-
egorized as grou p II. Grou p III in clu des gross residu al disease or in com plete resec-
t ion . Group IV disease in clu des cases w ith m et astasis at presen t at ion . Th e st age of
disease is depen den t n ot on ly on th e exten t of th e t u m or but largely on th e exten t
of resect ion . Th e sam e t u m or can be a group I or II versu s a grou p III depen ding
on w h eth er th e su rgeon perform ed an excision or in cision al biopsy, respect ively.
Th e recom m en ded regim en of radiat ion an d ch em oth erapy is based on th e stage
of disease an d is sum m arized follow ing h ere.
Becau se of rh abdom yosarcom as’ sen sit ivit y to ch em oth erapy an d radiat ion , an
in cision al biopsy follow ed by eith er ch em oth erapy, radiat ion , or both is p referred
by m ost oph th alm ologist s. Th is approach is especially pruden t w ith large t um ors
or t um ors in w h ich an excision al biopsy w ould likely h arm th e opt ic n er ve, ext ra-
ocu lar m uscles, or oth er im port an t orbital st ruct ures. Th e decision is m ore dif-
ficu lt for th ose sm aller, m ore an terior t um ors w h ere com plete excision w ith ou t
en dangering oth er vit al orbit al st ru ct u res is feasible. Most orbit al rh abdom yosar-
Fig. 3.9 (A) Three-year-old boy with rhabdomyosarcoma.

(B) Computed tomographic (CT) scan, sagit tal section.
com as are located su peron asally an d in th e ext racon al space w h ere excision w ould
n ot violate th e opt ic n er ve or ext raocular m u scles. Su ch an app roach m ay perm it
low er doses of radiat ion .
Judiciou s review of th e CT an d MRI scan s is crit ical for surgical plan n ing. In ci-
sion s directly overlying th e t um or are th e preferred approach to biopsy. For exam -
ple, m ore p osterior t um ors are best ap proach ed via a cu tan eou s in cision th rough
th e lid, w h ereas m ore an terior t u m ors th at are visible in th e conju n ct ival forn ices
m ay be ap proach ed via a t ran sforn iceal approach . For excision al biopsies, care
sh ould be taken to con t ain th e t u m or in it s pseudocapsu le an d n ot to violate th e
periosteu m to preser ve th e n at u ral barrier to spread ou tside th e orbit .
Irradiat ion for orbit al rh abdom yosarcom as plays a secon dar y role to ch em oth er-
apy in m an agem en t . Conven t ion al fract ion ated doses totaling 4000 to 5000 cGy
are u su ally su fficien t to con t rol t um or recurren ce. How ever, at th ese doses, ocular
com plicat ion s of orbit al irradiat ion , in clu ding radiat ion ret in op athy, cat aract , dr y
eyes, an d radiat ion -associated keratopathy, are relat ively com m on .
Fig. 3.9 (Continued) ( C) CT scan, coronal section. (D) CT

scan showing involvement of the eyeball.
With a com bin at ion of su rger y, ch em oth erapy, an d radiat ion , it is p ossible to
con t rol th e t u m or an d salvage th e eye in ~90% of cases of orbit al rh abdom yosar-
com a.
Supplem en t al ch em oth erapy h as su bstan t ially im proved su r vival rates of pa-
t ien t s w ith orbit al rh abdom yosarcom as. Pat ien ts w h o u n dergo surger y, radiat ion ,
an d ch em oth erapy for rh abdom yosarcom a in cluding but n ot lim ited to th e orbit
h ave been sh ow n to h ave a 2-year disease-free su r vival rate of 82%, com pared w ith
53% in th ose w h o un dergo su rger y an d radiat ion alon e.
Vin crist in e an d act in om ycin D h ave been th e m ain stays of ch em oth erap eut ic
agen ts em ployed in cases of orbit al rh abdom yosarcom a. New er agen ts such as ifos-
fam ide an d etoposide h ave been sh ow n to produce a favorable respon se.
Cavernous Hemangioma
Cavern ou s h em angiom a is usually seen in m iddle-aged w om en an d is th e m ost
com m on ben ign in t raorbit al t u m or in adult s.
Presentation
Th e t u m or is u n ilateral, solit ar y, an d t ypically located in th e in t racon al area. Pro-
ptosis is of th e axial t ype. Th ere is a predilect ion for m iddle-aged w om en , an d th e
t u m or m ay grow faster during pregn an cy. Th e t u m or m ay be associated w ith opt ic
disk edem a an d ret in al folds (st riae). Th e vision m ay decrease by on e or t w o lin es.
Th ere m ay be rest ricted m ovem en t s in ext rem e fields of gaze (Fig. 3.10A– C).
Oth er in t raorbit al m ass lesion s su ch as derm oid cyst , lym ph om a, sch w an n om a
Management
Com puted tom ograph ic scan con firm s th e diagn osis. Most cavern ou s h em angio-
m as can be obser ved. If su rger y is don e, th e t um or is seen to be a w ell-circum -
scribed, pu rp le, en capsu lated lesion w ith dist in ct vessels on it s surface.
Carotid Cavernous Fistula

Orbit al vascular abn orm alit ies are a grou p of orbit al disorders, congen it al or ac-
qu ired, arising from a variet y of un derlying con dit ion s. Arterioven ous m alform a-
t ion s, h aving feeder vessels from both in tern al an d extern al carot id circulat ion s,
m ostly occur after t rau m a (m ales, 15- to 30-year age range) bu t m ay also arise
spon tan eously (fem ales, 30- to 60-year age range). Carot id cavern ou s fist u las
(CCFs) occur m ostly after basal skull fract ures or pen et rat ing orbit al t rau m a an d
can occu r spon tan eously in person s w ith system ic hyperten sion . Th e h igh -flow
CCFs arise w h en th e in tern al carot id ar ter y develops a defect w ith in th e cavern -
ous sin us, w h ereas low -flow CCFs develop from th e com m u n icat ion bet w een th e
m en ingeal bran ch es of th e in tern al carot id ar ter y an d th e cavern ou s sin u s .
Presentation
Th e pat ien t n ot ices a sw ish ing n oise in th e h ead th at is syn ch ron ou s w ith th e
pulse. Becau se of th e proxim it y of th e ocu lar m otor n er ves to th e cavern ous sin u s,
im paired ocular m ot ilit y an d diplopia are early fin dings. Proptosis, lid an d orbital
edem a, an d dilated an d tor t u ou s conjun ct ival an d episcleral vein s develop later.
Elevated episcleral ven ou s pressu re leads to ocular hyper ten sion .
High-flow CCF: Ch em osis, corkscrew dilatat ion of th e epibulbar vessels, orbital
edem a, proptosis, pu lsat ile exoph th alm os, audible bru it , secon dar y glau com a,
ret in al vascu lar dilatat ion , papilledem a, rapid afferen t papillar y defect , de-
creased vision , occasion al n er ve palsies (III–VI are m ost com m on )
Low -flow CCF: Ch em osis, in creased episcleral ven ous pressure, ven ous dilata-
t ion ( Fig. 3.11A,B,C).
Fig. 3.10 (A) Young adult presenting with proptosis, a case

of cavernous hemangioma. (B) Coronal section computed to-
mographic (CT) scan, cavernous hemangioma. ( C) CT scan,
cavernous hemangioma.
Fig. 3.11 (A) Carotid cavernous fistula with left eye propto-
sis conjunctival congestion. (B) Restricted movements in the
left eye. (C) Computed tomographic scan showing cavernous
sinus involvement. Arrow marks cavernous sinus and also note
prominent superior ophthalmic vein.
Cavern ou s sin u s th rom bosis, pseudot um or, thyroid orbitopathy
Management
Com puted tom ograph ic scan w ith con t rast rem ain s th e in it ial procedu re of ch oice.
It sh ow s dilated su perior oph th alm ic vein w ith en largem en t of th e superior orbital
fissure an d erosion of th e an terior clin oid process. Ult rason ography of th e orbit
sh ow s a dilated superior oph th alm ic vein , m ild th icken ing of th e EOMs, an d m e-
diu m to h igh in tern al reflect ivit y from edem a. MRI w ith gadolin iu m or m agn et ic
reson an ce angiography is a u sefu l tool to invest igate fu rth er, su pplem en t ing th e
CT scan .
Sm all, spon t an eou s, low -flow fist ulas resolve frequen tly (u p to 40%) from
th rom bosis. Em bolizat ion is in dicated on ly w h en vision loss, glaucom a, or severe
pain is presen t . Traum at ic h igh -flow fist ulas rarely resolve on th eir ow n . With a
h igh rate of visual loss in th ese pat ien ts in ter ven t ion becom es th e ru le. Th e cu r-
ren t t ren d involves in ter ven t ion al radiology w ith in t ravascular balloon s or oth er
em bolizat ion via cath eter in th e in tern al carot id arter y.
Orbital Varices
Orbit al varices m ay represen t congen ial foci of an abn orm al vessel (th e m ost com -
m on site is th e upp er n asal qu adran t , an d it is usu ally un ilateral) or m ay be th e late
stage of oth er vascu lar abn orm alit ies.
Presentation
In term it ten t proptosis, w h ich is n onpu lsat ile an d n ot associated w ith bru it . It can
affect pat ien t s from early ch ildh ood to late m iddle age.
Derm oid cyst , lym ph angiom a
Management
Su rger y is difficu lt because th e lesion s are friable an d bleed easily, an d in m ost
cases, excision is incom plete. Indications for surgical intervention include re peated
episodes of pain , th rom bosis, severe proptosis, an d opt ic n er ve com pression . Con -
ser vat ive t reat m en t by CO2 laser, yt t riu m -alum in u m -garn et laser, or cau ter y is rec-
om m en ded. Em bolizat ion is possible if feeder vessels can be iden t ified.
Mucocele
Mu coceles are cyst ic lesion s origin at ing from prim ar y obst ru ct ion of a paran asal
sin us (m ost com m on ly fron t al or eth m oid sin uses) follow ing t rau m a, sin usit is, or,
rarely, a t um or, th at slow ly en large causing bon e deform it y w ith erosion s of th e
orbit . Con sist ing of a cyst ic m ass filled w ith m ucu s, m ucoceles m ay be bou n d by an
eggsh ell layer of bon e an d w h en in fected are referred to as pyoceles.
Presentation
Mu coceles m ost com m on ly arise from th e fron t al an d eth m oidal sin us. Pat ien ts
presen t w ith a com bin at ion of proptosis, a palpable flu ct u an t m ass, h eadach e, dip-
lopia, ptosis, an d epiph ora or globe displacem en t . Sw elling of th e u pper eyelid m e-
dially is com m on . Pain is n ot com m on in th e absen ce of in fect ion (Fig. 3.12A– D).
Derm oid cyst , osteom a, pseudot um or
Management
Com pu ted tom ograph ic scan sh ow s an op acified fron t al or eth m oidal sin u s, loss
of eth m oid septae, an d a bony deh iscen ce. Th e cyst ic con ten t sh ow s variable den -
sit y an d is n on en h an cing. Ult rason ography reveals a ver y w ell-defin ed m ass w ith
sh arp su rface spikes an d low in tern al reflect ivit y. Mucocele is associated w ith a
ver y large bony defect adjacen t to a paran asal sin u s. Treat m en t is su rgical rem oval
of th e cyst lin ing an d reestablish m en t of n orm al drain age. Obliterat ion of th e sin u s
w ith fat or m u scle m ay be n ecessar y to t reat recu rren ces.
Metastatic Orbital Lesions

Th ese lesion s represen t 2 to 10% of all orbital t um ors. In ch ildren , n euroblastom a,
Ew ing sarcom a, an d acute m yeloid leu kem ia are com m on . Th e m ost com m on pri-
m ar y sites in adu lt s are th e breast , bron ch u s, p rostate, skin m elan om a, gast roin -
test in al t ract , an d kidn ey.
Presentation
A m ass in th e an terior orbit cau sing axial or n on axial displacem en t of th e globe is
m ost com m on . In filt rat ion of orbit al t issue ch aracterized by ptosis, diplopia, an d
in durated skin su rroun ding th e orbit is com m on . In flam m ator y react ion is seen . It
m ay be seen presen t ing eith er as proptosis w ith decreased visual acuit y, diplopia,
pain , paresth esia, in creased in t raocular pressure, an d exp osure keratopathy, or in
cases of cicat rizing carcin om as su ch as cer t ain secon daries from th e breast , as en -
oph th alm os ( Fig. 3.13A– D).
Orbit al pseudot um or, rh abdom yosarcom a, leukem ias
Management
Treat m en t is aim ed at preser ving vision an d relieving p ain . Th e m ain opt ion s are
radioth erapy an d h orm on al th erapy (th e lat ter in cases of breast an d prost at ic m e-
t ast asis). Ch em oth erapy is often useful in con t rolling th e system ic disease. A bi-
opsy m ay som et im es be requ ired to est ablish th e n at u re of th e prim ar y. Gen erally,
on ly palliat ive th erapy can be offered.
Fig. 3.12 (A) Orbital abscess from

frontal sinus with pansinusitis. (B)
CT scan axial section showing the
orbital abscess. ( C) CT scan sagit tal
section showing spread of infection
behind the orbital septum . (D) The
patient also has ethmoidal and max-
illary sinusitis.
D
Fig. 3.13 (A) Eight-year-old girl suffering from leukemia.

(B) Computed tomographic scan, leukemia involving the
orbit. (C) Neuroblastoma. (D) Secondaries in orbit.
Lacrimal Gland Enlargement
Lacrimal Gland Inflammation

In flam m ator y cau ses, w h ich are n ot u n com m on , in clude dacr yoaden it is, sarcoid-
osis, an d orbital in flam m ator y pseudot u m or. A decreased Sch irm er test suggest s
an in flam m ator y lesion .
Lacrimal Gland Tumors
Benign Mixed Tumor (Pleomorphic Adenoma)

Th ese are slow ly grow ing lesion s u su ally seen in th e fou r th to fifth decades of life.
Presentation
A long h istor y of m ore th an 1 to 2 years is gen erally obtain ed, an d it u su ally pre-
sen ts as a n on in filt rat ing lesion in th e lacrim al glan d area w ith fulln ess of th e
su perotem poral lid an d orbit an d pain less in feron asal proptosis. Th e upper lid con -
tour m ay t ake an ̴ sh ape ( Fig. 3.14 ).
In flam m ator y lesion s, t u m ors of th e lacrim al glan d, derm oids
Management
Com pu ted tom ograp h ic scan sh ow s a w ell-circum scribed, pseu doen capsu lated le-
sion in th e lacrim al fossa.
Pat ien ts w ith a long-st an ding, pain less, slow ly grow ing m ass w ith a w ell-
circum scribed appearan ce on im aging st u dies are presu m ed to h ave a pleo-
m orph ic aden om a.
Treat m en t is ext irpat ion , con sist ing of a lateral orbitotom y w ith in t racapsular
rem oval of all lesion al t issue w ith carefu l at ten t ion to preven t violat ion of th e
pseu docapsu le.
Fig. 3.14 Mass in upper orbital region in young girl.

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

Aden oid cyst ic carcin om a is th e m ost com m on m align an t lacrim al glan d t u m or,
represen t ing 50%of m align an t t um ors of th e lacrim al glan d an d 25%of all lacrim al
glan d t u m ors.
Presentation
Most cases are seen in th e th ird decade of life w ith a secon d bim odal peak in th e
teen age years. Aden oid cyst ic carcin om as an d oth er m align an cies can also presen t
w ith pain secon dar y to perin eu ral or bony involvem en t . Dip lopia an d dim in ish ed
visu al acuit y can be seen w ith rapidly progressive lesion s. Aden oid cyst ic carci-
n om a u su ally p resen t s as an irregular m ass, produ cing bony erosion (70%) an d
occasion al calcificat ion (20%).
In flam m ator y lesion s, t u m ors of th e lacrim al glan d, derm oids
Management
Aden oid cyst ic carcin om as carr y a poorer progn osis becau se of bony exten sion
an d perin eu ral in filt rat ion . Th ese pat ien t s h ave a 50%at 5-year an d 75%at 15-year
m ort alit y rate. Death is com m on ly due to in t racran ial spread an d pu lm on ar y m e-
tast asis. Histological pat tern is also of progn ost ic sign ifican ce, w ith a cribriform
pat tern h aving a 70% at 5-year sur vival com pared w ith a 20% at 5-year su r vival
w ith a basaloid pat tern . CT scan , along w ith clin ical appearan ce, h elps in preopera-
t ive diagn osis. Treat m en t con sist s of en bloc com plete su rgical excision of th e orbit
an d its con ten t s ( Fig. 3.15A– D).
A B
Fig. 3.15 (A) Exenteration done in a 35-year-old woman with adenoid cystic carcin-
oma. (B) Spectacle prosthesis. (C) Exenteration done in a 35-year-old wom an with ad-
enoid cystic carcinoma. (D) Same patient, spectacle prosthesis.
4 External Diseases
Guillerm o Sim ón-Castellví, Pablo Gili-Man zanaro, Sarabel Sim ón-Castellví,
José María Sim ón-Castellví, Crist ina Sim ón-Castellví, and
José María Sim ón-Tor
Blepharitis and Ocular Rosacea
Anterior blepharitis is a bilateral chronic inflam m atory process of the eyelids, w hich
m ay secondarily result in corneal and conjunctival changes, w ith severe dry eye. It
m ay result in corneal and conjunctival irritation due to the secretion into the eye of
inflam m ator y substances and alteration of the oily layer of the tear film .
St aphylococcal bleph arit is, seborrh eic bleph arit is, an d acn e rosacea w ith lid an d
ocu lar involvem en t are com m on ly foun d in pat ien ts w ith bleph arit is. Hordeolum
an d ch alazion form at ion is also com m on ly seen .
Presentation
Presen tat ion in clu des itch ing, irritat ion , tearing, foreign body sen sat ion , crust ing
on th e lid m argin s, lash loss (m adarosis) or lash m isdirect ion (t rich iasis), u lcer-
at ion of th e lid m argin (t ilosis), red an d th icken ed eyelids, an d ch ron ic conju n ct ivi-
t is. Meibom it is (sebaceou s glan d dysfu n ct ion ) m ay also be presen t (Fig. 4.1A–D).
Infect ious: Fibrin collaret tes on th e lash es
Seborrheic: Seborrh eic derm at it is, tear film in st abilit y
Ocular rosacea : Greasy skin ; facial telangiect asia; er yth em a of th e ch eeks, fore-
h ead; an d n ose; rh in ophym a. Com m on ly, p eriph eral corn eal im m un e in filt rates
(asept ic, du e to staphylococcal t ype IV hypersen sit ivit y)
A B
C D
Fig. 4.1 (A) Fibrin collaret tes; (B,C) lid margin telangiectasia; (D) erythem a of the
cheeks in a patient with ocular rosacea.
100
4 External Diseases 101
Oth er kin ds of conju n ct ivit is (in fect ious, allergic, or toxic), an d dr y eye. Ru le ou t
sebaceou s glan d carcin om a in u n ilateral cases.
Management
Lid hygiene : Com m ercially available ready-for-use lid scru bs or w arm -w ater
soaks w ith dilu ted n eu t ral baby sh am poo in th e m orn ing an d at n igh t , tear
su pplem en t s, an t ibiot ic gels (e.g., fu sidic acid) or oin t m en t (e.g., er yth rom ycin
or bacit racin )
Diet : Vitam in an d om ega-3 su pplem en tat ion , salm on in t ake, olive oil in t ake
Flax oil: Th e best source is grou n d flax seeds so ligin an d fiber are in clu ded
Tapered topical steroids: For severe in flam m at ion or corn eal in filt rates or ph lyc-
ten u les. Dual-act ion topical an t iallergic m edicat ion provides relief (e.g., ketot i-
fen fu m arate 0.025%, azelast in e hydroch loride 0.05%, olopatadin e hydroch lo-
ride 0.1%.)
Oral system ic tet racyclines: For acn e rosacea in adu lts (oral doxycyclin e, 500 m g
1 g/ever y 6 h ours for 1 m on th of t reat m en t)
Abundant art ificial tears and topical lubricants: For dr y-eye sym ptom s (e.g.,
topical sodium hyalu ron ate 0.18%)
Treat m en t is t apered according to sym ptom s
Conjunctivitis
Any in flam m at ion of th e conju n ct iva is referred to as conjun ct ivit is.
Adenoviral Conjunctivitis (Epidemic Keratoconjunctivitis,

Pharyngoconjunctival Fever)
Adenoviral infections predom inate in sum m er m onths. Most textbooks refer to vi-
ral conjunctivitis as infections produced by adenoviruses (epidem ic conjunctivitis,
adenoviral conjunctivitis). But different viruses are responsible for different t ypes of
conjunctivitis. Picornavirus (m ainly enterovirus 70 and Coxsackievirus A24) are re-
sponsible for acute hem orrhagic conjunctivitis, w hich is clinically sim ilar to adeno-
viral conjunctivitis but m ore severe. It is highly contagious and occurs in epidem ics.
Epidem ic keratoconjunct ivit is: Cau sed by aden oviru ses 8 an d 19 w ith ch aracter-
ist ic preauricu lar lym ph n ode, p h ar yngit is, an d subepith elial in filt rates 5 to 10
days after th e in it ial sym ptom s
Pharyngoconjunct ival fever: Cau sed by aden oviru ses 3 an d 7, w ith fever an d
ph ar yngit is; associated w ith pu blic sw im m ing pools in sum m er
Presentation
Classic sign s in clu de red eye (u n ilateral or bilateral), ciliar y inject ion (m ild irit is),
an d epip h ora. In cases of aden oviral conjun ct ivit is, th e p at ien t refers to recen t ex-
posure to an in dividual w ith red eyes at h om e, sch ool, or w ork or h as a h istor y of
recen t sym ptom s of an upper resp irator y t ract in fect ion . Th e in cu bat ion period
is 5 to 12 days. Acute follicu lar conju n ct ival react ion m ain ly in th e in ferior tarsal
conju n ct iva, ch em osis, preauricu lar aden opathy, su bconjun ct ival petech iae (ver y
sm all h em orrh ages), an d som et im es early, n on specific m ild pu n ctate keratopathy
m ay be seen . Su bepith elial in filt rates develop 5 to 12 days after th e in it ial sym p-
tom s (suggest aden oviru s serot ypes 8 an d 19). Corn eal opacit ies can persist for a
few w eeks to m on th s (w e h ave seen u p to 2 years). Th ey can decrease visu al acuit y
an d cau se glare sym ptom s. Epith elial ulcerat ion (par t ial or total) m ay occu r. Eyelid
edem a an d su b-conju n ct ival h em orrh age suggest s acute h em orrh agic conju n ct i-
vit is. In severe cases, m em bran es an d pseu dom em bran es can lead to conju n ct ival
scarring an d sym bleph aron .
Sym ptom s in clude ph otoph obia an d eye pain if th ere is corn eal involvem en t
(aden oviru s), in ten se w ater y disch arge (serou s), an d itchy eye(s). It is usu ally be-
n ign an d self-lim ited an d gen erally h as a longer cou rse th an acute bacterial con -
jun ct ivit is, last ing for ~2 to 4 w eeks ( Fig. 4.2A,B).
Oth er epidem ic keratoconjun ct ivit is, h erpes sim plex in fect ion , h erpes zoster in -
fect ion , in fect iou s m on on u cleosis (w ith eye involvem en t), Epstein -Barr virus in -
fect ion , Dim m er kerat it is, bru cellosis
Management
Most cases are self-lim ited, w ith n o m orbidit y, an d requ ire n o specific t reat m en t .
High ly con tagious cases requ ire st rict hygien e m easu res. In st ru ct you r pat ien t th at
Fig. 4.2 (A, B) Adenoviral conjunctivitis.

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

(Epidemic Hemorrhagic Keratoconjunctivitis)
Th is t ype of conjun ct ivit is is also kn ow n as Apollo 11 conju n ct ivit is an d is due
to picorn aviru s (coxsackieviru s A24 or en teroviru s group 70). Acute h em orrh agic
conju n ct ivit is affect s m ostly ch ildren an d young adult s in th e low er socioecon om ic
classes.
Presentation
It begin s w ith an in it ial period of catarrh al in flam m at ion , follow ed, in a day or t w o,
by th e appearan ce of conju n ct ival petech iae th at coalesce to form subconju n ct ival
h em orrh ages. Th e explosive on set is a pain ful, rapidly progressive follicu lar con -
jun ct ivit is. It is self-lim ited in a couple of w eeks an d star ts w ith su bconjun ct ival
diffuse h em orrh age, m ore frequ en t in th e upper bulbar, an d sym ptom s of viral
conju n ct ivit is su ch as p reau ricu lar lym ph at ic n ode an d an terior segm en t in flam -
m at ion togeth er w ith flu like sym ptom s. Th ere is periorbital pain . Th e lids often
becom e sw ollen an d in durate (lid edem a). Ch em osis, serom u cou s disch arge, ph o-
toph obia, an d tearing are also seen ( Fig. 4.3 ).
Aden oviral or bacterial in fect ion , subconju n ct ival h em orrh age, kerat it is
Fig. 4.3 Acute hemorrhagic conjunctivitis.

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

Most textbooks refer to viral conjun ct ivit is as th at produced by aden oviruses (epi-
dem ic conjun ct ivit is, aden oviral conjun ct ivit is), bu t differen t viruses are respon -
sible for differen t t ypes of conju n ct ivit is or keratoconju n ct ivit is. Herpes sim plex
virus (HSV) is th e m ost dangerous cause, especially HSV t ype 1 (m ou th , gen it al).
HSV t ype 2 m ay also be a cau se, esp ecially in ch ildren an d n eon ates. HSV affects a
variet y of ocular t issu es an d m ay cau se ocular derm at it is, epith elial an d st rom al
kerat it is, an d even iridocyclit is ( Fig. 4.4A,B).
Presentation
Red pain fu l eye (un ilateral or bilateral, usually u n ilateral), ph otoph obia-epiph ora,
acute follicular conjun ct ival react ion m ain ly in th e in ferior tarsal conju n ct iva,
Fig. 4.4 (A) Herpetic dendritic cor-

B neal ulcer. (B) Associated skin lesions.
ch em osis, som et im es early n on specific m ild pu n ct ate keratopathy, classic den -

drite-like corn eal u lcerat ion w ith term in al bulbs. Flu orescein stain s th e u lcer base,
w h ereas rose bengal st ain s th e u lcer edges. Usually occu rs in sexu ally act ive adu lt s.
In cases left u n t reated or in late diagn osis or in m u n ocom prom ised pat ien t s it can
lead to disciform kerat it is, in terst it ial kerat it is, an d iridocyclit is.
Epidem ic keratoconjunctivitis, varicella-zoster herpetic infection, corneal abrasion
Management
Ophthalm ic ganciclovir gel (preferred) or acyclovir ointm ent (five tim es daily, 7 to 10
days is usually enough). Ganciclovir gel is m uch less toxic for the corneal epithelium
than acyclovir. Use in children m ay not be approved for herpes sim plex in som e
countries. Alternatives include 3%vidarabine ointm ent (five tim es daily, until reep-
ithelialization or 7 days), 1% trifluridine drops (1 drop every 2 hours, m axim um 9
drops), or 0.5%idoxuridine drops (one drop, five tim es per day, 7 days).
Epithelial debridem ent and patching: Use gan ciclovir an d an t ibiot ic oin t m en t .
Add topical antibiotic drops, gel, or ointm ent to avoid superinfection : Dark glasses,
antihistam ines (e.g., levocabastine every 8 to 12 hours) and vasoconstricting drops
provide relief. Oral fam ciclovir reduces duration and the risk of recurrence.
Concom itant use of topical ganciclovir, steroids, and oral fam ciclovir: Required for
disciform kerat it is, in terst it ial kerat it is, an d iridocyclit is. In th e case of iridocy-
clit is, prescribe cycloplegia.
Acute Bacterial Conjunctivitis

Becau se of th e excellen t defen se system s of th e eye, acute bacterial conju n ct ivit is
is un com m on , bu t it is st ill th e m ost com m on eye disease. In m ost cases self-lim -
ited an d ben ign , it is ch aracterized by th e presen ce of abun dan t m ucopu rulen t
disch arge in a pat ien t w ith red eye. Th ere is con siderable overlap in th e presen t ing
fin dings from differen t bacteria. On ly exper t clin ician s m ay be able to recogn ize
th e probable in fect ive agen t at a clin ical level.
Epidem iology /t ransm ission : In fect ious for th e first 48 h ours of t reat m en t
Presentation
Signs: Redn ess w ith variable conju n ct ival inject ion (hyperem ia), palpebral
conju n ct iva being m ore affected th an bu lbar, lid sw elling (m arked lid edem a
ch aracterist ic of Haem ophilus influenzae an d Corynebacterium diphtheriae ),
m em bran es th at are com m on ly seen w ith St reptococcus pyogenes an d Coryne-
bacterium diphtheriae , an d ch em osis (bulbar conju n ct iva an d forn iceal). Th ere
is u su ally n o preauricu lar aden opathy or skin involvem en t , th e corn ea is usu ally
clear (possible corn eal ulcerat ion if un t reated), an d th e pupil reacts n orm ally.
Sym ptom s: Unilateral, sudden onset of red eye w ith significant irritation and
foreign body or grit t y sensation (no outstanding pain), w hich progresses to the
other eye in 2 to 5 days. It is usual for sym ptom s to be present for several days
or w eeks at the tim e of presentation. An uncom m only long duration or frequent
recurrences suggest that other factors or conditions m ay be present (e.g., chronic
dacryocystitis, urethritis). The patient has sticky lids or m at ting of eyelashes, es-
pecially in the m orning, w ith serom ucoid (at the beginning) or m ucopurulent co-
pious grayish, yellow, or green discharge (later). W hite discharge is due to abun-
dant m ucus and suggests allergic reaction. Visual acuit y is preser ved except for
the expected m ild blur due to the discharge and debris in the tear film . Consider
gonococcal conjunctivitis if there is excessive purulent discharge.
A B
Fig. 4.5 (A) Conjunctival congestion.

(B) Mucopurulent discharge. (C) Con-
C junctival congestion and lid edem a.
Com plicat ions: Staphylococcus aureus blep h arit is is seen in ch ron ic bacterial
conju n ct ivit is an d extern al h ordeolu m (st ye). Gon ococcu s can pen et rate th e
in t act corn eal epith eliu m leading to perforat ion ( Fig. 4.5A– C).
Any cause of red eye (e.g., viral conju n ct ivit is, allergic conju n ct ivit is, any kerat it is,
uveit is, acu te angle closure glau com a)
Management
Lid cleansing: It is im por tan t to keep th e lids clean an d rem ove all disch arge.
Broad-spect rum topical ant ibiot ic: Most cases of bacterial conju n ct ivit is clear
in 48 to 72 h ou rs w ith a low dose of any broad-spect rum an t ibiot ic applied
topically. Con t in ue t reat m en t for at least 7 days. High ly respon sive to em pirical
t reat m en t (e.g., tobram ycin , n orfloxacin , levofloxacin ). Tw o drops in affected
eyes, ever y 3 to 4 h ours, for 1 w eek.
Oint m ents and gels: Ver y usefu l w ith ch ildren (day an d n igh t) or for overn igh t
use in adult s
Cult ures: Con sider for cases refractor y to topical t reat m en t
Chlamydia Trachoma
Ch lam ydial organ ism s are respon sible for n eon at al conju n ct ivit is, adu lt in clu sion
conju n ct ivit is, t rach om a, an d lym ph ogran ulom a ven ereu m (Nicolas-Favre disease,
in t ropical region s, rarely affects th e eye). Ch lam ydial (in clusion ) conju n ct ivit is
t ypically affects sexu ally act ive teen s an d you ng adult s, an d Chlam ydia is th e m ost
frequ en t in fect iou s cause of n eon atal conju n ct ivit is in th e Un ited States. Adult in -
clusion conju n ct ivit is, also called parat rach om a, is du e to Chlam ydia t rachom at is
serot ypes D to K, w h ereas t ru e t rach om a is du e to Chlam ydia t rachom at is sero-
t ypes A to C ( Fig. 4.6A,B).
Presentation ( Table 4.1 )

A B
Fig. 4.6 (A) Trachom a, cicatricial ptosis, lid everted following trachoma. (B) Trachom a
corneal ulceration.
Table 4.1 Presentatio n o f Chlam ydia Tracho m a
Adult Inclusio n
Co njunctivitis
(Paratracho m a o r
Oculo genital
Syndro m e) Tracho m a
Transmission Oculogenital (venereal) Ocular

Epidemiology Sporadic Endem ic
Flies and other fomites ease spreading
Endem ic region Developed countries Underdeveloped countries (Africa,
(Europe, United States) Asia, Middle East); hot, dry climates
Reservoir Eye and nose Eye and nose
Genital tract in males
and females
Age of infection Young adults (men and Children (100% are infected in
women, 15–30 years) endemic areas before age 2)
Sexually active
Incubation 8–10 days 5–8 days
period 6 days in children
Presentation Acute or chronic Acute or chronic
Lateralit y Uni-/bilateral (may be Almost always bilateral
asymmetric)
First signs Follicular conjunctivitis Red eye and mucopurulent discharge
Mucopurulent discharge (acute or chronic)
Follicular conjunctivitis (superior tarsal
follicles and papillae)
Very slow evolution (in years)
No membranes
Conjunctival injection, punctate
keratitis, superior corneal pannus,
follicles (most dense in the inferior
cul-de -sac) may be present
A palpable preauricular node is almost
always present (prominent
lymphoid reaction)
(Continued on page 108)
Table 4.1 (Con t in u ed) Presen tatio n o f Chlam ydia Tracho m a
Adult Inclusio n
Co njunctivitis
(Paratracho m a o r
Oculo genital
Syndro m e) Tracho m a
Ocular signs and symptoms include

the chief complaint that an eye
infection has persisted longer than
3 weeks despite treatm ent with
topical antibiotics
Complications No scarring of the Conjunctival scarring (limbal Herbert
conjunctiva pits, von Arlt lines in superior
and sometim es inferior tarsal
conjunctiva)
Epithelial super cial keratopathy, pannus:
super cial corneal vascularization
Dry eye
Bacterial superinfection
Blindness
Course Weeks (may have Chronic (years)
acute start) May have acute start
Rule out other venereal
infections (gonorrhea
and syphilis)
Prognosis Good Poor in chronic untreated cases or
reinfection (common)
Reinfection with other pathogens is
frequent
Management
Salin e rin sing clears th e m u copur ulen t debris from th e lids an d conjun ct iva.
Azithromycin (Zithrom ax) 1 g by m outh is the drug of choice. One dose has been
docum ented as being as effective as doxycycline for the treatm ent of genital chla-
mydial infection. For greater safety we prescribe 500 m g, once every day for 3 days.
Altern at ives:
Oral am oxicillin or er yth rom ycin 250 to 500 m g, orally, fou r t im es daily, for 3
or 4 w eeks
Oral doxycyclin e 100 m g, orally, t w ice daily, for 1 w eek (be aler t for p reg-
n an cy or lactat ion )
Oral tet racyclin e 250 to 500 m g, orally, fou r t im es daily, for 1 w eek (con t rain -
dicated in pregn an t or lact at ing w om en an d ch ildren u n der age 8)
Topical antibiotics are relatively ineffective (but useful). Topical therapy is ad-
junctive but not essential: rifam picin (drops or ointm ent), or erythromycin oint-
m ent (drug of choice for pregnant wom en), or tetracycline drops or ointm ent,
three tim es daily, for 7 days. We always prescribe adjunctive topical therapy.
In ch ildren u se oral er yth rom ycin su spen sion , 40 m g/kg/day, in fou r divided
doses for 2 w eeks.
In pregn an t w om en use er yth rom ycin 250 to 500 m g, orally, fou r t im es daily,
for 3 w eeks.
Topical flu oroquin olon e (ofloxacin or ciprofloxacin ) if th ere is a corn eal in -
fect ion
Topical steroids can give tem porar y relief (st art a few days after topical an t i-
biot ics).
Treat sexual partners. A condom does not protect against chlamydial conjunctivitis.
Chlamydia/Adult Inclusion Conjunctivitis

Th is con dit ion is sexually t ran sm it ted an d t ypically foun d in young adu lts. It pres-
en ts as ch ron ic follicular conju n ct ivit is cau sed by Chlam ydia t rachom at is serot ypes
D to K. A h istor y of vagin it is, cer vicit is, or u reth rit is m ay or m ay n ot be presen t .
Presentation
Presen tat ion in clu des ch ron ic, bilateral, red, an d irrit ated eye. In ferior tarsal or
bulbar conjun ct ival follicles, su perior corn eal pan n us, palpable p reau ricu lar n ode,
or t iny, gray-w h ite periph eral subepith elial in filt rates m ay be presen t . A st ringlike,
m u cous disch arge is t ypical.
All oth er form s of ch ron ic conjun ct ivit is
Management
Diagn ost ic test s in clu de slit-lam p exam in at ion , direct ch lam ydial im m u n o-
flourescen ce test , or Chlam ydia cu lt ure of th e conju n ct iva. Conju n ct ival scrap-
ing w ith Giem sa st ain sh ow s basoph ilic in t racytop lasm ic in clusion bodies in
epith elial cells, polym orph on u clear leukocytes, an d lym ph ocytes in n ew born s.
Azith rom ycin 1 g by m ou th single dose, doxycyclin e 100 m g by m outh t w ice a
day, or er yth rom ycin 500 m g by m outh four t im es a day for 7 days is given to th e
pat ien t an d sexual part n ers.
Topical er yth rom ycin an d tet racyclin e t w o to th ree t im es a day for 2 to 3 w eeks
are oth er t reat m en t opt ion s.
Chronic Conjunctivitis and Recurrent Corneal Abrasions

Ch ron ic conju n ct ivit is an d recurren t corn eal abrasion s m ay h ave th eir origin s in
a lid disease (e.g., bleph arit is), a lid m alp osit ion (e.g., en t ropion ), or a lacrim al
w ay dysfu n ct ion (e.g., dacr yocyst it is). Left un t reated, th ese con dit ion s can resu lt
in severe dam age to th e ocu lar su rface as a resu lt of differen t corn eal an d con -
jun ct ival com plicat ion s. Determ in ing th e cause is essen t ial to in dicate adequ ate
t reat m en t .
Presentation
Sym ptom s in clu de recu rren t or ch ron ic conjun ct ivit is (red eye, disch arge, etc.),
spasm odic inversion of th e eyelid m argin (spasm odic en t ropion ), foreign body
sen sat ion , ocu lar su rface irrit at ion , corn eal abrasion s, an d scars. In advan ced cases
th ere can be vision problem s (ast igm at ism , leu kom as in th e visu al axis), rest ric-
Fig. 4.7 Spasmodic entro-

pion with corneal ulceration
sequelae.
t ion of ocu lar m ot ilit y (pseudopter ygiu m , sym bleph aron ), an d blin dn ess (in un -
derdeveloped coun t ries w h ere su rger y is un available) ( Fig. 4.7 ).
Trich iasis, dist ich iasis, dacr yocyst it is, t rach om a, ch em ical bu rn s, au toim m u n e dis-
orders (e.g., pem ph igoid)
Management
Treat m en t varies w ith th e cause of recu rren t conju n ct ivit is. Un t il su rger y can be
perform ed, bot u lin u m toxin inject ion s provide im m ediate tem porar y relief of
spast ic en t ropion by w eaken ing th e pretarsal orbicularis oculi m u scle. Dacr yocys-
torrh in ostom y is perform ed in com plete obst ru ct ion of lacrim al p ath w ays. Treat
com plicat ion s w h en n ecessar y (e.g., topical an t ibiot ic drops an d oin t m en t s for in -
fect ious conjun ct ivit is). Provide ext raocular lubricat ion w ith gels an d oin t m en t s to
reduce th e risk of abrasion s.
Giant Papillary Conjunctivitis

Papillae m ay be presen t in an oth er w ise h ealthy person . Papillae are gen erally
presen t in th e sup erior lid t arsal conju n ct iva, m ost often papillae are due to an in -
ten se allergic respon se, eith er con t act (con t act len s u sers, as part of a Gell- Coom bs
t ype 1 im m u n oglobulin E–m ediated hypersen sit ivit y react ion ) or spring allergy
(vern al keratoconju n ct ivit is). Gian t pap illar y conju n ct ivit is is a ch ron ic reversible
in flam m ator y con dit ion . In th e presen ce of gian t papillae con sider th e follow ing:
Vern al conjun ct ivit is
Atopic keratoconju n ct ivit is
Gian t papillar y conjun ct ivit is of con tact len ses
Gian t papillae of prosth eses, corn eal scleral sh ields
En ds of nylon sut ures, cyan oacr ylate glue, ext ruded scleral buckles
Presentation
Pat ien ts presen t w ith itch ing, red eyes, foreign body sen sat ion , m ucou s disch arge,
an d th e presen ce of gian t papillae at slit-lam p exam in at ion (cobbleston e aspect),
usually in prosth eses or con tact len ses w earers (associated w ith u se of all t ypes
Fig. 4.8 Giant papillae. Vernal conjunctivitis.
of con tact len ses—h ard, hydrogel, scleral, prosth et ic, etc.). Th e con dit ion can be
m ore aggressive in ch ildren , w ith lid sw elling an d pseudoptosis of th e su perior
lid ( Fig. 4.8 ).
Atopic conju n ct ivit is, vern al conju n ct ivit is
Management
Discon t in ue con t act len s or prosth esis w ear for at least 3 to 4 w eeks. Most pat ien ts
do n ot require m ore aggressive t reat m en t . Topical m ast cell–st abilizing solu t ion s,
an t ih istam in es, an d top ical steroids (drops an d oin t m en t s) can be used. Excep-
t ion ally, gian t papillae m ay requ ire steroid depot inject ion at th e t arsu s. Silver n i-
t rate or radiosu rger y su rgical curet tage of large/gian t papillae m ay be useful in
som e cases (e.g., corn eal ulcerat ion ). Plasm aph eresis h as been su ccessfully u sed
as adjun ct th erapy for pat ien ts w ith h igh im m u n oglobu lin E levels (e.g., vern al
conju n ct ivit is). En cou raging th e pat ien t to replace con tact len ses frequen tly, u se
preser vat ive-free len s solut ion , an d in crease len s hygien e all are good preven t ive
m easu res.
Cicatrizing Disorders
Ocular Cicatricial Pemphigoid

Cicat ricial pem ph igoid is an autoim m u n e disorder of un kn ow n et iology, w h ere
circulat ing an t ibodies an d com plem en t bin d to th e basem en t m em bran e of m uco-
sal t issues. It is a ch ron ic, bilateral, papillar y cicat rizing conjun ct ivit is ( Fig. 4.9 ).
Fig. 4.9 Benign pemphigoid.
Presentation
Age of presentat ion : Aged adu lts (rarely seen in ch ildren )
Sex : Wom en are affected t w ice as frequ en tly as m en .
Main early feat ure : Ch ron ic bilateral papillar y cicat rizing conju n ct ivit is
Ocular findings:
Conju n ct ival hyp erem ia w ith dr y eye sym ptom s, tearing, itch ing, ph otoph o-
bia, foreign body sen sat ion s
Disch arge (i.e., catarrh al, m ucou s, m em bran ou s)
Eyelids m an ifest t rich iasis, dist ich iasis, m eibom ian glan d dysfun ct ion , bleph -
arit is
Conju n ct iva m an ifest s pap illae, follicles, kerat in izat ion , su bepith elial fibro-
sis, conju n ct ival kerat in izat ion w ith foresh orten ing of th e forn ices an d sym -
bleph aron an d/or an kylobleph aron (en d stage, im m obilizes th e eye)
Corn ea m an ifests su perficial pu n ctate kerat it is, epith elial defect , u lcerat ion s,
n eovascu larizat ion , kerat in izat ion , st rom al opacit ies, perforat ion (pseu do-
t rach om a)
Chlamydial infections (trachom a, inclusion conjunctivitis), atopic keratoconjunctivi-
tis, adenoviral conjunctivitis, long-term ocular m edication (old antiglaucom a m edi-
cations, e.g., epinephrine), chem ical (alkali) or therm al burns, radiation exposure
(therapeutic or not), Sjögren syndrom e, sarcoidosis, derm atobullous disorders (toxic
epiderm al necrolysis, congenital ichthyosiform erythroderm a, epiderm olysis bullosa),
erythem a m ultiform e, Corynebacterium diphtheriae conjunctivitis, epitheliom a
Management
No topical agen t is really effect ive in stop ping act ivit y (dexam eth ason e oin t-
m en t m ay h elp). Best con t rolled w ith system ic th erapy.
To slow disease p rogression t r y th e follow ing:
Subconjunctival steroid injections such as triam cinolone (Trigon, Bristol-Myers
Squibb, New York) or betam ethasone (Celestone, Schering Corp., Kenilw orth,
NJ). We use prolonged-release betam ethasone, injecting as m uch as possible,
subconjunctivally (Celestone-Cronodose, Schering Corp., Kenilw orth, NJ the
strongest “depot” steroid).
Abu n dan t ar t ificial tears an d topical lu brican ts in pat ien t s w ith dr y eye
sym ptom s
Steroid oin t m en t s (e.g., dexam eth ason e oin t m en t , t w o to four t im es daily)
System ic im m u n osu ppressive th erapy, u su ally con t in ued for 9 to 12 m on th s.
Mu st be con t rolled by a specialist in im m u n osup pressive th erapy (e.g., di-
am in odiph enylsu lfon e or cycloph osph am ide + system ic predn ison e)
System ic cor t icosteroids for severely in flam ed eyes th at do n ot respon d to
im m u n osu ppression alon e
Treat com plicat ion s su ch as epilat ion of an aberran t lash (m ech an ical, laser,
cr yodest ru ct ion ), pu n ctal occlu sion for dr y eye, or lid su rger y for en t ropion .
Stevens-Johnson Syndrome
Stevens-Johnson syndrom e is an acute hypersensitivity reaction consisting of an inflam -
m atory vesiculobullous reaction of the skin and m ucous m em brane. Im m une complex
deposition is incited by m edications, including sulfonam ides, anticonvulsants, aspirin,
penicillin, isoniazid, diam ox (acetazolam ide), and m any m ore, and som etim es infec-
tious organism s (herpes simplex virus, streptococci, adenovirus, mycoplasm a).
Presentation
Th ere is an acu te on set of fever, rash , red eyes, m alaise, ar th ralgias, an d respirator y
t ract sym ptom s. Classic “t arget” skin lesion s (m aculop apu les w ith a red cen ter an d
a w h ite surroun d on an er yth em atous base) are con cen t rated on th e h an ds an d
feet . Oth er sym ptom s in clu de u lcerat ive stom at it is an d h em orrh agic lip cru st ing.
Th e m or talit y rate is 10 to 33%.
Ocu lar sign s in clu de m ucopu ru len t or pseu dom em bran ou s conjun ct ivit is,
episclerit is, an d irit is in th e acu te ph ase. Late com plicat ion s in clu de conju n ct ival
scarring or sym bleph aron , t rich iasis, eyelid deform it ies, tear deficien cy, corn eal
n eovascularizat ion , u lcer, perforat ion , or scarring.
Erythem a m ult iform e m ajor (Stevens-Johnson syndrom e) : Im m u n e com plex de-
posit ion in th e derm is w ith su bepith elial vesiculobu llou s react ion of th e skin
an d m u cou s m em bran es
Erythem a m ult iform e m inor: On ly skin involvem en t
Toxic epiderm al necrolysis: Th e m ost severe form , causing exten sive in t raepith e-
lial vesicu lobu llou s erupt ion s an d epiderm al n ecrosis; m ore com m on in ch il-
dren an d im m un osu ppressed pat ien ts
Ocular cicatricial pem phigoid, chem ical burns, radiation, squam ous cell carcinom a
Management
Taking a h istor y to r ule out a precip itat ing factor is im por tan t . Slit-lam p exam in a-
t ion , in clu ding eyelid eversion w ith exam in at ion of th e forn ices, conjun ct ival or
corn eal cu lt ures, an d con su ltat ion w ith in tern al m edicin e, is a m u st . Hospit alize
th e pat ien t , rem ove th e in it iat ing factor, an d provide sup por t ive care as th e m ain -
stays of t reat m en t .
Ocu lar m an agem en t in cludes m an agem en t of associated dr y eye, irit is, topical
steroids for ocular surface in flam m at ion , daily pseu dom em bran e peel, an d sym -
bleph aron lysis w ith a glass rod or m oisten ed cot ton sw ab, system ic or topical
vitam in A, an d in t raven ous im m u n oglobulin . To m an age th e late com plicat ion s
pen et rat ing keratop last y w ith stem cell t ran splan t , an d am n iot ic m em bran e t ran s-
plan t , or perm an en t keratoprosth esis m ay be requ ired.
Dry-Eye Syndrome
Most dr y eyes are m u lt ifactorial. Th e eye can be dr y as a result of defect ive produc-
t ion of tears (e.g., age-related dr y eye in m en opau sal w om en ) or excessive evapora-
t ion (e.g., exoph th alm os).
Presentation
Sym ptom s: Foreign body sen sat ion , sen sat ion of ocu lar dr yn ess an d grit t in ess
(in it ially at th e en d of th e day an d later th rough out th e w h ole day), hyperem ia
an d ocu lar irrit at ion (exacerbated by sm oky or dr y environ m en t s, in door h eat-
ing system s, prolonged reading, or u se of com puters), m u cous disch arge, exces-
sive tearing (secon dar y to reflex secret ion )
Signs: Red eyes, conju n ct ival hyperem ia, decreased tear m en iscus, in creased
debris in th e tear film , su perficial pu n ctate keratopathy (w ith flu orescein , lis-
sam in e green , an d/or rose bengal posit ive st ain ing), m u cous plaques an d dis-
ch arge, corn eal filam en t s (severe cases), corn eal epith elial defect s or ulcers (in
m ore severe cases) ( Fig. 4.10 ).
Any conjun ct ivit is (especially toxic form s, w h ich are m ore difficult to differen t i-
ate). Careful h istor y an d w orku p sh ou ld h elp to establish th e origin of th e dr y eye
(e.g., air con dit ion ing, Sjögren syn drom e)
Management
Tru e dr y eye can n ot be cu red, bu t eye sen sit ivit y can be lessen ed an d m easu res
taken so th e eyes rem ain h ealthy by m ean s of art ificial tears or tear su bst it u tes.
No con t act len s pat ien t or glau com a t rabeculectom ized pat ien t sh ould be out of
ar t ificial tear lu bricat ion or tear subst it utes!
First step : Sup plem en tal lubricat ion (m ild an d m oderate kerat it is sicca)
Art ificial tears: Preferably preser vat ive-free ar t ificial tears (drops 4 to 14
t im es a day, depen ding on th e severit y of th e case). Our preferred ocu lar lu -
brican t is sodiu m hyaluron ate (0.18 to 0.4%).
Fig. 4.10 Dry eye, lagophthalm os, corneal and conjunctival

rose bengal staining.
Viscous artificial tear drops or gels: How ever, they tem porarily blur the vision.
Lubricat ing oint m ents: For m ore severe cases (gen erally reser ved to bedt im e,
becau se vision blur lasts m in utes or h ours). Not to be used w ith con t act
len ses.
In severe cases: Use a patch w ith lubricat ing oin t m en t at n igh t .
In case of abundant m ucus: Rem ove th e m ucou s an d add 10% N-acet ylcyste-
in e, th ree to fou r t im es daily.
Art ificial tear insert (e.g., Lacrisert, Aton Pharm a, Inc., Law renceville, NJ) : Place
in to th e in ferior cu l-de-sac ever y m orn ing.
Special goggles, m oist ure cham ber glasses: To redu ce evap orat ion an d ret ain
h um idit y aroun d th e globe.
In case of suspected inflam m at ion or in case of unsat isfactory results : Tr y topi-
cal steroids (before cyclosporin e).
Treat any associated abnorm alities: (e.g., in blepharitis, suppress inflam m ation
w ith topical steroids and local antibiotics, and/or system ic tetracyclines)
Interm ediate step :
Tem porary punctal occlusion : With collagen (dissolvable) or silicon e (perm a-
n en t) plugs in case of severe aqueou s tear deficien cy (to preser ve en dog-
en ou s w ater)
In-office cauterizat ion of the inferior lacrim al punct i: If th e pat ien t is sat isfied
w ith tem poral occlusion result s
Minim ize exposure: Consider tarsorrhaphy or botulinum -toxin-induced ptosis.
Last step : Su rgical t reat m en t (on ly for ver y severe cases, w ith u lcerat ion or cor-
n eal perforat ion )
Closure of perforat ion or descem etocele : Cyan oacr ylate t issu e adh esive
Corneal or corneoscleral patch : For an im pen ding or fran k perforat ion (am n i-
ot ic m em bran e, fascia lat a)
Lateral tem porary tarsorrhaphy : For exam ple, after facial n er ve paralysis,
after t rigem in al n er ve lesion s, or for severe exoph th alm os secon dar y to thy-
roid disease
Conjunct ival flap: Aids in preven t ing corn eal m elt an d perforat ion by cover-
ing th e corn ea w ith conjun ct iva
Several variet ies of con tact len ses can aid in th e t reat m en t of dr y eye. Hard con -
tacts m ay st im u late reflex tearing an d th u s in crease th e volu m e of tears. Som e
h ard scleral con t act len ses m ay be ben eficial by preven t ing evap orat ion from a
large por t ion of th e ocular su rface. Th e U.S. Food an d Drug Adm in ist rat ion h as ap-
proved on e t ype of con tact len s (Proclear Toric XR len s, CooperVision , Fairpor t , NY)
w ith an in dicat ion for dr y eyes. Th is is a soft len s th at h as som e u n iqu e proper t ies.
It has a h igh w ater con ten t like oth er soft len ses, bu t it also h as a com pon en t th at
retain s w ater bet ter th an m ost oth er soft len ses. Th is reduces th e dehydrat ion th at
occu rs w ith m ost soft con tact len ses. Clin ical st u dies h ave dem on st rated im prove-
m en t in com fort an d sign s of dr yn ess on th e surface of th e eye w ith th is con t act
lens w h en com pared w ith a grou p of oth er len ses w ith w h ich it w as tested. Never-
th eless, con tact len ses alon e h ave n o place in th e t reat m en t of dr y eyes; con com i-
tant u se of art ificial teardrops (an d periodic ch ecku ps) is essen t ial.
In som e cases, sm all pu n ctal plugs m ay be in ser ted in th e in ferior lacrim al
pun ct u m to slow drain age an d loss of tears.
Cyclosporine 0.05% ophthalm ic em ulsion (Restasis, Allergan, Inc., Irvine, CA) : Can
be u sed to relieve dr y eyes caused by su ppressed tear produ ct ion secon dar y to
ocu lar in flam m at ion . Safet y for u se in ch ildren an d du ring p regn an cy h as n ot
been establish ed.
Autologous serum : It h as a ben eficial effect on corn eal epith elium (20% au tolo-
gou s serum dilu ted in n orm al salin e).
Tet racyclines: Tradit ion ally u sed as an t ibiot ics, th ey also h ave im port an t an -
t iin flam m ator y proper t ies. Ver y u sefu l (doxycyclin e) in t reat ing acn e rosacea
dr y eye. Tet racyclin es are also effect ive again st recu rren t corn eal erosion s an d
ph lycten u lar keratoconju n ct ivit is.
Oral pilocarpine (Salagen , Novart is Pharm aceut icals UK Ltd., Surrey, UK) : In it ially
u sed in xerostom ia (salivar y glan d dysfu n ct ion , com m on after irradiat ion of th e
n eck in on cology), it h as been fou n d u sefu l in severe xeroph th alm ia by st im u-
lat ing tear secret ion (5 to 10 m g/8 h ). Good result s are seen in Sjögren syn drom e
after a few w eeks of t reat m en t .
Salivary gland t ransplantat ion into the inferior tarsal conjunct iva : Has been re-
por ted to be usefu l in dr y-eye con dit ion s w ith severe perm an en t lacrim al glan d
dysfu n ct ion s (n ot u sefu l in Sjögren syn drom e). Tech n ically com p lex.
Symblepharon
Sym bleph aron is an adh esion bet w een th e palpebral conjun ct iva an d th e bulbar
conju n ct iva or corn ea. Sym bleph aron is usually th e result of a t raum a (surger y,
ch em ical or radiat ion bu rn s) or sup erficial eye in flam m at ion (er yth em a m u lt i-
form e, t rach om a, burn s, pem ph igoid, Steven s-Joh n son syn drom e).
Presentation
Sym ptom s in clu de adh esion bet w een th e palpebral conju n ct iva an d th e bulbar
conju n ct iva or corn ea, lid deform it ies, fu n ct ion al lacrim al occlu sion an d tearing,
en t ropion , an d dist ich iasis ( Fig. 4.11 ).
Diagnosis is apparently clinical and is seldom confused w ith other disease entities .
Management
Treatm ent is often frustrating, and progressive scarring cannot be com pletely con-
trolled or reversed. Grow th prevention includes breaking early adhesions w ith a
rectal therm om eter t w ice a day and steroid ointm ents. Conform ers can be tried.
Surgical Z-plast y w ith am niotic m em brane or m ucosal graft can be tried. The use of
A B
Fig. 4.11 Symblepharon: (A) bet ween bulbar and palpebral conjunctiva; (B) at the lat-
eral canthal angle.
am niotic m em brane can be beneficial in that it facilitates epithelialization, m aintains

norm al epithelial phenot ype (w ith goblet cells w hen perform ed on the conjunctiva),
and effectively helps in reducing inflam m ation, vascularization, and scarring. Im m u-
nosuppressant therapies (m ethotrexate, cyclophospham ide, cyclosporine, azathio-
prine, etc.) can help in the acute episodes of the illness. In late cicatricial stages, treat
collateral effects such as dr y eye syndrom e, and punctal occlusion.
Pinguecula, Pterygium, and Lipoid Degeneration
Pinguecula
Pinguecu lae are th e m ost com m on ben ign conjun ct ival t u m ors. Th ey u su ally af-
fect m iddle-aged in dividuals bu t can also be foun d in ch ildren an d young peop le,
especially th ose w ith dyslipidem ia. Pingu ecu lae h ave n o sex or racial predilect ion .
Th ey seem related to ch ron ic exposure to th e su n .
Presentation
Pingueculae are usually asym ptom at ic, yellow ish /w h it ish , sligh tly raised, in terpal-
pebral lipid-like dep osits in th e n asal (m ore frequen t) an d tem poral lim bal con -
jun ct iva. Un i- or bilateral (usually bilateral an d asym m et rical), th ey m ay becom e
vascu larized an d in flam ed (pingu eculit is). In a case of in flam m at ion , pat ien t s ex-
perien ce foreign body sen sat ion an d conju n ct ival redn ess arou n d th e pingu ecu la,
corn eal p un ct ate epith eliopathy, an d corn eal th in n ing secon dar y to dr yn ess (del-
len u lcerat ion ). Th ese pat ien ts m ay com plain of dr y-eye sym ptom s an d foreign
body sen sat ion ( Fig. 4.12A,B).
Fig. 4.12 (A) Pingueculae.

(Continued on page 118) A
Fig. 4.12 (Continued) (B) Pinguecula and melanoma.
Pter ygiu m , conju n ct ival derm oid, conju n ct ival n eoplasia (a u n ilateral, w h ite, vas-
cularized m ass), p h lycten ule, pan n u s, conju n ct ival reten t ion cyst (a clear, fluid-
filled sac), lim bal follicles
Management
No t reat m en t is n ecessar y in cases w ith good lubricat ion resu lts, w h ich h ave a
low er risk of pingu eculae form at ion (preser vat ive-free tear subst it u tes are pref-
erable).
Con sider surgical part ial or tot al resect ion u n der local an esth esia in th e follow -
ing:
Severe cases (gian t pingueculae) w h ere pter ygiu m is presen t an d in terfering
w ith vision
Ch ron ically in flam ed severe cases
Un com for table con t act len s w ear
Bad corn eal lu bricat ion w ith dellen form at ion
Cosm et ic problem s
In th e case of in flam m at ion , u se ocu lar lu bricat ion an d m ild topical steroids
(e.g., flu orom eth olon e), w ith decongest an ts (e.g., n aph azolin e).
Preven t ion is possible for peop le w ith occupat ion s or h obbies th at in crease
th e risk of pinguecu la (sailing, fish ing, skiing, garden ing, outdoor con st ru ct ion
w ork). Sun goggles, u lt raviolet-blocking coat ings, or goggles th at lim it dust ex-
posure are h elpful.
Pterygia
Like pinguecu lae, pter ygia h ave been related to exposu re to u lt raviolet ligh t (both
ult raviolet A [UV-A] an d UV-B). Risk factors in clu de living in su bt ropical an d t ropi-
cal clim ates, ou tdoor act ivit ies (e.g., golf, sailing, fish ing), dr y w in dy clim ates, dust ,
an d fu m es. A gen et ic predisposit ion h as been described. Th ere are t w o groups of
pter ygium pat ien t s: pter ygia w ith m in im al proliferat ion an d at roph ic appearan ce
Fig. 4.13 (A) Pterygium. (B) Pte -

rygium postsurgical papilloma. (C)
Terrien’s marginal degeneration.
C
(slow -grow ing pter ygia, w ith low in ciden ce of recu rren ce after excision ) an d el-
evated fibrovascu lar pter ygia (rapid grow th , aggressive clin ical cou rse, an d h igh
rate of recu rren ce after excision ) ( Fig. 4.13A,B,C)..
Th ere is a predilect ion for m ales, an d occu rren ce is m ore frequ en t in th e n asal
conju n ct iva.
Presentation
A t riangular, fleshy, elevated m ass of bu lbar conjun ct iva grow s over th e corn ea
w ith in th e in terpalpebral fissure. Sm all pter ygia are asym ptom at ic. Th ey can be-
com e in flam ed, w ith redn ess, foreign body sen sat ion , an d ocular irrit at ion (dr y-
eye sym ptom s). In advan ced cases, pat ien t s experien ce vision problem s (ast igm a-
t ism , progression over th e visu al axis), rest rict ion of ocular m ot ilit y, an d blin dn ess
(in u n derdeveloped cou n t ries w h ere surger y is u n available).
Pinguecu lae, am yloidal degen erat ion of th e conju n ct iva, pseu dopter ygium , n eo-
plasia (e.g., carcin om a in sit u , squ am ous cell carcin om a)
Management
Good in ten sive lubricat ion is essen t ial.
Topical steroids su ch as predn isolon e acetate (Pred For te, Allergan , In c., Ir vin e,
CA) 1%, th ree to fou r t im es daily an d an t ih ist am in es provide relief of in flam m a-
t ion .
In dicat ion s for surger y in clude th e follow ing:
Cosm et ic reason s
Discom for t due to recu rren t in flam m at ion
Before it en croach es on th e pu pillar y area
Mult ip le differen t surgical procedu res w ork in th e first grou p of pter ygiu m pa-
t ien t s (at roph ic, slow grow ing), bu t n on e can be said to w ork sat isfactorily in
th e secon d group of pat ien ts (fast grow ing, aggressive clin ical course):
Sim p le excision
Sim p le excision an d repair w ith conju n ct iva autop last y
Sim p le excision an d sliding flaps of conjun ct iva
With an d w ith ou t adjun ct ive extern al β radiat ion th erapy (1000 to 2000 rep s
at lim bu s or th iotepa 1:2000 solu t ion ) an d/or in t raoperat ive topical m itom y-
cin - C or postoperat ive 5-flu orou racil
Prim ar y excision plus free graft s of conjun ct iva an d lim bal t issu e (lim bal au -
tograft) or am n iot ic m em bran e: for aggressive or recu rren t pter ygia
Lipoid/Lipid Degeneration of the Cornea and Conjunctiva

Lipid degen erat ion of th e corn ea an d conju n ct iva m ay occur in prim ar y or secon d-
ar y form . Th e prim ar y form is usu ally bilateral an d m ore diffu se an d is cau sed by
lipid seru m dyscrasias su ch as Tangier fam ilial h igh -den sit y lipoprotein deficien cy
or lecith in ch olesterol acylt ran sferase deficien cy. Th e secon dar y form is by far th e
m ost com m on form of th is rare disease, is m ore localized, an d is due to th e pres-
en ce of corn eal blood vessels after ocu lar t rau m a or in terst it ial or h erpet ic kera-
t it is.
Presentation
W h ite, yellow, or cream -colored den se opacificat ion of th e corn eal st rom a is seen
in a diffu se or localized m an n er. Ch olesterol cr yst als occu r in th e corn eal or con -
jun ct ival st rom a su rrou n ding aberran t blood vessels. Th e conju n ct ival feeder ves-
sels are dilated, an d th ere are sym ptom s of dr y eye ( Fig. 4.14 ).
Aspect at slit lam p is diagn ost ic. Ru le out degen erated epith eliom a.
Fig. 4.14 Cornea conjunctival li-

poid degeneration.
Management
Treat th e u n derlying con dit ion to redu ce th e risk of progression (reduce fat se-
rum levels). At tem pt closing th e feeder vessels w ith argon laser ph otocoagu lat ion .
Severe cases w ith affected pu pillar y area m ay requ ire pen et rat ing keratoplast y,
alth ough th is degen erat ion m ay recur in th e graft .
Conjunctival Retention Cysts
Conjun ct ival lym ph at ic cysts are soft , t ran slu cen t , an d m obile. Large cyst s m ay
give dr y eye or foreign body sen sat ion (eye discom for t or tearing). A conju n ct ival
cyst m ay reveal differen t possible origin s an d can be due to parasit ic in festat ion
(e.g., cist icercosis), w h ich h as to be ruled ou t w h en th e p at ien t h as t raveled to en -
dem ic region s, or it m ay be an accessor y lacrim al glan d, a post t raum at ic in clusion
cyst (of conjun ct ival epith eliu m ), or sim ply a lym ph at ic cyst (th e m ost com m on ).
Presentation
Soft , t ran slucen t , an d m obile flu id-filled m ass w ith in th e conjun ct iva, alon e or in
groups, fou n d at slit-lam p exam in at ion . More frequ en t in th e bu lbar conju n ct iva
an d in th e in ferior tarsu s or in ferior cul-de-sac ( Fig. 4.15 ).
Fig. 4.15 Conjunctival lymphatic retention cyst.

Cyst of m align an t origin (ru le out parasit ic in festat ion an d h em at ic dyscrasia)
Management
Th ese cysts n eed n o t reat m en t u n less th ey cau se persisten t dr y eye or foreign
body sen sat ion . Topical lubrican t s provide relief. Th eir w all can be easily broken
in feriorly w ith a few sh ot s of n eodym ium :yt t rium -alu m in um -garn et laser app lied
in feriorly on th e cyst w alls, w ith care to avoid conju n ct ival vessels. Postsu rgical
cyst can be excised.
Superior Limbic Keratoconjunctivitis
Th is con dit ion is of un kn ow n et iology an d associated w ith thyroid disease. In su -

perior lim bic keratoconjun ct ivit is, conju n ct ival laxit y m igh t in du ce in flam m ator y
ch anges from m ech an ical t raum a of hyper t roph ic pap illae of th e u pper t arsal con -
jun ct iva. It affects m ain ly adu lt w om en . A sim ilar con dit ion is foun d in soft con tact
len s w earers using th im erosal-preser ved solut ion s.
Presentation
Sym ptom s in clu de tearing, bu rn ing, superior hyp erem ia, an d foreign body sen -
sat ion . Pat ien ts w ith filam en ts are ext rem ely sym ptom at ic. In flam m at ion of th e
su perior bu lbar conjun ct iva result s in edem a w ith redun dan t conjun ct iva. Th ere
is predom in an t involvem en t of th e superior lim bu s, adjacen t epith elial kerat it is,
an d papillar y hyper t rophy of th e u pper t arsal conjun ct iva. Th e in ferior conjun ct iva
an d corn ea appear n orm al. Th e age range is 20 to 70 years. Occu rren ce is predom i-
n an tly in fem ales, is usu ally bilateral, an d m ay be asym m et rical, w ith rem ission s
an d exacerbat ion s. Dr y eye is often presen t ( Fig. 4.16 ).
Fig. 4.16 Superior limbal keratoconjunctivitis.

Clin ical fin dings are diagn ost ic (in flam m at ion of th e su perior bu lbar conju n ct iva
w ith absolutely n orm al appearan ce of th e in ferior conjun ct iva an d corn ea).
Management
Con ser vat ive t reat m en t offers on ly tem porar y relief of sym ptom s (pressu re patch -
ing, Plan o T ban dage con tact len s, Baush & Lom b In c., St . Lou is, MO). Silver n i-
t rate (0.5 to 1.0% solut ion ) is applied w ith a sat urated cot ton sw ab. It u su ally re-
lieves sym ptom s for 1 m on th an d can be repeated safely. Topical an t ih istam in es
an d m ast cell st abilizers (e.g., olopat adin e hydroch loride 0.1%, ketot ifen fu m arate
0.025%, azelast in e hydroch loride 0.05%) are h elpfu l. Moist urizing drop s an d oin t-
m en ts provide on ly m in im al relief. Cr yoth erapy an d a su rgical approach can be
taken in recalcit ran t cases involving resect ion or recession of th e su perior bu lbar
conju n ct iva or in -office th erm ocau terizat ion of th e su perior bulbar conju n ct iva
w ith a disposable oph th alm ic cauter y u n der topical an esth esia. Th e u se of topical
steroids is usually in effect ive.
Episcleritis
Episclerit is is a t ran sien t , self-lim ited in flam m ator y process of th e episclera. Most
episclerit is is idiopath ic, but it can also be associated w ith system ic disorders su ch
as rh eu m atoid ar th rit is, acn e rosacea, an d atopy.
Presentation
Th ere is an acute on set an d diffuse or n odu lar ocu lar redn ess w ith out irrit a-
t ion or pain . In flam ed ep iscleral vessels radiate posteriorly from th e lim bus (Fig.
4.17A,B).
Fig. 4.17 (A) Episcleritis. (Contin-

ued on page 124)
Fig. 4.17 (Continued) (B) Nodular episcleritis.
Sclerit is, uveit is, conjun ct ivit is
Management
Th e con dit ion is self-lim ited; n o t reat m en t is n eeded. Topical steroids or n on ste-
roidal an t iin flam m ator y drugs (NSAIDs) or a com bin at ion of th ese can accelerate
recover y. Vasocon st rictors an d refrigerated art ificial tears p rovide relief.
Scleritis
Sclerit is is a gran u lom atous in flam m at ion of th e sclera th at m ay presen t in as-

sociat ion w ith system ic diseases such as rh eu m atoid ar th rit is, system ic lu pu s
er yth em atosu s, polyarterit is n odosa, or Wegen er gran ulom atosis. Sclerit is can be
self-lim it ing or can progress to a p oten t ially blin ding n ecrot izing process. Possible
com plicat ion s in clude scleral th in n ing (especially in recurren t sclerit is), sclerom a-
lacia perforan s, sclerosing kerat it is, perip h eral corn eal m elt ing, uveit is, cat aract ,
m acu la edem a, ch oroidal gran ulom as, an d ret in al det ach m en t . Posterior sclerit is
occurs m u ch less frequen tly th an an terior sclerit is an d m ay exten d in to th e an te-
rior segm en t of th e eye. In cases of n ecrot izing sclerit is, in fect ion h as to be ruled
out .
Fig. 4.18 Scleritis table.
Presentation
Th ere is severe ocu lar pain w ith or w ith ou t decreased vision (suspect posterior
involvem en t if vision is com prom ised). On set of sclerit is is m ore gradu al th an is
seen in episclerit is an d is accom pan ied by u n i- or bilateral red eye, lacrim at ion ,
an d ph otoph obia ( Fig. 4.18 ).
Conju n ct ivit is, uveit is, episclerit is
Management
Man agem en t in cludes system ic NSAIDs (e.g., in dom eth acin , ibuprofen , n aproxen ),
topical steroids (e.g., bet am eth ason e, dexam eth ason e) an d system ic oral steroids
(oral steroids preferred over topical), an d im m u n osup pressan t th erapies (e.g.,
m eth ot rexate, cycloph osph am ide, cyclosporin e, azath ioprin e). System ic t reat m en t
w orks bet ter th an topical, w h ich h as to be con sidered accessor y to a system ic an -
t iin flam m ator y regim en .
Dilated Vessels
Dilated Vessels and Ocular Vein Varicosities

Dilated vessels are com m on ly seen in h ealthy pat ien t s, especially w om en w ith ve-
n ou s in su fficien cy. Th ey m ay be a sign of ven ous congest ion or raised episcleral
pressure, eith er due to carot id cavern ous fist u la or to any orbit expan sive process
or in flam m ator y disease of th e eye. Dilated episcleral sen t in el vessels can be pres-
en t in cases of m align an t m elan om a of th e ch oroid or ciliar y body.
Fig. 4.19 Dilated vessels.
Presentation
Presen t at ion is asym ptom at ic. Foreign body sen sat ion occurs in th e case of vari-
cosit ies. Oth er sign s an d sym ptom s of red eye or orbit expan sive process m ay also
be seen . Thyroid disease presen t s w ith dilated vessels, m ain ly over th e extern al
rect us m u scles ( Fig. 4.19 ).
Any cau se of red eye (conjun ct ivit is, idiopath ic raised ep iscleral pressure, ven ous
in sufficien cy, carot id cavern ou s sin us fist u la, episclerit is, sclerit is, ch ron ic irrita-
t ion , glau com a su rger y)
Management
No t reat m en t is n eeded if th e con dit ion is idiopath ic. Varicosit ies can be success-
fully redu ced using argon laser ph otocoagulat ion . Th e occasion al u se of vasocon -
st rictors im proves cosm et ics but th ere is n o defin it ive t reat m en t . Treat th e con -
com it an t illn ess.
Dilated Episcleral Vessels in Sturge -Weber Syndrome

St u rge-Weber syn drom e is a rare n eu rological disorder presen t at birth , ch aracter-
ized by a birth m ark (u sually on th e face) kn ow n as a port-w in e st ain cau sed by an
overabun dan ce of capillaries arou n d th e t rigem in al n er ve ben eath th e surface of
th e face. Neu rological problem s arise du e to a loss of n er ve cells an d calcificat ion
of t issu e in th e cerebral cortex of th e brain on th e sam e side of th e body as th e
bir th m ark (angiom atosis of th e cen t ral n er vous system ). Neu rological sym ptom s
in clu de seizu res th at begin in in fan cy an d m ay w orsen w ith age. Convulsion s usu -
ally h appen on th e side of th e body opposite th e birth m ark an d var y in severit y.
Th ere m ay be m u scle w eakn ess on th e sam e side. Som e ch ildren w ill h ave devel-
opm en t al delays an d m en tal retardat ion . St u rge-Weber syn drom e rarely affects
oth er body organ s.
Fig. 4.20 Sturge-Weber syndrom e table.
Presentation
Ch aracterist ic facial bir th m ark (can var y in color from ligh t pin k to deep purple).
Most pat ien t s w ith angiom a th at affect s th e su perior lid h ave glaucom a at bir th or
w ill develop it later. Dilated episcleral vessels are seen , along w ith bup h th alm os,
seizu res, convulsion s, m uscle w eakn ess, m en tal ret ardat ion or learn ing disabili-
t ies, an d possible developm en t al delay (Fig. 4.20 ).
Facial angiom a w ith ou t n eu rological m an ifestat ion s, oth er cau ses of elevated epi-
scleral pressu re (e.g., thyroid oph th alm opathy, th rom bosis of th e cavern ous sin u s,
carot id-cavern ou s fist ula)
Management
Treat th e sym ptom s. Treat in creased in t raocu lar pressu re w ith eyedrops (avoid
m iot ics). Su rger y can be perform ed on serious cases of glaucom a. Laser t reat m en t
can ligh ten or rem ove th e facial bir th m ark. An t iconvu lsan t m edicin es are u sed to
con t rol seizu res. Physical th erapy is h elpfu l in ch ildren w ith m u scle w eakn ess.
Carotid Cavernous Sinus Fistula

Carot id cavern ous fist u las are un i- or bilateral abn orm al com m u n icat ion s (sh u n t s)
of th e carot id ar teries directly or in directly in to th e vein s of th e cavern ous sin u s.
Th ey are frequ en tly cau sed by t raum a, alth ough th ey can be spon t an eou s in m en o-
pau sal w om en . Depen ding on th e am ou n t of blood injected in to th e sin u ses, th ey
can produce differen t am ou n t s of n eu ro-oph th alm ological m an ifest at ion s.
Presentation
Patients present w ith arterialized dilated vessels of the conjunctiva and orbit, unilat-
eral or bilateral, proptosis, lid edem a, papilledem a, retinal edem a w ith hem orrhages,
uveitis, secondary glaucom a associated w ith elevated venous pressure, anterior seg-
m ent ischem ia, and iris atrophy. There can also be visual loss and dysfunction of
the extraocular m uscles. Pulsating exophthalm ia occurs in advanced (i.e., high-flow )
cases. Patient refers to an audible sound that follow s the heart rate. Cavernous sinus
syndrom e (cranial ner ve II, IV, and VI palsy) and Tolosa-Hunt syndrom e m ay also
present (Fig. 4.21A,B).
Cavern ous sin u s th rom bosis, du ral sin u s ar terioven ou s fist u la, cavern ou s sin us
ar terioven ou s m alform at ion s, orbital pseudot um or, thyroid orbitopathy, orbital
am yloidosis, orbital t u m or, orbit al cellu lit is, m ucorm ycosis
Fig. 4.21 (A) Carotid cavernous fistula. (B) Carotid cavern-

ous fistula.
Management
Neu rosu rger y by a n eu roradiologist (det ach able balloon or m etallic coil em boli-
zat ion ) accom plish es reversal of ocu lar m an ifest at ion s in p at ien ts w ith vision at
risk or pat ien ts w ith in tolerable sym ptom s (h igh -flow sh un ts). Low -flow sh u n t s
(m ain ly t raum at ic) spon t an eou sly occlude w ith t im e. Th e gam m a kn ife can be
used w h ere available.
Pigmented Conjunctival Lesions
Ocular or Oculodermal Melanocytosis (Congenital Melanosis Oculi)
Ocular Melanocytosis
Th is n on h eritable congen it al hyperpigm en tat ion of th e eye h as in creased fre-
qu en cy in w h ites. Path ology sh ow s an in creased n um ber of m elan ocytes in th e
affected t issu es. Th e con dit ion can progress w ith th e u se of topical prostaglan din
an alogues (e.g., latan oprost) u sed to t reat glau com a.
Presentation
Presen tat ion in clu des in creased gray or bluish pigm en tat ion of th e globe (sclera
an d episclera) th at is u su ally un ilateral ( Fig. 4.22 ).
Prim ar y acqu ired m elan osis (affect s th e conju n ct iva on ly an d oth er m u coses), ocu -
loderm al m elan ocytosis (n evus of Ot a), n evu s, m elan om as, pigm en ted dep osits,
foreign body iron deposit s
Fig. 4.22 Ocular melanocytosis.

Management
No t reat m en t is n eeded. Carefu l periodic ocular exam in at ion s are required becau se
of th e in creased risk of uveal m elan om as in affected in dividuals.
Oculodermal Melanocytosis
Th is con dit ion is caused by diffuse dist ribut ion of proliferated m elan ocytes an d is
m ore com m on in Asian s an d blacks. It is associated w ith ip silateral glau com a.
Presentation
Asym ptom at ic. Un ilateral facial an d/or blue-gray pigm en t at ion of th e globe, usu-
ally follow ing th e ipsilateral dist ribu t ion of th e t rigem in al n er ve (V1 an d V2) (Fig.
4.23 ).
Prim ar y acqu ired m elan osis, n evu s, m elan om as, pigm en ted dep osits, foreign body
iron dep osits, facial angiom a
Management
No t reat m en t is n eeded. Ru le out glaucom a, ret in it is pigm en tosa, an d congen it al
cataract . Ocu loderm al m elan ocytosis rarely u n dergoes m align an t t ran sform at ion .
Fig. 4.23 Oculoderm al melanocytosis. Heterochromia.

Conjunctival Pigmentations 1
Melan ocyt ic ben ign conjun ct ival lesion s are ver y com m on , especially in darker-
skin n ed in dividu als, in clu ding blacks or African Am erican s, Hispan ics, an d Git a-
n os, in th e lim bal area or caru n cle. Presen t at ion greatly differs from on e pat ien t
to an oth er. Th e lesion s h ave n o m align an t poten t ial, alth ough n evi ver y rarely give
rise to m align an cy (jun ct ion al an d com pou n d n evi). Ch ron ic u se of topical epi-
n eph rin e (as a cosm et ic), silver-con tain ing com pou n ds (argyrosis), ret ain ed iron
foreign body, atabrin e, tet racyclin es, clofam icin e, im bibed m ascara gran u les, ra-
diat ion , h orm on es (pregn an cy, Addison disease), ch em icals (arsen ic, th orazin e),
an d som e ch ron ic disorders (like t rach om a or xeroderm a pigm en tosu m ) are po-
ten t ial cau ses of pigm en t at ion in crease (darken ing of th e lesion s). Never th eless,
th e m ost com m on cau se of pigm en t at ion in crease is th e topical use of prostaglan -
din an alogues to low er in t raocular pressure. Conjun ct ival pigm en t at ion is n o sign
of m align an cy.
Presentation
Conju n ct ival ben ign epith elial m elan osis presen t s as a flat brow n ish pigm en t at ion
seen especially in blacks or African Am erican s, in th e lim bal area or caru n cle. Con -
jun ct ival p igm en ted lesion s close to th e lacrim al glan d can be ben ign , bu t alw ays
con sider m align an cy because m align an cy m ay be m ore exten sive th an is clin i-
cally apparen t . Su spect m align an cy if pigm en t at ion in creases in parallel to su rface
grow th ( Fig. 4.24A– D).
Conju n ct ival m elan om a
A B
C D
Fig. 4.24 (A–D) Conjunctival pigm entations.
Nom en clat u re m ay be con fu sin g, an d classificat ion is often con t roversial: w e h ave u sed th e
1
m ost w id ely accepted n am es.

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

Malign an t m elan om a of th e conjun ct iva is rare, but it is th e m ost com m on pig-
m en ted m align an cy of th e conjun ct iva an d accou n t s for 2% of all ocu lar m align an -
cies. It is far less com m on th an in t raocular ch oroidal m elan om a. It can appear in a
previou sly h ealthy par t of th e conju n ct iva (de n ovo, from m elan ocyt ic cells of th e
basal layer of conju n ct iva, in ~20 to 25% of cases), from a preexist ing conju n ct ival
jun ct ion al or com p oun d n evu s (~20%of cases), from a prim ar y acquired conju n ct i-
val m elan osis (~60% of cases). Th e in ciden ce of conjun ct ival m elan om a is in creas-
ing am ong w h ite m en in a t ren d sim ilar to th at of skin m elan om a. Conju n ct ival
m elan om a is probably related to su n exposu re ( Fig. 4.25 ).
Presentation
Clin ical presen tat ion is variable, w ith a raised, pigm en ted or n onpigm en ted lesion
(som e h ave lit tle or n o pigm en t , w h ich is rare) th at grow s an d/or bleeds an d/or
fixates to th e un derlying t issu es. Periph eral corn eal in filt rat ion is possible. Som e
grow aroun d th e lim bu s. Som et im es th ere is pigm en t at ion of th e lid m argin s or lid
skin (rare, poor progn osis). Region al lym ph n odes (parot id, p reau ricu lar, subm an -
dibu lar, an d cer vical) m ay be affected. Distan t m et ast ases are possible, w ith spread
by th e lym ph at ic vessels an d bloodst ream . Direct exten sion to th e eyeball an d orbit
is possible if th ere is late diagn osis.
Susp ect m elan om a if
a preexist ing n evu s grow s or h as in creased vascularit y
a preexist ing conjun ct ival n evus at th e lim bu s h as rap id ver t ical grow th
a p reexist ing n evu s grow s or h as in creasing n odu larit y or ch anges in pigm en t a-
t ion or bleeds, or develops in flam m at ion
a previou sly flat area of pigm en tat ion develop s n odu lar th icken ing
th ere is local conjun ct ival in creased vascu larit y (w ith or w ith out a pigm en ted
lesion )
Fig. 4.25 Semilunar fold melanoma.

th e conju n ct iva fixates to th e sclera

you obser ve h em orrh age in a pigm en ted lesion
you obser ve an u lcerat ive area in th e conjun ct iva th at does n ot h eal w ith topical
m edicat ion
Ben ign pigm en ted lesion s [n evu s, m elan osis, foreign body, pigm en ted dep osits
(ch em icals, m ascara gran u les)]. Suspect m align an t m elan om a in th e case of a
grow ing pigm en ted lesion . Not all conju n ct ival m elan om as are pigm en ted; th ey
can look like a squam ou s or sebaceous glan d carcin om a, a papillom a, lym ph oid hy-
perplasia, an d even a pter ygiu m . Metastat ic t u m or to th e conju n ct iva (ext rem ely
rare, e.g., secon dar y m elan om a from cut an eou s t um or).
Management
Man agem en t depen ds on path ological staging [varies in differen t cou n t ries: clin i-
cal t um or, n ode, m et ast ases (TNM) classificat ion , path ological classificat ion , an d
h istopath ological t ype an d grade].
Can be t reated con ser vat ively w ith th e proton accelerator.
Com plete resect ion of t u m or (w ith care to avoid t issue dissem in at ion ). Path o-
logical exam in at ion is requ ired at th e t im e of excision .
Conju n ct ival m elan om as at th e lim bu s: Absolu te alcoh ol epith eliectom y +
w ide local resect ion (par t ial lam ellar scleroconju n ct ivoten on ectom y w ith a
2–3 m m clear zon e) + cr yoth erapy of th e bed an d borders of excision
Palpebral conju n ct ival m elan om as (or forn ical): Surgical resect ion w ith ab-
solute alcoh ol t reat m en t to th e scleral base an d cr yoth erapy to th e surrou n d-
ing conju n ct iva
Large invasive m elan om as: Orbit al exen terat ion ? (alm ost alw ays m etast ases
h ave already occurred at th e t im e of t reat m en t). We prefer th e proton accel-
erator.
A con ser vat ive approach w ith topical m itom ycin - C is un der research .
Adju n ct ive radioth erapy an d/or ch em oth erapy m ay be n eeded.
Life-long close obser vat ion follow -up w ith palpat ion of lym ph n odes (th ree to
fou r t im es yearly)
Blue Sclera and Scleral Staphyloma
Blue Sclera in Osteogenesis Imperfecta

Blu e sclerae in in fan cy an d ch ildh ood reflect eith er an abn orm al th in n ess of th e
sclera or an in creased scleral t ran sparen cy. Th e u n derlying uveal pigm en t pro-
du ces th e blu e-gray appearan ce. Sligh t blue sclerae are com m on in n eon ates, par-
t icu larly if th ey are prem at u re. It can be con sidered a n orm al varian t in th e first
several m on th s of life. In case of persist ing pron oun ced blu en ess, op h th alm ologi-
cal evaluat ion is n eeded to ru le ou t th e presen ce of elevated in t raocular pressu re
(IOP). Pediat ric an d or th opedic evalu at ion is n eeded to rule ou t in h erited diseases
resp on sible for th e blue-t in ted sclerae, such as osteogen esis im perfect a.
Osteogen esis im p erfect a is a rare in h erited congen it al disorder w ith ext rem e
bon e fragilit y, caused by m ut at ion s in th e gen es th at codify for t yp e I procollagen
( COL1A1 an d COL1A2 ). At least four t ypes of osteogen esis im perfect a h ave been
Fig. 4.26 Osteogenesis imperfecta. Blue sclera.
described. Th e age w h en fract ures begin varies w idely. Pat ien t s w ith m ild form s
m ay presen t w ith fract ures in in fan cy or m ay n ot h ave fract ures u n t il adu lth ood.
Th e m ore severe cases m ay h ave fract u res in u tero.
Presentation
Presen t at ion is asym ptom at ic; th ere are n o ocu lar sym ptom s. In pat ien ts w ith os-
teogen esis im perfect a, th e sclera can be blue or w h ite ( Fig. 4.26 ).
Blue sclera also m ay occu r in oth er disorders, su ch asalkapton uria, progeria, clei-
docran ial dysplasia, cu t is laxa (pseu doxan th om a elast icum ), Eh lers-Dan los syn -
drom e, Marfan syn drom e, Ch en ey syn drom e, Men kes syn drom e, an d pykn odysos-
tosis. Som e h igh or ext rem e m yopes m ay presen t w ith “blu e-sclera” due to scleral
th in n ing.
Management
No ocu lar t reat m en t is n eeded. Oth er system ic invest igat ion s an d t reat m en t are to
be don e. Gen et ic coun seling is h elpfu l.
Blue Sclera and Scleral Staphylomas

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

tal glaucoma buphthalmos.
Presentation
Epiph ora, p h otoph obia, an d blep h arospasm are seen in a n ew born or you ng in -
fan t . Bu ph th alm os, ectasia, st aphylom as, an d in creased corn eal diam eter m ay also
be presen t . Corn eal edem a or Haab st riae m ay be seen at slit-lam p exam in at ion ,
(w h ich m ay n ot alw ays easy to perform in babies).
With causes of blu e sclera (e.g., osteogen esis im perfecta), n eoplasias, an d causes
of congen ital glaucom a:
Dysgen esis of th e iris, angle, an d periph eral corn ea w ith or w ith out system ic
abn orm alit ies (e.g., Rieger an om aly syn drom e, Axen feld an om aly syn drom e,
Peter an om aly, an iridia, Marfan syn drom e, Weill-March esan i syn drom e)
Ph akom atoses (e.g., n eu rofibrom atosis, St urge-Weber syn drom e)
Metabolic disease (e.g., ocu locerebroren al syn drom e or Low e disease, h om ocys-
t in u ria)
In flam m ator y (e.g., r ubella, juven ile xan th ogran ulom a)
Ch rom osom al delet ion /du plicat ion (e.g., Tu rn er syn drom e, t risom y 13–15)
Management
Topical m edical t reat m en t is used to redu ce IOP un t il surger y can be safely p er-
form ed. Beta-blockers an d m iot ics rarely w ork (th ough th ey m ay h elp). Carbon ic
an hydrase in h ibitors can be u sed to h elp redu ce corn eal edem a an d allow a bet ter
in spect ion of an terior ch am ber st ru ct u res. Modern drugs (e.g., prostaglan din an a-
logues, brim on idin e tar t rate) can be t ried, alth ough th eir u se in in fan t ile glaucom a
is n ot curren tly accepted.
Surger y aim s at redu ct ion of com p licat ion s of IOP in crease (e.g., am blyopia,
staphylom as, opt ic n er ve at rophy). We use ou r ow n su rgical tech n ique, called Si-
m on’s pect in otom y (surgical ablat ion of t rabecu lodysgen et ic t issue–iridopect in eal
t issue by m ean s of an in st ru m en t of our ow n design ), com bin ed, or n ot , w ith a
gon iotom y, t rabecu lotom y, or t rabeculectom y. Glau com atous cu pping in in fan ts
h as proven to be reversible w h en su ccessful su rger y is perform ed early.
Conjunctivalization of the Cornea
Den se leu kom as, severe scarring, an d conjun ct ivalizat ion of th e corn ea can be
fou n d after bon e m arrow t ran splan t at ion , severe corn eal or in t raocu lar in fect ion s,
or ocular radiat ion th erapy. A sim ilar con dit ion can be th e result of severe ch em i-
cal burn s or au toim m un e disorders.
Presentation
Severe scarring, leukom a, an d conjun ct ivalizat ion of th e globe are seen (Fig.
4.28 ).
Ph th isis bu lbi, at rofia bu lbi, en doph th alm it is sequ elae, Steven s-Joh n son syn drom e,
er yth em a m ult iform e, ch em ical bu rn s
Management
Palliat ive t reat m en t to alleviate discom for t con sists of surface lubricat ion w ith
n onp reser vat ive art ificial tears or oin t m en t s, pun ct al occlu sion , an d h u m idifiers
or m oist u re ch am bers to decrease tear film evaporat ion . Surgical tarsorrh ap hy
is used for in t ractable dr y eye. Mydriat ics an d covering th e globe w ith am n iot ic
m em bran e can provide relief. Cosm et ic prosth esis after en ucleat ion is p erform ed
if n eeded.
Fig. 4.28 Massive corneal neovascularization and opacifica-

tion following radiotherapy.
Conjunctival Metaplasia in Ectropion
Metaplasia of th e conju n ct iva is an un com m on disease w h ere th e conju n ct ival m u -

cosa ch anges in to a skin like surface due to ch ron ic exposu re of th e conju n ct iva to
air an d ligh t . We also describe kerat in izat ion of th e conju n ct iva.
Presentation
Conjun ct ival hyp erem ia an d lacrim at ion are seen in a pat ien t su ffering from in -
ten se ch ron ic lid eversion (ect rop ion ). Th ere is also foreign body sen sat ion (Fig.
4.29 ).
Squ am ous cell carcin om a of th e conju n ct iva
Management
In ten se lu bricat ion w ith eye drops, gels, an d oin t m en ts u n t il su rger y of ect ropion
is perform ed.
Fig. 4.29 Conjunctival m etaplasia in ectropion.

Chemosis
Ch em osis con sists of conjun ct ival an d subconju n ct ival edem a. In gen eral, ch em o-
sis is a n on specific sign of eye irrit at ion .
Presentation
Liquid collect ion is seen u n der th e conjun ct iva. Som et im es th e conjun ct iva be-
com es so sw ollen th at th e eyes can n ot close properly. Oth er sign s an d sym ptom s
depen d on th e cause of ch em osis ( Table 4.2 ) ( Fig. 4.30A– C).
Any cau se of ch em osis ( Table 4.2 )
Table 4.2 Co m m o n Causes o f Che m o sis
Severe local in am mation:

Gonococcus, viral or chlamydial conjunctivitis
Allergic or hypersensitivit y reaction
Endophthalm itis
Panophthalmia
Local irritation
Trauma:
Ruptured globe
Post trabeculectomy
Postsurgical after scleral or vitreoretinal surgery
Generalized edema:
Nephropathy (of any origin)
Heart disease (congestive)
Quincke edema
Urticaria
Myxedema
Venous congestion
Cavernous sinus
Orbital tumor
Orbital cysts
Orbital in am mation:
Orbital cellulitis
Endocrine thyroid disease (thyrotoxicosis)
Drugs:
Atropine
Epinephrine
Glaucoma medications
Tri uridine
Parasites:
Trichinella spiralis dissemination
Fig. 4.30 (A) Mild chem osis. (B) Massive chem osis. (C) Mas-
sive chem osis.
Management
Treat th e un derlying con dit ion (e.g., t reat allergic conju n ct ivit is if ch em osis is due
to an allergic react ion ). Ch em osis after t rabecu lectom y is h igh ly desirable an d
does n ot n eed to be t reated: th e n ovice m ay be tem pted to perform early surgi-
cal revision of th e filtering bleb, w h ich is on ly n ecessar y if atalam ia w ith corn eal
edem a is presen t .
Hyaline Pillat Scleral Plaque
Pillat scleral plaqu e is an involut ive th in n ing of th e sclera, w ith hyalin e degen era-
t ion , resu lt ing from t ract ion forces of ext raocu lar m uscles, especially th e lateral
rect us.
Presentation
A grayish pun ch ed-ou t area is seen an terior to th e in ser t ion of th e lateral rect us
m u scles. Slit-lam p exam in at ion is diagn ost ic. Th ere is n o clin ical m an ifestat ion ,
but th e p laqu es are visible in older pat ien ts ( Fig. 4.31 ).
Sclerom alacia, m elan osis bulbi, scleral ect asia, staphylom as
Management
No t reat m en t is n eeded.
Fig. 4.31 Hyaline scleral Pillat’s plaque.

5 Cornea
W. Barry Lee and Ivan R. Schw ab
Cornea Infections
Herpes Simplex Virus

Herpes sim plex viru s (HSV) is a DNA viru s th at involves various stages of in fect ion ,
in clu ding a prim ar y system ic in fect ion , an in act ive laten t stage, an d a recu rren t
in fect iou s st age follow ing react ivat ion of th e laten t viral st ate. Un ilateral in fect ion
is m ost com m on , bu t bilateral in fect ion can occu r in up to 10%of cases (m ore likely
in atopic in dividu als) ( Fig. 5.1A,B).
Presentation
Prim ary infect ion stage : Upp er respirator y in fect ion sym ptom s w ith or w ith ou t
prodrom al sign s of fever, m alaise, an d fat igu e. Associated preauricular lym ph -
aden opathy is n ot un com m on . Ocular involvem en t in th e prim ar y stage m ost
com m on ly involves vesicles on th e periorbital skin w ith or w ith ou t a follicular
bleph aroconjun ct ivit is. In fect ion m ay rarely presen t in th e conju n ct iva or cor-
n ea w ith a follicular conjun ct ivit is an d pu n ctate kerat it is or den drit ic kerat it is.
In th e laten t stage, th e virus rem ain s dorm an t in th e sen sor y n er ve ganglia.
Fig. 5.1 (A) Corneal dendrite high-

lighted by rose bengal from herpes
simplex virus (HSV) intraepithelial
keratitis. (B) Corneal scar and iris at-
rophy from HSV stromal keratitis. B
141
Recurrent infect ion stage : Th e virus t ravels dow n n er ve a xon s to sen sor y n er ve
en dings to in fect th e ocular su rface. Corn eal fin dings in clu de th e follow ing:
Pu n ctate epith elial keratopathy
Dendrit ic int raepithelial kerat it is : An u lcer of th e epith eliu m w ith th in ,
bran ch ing figures an d term in al bu lbs at th e en d of each bran ch w ith sw ollen
borders
Im m une strom al kerat it is : Grou n d-glass–like corn eal h aze, scarring, an d po-
ten t ial th in n ing w ith late n eovascularizat ion an d lipid deposit ion . Th e epi-
th elium is often in t act but m ay break dow n in severe cases w ith p rogression
to n ecrot izing kerat it is.
Necrot izing st rom al kerat it is: A corn eal ulcer w ith an epith elial defect , st ro-
m al in filt rat ion , th in n ing, an d n ecrosis. Th ere is a h igh risk for perforat ion .
Marginal ulcer: A perilim bal epith elial lesion w ith st rom al in filt rat ion , pan -
n us, an d th in n ing
Neurot rophic ulcer: A corn eal ulcer result ing from corn eal an esth esia from
viral dam age to sen sor y corn eal n er ves. Im p aired corn eal in n er vat ion leads
to an u lcer w ith an epith elial defect con tain ing sm ooth , rolled borders.
Endothelit is: Focal or disciform corn eal st rom al edem a w ith en doth elial ke-
rat ic precipitate an d an terior ch am ber cellular react ion
Differen t ial diagn osis of den drit ic epith elial kerat it is:
Oth er viruses [h erpes zoster vir us, Epstein -Barr viru s, epidem ic keratocon -
jun ct ivit is (EKC)]
Healing epith elial defect s
Soft con tact len s w ear
Tyrosin em ia t ype II
Acantham oeba
Rosacea
Superficial hyper t roph ic den driform epith eliopathy (SHDE)
Differen t ial diagn osis of st rom al kerat it is
Herpes zoster
Bacterial kerat it is
Acantham oeba
St aphylococcal m argin al kerat it is (m argin al u lcer cases)
Rosacea
Collagen vascular disease
Mooren ulcer (m argin al ulcer cases)
Management
Diagn osis is u sually clin ical, bu t scrapings can be perform ed w ith Giem sa stain or
Papan icolaou sm ear. Polym erase ch ain react ion or an t igen detect ion can be used
as a diagn ost ic tool to detect viral par t icles from scrapings or cult u re.
Dendrit ic int raepithelial kerat it is : Debridem en t of den drite m ay or m ay n ot be
perform ed. Begin t riflu ridin e 1%, on e drop ever y 2 h ou rs (n in e t im es daily) or
vidarabin e oin t m en t five t im es daily for 10 to 14 days. Th e Herpet ic Eye Disease
St udies (HEDS) foun d oral an t ivirals do n ot preven t th e subsequ en t risk of st ro-
m al kerat it is. Con sider cyclop legic agen ts an d avoid cort icosteroids.
Im m une st rom al k erat it is : An t ivirals w it h eit h er oral agen t s (acyclovir, valacy-
clovir, or fam ciclovir) or top ical t r iflu r id in e can be u sed in conju n ct ion w it h
top ical cor t icosteroid s in cases w it h severe st rom al scar r ing an d d ecreased
visu al acu it y; h ow ever, top ical an t ivirals d o n ot p en et rate in t act ep it h eliu m
5 Cornea 143
w ell; t h u s for d eep st rom al kerat it is cases, oral agen t s m ay w ork best . Th e
HEDS t r ial sh ow ed t h at top ical cor t icosteroid toget h er w it h an t ivirals red u ced
p ersisten ce an d p rogression of st rom al in flam m at ion an d sh or ten ed t h e d u ra-
t ion of st rom al kerat it is. Oral an t ivirals (valacyclovir 500 m g on ce daily or acy-
clovir 400 m g t w ice a day) can be u sed for long-ter m su p p ression an d avoid
cor n eal toxicit y. Long-ter m su p p ression is esp ecially h elp fu l for p at ien t s w it h
m u lt ip le recu r ren ces of st rom al kerat it is. High er d oses sh ou ld be u sed for ac-
t ive d isease. Cor t icosteroid s sh ou ld be t ap ered to t h e low est d ose t h at con t rols
in flam m at ion .
Neurot rophic kerat it is: Preser vat ive-free lu brican t drop s an d oin t m en ts can be
ben eficial w ith or w ith ou t blan d an t ibiot ic oin t m en t use to preven t secon d-
ar y bacterial in fect ion (i.e., er yth rom ycin oin t m en t). Persisten t epith elial de-
fects can be t reated w ith t arsorrh aphy or au tologous seru m drops. Th erapeut ic
ban dage len s w ear can be used tem porarily w ith close obser vat ion an d con -
siderat ion of prophylact ic an t ibiot ic t reat m en t . Em ergen t surgical t reat m en t
m ay in clude lam ellar or pen et rat ing keratop last y, cyan oacr ylate glu e patch ing,
am n iot ic m em bran e graft ing, or conjun ct ival flaps in sit uat ion s w h ere visual
reh abilit at ion is poor.
Herpes Zoster Virus

Herpes zoster viru s (HZV) is a DNA virus also kn ow n as varicella virus. It h as a
prim ar y in fect ion m an ifested as a self-lim ited in fect ion of ch ildh ood (ch icken pox)
follow ed by a p eriod of laten cy in w h ich th e viru s is dorm an t in th e n eural ganglia.
React ivat ion cau ses h erpes zoster (sh ingles) in ~20% of in dividuals. Herp es zoster
cases in clu de 15% th at affect th e oph th alm ic n er ve dist ribut ion of th e t rigem in al
n er ve (cran ial n er ve V1 division ). In fect ion alw ays involves a single derm atom e,
m aking it un ilateral.
Presentation
Prim ary infect ion stage : Ch icken pox. Occurs as a self-lim ited in fect ion in ch il-
dren . Sym ptom s in clude a m aculopapu lar rash , along w ith p rodrom al sym p-
tom s of fever, m alaise, an d upp er respirator y in fect ion sym ptom s. A vaccin e is
n ow available for ch ildren t ypically at 12 m on th s of age to p reven t ch icken pox.
A vaccin e is also available for im m un ocom p eten t in dividuals over th e age of 65.
Can be life th reaten ing if a prim ar y in fect ion develops in adu lth ood or in im -
m u n osu ppressed pat ien t s.
Latent stage : Viru s is dorm an t in th e n eural ganglia.
Recurrent or react ivat ion stage : Viru s t ravels dow n a single derm atom e w ith
pain , paresth esias, an d dysesth esia, follow ed by a u n ilateral m aculopapular
rash along th e involved derm atom e. Ocular involvem en t occu rs in m ore th an
70% of pat ien ts w ith cran ial n er ve V1 involvem en t . Corn eal fin dings in clu de
a pu n ctate or den drit ic epith elial kerat it is, n u m m u lar su bepith elial in filt rates,
st rom al kerat it is, disciform kerat it is, an d gran u lom atou s kerat ic precipit ate
from uveit is. Neu rot roph ic keratopathy, corn eal scarring, corn eal n eovascu lar-
izat ion , in terst it ial kerat it is, lipid keratopathy, an d keratolysis can also occu r
( Fig. 5.2A,B,C).
HSV, bacterial, or fungal in fect ion ; collagen vascular disease or im m u n e-related
corn eal u lcers; exposure-related corn eal ulcers
Fig. 5.2 (A) Dendrite from primary her-

pes zoster virus (HZV) infection. (B) Neu-
rotrophic ulcer following herpes zoster
ophthalm icus. (C) Corneal melt after HZV
C necrotizing stromal keratitis.
Management
Oral an t ivirals (acyclovir, valacyclovir, or fam ciclovir) reduce viral sh edding from
lesion s an d decrease th e in ciden ce an d severit y of th e m ost com m on ocular com -
plicat ion s for h erpes zoster oph th alm icu s. Oral th erapy, if begu n w ith in 72 h ou rs
of sym ptom on set , m ay redu ce th e in ciden ce an d durat ion of posth erpet ic n eu ral-
gia. Treat m en t is t ypically used for 7 to 10 days (valacyclovir, 1 g th ree t im es a day;
acyclovir, 800 m g five t im es daily; or fam ciclovir, 500 m g th ree t im es a day. Topical
an t ivirals are n ot effect ive in h erpes zoster. In t raven ous an t ivirals are in dicated
for p at ien ts at risk for system ic dissem in at ion due to severe im m u n osu ppression .
5 Cornea 145
Topical cycloplegia an d topical cor t icosteroids can be ben eficial for severe st rom al
scarring an d uveit is.
Epstein-Barr Virus
Epstein -Barr viru s (EBV) is a DNA virus in th e h erpesviru s fam ily. Typically t ran s-
m it ted from saliva w ith a su bclin ical in fect ion m ost com m on ly occu rring in th e
first decade of life. In th e laten t period th e viru s is dorm an t in B lym ph ocytes an d
m u cosal epith elial cells of th e ph ar yn x. Ocular disease is un com m on bu t can occu r.
In fect ion later in life causes m on on ucleosis.
Presentation
Pain , redn ess, an d decreased vision along w ith an acu te follicular conju n ct ivit is
can occu r w ith en largem en t of th e lacrim al glan ds an d lym p h aden opathy. Corn eal
fin dings in clude epith elial den drites or pu n ctate kerat it is, corn eal n eovasculariza-
t ion , an d in terst it ial an d/or st rom al kerat it is. A n um m ular kerat it is as seen w ith
HZV or HSV can occur ( Fig. 5.3 ).
See th e differen t ial for den drit ic kerat it is an d st rom al kerat it is listed earlier.
Management
Diagn osis is depen den t on th e detect ion of EBV an t ibodies to variou s viral com -
pon en ts. Viral capsid an t igen s app ear in acu te in fect ion w ith im m u n oglobulin M
(IgM) an t ibodies appearing first follow ed by IgG an t ibodies. An t ibodies to early
an t igen s also occur in acute in fect ious st ages an d decrease to low or u n detect able
after in it ial in fect ion . An t ibodies to EBV n u clear an t igen s ap pear w eeks to m on th s
after in fect ion an d can be used as a m arker of previou s EBV in fect ion . Support ive
t reat m en t w ith topical lu brican t drop s, gels, or oin t m en t s can im prove epith elial
kerat it is. Oral an t ivirals rem ain un st udied w ith EBV st rom al kerat it is. Topical cor-
t icosteroids m ay redu ce su bepith elial in filt rates, n u m m u lar kerat it is, an d corn eal
n eovascularizat ion .
Fig. 5.3 Multiple cornea stromal opacities from Epstein-Barr

virus stromal keratitis.
Bacterial Corneal Ulcer

An in fect iou s in filt rate of th e corn ea, bacterial corn eal ulcer is also kn ow n as a bac-
terial corn eal u lcer. Risk factors in clude con tact len s w ear (m ost com m on cause),
corn eal t rau m a, an d an altered corn eal surface such as a corn eal abrasion or
pun ct ate keratopathy. Any disorder com prom ising th e corn eal epith elial in tegrit y
can lead to a bacterial in fect ion . Com m on gram -posit ive bacterial cau ses in clude
Staphylococcus species, St reptococcus species, an d Bacillus species (com m on af-
ter t rau m a). More com m on gram -n egat ive bacterial causes in clude Pseudom onas
aeruginosa , Proteus, Enterobacter, an d Serrat ia .
Presentation
Often acu te pain , redn ess, ph otoph obia, tearing, an d disch arge. A w h ite spot m ay
be visible to th e pat ien t th at m ay represen t eith er th e in filt rate w ith in th e corn ea
or a layered hypopyon if in th e low er port ion of th e corn ea. Bacterial kerat it is m ay
presen t w ith con curren t corn eal edem a, corn eal st rom al th in n ing, n ecrosis, pu ru-
len t debris w ith in th e u lcer, an d w h ite blood cell in filt rat ion w ith in th e corn ea or
th e an terior ch am ber ( Fig. 5.4 ).
Viral cau se (part icu larly HSV or HZV), fu ngal cause, Acantham oeba , sh ield ulcer
from vern al keratoconjun ct ivit is, im m un e-m ediated corn eal u lcer, sterile m argin al
ulcer, staphylococcal m argin al kerat it is
Management
Obtain a h istor y of con t act len s w ear, t raum a, or foreign body.
Obtain an ocular history of system ic disease or previous eye conditions that m ay pre-
dispose to breakdow n or instability of the corneal epithelium (exposure keratopathy,
recurrent erosion, dry-eye disease, corneal abrasions, eyelid abnorm alities).
Obt ain a corn eal scraping for various cu lt ure m edia an d a Gram st ain or pot as-
sium hydroxide prep on glass slides. Con sider cu lt u ring con t act len ses, cases,
an d solut ion w h en available.
Fig. 5.4 Central bacterial corneal ulcer, epithelial/mucous

debris, and hypopyon from Pseudom onas aeruginosa .
5 Cornea 147
Large cen t ral u lcers sh ould be placed on broad-spect rum for t ified topical an -
t ibiot ics u sing eith er cefazolin (50 m g/m L) or van com ycin (25 or 50 m g/m L),
on e drop ever y 30 m in u tes for gram -p osit ive coverage along w ith a topical
am in oglycoside for gram -n egat ive coverage. A com m on agen t is tobram ycin
(14 m g/m L), on e drop ever y 30 m in utes. Sm all periph eral u lcers can be t reated
w ith m on oth erapy w ith a fluoroquin olon e u sed on e drop ever y 30 m in utes.
Treat m en t can be adju sted w ith close follow -up exam in at ion s an d by ut ilizat ion
of bacterial cu lt u re resu lt s, in cluding iden t ificat ion an d m edicat ion sen sit ivi-
t ies/su scept ibilit ies. Som e physician s m ay also u se a loading dose of an t ibiot ics
ever y 15 m in u tes for th e first 2 h ou rs.
Cycloplegia (h om at ropin e or scopolam in e, on e drop t w ice a day) is h elpfu l for
pain relief from ciliar y spasm an d preven t ion of posterior syn ech iae.
Con tact len s w ear sh ould be discon t in u ed w h en app licable.
Hospit alizat ion sh ould be con sidered if com p lian ce is an issue w ith m edicat ion
use.
Aca ntha moeba

Acan th am oeba kerat it is is a corn eal in fect ion or u lcerat ion caused by a u biqu itou s
protozoan foun d in fresh w ater an d soil. It exist s as a dorm an t cyst or an act ive
m obile form kn ow n as a t roph ozoite. Most cases occu r in associat ion w ith p oor
con tact len s hygien e, con t act len s w ear in fresh w ater sources, or clean ing con tact
len ses in fresh w ater, w ell w ater, or h om em ade salin e solut ion s.
Presentation
Pat ien ts experien ce acu te, severe pain , often ou t of proport ion to corn eal fin d-
ings. Associated redn ess, ph otoph obia, tearing, an d blurred vision p rogress over
th e cou rse of several w eeks. Seek a con t act len s h istor y or fresh w ater exp osure
in associat ion w ith con t act len s w ear or clean ing. Fin dings begin w ith a pu n ctate
epith elial kerat it is follow ed by su bepith elial h aze an d in filt rat ion . A radial peri-
n eurit is m ay be seen along w ith pseudoden drit ic kerat it is. A late an d om in ous
fin ding is st rom al ulcerat ion , n ecrosis, an d su bsequen t ring u lcerat ion w ith kera-
tolysis ( Fig. 5.5A,B,C).
HSV, HZA, bacterial kerat it is, fu ngal kerat it is, im m un e-related u lcer
Management
Obtain a corn eal scraping or corn eal biopsy. Ut ilize calcofluor w h ite, Giem sa,
H&E, or Gram st ain to iden t ify cyst s of t roph ozoites. Corn eal cult u re on n on -
n ut rien t agar w ith Escherichia coli overlay can detect in fect ion . Con sider cult u r-
ing th e con t act len s or case.
Con focal m icroscopy w h en available can be ben eficial for iden t ificat ion of th ese
st ru ct u res in th e absen ce of severe st rom al u lcerat ion .
Discon t in ue con t act len s w ear.
Com bin at ion th erapy is m ost effect ive, bu t early diagn osis is th e m ost crit ical
factor for su ccessful t reat m en t:
Diam idin es (propam idin e, h exam idin e)
Biguan ides (ch lorh exidin e diglu con ate, polyh exam ethylen e bigu an ide, alexi-
din e)
Fig. 5.5 (A) Acantham oeba keratitis with severe punctate

keratopathy and early central corneal haze. (B) Central corneal
ulcer and radial perineuritis from Acantham oeba . (C) Ring ul-
cer and stromal necrosis from late Acantham oeba keratitis.
Am in oglycosides (parom om ycin , n eom ycin )

Im idazoles/t riazoles (clot rim azole, it racon azole, ketocon azole, m icon azole,
m et ron idazole)
5 Cornea 149
Biguan ides h ave becom e first-lin e th erapy becau se of cyst icidal act ivit y, an d
th ey can be u sed alon e or in com bin at ion plus a diam idin e an d/or a topical or
oral am in oglycoside agen t .
Treat m en t is often n eeded for m any m on th s.
Cor t icosteroids sh ould be avoided if possible un less severe in flam m at ion an d
pain do n ot im prove. Careful obser vat ion m ust occu r if cor t icosteroids are used
becau se of poten t ial react ivat ion of t roph ozoites from dorm an t cyst s.
Keratoplast y tech n iques m ay be n eeded, bu t outcom es im prove if surger y can
be delayed after several m on th s of an t iam oeba t reat m en t .
Fungal Keratitis
Fu n gal in fe ct ion involvin g t h e cor n ea. Risk factors m ost com m on ly in clu d e
t rau m a involvin g breakd ow n of t h e cor n eal ep it h eliu m from p lan t or veget a-
ble m at te r. Trau m a from con t act le n s w ear is an ot h er r isk factor, alt h ough a
relat ively n ew r isk factor from con t act le n s w ear in clu d es clean in g solu t ion s,
as se en w it h t h e n ow d iscon t in u e d ReNu w it h Moist u re Loc solu t ion (for m erly
m an u fact u re d by Bau sch & Lom b, Roch ester, NY), an d t h e d evelop m e n t of a Fu -
sariu m kerat it is ou t break w orldw id e. System ic an d top ical cor t icosteroid s are
ad d it ion al r isk factors to fu n gal in fe ct ion an d can in cite d ram at ic p rogression
of a fu n gal kerat it is or u lce rat ion . Com m on fu n gal agen t s m ay in clu d e Candida ,
Fusarium , Paecilom yces , an d Aspergillus sp e cies am on g ot h ers.
Presentation
Fungal kerat it is in fect ion s are m ore in dolen t th an bacterial or viral corn eal in fec-
t ion s un less topical cor t icosteroids h ave been u sed. Sym ptom s in clu de eye pain ,
redn ess, tearing, ph otoph obia, an d decreased vision . Th e h istor y can be an im p or-
tan t factor in assessing th e risk of fungal in fect ion s. A w h ite spot over th e corn ea
is com m on ly seen from eith er th e st rom al ulcer or hypopyon . An terior uveit is,
corn eal edem a, posterior corn eal plaques, an d a layered hypopyon are com m on
( Fig. 5.6 ).
Bacterial, viral, or Acantham oeba kerat it is
Fig. 5.6 Fungal corneal ulcer and hypopyon from Candida

albicans in im munosuppressed patient.
Management
All corn eal ulcers sh ou ld be t reated as w ith bacterial kerat it is in it ially an d star ted
on broad-sp ect ru m topical an t ibacterial m edicat ion s un t il cu lt ure-proven fu ngal
in fect ion is iden t ified from corn eal scrapings or cu lt ure m edia. Scrapings w ith Gi-
em sa, periodic acid–Sch iff, or Grocot t- Gom ori m eth en am in e-silver n it rate st ain s
can iden t ify fu ngal elem en ts. Cult ure m edia sh ou ld in clu de eith er Sabou raud agar
or brain –h ear t in fu sion broth . Nat am ycin 5%is th e t reat m en t of ch oice for filam en -
tous Fusarium in fect ion s, w h ereas topical m icon azole 1%is th e t reat m en t of ch oice
for Paecilom yces. Am ph otericin B 0.15% or topical voricon azole is th e t reat m en t
of ch oice for yeast kerat it is an d Aspergillus in fect ion . Drops are t ypically star ted
ever y 1 to 2 h ou rs an d often supp lem en ted w ith oral an t ifu ngal im idazoles or vori-
con azole. Corn eal debridem en t m ay be n eeded in su bsequ en t visit s to im prove
topical m edicat ion pen et rat ion . Surgical keratoplast y tech n iqu es m ay be n eeded
in severe cases w ith keratolysis or sign ifican t n on respon sive posterior corn eal
plaqu es. Treat m en t is often n eeded for several m on th s, an d cor t icosteroids sh ould
be avoided du ring th is t im e. Cycloplegia m ay be ben eficial in cases w ith sign ifican t
uveit is or hypopyon to preven t p osterior syn ech iae form at ion .
Corneal Inflammation and Surface Disorders
Interstitial Keratitis
Th is is a n on suppu rat ive in flam m at ion of th e corn eal st rom a th at t ypically in -
volves corn eal n eovascularizat ion an d even t ual lipid deposit ion an d scarring. Most
cases resu lt from a hypersen sit ivit y react ion to in fect ious m icrobes or associated
an t igen w ith in th e corn ea. Several viral, bacterial, an d h elm in th in fect ion s are as-
sociated w ith th is con dit ion .
Presentation
In it ial sym ptom s in clu de pain , tearing, p h otoph obia, an d perilim bal inject ion . Ke-
rat ic precipit ate an d an terior uveit is m ay develop. With progression , deep st rom al
n eovascularizat ion develops w ith cen t ral spread. Corn eal scarring an d edem a m ay
en sue. A salm on -pin k patch m ay appear w ith in th e corn ea from severe st rom al
n eovascularizat ion cases. Gh ost vessels even t u ally develop w ith lipid an d scar for-
m at ion . St rom al scarring an d opacificat ion can becom e severe. ( Fig. 5.7A,B).
Congenital syphilis (usually bilateral and develops late in the first decade of life)
Acqu ired syph ilis (rare an d usu ally u n ilateral w ith occu rren ce in adu lth ood)
Viral corn eal in fect ion s (HSV, HZV, EBV, m u m ps, rubeola)
Tu bercu losis (con firm w ith Tb skin test)
Leprosy
Chlam ydia t rachom at is (h istor y of sexu al con t act)
Helm in th in fect ion s [Onchocerca volvulus (on ch ocerciasis) from foreign t ravel in
en dem ic region ]
Lym e disease (h istor y of t ick exposu re; ch eck Lym e t iters)
Sarcoidosis (ch est x-ray, ser um calcium , lysosym e, an d angioten sin -conver t ing
en zym e level)
Cogan syn drom e (autoim m u n e disorder w ith in terst it ial kerat it is, ver t igo, an d
h earing loss)
5 Cornea 151
Fig. 5.7 (A) Interstitial keratitis from congenital syphilis with

ghost vessels on retroillum ination. (B) Herpes simplex virus
interstitial keratitis and corneal scarring.
Management
Treat m en t begin s w ith iden t ificat ion of th e cau se. Serological test ing is ver y im -
por tan t in m aking a diagn osis. A screen ing test such as a Rapid Plasm a Reagin (RPR)
or a t repon em e-specific test su ch as Flu orescen t Tropon em al An t ibody (FTA-ABS)
or m icroh em agglut in at ion assay (MHA-TP) can detect syph ilis. System ic t reat m en t
is in dicated depen ding on th e u n derlying cau se (e.g., system ic an t ibiot ics w ith
syph ilis, t u bercu losis, Lym e disease). In flam m at ion in in terst it ial kerat it is cases is
con t rolled w ith topical cor t icosteroids an d cycloplegic agen t s on ce an in fect ious
cau se is elicited. See th e sect ion on HSV an d HZV for t reat m en t .
Fig. 5.8 Punctate keratitis and

multiple corneal opacities t ypical
of Thygeson superficial punctate
keratitis.
Thygeson Superficial Punctate Keratitis

Th is n on specific su perficial pun ct ate kerat it is t ypically occurs in you ng ch ildren to
older adu lts. Th e cau se is u n kn ow n , alth ough specu lat ion exists as to w h eth er a vi-
rus is th e u n derlying agen t . Mult iple corn eal lesion s develop bu t t ypically respon d
rapidly to cor t icosteroids, suggest ing an im m un ological basis for kerat it is.
Presentation
Sym ptom s in clude recu rren t episodes of tearing, ph otoph obia, foreign body sen sa-
t ion , an d m ild blurred vision . Conju n ct ival inject ion is usually m in im al or absen t .
Corn eal fin dings in clu de m u lt iple raised epith elial lesion s th at are gray to w h ite
gran ular opacit ies th at w a x an d w an e in locat ion an d n u m ber an d are u sually bi-
lateral w ith poten t ial asym m et r y ( Fig. 5.8 ).
Subepith elial in filt rates w ith epidem ic keratoconju n ct ivit is (EKC), EBV, staphylo-
coccal hypersen sit ivit y, con tact len s over w ear kerat it is
Management
Su ppor t ive t reat m en t w ith topical lubrican t tears or gels w ith or w ith out ban dage
con tact len s w ear can be u sefu l in m ilder cases. Low -dose topical cort icosteroids
can prom ote rapid resolut ion of corn eal lesion s; h ow ever, lesion s t ypically recur
w ith discon t in uat ion of drops. Long-term cort icosteroid t reat m en t w ith flu oro-
m eth olon e 0.1% or lotepredn ol 0.5% can w ork w ell at var ying dose regim en s w ith
slow ly tapering doses from an in it ial dosing of fou r t im es a day. Topical cyclosp o-
rin e an d top ical t rifluridin e h ave also been u sed w ith an ecdot al success. Th e le-
sion s even t u ally resolve, bu t th is m ay take m on th s to years for perm an en t resolu -
t ion of recu rren t episodes.
Shield Ulcer
Sh ield ulcer is a n on in fect ious corn eal u lcer in th e set t ing of vern al keratoconju n c-
t ivit is th at is often bilateral an d season al (m ore com m on in spring). A h istor y of
atopy is com m on . Th e con dit ion m ore often presen ts in m ales.
5 Cornea 153
Fig. 5.9 Peripheral vernal shield ulcers seen in vernal kerato-

conjunctivitis.
Presentation
Presen tat ion in cludes itch ing, ph otoph obia, foreign body sen sat ion , blu rred vision ,
redn ess, an d m ucou s disch arge. Sh ield ulcer can be associated w ith gian t papillar y
conju n ct ivit is an d lim bal follicles kn ow n as Horn er-Tran t as dots (collect ion s of de-
gen erated eosin op h ils an d epith elial cells). A su perior pu n ctate keratopathy t yp i-
cally develops follow ed by a breakdow n of epith elium an d even t ual oval-sh aped
ulcers (sh ield ulcers) w ith u n derlying st rom al opacificat ion . Associat ion can occur
w ith keratocon u s an d floppy eyelid syn drom e ( Fig. 5.9 ).
Atopic keratoconju n ct ivit is, in fect ious u lcer, im m u n e-m ediated u lcer
Management
Pat ien t s sh ou ld avoid p oten t ial allergen s an d u se cool com p resses, p reser va-
t ive-free lu br ican t s, top ical an d /or system ic an t ih ist am in e or m ast cell st abiliz-
ers, an d top ical or system ic cor t icosteroid s. Alter n at ive top ical or system ic im -
m u n osu p p ressan t agen t s su ch as cyclosp or in e A m ay p rovid e ben efit w it h close
follow -u p .
Exposure Keratopathy
Exposu re keratopathy is also referred to as n europaralyt ic keratopathy. Causes in -

clude facial n er ve palsy, severe proptosis, an d scarring of th e lids. Th e con dit ion
m ay be associated w ith w eak Bell p h en om en on . It is th e resu lt of im proper w et t ing
of th e ocu lar su rface by th e tear film .
Presentation
Sym ptom s range from m ild in ferior pu n ctate epith elial ch anges to severe corn eal
m elt ing w ith corn eal perforat ion .
Neu rot roph ic keratopathy, recu rren t erosion s, in fect iou s keratopathy, dr y eye, au -
toim m un e diseases
Management
Frequen t in st illat ion s of lubrican t s an d t aping th e lids form th e m ain stay of th e
t reat m en t . Lid surgeries to correct th e lid abn orm alit ies m ay be required.
Filamentary Keratitis
An ocu lar surface in flam m ator y con dit ion ch aracterized by m ucou s plaques an d
st ran ds of th e corn eal surface. Filam en ts represen t st ran ds of epith elial cells at-
tach ed to th e corn eal su rface over a core of m u cus w ith firm at t ach m en t to th e
adjacen t corn eal n er ves.
Com m on con dit ion s associated w ith th is fin ding in clude eyelid m alposit ion su ch
as lagop h th alm ia an d poor eyelid blin k, con tact len s over w ear kerat it is, atop ic con -
dit ion s, an d ch ron ic ocu lar surface in flam m ator y con dit ion s such as su perior lim -
bic keratoconju n ct ivit is, aqueou s tear deficien cy, an d dr y-eye syn drom e.
Presentation
Pat ien ts experien ce pain , foreign body sen sat ion , redn ess, dr yn ess, an d ph otop h o-
bia. Mu cous plaqu es in st ran ds or globules are visu alized en t rapped w ith in th e
corn eal epith elium . Th e filam en t s st ain w ith fluorescein dye. Conju n ct ivoch alasis,
pun ct ate corn eal st ain ing bet w een filam en ts, an d a low tear m en iscus are com -
m on associated fin dings ( Fig. 5.10 ).
Fig. 5.10 Filamentary keratitis.

5 Cornea 155
Recurren t erosion s, epith elial defect s
Management
Debridem en t of th e filam en t s can be perform ed w ith a cot ton -t ipped applicator
follow ing a topical an esth et ic. Lu brican t drops, gels, or oin t m en ts can be ben efi-
cial from four t im es a day to h ou rly dosing. Sodium ch loride drops or acet ylcys-
tein e drops can also be used four t im es a day. Addit ion al t reat m en t s m ay in clu de a
th erapeu t ic ban dage len s an d autologou s ser um drops. Pun ct al plugging sh ou ld be
avoided u n t il filam en t recu rren ce is preven ted.
Neurotrophic Keratopathy
Neu rot roph ic keratopathy is a ch ron ic surface disorder resu lt ing from hypoesth e-
sia or an esth esia of th e t rigem in al n er ve. A variet y of factors, in cluding HSV, HZV,
radiat ion th erapy, previou s brain t um or resect ion , or t rau m a to th e t rigem in al
n er ve, as w ell as diabetes, topical an esth et ic or preser vat ive abu se, ch em ical burn s,
an d leprosy are associated w ith th is con dit ion .
Presentation
Blurred vision , redn ess, an d ch ron ic epith elial defects or ulcerat ion are seen at pre-
sen tat ion , along w ith pu n ctate corn eal stain ing in th e cen t ral or in ferior corn ea
w ith or w ith out associated epith elial defects. Corn eal th in n ing, filam en t ar y kerat i-
t is, scarring, corn eal surface asperit y, an d an elevated hyperplast ic epith eliopathy
can be associated fin dings ( Fig. 5.11 ).
Fig. 5.11 Neurotrophic keratopathy.

Corn eal abrasion , recu rren t erosion , in fect ious u lcer, dr y-eye syn drom e, an d au-
toim m un e disease
Management
Identify the underlying cause. Lubricant drops, gels, and ointm ents can protect the cor-
neal surface from further breakdow n. Temporary or perm anent punctal closure can also
benefit. A tarsorrhaphy is often needed to decrease the am ount of exposed surface area
to the cornea. Therapeutic bandage lens wear should be used cautiously w ith close fol-
low-up. A conjunctival or am niotic m em brane graft can be used in severe situations,
w ith lam ellar or penetrating keratoplasty used in cases of severe corneal m elting or per-
foration. Autologous serum drops can also be a useful adjunct to treatm ent.
Recurrent Erosion
Recurren t corn eal erosion (RCE) syn drom e is ch aracterized by a dist urban ce at th e
level of th e corn eal epith elial basem en t m em bran e, resu lt ing in defect ive adh e-
sion s an d recu rren t breakdow n s of th e epith elium . Com m on cau ses of recu rren t
erosion s in clude epith elial basem en t m em bran e dyst rophy, corn eal inju ries, alkali
burn s, foreign bodies, post in fect iou s ulcers from h erp es sim plex, Cockayn e syn -
drom e, Reis-Bü cklers dyst rophy, p ost vit rectom y, ph otocoagu lat ion , an d con tact
len ses, am ong oth ers.
Presentation
Many pat ien t s (80 to 90%) are asym ptom at ic. Som e can presen t w ith pain , blurred
vision , ast igm at ism , epith elial blebs, an d foreign body sen sat ion w ith recu rren t
erosion . Pat ien t s can presen t w ith a variet y of sign s such as epith elial loss, epi-
th elial m icrocyst s, bu llae, lack of adh eren ce of sh eet s of epith elium , epith elial
filam en t form at ion , corn eal abrasion , brow n ish gran u lar edem a (braw ny edem a),
an d areas of h ealed epith eliu m , w h ich m ay even resem ble a den drit ic figu re, a
pseu doden drite.
Corn eal abrasion , corn eal dyst rophy, corn eal foreign bodies, dr y eye, an d in fect ive
kerat it is
Management
Man agem en t of RCE syn drom e is u su ally aim ed at regen erat ing or repairing th e
epith elial basem en t m em bran e to restore th e adh esion bet w een th e epith elium
an d th e an terior st rom a. Recurren t corn eal erosion s respon d to top ical lu bricat ion
th erapy, ban dage soft con tact len ses, debridem en t , an t ibiot ic oin t m en t , an d patch -
ing. Su rgical opt ion s in clu de an terior st rom al pu n ct ure, excim er laser diam on d
burr keratectom y, n eodym iu m :yt t riu m -alu m in u m -garn et (Nd:yAG) laser t reat-
m en t , an d su perficial ph ototh erapeut ic keratectom y.
5 Cornea 157
Congenital Anomalies
Microcornea
Th e n orm al corn eal diam eter is n orm ally 9.5–10m m at bir th an d 10–12.5m m at
adulth ood.
Presentation
Th e term m icrocorn ea is given to an adu lt corn ea w ith less th an 10m m diam eter.
Most cases occur sporadically. Microcorn ea m ay occu r in isolat ion or par t of a gen -
erally sm all eye (m icroph th alm os). If it occurs as an isolated en t it y, th e n orm al len s
size cau ses a disparit y w ith th e sm all corn ea resu lt ing in angle-closu re glau com a.
Nan oph th alm os, m icrop h th alm os, sclerocorn ea
Management
Treat m en t is directed at any associated ocular abn orm alit ies
Megalocornea
Presentation
Megalocornea is the term given to the condition in w hich the corneal diam eter ex-
ceeds 11m m at birth, or 12m m after 2 years of age. It has been associated w ith ecto-
pia lentis, iris transillum ination, pigm ent dispersion, arcus and m ental retardation.
Buph th alm os, an terior ch am ber dysgen esis syn drom es. As com pared to buph th al-
m os, th e size of th e globe an d th e in t raocular pressu re are n orm al.
Management
Treat m en t is directed at any associated ocular abn orm alit ies
Anterior Embryotoxon
It is a congen ital an om aly of th e corn ea w h ich is n ot visually sign ifican t .
Presentation
An terior em br yotoxon refers to a congen it al broad superior lim bu s w ith an oth er-
w ise n orm al an terior ch am ber. It is also u sed to describe a congen it al arcu s, arcu s
juven ilis, sim ilar to th e arcus sen ilis th at occu rs in old in dividu als.
Posterior em br yotoxon , Arcus sen ilis, Axen feld Reiger syn drom e.
Management
Obser vat ion an d follow -u p.
Posterior Embryotoxon
It represents a thickened and anteriorly displaced Schwalbe’s line that is easily visible.
Presentation
Posterior em br yotoxon is th e m ost com m on an om aly of th e corn ea. It m ay be seen
as a w h ite ring n ear th e lim bus on slit-lam p exam in at ion an d as a prom in en t , an -
teriorly displaced Sch w albe’s lin e on gon ioscopy.
An terior em br yotoxon , Arcus sen ilis, Axen feld Reiger syn drom e.
Management
Obser vat ion an d follow -u p.
Sclerocornea
It is a congen ital an om aly of th e corn ea resu lt ing in decreased vision .
Presentation
Sclerocorn ea refers to a sclera-like appearan ce of th e corn ea, w h ich m ay be p e-
riph eral or involve th e w h ole corn ea. It is associated w ith corn eal flat ten ing an d
oth er an om alies of an terior ch am ber developm en t .
Microph th alm os, bu ph th alm os, an d oth er cau ses of opaque corn ea.
Management
Visual progn osis in severe cases is ver y p oor.
Dystrophies
Anterior Corneal Dystrophies
Epithelial Basement Membrane Dystrophy (EBMD)

Also kn ow n as Cogan m icrocyst ic edem a or m ap-dot-fingerprin t corn eal dyst ro-
phy, th is is perh ap s th e m ost com m on of all corn eal dyst roph ies. Bilateral (bu t
m ay be asym m et rical) w ith autosom al dom in an t in h erit an ce, bu t m ost cases are
sporadic. Th e con dit ion resu lts from abn orm al epith elial t urn over an d redun dan t
basem en t m em bran e.
Presentation
Pat ien ts m ay rem ain asym ptom at ic or m ay develop decreased vision , foreign body
sen sat ion , ph otop h obia, tearing, an d poten t ially pain fu l corn eal erosion s. Slit-lam p
exam in at ion of EBMD sh ow s gray-w h ite su bepith elial patch es (m aps), gray-w h ite
5 Cornea 159
Fig. 5.12 (A) Fingerprint pat tern seen with epithelial base-
ment m em brane dystrophy (EBMD). (B) Map pat tern seen
with EBMD.
parallel lin es (fingerprin ts), or su bepith elial m icrocysts (dots). Th e fin dings are
best seen on re t roillu m in at ion ( Fig. 5.12A,B).
Trau m at ic corn eal erosion , Meesm an n dyst rophy, early Reis-Bü cklers dyst rophy,
corn eal in t raepith elial n eop lasia (CIN)
Management
No t reat m en t is required for asym ptom at ic cases. For sym ptom at ic cases, sodiu m
ch loride drops or oin t m en t can be ben eficial. Pat ien ts w ith con curren t dr y-eye
disease sh ould use addit ion al preser vat ive-free ar t ificial tears or gels. Th erapeu -
t ic ban dage len ses can provide tem porar y relief. Surgical opt ion s in clu de an terior
st rom al pun ct u re, Na:Yag st rom al pu n ct u re, epith elial debridem en t , ph ototh era-
peut ic keratectom y, or diam on d bu rr keratectom y.
Fig. 5.13 Meesmann dystrophy.
Meesmann Dystrophy
Meesm an n dyst rophy is a rare bilateral au tosom al dom in an t con dit ion occu rring
early in life th at resu lts from a th icken ed basem en t m em bran e w ith a fibrogran u -
lar “pecu liar su bstan ce” (possibly hyalin e) w ith in th e ep ith elial cells.
Presentation
Pat ien ts m ay presen t w ith pain , blu rred vision , ph otoph obia, tearing, an d redn ess
from recu rren t erosion s. Tiny epith elial vesicles are seen from lim bus to lim bu s,
m ost clearly on ret roillu m in at ion ( Fig. 5.13 ).
Cyst in osis, corn eal gu t tae, epith elial basem en t m em bran e dyst rop hy (EBMD),
t raum at ic recu rren t erosion
Management
No t reat m en t is requ ired u n less recu rren t erosion s develop. Sim ilar t reat m en ts
m ay be perform ed as w ith EBMD, alth ough a deep an terior lam ellar keratoplast y
can also be useful in cases recalcit ran t to recurren t erosion t reat m en t opt ion s.
Reis-Bücklers Dystrophy
Reis-Bü cklers dyst rophy is a progressive bilateral autosom al dom in an t dyst rophy
th at develops early in life. Gen et ic lin kage occurs from a m u tat ion of th e BIGH3
gen e on ch rom osom e 5q31.
Presentation
Pat ien ts presen t w ith recurren t erosion sym ptom s of blurred vision , pain , ph oto-
ph obia, tearing, an d redn ess. Exam in at ion reveals a superficial gray-w h ite ret icu-
lar h aze m ore con cen t rated in th e cen t ral corn ea. Sign ifican t scarring can lead to
Salzm an n n odules an d an irregu lar corn eal su rface ( Fig. 5.14 ).
5 Cornea 161
Fig. 5.14 Reis-Bücklers dystrophy.
Meesm an n dyst rophy, EBMD, gran u lar dyst rophy
Management
In it ial t reat m en t is directed at recu rren t corn eal erosion t reat m en t as w ith EBMD.
Surgical p rocedu res m ay in clude lam ellar keratoplast y, pen et rat ing keratoplast y,
or ph ototh erapeut ic keratectom y. Recu rren ce is com m on w ith p en et rat ing kera-
toplast y.
Stromal Corneal Dystrophies
Granular Dystrophy
Gran u lar dyst rophy is a com m on bilateral au tosom al dom in an t st rom al dyst rophy
lin ked to a m ut at ion in th e BIGH3 gen e on ch rom osom e 5q31. St rom al deposits
con sist of hyalin e, w h ich can be h igh ligh ted w ith Masson t rich rom e stain on cor-
n eal h istopath ology. Several form s of th is dyst rophy exist .
Presentation
Sym ptom s t ypically occur as a result of recurren t erosion s an d m ay in clude blu rred
vision , pain , foreign body sen sat ion , ph otoph obia, an d tearing. Exam in at ion sh ow s
bread crum b –like op acit ies, t yp ically in th e cen t ral por t ion of th e an terior st rom a,
w ith sparing of th e lim bu s ( Fig. 5.15 ).
Avellin o dyst rophy, m acular dyst rophy
Management
Treat m en t is directed at recurren t erosion s. Ph ototh erapeu t ic keratectom y, lam el-
lar keratoplast y, an d pen et rat ing keratoplast y can be u sed for t reat m en t . Kerato-
plast y h as a good progn osis in cases w ith poor vision , bu t recu rren ce is possible.
Fig. 5.15 Granular corneal dystrophy.
Lattice Dystrophy
Th is is a bilateral autosom al dom in an t st rom al dyst rophy lin ked to a m u t at ion in
th e BIGH3 gen e on ch rom osom e 5q31. St rom al deposits con sist of am yloid, w h ich
can be h igh ligh ted on corn eal h istopath ology w ith Congo-red stain . Several t ypes
of th is dyst rophy exist , in clu ding an au tosom al recessive form .
Presentation
Pat ien ts are asym ptom at ic un less recurren t erosion s develop . Decreased vision
can resu lt from deposit s. Exam in at ion reveals cen t ral refract ile bran ch ing lin es
w ith in th e an terior corn eal st rom a th at are best seen on ret roillu m in at ion . Th e
lim bus is spared ( Fig. 5.16A,B).
A B
Fig. 5.16 (A) Lat tice corneal dystrophy. (B) Lat tice corneal dystrophy on retroillumina-
tion. (Both im ages courtesy of Mark J. Mannis, MD)
5 Cornea 163
En larged corn eal n er ves, gh ost vessels of in terst it ial kerat it is
Management
No m an agem en t is required if th e p at ien t is asym ptom at ic. Treat recurren t ero-
sion s as described in sect ion on Recu rren t Erosion s (p . 156). Recurren ce is com -
m on despite corn eal t ran sp lan tat ion , bu t th e procedure can be ben eficial in som e
pat ien t s w ith a good long-term progn osis.
Macular Dystrophy
Macu lar dyst rophy is a rare bilateral au tosom al recessive corn eal dyst rophy w ith
m u copolysacch aride deposit ion in any or all p or t ion s of th e st rom a. Alcian blue
stain can detect th e dep osits on corn eal h istopath ology.
Presentation
Th ere m ay be recu rren t erosion sym ptom s. Decreased vision can occu r early in th e
cou rse. Exam in at ion sh ow s gray-w h ite opacit ies w ith p oor m argin s, separated by
in ter ven ing h aze or clou din ess w ith in th e st rom a. Lesion s often coalesce w ith t im e
an d often involve th e en t ire corn ea, lim bu s to lim bu s (Fig. 5.17 ).
At ypical lat t ice or gran u lar dyst rophy
Management
Recurren t erosion sym ptom s can be t reated. Pen et rat ing keratoplast y or deep la-
m ellar keratoplast y is often th e on ly surgical opt ion because of th e depth of st ro-
m al opacit ies. Larger graft sizes m ay lim it recu rren ces.
Fig. 5.17 Early macular corneal dystrophy.

Fig. 5.18 Central crystalline dystrophy of Schnyder. (Cour-

tesy of Mark J. Mannis, MD)
Schnyder Corneal Dystrophy

Th is con dit ion is a bilateral, slow ly progressive autosom al dom in an t st rom al dys-
t rop hy, w h ich is often referred to as Sch nyder cr ystallin e corn eal dyst rophy. It can
be detected as early as 1 year of age w ith accum ulat ion of ch olesterol an d ph os-
ph olipids w ith in th e st rom a secon dar y to abn orm al corn eal lipid m et abolism .
Presentation
Decreased vision is a presen t ing sym ptom . Exam in at ion sh ow s cen t ral corn eal
opacificat ion w ith sparing of th e lim bu s. Th e epith elium rem ain s in tact w ith as-
sociated corn eal arcus developm en t in th e secon d or th ird decade of life. Su bepi-
th elial cr ystals m ay be presen t along w ith m idperiph eral corn eal opacificat ion in
som e cases ( Fig. 5.18 ).
Oth er st rom al dyst roph ies, cen t ral corn eal scars, hyp erlip oprotein em ia
Management
Ch eck th e pat ien t’s fast ing lip id p rofile because 50% h ave elevated ch olesterol. If
vision declin e progresses, pen et rat ing keratoplast y is w arran ted, w ith a p oten t ial
for recurren ce.
Fleck Dystrophy
Fleck dyst rophy is an un com m on n onprogressive au tosom al dom in an t con dit ion
th at begin s early in life. Affected keratocytes con tain t w o abn orm al subst an ces:
glycosam in oglycan an d lipids.
5 Cornea 165
Presentation
Discrete, flat , gray-w h ite, dan dru ff-like opacit ies ap pear th rough out th e st rom a.
Sym ptom s are m in im al, an d vision is n ot affected. Th e con dit ion m ay be associated
w ith decreased corn eal sen sat ion , lim bal derm oid, keratocon u s, atopy, or pseudo-
xan th om a elast icum .
Posterior polym orph ou s dyst rophy, pre–Descem et dyst rophy, ich thyosis
Management
No t reat m en t is requ ired.
Central Cloudy Dystrophy of François

Th is is a bilateral sym m et rical st rom al dyst rophy th at is slow ly progressive an d
au tosom al dom in an t . It can be associated w ith m egalocorn ea.
Presentation
Patients are asym ptom atic. Exam ination shows central corneal opacities w ith poly-
gonal gray areas separated by intervening clear zones resem bling cracks. Haze extends
into the anterior corneal strom a unlike w ith posterior crocodile shagreen (Fig. 5.19 ).
Posterior crocodile sh agreen , oth er st rom al dyst roph ies
Management
No t reat m en t is n ecessar y because vision is usu ally n ot redu ced.
Pre–Descemet Dystrophy
Th is is an acqu ired con dit ion .
Fig. 5.19 Central cloudy dystrophy of François.

Presentation
Pat ien ts are gen erally asym ptom at ic bu t visu al acu it y can be affected. Sm all lin ear
or pu n ctate gray-w h ite flecks are appreciated in th e deep st rom a. Th e con dit ion
can be associated w ith keratocon us, posterior polym orph ous dyst rophy, an d epi-
th elial basem en t m em bran e dyst rophy.
Fleck dyst rophy, posterior polym orph ou s dyst rophy, ich thyosis
Management
No t reat m en t is requ ired .
Posterior Amorphous Stromal Dystrophy

Th is is a rare, bilateral, slow ly progressive, au tosom al dom in an t con dit ion presen t-
ing in ch ildh ood.
Presentation
Pat ien ts p resen t w ith diffu se, gray-w h ite lesion s in th e posterior st rom a, usu ally
involving th e cen t ral area, bu t m ay involve up to th e lim bu s. Corn eal th in n ing w ith
flat topography an d result ing hyperopia, cen t ral corn eal th in n ing, an d periph eral
iris processes m ay be seen .
Management
Rigid gas perm eable con t act len ses are u sed to correct th e ast igm at ism . Visu al acu-
it y is rarely affected.
Congenital Hereditary Stromal Dystrophy

Th is is an au tosom al dom in an t , bilateral, sym m et rical, n onprogressive st rom al
dyst rop hy seen in th e n ew born .
Presentation
Cen t ral corn eal clou ding is seen in th e n ew born w ith sparing of th e periph eral
st rom a. In fan t s develop am blyopia w ith nystagm u s an d squin t .
Congen it al h ereditar y en doth elial dyst rophy, congen it al glau com a, m ucopolysac-
ch aridosis, bir th t rau m a, posterior polym orph ous corn eal dyst rophy
Management
Pen et rat ing keratoplast y is th e t reat m en t of ch oice.
5 Cornea 167
Posterior Corneal Dystrophies
Corneal Guttae
Th is is a focal accum u lat ion of collagen on th e posterior surface of th e Descem et
m em bran e associated w ith en doth elial dysfun ct ion , corn eal clou ding, an d poten -
t ial visu al loss.
Presentation
Th e lesion appears as a w art or excrescen ces in relat ion w ith th e en doth elial cells.
Th e lesion s, called Hassall–Hen le bodies, ap pear at th e periph er y of th e corn ea an d
are associated w ith aging. A “beaten m et al” appearan ce, visible during th e specu lar
reflect ion , is associated w ith m elan in deposits.
Fu ch s en doth elial dyst rophy, Hassall-Hen le bodies
Management
Con sider specu lar m icroscopy. Han dling of th e corn ea m u st be gen tle during in t ra-
ocu lar surgeries becau se th is m ay h asten en doth elial com prom ise.
Fuchs Endothelial Dystrophy

Th is is a bilateral en doth elial dyst rophy result ing in progressive dam age to th e en -
doth elium . It is rarely sym ptom at ic before 50 years of age. Reduct ion in th e n u m -
ber an d fun ct ion of sodium an d pot assiu m aden osin e t riph osph at ase pum ps in th e
en doth eliu m occu r, creat ing progressive corn eal edem a an d gut tae. Th e con dit ion
is autosom al dom in an t or sporadic.
Presentation
Sym ptom s in clude decreased vision (w orse in th e m orn ing), foreign body sen sa-
t ion , ph otoph obia, tearing, an d pain . Exam in at ion resu lts can var y from m ild gut-
tae to severe m icrocyst ic an d st rom al edem a w ith bu llae form at ion . Th e Descem et
m em bran e becom es th icken ed w ith an in crease in corn eal th ickn ess as fluid reten -
t ion in creases w ith in th e corn eal st rom a. Su bepith elial fibrosis an d scarring m ay
occur in later st ages ( Fig. 5.20A,B,C).
Hassall-Hen le bodies, pseu doph akic or aph akic bullous keratopathy, Ch an dler syn -
drom e, h erpes sim plex kerat it is
Management
Begin w ith su ppor t ive th erapy, in cluding art ificial tears an d in part icu lar sodium
ch loride drops or oin t m en t . Hair dr yers can in crease fluid evaporat ion from th e
corn ea if used carefu lly. Defin it ive surgical t reat m en t in clu des eith er a pen et rat-
ing keratoplast y or en doth elial keratoplast y (becom ing a preferred tech n iqu e w ith
Fu ch s dyst rophy). Graft su r vival progn osis is good.
Fig. 5.20 (A) Gut tae seen on retroillumination in Fuchs en-

dothelial dystrophy. (B) Microcystic and strom al edema seen
in Fuchs endothelial dystrophy. (C) Fuchs endothelial dystrophy
with subepithelial bullae.
5 Cornea 169
Posterior Polymorphous Dystrophy

Th is t ype of dyst rophy is a bilateral en doth elial dyst rophy w ith gradu al progres-
sion . It m ay be au tosom al dom in an t or recessive an d h as been m apped to ch rom o-
som e 20q11.
Presentation
Decreased vision an d pain m ay develop, bu t pat ien t s are often asym ptom at ic. Ex-
am in at ion of th e posterior corn ea reveals grou ped vesicles, geograph ic gray le-
sion s, an d/or broad ban ds w ith scallop ed edges. Associated fin dings can in clu de
corn eal edem a, h aze, corectopia, an d iridocorn eal adh esion s as w ell as glau com a.
In fan t s m ay p resen t w ith cloudy corn eas ( Fig. 5.21A,B).
Iridio-corneal endothelial syndrom e, congenital hereditary endothelial dystrophy,
aphakic or pseudophakic bullous keratopathy, Fuchs dystrophy
Management
Pen et rat ing keratop last y is required for cases w ith sym ptom at ic decreased vision .
Obser ve carefu lly for con curren t open -angle glau com a.
Fig. 5.21 (A) Edema and haze seen

in posterior polymorphous dystro-
phy. (B) Broad bands seen on slit illu-
mination in posterior polymorphous
dystrophy. B
Fig. 5.22 Diffuse epithelial and

stromal edem a in child with congeni-
tal hereditary endothelial dystrophy.
Congenital Hereditary Endothelial Dystrophy

Th is is a bilateral corn eal dyst rophy w ith au tosom al dom in an t an d recessive form s.
It cau ses en doth elial dysfu n ct ion .
Presentation
Sym ptom s in clu de bilateral clou dy corn eas an d diffuse epith elial an d st rom al
edem a w ith a n orm al in t raocular p ressure an d th icken ed Descem et m em bran e.
Th e recessive form p resen t s w ith bilateral corn eal edem a at birth w ith nystagm u s.
Th is con dit ion appears w ith out ph otoph obia an d tearing, an d th ere is a lack of dys-
t rophy progression . Th e dom in an t form is eviden t by age 2 w ith gradual progres-
sion of th e dyst rophy. Pain an d tearing are presen t in th e absen ce of ph otoph obia
( Fig. 5.22 ).
Congen ital glau com a, m u copolysacch aridosis, congen it al h eredit ar y st rom al dys-
t rop hy, posterior polym orph ou s corn eal dyst rophy, birth t raum a
Management
Topical sodium ch loride drops or oin t m en t s are h elpfu l. Pen et rat ing keratoplast y
is used in cases w ith corn eal decom pen sat ion . Th ere is a con cern for am blyopia in
in fan t s.
Ectatic Disorders
Keratoconus
Keratocon u s is a com m on bilateral, but often asym m et rical, disorder of corn eal
th in n ing in w h ich th e cen t ral or in ferior paracen t ral corn ea un dergoes progressive
th in n ing to t ake on th e sh ape of a con e. It begin s in adolescen ce an d progresses
slow ly w ith st abilit y in late adu lth ood. A gen et ic predisp osit ion is suspected w ith
gen e lin kages in cer t ain fam ilies, bu t eye ru bbing an d eye t rau m a rem ain a sig-
5 Cornea 171
Fig. 5.23 Central corneal thinning

and protrusion with keratoconus.
n ifican t factors. Th ere is a h igh associat ion w ith con dit ion s su ch as atopy, Dow n
syn drom e, Marfan syn drom e, floppy eyelid syn drom e, an d Leber congen it al h e-
reditar y opt ic n eu ropathy. Acu te hydrops can occur.
Presentation
Vision is decreased as a result of progressive m yopic ast igm at ism from alterat ion
of th e sh ape of th e corn ea. Keratocon us t ypically develops in th e oblique axis an d
is associated w ith irregular ast igm at ism . Exam in at ion sh ow s scissoring of th e
red reflex an d apical corn eal th in n ing. Associated fin dings m ay in clude breaks in
th e Descem et m em bran e, apical corn eal scarring, st ress lin es in th e st rom a (Vogt
st riae), an d iron lin e form at ion (Fleisch er ring) (Fig. 5.23 ).
Con tact len s scarring, pellu cid, keratoglobus
Management
Corn eal topography, keratom et r y, or ph otokeratoscopy can im prove accu racy of
diagn osis by dem on st rat ing in ferior corn eal steepen ing. Scissoring reflex on ret i-
n oscopy is path ogn om on ic. Treat w ith spectacle correct ion of m yopic ast igm at ism
or rigid gas perm eable or hybrid con tact len ses w h en spectacle correct ion is poor
ow ing to irregu lar ast igm at ism . In tacs corn eal im plan t s (Addit ion Tech n ology, In c.,
Sun nyvale, CA) can h elp in som e cases of con tact len s in toleran ce an d poten t ially
provide in creased corn eal stabilit y w ith or w ith ou t corn eal collagen cross-lin king.
Pen et rat ing or deep an terior lam ellar keratoplast y provides th e u lt im ate cure.
Keratoglobus
Keratoglobus is a rare n on in flam m ator y con dit ion th at presen t s at bir th as op-
posed to oth er corn eal ect at ic disorders w ith m idperiph eral corn eal th in n ing.
Acute hydrops can occu r.
Fig. 5.24 Corneal hydrops in keratoglobus.
Presentation
Exam in at ion sh ow s a globular sh ape of both corn eas w ith large corn eal diam eters.
Associated gen eralized th in n ing occu rs, m ore con cen t rated in th e m idperiph er y.
Th e an terior ch am ber is ver y deep ( Fig. 5.24 ).
Keratocon u s
Management
A lam ellar or pen et rat ing keratoplast y is u su ally required. Progn osis is gu arded
becau se of th e n ecessar y large graft diam eter.
Pellucid Marginal Degeneration

Pellu cid m argin al degen erat ion is a n on h eredit ar y, bilateral, periph eral corn eal
th in n ing disorder th at m ost often involves th e in ferior corn eal periph er y but m ay
occur in th e superior periph eral corn ea. It presen t s in early adulth ood, t ypically
bet w een 20 an d 40 years of age.
Presentation
Pat ien ts experien ce blurred vision as a resu lt of progressive, again st th e rule ast ig-
m at ism or oblique ast igm at ism . Exam in at ion dem on st rates corn eal prot rusion
above th e area of th in n ing. St rom al scarring can occur w ith in th e th in n ed areas,
an d rarely p erforat ion m ay develop ( Fig. 5.25 ).
Keratocon u s, Terrien m argin al degen erat ion , collagen vascular disease
5 Cornea 173
Fig. 5.25 Inferior corneal thinning with

pellucid marginal degeneration.
Management
Rigid gas perm eable len ses are recom m en ded. Spect acle correct ion is often n ot
h elpfu l. Crescen t ic lam ellar or pen et rat ing keratoplast y can occur in severe cases
of vision loss or corn eal ectasia.
Corneal Degenerations and Deposits
Arcus Senilis
Arcu s sen ilis is an accu m u lat ion of ext racellular lipid in th e periph eral corn eal
st rom a. It can be associated w ith hyerlipidem ic states.
Presentation
Pat ien t s are asym ptom at ic, bu t th e con dit ion can be visible cosm et ically as a ring
of periph eral opacificat ion th at begin s in th e su perior an d in ferior periph eral cor-
n eal st rom a w ith a th in , clear zon e bet w een th e lim bu s. It t ypically presen ts in a
360-degree ring an d can be associated w ith correspon ding carot id ar ter y disease
( Fig. 5.26 ).
Hyp erlipoprotein em ia, pseu dogeron toxin , arcu s juven ilis
Management
No ocu lar t reat m en t is n ecessar y. Con sider obt ain ing a lipid profile in you ng
adu lt s.
Fig. 5.26 Peripheral lipid deposition with arcus senilis.
Limbal Girdle of Vogt

An elastot ic degen erat ion of collagen develops in th e periph eral corn ea an d m ay
con tain par t icles of calciu m .
Presentation
An asym ptom at ic, periph eral corn eal opacit y t yp ically begin s at 3 an d 9 o’clock
along th e lim bu s. Th ere m ay be a clear lucid in ter val bet w een th e opacit y an d
lim bus, depen ding on w h ich t ype of lim bal girdle develops. Ch alklike opacificat ion
is com m on ( Fig. 5.27 ).
Management
No t reat m en t is n eeded becau se visual fun ct ion is n ot affected.
Fig. 5.27 Vogt limbal girdle.

5 Cornea 175
Fig. 5.28 Dense primary lipid keratopathy.
Primary Lipid Keratopathy

A yellow - or cream -colored lipid deposit ion com posed of ch olesterol develops in
th e su perficial or deep corn eal st rom a. Un like secon dar y lipid keratopathy w h ere
an an teceden t corn eal in flam m ator y con dit ion such as h erpes sim plex, h erpes
zoster, or t rach om a is presen t , prim ar y cases lack corn eal n eovascu larizat ion an d
previou s in fect ion or in flam m at ion .
Presentation
Pat ien ts are asym ptom at ic un less decreased vision develops from obscu rat ion of
th e visual axis. Cream or yellow deposits develop in th e paracen t ral or cen t ral cor-
n ea ( Fig. 5.28 ).
Crocodile sh agreen , cen t ral clou dy dyst rophy of Fran çois, Sch nyder corn eal dyst ro-
phy, hyperlipoprotein em ia
Management
No t reat m en t is n ecessar y un less th e visual a xis causes decreased vision . A deep
an terior lam ellar keratoplast y or pen et rat ing keratoplast y m ay be n eeded depen d-
ing on th e depth of lipid deposit ion in th e corn eal st rom a.
Corneal Keloid
Corn eal opacit ies are com posed of irregu lar pat tern s of collagen ase bu n dles. Th ey
can be associated w ith ocu locerebroren al (Low e) syn drom e, an autosom al dom i-
n an t con dit ion in ch ildren , in w h ich th ey are bilateral. Adu lt cases t ypically occu r
after previou s corn eal perforat ion or corn eal t rau m a.
Fig. 5.29 Corneal keloid.
Presentation
Pat ien ts presen t w ith decreased visual acu it y an d foreign body sen sat ion . A w h ite,
elevated, corn eal lesion can progressively en large an d cover th e visual axis of th e
corn ea ( Fig. 5.29 ).
Scar from in fect ious corn eal ulcer; derm oid
Management
If visu al acu it y is com prom ised, a lam ellar keratectom y or pen et rat ing keratop last y
m ay be n eeded for vision reh abilit at ion . Bilateral cases in ch ildren sh ou ld receive a
system ic evaluat ion for Low e syn drom e w ith referral to a pediat rician .
Calcific Band Keratopathy

Th is con dit ion con sist s of calcific degen erat ion of th e su perficial corn ea involv-
ing th e Bow m an layer. System ic cau ses m ay in clu de ren al disease, hypercalcem ic
states, gou t , sarcoidosis, an d elevated p h osph oru s levels. Con dit ion s of ch ron ic
ocu lar disease such as glau com a, kerat it is, uveit is, an d th e p resen ce of in t raocular
silicon e oil m ay also cau se th is con dit ion . A h eredit ar y form is presen t . Ch ron ic
m ercur y exposure m ay be an oth er cause.
Presentation
Fin e gray-w h ite du stlike opacit ies develop in th e Bow m an layer in th e periph eral
corn ea, t ypically at 3 an d 9 o’clock. A lu cid in ter val separates th e opacit ies from
th e adjacen t lim bu s. Th e dep osits t ypically coalesce to form a h orizon tal ban d of
corn eal op acificat ion in th e in terpalp ebral zon e of th e corn ea ( Fig. 5.30 ).
Management
Determ in e th e un derlying cause. Any un derlying elect rolyte or ren al disease m u st
be corrected. If pain or foreign body sen sat ion is sign ifican t , a lam ellar keratec-
tom y w ith adju n ct disodium ethylen ediam in etet raacet ic acid (EDTA) (1, 1.5, or 2%)
can be u sed to rem ove calcific deposit s. If un derlying con dit ion s are n ot corrected,
5 Cornea 177
Fig. 5.30 Band keratopathy.
th e calcific deposits w ill recur. Ph ototh erapeu t ic keratectom y m ay also be u sed to

rem ove th e dep osits.
Salzmann Nodular Degeneration

A n on in flam m ator y corn eal degen erat ion develops eith er from idiopath ic cau ses
or as a sequela of prior ch ron ic kerat it is. Som e causes m ay in clu de ph lycten ulosis,
bleph arit is, t rach om a, con tact len s kerat it is, or in terst it ial kerat it is. It m ay also
be associated w ith recurren t corn eal erosion s an d ep ith elial basem en t m em bran e
dyst rop hy.
Presentation
Pat ien ts eith er m ay be asym ptom at ic or m ay presen t w ith foreign body sen sat ion ,
tearing, an d decreased vision . Elevated gray-w h ite or blu ish n odules develop rep -
resen t ing fibrillar m aterial th at h as replaced th e Bow m an layer. Th e n odules often
develop in a circu lar fash ion in th e cen t ral or paracen t ral corn ea, alth ough th ey
m ay also be presen t ing adjacen t to th e lim bus ( Fig. 5.31 ).
Ph lycten u losis, keloid, staphylococcal m argin al u lcer, corn eal scar from in fect ious
kerat it is
Fig. 5.31 Salzmann’s nodular de-

generation.
Management
Support ive care w ith art ificial tears, gels, or oin t m en t can h elp. If recurren t ero-
sion s are presen t , a tem porar y ban dage len s or topical osm ot ic agen t su ch as so-
diu m ch loride drops m ay im prove th e sym ptom s. If visual acu it y is decreased or
sign ifican t discom fort develops, a su perficial keratectom y can be perform ed to
rem ove th e n odules. Recurren ce m ay develop despite debridem en t . Pen et rat ing
procedu res are n ot com m on ly n eeded for th is con dit ion .
Spheroidal Degeneration
Sph eroidal degen erat ion is also referred to as corn eal elastosis, Labrador keratop-
athy, clim at ic droplet keratopathy, an d Biet t i n odu lar dyst rop hy. It is a bilateral
con dit ion seen m ain ly in m en , kn ow n to h ave an associat ion w ith ult raviolet ex-
posure.
Presentation
Th e pat ien t presen ts w ith irrit at ion an d foreign body sen sat ion . In ext rem e cases
visu al im pairm en t m ay occur. Clin ical exam in at ion reveals sm all am ber-colored
gran ules in th e su perficial st rom a of th e periph eral in terpalpebral corn ea. In -
creased opacificat ion , coalescen ce, an d cen t ral spread occur in th e late presen t a-
t ion s.
Management
Lam ellar keratoplast y an d pen et rat ing keratoplast y are opt ion s w h en th ere is vi-
su al im pairm en t .
Polymorphic Amyloid Degeneration

Bilateral corn eal opacit ies con tain ing am yloid develop in late adu lth ood w ith in th e
corn eal st rom a.
Presentation
Pat ien ts are often asym ptom at ic w ith n o vision reduct ion . Gray to w h ite polym or-
ph ic an d/or filam en tous flecks appear in th e m id- to deep st rom a w ith in th e cen -
t ral or paracen t ral corn ea. Th e dep osits appear refract ile bu t are t ran slu cen t on
ret roillu m in at ion . Th e in ter ven ing st rom a is clear ( Fig. 5.32 ).
Corn eal gu t t ae, corn eal farin at a, lat t ice dyst rophy
Management
Visual acu it y is t ypically n ot involved so n o t reat m en t is n ecessar y.
5 Cornea 179
Fig. 5.32 Polym orphic amyloid deposits seen on ret-

roillumination.
Iron Lines
Iron deposit ion occu rs in th e epith eliu m as a result of tear pooling abn orm alit ies
from asperit y of th e corn eal su rface ( Fig. 5.33 ).
Presentation
Hudson-Stähli line : A h orizon t al lin e at th e ju n ct ion of th e low er th ird an d upper
t w o th irds of th e corn ea
Ferry line : A lin e an terior to th e edge of th e conju n ct iva from a filtering bleb
Stocker line : A lin e an terior to th e h ead of a pter ygiu m
Fleischer ring: A con t in uous circular or ellipt ical pat tern surroun ding th e area of
corn eal steepen ing in keratocon us
Mannis line : A con t in uous 360-degree ring ju st an terior to th e sut u res of a cor-
n eal graft
Fig. 5.33 Fleischer ring from keratoconus outlined with co-

balt blue light filter.
Management
No m an agem en t is n ecessar y.
Kayser-Fleischer Ring
Th is is a rare au tosom al recessive disorder in w h ich copp er deposit ion occu rs
th rough out th e body, in clu ding th e Descem et m em bran e. It is also kn ow n as h epa-
tolen t icular degen erat ion .
Presentation
Corn eal fin dings are asym ptom at ic, but exam in at ion by gon ioscopy or slit lam p
reveals a Kayser-Fleisch er ring (a golden brow n to green 360-degree ring pat tern
at th e lim bu s w ith in th e Descem et m em bran e).
Management
System ic pen icillam in e causes th e ring to disappear gradu ally. Liver t ran splan ta-
t ion is often requ ired.
Corneal Verticillata
Lysosom al or lipid deposits occur in th e basal epith elial layer of th e corn ea in as-
sociat ion w ith several system ic m edicat ion s. Th is can also occu r in Fabr y disease.
Presentation
Pat ien ts are u sually asym ptom at ic but m ay experien ce blurred vision .
Fabr y disease ( Fig. 5.34A), am iodaron e ( Fig. 5.34B), ch loroquin e, hydroxych lo-
roqu in e, ph en oth iazin es, in dom eth acin , n aproxen , st riate m elan okeratosis ( Fig.
5.34C)
Management
Obser ve or perform epith elial debridem en t if vision is decreased. If th e con dit ion
is severe, con sider discon t in u at ion of system ic m edicat ion un less it is essen t ial for
t reat m en t of system ic disease.
Peripheral Thinning
Mooren Ulcer
Th is is an aggressive p eriph eral u lcerat ive kerat it is w ith a h igh risk of corn eal
m elt ing an d perforat ion n ot associated w ith system ic collagen vascular disease.
An au toim m un e role is th ough t to play a role in th e developm en t of u lcerat ion
an d st rom al m elt ing based on kn ow n su ppressor T-cell deficien cy, in creased im -
m u n oglobu lin A an t ibody levels, an d in creased levels of plasm a cells, lym ph ocytes,
im m u n oglobu lin s, an d com p lem en t factor in th e periph eral corn ea an d adjacen t
5 Cornea 181
Fig. 5.34 (A) Corneal verticillata seen with

Fabry disease. (B) Corneal verticillata from
amiodarone. (C) Striate melanokeratosis
with melanin deposition in the corneal epi-
thelium. B
conju n ct iva. Risk factors in clu de prior ocular t rau m a or ocular surger y as w ell as a
su bset w ith prior parasit ic in fect ion w ith h elm in th s.
Presentation
Sym ptom s in clude in ten se pain , redn ess, tearing, an d ph otop h obia. A ch ron ic an d
progressive corn eal ulcerat ion begin s in th e periph eral corn ea w ith circu m feren -
t ial spread follow ed by cen t ripet al spread. A leading edge of deepith elialized t is-
su e is presen t w ith keratolysis. Sign ifican t corn eal n eovascu larizat ion an d fibrosis
can develop. Pat ien ts are often in late adu lth ood w ith u n ilateral presen tat ion . A
secon d form of u lcerat ion occurs in p at ien ts w ith preceding parasit ic h elm in th
Fig. 5.35 Mooren ulcer.
in fect ion ; th is form is m ore com m on in en dem ic areas of Africa w ith h igh popula-
t ion s of parasitem ia. Th is form is often bilateral an d h igh ly associated w ith corn eal
perforat ion . Mooren u lcer can be associated w ith con curren t h epat it is C in fect ion
( Fig. 5.35 ).
Perip h eral u lcerat ive kerat it is, in fect iou s kerat it is, rosacea, st aphylococcal m ar-
gin al ulcerat ion
Management
A conjun ct ival recession can be ut ilized for in it ial t reat m en t of ulcerat ion , perh aps
by severing con n ect ion of th e lim bal vasculat ure an d associated in flam m ator y
cells from th e region of u lcerat ion . Lam ellar keratoplast y is often n eeded in cases
w ith im p en ding or fran k perforat ion . System ic im m un osuppressive agen t s such
as oral cort icosteroids, m eth ot rexate, cyclosporin e, an d cycloph osph am ide h ave
sh ow n prom ise in t reat m en t . Hep at it is C–associated cases h ave sh ow n im prove-
m en t w ith in terferon th erapy. Despite t reat m en t opt ion s, th is form of ulcerat ion
often h as a poor progn osis w ith a h igh corn eal perforat ion rate.
Peripheral Ulcerative Keratitis

A periph eral corn eal u lcerat ion is associated w ith epith elial breakdow n an d kera-
tolysis. Th e con dit ion can be associated w ith any con n ect ive t issue disorder (col-
lagen vascu lar disease) bu t is m ost com m on ly seen w ith rh eu m atoid ar th rit is.
Presentation
Presen tat ion in cludes pain , redn ess, an d decreased vision in th e set t ing of a con -
n ect ive t issu e disorder. Periph eral corn eal in filt rat ion is presen t w ith an associated
epith elial defect except in th e early st ages. St rom al m elt ing m ay be th e first sign of
system ic disease an d is correlated w ith exacerbat ion s of system ic disease act ivit y.
Sym ptom s are u sually u n ilateral but m ay be bilateral in presen t at ion . Associated
lim bal vaso-occlu sion can be seen w ith th e adjacen t lim bal vessels ( Fig. 5.36A,B).
5 Cornea 183
Fig. 5.36 (A) Peripheral ulcerative keratitis from rheumatoid

arthritis. (B) Peripheral corneal ulcer associated with system ic
lupus erythem atosis.
In fect ious kerat it is, Mooren ulcer, Terrien m argin al degen erat ion , furrow degen -
erat ion , rosacea, exposu re kerat it is
Management
Th e goal of th erapy is to preven t corn eal m elt ing an d p rom ote reepith elializat ion .
Surface lu brican t s su ch as art ificial tears, gels, or oin t m en t s sh ould be u sed ever y
1 to 2 h ours w ith or w ith ou t a blan d oph th alm ic an t im icrobial an t ibiot ic such
as er yth rom ycin to t reat th e im m un e-m ediated dr y-eye disease. Tem porar y or
perm an en t pu n ctal cau ter y can also in crease th e su rface m oist u re. System ic col-
lagen ase in h ibitors (m acrolides) m ay be u seful. Con t rol of system ic in flam m at ion
is essen t ial w ith im m u n osu ppression m edicat ion s such as predn ison e, cyclosp o-
rin e, azath iop rin e, cylcoph osph am ide, or m eth ot rexate; th is can be adm in istered
in con cer t w ith a rh eum atologist depen ding on th e oph th alm ologist’s com fort
w ith system ic th erapy. Cyan oacr ylate glu e or th erapeu t ic ban dage len ses m ay be
n eeded in cases of severe st rom al th in n ing. A conju n ct ival recession of adjacen t

lim bal conju n ct iva can prom ote h ealing of th e adjacen t periph eral st rom al m elt ing
an d ulcerat ion , perh ap s becau se of elim in at ion of a sou rce of in flam m ator y cells
an d collagen olyt ic en zym es from severing th e con n ect ion to th e lim bal vessels.
Lam ellar an d pen et rat ing keratoplast y m ay be n eeded in cases of im pen ding or
fran k perforat ion .
Terrien Marginal Degeneration

Th is is an idiopath ic periph eral corn eal th in n ing disorder th at can be localized or
diffuse. Th e ocu lar surface t ypically sh ow s on ly m ild in flam m at ion in associat ion
w ith th e periph eral corn eal th in n ing. Th e con dit ion is u sually bilateral but m ay be
un ilateral or asym m et rical in presen tat ion . Elect ron m icroscopy reveals th e p res-
en ce of h ist iocytes in th e corn eal lam ellae, in dicat ing a possible im m u n e-m ediated
role.
Presentation
Sym ptom s in clude foreign body sen sat ion , blurred vision , an d on ly m in im al sign s
of redn ess or conju n ct ival inject ion . Periph eral corn eal th in n ing occu rs m ost often
su periorly an d p rogresses in an an n ular pat tern w ith an overlying in t act epith e-
lium . Th e th in n ing is accom pan ied by an an terior lipid border an d bridging vessels
exten ding tow ard th e lipid base. Th e an terior lipid border is often steep, w ith slop -
ing of th e lim bal border. Again st-th e ru le-ast igm at ism often develops as th e th in -
n ing progresses. Spon tan eous perforat ion is rare bu t m ay occu r, especially w ith
ocu lar t rau m a ( Fig. 5.37 ).
Perip h eral ulcerat ive kerat it is, fu rrow degen erat ion , at ypical pellucid m argin al de-
gen erat ion , Fu ch s su perficial m argin al kerat it is
Fig. 5.37 Terrien marginal degeneration.

5 Cornea 185
Management
Man agem en t often con sists of suppor t ive care w ith lu brican t tears, gels, or oin t-
m en ts. Topical cyclosporin e m ay add ben efit in som e cases. Lam ellar corn eal patch
graft s can be useful in cases of im pen ding perforat ion . An n u lar lam ellar graft s
h ave also been used in severe cases w ith 360 degrees of progressive p eriph eral
th in n ing.
Furrow Degeneration
Th is m ay be an opt ical illu sion , th ough som et im es t ru e th in n ing does occur.
Presentation
Th e pat ien t is u su ally asym ptom at ic. Th is m ay occu r as an idiopath ic con dit ion in
th e elderly as a lu cid area separat ing th e corn eal arcu s from th e lim bu s. Corn eal
th in n ing is eviden t . Th e ep ith eliu m is in tact w ith n o vascularizat ion .
Terrien m argin al degen erat ion
Management
No t reat m en t is requ ired.
Aphakic and Pseudophakic Bullous Keratopathy
Refer to th e ch apter 7 sect ion on corn eal edem a as a com plicat ion of cataract su r-
ger y.
Corneal Surgery
Penetrating Keratoplasty
Pen et rat ing keratoplast y con sists of a fu ll-th ickn ess replacem en t of diseased cor-
n ea using a don or corn ea h ar vested from a h ealthy corn eoscleral don or rim . In di-
cat ion s for surger y in clu de corn eal edem a, corn eal scarring, corn eal dyst roph ies,
keratocon u s, corn eal u lcerat ion or perforat ion , in fect ion , an d failed corn eal t ran s-
plan ts, am ong oth ers. Th e don or t issue is secu red to th e periph eral h ost corn eal
rim w ith a variet y of su t ure p lacem en t tech n iques, in cluding in terrupted su t ures,
a con t in uous r un n ing sut ure, or com bin ed tech n iqu es.
Risks of su rger y in clu de but are n ot lim ited to sut u re in fect ion s an d graft rejec-
t ion . Graft reject ion m ay be epith elial, subepith elial, or en doth elial in n at ure (Fig.
5.38A,B).
Fig. 5.38 (A) Penetrating kerato-

plast y with com bined interrupted
and running suture technique. (B)
B Castroviejo square graft.
Fig. 5.39 Lam ellar keratoplast y for recurrent pterygium.

5 Cornea 187
Lamellar Keratoplasty
Th is is a part ial replacem en t of th e corn ea w ith don or corn eal t issue. Th e proce-
du re can be u sed in keratocon us, an terior corn eal dyst roph ies, an terior corn eal
scars, recurren t pter ygia, an d corn eal m elt s. Lam ellar grafts can be perform ed us-
ing an ar t ificial an terior ch am ber an d m icrokeratom e or using a w h ole globe w ith
h an dh eld p ar t ial-th ickn ess don or t issu e dissect ion ( Fig. 5.39 ).
Endothelial Keratoplasty
Th is corn eal t ran splan t tech n iqu e replaces th e diseased en doth eliu m w ith a pos-
terior don or corn eal but ton con sist ing of en doth elium , Descem et m em bran e, an d
a th in layer of posterior corn eal st rom a. It is used for diseases of th e en doth eliu m
w h en th e epith eliu m an d st rom a are essen t ially n orm al, su ch as Fuch s dyst rophy,
bullous keratopathy, en doth elial graft failu re, an d iridocorn eal en doth elial syn -
drom e. Tissue can be prepared u sing an ar t ificial an terior ch am ber an d m icro-
keratom e or by h an dh eld dissect ion . Descem et st ripp ing en doth elial keratoplast y
(DSEK) h as becom e a popu lar m eth od of perform ing th is tech n iqu e w ith st ripping
of th e Descem et m em bran e an d en doth elium , follow ed by replacem en t w ith a th in
don or posterior corn ea. Th e part ial-th ickn ess don or corn ea is h eld in place w ith
air t am pon ade ( Fig. 5.40A,B).
Fig. 5.40 (A) Endothelial keratoplast y. Preoperative

corneal edema from Fuchs dystrophy. (B) Endothelial
keratoplast y. Postoperative Descemet stripping auto-
m ated endothelial keratoplast y.
Fig. 5.41 Khodadoust line with

endothelial rejection of penetrating
keratoplast y.
Graft Rejection
Th e 5-year failu re rate for corn eal graft s is ~35% across th e Un ited States. Corn eal
graft reject ion is th e m ost com m on cau se of graft failure in th e late postoperat ive
period. Diagn osis of corn eal graft reject ion sh ould be m ade on ly in graft s th at h ave
rem ain ed clear for at least 2 w eeks follow ing keratoplast y.
Presentation
Pat ien ts m ay com p lain of a decrease in visu al acuit y, redn ess, ph otoph obia, p ain ,
an d irrit at ion . Epith elial reject ion is m arked by an elevated epith elial reject ion lin e
th at stain s w ith flu orescein or rose bengal or by th e presen ce of subepith elial in -
filt rates. St rom al reject ion is ch aracterized by periph eral full-th ickn ess h aze w ith
lim bal inject ion in a previou sly clear graft . An arc-sh aped in filt rate m ay be n oted
periph erally at th e graft–h ost ju n ct ion th at progresses cen t rally. Classic en doth e-
lial reject ion presen ts w ith an en doth elial reject ion lin e (Kh odadoust lin e) th at
usually begin s at a vascularized por t ion of th e perip h eral graft–h ost ju n ct ion an d
progresses ( Fig. 5.41 ). Th e com bin at ion of kerat ic precipit ates, an an terior ch am -
ber react ion , circu m corn eal inject ion , an d region s of corn eal edem a sh ould be di-
agn osed as corn eal graft reject ion .
Management
Reject ion m ay be preven ted w ith steroids, cyclosporin e A, an d oth er im m u n o-
m odu lators.
Keratoprosthesis
An ar t ificial device is u sed to restore vision in con dit ion s of corn eal blin dn ess.
Com m on ly u sed devices in clu de th e Boston Doh lm an Keratoprosth esis an d Alph a-
Cor (Addit ion Tech n ology, In c., Des Plain es, IL; th e Type I device is m an u fact u red
an d curren tly available th rough th e Massach u set t s Eye an d Ear In firm ar y at cost:
h t t p://w w w.m asstech por tal.org/IP1416.aspx). In dicat ion s in clu de m u lt iple failed
cadaveric allograft su rgeries w ith lit tle h ope for su ccess u sing fut u re cadaveric t is-
su e. Th is m eth od avoids allograft reject ion , bu t corn eal m elt ing, in fect ion s, an d
glaucom a rem ain a con cern w ith th is device. It can also be u sed for stem cell dis-
ease in pat ien t s w h o are poor can didates for im m un osupp ression ( Fig. 5.42A,B).
5 Cornea 189
Fig. 5.42 (A) Dense corneal opacification prior to

Dohlman keratoprosthesis placement. (B) Postopera-
tive Dohlman keratoprosthesis with 20/40 uncorrected
Snellen visual acuit y.
Anterior Staphyloma Managed by Anterior Segment Transplantation

An an terior staphylom a is a ch allenging en t it y. Mult ip le causat ive factors h ave
been n oted in th e literat u re, th e com m on on es being kerat it is an d congen ital
m alform at ion s, w ith sp oradic report s of disorders su ch as n eu rofibrom atosis an d
sarcoidosis .Th e surgical opt ion s for an terior st aphylom a an d sim ilar diffu se cor-
n eal lesion s in clu de a conven t ion al kerat roplast y, overlay graft s, an d par t ial- or
full-th ickn ess sclerokeratoplast y. How ever, an terior st aphylom a is associated w ith
addit ion al p roblem s oth er th an corn eal ectasia. Th e len s is often cataractous w ith
com prom ised zon ules, w h ich h as to be t aken care of at th e t im e of th e surger y. In
addit ion , staphylectom y leads invariably to loss of iris t issue, creat ing an iat rogen ic
an iridia. Th erefore, an ideal t ran splan t for an an terior staphylom a or a sim ilar dif-
fuse an terior path ology sh ould address th ese issues. Soosan Jacob h as develop ed a
n ew m eth od of an terior segm en t t ran splan t at ion th at can be u sed to t reat a m al-
form ed an terior segm en t an d t ran splan t a n ew, bioprosth et ic graft sim u lat ing th e
an terior segm en t ( Figs. 5.43A,B). Th is tech n iqu e is an exten sion of th e glu ed in t ra-
ocu lar len s (IOL) tech n iqu e inven ted by Am ar Agar w al.
Fig. 5.43 (A) Anterior segm ent trans-

plantation done on a 4-m onth-old child
with anterior staphylom a. (Top) Preopera-
tive appearance. (Bottom ) Postoperative
day 1 appearance. (B) Biosynthetic graft
being prepared for transplantation. One
haptic of the aniridia IOL being external-
ized (top). Biosynthetic graft seen after
both haptics have been externalized (cen-
ter). The bioprosthetic graft prepared for
anterior segment transplantation. Note
the aniridia intraocular lens haptics exter-
nalized at the scleral level below scleral
flaps (bot tom). The graft has biological
B
components: the cornea and sclera, and
synthetic components: the IOL optic, the
artificial iris, and the edge of the artificial
iris form ing the pupil.
Presentation
Diffuse corn eal involvem en t an d cicat rizat ion of th e uveal t issu e along w ith h igh
in t raocular pressu re resu lts in a large ect at ic area of th e corn ea an d lim bus.
Peters an om aly, diffuse corn eoscleral kerat it is
5 Cornea 191
Management
Th e bioprosth et ic graft con sists of a biological an d a prosth et ic p ar t . Th e biological
par t is fash ion ed from a cadaveric w h ole globe (corn ea an d sclera), an d th e pros-
th et ic par t con sists of an an iridia IOL (iris, pu pil, an d IOL). Tw o par t ial-th ickn ess,
lim bal-based scleral flaps of 3-m m size are created 180 degrees apart on th e don or
sclera. Tw o st raigh t sclerotom ies are th en m ade w ith an 18-gauge n eedle 1.5 m m
from th e lim bu s un der th e exist ing scleral flaps. A corn eo-scleral rim is th en cu t
th e en t ire length of th e sclera, in clu ding in th e scleral flaps. A cyclodialysis is th en
in duced to separate th e uveal t issue from th e dissected corn eoscleral but ton . Th e
corn eoscleral graft is placed con cave (i.e., en doth elial side up ), an d th e an iridia
IOL h apt ics are extern alized th rough th e sclerotom y un der th e scleral flaps u sing
a 23-gauge forceps. Th e biosyn th et ic assem bly th u s con sist s of a don or corn ea an d
sclera an d an ar t ificial iris an d len s (an iridia IOL). In th e recipien t eye, th e staphy-
lom atous corn ea is excised. Un derlying cat aract is m an aged w ith len sectom y an d
vit rectom y. Th e biosyn th et ic graft is th en placed on th e h ost an d sut u red. Th e IOL
h apt ics are th en t ucked in to a scleral p ocket created at th e edge of th e scleral flaps,
an d th e flap is th en glu ed dow n w ith t issu e glu e (Tisseel, Baxter, Deerfield, IL). Th e
conju n ct iva is th en closed by gluing.
Enlarged Corneal Nerves
Presentation
Prom in en t corn eal n er ves m ay be seen radiat ing cen t rally from th e corn eal pe-
riph er y.
Mu lt ip le en docrin e n eop lasia t ype IIb, icthyosis, Refsu m disease, leprosy, kerato-
con us, Fuch s en doth elial dyst rophy, osteogen esis im p erfect a, ocu lar pem ph igu s,
n eurofibrom atosis, ph th isis bu lbi, posterior polym orp h ou s dyst rophy, h erpes sim -
plex, h erp es zoster, p rim ar y am yloidosis
Management
Man agem en t is aim ed at th e un derlying con dit ion .
Corneal Neovascularization
Th e corn ea is t yp ically devoid of blood vessels; h ow ever, in con dit ion s of in fect ion ,
in flam m at ion , or ocu lar surface in sult , blood vessels can abn orm ally exten d in to
th e corn ea. Th is is often referred to as corn eal n eovascularizat ion or pan n us. It is
m ain ly seen in in flam m ator y corn eal diseases such as t rach om a.
Presentation
Pan n u s presen t s as a vascu lar ingrow th in to th e corn ea from th e lim bal vascu la-
t u re. It can be superficial or deep ( Fig. 5.44 ).
Fig. 5.44 A slit-lamp photograph

demonstrating severe corneal neo-
vascularization from diffuse stem
cell deficiency.
Trach om a, leprosy, h erpes sim plex or h erpes zoster kerat it is, ph lycten u lar kerato-
conju n ct ivit is, toxic conjun ct ivit is, acn e rosacea, bu llou s keratopathy, m ollu scum
con tagiosum , vern al conjun ct ivit is, keratoconju n ct ivit is sicca, con tact len s u se, in -
clusion conju n ct ivit is (m icrop an n u s), su perior lim bic keratoconjun ct ivit is, con tact
len s kerat it is, Fu ch s m argin al kerat it is, hypoparathyroidism , vitam in B deficien cy,
an d pellagra .
Management
Man agem en t is aim ed at th e un derlying con dit ion .
Leukocornea
Leu kocorn ea m ay arise due to a w ide variet y of con dit ion s in clu ding in fect ion ,
in flam m at ion , an om alies, h eredit ar y con dit ion s, an d t rau m a.
Presentation
Opacificat ion of th e corn ea is eviden t , even w ith out th e slit-lam p exam in at ion .
Infect ions: Bacterial, fu ngal, h erpet ic u lcers, st rom al scarring, t rach om a, Acan-
tham oeba
Inflam m atory : aph akic an d pseudoph akic bullous keratopathy, Steven Joh n son s
syn drom e, graft reject ion , ocular cicat ricial pem ph igoid
Congenital: An terior ch am ber cleavage syn drom es, sclerocorn ea, congen it al
glaucom a, derm oid, am yloidosis
Hereditary : Congen it al h eredit ar y corn eal dyst rophy, Dow n syn drom e, Patau
syn drom e, in born errors of m etabolism (m u copolysacch aridosis, Low e syn -
drom e, m ucolipoidosis)
Traum a : Descem et tear (bir th t rau m a), th erm al inju r y, ch em ical burn s
5 Cornea 193
Management
Man agem en t con sist s m ain ly of doing a corn eal t ran sp lan tat ion in su itable cases.
Any un derlying cause sh ould also be addressed.
Chemical Burn
See th e ch apter 1 sect ion on ch em ical exposu re bu rn s.

6 Intraocular Inflammation
Soosan Jacob , Dhivya Ashok Kum ar, Athiya Agarw al, and Am ar Agarw al
Acute Anterior Nongranulomatous Uveitis
HLA-B27-Associated Uveitis
Ankylosing Spondylitis
An kylosing spon dylit is (AS) is an ar th ropathy ch aracterized by back pain an d st iff-
n ess after in act ivit y. HLA-B27 is fou n d in u p to 90%of pat ien ts w ith AS. Th e ch an ce
th at an HLA-B27–posit ive pat ien t w ill develop spon dyloar th rit is or eye disease is
1 in 4. Not all HLA-B27–posit ive pat ien ts develop disease.
Sacroiliac x-ray film s sh ould be obt ain ed w h en in dicated by a suggest ive h istor y
in a p at ien t w ith ocular disease con sisten t w ith HLA-B27 syn drom e. Sacroiliac x-
ray film s sh ow sclerosis an d even t ual n arrow ing of th e join t space, ligam en tou s
ossificat ion .
Complications
Bony deform it y, pu lm on ar y apical fibrosis, aort it is, aor t ic valvular in su fficien cy
Uveitis
Management
Topical steroids an d cycloplegics. Sacroiliac join t radiograph , HLA-B27 screen ing
rh eu m atology con su ltat ion
Reiter Syndrome
Reiter syn drom e is ch aracterized by th e follow ing:
Non specific u reth rit is
Polyar th rit is
Conju n ct ival in flam m at ion , often accom pan ied by irit is
Presentation
Th is is com m on ly seen in young adult m ales (90%), w h ereas fem ales con st it u te
on ly 10%. Arth rit is is t ypically asym m et ric an d in oligoart icular dist ribu t ion , in -
volving kn ees, an kles, feet , an d w rist s. Sacroiliit is is presen t in as m any as 70% of
pat ien t s.
Keratoderm a blen n orrh agicum (a scaly, er yth em atous disorder of th e palm s an d
soles) an d circin ate balan it is (a persisten t , scaly, er yth em atous, circu m feren t ial
rash of th e dist al pen is) m ay be foun d.
Less com m on fin dings are plan t ar fasciit is, Ach illes ten din it is, sacroiliit is, n ailbed
pit t ing, p alate u lcers, an d tongu e ulcers.
Ocu lar involvem en t ranges from conju n ct ivit is (m u copur ulen t an d papillar y) to
kerat it is (pu n ct ate an d subep ith elial) to an terior, n ongran ulom atou s in flam m a-
t ion .
194
6 Intraocular In am m ation 195
Uveitis
Management
Topical steroids an d cycloplegics
HLA-B27 screen ing becau se it is presen t in 85 to 95% of pat ien t s
Rh eu m atology con sult at ion
Inflammatory Bowel Disease (IBD)

Ulcerat ive colit is (diffu se in flam m at ion of th e colon ic m u cosa) an d Croh n disease
(gran ulom atous iliocolit is) are both associated w ith acute irit is.
Presentation
Bet w een 5 an d 12% of pat ien ts w ith u lcerat ive colit is, an d 2.4% of pat ien ts w ith
Croh n disease develop acu te an terior uveit is (AAU). Of pat ien t s w ith in flam m a-
tor y bow el disease, 20% m ay h ave sacroiliit is, an d of th ese pat ien t s, 60% are HLA-
B27 posit ive. Ocular involvem en t m ay occu r first an d m ay in clu de an terior uveit is,
conju n ct ivit is, kerat it is, ep isclerit is, sclerit is, ext raocu lar m u scle palsies, opt ic
n europathy, ret in al vascu lit is, n eu roret in it is, an d orbital in flam m at ion . System ic
sym ptom s in clu de bloody diarrh ea, cram py abdom in al pain , skin rash , ar th ralgia,
er yth em a n odosu m , pyoderm a gangren osum , sacroiliit is, ren al ston es, an d h epa-
tobiliar y abn orm alit ies.
Uveitis
Management
Topical steroids an d cycloplegics. Treat in conjun ct ion w ith an in tern ist .
Psoriatic Arthritis
Ocular involvem ent : Nongran u lom atou s, an terior in flam m at ion , n odular
episclerit is, kerat it is, keratoconjun ct ivit is sicca
Uveit is is n ot associated w ith psoriasis w ith ou t arth rit is.
System ic: Er yth em atous, hyperkeratot ic rash , n ail pit t ing, an d distal in terp h a-
langeal join t ar th rit is
Uveitis
Management
Topical steroids, cycloplegics, im m un osup pressives. Treat in conju n ct ion w ith an
in tern ist .
Glaucoma-Related Uveitis
Posner-Schlossman Syndrome
Presentation
Man ifests as u n ilateral m ild acu te irit is sym ptom s th at in clu de discom for t , blurred
vision , or h aloes. Sign s in clu de m arkedly elevated in t raocular pressure, corn eal
edem a, fin e keriat ic p recipit ates, low -grade, an d sligh tly dilated pu pil.
Posn er-Sch lossm an syn drom e m ay be associated w ith HLA-B54 gen e locus.
Management
Topical steroids an d in t raocular p ressure–low ering agen ts
Uveitis-Glaucoma-Hyphema Syndrome
Irritat ion of th e iris root by th e w arped foot plates of poorly m ade, rigid, an terior
ch am ber in t raocular len s im plan t s cau ses th e uveit is-glaucom a-hyp h em a t riad.
Presentation
In creased in t raocular pressu re, an terior ch am ber in flam m at ion , an d hyph em a for-
m at ion in th e presen ce of an in t raocular len s
Management
Topical steroids, cycloplegics, an d in t raocu lar pressure–low ering agen t s
Phacolytic Uveitis/Glaucoma
Th is involves an acu te in crease in in t raocu lar pressure cau sed by blockage of th e
t rabecu lar m esh w ork by len s protein an d engorged m acroph ages.
Presentation
Low -grade anterior cham ber inflam m ation, increased intraocular pressure, lack of
keratic precipitates, and synechiae. Aqueous tap m ay reveal swollen m acrophages.
Management
In t raocu lar pressu re redu ct ion w ith osm ot ic agen t s as w ell as topical m edicat ion s.
Th e cat aract n eeds to be rem oved.
Chronic Nongranulomatous Uveitis
Juvenile Rheumatoid Arthritis–Associated Uveitis

Juven ile rh eum atoid ar th rit is (JRA) is a grou p of diseases w ith on set before 16
years of age.
Presentation
System ic onset : Usu ally seen in ch ildren u n der th e age of 5 years, of w h ich < 6%
presen t w ith uveit is; rash , fever, lym ph aden opathy, h epatosplen om egaly, peri-
cardit is, an em ia, psoriasis; pat ien t s presen t ing w ith system ic on set accou n t for
~20% of all cases of JRA.
Polyart icular onset : Sh ow s involvem en t of five or m ore join ts in th e first 6 w eeks
of th e disease. It con st it utes 40%of JRA cases overall bu t on ly 7 to 14%of cases of
JRA-associated iridocyclit is.
Panciart icular onset : Th is in cludes th e vast m ajorit y (80 to 90%) of pat ien ts w ith
JRA w h o h ave uveit is.
Type 1 : Girls un der age 5, posit ive for an t in u clear an t ibody (ANA). Ch ron ic
iridocyclit is occurs in up to 25% of th ese pat ien t s.
Type 2 : Older boys, seronegative spondyloarthropathy (75%are HLA-B27 posi-
tive). Uveitis tends to be acute and recurrent rather than chronic as in t ype 1.
Ocular involvem ent : It usu ally occu rs w ith in 5 to 7 years of on set of arth rit is, but
th e risk rem ain s in to adulth ood. Ocular in flam m at ion occu rs in 2 to 12% of all
cases an d is u sually bilateral. Un ilateral cases often progress to bilateral w ith in
12 m on th s. Th e ocu lar an d join t disease act ivit y are n ot associated. It m ay be
an t in u clear an t ibody test posit ive, rh eum atoid factor n egat ive.
Ch ildren are often asym ptom at ic w ith in sidious disease on set . Th ere m ay be
m ild ocular pain , h eadach e, ph otoph obia, an d decreased vision . A w h ite eye w ith
act ive an terior ch am ber in flam m at ion , kerat ic precipit ates, posterior syn ech ia,
cataract form at ion , glau com a, an d ban d sh aped keratopathy m ay even be fou n d
on th e first exam in at ion .
Management
Uveitis is treated w ith topical steroids and cycloplegics. System ic or periocular ste-
roids are som etim es needed. Ethylenediam inetetraacetic acid (EDTA) chelation is
done for band-shaped keratopathy. Rheum atology consultation is often necessar y.
Fuchs Heterochromic Iridocyclitis
Presentation
Presen t at ion is u sually u n ilateral; sym ptom s var y from n on e to m ild blu rring an d
discom fort .
Signs
Diffuse iris st rom al at rophy w ith variable pigm en t epith elial layer at rophy
Sm all w h ite stellate kerat ic precip itates scat tered diffu sely over th e en t ire en -
doth elium
Cells presen t in th e an terior ch am ber as w ell as th e an terior vit reou s.
Syn ech iae alm ost n ever form .
Glaucom a an d cat aract s occu r.
Abn orm al vessels m ay bridge th e angle on gon ioscopy.
Fu n dus lesion s are absen t; som et im es toxoplasm a scars h ave been reported.
Management
Cat aract su rger y an d in t raocular len s im plan t can be don e successfu lly.
Glaucom a is difficult to con t rol an d m ay n eed surger y.
Granulomatous Uveitis
Syphilis
Presentation
Syph ilit ic involvem en t of th e uveal t ract can presen t in t w o w ays:
1. Congenital syphilit ic uveit is: Here th e involvem en t is bilateral, th e periph er y of
th e ret in a is involved, an d occasion ally on e or t w o qu adran ts of th e posterior
pole can be involved rarely. Th us th e vision is n ot affected. Th e t yp ical appear-
an ce is th at of a salt an d pepper fun du s du e to areas of p igm en t accum ulat ion
in terspread w ith areas of pigm en t loss. An associated ocu lar feat ure is th e pres-
en ce of bilateral in terst it ial kerat it is.
Secondary ret inal pigm ent degenerat ion m ay be seen , w h ich is a con dit ion th at is
progressive an d is associated w ith con st rict ion of th e blood vessels of th e ret in a
an d th e ch oroids in th e form of sclerosis. Th e opt ic disk is pale w ith sh arp ly
defin ed borders. Pigm en t s dispersed are also sh arply dem arcated w ith a st ar
sh ape or bony corpu scle form at ion . Th e posterior pole or th e periph er y can be
affected, an d th e con dit ion is bilateral.
2. Acquired syphilis: Th is h as th ree com pon en t s: irit is, ch orioret in it is, an d n eu -
roret in it is. Th e irit is is ch aracterized by th ree form s: irit is papulosa, irit is n o-
dosa, an d irit is roseat a. Ch orioret in it is is ch aracterized by vit reou s h aze, fin e
pun ct ate gray to yellow exu dat ion areas, pigm en t accum ulat ion along th e opt ic
n er ve an d blood vessels, an d flam e-sh aped h em orrh ages w ith ch orioret in al
edem a. Neu roret in it is con sists of opt ic n er ve h ead involvem en t w ith vascular
involvem en t of th e su rrou n ding ret in al vessels.
Management
Blood tests in th e form of Ven ereal Disease Research Laborator y test (VDRL) an d
th e fluorescen t t repon em al an t ibody–absorpt ion test (FTA-ABS) are don e. Uveit is
is t reated w ith topical steroids an d cycloplegics. Th e system ic disease sh ou ld be
t reated in conju n ct ion w ith an in tern ist .
Sarcoidosis
Sarcoidosis is a ch ron ic gran u lom atous uveit is of u n kn ow n et iology. It m ay also
affect th e lu ngs, eyes, an d skin .
Presentation
Ocular: Sym ptom s of uveal involvem en t are variable an d frequ en tly in clude m ild
to m oderate blurring of vision . It m ay involve all st ru ct ures of th e eye. A sizable
propor t ion of pat ien t s develop ch ron ic gran ulom atou s iridocyclit is. Typical fin d-
ings are m u t ton fat kerat ic precipitates, Koeppe an d Bu sacca iris n odules, an d
sn ow balls in th e in fect iou s an terior vit reous. Nu m m u lar corn eal in filt rates, en -
doth elial op acificat ion , an d large iris gran ulom as also occu r. Posterior syn ech iae
can be exten sive an d m ay lead to iris bom bé an d angle closure glaucom a. Pe-
riph eral an terior syn ech ia m ay be exten sive, involving 360 degrees in advan ced
cases. Secon dar y glaucom a can be severe.
Posterior segm ent involvem ent is characterized by nodular granulom as m easuring

¼ to 1 disc diam eter that occur in both the retina and the choroid. Irregular nodu-
lar granulom as along venules have been term ed candlew ax drippings or taches de
bougie. Linear or patchy retinal periphlebitis presents as sheathing. Cystoid m acular
edem a is com m on; retinal neovascularization, disk edem a, and optic nerve granu-
lom as also occur. Palpebral and bulbar conjunctival nodules also occur.
Lungs: Hilar aden opathy.
Central nervous system (CNS): Cran ial an d periph eral n europathy, asept ic m en -
ingit is.
Cardiovascular system : Cardiac arrhyth m ias, pericardit is, ar th rit is, m yosit is,
er yth em a n odosa, ren al involvem en t h epatosplen om egaly an d bon e m arrow
in filt rat ion .
Management
Serum angioten sin -conver t ing en zym e an d lysozym e levels are in creased. Ch est
x-ray sh ow s h ilar aden opathy.
Uveitis
Topical, periocu lar, an d system ic steroids, topical cycloplegics. Treat in conju n ct ion
w ith an in tern ist .
Tuberculosis
Presentation
Tu bercu lou s uveit is can presen t in clu de th e follow ing w ays:
Acute n ongran ulom atous in fect ion —im m u n ological or allergic in n at ure
Nodu lar gran u lom atous in filt rates th at are fulm in at ing an d caseat ing. Tw o
t ypes of n odu les are presen t as sh ow n in Table 6.1 .
Relapsing an d recurren t irit is
Low -grade ch ron ic in flam m at ion th at presen ts w ith cataract , glaucom a, or
pth isis bu lbi
Ch oroidal lesion s appear as raised m ult iple yellow n odules w ith blurred m ar-
gin s. Ret in al periph lebit is an d su bret in al vascu larizat ion s are seen .
Oth er ocu lar feat ures of t uberculosis in clu de ph lycten u losis, episclerit is, n odu -
lar sclerit is, an d opt ic n eu rit is.
Management
An t it uberculous t reat m en t is started in conjun ct ion w ith an in tern ist . Steroids
sh ould be started on ly after star t ing an t it u bercu lou s t reat m en t .
Table 6.1 Tw o Types o f No dular Granulo m ato us In ltrates
So litary o r co nglo m erate no dule Miliary no dules
Otherwise healthy immune Im munocompromised patients,

responsive patients severely debilitated patients
Single large tum orlike nodule Multiple nodules over the iris at the
yellow to white papillary margin and ciliary body
with dissemination of bacilli
Intermediate Uveitis/Pars Planitis
Presentation
The disease is t ypically seen in children. It is also know n as chronic cyclitis. Patients
have visual sym ptom s like blurred, distorted vision secondar y to m acular edem a.
They also com plain of floaters. The active vitreous inflam m ation show s cells that are
sm all, round, nonpigm ented, and num erous. The old vitritis has cells that are irregu-
lar, pigm ented, and rem ain in the form ed vitreous. At the edges of the ora serrata
there is snow banking that is characteristic of deposition of inflam m atory cells at
this region. Anterior segm ent inflam m ation signs such as congestion and tenderness
are absent. The sym ptom s of pain or photophobia are also absent or m inim al.
Th ere are several key differen t iat ing feat u res:
Periph eral ret in it is
Perivasculit is
Vit rit is
Sn ow ban king at th e in ferior periph eral ret in a
Mu lt iple sclerosis, sarcoidosis, t u bercu losis, toxocariasis, syph ilis, Lym e disease
Management
Steroids are given via topical, su bten on s, or oral route. System ic im m u n osup pres-
san t s an d vit rectom y m ay also be n ecessar y in som e cases.
Posterior Uveitis
Toxoplasmosis
Toxoplasm osis is th e m ost com m on cau se of ch orioret in it is, congen it al in origin .
W h en th e m oth er is affected in th e first t rim ester, th ere is a 40% risk of t ran sm is-
sion to th e fet u s.
Presentation
Tw o form s of toxoplasm osis exist: congen ital an d adult . Both form s are ch aracter-
ized by acu te focal ch orioret in it is ( Fig. 6.1 ).
Congenital toxoplasm osis: It is bilateral in 85% of ch ildren an d 80% h ave ch o-
rioret in it is. W h en CNS sym ptom s su ch as convulsion s, hydrocep h alus, m en tal
retardat ion , an d in t racran ial calcificat ion s are absen t , congen ital lesion s are
diagn osed w h en th e ch ild p resen t s w ith esot ropia or exot ropia or decreased
vision .
Th e clin ical ch aracterist ics of th e congen it al form are as follow s:
Bilateral
Mu lt ip le ch orioret in al lesion s, especially in th e m acular area
Pu n ch ed-ou t lesion du e to fu ll-th ickn ess n ecrosis
Heavily p igm en ted scar, m ist aken to be congen ital colobom as
A B
C D
Fig. 6.1 (A) Arterial phase fluorescein angiogram (FA) demonstrating active toxo-
plasma chorioretinitis adjacent to a classic chorioretinal scar. (B) Arteriovenous phase
FA demonstrating the classic old pigmented lesion with hypofluorescence at the center
and hyperfluorescence at the margins of the lesion. (C) Late -phase FA showing juxta-
papillary multiple hyperfluorescent areas corresponding to new, active lesions. (D) Ac-
tive lesions, areas of hyperfluorescence, are usually seen in the vicinit y of old scars, as
evidenced here. (Courtesy of J. Fernando Arevalo, Venezuela)
Adult toxoplasm osis: Th ere is react ivat ion of in flam m at ion at th e edges of a pre-
exist ing scar; th is is called a satellite lesion . Vit rit is is severe an d th e m argin s
of th e lesion are yellow an d blurred. Th ere is a hypersen sit ivit y react ion to th e
t roph ozoites th at are released from th e cysts.
Th e follow ing term s h ave been used to describe th e lesion :
Searchlight in fog: Yellow lesion s seen th rough th e vit reous h aze
Grapevines: Of th e m em bran es an d cells of vit rit is
W et snow : St icky vit reou s exudates in vit rit is
In ch ildren , differen t ials in clude congen it al colobom as, cytom egaloviru s in clusion
ch orioret in it is, h erp es sim plex ch orioret in it is, toxocariasis, ret in oblastom a, an d
cerebral t rau m a.
In adu lt s, differen t ials in clu de t u berculous ch orioret in it is, can didiasis, an d h is-
t ioplasm osis.
Management
An t ibiot ics in th e form of Bact rim DS (dou ble st rength ), clin dam ycin , or pyrim eth -
am in e w ith folin ic acid are given for 6 w eeks in conjun ct ion w ith an in tern ist . Oral
steroids m ay be in dicated w h en th e disease spreads close to th e opt ic n er ve or
m acu la.
Presumed Ocular Histoplasmosis

Histoplasm a capsulat um causes th e ch aracterist ic presu m ed ocu lar h istop lasm osis
syn drom e (POHS). Im m un ocom prom ised pat ien t s are affected by th e presen ce of
an isolated gran u lom a or en doph th alm it is.
POHS t ypically affects you ng people an d is respon sible for bilateral visu al loss.
Th e ch aracterist ic feat u res of th e syn drom e are th e appearan ce of isolated disci-
form m acular lesion s an d scars th at are w ell circu m scribed. Th ese are quiet lesion s
w ith ou t th e presen ce of act ive in flam m at ion an d th e presen ce of peripapillar y
pigm en t at rophy ( Fig. 6.2 ).
A B
C D
Fig. 6.2 (A) Disseminated choroiditis (histo spots), maculopathy, and peripapillary cho-
rioretinal degenerative changes in a patient with the presumed ocular histoplasmosis syn-
drome. (B) Disseminated choroiditis (histo spots), maculopathy, and peripapillary chorio-
retinal degenerative changes in another patient with the presumed ocular histoplasmosis
syndrome. (C) Fluorescein angiography of the patient in Fig. 6.2B, revealed serosangui-
nous retinal detachment with faint pigment halo in right macula and choroidal neovascu-
larization. (D) Fluorescein angiography of patient in Fig. 6.2B, revealed hypofluorescence
after photodynamic therapy with verteporfin. (Courtesy of Steve Bloom, MD).
Management
Follow up for developm en t of ch oroidal n eovascu lar m em bran e an d t reat accord-
ingly.
White Dot Syndrome
Multifocal Choroiditis
Mu lt ifocal ch oroidit is is a rare disorder involving idiopath ic in flam m at ion of th e
ch oroid an d ret in al pigm en t epith eliu m (RPE).
Presentation
Presen t at ion is u sually bilateral, w ith sym ptom s such as ph otoph obia, blu rred vi-
sion , eye pain , an d decreased visual acu it y. Th e pat ien t m ay also h ave m etam or-
ph opsia, floaters, scotom as, an d ph otopsia. Th ere m ay be sign s an d sym ptom s of
an terior uveit is.
Mu lt iple, sm all, yellow /gray-w h ite spot s m ay be seen along w ith cystoid m acu -
lar edem a, ch oroidal n eovascular m em bran e, an d subret in al fibrosis.
Management
Cor t icosteroids are h elpfu l in resolving th e lesion s.
Multiple Evanescent White Dot Syndrome

Mu lt iple evan escen t w h ite dot syn drom e (MEW DS) is an acute bu t ben ign , rare,
un ilateral disease of u n kn ow n et iology involving th e RPE an d ch oroidal capillaries.
It u su ally affect s young fem ales. It can rarely be bilateral ( Fig. 6.3 ).
A B
Fig. 6.3 Multiple evanescent white dot syndromes (MEWDS). (A) Fundus examination
revealed sm all and large spots scat ted through the fundus in the left eye. (B) Fluorescein
angiography showed punctate hyperfluorescence. (Courtesy of Antonio Ciardella, MD).
C D
Fig. 6.3 (Continued) Multiple evanescent white dot syndromes (MEWDS). (C) Indocya-
nine green videoangiography demonstrated small and large hypofluorescent spots. A
diagnosis of MEWDS was made. (D) One month later the spots in the fundus had disap-
peared, and visual acuit y recovered. (Courtesy of Antonio Ciardella, MD).
Presentation
Mu lt iple w h ite dot s at th e level of th e RPE an d opt ic n er ve h ead sw elling m ay be
seen . Th e m acula often h as a gran u lar appearan ce. Visual-field defects in th e form
of an en larged blin d spot an d paracen t ral an d cen t ral scotom as m ay also be seen .
Visual loss, even if sign ifican t in it ially, alm ost alw ays ret urn s to n orm al later. Th e
pat ien t m ay also h ave ph otopsia.
Management
Fu n du s fluorescein angiography sh ow s focal areas of early pu n ct ate hyperfluo-
rescen ce th at corresp on d to th e w h ite dots. Th e ch anges on in do-cyan in e green
angiography m ain tain longer th an fu n dus ph oto an d FFA. Th e progn osis for visu al
acuit y in MEW DS is ver y good, an d it resolves on its ow n .
Acute Multifocal Posterior Placoid Pigment Epitheliopathy

Th is con dit ion closely resem bles MEW DS. It m ay be associated w ith HLA-B7.
Presentation
Sim ilar to MEW DS, it can also presen t w ith a viral predrom e follow ed by t ran sien t
visu al loss in young to m iddle-aged adu lt s. It is u su ally bilateral. Ret in al lesion s
con sist of m u lt iple yellow -w h ite placoid lesion s, w h ich are larger th an in MEW DS,
involving th e RPE ( Fig. 6.4 ).
A B
C D
Fig. 6.4 (A) Early phase fluorescein angiography in acute posterior multifocal placoid
pigment epitheliopathy (APMPPE) shows irregular areas of blocked fluorescence charac-
teristic of acute lesions. (B) At the arteriovenous phase, acute lesions still block fluores-
cence and are well demarcated. (C) Mid- and (D) late -phase angiograms dem onstrate
progressive, diffuse, even staining of the acute lesions. (Courtesy of J. Fernando Arevalo,
Venezuela)
Management
FFA sh ow s early blocked fluorescen ce w ith late st ain ing. Th e lesion s slow ly h eal
w ith scarring an d leave beh in d exten sive RPE defects th at persist .
Serpiginous Choroiditis
Th is is a recurren t in flam m ator y disease of th e RPE an d ch oroid th at is gen erally
bilateral.
A B
Fig. 6.5 (A) Fundus photograph and (B) hyperfluorescence on fluorescein angiogra-
phy of a patient with serpiginous choroiditis demonstrating choroidal neovasculariza-
tion. (Courtesy of J. Fernando Arevalo, Venezuela)
Presentation
Serpigin ou s ch oroidit is is seen m ost com m on ly in adu lts in th e fou rth to sixth de-
cade of life. Blu rred vision is th e first sym ptom . Vit reou s varies from clear to m ildly
cellular. A serpigin ou s or geograph ic (m aplike) pat tern of scars m ay presen t in th e
posterior fun du s. Edges of th ese lesion s m ay be act ive, w ith a yellow -gray an d
edem atou s appearan ce. As act ive lesion s becom e at ioph ied over w eeks to m on th s,
n ew lesion s can occur elsew h ere or con t iguously in a sn ail-like pat tern . Scotom as
m ay be seen . FFA sh ow s early hypoflu orescen ce w ith late hyp erflu orescen ce of le-
sion s during act ive disease ( Figs. 6.5 an d 6.6 ).
Management
Oral steroids along w ith im m u n osu ppressan t s
Birdshot Retinochoroidopathy (Vitiliginous Chorioretinitis)

Th is is a cau se of ch ron ic p osterior uveit is, w ith a fem ale predilect ion . It is also
associated w ith HLA-A29.
Presentation
Mu lt iple sm all w h ite/yellow spot s are seen scat tered about th e posterior pole in
th e deep ret in a an d ch oroid. Vit rit is an d m acular edem a w ith or w ith ou t epiret in al
m em bran e form at ion m ay be seen . Disk edem a an d opt ic n er ve in flam m at ion w ith
peripapillar y at rophy m ay be seen ( Fig. 6.7 ).
Management
Th ough it gen erally ru n s a ben ign course, it is poten t ially blin ding secon dar y to
m acu lar in flam m at ion an d perm an en t dam age. Hen ce, in cases w ith sign ifican t
in flam m at ion or vision -affect ing m acular edem a, aggressive t reat m en t sh ou ld be
given .
A B
C D
Fig. 6.6 Serpiginous choroiditis. (A) Color fundus photo. (B) Fluorescein angiography
shows early blockage. (C) As the angiogram proceeds, the active margins progressively
becom e hyperfluorescent (D) and spread toward the center of the lesion as it absorbs
dye from the choriocapillaris. (Courtesy of J. Fernando Arevalo, Venezuela)
A B
C D
Fig. 6.7 Birdshot retinochoroidopathy. (A) Color fundus photo of right and (B) left eye.
(C) Early fluorescein angiography with choroidal infiltration and m inim al retinal pigm ent
epithelium atrophy. The spots are hypofluorescent. (D) The lesions become m ildly hy-
perfluorescent in the late phases of the study as dye from the choriocapillaris stains the
extrachoroidal vascular space. (Courtesy of J. Fernando Arevalo, Venezuela)
Cytomegalovirus (CMV) Retinitis
Presentation
Congenital CMV: Most com m on ly seen at th e posterior pole w ith overlying vit reous
h aze an d pigm en t clum ps. Th ere can be m ild ch orioret in it is or a severe n ecrot izing
ch orioret in it is. Th e lesion s are m u lt iple, an d hyperplast ic m acular scarring can be
seen . Th e vision loss can be secon dar y to opt ic n er ve abn orm alit y.
Im m unosuppressed CMV ret init is: Ret in al in farcts m an ifest ing as cot ton w ool
spot s, ret in al h em orrh ages, ret in al n ecrosis, vit reou s h aze, an d ret in al vasculit is
are seen ( Figs. 6.8 an d 6.9 ).
Management
CMV ret in it is is t reated w ith in t raven ou s gan ciclovir (5 m g/kg ever y 12 h ou rs) or
in t raven ous foscarn et (60 m g/kg ever y 8 h ours) or cidofovir. In t ravit real inject ion s
m ay also be given .
Fig. 6.8 Cytomegalovirus retinitis.

A B
Fig. 6.9 Cytomegalovirus (CMV) retinitis in an AIDS patient. (A) Early indocyanine
green angiography (ICG-V) frame shows the onset of fluorescence. Engorged leaking
choroidal vessels (white arrows) indicating inflammatory choroidal vasculopathy. (B)
Maximum fluorescence on ICG-V of leaking choroidal vessels (white arrow). (Courtesy of
J. Fernando Arevalo, Venezuela)
Acute Retinal Necrosis Syndrome
Presentation
Th is syn drom e is a form of severe posterior uveit is th at is cau sed by varicella zos-
ter virus an d h erp es sim plex viru s t ypes 1 an d 2. It is defin ed by th e follow ing ver y
defin ite clin ical ch aracterist ics: presen ce of focal, defin ed areas of ret in al n ecrosis
in th e perip h eral ret in a out side th e vascular arcades, rapid p rogression to con flu-
en t circu m feren t ial n ecrosis, occlusive vasculopathy, m arked vit rit is, an d irit is.
Oth er feat ures are opt ic at rophy, sclerit is, an d p ain .
Management
System ic an t ivirals su ch as acyclovir an d valacyclovir are given in it ially follow ed
by oral steroids. An t icoagu lan t th erapy m ay be requ ired. Prophylact ic laser ph oto-
coagulat ion or pars plan a vit rectom y m ay be required.
Vogt-Koyanagi-Harada Syndrome
Presentation
Headach e, n au sea
Bilateral vit rit is, ch oroidit is, an d m ult iple oval det ach m en t s of th e ret in a th at
are exu dat ive
Ret in al an d peripapillar y n eovascu larizat ion s
Mu t ton fat kerat ic precipit ates
Papillar y n odules an d sh allow an terior ch am bers
Differen t ial diagn osis: sym path et ic oph th alm ia, sarcoidosis, acu te posterior
m u lt ifocal placoid pigm en t epith eliopathy
Associated fin dings in clude alopecia, vit iligo, poliosis, an d h earing loss ( Figs.
6.10 an d 6.11 ).
Management
Treat aggressively w ith cycloplegics; top ical, periocular, an d system ic steroids;
an d/or im m u n osu ppressive agen t s.
Fig. 6.10 Occlusion (arrows) of choroidal vessels in a patient

with Vogt-Koyanagi-Harada syndrome. (Courtesy of J. Fernando
Arevalo, Venezuela)
A B
C D
Fig. 6.11 Vogt-Koyanagi-Harada syndrome. (A) Individual leaking choroidal vessels

(black arrow s) indicating inflamm atory choroidal vasculopathy and patchy hypofluo-
rescent areas (w hite arrow s) were visible in the early-phase indocyanine green videoan-
giography (ICG-V) in the acute disease. (B) Note m arked decrease in the number of large
choroidal vessels (black arrow s) in the early phase ICG-V in the acute disease. (C) Evenly
sized hypofluorescent spots are observed in the intermediate phase ICG-V at the acute
phase of the disease. (D) Some of the spots are persisting into the late phase. (Courtesy
of Leyla Atmaca, MD)
Behçet Syndrome (Oculo-Oro-Genital Syndrome)
It w as first described by Beh çet as a syn drom e ch aracterized by recurren t oral aph -
th ous u lcers, gen ital u lcers, an d uveit is ( Fig. 6.12 ).
Presentation
Major feat u res
Recurren t aph th ou s ulcerat ion of th e oral m ucou s m em bran e
Skin lesion s (er yth em a n odosum –like lesion s), su bcu tan eous th rom boph le-
bit is, folliculit is (acn elike lesion s), cu t an eou s hyp ersen sit ivit y
Eye lesion s (iridocyclit is, ch orioret in it is, ret in ouveit is) an d a defin ite h istor y
of ch orioret in it is or ret in ouveit is
Gen ital u lcers
Mino r features
Ar th rit is w ith out deform it y an d an kylosis
Gast roin test in al lesion s ch aracterized by ileocecal u lcers
Epididym it is
Vascular lesion s
CNS sym ptom s
A B
C D
Fig. 6.12 Behçet syndrome. (A) Hypofluorescent spots in the late phase of indocya-
nine green videoangiography (ICG-V). (B) These spots cannot be seen on fluorescein
angiography. Hyperfluorescence due to pigment epithelial changes can be observed.
(C) Hyperfluorescent spots in the late phase of ICG-V. (D) These spots cannot be seen
on fluorescein angiography. (Courtesy of Leyla Atmaca, MD)
Com plete : Fou r m ajor feat u res
Incom plete : (1) Th ree m ajor feat ures, (2) t w o m ajor an d t w o m in or feat u res, or
(3) t ypical ocu lar sym ptom an d on e m ajor or t w o m in or feat ures
Possible : (1) Tw o m ajor feat ures or (2) on e m ajor an d t w o m in or feat u res
Management
Cor t icosteroids an d oth er im m u n osup pressives h ave been t ried. Em pirical t reat-
m en t is given .
Sympathetic Ophthalmia
Presentation
Characterist ic sym ptom s: Pain , ph otoph obia, an d decreased accom m odat ion
Characterist ic signs: In flam ed an d th icken ed uveal t issu e w ith edem atous iris,
disk sw elling, n odu les, papillit is, an d yellow n odu les called Dalen -Fuch s n od-
ules
Characterist ic associat ions: vit iligo, poliosis, alopecia
Different ial diagnosis: Vogt Koyan agi Harada, len s-in duced uveit is
Treatment
Steroids via all routes an d im m un osu ppressan ts are in dicated.
Endophthalmitis
Traumatic Endophthalmitis
Bacteria or fungi are in t rodu ced at th e t im e of inju r y in t rau m at ic en doph th alm it is.
It can occur in u p to 13%of cases of pen et rat ing globe t rau m a. Th e cau sat ive organ -
ism differs from oth er en doph th alm it is w ith gram -p osit ive bacteria accou n t ing
for 61.0% cases, gram -n egat ive bacteria for 10.2% of cases, fungi in 8.3% cases, an d
polym icrobial in fect ion s in 15.6% cases. Th e m ost com m on gram -posit ive organ -
ism s w ere coagu lase-n egat ive Staphylococcus (21.5%) an d Bacillus species (18.5%),
follow ed by St reptococcus species (14.8%) an d Staphylococcus aureus (8%).
Presentation
Becau se ocular t raum a gen erally occu rs in a n on sterile environ m en t , m ost inju ring
object s are con tam in ated w ith m ult iple in fect ious agen ts. Pat ien t s w ith a larger
area of lacerat ion , delay in su rger y, rupt u red len s capsu le, retain ed in t raocular
foreign body, n on m et allic foreign body, an d dir t y w ou n d are m ore com m on ly
associated w ith post t rau m at ic en doph th alm it is. Th e pat ien t presen t s w ith pain ,
ph otop h obia, an d decreased vision occurring a variable period, even years, after
pen et rat ing ocu lar t raum a. Sign s of in t raocu lar in fect ion are seen .
Management
Early repair of th e injur y is n ecessar y. A retain ed in t raocu lar foreign body m ay
requ ire a vit rectom y. If th e risk of en doph th alm it is is greater, con sider t aking an
aqu eou s an d vit reous t ap for cu lt ure an d sen sit ivit y. In t ravit real an t ibiot ics such
as van com ycin hydroch loride (1 m g in 0.1 m L) an d am ikacin (0.4 m g in 0.1 m L) or
ceftazidim e (2.25 m g in 0.1 m L) m ay be given at th e t im e of su rger y along w ith
in t racam eral an d in ten sive postop erat ive an t ibiot ics, w h ich are ch anged according
to cult u re an d sen sit ivit y report s.
On ce th e in fect ion is con t rolled, steroids m ay be added to decrease th e in flam -
m at ion .
Postoperative Endophthalmitis
Any su rgical procedure on th e eye th at disru pts th e in tegrit y of th e globe, h ow ever
m in or th e breach m ay be, can lead to postop erat ive en dop h th alm it is, su ch as cat a-
ract , glau com a, vit rectom y, an d radial keratotom y. Postoperat ive en doph th alm it is
represen ts 70% of in fect ive en doph th alm it is. Th e large m ajorit y of cases follow
cataract su rger y, w ith an approxim ate prevalen ce of 0.082 to 0.1%. It can occur
secon dar y to periocu lar flora gain ing access in to th e eye du ring su rger y, organ ism s
being carried in to th e eye as su rface flu id reflu xes th rough th e w oun d du ring sur-
ger y, secon dar y to in t raocu lar len s con t am in at ion if it tou ch es th e ocular surface,
or w ith th e use of con tam in ated irrigat ion solut ion s (Fig. 6.13 ).
Presentation
Th e pat ien t gen erally presen t s w ith pain , redn ess, decreased vision , lid edem a,
h azy corn ea, an d hypopyon . Th ree form s of presen tat ion m ay be seen :
Acute form : Usu ally fulm in an t , occurs 2 to 4 days postop, m ost com m on ly du e
to S. aureus or st reptococci
Delayed form : Moderately severe, occurs 5 to 7 days postop, due to Staphylococ-
cus epiderm idis, coagu lase-n egat ive cocci, rarely fungi
Chronic form : Occu rs as early as 1 m on th postop, due to Propionibacterium
acnes, Staphylococcus epiderm idis, or fungi
Fig. 6.13 Endophthalmitis—conjunctivitis.

Management
Cer tain m easures, such as 3 days of p reoperat ive prophylact ic an t ibiot ics an d pre-
operat ive povidon e-iodin e (5%) scr ub, can h elp reduce th e risk of postoperat ive
en doph th alm it is (POE). Prim ar y use of preoperat ive topical fou rth -gen erat ion
flu oroqu in olon es su ch as m oxifloxacin an d gat ifloxacin is ben eficial an d is bet ter
for preven t ing resistan ce.
Th e En dop h th alm it is Vit rectom y St udy w as a m u lt icen ter ran dom ized t rial per-
form ed at 24 cen ters in th e Un ited St ates (1990 to 1994) to determ in e th e role of
in t raven ou s an t ibiot ics in th e m an agem en t of POE an d th e role of in it ial vit rec-
tom y in m an agem en t . Th e st udy con clu ded th at system ic an t ibiot ics w ere of n o
ben efit an d th at in it ial vit rectom y w as on ly ben eficial for pat ien t s presen t ing w ith
a ver y poor visu al acu it y.
Cu lt u res sh ou ld be t ake n from t h e aqu eou s an d vit re ou s. Th e p ossibilit y of
isolat in g an organ ism from t h e vit reou s is 56 to 70%, w h ereas from t h e aqu eou s
it is 36 to 40%. In est ablish ed e n d op h t h alm it is, oral or in t rave n ou s an t ibiot ics
h ave p oor p e n et rat ion in to t h e vit re ou s cavit y. Hen ce in t ravit real inject ion s are
t h e t reat m en t of ch oice. For gram -p osit ive organ ism s, a sin gle d ose of in t ravit-
real van com ycin 1 m g in (0.1 m L) h as ad e qu ate an t ibiot ic con cen t rat ion s for
over 1 w eek. Am ikacin (0.4 m g in 0.1 m L) an d ceft azid im e (2.25 m g/0.1 m L)
are effe ct ive again st gram -n egat ive organ ism s. Th u s van com ycin com bin e d w it h
am ikacin or ceft azid im e ap p ears to be t h e best com bin at ion for t h e t reat m e n t
of POE.
Dose of subconjunct ival ant ibiot ics: Van com ycin (25 m g in 0.5 m L) an d ceftazi-
dim e (100 m g in 0.5 m L) or am ikacin (25 m g in 0.5 m L)
Dose of topical fort ified ant ibiot ics: Van com ycin (50 m g/m L) an d am ikacin (20
m g/m L), altern at ing ever y 1 to 4 h ours
Dose of cort icosteroids : Topical, sub-conju n ct ival inject ion (dexam eth ason e, 6
m g in 0.2 m L), oral (p redn isolon e 30 m g by m ou th t w ice a day for 5 to 10 days),
or in t ravit real
Endogenous Endophthalmitis
En dogen ou s en doph th alm it is is gen erally of h em atogen ou s origin an d u su ally
affect s adult s w ith predisposing con dit ion s such as diabetes, u rogen it al an d gas-
t roin test in al t ract disorders, en docardit is, an d pat ien ts on im m u n osu ppressives
or h aving u n dergon e invasive procedures. Th e et iological organ ism m ay be gram -
posit ive or n egat ive bacteria or fungi. In th e p ediat ric age grou p, n eon atal in fect ion
h as been seen w ith grou p B st reptococcal or Candida albicans.
Presentation
It m ay presen t acutely or as a slow ly progressive con dit ion . Th e p at ien t m ay pre-
sen t w ith pain an d decreased visual acu it y. Exam in at ion m ay sh ow a spect rum
of clin ical sign s ranging from m in im um sign s of in flam m at ion , hypopyon , vit rit is,
Roth spot s, ret in al periph lebit is, to pan oph th alm it is in severe cases. W h ite ch orio-
ret in al in filt rates w ith fluffy w h ite vit reou s opacit ies (“st ring of pearls” appear-
an ce) m ay be seen in Candida endophthalm it is.
Management
Blood cu lt u res, in t raocu lar cu lt u res obtain ed from both ch am bers, an d cu lt ures
from oth er sites su ch as an in dw elling cath eter are taken . Early in t raven ou s an -
t ibiot ic th erapy is crucial. Th e pat ien t sh ou ld be w orked up system ically to de-
term in e th e et iology, source, an d cau se for th e en dogen ou s en dop h th alm it is. Th e

role of in t ravit real an t ibiot ics an d vit rectom y is con t roversial u n like in exogen ous
en doph th alm it is. Top ical an d periocu lar an t ibiot ic/an t ifungal agen t s along w ith
cycloplegics m ay be used.
Masquerade Syndromes
Masquerade syn drom es are disorders th at occu r w ith in t raocu lar in flam m at ion
an d are often m isdiagn osed as a ch ron ic idiopath ic uveit is. Th ese are m align an cies
th at presen t w ith react ion s in th e an terior an d posterior segm en t of in flam m at ion
th u s m asqu erading as cases of uveit is ( Table 6.2 ).
Intraocular Lymphoma
Most pat ien t s w ith in t raocu lar lym ph om a h ave im m un osuppression . Th ey gen -
erally p resen t w ith blurr y vision an d floaters, w ith slit lam p exam in at ion often
sh ow ing m ild cells an d flare an d kerat ic precipitates. Vit rit is m ay also be seen w ith
su bret in al yellow in filt rates an d som et im es h em orrh agic ret in al vascu lit is.
Intraocular Leukemia
In t raocu lar leu kem ia m ay presen t w ith hypopyon , vit reous an d opt ic n er ve in fil-
t rat ion , an d vascu lit is.
Retinoblastoma
Ret in oblastom a sh ou ld be ruled ou t in ch ildren less th an 3 years of age presen t ing
w ith uveit is. Pseudohypopyon m ay be seen along w ith vit reous seedlings.
Choroidal Melanoma
Approxim ately 5% of uveal m elan om as p resen t w ith ocu lar in flam m ator y reac-
t ion s. A black hypopyon m ay be seen (see ch apter 11, sect ion on Ch oroidal Mela-
n om a).
Table 6.2 Masquerading Cases o f Uveitis
Juvenile Malign an t leukem ias

Retino blasto m a xantho granulo m as and lym pho m as
Pseudohypopyon Recurrent hyphema Anterior cham ber

nodules on iris yellow, poorly de ned Aspirate
Retinal lesions tumors. Iris tissue biopsy
X-ray shows calci cation Central nervous system
lymphoma presents
with vitritis and
choroidal nodules
Intraocular Foreign Body

A retain ed in t raocu lar foreign body can cau se siderosis, m an ifest ing as a focal low -
grade in flam m at ion , cat aract form at ion , or ch ron ic in flam m at ion .
Schw artz-Jampel Syndrome

Rh egm atogen ous ret in al detach m en t m ay be associated w ith an terior ch am ber
react ion an d glau com a.
Juvenile Xanthogranuloma
Juven ile xan th ogran ulom a is a rare pediat ric disorder affect ing th e h ist iocytes of
th e skin . It m ay som et im es affect th e eye an d can presen t w ith m any differing
ocu lar m an ifest at ion s su ch as m asquerade uveit is, h eteroch rom ia, hyph em a, or
glaucom a.
Paraneoplastic Syndrome
Tu m or-associated ret in op athy secon dar y to cu tan eous m elan om a or bron ch ial car-
cin om a m ay som et im es occu r. Su ch pat ien t s m ay h ave bilateral ret in opathy an d
loss of vision .
7 Lens and Cataract
Athiya Agarw al, Soosan Jacob , and Am ar Agarw al
Posterior Polar Cataract
Th e posterior polar cat aract h as a un ique circular w h orl-like app earan ce located in
th e cen t ral axis n ear th e n odal poin t of th e eye w ith th e rest of th e len s rem ain ing
clear. It is frequ en tly associated w ith a w eaken ed or deficien t posterior capsule.
Missing th e diagn osis in a posterior polar cataract can be cat ast roph ic an d a n igh t-
m are.
Presentation
Th e bu ll’s-eye appearan ce is path ogn om on ic of posterior polar cataracts. How ever,
th is en t it y could be cam ouflaged u n der a den se n uclear sclerosis or a tot al w h ite
cataract ( Fig. 7.1A).
In terdigit at ion w ith th e posterior cap su le is ch aracterist ic as opposed to a poste-
rior su bcapsular cat aract .
Management
A sm all, con t in u ous cur vilin ear cap su lorh exis is aim ed for in th e even t u alit y of
th e in t raocu lar len s h aving to be placed in th e su lcu s. Hydrodissect ion m ay cau se
hydraulic perforat ion at th e w eaken ed area of th e capsu le; h en ce on ly a careful,
con t rolled hydrodelin eat ion is p referred. Th is epin uclear sh ell provides addit ion al
protect ion by tam pon ading any vit reous or capsular breach du ring ph acoem ulsi-
A B
Fig. 7.1 (A) Posterior polar cataract. Note hydrodelineation only done. No hydrodis-
section has been done. (B) Microphakonit is started. Note the 0.7-mm irrigating chop-
per and 0.7-m m phako tip without the sleeve inside the eye. All instruments are made
by MicroSurgical Technology, Redmond, WA. The assistant continuously irrigates the
phaco probe area from outside to prevent corneal burns.
218
7 Lens and Cataract 219
ficat ion . A sm all am oun t of viscoelast ic can be injected ju st u n der th e rim of th e

rh exis all arou n d to form a m ech an ical barrier for flu id from acciden tally en tering
th e subcapsular plan e w h ile perform ing hydrodelin eat ion . Because th e n u cleu s af-
ter hydrodelin eat ion is ver y sm all, it can be rem oved easily eith er by carou selling
(con st an tly rot at ing th e n ucleus to prolong occlusion an d allow m ore effect ive
breakdow n of th e cataract) it ou t w ith th e ph aco t ip or by u sing a ch op m an euver.
Ph aco ch op is esp ecially h elpfu l in case of associated n uclear sclerosis. If th e cen -
t ral plaque w as n ot rem oved at th e t im e of su rger y, it can be tackled by a yit t riu m
alu m in u m garn et capsu lotom y postoperat ively.
Sub 1 mm 700-Micrometer Ca ta ra ct Surgery—Micropha konit
Microph akon it or bim an ual ph acoem u lsificat ion th rough t w o 0.7-m m in st ru-
m en ts (an irrigat ing ch opper an d a ph aco t ip) can be u sed effect ively to t ackle a
posterior polar cat aract . Hydrodelin eat ion can be don e th rough both por ts h ere.
An oth er advan t age of th is tech n ique is th at on e can easily rever t to bim an ual vit-
rectom y in case of vit reou s loss. Th e advan tage of m icroph akon it over ph aco is
th at on e h as a closed ch am ber th rough ou t su rger y becau se both th e in cision s are
so sm all ( Fig. 7.1B).
Subluxated Cataract
Sublu xated cataracts pose a risk of n u cleu s drop du ring cataract su rger y, an d h en ce
requ ire sp ecial precau t ion s.
Presentation
Th ere can be a zon u lar deh iscen ce or w eakn ess presen t .
Colobom a of th e len s. Th ere can be colobom a w ith a su blu xat ion ( Fig. 7.2A).
A B
Fig. 7.2 (A) Subluxated colobomatous lens. (B) Aniridia rings being implanted. ([A]
Courtesy of Lincoln L. Freitas)
Management
Cat aract su rger y in th e presen ce of zon ular w eakn ess or a su blu xated len s is a
great ch allenge. In th e past , su rgical in ter ven t ion in th ese cases w as difficu lt , lead-
ing to com plicat ion s. Th e u se of an en docapsular flexible polym ethyl m eth acr ylate
(PMMA) ring h as ch anged th e su rgical approach to su blu xated cataracts. Im plan ta-
t ion of a capsular ten sion ring (CTR) st abilizes a loose len s an d allow s th e surgeon
to place th e in t raocu lar len s in th e m ost desirable p lace—th e capsular bag. Th ere
are n u m erous oth er advan tages: vit reous h ern iat ion to th e an terior ch am ber is re-
du ced, a t aut capsu le gives coun tert ract ion to all t ract ion m an euvers m aking th em
easier to perform , capsular su ppor t for an “in th e bag” im plan t is obtain ed, an d
m ost im por tan tly, th e capsu lar bag m ain t ain s its sh ape, avoiding capsu lar fibrosis
syn drom e an d in t raocu lar len s decen t rat ion .
Do n ot use t r ypan blue in sublu xated cataracts because th e tr ypan blue w ill go
into th e vit reous cavit y through th e zonular dehiscen ce an d m ake th e w hole vitre-
ous cavit y blue. W h en zonular deh iscence is large in extent or progressive in n at ure,
capsular bag shrin kage resulting in in traocular len s decen tration and pseudoph a-
kodonesis m ay occur even after a successful surger y w ith a capsular ring. Com plete
lu xation of the bag an d its con ten ts has also been reported. For such cases, Cionn i’s
m odified design w ith a fixat ion h ook is a good solution . The h ook is kept in th e area
of dialysis an d is pulled periph erally using a t ran sscleral fixation sut ure to counter-
act capsular bag decen trat ion an d tilt . In severe cases, t w o such rings or th e t w o-
hooked m odel can be used. An altern ative in cases of severe decentration is to m ake
a sm all equatorial capsulorrhexis through w h ich a standard capsular tension ring
can be in serted. A scleral sut ure can th en be passed around th e exposed capsular
ten sion ring, w h ich is th en used to cen ter th e lens before capsulorrh exis. Peribulbar
anesth esia is suitable for creat ion of scleral w indow s an d transscleral sut uring of
th e capsular ring or of th e in traocular lens if n ecessar y.
CTRs can also be placed to cover sector iris defects or colobom a. Th ese colobom a
sh ields h ave an in tegrated 60- to 90-degree sector sh ield to protect again st glare
an d m on ocu lar diplopia. More th an on e capsu lar ten sion ring can be used if m ore
th an 90 degrees of defect is presen t . Mult isegm en ted colobom a rings are available
for an iridia as w ell as for cases w ith large perm an en tly dilated pu pils secon dar y
to any cause. In sert ion of t w o of th ese rings so th at th e in terspaces of th e first ring
are covered by th e sector sh ields of th e secon d m akes a con t iguous ar t ificial iris
possible ( Fig. 7.2B).
Miotic Pupil Cataract
A w ell-dilated pu pil is th e gatew ay to a sm ooth , easy, an d rew arding cataract

su rger y. Bu t th e su rgeon m ay n ot be lucky en ough to sail sm ooth ly th rough th e
w ell-ch arted path of a dilated pu pil each t im e. Som et im es th e door looks n arrow
an d un invit ing even for th e best . A m iot ic pu pil is a com m on bugbear th at ever y
su rgeon faces at som e t im e.
Presentation
A sm all pu pil affects all steps of ph acoem ulsificat ion , righ t from capsu lorrh exis
to in t raocu lar len s in ser t ion . Difficult m an euvering cau ses iris dam age, sph in cter
tears, zon u lar dialysis, bleeding, an d so on . Poor exposure th rough a sm all pu pil
forces th e surgeon to m ake a sm aller rh exis, adding to th e difficu lt y an d frequen tly
leading to capsu lar deh iscen ce an d n ucleus drop—th e w orst n igh t m are. Th e pro-
longed surgical t im e takes it s toll th ereafter. Corn eal edem a, uveit is, secon dar y
glaucom a, cystoid m acu lar edem a, distor ted pu pil—th e list is en dless.
Causes of sm all pup il
Management
Sphincter-Spa ring Techniques

Ph arm acological m ydriasis alon e m ay n ot be effect ive in cases w ith posterior syn -
ech iae, pu pillar y m em bran e, or scarred pupils. Su ch pu pils n eed in t raoperat ive
procedu res su ch as h igh -m olecu lar-w eigh t coh esive viscoelast ics, syn ech iolysis,
viscom ydriasis, an d/or pupillar y m em bran e st ripping.
Sphincter-Involving Techniques
Min isp h in cterotom ies (less th an 1 m m ) lim ited to th e sph in cter t issu e can be
m ade w ith eith er Van ass scissors or th e vit reoret in al scissors. Dilat at ion can also
be ach ieved by pu pillar y st retch ing u sing push -pull in st ru m en t s. Bip ronged, t ri-
pronged, an d quadripronged pu pil st retch ers are also ver y effect ive. Oth er altern a-
t ives are com m ercially available iris h ooks, pupil ring expan ders, an d th e Malyugin
ring ( Fig. 7.3A,B).
Fig. 7.3 (A) Tri-pronged pupil

stretchers. (B) Iris hooks inserted to
enlarge the pupil. B
Mature Cataract
One of the biggest bugbears for a phaco surgeon is to perform a rhexis in a m a-

ture cataract. Once one perform s rhexis in m ature and hyperm ature cataracts, then
phaco can be done in these cases and a foldable intraocular lens can be im planted.
Presentation
Th e solu t ion to m at u re cat aract s is to h ave a dye th at st ain s th e an terior capsu le.
Th is dye is t r ypan blu e (Fig. 7.4A,B). On e can also use in docyan in e green .
Ch eck for variou s causes of m at u re cataracts.
Management
Th e problem w h en on e operates a m at ure cataract is th e creat ion of th e rh exis,
w h ich can be solved u sing a dye. Th e oth er problem is surge. On e sh ou ld un der-
stan d th e w orking of a ph aco m ach in e for un derstan ding surge. W h en an occlu ded
fragm en t is h eld by h igh vacu um an d th en abruptly aspirated, fluid ru sh es in to th e
Fig. 7.4 (A) Rhexis being done in

a mature cataract with cystotome.
(B) Rhexis forceps (MicroSurgical
Technology, Redmond, WA) used to
perform the rhexis in a m ature cata-
ract. Note the trypan blue (Blurhex,
Dr. Agarwal Pharm a Ltd., Chennai,
B India) staining the anterior capsule.
ph aco t ip to equ ilibrate th e bu ilt-up vacu um in th e asp irat ion lin e, causing su rge.
Th is leads to sh allow ing or collapse of th e an terior ch am ber. Differen t m ach in es
em ploy a variet y of m eth ods to com bat surge. An oth er m eth od is th e air pu m p. An
au tom ated air pu m p is used to pu sh air in to th e in fu sion bot tle, th us in creasing th e
pressure w ith w h ich th e fluid flow s in to th e eye. Th is in creases th e steady-state
pressure of th e eye, m aking th e an terior ch am ber deep an d w ell m ain tain ed dur-
ing th e en t ire procedure. It m akes ph akon it an d ph acoem ulsificat ion a relat ively
safe p rocedu re by reducing su rge even at h igh vacu um levels ( Figs. 7.5 , 7.6 , 7.7 ,
an d 7.8 ).
Fig. 7.5 The principles of how the phaco m achine works. This conceptual view shows
the three main elements of m ost phaco systems. (1) The irrigation (red): Intraocular
pressure is maintained and irrigation is provided by the bot tle of balanced salt solution
(B) connected via tubing to the phaco handpiece (F). It is controlled by the surgeon. Ir-
rigation enters the eye via an infusion port (H) located on the outer sleeve of the bi-tube
phaco probe. Height of the bot tle above the eye is used to control the inflow pressure.
(2) Aspiration (blue): (I) enters through the tip of the phaco probe, passes within the
inner tube of the probe, travels through the aspiration tubing, and is controlled by the
surgeon by way of a variable-speed pump (J). The peristaltic t ype pump is basically a mo-
torized wheel exerting rotating external pressure on a portion of the flexible aspiration
line, which physically forces fluid through the tubing. Varying the speed of the rotating
pump controls the rate of aspiration. Aspirated fluid passes to a drain (l). (3) Ultrasonic
energy (green) is provided to the probe tip via a connection (M) to the unit. All three of
these m ain phaco functions are under control of the surgeon by way of a multicontrol
foot pedal (N). (Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of
Cataract Surgery.” Highlights of Ophthalmology, English Edition, 2001.)
Fig. 7.6 Mechanism of the undesirable surge phenomenon. One problem area of the
closed phaco system occurs during abrupt dislodging of an occluding piece of lens ma-
terial so that it no longer occludes the aspiration port of the phaco tip. A sudden drop in
intraocular pressure occurs as the fluid rate into the eye fails to imm ediately match the
sudden fluid rate out of the eye. This is known as the surge phenomenon. (A) A piece of
lens material occluding the aspiration port of the phaco tip is held in place by vacuum
pressure created by the operating pump (D). (Note there is no drainage (E) from the
blocked system.) Infusion from the irrigating bot tle (C) has ceased, but it is still pro-
viding controlled intraocular pressure due to its elevated position above the eye. With
sufficient vacuum pressure from the pump and/or emulsification from the ultrasonic
energy, the nuclear piece will abruptly enter the aspiration port and the fluid system
will once again open (B). Because the plastic infusion/aspiration lines and the eye walls
are flexible in absorbing the sudden inflow–outflow pressure differential, there occurs
a moment when the infusion fluid (G, small arrow) does not effectively enter the eye
fast enough to replace the fluid suddenly moving out of the unblocked system (F, large
arrow). Outflow rate from the force of the pump is momentarily greater than the replac-
ing infusion rate. This out-of-balance system (out of balance in not providing constant
intraocular pressure) in which the eye m omentarily absorbs the inflow–outflow rate dif-
ferential may traumatically collapse the eye for a short period. (Courtesy of Benjamin F. Boyd,
MD, FACS, Editor-in-Chief, “The Art and the Science of Cataract Surgery.” Highlights of Ophthalmology,
English Edition, 2001.)
Fig. 7.7 Technical solution to prevent the undesirable surge phenomenon. One techni-
cal solution for elim inating the surge phenomenon involves the use of a high-tech micro-
processor. When a nuclear piece (F) occludes the aspiration port and then suddenly (B) is
aspirated (F, arrow) by the vacuum pressure of the pump (P), a sensor (E) located on the
aspiration line signals a microprocessor (G) in the unit that an abrupt surge in aspiration
flow has begun to take place. Within milliseconds, the microprocessor directs the motor
of the pump (P) to slow down. The reduction in aspiration rate resulting from the slowed
pump occurs before the eye can collapse from any volum e differential encountered be-
t ween sudden inflow and outflow rates. The potentially dangerous surge phenomenon
is avoided. This elimination of the surge phenomenon allows the surgeon to safely use
higher vacuum rates (necessary in some situations) with a reduction in the need to use
potentially damaging high ultrasonic power set tings. Surgery becomes safer and faster.
(Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of Cataract Surgery.”
Highlights of Ophthalmology, English Edition, 2001.)
Fig. 7.8 Diagrammatic represen-

tation of the connection of the air
pump to the infusion bot tle.
Senile Cataract
On e sh ould perform cat aract su rger y w ell in sen ile cataracts (Fig. 7.9 ).
Presentation
Th e fu n dam en tal goal of Ph aco is to rem ove th e cat aract w ith m in im al dist urban ce
to th e eye u sing th e least n um ber of su rgical m an ipulat ion s. Each m an euver sh ou ld
be perform ed w ith m in im al force, an d m a xim al efficien cy sh ould be obtain ed.
Fig. 7.9 Rhexis being done in a

case of senile cataract.
Oth er system ic causes of cataract
Management
After viscoelast ic is injected in to th e eye th rough a 26-gauge n eedle, a globe stabi-
lizat ion rod is in ser ted an d a clear corn eal in cision is m ade. On e can th en rem ove
th e cataracts u sing th e divide an d con quer tech n iqu es. We prefer th e ch opping
tech n ique ( Figs. 7.10 an d 7.11 ). Th e Ph aco probe is in ser ted th rough th e in cision
sligh tly sup erior to th e cen ter of th e n u cleu s, an d th e ph aco t ip is em bedded in
th e n u cleu s w ith th e t ip directed obliqu ely dow nw ard tow ard th e vit reou s an d n ot
h orizon t ally tow ard th e iris. On ce th e t ip is em bedded, w h ile in foot posit ion 2,
th e n u cleus is ch opped w ith a st raigh t dow nw ard m ot ion at th e en d of w h ich th e
ch op per m oves to th e left on reach ing th e cen ter of th e n ucleus (i.e., like a laterally
reversed L) ( Fig. 7.12 ). Th e n u cleu s is th en rot ated 180 degrees an d cracked again
to get t w o h alves of th e n u cleu s. Th is is th en repeated to fu r th er ch op th e cataract
in to sm aller pieces, w h ich are th en brough t in to th e an terior ch am ber on e by on e
an d em ulsified. On ce th e n u cleus an d cor tex are rem oved th e foldable in t raocular
len s is im plan ted ( Fig. 7.13 ).
Fig. 7.10 A diam ond knife blade (D) enters the first incision (1), the second tunnel inci-
sion (2), and is then directed slightly oblique to the iris plane and advanced (arrow) into
the anterior chamber. This forms the internal aspect of the incision into the cham ber
(A). This is the third step (3) in the three -step self-sealing incision. (Courtesy of Benjamin F.
Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of Cataract Surgery.” Highlights of Ophthalmol-
ogy, English Edition, 2001.)
Fig. 7.11 Emulsification of lens fragments: This surgeon’s view shows the manage -
ment of the lens quadrants. The apex of each of the four loose quadrants is lifted with
the second instrument (S), the ultrasound phaco tip (P) is em bedded into the posterior
edge of each, and by m eans of aspiration the surgeon centralizes each quadrant for
emulsification. U.S., ultrasound; Asp., aspiration flow rate; Vac., vacuum; C, capsule;
F, fragment. (Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of
Cataract Surgery.” Highlights of Ophthalmology, English Edition, 2001.)
Fig. 7.12 This cross-section view

shows the phacoemulsification
probe removing the nucleus frag-
m ents within the capsular bag. Note
the apex of one of the fragments
created in the nucleus being lifted
with the second instrument (arrow)
and the ultrasound tip embedded
into the posterior edge of each seg-
m ent ready for emulsification. The
epinucleus and cortex will then be
removed during the phaco process.
If we operate on a softer cataract,
the freed fractured pieces are emul-
sified immediately. (Courtesy of Benja-
min F. Boyd, MD, FACS, Editor-in-Chief, “The
Art and the Science of Cataract Surgery.”
Highlights of Ophthalmology, English Edi-
tion, 2001.)
Fig. 7.13 This cross-section view shows the movement of

the foldable intraocular lens during insertion. Folding forceps
removed for clarit y. (1) Folded lens outside the eye. (2) Folded
lens passing through small incision. (3) Folded lens placed pos-
teriorly into the capsular bag through anterior capsule open-
ing and then rotated 90 degrees. (4) Lens slowly unfolded in
the bag. (5) Final unfolded position of lens within the capsular
bag. (Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art
and the Science of Cataract Surgery.” Highlights of Ophthalmology, English
Edition, 2001.)
Glued Intraocular Lens
In t raocu lar len s (IOL) im plan t at ion in eyes th at lack posterior capsular su ppor t h as
been accom plish ed in th e past by m ean s of an iris-fixated IOL, an terior ch am ber
IOL, an d t ran sscleral IOL fixat ion th rough th e ciliar y su lcus or pars plan a. Su rgical
exper t ise, prolonged su rgical t im e, su t u re-in du ced in flam m at ion , su t u re degrada-
t ion , an d delayed IOL sublu xat ion or dislocat ion due to broken su t u re are som e of
th e lim itat ion s in sut u red scleral fixated IOLs.
It is possible to p lace a posterior ch am ber IOL in eyes w ith a deficien t posterior
capsule using a glu ed IOL tech n iqu e.
Technique
After clam ping th e su perior rect us, an in fu sion can n u la or an terior ch am ber m ain -
tain er is in serted, preferably in th e in feron asal quadran t to preven t in terferen ce in
creat ing th e scleral flap s, w h ich is th e n ext step in th e surger y. Tw o part ial-th ick-
n ess lim bal-based scleral flaps ~2.5 × 3.0 m m are th en created exactly 180 degrees
A B
Fig. 7.14 (A) Scleral flaps (sf) of 2.5 x 3.0 mm made ~ 1.5 m m from the limbus. (B)
Two flaps are made exactly 180 degrees diagonally apart.
diagon ally apar t ( Fig. 7.14 ) up to th e lim bus. Th is is follow ed by vit rectom y via
a pars plan a or an terior route to rem ove all vit reous t ract ion . Tw o st raigh t scle-
rotom ies w ith a 22-gauge n eedle are m ade ~1.5 m m from th e lim bu s un der th e
exist ing scleral flaps. A corn eo-scleral or scleral t u n n el in cision is th en prepared
for in t rodu cing th e IOL in secon dar y IOL im plan tat ion .
W h ile th e IOL is being in t rodu ced w ith on e h an d u sing a McPh erson forceps, an
en d-gripping 23-gauge MST m icro rh exis forceps (MicroSurgical Tech n ology, Red-
m on d, WA) is passed th rough th e in ferior sclerotom y w ith th e oth er h an d. Th e t ip
of th e leading h apt ic is th en grasped w ith th e m icro rh exis forceps, pulled th rough
th e in ferior sclerotom y follow ing th e cu r ve of th e h apt ic (Figs. 7.15 an d 7.16 ), an d
extern alized un der th e in ferior scleral flap. Sim ilarly, th e t railing h apt ic is also ex-
tern alized th rough th e su perior sclerotom y u n der th e scleral flap. Scleral t u n n els
are m ade w ith a 26-gauge n eedle at th e edge of th e scleral flap an d th e h apt ics are
th en t u cked in to th ese scleral t u n n els for addit ion al stabilit y of th e IOL (Fig. 7.17).
Recon st it u ted fibrin glu e is th en injected th rough th e can n ula of th e dou ble-sy-
ringe deliver y system un der th e su perior ( Fig. 7.18 ) an d in ferior scleral flap s. Local
pressure is given over th e flap s for ~10 to 20 secon ds for th e form at ion of fibrin
polypept ides. In pat ien t s w h o h ave a lu xated IOL, sim ilar lam ellar scleral flaps as
A B
Fig. 7.15 (A) The IOL is inserted with a McPherson forceps, and the leading haptic is
grasped by an end-gripping 23-gauge micro rhexis forceps (f) passed through a scle -
rotomy wound. The haptic is then externalised under the scleral flap (sf). (B) The trailing
haptic is also externalized in a similar manner.
A B
Fig. 7.16 (A) One haptic of the IOL is grasped by an end-gripping 23-gauge micro rhexis
forceps (f) passed through sclerotomy wound. The haptic (h) is then externalised under
the scleral flap (sf). (B) The second haptic is also externalized in a similar manner.
Fig. 7.17 (A) The haptics are externalized and a 26-gauge needle is taken to create a
scleral tunnel. (B) 26-gauge needle is seen making a scleral tunnel near the edge of the
flap, parallel to the limbus. (C) The haptic is tucked into the tunnel made with the 26-
gauge needle. This provides it additional stabilit y.
A B
Fig. 7.18 (A) Reconstituted fibrinogen and thrombin preparation of fibrin glue (FG)
injected beneath the scleral flaps. (B) Scleral flaps (arrow) sealed well with the scleral
bed.
Fig. 7.19 Conjunctiva closed with

fibrin glue.
described earlier are m ade an d th e lu xated IOL h apt ic is th en grasped w ith th e 23-
gauge m icro rh exis forceps an d extern alized, t ucked in to th e scleral t u n n el m ade at
th e edge of th e flaps, an d th en glu ed un der th e scleral flaps. Th e in fu sion can n ula
is th en rem oved. Conjun ct iva is also closed w ith th e sam e fibrin glu e ( Fig. 7.19 ).
Th is tech n ique is u seful in a m yriad of clin ical sit uat ion s w h ere scleral-fixated
IOLs are in dicated, such as a lu xated IOL, dislocated IOL, zon ulop athy, or secon d-
ar y IOL im plan t at ion . In dislocated posterior ch am ber polym ethyl m eth acr ylate
(PMMA) IOL, th e sam e IOL can be reposit ion ed, th ereby redu cing th e n eed for fur-
th er m an ipu lat ion . It can be perform ed w ell w ith a rigid PMMA IOL, IOLs w ith
m odified PMMA h apt ics, or m u lt ifocal glued IOLs. On e th erefore does n ot n eed
to h ave special scleral fixated IOLs w ith eyelet s or n ew er h apt ic design s. Becau se
Fig. 7.20 Im age of the scleral flap as seen by anterior seg-

ment optical coherence tomography on day 1 (above) and
well sealed scleral flaps at 6 weeks (below).
Fig. 7.21 Postoperative anterior segm ent optical coherence tomography showing 360
degrees well-centered intraocular lens at 6 weeks.
th e h apt ic is being placed in its n orm al cur ved con figu rat ion w ith ou t any t ract ion ,
th ere is n o distort ion or ch ange in sh ape of th e IOL opt ic. Extern alizat ion of th e
greater par t of th e h apt ics along it s cu r vat u re stabilizes th e a xial posit ion ing of th e
IOL an d th ereby preven ts any IOL t ilt ( Figs. 7.20 an d 7.21 ).
Temporary Haptic Externalization
On e of th e m ost difficult steps of reposit ion ing a dislocated posterior ch am ber IOL
is secu ring a sut u re on th e h apt ics. In 1992, Clem en t Ch an first in t roduced th e con -
cept of tem porar y h apt ic extern alizat ion to en h an ce th e ease of su t ure placem en t
an d ch anged IOL reposit ion ing from an un con t rolled to a h igh ly con t rolled set t ing
th at allow s fixat ion of th e dislocated IOL in th e ciliar y su lcu s on a con sisten t basis
( Fig. 7.22 ).
A th ree-port pars p lan a vit rectom y is perform ed for th e rem oval of th e an terior
an d cen t ral vit reou s adjacen t to th e dislocated IOL to p reven t any vit reoret in al
t ract ion during th e process of m an ipu lat ing th e IOL. Tw o diam et rically opposed
lim bal-based part ial-th ickn ess t riangu lar scleral flaps are prepared along th e h ori-
zon tal m eridian s at 3 an d 9 o’clock. Cau terizat ion is don e to preven t any bleeding
An terior sclerotom ies w ith in th e beds un der th e scleral flaps are m ade at 1 to 1.5
m m from th e lim bus. As an altern at ive to th e scleral flaps, th e an terior scleroto-
m ies m ay be m ade w ith in th e scleral grooves at 1.0 to 1.5 m m from th e h orizon tal
lim bu s.
A fiberopt ic ligh t pipe is in serted th rough on e of th e posterior sclerotom ies,
w h ile a pair of fin e, n on angled posit ive-act ion forceps (e.g., Griesh aber 612.8,
Alcon Griesh aber, Ltd., Sch affh au sen , SZ) is in ser ted to h old th e IOL an d bring it
an teriorly. A forceps is th en passed th rough th e an terior sclerotom y of th e oppos-
A B
Fig. 7.22 (A) A forceps is passed through a sclerotomy made under a scleral flap in
the opposite quadrant and one haptic of the dislocated IOL is caught for temporary
externalization. (B) A double -armed 9–0 or 10–0 polypropylene suture is tied around
the externalized haptic to make a secured knot and it is re -introduced into the vitreous
cavit y. The same process is then repeated for the other haptic. (C) The internalized hap-
tics are anchored securely in the ciliary sulcus by taking scleral bites with the external
suture needles.
ing quadran t to engage on e h apt ic of th e dislocated IOL for th e tem porar y exter-
n alizat ion . A dou ble-arm ed 9–0 (Eth icon TG 160–8 plus, Som er ville, NJ) or 10–0
polypropylen e sut u re (Eth icon CS 160–6, Som er ville, NJ) is t ied arou n d th e ex-
tern alized h apt ic to m ake a secured kn ot . Th e sam e process is repeated for th e
oth er h apt ic after th e su rgeon sw itch es th e in st ru m en ts to th e opposite h an ds.
Th e extern alized h apt ics w ith th e t ied sut u res are rein tern alized th rough th e cor-
resp on ding an terior sclerotom ies w ith th e sam e forceps. Th e in tern alized h apt ics
are an ch ored securely in th e ciliar y sulcus by taking scleral bites w ith th e extern al
su t u re n eedles on th e lips of th e an terior sclerotom ies. By adjust ing th e ten sion of
th e op posing sut u res w h ile t ying th e polypropylen e sut u re kn ot s by th e an terior
sclerotom ies, th e opt ic is cen tered beh in d th e p upil, an d th e h apt ics are an ch ored
in th e ciliar y sulcus.
Intraoperative Complications
Posterior Capsular Rupture

If a capsular tear does occu r, several steps can h elp m in im ize vit reou s loss. A
closed system sh ould be m ain t ain ed by inject ing viscoelast ic before w ith draw ing
th e ph aco t ip. Th is h elps to tam pon ade th e vit reou s backw ard w h ere a capsu lar
deh iscen ce is presen t . Cor t ical rem oval sh ould th en proceed w ith eith er low in fu-
sion or by a dr y tech n ique filling th e ch am ber w ith viscoelast ic m aterial an d us-
A B
Fig. 7.23 (A) Bimanual vitrectomy being done in a case after posterior capsular rup-
ture and vitreous loss. One should never do a coaxial vitrectomy. (B) Case of a dropped
intraocular lens and nucleus (see the white reflex) after a posterior capsular rupture.
ing a Sim coe aspirator. If th e hyaloid face is broken an d vit reou s presen ts th rough
th e ru pt u re, a rout in e t w o-por t m ech an ized vit rectom y using an Accur us Vit rec-
tor (Accuru s 800 CS, Alcon Laboratories, For t Worth , TX) using low -flow an d low -
vacu um param eters is perform ed. Th e tear can th en be converted in to a roun d an d
stable open ing. Th e IOL can th en be safely placed in to th e capsu lar bag. An u n con -
t rolled tear n ecessitates im plan t at ion in to th e ciliar y su lcu s. A 6-m m opt ic acr ylic
len s is preferred w h en im plan t ing in th e bag as it un folds slow ly, th us causing less
st ress on th e posterior capsu le. Th e leading edge of th e len s sh ould be directed
aw ay from th e area of a w eak capsu le. On e can develop a dropped IOL an d n ucleu s
also ( Fig. 7.23A,B).
Iridodialysis
On e can develop an iridodialysis, w h ich w ill requ ire su t u ring (Fig. 7.24 ).
Fig. 7.24 Iridodialysis.

Fig. 7.25 Torn intraocular lens.
Torn Intraocular Lens

An IOL can get torn during im plan t at ion . In such a case it sh ould be explan ted an d
replaced ( Fig. 7.25 ).
Postoperative Complications
Corneal Edema after Cataract Extraction

Corn eal edem a after a cataract surger y can be iden t ified im m ediately after th e
cataract su rger y. It is best appreciated a few h ou rs after th e cataract surger y. Most
com m on causes th at lead to epith elial or st rom al edem a after th e cataract surger y
are m ech an ical t rau m a, prolonged in t raocular irrigat ion , in flam m at ion , in creased
in t raocular pressu re (IOP), Descem et m em bran e det ach m en t , in t raocu lar toxin s,
an d a com p licated cataract su rger y.
Presentation
An associated en doth elial abn orm alit y or decom p en sat ion h as to be ruled out pre-
operat ively. Th ere is an associated in crease in th e corn eal th ickn ess. An in creased
epith elial th ickn ess w ith associated h azy corn ea, irregu lar surface, an d epith elial
defect form th e h allm arks of epith elial edem a. Th e th ickn ess ret urn s to n orm al
after a few h ou rs of su rger y. St rom al edem a is associated w ith a m arked in crease
in th e corn eal th ickn ess, corn eal h aze, an d en doth elial folds involving m ain ly th e
cen ter of th e corn ea ( Fig . 7.26 ). Brow n -McLean syn drom e, a clin ical con dit ion
arising after cat aract surger y, is ch aracterized by periph eral corn eal edem a w ith
a clear cen ter.
Fig. 7.26 Postoperative striate stri-

ate keratitis. B
Management
As a r ule, if th e corn eal p eriph er y is clear, th e corn eal edem a reduces in a few
h ou rs. Cases w ith epith elial edem a requ ire a m edical reduct ion of IOP if th e edem a
does n ot resolve in a few h ou rs. A st rom al corn eal edem a p ersist ing for a few days
is requ ired to be m an aged w ith hyper ton ic eyedrops an d steroids. A vit rectom y is
in dicated in pat ien t s w ith exist ing vit reocorn eal adh eren ce w ith associated cor-
n eal edem a. A Descem et m em bran e detach m en t can be h an dled by inject ing air
or gas (C3 F8 or SF6 ). Edem a exist ing for m ore th an 3 m on th s u sually requ ires a
p en et rat ing keratoplast y.
Hypotony and Wound Leak after Cataract Extraction

Hyp otony due to w ou n d leak after cataract su rger y is a com m on com plicat ion of
th e cat aract surger y. Th e leakage can occur from th e in cision site, th e side por t , or
th e bleb in cases w ith th e com bin ed t rabecu lectom y su rger y.
Presentation
Wou n d leakage can be picked u p on slit lam p an d by em ploying a Siedel test . Mild
to m oderate leaks presen t w ith a sh allow an terior ch am ber, leaking w oun d, irriga-
t ion , tearing, con t act len s in toleran ce, in fect ion , an d sign ifican t hypotony. Severe
leaks m ay be associated w ith iridocorn eal touch w ith severe corn eal edem a.
Management
Most of th e leaks are self-lim it ing an d requ ire obser vat ion w ith patch ing an d use
of topical an d system ic aqu eou s suppressan ts. Tem porar y redu ct ion of th e topical
steroids h elps in prom pter h ealing by in creasing in flam m at ion . In ch ron ic cases
use of cyan oacr ylate glu e, sim ple w oun d sut uring, an d an terior ch am ber reform a-
t ion w ith salin e or air follow ed by w ou n d sut u ring h elps.
Elevated IOP after Cataract Extraction

An IOP fluct uat ion im m ediately after cataract surger y is a com m on bu t self-lim -
it ing p roblem in m ost of th e cases. Reten t ion of viscoelast ic m aterial in th e eye
du ring th e su rger y is on e of th e m ost com m on cau ses of p ostop erat ive in flam -
m at ion an d IOP rise. Oth er cau ses of in creased IOP after cat aract su rger y in clude
pupillar y block, hyph em a, ciliar y block, en doph th alm it is, retain ed len s m aterial,
iris pigm en t release, preexist ing glaucom a, use of steroids, an d periph eral an terior
syn ech iae.
Presentation
Th e pat ien t u sually com plain s of h azy vision an d pain . Hazy corn eas du e to epi-
th elial or st rom al edem a an d associated an terior ch am ber in flam m at ion are com -
m on .
Management
Most of th e cases are m ild an d self-lim it ing an d requ ire on ly m on itoring. A rise
in IOP n oted in t raoperat ively can be m an aged by depressing th e low er lip of th e
paracen tesis site to evacuate som e of th e fluid out of th e eye. How ever, a sign ifi-
can t an d su stain ed rise in IOP m ay n ecessitate t im ely an d specific m an agem en t of
several circu m stan ces. Secon dar y glau com as are h an dled by t reat ing th e u n derly-
ing cau se.
Cystoid Macular Edema after Cataract Extraction

Cystoid m acu lar edem a (CME) is a pain less con dit ion in w h ich cyst ic sw elling or
th icken ing occurs of th e cen t ral ret in a (m acu la) an d is u su ally associated w ith
blurred or distor ted vision . W h en CME develops follow ing cat aract su rger y an d its
cau se is th ough t to be directly related to th e surger y, it is referred to as Ir vin e- Gass
syn drom e. Th e Mü ller cells in th e ret in a becom e over w h elm ed w ith fluid, leading
to th eir lysis. Th is result s in an accu m ulat ion of fluid in th e ou ter plexiform an d
in n er n u clear layers of th e ret in a. Usual cau ses of CME are vascu lar in st abilit y,
in t raocular in flam m at ion , an d m ech an ical forces (e.g., epiret in al m em bran e, vit-
reom acu lar t ract ion ). Th e in ciden ce of CME is 1% after ph acoem u lsificat ion an d
20% after ext ra-capsular cat aract ext ract ion (ECCE).
Presentation
Pat ien ts w ith CME u sually presen t w ith decreased or blurr y vision . Slit-lam p bio-
m icroscopy reveals blun ted or irregu lar foveal ligh t reflex, ret in al th icken ing, an d/
or in t raret in al cyst s in th e foveal region . Opt ic disk edem a, epiret in al m em bran e/
m acu lar pu cker, diabet ic ret in opathy, an d uveit is m u st be looked for to rule out
oth er causes. On fu n dus fluorescein angiograp hy a pet aloid pat tern of leakage in
th e m acu la occurs. Opt ical coh eren ce tom ography (OCT) is h elpful in establish ing
a diagn osis an d in m easu ring th e th erapeut ic respon se (Fig. 7.27 ).
A B
C D
Fig. 7.27 (A) Color photograph of the fundus shows an absent foveal reflex
with cystoid edema. (B) Fundus fluorescein angiography shows a characteristic
patelloid pat tern of hyperfluorescence in the early venous phase, which increases
in intensit y in the later phases (C,D) of the angiogram.
Diabet ic m acu lar edem a, ret in it is pigm en tosa, juven ile ret in osch isis, vit reom acu-
lar t ract ion syn drom e, epiret in al m em bran e, Goldm an n -Favre syn drom e, m acu lar
cyst , an d foveal sch isis in h igh m yop es
Management
Treat m en t is aim ed at th e u n derlying et iology; h ow ever, several of th e com m on
t reat m en ts m ay h elp differen t cau ses of CME. Medical t reat m en t m odalit ies in -
clude cor t icosteroids (topical, oral, in t ravit real, or in th e su b-Ten on sp ace), n on ste-
roidal an t iin flam m ator y drugs, carbon ic an hydrase in h ibitors, an d YAG laser lysis
of th e vit reou s st ran ds.
Surgical th erapy in clu des pars p lan a vit rectom y to rem ove vit reou s st ran ds
t racking to th e surgical w ou n d or pup il st at u s after com p licated ocular su rger y,
peeling of th e p osterior hyaloid face from th e su rface of th e m acula in vit reom acu-
lar t ract ion syn drom e, peeling of th e ep iret in al m em bran es, rem oval of in flam -
m ator y m ediators from th e vit reou s cavit y, rem oval of retain ed n u clear len s frag-
m en ts, an d reposit ion ing of a dislocated or sublu xed IOL.
Retinal Detachment, Suprachoroidal Hemorrhage/ Effusion after

Cataract Extraction
In t raocu lar su rger y is a m ajor risk factor in th e developm en t of rh egm atogen ou s
ret in al det ach m en t (RRD). Becau se cataract surger y is th e m ost com m on in t raocu -
lar procedu re, it is also th e m ost com m on risk factor for RRD. It h as been est im ated
Fig. 7.28 Aphakic retinal detach-

ment.
th at 20 to 40% of RRDs occur in eyes th at h ave un dergon e cataract ext ract ion .
Aph akia an d p seu doph akia, especially after YAG capsulotom y, predispose to p os-
terior vit reou s det ach m en t (PVD). Previou s st u dies h ave sh ow n th at th e in ciden ce
of PVD in creased w ith age an d w ith durat ion of th e aph akia. In pseudoph akic pa-
t ien t s, perip h eral capsu lar op acificat ion , len t icular rem n an t s, an d opt ical effects
in duced by th e rim of th e IOL m ay im pair visu alizat ion of th e sm all periph eral
ret in al breaks by in direct oph th alm oscopy, leading to m issed breaks during su rgi-
cal repair.
Presentation
Because m ost postoperat ive ret in al detach m en ts are rh egm atogen ous in n at u re,
sim ilar sym ptom s, such as ph otopsias, floaters, visual-field defects, an d cen t ral vi-
su al loss are experien ced by p at ien t s. Th e ret in al breaks are often sm all, difficu lt to
visu alize, an d located along th e posterior border of th e vit reous base. RRD is often
exten sive an d com m on ly involves th e m acu la. Th e m ost im p or tan t p ostop erat ive
factor is YAG capsu lotom y. An terior ch am ber IOL an d iris clip len ses in duce m ore
in flam m at ion , resu lt ing in a h igh er in ciden ce of p roliferat ive vit reoret in opathy
( Fig. 7.28 ).
IOL dislocat ion , lat t ice degen erat ion , an d oth er t ypes of ret in al detach m en t
Management
B-scan u lt rasou n d is th e m ain invest igat ion . As w ith all RRDs, th e goal is to iden t ify
an d close all th e ret in al breaks. Vit rectom y w ith fluid-air exch ange, vit rectom y an d
scleral bu ckling, an d pn eum at ic ret in opexy are som e of th e t reat m en t m odalit ies.
Endophthalmitis after Cataract Extraction

Postoperat ive en doph th alm it is is defin ed as severe in flam m at ion involving both
th e an terior an d p osterior segm en t s of th e eye after in t raocu lar surger y. Typically,
postoperat ive en doph th alm it is is cau sed by th e perioperat ive in t rodu ct ion of m i-
crobial organ ism s in to th e eye eith er from th e pat ien t’s n orm al conjun ct ival an d
skin flora or from con tam in ated in st ru m en t s. Alth ough m ost cases of postopera-
t ive en doph th alm it is occur w ith in 6 w eeks of su rger y, in fect ion s seen in h igh -risk
pat ien ts or in fect ion s caused by slow -grow ing organ ism s m ay occur m on th s or
years after th e procedu re.
Presentation
Pat ien ts w ith acute postoperat ive en doph th alm it is t ypically p resen t w ith in 6
w eeks of in t raocu lar su rger y w ith m oderate to severe eye pain an d decreased vi-
sion . Th e h allm ark fin dings on oph th alm ic exam in at ion are posterior an d an terior
ch am ber in flam m at ion , hypopyon , conju n ct ival hyperem ia an d ch em osis, corn eal
edem a, w oun d abn orm alit ies, an d associated eyelid or orbit al in flam m at ion . In
rare circu m st an ces, pat ien ts m ay develop ch ron ic, in fect ious en doph th alm it is
m on th s to years after in t raocu lar surger y ( Fig. 7.29 ).
Risk factors for developm en t of postoperat ive en doph th alm it is m ay in clu de
in creased operat ive t im e, posterior capsu le ru pt u re/vit reous loss, ret ain ed len s
fragm en t s, in adequate sterilizat ion of th e operat ive field, an d con t am in at ion of
su rgical in st ru m en ts. In th e en doph th alm it is vit rectom y st u dy, th e m ost com m on
organ ism s isolated w ere coagu lase-n egat ive staphylococci (70%), Staphylococcus
aureus (9.9%), an d st reptococci species (9.0%). In fect ion s cau sed by gram -n egat ive
organ ism s w ere seen in 6% of cases. In ch ron ic postop erat ive en doph th alm it is, an
im port an t causat ive organ ism is Propionibacterium acnes, a slow -grow ing, gram -
posit ive bacillu s th at is associated w ith a ch aracterist ic w h ite, in t racapsu lar plaqu e
th at develops w eeks to m on th s after cataract su rger y.
Fig. 7.29 Postoperative endophthalmitis with corneal melt

and intraocular lens extrusion.
En dogen ou s en doph th alm it is, in t raocu lar foreign body, vit reou s h em orrh age,
glaucom a (len s p ar t icle, ph acolyt ic, ph acom orph ic, an d uveit is), an d vit reou s w ick
syn drom e
Management
Obt ain vit reou s, aqu eou s sam p les an d conju n ct ival cu lt u res for m icrobiological
id en t ificat ion of t h e offen d ing organ ism . B-scan is d on e to con fir m t h e d iagn osis.
Th e resu lt s of an en d op h t h alm it is vit rectom y st u dy d em on st rated n o d ifferen ce
in fin al visu al ou tcom es in p at ien t s w h o u n d er w en t in it ial in t raocu lar an t ibiot ic
inject ion (vit reou s t ap ) or im m ed iate p ars p lan a vit rectom y (vit rectom y) if p re-
sen t ing visu al acu it y w as bet ter t h an ligh t p ercept ion . How ever, in p at ien t s p re-
sen t ing w it h ligh t p ercept ion vision , t h ose w h o u n d er w en t in it ial VIT w ere t h ree
t im es m ore likely to ach ieve 20/40 vision , t w ice as likely to m ain t ain 20/100 vi-
sion , an d h ad a n early 50% red u ct ion in t h e r isk of severe visu al loss (<5/200),
com p ared w it h p at ien t s w h o u n d er w en t TAP. No lon g-ter m d ifferen ce occu r red
in m ed ia clar it y bet w een t reat m en t grou p s. In t raven ou s an t ibiot ics h ad n o effect
on eit h er t reat m en t ou tcom e.
Van com ycin h as been sh ow n effect ive again st greater th an 99%of gram -posit ive
en doph th alm it is isolates. Th e am in oglycoside am ikacin (0.4 m g in 0.1 m L) is u se-
ful for gram -n egat ive coverage. Approxim ately 90% of gram -n egat ive isolates are
su scept ible to th is agen t . Ceft azidim e is a reason able altern at ive for gram -n egat ive
coverage. Th e use of in t ravit real dexam eth ason e in th e t reat m en t of acute post-
operat ive en doph th alm it is rem ain s con t roversial. In ch ron ic postoperat ive en do-
ph th alm it is from Propionibacterium acnes, in t raocu lar van com ycin alon e h as been
associated w ith h igh rates of persisten t in flam m at ion .
Th e en doph th alm it is vit rectom y st u dy recom m en ds rapid in ter ven t ion w ith
su rger y for pat ien t s w ith severe vision loss on presen tat ion .
Intraocular Lens Opacification

It h as been m ore th an 50 years sin ce Harold Ridley’s first im plan t , an d th e cata-
ract-IOL procedu re h as reach ed an ext raordin arily h igh level of qu alit y an d perfor-
m an ce. How ever, com p licat ion s su ch as IOL opacificat ion do occu r ( Fig. 7.30A,B).
A B
Fig. 7.30 (A) Opacified intraocular lens. (B) Opacified intraocular lens explanted.
Presentation
Th e opacit ies of th e IOL opt ics m ay st ar t as scat tered w h ite-brow n spot s w ith in
th e su bst an ce of th e IOL opt ic an d rem ain stable or slow ly progressive. Som e m ay
gradu ally in crease in in ten sit y an d n um bers, even t u ally reach ing a poin t w h ere a
visu al acuit y loss m ay n ecessitate rem oval or exch ange of th e IOL. In addit ion to
visu al loss, th e repor ted sym ptom s in clu ded decrease in con t rast sen sit ivit y an d
variou s visu al dist u rban ces an d aberrat ion s, in clu ding glare. In th e early stages
th ere w as usually n o effect on Sn ellen visu al acu it y, bu t a gradual decrease of vi-
su al acu it y w as n oted in th e late st age of th e process.
Cat aract
Management
If n ecessar y, on e sh ould explan t an d rem ove th e opacified IOL an d replace it w ith
a n ew on e.
8 Glaucoma
Primary Open-Angle Glaucoma
Open -angle glaucom a (OAG) is ch aracterized by a gon ioscopically open angle an d

reduced facilit y of ou tflow n ot due to any obvious disease of th e eye. It h as a fa-
m ilial ten den cy.
Presentation
OAG is a bilateral, in sidious, slow ly progressive, often asym m et ric, asym ptom at ic
disease, often n ot recogn ized by th e p at ien t un t il late in th e disease w h en vision
is lost . Pat ien t s h ave an elevated in t raocu lar p ressure (IOP) w ith open angles all
arou n d. Th e opt ic n er ve sh ow s ch aracterist ic ch anges th at in clude a gen eralized
en largem en t of th e cup w ith docum en ted th in n ing of th e n eu rosen sor y rim over
t im e progressing from in ferior to su perior to n asal an d th en fin ally to th e tem poral
rim , focal (especially ver t ical) en largem en t of th e cu p, n otch ing in th e rim , acquired
pit of th e opt ic n er ve, cu p:disk (C:D) rat io asym m et r y greater th an 2, en larged C:D
rat io m ore th an 0.6, n er ve fiber layer h em orrh age crossing th e rim m argin , n er ve
fiber layer defect , perip apillar y at rophy, an d bayon et ing sign . In it ially, th e su perior
an d in ferior poles of th e opt ic n er ve are affected ( Fig. 8.1 ).
Physiological cupping of th e opt ic n er ve, secon dar y OAG, creeping angle-closure
glaucom a, ocu lar hyper ten sion , oth er cau ses of opt ic at rophy, congen ital opt ic
n er ve defect s, an d dru sen of th e opt ic n er ve.
Management
Applan at ion ton om et r y, visu al fields, an terior-segm en t opt ical coh eren ce tom og-
rap hy (OCT), opt ic n er ve-h ead an alysis an d ret in al n er ve fiber layer an alysis by
posterior segm en t OCT, scan n ing laser p olarim et r y or Heidelberg ret in al tom o-
gram , an d stereoscopic evaluat ion of th e disk form part of th e evaluat ion of th e
pat ien t .
Medical m an agem en t of glau com a is based on th e am ou n t of dam age already
presen t , th e rate of dam age progression , an d th e risk factors. Th e on ly proven
m eth od of stopping or slow ing opt ic n er ve dam age is by reducing IOP. An t iglau-
com a m edicat ion s can be used for th is such as topical parasym path om im et ics, B-
adren ergic blockers, epin eph rin e derivat ives, carbon ic an hydrase in h ibitors, alp h a
adren ergic agon ist , an d prost aglan din an alogues. Non m edical m an agem en t con -
sists of argon laser t rabecu loplast y, select ive laser t rabecu loplast y, an d su rger y.
Surgical procedu res in clude t rabeculectom y, seton s, an d n onpen et rat ing proce-
du res su ch as viscocan alostom y an d deep sclerectom y.
244
8 Glaucom a 245
Fig. 8.1 Normal anatomy and fluid dynamics compared with the anatomy and fluid dy-
namics of a glaucoma patient. (A) The normal flow of aqueous humor through the tra-
becular meshwork (T) to the floor (F) of the Schlemm canal (SC). Active transport of the
aqueous humor occurs through the norm al endothelium (E) to the lumen of the canal. It
is then drained through small openings in the external wall or roof of the Schlemm canal
(SC), into scleral collector channels and then into capillaries and veins within the sub-
conjunctival tissues. (B) In a diseased eye with open-angle glaucom a, the endothelium
(E) of the Schlem m canal is more resistant to aqueous outflow, as is the immediately
adjacent trabecular meshwork. This is the site of highest resistance to aqueous flow.
Passage of aqueous humor is very slow, resulting in the increased intraocular pressure
of glaucoma. (Inset ) Anatomically, the Schlemm canal (SC) is located slightly behind the
lim bus. (Courtesy of Highlights of Ophthalmology, “Innovations in the Glaucomas: Etiology, Diagnosis
and Management,” English Edition, 2002. Eds: Benjamin F. Boyd, MD, FACS; Maurice H. Lunt z, MD, FACS;
Co-Editor: Sam uel Boyd, MD.)
Normal-Tension (or Low -Tension) Glaucoma
Th is is th e con dit ion in w h ich opt ic n er ve h ead loss, loss of n er ve fiber layer, an d
th e visual field defect s are th e sam e as seen in ch ron ic glaucom a, bu t th ere is n o
rise in IOP. Norm al-ten sion glau com a (NTG) form s 16% of all th e glau com as.
Presentation
Pat ien ts are m ostly asym ptom at ic. Occasion ally th e pat ien t presen ts w ith scotom a.
Th ere m ay be an associated h istor y of episodes of isch em ic opt ic n europathy, n eu -
rological sym ptom s, m igrain e, or Rayn au d disease.
Fig. 8.2 (A) Posterior segment optical coherence tomography (OCT) showing optic
nerve head analysis in a patient with normal-tension glaucoma and advanced glauco-
matous cupping.
8 Glaucom a 247
IOPs are recorded as n orm al. Th e opt ic disk is t ypically large in NTG. Cu pping is
p ropor t ion ally larger th an visual-field loss. A sloping n euroret in al rim edge w ith
sh allow cu p, h igh er in ciden ce of opt ic disk pit associated w ith it , peripap illar y
at rophy, opt ic disk h em orrh ages, steeper an d deep er visual-field defect s close to
fixat ion , a posit ive fam ily h istor y, m yopia, n oct u rn al hypoten sion , an d a h istor y of
carot id ar ter y disease are som e of th e associated feat ures ( Figs. 8.2A,B).
In cludes cases w ith in term it ten t pressu re elevat ion or diu rn al variat ion s in pri-
m ar y open -angle glaucom a (POAG), pat ien t s w ith long-term flu ct u at ion s in pres-
su re elevat ion s th at h ave been m asked by system ic m edicat ion s such as β-block-
ers; oth er n onglaucom atous opt ic n europathy; congen ital abn orm alit ies of th e
opt ic n er ve h ead
Fig. 8.2 (Continued) (B) Posterior segment OCT showing retinal nerve fiber layer anal-
ysis in a patient with bilateral advanced normal-tension glaucoma.
Management
A com plete ocular an d system ic h istor y is im por tan t . Histor y of drug in t ake, vas-
cular problem s, n eu rological problem s, an d cardiological problem s m u st be re-
corded. Carefu l ocular exam in at ion w ith stereoscopic opt ic n er ve h ead evaluat ion ,
periph eral fu n dus exam in at ion , an d gon ioscopic an d field exam in at ion m ust be
don e.
A 20 to 25% redu ct ion in th e IOP is aim ed in early an d m oderate field ch anges,
an d it sh ou ld be less th an 25% in pat ien ts w ith severe field ch anges. Miot ics, α -2
agon ist , β-blockers, dorzolam ide an d lat an oprost are good opt ion s to redu ce IOP.
Calcium ch an n el blockers an d n europrotect ion play a prom in en t role in th e m an -
agem en t of NTG. Filt rat ion surger y is an opt ion in advan ced field defects.
Secondary Open-Angle Glaucoma
Secon dar y open -angle glau com a is OAG th at occu rs secon dar y to som e oth er
cau se.
Pigmentary Dispersion Glaucoma

Pigm en t dispersion syn drom e (PDS) is gen erally an asym ptom at ic disorder dis-
covered du ring rout in e oph th alm ic evaluat ion . Pigm en t ar y glaucom a is a sequ ela
of PDS. Developm en tal abn orm alit ies of th e iris pigm en t epith eliu m are th e fun -
dam en t al defect respon sible for th e pigm en t dispersion . Th e con dit ion is seen in a
relat ively younger age grou p an d is m ore com m on in m en th an in w om en . Myopia
an d a deep an terior ch am ber are th e risk factors for th e developm en t of PDS. It is
com m on in person s of Europ ean descen t .
Presentation
Th e con dit ion is diagn osed in ciden t ally in m ost of th e cases. Few pat ien t s com plain
of colored h alos an d sm oky vision in th e dim ligh t con dit ion s an d after vigorous
physical exercise. Pat ien t s h ave a raised IOP, defect ive fields, an d opt ic disk cu p-
ping. Oth er associated fin dings in clu de Kruken berg spin dle, raised cen t ral corn eal
th ickn ess, iris t ran sillum in at ion defect , con cave periph eral iris, an isocoria, ring of
pigm en t at ion over th e periph eral su rface of th e len s, an d a h igh ly pigm en ted t ra-
becu lar m esh w ork ( Fig. 8.3 ).
Pseu doexfoliat ive glau com a
Management
Pat ien ts w ith PDS are alw ays con sidered as glaucom a su spect s an d IOP sp ikes, vi-
su al fields, cu pping, an d gon ioscopy are ch ecked an n u ally. Myopic pat ien t s w ith
PDS h ave to be ch ecked for periph eral ret in al lesion s, ret in al breaks, an d ret in al
detach m en t . Miot ics, topical β-blockers, an d carbon ic an hydrase in h ibitors form
th e m ain st ay of th e t reat m en t of pigm en t ar y glaucom a. Pat ien t s u su ally respon d
w ell to argon laser t rabecu loplast y (ALT). Filtering su rger y w ith an t im etabolite is
don e in advan ced an d refractor y cases.
8 Glaucom a 249
Fig. 8.3 Proper placement of laser application in laser trabeculoplast y. This magnified
cross section of the angle area shows a properly placed laser beam (L) being applied to
the center of the posterior trabecular m eshwork (P) or pigmented band. Notice the laser
burns (B) centered on this pigmented band (P). If one were to divide the space bet ween
the scleral spur (S) and the Schwalbe line (A) in half (X), the laser burns (B) fall on the
center of the posterior half [area bet ween (X) and (S)]. The anterior half of the m esh-
work [area bet ween (X) and (A)] is left untreated. Posterior to the scleral spur (S) is the
uveal meshwork (U). Schlemm canal (C). (Courtesy of Highlights of Ophthalmology, “Innovations
in the Glaucomas: Etiology, Diagnosis and Management,” English Edition, 2002. Eds: Benjamin F. Boyd,
MD, FACS; Maurice H. Lunt z, MD, FACS; Co-Editor: Samuel Boyd, MD.)
Pseudoexfoliation Syndrome
Pseu doexfoliat ion syn drom e (PEX) is an age-related gen eralized disorder of ex-
t racellu lar m at rix ch aracterized by produ ct ion an d progressive accu m ulat ion of
fibrillar m aterial in th e t issues th rough out th e an terior segm en t . PEX is th e single
m ost com m on iden t ifiable cause of OAG, an d it is also a risk factor for cataract
su rger y. In ciden ce in creases w ith age an d is m ore com m on in fem ales.
Presentation
Deposit ion of th e grayish w h ite m aterial on th e surface of th e an terior len s capsu le
is a com m on , con sisten t , an d diagn ost ic fin ding. Th e deposit ion h ad a classic pat-
tern of th ree zon es (i.e., a relat ively h om ogen eou s cen t ral disk, a gran u lar layered
periph eral zon e, an d a clear area separat ing th e preceding t w o).
Th e PEX m aterial is fou n d deposited on th e pupillar y border, corn eal en doth e-
lium , iris fu rrow s, an d som et im es even at th e ext raocular m uscles. Oth er associ-
Fig. 8.4 Pseudoexfoliative material seen in a sublux-

ated lens. (Courtesy of Lincoln Freitas)
ated feat u res can be peripapillar y t ran sillum in at ion defect , in sufficien t m ydriasis,
posterior syn ech iae, Sam p aolesi lin e, less den se bu t patchy pigm en t at ion of th e
t rabecu lar m esh w ork, ph acodon esis, an d iridodon esis ( Fig. 8.4 ).
Tru e exfoliat ion , pigm en t ar y glau com a, oth er cau ses for m elan in dispersion , pri-
m ar y am yloidosis, sen ile iridosch isis, POAG.
Management
PEX pat ien ts require frequ en t m on itoring of IOP, opt ic disk, an d visu al fields. Th e
m edical lin e of m an agem en t form s th e in it ial lin e of t reat m en t . ALT is a h igh ly
su ccessive t reat m en t for th ese pat ien t s. Filtering su rger y is recom m en ded for th e
advan ced cases. Caut ion sh ou ld be t aken w h ile operat ing on pat ien ts w ith PEX for
cataract su rger y, as PEX is associated w ith an in creased in ciden ce of len s su blu x-
at ion an d vit reou s loss.
Lens-Induced Glaucoma
Lens-induced glaucom as are either OAGs or angle-closure glaucom a. OAGs include lens
protein glaucom a, lens particle glaucom a, and phacoanaphylactic glaucom a. Closed-
angle glaucom as include intum escent lens and lens sublu xation or dislocation.
Lens Pa rticle Gla ucoma
Th e pat ien t h as u n ilateral pain , defect ive vision , lacrim at ion , an d p h otoph obia. In -
creased IOP, cells an d flare, w h ite fluffy pieces of len s cor tex in th e an terior ch am -
ber, an d open angles are seen . A disru pt ion in th e len s capsule by t raum a or su rger y
liberates len s m aterial th at obst ruct s th e t rabecular m esh w ork. In flam m at ion also
con t ributes to th e glaucom a. Man agem en t con sists of reduct ion of in flam m at ion
an d IOP follow ed by rem oval of th e residu al len s m at ter if n ecessar y.
8 Glaucom a 251
Pha colytic Gla ucoma

Th e pat ien t h as u n ilateral pain , defect ive vision , lacrim at ion , an d ph otoph obia.
Open angles an d a hyperm at u re or m at u re cataract are seen along w ith in creased
IOP, iridescen t par t icles, an d w h ite m aterial in th e an terior ch am ber an d on th e
len s cap su le. Len s protein leaks from an in t act cataract an d obst ru ct s th e t rabecu-
lar m esh w ork. In flam m at ion also con t ribu tes to th e glaucom a. Aging an d cataract
form at ion are th e risk factors. Man agem en t con sists of redu ct ion of in flam m at ion
an d IOP follow ed by cat aract ext ract ion .
Pha coa naphyla ctic Gla ucoma

Gran u lom atous in flam m at ion occu rs secon dar y to a hyp ersen sit ivit y react ion to
len s par t icle released after pen et rat ing t raum a or su rger y.
Pha comorphic Gla ucoma

It is caused by an in t u m escen t len s leading to angle closure glau com a, secon dar y
to eith er an en h an ced pup illar y block m ech an ism or du e to for w ard disp lacem en t
of th e len s–iris diaph ragm .
Ectopia Lentis
Can also cau se an angle closu re glaucom a. Pu pillar y block m ay occu r due to th e
len s being dislocated in to th e pu pil or an terior ch am ber or th e vit reou s h ern iat ing
in to th e an terior ch am ber.
Uveitic Glaucoma
Inflam m ator y cells m echanically obstruct the trabecular m eshw ork (TBM) and are
directly cytotoxic to the TBM. They cause a com bination of closed-angle glaucom a
secondary to peripheral anterior synechiae (PAS), posterior synechiae, or total syn-
echiae, form ation and OAG secondary to increased protein content of the aqueous,
aqueous hypersecretion, obstruction by inflam m atory cells and debris, trabeculitis,
scarring of the TBM, increased episcleral venous pressure, or as a steroid response.
Presentation
It is often u n ilateral, presen t ing w ith pain , ph otoph obia, redn ess, an d decreased
vision . On exam in at ion , aqueous cells an d flare, open angles, irregular pu pil, pos-
terior syn ech iae, PAS, in flam m ator y precip itates on th e p osterior corn eal su rface
or t rabecular m esh w ork, ciliar y flu sh , an d oth er sign s of uveit is along w ith sign s of
glaucom atou s dam age to th e eye m ay be seen .
Acute angle-closure glau com a, n eovascular glaucom a, Posn er-Sch lossm an syn -
drom e, Fu ch s h eteroch rom ic iridocyclit is, steroid respon se glau com a
Fig. 8.5 Uveitic evidence seen in an eye with in-

creased intraocular pressure.
Management
Topical steroids an d cycloplegics are given for th e in flam m at ion , an d th e glaucom a
is m an aged m edically (m iot ics an d p rostaglan din an alogues are n ot to be u sed be-
cau se th ey m ay cau se a w orsen ing of th e in flam m at ion ) an d if requ ired su rgically
w ith an t im et abolites or seton ( Figs. 8.5 an d 8.6 ).
Elevated Episcleral Venous Pressure

Ven ous drain age obst ruct ion secon dar y to ret robu lbar t u m ors, thyroid eye disease,
pseu dot u m or, cavern ou s sin u s th rom bosis, jugu lar vein obst ru ct ion , superior ven a
cava obst ru ct ion , an d in t racran ial (St u rge-Weber syn drom e, carot icocavern ous
fist u la, dural fist ula, ven ous varix) or orbital ar terioven ou s fist ulas can all lead
to in creased episcleral ven ous pressu re. It can also in crease w ith ou t any obvious
cau se.
Presentation
Th e pat ien t presen ts w ith a red eye. Th e ep iscleral vein s are gen erally dilated an d
tort u ou s w ith a corkscrew appearan ce. Th e angles are open an d blood m ay be seen
in th e Sch lem m can al.
Conju n ct ivit is, episclerit is, sclerit is, orbital in flam m at ion
8 Glaucom a 253
Fig. 8.6 Seton implantation procedure. A fornix-based conjunctival flap (C) is raised
and the m ethylm ethacrylate baseplate (P) of the Seton is pushed under the conjunctival
flap posteriorly and sutured to the scleral surface. The implant has a biconcave shape
with the inferior surface shaped to fit the sclera. A small 3-mm-square half-thickness
lamellar scleral flap (D) is raised just as in a trabeculectomy. An incision (F) is made into
the anterior chamber under this scleral flap, and the long silicone tube (S) of the Seton
is placed into the anterior chamber (the end of the silicone tube can be seen in the ante-
rior chamber near the tip of the white arrow). Next, the scleral flap (D) is sutured down
around the tube (S) of the Seton. Finally, the conjunctiva is sutured back in place. Aque-
ous then drains from the anterior chamber (white arrow) down through the tube (S) to
the baseplate (P) (black arrow), where a bleb forms. (Courtesy of Highlights of Ophthalmology,
“Innovations in the Glaucomas: Etiology, Diagnosis and Management,” English Edition, 2002. Eds: Benja-
min F. Boyd, MD, FACS; Maurice H. Lunt z, MD, FACS; Co-Editor: Samuel Boyd, MD.)
Management
Approach to m an agem en t depen ds on th e cause. Filtering su rger y carries th e risk
of in t raoperat ive ch oroidal effusion , exp ulsive h em orrh age, an d postoperat ive flat
an terior ch am ber. Prophylact ic sclerotom ies in th e in ferior quadran t s sh ould be
m ade prior to st art ing th e filtering su rger y. Th ese are left op en an d covered on ly
w ith conju n ct iva at th e en d of surger y.
Angle -Closure Glaucoma
Angle-closure glaucom a can be classified as angle-closu re glau com a w ith pupil-

lar y block an d angle-closu re glaucom a w ith ou t pu pillar y block, each of w h ich can
be fu r th er divided in to prim ar y an d secon dar y form s.
Acute Angle -Closure Glaucoma

Any in crease in th e n orm al con dit ion of relat ive pupillar y block because of th e
cen t ral iris h ugging th e an terior len s surface causes th e periph eral iris to com e in
con tact w ith th e t rabecu lar m esh w ork blocking th e aqu eou s outflow, causing an
acute rise in pressu re.
Presentation
Th e pat ien t p resen t s w ith decreased vision , severe pain , redn ess, blu rred vision ,
colored h aloes aroun d ligh ts, n ausea, an d vom it ing. Circum corn eal congest ion ,
corn eal m icrocyst ic edem a, sh allow an terior ch am ber, m ild an terior ch am ber re-
act ion , m id-dilated, vert ically oval pu pil, an d th e like are seen . In case of recurren t
at t acks, posterior syn ech iae, periph eral an terior syn ech iae, glaukom flecken (sm all
an terior su bcapsu lar len s op acit ies), sector or gen eralized iris at rophy, opt ic n er ve
pallor an d cu pping, perm an en tly in creased IOP, an d visu al-field loss m ay be seen
( Figs. 8.7 an d 8.8 ).
Fig. 8.7 Anterior segm ent optical coherence tomography in a patient showing narrow
angles
8 Glaucom a 255
Fig. 8.8 Abraham’s argon laser iridectomy t wo-step technique—surgeon’s view of the
second burn. The second burn is a penetrating burn aimed at the crest or peak of one of
the iris humps (B), which resulted from the first burn. This second burn has now created
a hole or iridectomy (D) through the peak of the iris hump (B). The first burn, which was
partially penetrating, is shown in (A). Note the iris pigm ent drifting down while a gas
bubble floats superiorly (arrow). Use the plano-convex but ton of the lens only for second
coagulation. (Courtesy of Highlights of Ophthalmology, “Innovations in the Glaucomas: Etiology, Diag-
nosis and Management,” English Edition, 2002. Eds: Benjamin F. Boyd, MD, FACS; Maurice H. Lunt z, MD,
FACS; Co-Editor: Samuel Boyd, MD.)
Management
Medical m an agem en t con sists of pilocarpin e, topical steroids, an t iglau com a m edi-
cat ion s, pain killers, an d an t iem et ics. Th e defin it ive t reat m en t is laser iridotom y
perform ed w ith eith er w ith an argon or YAG laser. In th e case of exten sive syn -
ech iae w ith perm an en tly elevated IOP, a t rabecu lectom y m ay be required.
Chronic Angle -Closure Glaucoma

Angle syn ech iae u sually begin superiorly an d progress in both direct ion s tow ard
th e 6 o’clock p osit ion .
Presentation
Presen t at ion is sim ilar to prim ar y open -angle glau com a (POAG). Th e pat ien t h as
in creased IOP th at is asym ptom at ic un t il dam age h as occurred. Diagn osis is by go-
n ioscopy ( Fig. 8.9 ).
Prim ar y or secon dar y OAG
Management
Medical m an agem en t an d laser iridotom y are used in early cases an d t rabecu lec-
tom y in advan ced cases.
Neovascular Glaucoma
Secon dar y glau com a is caused by developm en t of n ew vessels in th e angle. De-
layed diagn osis an d poor m an agem en t can cause loss of vision . Ret in al isch em ia
or hypoxia an d subsequ en t release of angiogen ic factors cau ses n eovascular glau-
com a. Elevat ion of IOP is due to fibrovascu lar m em bran es an d an terior syn ech iae
blocking th e angle.
Presentation
Th e eye is pain fu l, ph otoph obic, an d red. Visu al acuit y m ay be as low as coun t ing
fingers or n o p ercept ion of ligh t . IOP m ay be as h igh as 60 m m Hg. Conju n ct ival
congest ion an d steam y corn ea are associated fin dings (Fig. 8.10 ).
A tiny early t uft of n ew vessels is visible first at the m argin of the pupillar y border.
Th e vessels later en large to form knuckles. Th e pat ien t’s eye is exam ined under high
Fig. 8.9 Cupping of the disk.

8 Glaucom a 257
Fig. 8.10 Trabeculectomy with a fornix-based flap—removing the trabecular window—

surgeon’s view. This is a surgeon’s view of the final incision to remove the trabecular
window. It also reveals the surgeon’s view of the structures most important to proper
trabeculectomy. The trabeculectomy flap, which is being excised, has been hinged
backward exposing its deep surface to the surgeon’s view. The Vannas scissors (SC),
make the final cut just in front of the scleral spur (S), on the trabecular tissue, which is
here being reflected back with forceps (FP). The scleral spur is localized externally (E) by
the junction of the white sclera and gray band (B). A, clear cornea; F, scleral flap; IR, iris
root; T, trabeculum. (Courtesy of Highlights of Ophthalmology, “Innovations in the Glaucomas: Etiol-
ogy, Diagnosis and Management,” English Edition, 2002. Eds: Benjam in F. Boyd, MD, FACS; Maurice H.
Lunt z, MD, FACS; Co-Editor: Samuel Boyd, MD.)
m agn ification . A sligh t pressure w ith th e gon ioscope or dilat ion of the pupil m ay
obscure th e fin ding. Th e n ew vessels then appear on the surface of the iris to reach
th e iris collaret te. Later n ew vessels extend from th e root of the iris to the ciliar y
body and the scleral spur to arborize the t rabecular m eshw ork. Fibrovascular m em -
bran es associated w ith neovascularization start cont racting to ten t the iris tow ard
th e angle. Anterior synech iae an d iridocorneal adh esions are com m on fin dings.
Uveit ic glau com a, acute angle-closu re glau com a
Management
A th orough h istor y an d op h th alm ic an d ret in al exam in at ion s are m an dator y. Con -
t rol of IOP an d in flam m at ion is th e first lin e of m an agem en t . Hyperosm ot ic agen t s
an d m ydriat ic agen t s play an im por t an t role. Pan ret in al ph otocoagulat ion or pe-
riph eral cr yopexy m u st be perform ed at th e earliest oppor t un it y. Trabeculectom y
w ith a sh u n t procedu re an d applicat ion of an t im et abolites is preferred. If th e IOP is
ver y h igh an d th e pat ien t experien ces pain w ith n o usefu l vision , cyclodest ruct ive
procedu res can be con sidered.
Iridocorneal Endothelial Syndrome
Th is is a un ilateral con dit ion , seen m ain ly in w om en aged 30 to 50 years.
Presentation
Th e p at ien t u sually p resen t s w ith dim in ish ed vision an d pain . Th ere is m ovem en t
of th e corn eal en doth elium on to th e iris. Loss of cells from th e corn ea w ou ld lead
to corn eal sw elling, distor t ion of th e iris, m elt h oles, st retch h oles, an d a variable
degree of distor t ion of th e pupil. Th e en doth eliu m becom es several layers th ick
an d spreads over th e t rabecu lar m esh w ork, cau sing glau com a.
Th e slit-lam p exam in at ion reveals a “fin e h am m ered silver” ap pearan ce of th e
corn eal en doth eliu m . Sp ecular m icroscopy dem on st rates pleom orp h ism in size
an d sh ape w ith in cert ain cells an d a loss of clear h exagon al m argin s. Based on th e
involvem en t of th e corn ea, th e disorder is called tot al, dissem in ated, or su btotal.
Based on th e st ru ct u re involvem en t th e disorder is classified as follow s:
Ch an dler syn drom e, w h ich m ain ly involves th e corn ea
Cogan -Reese syn drom e, w h ich m ain ly involves th e angle, w ith a pigm en ted an d
pedu n cu lated n odu le p resen t over th e iris
Progressive iris at rop hy
Ch an dler syn drom e, Cogan -Reese syn drom e, an terior ch am ber cleavage syn drom e,
previou s ocu lar t rau m a, posterior polym orph ous dyst rophy
Management
Corn eal edem a is m an aged by low ering th e IOP, u se of hyper ton ic salin e an d soft
ban dage con t act len ses, an d, fin ally, pen et rat ing keratoplast y. Glaucom a is best
approach ed w ith m edical m an agem en t (aqueous suppressan ts), an d, fin ally, w ith
t rabecu lectom y in th e late st ages. Im m u n otoxin s h ave been foun d to be effect ive.
Malignant Glaucoma
Malign an t glau com a m ay be seen in th e postsurgical period on discon t in uat ion of

cycloplegics or on addit ion of m iot ics. Th ere is posterior m isdirect ion of th e aque-
ous, in to th e vit reou s w h ere it accu m ulates an d displaces th e vit reou s for w ard,
push ing th e ciliar y processes, cr yst allin e len s, in t raocu lar im plan t , or an terior vit-
reou s face for w ard, cau sing secon dar y angle closu re.
Presentation
Th e pat ien t presen ts w ith pain , redn ess, ph otoph obia, an d defect ive vision along
w ith h igh IOP; a flat or ver y sh allow an terior ch am ber in th e presen ce of a paten t;
periph eral iridectom y an d in th e absen ce of a ch oroidal detach m en t .
8 Glaucom a 259
Pu pillar y block glau com a, w oun d leak, ch oroidal detach m en t , an d su prach oroidal
h em orrh age
Management
If th ere is n o iridectom y or if it is n ot paten t , a PI is perform ed to r ule ou t pu pil-
lar y block glau com a. Medical m an agem en t con sist s of topical at ropin e an d ph en -
yleph rin e as w ell as an t iglau com a m edicat ion s to con t rol th e IOP. All m iot ics are
stopped. Hyperosm ot ics are given to sh rin k th e vit reous. Surgical m an agem en t is
often requ ired. In aph akic/pseudoph akic eyes, th e YAG laser m ay be u sed to p er-
form an an terior hyaloidotom y an d posterior capsulotom y. If th e pat ien t is ph akic,
a vit rectom y m ay be required to rem ove th e t rapped aqu eous. A PI sh ould be per-
form ed in th e con t ralateral eye. All m iot ics sh ould be avoided in th e pat ien t .
9 Medical Retina
Mandeep Lam ba , Soosan Jacob , and Am ar Agarw al
Hypertensive Retinopathy
A gen eralized respon se to system ic hyp erten sion is vasocon st rict ion . Th is causes
ret in al vasculopathy in both th e acute an d th e ch ron ic st ages of system ic hyper-
ten sion .
Presentation
Most pat ien t s are asym ptom at ic. How ever, sym ptom s can range from blurring
of vision to profoun d loss of vision . Oph th alm oscopic exam in at ion reveals sign s
of ar teriosclerosis, ch anged ligh t reflex, cop per w iring, sh eath ing of th e vessels,
pipestem sh eath ing, vascular at ten uat ion , ar terioven ou s n icking, Gu n n sign , Salus
sign , ext ravascular ret in al lesion s (m icroan eu r ysm s, ret in al h em orrh ages, m acu -
lar edem a, ret in al lipid deposit s, an d m acu lar st ar), in n er ret in al isch em ic spot s,
focal in t raret in al periarteriolar t ran sudate, an d associated disk edem a in m alig-
n an t hyperten sion . At ar terioven ou s crossings, ret in al ar teries an d vein s sh are a
com m on adven t it ial lin ing. Obscu rat ion of th e ret in al vein s at th ese crossings (AV
n icking) is con sidered a h allm ark of hyper ten sive ret in opathy. Ret in al pigm en t
epith eliu m (RPE) in farct ion s becom e hyperpigm en ted w ith t im e, form ing Elsch n ig
Fig. 9.1 Hypertensive retinopathy. Hypertensive retinopathy grade 4.
260
9 Medical Retina 261
spot s (periph eral hyperpigm en ted spots th at m ay be su rrou n ded by a sm all h alo of
hypop igm en tat ion ). Siegrist st reaks are lin ear hyperpigm en ted areas directly over
ch oroidal vessels th at m ay h ave a sim ilar path ophysiology as Elsch n ig spots. In
ext rem e cases pat ien ts can presen t as hyper ten sive ch oroidopathy, serous ret in al
detach m en t , an d hyp erten sive opt ic n eu ropathy (h em orrh ages at th e opt ic disk
m argin , blurring of disk m argin s, congest ion of ret in al vein s, m acu lar exu dates,
an d florid disk edem a) ( Fig. 9.1 ).
Diabet ic ret in opathy, collagen vascu lar diseases, an em ia, radiat ion ret in opathy,
bran ch ret in al vein occlusion (BRVO), cen t ral ret in al vein occlusion (CRVO), isch -
em ic opt ic n eu ropathy, oth er cau ses of n eu roret in it is, papilledem a, an d ch err y-red
spot
Management
Th e pat ien t is referred for a cardiological w orkup. Ph arm acoth erapy an d lifest yle
ch anges are required to preven t fur th er en d organ dam age.
Diabetic Retinopathy
Ret in al vasculopathy affect s 25% of th e total diabet ic populat ion . Diabetes can af-
fect eyes in various w ays, m ost com m on ly corn eal abn orm alit ies, glaucom a, iris
n eovascularizat ion , cat aract s, an d n eu ropath ies. How ever, diabet ic ret in op athy is
th e m ost com m on an d poten t ially th e m ost blin ding of th ese com plicat ion s. Na-
t ive Am erican s an d African Am erican s are at in creased risk of developing diabet ic
ret in opathy ( Fig. 9.2A,B,C,D).
Presentation
Nonproliferat ive diabet ic ret inopathy (NPDR) : Most com m on ly th e con dit ion is
detected in ciden t ally. How ever, sym ptom s can range from m ild blu rring vision
to distor t ion to visu al acuit y loss. Com m on clin ical feat ures in clu de th e pres-
en ce of m icroan eu r ysm s, dot/blot h em orrh ages, flam e-sh aped h em orrh ages,
ret in al edem a, h ard exu dates, cot ton w ool spots, ven ous loops/beadings, in t ra-
ret in al m icroangiopath ies. On th e basis of clin ical pict ure, Early Treat m en t Dia-
bet ic Ret in opathy St u dy (ETDRS) h as classified NPDR in to m ild, m oderate, an d
severe t ypes.
Proliferat ive Diabet ic Ret inopathy ( PDR) : Th is is classified as early an d h igh -risk
PDR. Un t reated PDR h as a 70% ch an ce of leading to tot al blin dn ess. Sym ptom s
can var y from m ild blurring of vision to severe vision loss. Com m on clin ical fea-
t u res in clu de n eovascularizat ion of disk (NVD), n eovascularizat ion elsew h ere
(NVE), p reret in al an d vit reous h em orrh age, fibrovascu lar t issu e proliferat ion ,
an d t ract ion or com bin ed ret in al det ach m en t s.
Maculopathy : Th is is th e leading cau se of vision loss in pat ien ts w ith diabet ic
ret in opathy. It is du e to fu n ct ion al dam age an d n ecrosis of ret in al capillaries.
Edem a can also be cau sed by t ract ion in th e case of PDR. Th e defin it ion of clin i-
cally sign ifican t m acu lar edem a (CSME) is given by ETDRS (Fig. 9.2E,F,G,H).
A B
C D
E F
Fig. 9.2 (A) Severe nonproliferative diabetic retinopathy (NPDR) with diffuse diabetic
m aculopathy. (B) Moderate NPDR. (C) Proliferative diabetic retinopathy (PDR) with clini-
cally significant macular edema. (D) PDR–FFA. (E) PDR with preretinal hemorrhage–
color fundus photo. (F) PDR with preretinal hemorrhage–FFA.
G H
Fig. 9.2 (Continued) (G) PDR with

preretinal hemorrhage. (H) Severe
PDR with tractional retinal detach-
m ent. (I) Grid laser for diffuse retinal
I thickening.
NPDR: CRVO, BRVO, ocu lar isch em ic syn drom e, hyper ten sive ret in opathy, radia-
t ion ret in opathy, sickle cell disease
PDR: Neovascu larizat ion due to vein occlusion , sickle cell ret in opathy, drug
abu se, sarcoidosis, Valsalva ret in opathy
Maculopathy : Cystoid m acular edem a (CME), cen t ral serous ret in op athy (CSR),
n euroret in it is, parafoveal telangiect asia, m acroan eu r ysm
Management
Fast ing glucose level an d th e h em oglobin A1c (HbA1c) levels are im por tan t in -
dicators to m on itor con t rol of diabetes m ellit us. HbA1c m ust be m ain tain ed be-
t w een 6 an d 7%. Fun du s flu orescein angiography is don e to diagn ose st age an d
for follow -u p of cases of diabet ic ret in opathy. B-scan is u sed in diagn osis an d
follow -up of cases w ith vit reou s h em orrh age.
Laser photocoagulation form s the m ainstay of treatm ent. Focal and grid lasers are
done to treat m acular edem a depending on the num ber and localization of the
leaks. Panretinal photocoagulation is done in PDR. W hen both the lasers need to
be com bined, grid laser is done 2 to 3 w eeks prior to the PRP (Fig. 9.2I).
In t ravit real t riam cin olon e aceton ide an d bevacizum ab h ave been t ried for t reat-
ing m acu lar edem a. In t ravit real inject ion s of bovin e hyaluron idase are used in
t reat m en t of vit reous h em orrh ages.
Vit rectom y is n ecessar y in cases w ith longst an ding vit reous h em orrh age, t rac-
t ion , or com bin ed ret in al detach m en t .
Follow -u p depen ds on th e st age of th e diabet ic ret in opathy (diabet ic ret in o-

pathy). Mild NPDR is follow ed u p on ce in 1 year, m oderate NPDR in 6 to 8
m on th s, severe NPDR in 3 to 4 m on th s, CSME in 2 to 3 m on th s, early PDR in 2 to
3 m on th s, an d h igh -risk PDR ever y 1 to 2 m on th s.
Central Retinal Vein Occlusion
Th e et iology is th rom bus form at ion posterior to th e lam in a cribrosa of th e opt ic

n er ve in th e predisposed popu lat ion . Here vessels lie in a com p ar t m en t w ith lim -
ited sp ace for displacem en t . Ret in al vein occlu sion can be a cen t ral or a bran ch vein
occlusion . Th e path ogen esis of th rom bosis can be an alyzed by th e classic Virch ow
t riad: stasis of blood flow, hypercoagu lat ion , an d vessel w all abn orm alit ies. (Fig.
9.3A,B,C,D).
Presentation
A det ailed h istor y is t aken to rule out possible causes. Th e presen t ing pat ien t can
be asym ptom at ic or w ith decreased vision , ph otoph obia, pain fu l blin d eye, an d
redness. Visual loss can be sudden or gradual, over a period of days to w eeks. Visual
loss ranges from m ild to severe. Pat ien t s can presen t w ith t ran sien t obscurat ion s
of vision in it ially, later progressing to con st an t visual loss. Best-corrected vision is
a ver y good progn ost ic in dicator. Conju n ct ival congest ion , corn eal edem a, relat ive
afferen t pupillar y defect (RAPD), iris an d angle n eovascularizat ion , an d periph -
eral an terior syn ech iae are im port an t associated fin dings. Fu n du s exam in at ion
reveals su perficial, dot an d blot , an d/or deep ret in al h em orrh ages possible in all
fou r qu adran t s. W h ole fu n du s gives a “blood an d th u n der” appearan ce. Vein s m ay
be dilated an d tort u ous. Oth er associated fin dings can be opt ic disk edem a, cot ton -
w ool spot s, NVD, NVE, optociliar y sh u n t vessels, m acular edem a w ith or w ith out
exu dates, cystoid m acular edem a, lam ellar or fu ll-th ickn ess m acu lar h ole, opt ic
at rophy, an d p igm en tar y ch anges in th e m acu la ( Fig. 9.3E,F).
Diabet ic ret in opathy, acute hyper ten sive ret in opathy, ocu lar isch em ic syn drom e,
pap illedem a, p apillit is, radiat ion ret in opathy, acu te ret in it is
Management
Medical exam in at ion an d laborator y test s are don e to fin d th e system ic causes
leading to th e episode. Ocu lar hyperten sion an d glaucom a m ust be ru led out . Dop-
pler color im aging, opt ical coh eren ce tom ography (OCT), FFA, an d elect roret in o-
gram (ERG) con firm th e stat us of th e disease an d th e likely progn osis. Aspirin , an -
t iin flam m ator y agen ts, system ic an t icoagu lan t s, fibrin olyt ic agen t s, an d system ic
cor t icosteroids are used as th e m edical lin e of t reat m en t .
In t ravit real inject ion of t riam cin olon e is th e th erapy of ch oice in pat ien t s w ith
severe m acu lar involvem en t . Ch orioret in al ven ous an astom osis, radial opt ic n eu-
rotom y, an d vit rectom y to rem ove th e posterior hyaloid ph ase are th e surgical ap-
proach es u sed so far. Cen t ral vein obst ruct ion st udy (CVOS) advocated a frequ en t
follow -up in th e early m on th s of th e episode to look for th e iris n eovascularizat ion .
Neovascu larizat ion , m acular pu cker, m acular edem a, celloph an e m aculopathy, an d
opt ic at rophy are th e com plicat ion s to be n oted in ever y visit .
A B
C D
E F
Fig. 9.3 (A) Central retinal vein occlusion (CRVO)—color fundus photo. (B) CRVO—fun-
dus fluorescein angiography (FFA) late phase. (C) Branch retinal vein occlusion (BRVO)
involving the m acula—color fundus photo. (D) BRVO involving macula—FFA. (E) Macular
retinal vein occlusion—color fundus photo. (F) Macular retinal vein occlusion—FFA.
Branch Retinal Artery Occlusion
Of all th e acu te ret in al arterial occlu sion s, 40%are bran ch ret in al arter y occlu sion s
(BRAOs). Path ophysiologically, BRAO resem bles cen t ral ret in al ar ter y occlusion
(CRAO), but it involves on ly a sector of th e ret in a. Isch em ia of th e in n er layers
of th e ret in a leads to cellular injur y an d n ecrosis. It is m ost com m on in th e sev-
en th decade of life. Ch olesterol em boli, called Hollen h orst p laqu es, are th e em boli
m ost com m on ly associated w ith BRAO. Platelet fibrin em boli appear as w h it ish
gray p lugs an d in creases in n u m ber w ith repeated episodes. Calcific em boli ap-
pear as large w h ite plaques an d u su ally involve th e large ar terioles arou n d th e
disk. Oth er u n com m on sources of em boli are also fou n d, such as leukoem boli
(vascu lit is, Pur t sch er ret in op athy, sept ic en docardit is), fat em boli follow ing long-
bon e fract u res, am n iot ic flu id em boli (com plicat ion of pregn an cy), t um ors (at rial
m yxom a, m it ral valve papillar y fibroelastom a), talc em boli (long-term in t raven ous
drug abusers), cort icosteroid em boli (com plicat ion of in t ralesion al or ret robulbar
steroid inject ion ), air em boli follow ing t rau m a or surger y, syn th et ic m aterials u sed
in cardiac an d vascular p rocedu res (e.g., syn th et ic par t icles from art ificial cardiac
valves).
Cer t ain n on em bolic cau ses are also respon sible for th e arterial occlu sion s, for
exam ple, th rom bosis associated w ith ath erosclerosis, vascu lit is (gian t cell ar terit is,
system ic lu pus er yth em atosu s), posterior in flam m ator y con dit ion s (toxoplasm a
ret in och oroidit is, Beh çet syn drom e).
Presentation
Th e pat ien t usually presen ts w ith acu te, u n ilateral, pain less, par t ial loss of vision .
Many t im es it can be an in ciden t al fin ding. A h istor y of associated risk factors like
sm oking, hyper ten sion , hyperch olesterolem ia, diabetes, coron ar y ar ter y disease,
or h istor y of st roke or t ran sien t isch em ic at t ack an d am aurosis fugax m ay be pres-
en t . Th ere m ay be a part ial field loss respect ing th e h orizon t al m eridian .
Fu n du s exam in at ion reveals a grayish w h ite ret in a along th e dist ribut ion of th e
affected vessel. An em bolus can be seen in th e vessel involved, usu ally at th e bifu r-
cat ion s. A n arrow ed bran ch ret in al ar ter y, boxcarring, segm en t at ion of th e blood
colum n s, an d cot ton -w ool spot s are th e com m on associated fin dings.
CRAO, cilioret in al ar ter y occlu sion , ret in it is, ret in al con t usion , n eoplasia, an d m y-
elin ated n er ve fiber layer
Management
Th e con dit ion is broadly invest igated an d t reated along th e sam e lin es as CRAO. All
th e t reat m en t m odalit ies used in th e case of CRAO h ave been t ried but w ith ver y
lit tle success. Prop er system ic invest igat ion s are don e frequen tly to diagn ose an d
elim in ate th e et iological factors. FFA an d elect roret in ography (ERG) play a role in
diagn osis of th e con dit ion , localizat ion of n onperfu sion areas, an d viabilit y of th e
involved ret in a. An t icoagulan ts, th rom bolysis, an d carot id en dar terectom y form
th e t reat m en t opt ion s, depen ding on th e resu lt s of various invest igat ion s.
Central Retinal Artery Occlusion/Ophthalmic

Artery Occlusion
Vision loss du e to CRAO is at t ributed to isch em ia of th e in n er ret in al layers an d

pykn osis of th e ganglion cell layer. Subsequ en t isch em ic n ecrosis of th e ret in a
cau ses opacificat ion . Fou rteen percen t of th e popu lat ion h as a cilioret in al ar ter y
th at is a com pon en t of ch oroidal circulat ion an d supplies th e m acu la w ith all th e
com pon en t s of th e m acu lopapillar y bu n dle. Men in th eir early sixt ies are m ost
com m on ly involved ( Fig. 9.4A,B,C,D).
A B
C D
Fig. 9.4 (A) Central retinal artery occlusion (CRAO) showing cherry-red spot.
(B) CRAO—color fundus photo. (C) CRAO fundus fluorescein angiography showing poor
filling even after 6 minutes. (D) CRAO FFA showing poor filling even after 10 m inutes.
Presentation
CRAO presen ts as u n ilateral, acu te, persisten t , pain less loss of vision . It can be bi-
lateral in 2% of th e p opulat ion . Am au rosis fugax an d oth er sym ptom s of tem po-
ral ar terit is are oth er associated fin dings. A h istor y of drug in t ake an d em bolic
ph en om en a are com m on ly presen t . Visu al acu it y m ay var y from h an d m ovem en t s
to a few m eters. Th e presen ce of a cilioret in al ar ter y por ten ds a bet ter progn osis
becau se th e cen t ral vision in th ese pat ien t s is w ell preser ved. Afferen t pup illar y
defects, pale disk w ith splin ter h em orrh ages, ch err y-red spot , grou n d-glass ap-
pearan ce of th e ret in a, boxcar segm en t at ion of th e arteries an d vein s, an d em bolu s
in ~20% of pat ien ts are com m on fin dings. A cardiac exam in at ion can reveal th e
presen ce of a bru it or m urm urs. A h istor y of tem poral ten dern ess, jaw clau dica-
t ion , m u scle w eakn ess, raised er yth rocyte sedim en t at ion rate (ESR), an d fever m ay
be associated.
An terior isch em ic opt ic n eu ropathy, in adver ten t in t raocu lar inject ion of gen tam i-
cin , blun t t raum a, an d oth er cau ses of ch err y red spot like Tay-Sach s disease, oth er
sph ingolipidoses, qu in in e toxicit y, m acular h em orrh age, hyper ten sion , Hurler syn -
drom e, m acular h ole, steroid inject ion s
Management
Laborator y st u dies su ch as com plete blood cou n t , ESR, fibrin ogen , an t iph os-
ph olipid an t ibodies, p roth rom bin t im e/act ivated part ial th rom bop last in t im e
(PT/aPTT), seru m protein elect rop h oresis, fast ing blood sugar, lipid profile, an d
blood cult ures are h elpful in th e et iological diagn osis. Im aging tech n iqu es such
as carot id u lt rasou n d, w h ich is su pposedly m ore specific th an carot id Doppler;
m agn et ic reson an ce im aging; an d FFA h elp in diagn osing th e site of occlu sion an d
th e progn osis. FFA sh ow s a gen eralized delay in th e dye flow in all th e ph ases of
angiogram . Elect rocardiography an d ech ocardiography are in dicated to ru le ou t
cardiac causes. ERG sh ow s a dim in ish ed B-w ave respon se.
CRAO w ith durat ion of onset less than 24 hours: Treat as an em ergen cy.
Ocular m assage: Apply direct pressure over the eye digitally or w ith a fundus con-
tact lens. The em bolus can lodge dow n, giving a better perfusion to the retina.
Anterior cham ber paracentesis: In t raocu lar pressu re (IOP) is ch ecked before th e
procedu re. Topical an esth esia an d a topical an t ibiot ic are given to th e pat ien t .
After w ith draw ing 0.1 to 0.2 m L of aqueous from th e an terior ch am ber th e IOP
is rech ecked. Th e procedure is repeated u n t il th e pat ien t’s IOP is n ot less th an 10
m m Hg.
Acetam ide : 500 m g in t raven ou s
Topical drugs: To low er IOP
Carbogen therapy (5% CO2 w ith 95% O2 ) : Carbon dioxide dilates th e ret in al ar te-
rioles. Oxygen in creases th e viabilit y of th e isch em ic t issues. It is given for 10
m in utes ever y 2 h ou rs for 48 h ou rs.
Hyperbaric oxygen : Most usefu l if started w ith in 2 h ou rs of th e episode; 100%
oxygen at 2 at m osph eric absolute provides an arterial PO2 of 1000 to 1200 m m
Hg resu lt ing in a th reefold in crease in th e oxygen su pply to th e isch em ic t is-
su es. St u dies sh ow a t w o-lin e im provem en t in 40% of th e pat ien t s subjected to
hyperbaric oxygen .
Int raarterial fibrinolysis: Tissu e plasm in ogen act ivator is preferred.
Approxim ately 20%of pat ien t s presen t w ith n ew vessels of iris w ith in a period of
4 to 5 w eeks. To preven t th is com p licat ion th e pat ien t h as to be review ed ever y 2
w eeks after th e episode. If any eviden ce of develop m en t of n ew vessels elsew h ere
or n ew vessels at th e disk is n oted, PRP is don e to preven t n eovascular glau com a.
Ocular Ischemic Syndromes
Th is ch ron ic vascular in su fficien cy occurs secon dar y to ath erosclerot ic disease of

th e in tern al or com m on carot id ar ter y. Gian t cell arterit is leads to a state of hy-
poxia to th e ocu lar st ru ct ures, w h ich causes n eovascu larizat ion an d oth er sym p-
tom s ( Fig. 9.5 ).
Presentation
Pat ien ts presen t largely in th eir fift ies an d sixt ies w ith t ran sien t obscu rat ion of
vision (am au rosis fugax) or w ith gradu al or abru pt loss of vision an d ocu lar pain .
An terior segm en t exam in at ion sh ow s sign s of congest ion , an terior uveit is, iris n ew
vessels, an d cat aract . Posterior segm en t exam in at ion sh ow s n arrow ing of th e ret i-
n al ar teries, dilated ret in al vein s, dot an d blot h em orrh age, cot ton -w ool spots, an d
opt ic n er ve or ret in al n ew vessels.
Fig. 9.5 Anterior segm ent neovascularization in ocu-

lar ischemic syndrome seen on anterior segment fluo-
rescein angiography.
CRVO, diabet ic ret in opathy, pan uveit is, an d vasculit is
Management
Cardiac w orku p, ESR, an d C-react ive protein (CRP) to exclude gian t cell arterit is,
FFA, an d oph th alm odyn am om et r y
Macroaneurysm
Macroan eur ysm is an acqu ired saccu lar dilatat ion of th e large ar terioles of th e
ret in a due to w eaken ing of th e ar teriolar w all seen com m on ly in w om en aged 60
to 70 years. Hyper ten sion , arteriosclerosis, an d seru m lipid abn orm alit ies are th e
com m on cau ses. Oth er cau ses are radiat ion ret in op athy, ven ou s occlu sive disease,
sickle cell, an d diabetes.
Presentation
Pat ien ts presen t w ith an asym ptom at ic, sudden , pain less decrease in vision du e to
vit reous h em orrh age, ret in al h em orrh age, or lipid exu dates in to th e m acu la. Th e
fun du s sh ow s an eur ysm al dilat ion of ret in al arterioles, circin ate exu dates, h em or-
rh age, m acu lar edem a, an d serou s ret in al det ach m en t .
Age-related m acular degen erat ion (ARMD), parafoveal telangiect asis, diabet ic
m acu lopathy, ret in al capillar y h em angiom a, BRVO
Management
Evalu ate cardiovascular disease, con t rol hyper ten sion , low er seru m lipids. Fu n dus
flu orescein angiograp h ic evaluat ion m ust be don e. Spon t an eou s resolu t ion follow -
ing th rom bosis is th e n at u ral cou rse. Lipid exu dates an d h em orrh age th reaten ing
th e fovea m ay be t reated w ith argon laser or yellow -dye laser applied arou n d th e
an eur ysm . Com p licat ion s in clu de h em orrh age an d occlusion of th e arteriole.
Vitreous Hemorrhage
Vit reous h em orrh age (VH) is th e ext ravasat ion of blood from th e ret in al vessels or
n ew vessels in to th e vit reous cavit y. Causes in clu de diabet ic ret in opathy, posterior
vit reous det ach m en t (PVD) w ith or w ith out a ret in al break, hyper ten sion , ret in al
vein occlu sion , exu dat ive age-related m acu lar degen erat ion (ARMD), proliferat ive
sickle cell ret in opathy, t raum a, in t raocular t u m or, an d Eales disease.
Presentation
Pat ien ts exp erien ce a su dden pain less loss of vision , floaters, an d cobw ebs. Den se
VH m ay redu ce th e visu al acuit y to th e percept ion of ligh t . On exam in at ion , th ere
is n o fun du s view w ith absen t red reflex. Ch ron ic vit reou s h em orrh age h as a
ch icken -fat app earan ce ow ing to organ ized blood.
Oth er causes of sudden loss of visu al acu it y
Management
Ult rason ography is don e w h en th e view of th e fu n dus is obst ru cted by VH. Histor y
an d physical exam in at ion are im por tan t , as w ell as exam in at ion of th e oth er eye.
If system ically feasible, an t icoagu lan t s an d oth er an t iplatelet agen t s m ay n eed to
be stopped. Treat m en t in clu des lim it ing physical act ivit y, bilateral patch ing, an d
sleeping in an u prigh t posit ion . Par t ial clearan ce of th e VH w ith som e ret u rn of
vision m ay occur over a few m on th s. Pars plan a vit rectom y m ay be requ ired if
spon tan eous clearing doesn’t occur.
Angioid Streaks
Angioid st reaks represen t deh iscen ces in th e Br uch m em bran e an d ap pear as dark
red ban ds of irregular con tour th at radiate from th e opt ic n er ve h ead. Angioid
st reaks are associated w ith Pseudoxanthom a elast icum (Grön blad-St ran dberg syn -
drom e), osteit is deform an s (Paget disease), sickle cell an em ia, hyper ten sive car-
diovascu lar disorders, an d Eh lers-Dan los syn drom e ( Fig. 9.6A,B).
Presentation
Th e pat ien t is asym ptom at ic. St udies repor t th at ch oroid n eovascularizat ion
m em bran e (CNV) occurs in 42 to 86%of pat ien ts during follow -u p, w ith m ost eyes
Fig. 9.6 (A) Angioid streaks—color fun-

dus photo. (B) Angioid streaks—fundus
fluorescein angiography.
progressing to legal blin dn ess. Pseudoxanthom a elast icum is associated w ith peau
d’orange an d m ot tled backgrou n d fun du s appearan ce. Focal m idp eriph eral fin e
exu dates an d opt ic n er ve h ead dru sen are also associated. Loose skin folds an d
plaqu elike lesion s can be seen in th e n eck (ch icken skin appearan ce), an d on th e
flexor aspect s of th e join ts.
Myopic lacquer cracks, ch oroidal ru pt u re
Management
FFA is ver y h elpfu l in delin eat ing angioid st reaks. Fluorescein angiography (FA) re-
veals early hyperfluorescen ce du e to t ran sm ission defects th rough at roph ic RPE
presen t n ext to th e st reak. Late-ph ase FFA sh ow s leakage at th e m argin of angioid
st reaks from adjacen t ch oriocapillaries an d deep ch oroidal vessels. Any eviden ce
of ch oroidal n eovascu lar m em bran e is also picked on FFA. In docyan in e green an -
giography is superior to fu n dus flu orscein in diagn osing th e details of a ch oroidal
n eovascular m em bran e. Laser ph otocoagulat ion for ju xtafoveal an d p arafoveal le-
sion s an d ph otodyn am ic th eor y (w ith in t ravit real t riam cin olon e aceton ide) for
su bfoveal CNVM h ave been recom m en ded.
Skin biopsy an d cardiological w orkup for su spected Pseudoxanthom a elast icum ,
seru m alkalin e ph osph atase, an d calcium levels for su spected Paget disease an d
h em oglobin elect roph oresis for su spected sickle cell an em ia are recom m en ded.
Degenerative Myopia
Progressive m yopia of m ore th an –6D or eyes w ith an axial length greater th an

26 m m h ave st ru ct u ral alterat ion s of th e globe result ing in axial length en ing, es-
pecially in th e posterior pole, leading to st retch ing of th e ret in a, th in n ing of th e
sclera, ch oroidal degen erat ion , an d poten t ial loss of vision .
Presentation
Decreased best-corrected visual acu it y an d progressive ch ange in p ow er are seen ,
usually beyon d th e fifth decade. Posterior segm en t fin dings in clu de vit reou s liq-
uefact ion , m yopic crescen t , t ilted disk, posterior st aphylom a, RPE abn orm alit ies,
patchy ch oroidal at rophy, Fu ch s spot (dark area cau sed by CNV), lat t ice degen era-
t ion in th e periph er y, an d lacquer cracks. Risk for rh egm atogen ous ret in al det ach -
m en t is in creased, especially w ith con cu rren t lat t ice degen erat ion ( Fig. 9.7 ).
Ocu lar associat ion s in clude cat aract , prim ar y op en -angle glaucom a, p igm en tar y
glaucom a, an d ret in opathy of prem at u rit y. System ic associat ion s in clu de Marfan
syn drom e, Eh lers-Dan los syn drom e, an d St ickler syn drom e.
Ocu lar h istoplasm osis, gyrate at rophy, congen ital staphylom a, age-related ch oroi-
dal at rophy, ARMD, an d angioid st reaks
Management
No t reat m en t w ill regress th e progression of staphylom a. An n u al to bian n u al care-
ful exam in at ion of th e fu n dus is recom m en ded. Sym ptom at ic ret in al breaks sh ou ld
be t reated w ith laser ph otocoagulat ion , cr yoth erapy, or scleral-bu ckling surger y.
Fig. 9.7 High myopia with chorio-

retinal degeneration and parapapil-
lary atrophy.
Central Serous Chorioretinopathy
Cen t ral serou s ch orioret in opathy (CSCR) is a localized serou s detach m en t of th e

n eurosen sor y ret in a overlying an area of leakage from th e ch oriocapillaris th rough
th e RPE. Leaks m ay be single or dou ble or m ay be m u lt iple in diffu se RPE dysfu n c-
t ion . Recu rren ce of 40 to 50% is n oted, w ith 5% of pat ien t s developing a ch oroi-
dal n eovascu larizat ion or progressive RPE at rophy. It is com m on in Hispan ics an d
Asian m en bet w een 20 an d 55 years of age. Type A person alit ies, pat ien ts on ex-
ogen ou s steroids, Cu sh ing syn drom e, system ic hyperten sion , system ic lu pu s er y-
th em atosus, pregn an cy, gast roesoph ageal reflu x disease, use of silden afil cit rate,
an d use of psych oph arm acological m edicat ion s are som e precipitat ing factors for
CSCR ( Fig. 9.8A,B).
Presentation
Th e pat ien t com m on ly presen t s w ith sym ptom s of vision loss, m icropsia, m et a-
m orph opsia, an d posit ive scotom a. Visual acu it y ranges from 20/20 to 20/80. Th e
pat ien t usu ally h as a sm all hyperopic correct ion w ith abn orm al ph oto st ress test ,
loss of color sat u rat ion , an d loss of con t rast sen sit ivit y. Clin ically CSCR can be asso-
ciated w ith pigm en t epith elial detach m en t s, RPE m ot tling an d at rop hy, subret in al
fibrin , subret in al lipid, or lipofuscin oid flecks.
Fig. 9.8 (A) Central serous reti-

nopathy—color fundus photo. (B)
Central serous retinopathy—fundus
fluorescein angiography. B
Macu lar h ole, differen t cau ses of CNV, ARMD, ch oroidal t um ors an d m et astasis,
CME, an d ret in al detach m en t
Management
Th e Am sler grid sh ow s distorted lin es or closely crow ded lin es. FFA dem on st rates
th e site of th e leak in a t ypical sm okest ack pat tern . OCT sh ow s a hyporeflect ive
flu id accu m ulat ion bet w een th e n eurosen sor y ret in a an d th e RPE layer. A deposi-
t ion of lipofuscin m ay be associated w ith CSCR, con fu sing it w ith vitelliform dys-
t rophy. OCT con firm s th e diagn osis by dem on st rat ing th e lipofuscin deposited be-
low RPE. ICG angiography m ay h elp in som e cases. ERG sh ow s a deficit in both eyes
in a pat ien t w ith u n ilateral CSCR poin t ing tow ard a system ic path ology.
Progn osis is gen erally good, w ith 85% of th e p at ien ts gain ing back 20/20 vision
in 6 to 8 w eeks. Laser ph otocoagu lat ion of th e leaking area sh or ten s th e disease
cou rse. It is don e on ly w h en th e leak is n oted 300 µm from th e cen ter of th e fovea
an d w h en th e disease process th reaten s to be ch ron ic. Laser is an opt ion in cases
w h ere th ere is a persisten t fluid level for m ore th an 4 m on th s, recurren ce in an eye
w ith visual deficit in th e oth er eye, presen ce of visu al deficit s in th e opposite eye
from previou s episodes of CSCR, an d occup at ion al n eed requiring prom pt recover y
of vision .
Plasm in ogen act ivator in h ibitor 1 h as been fou n d to h ave associat ion w ith CSCR.
Car valh o-Recch ia et al an d Haim ovici et al h ave derived a sign ifican t relat ion sh ip
bet w een pat ien ts on exogen ous steroids an d CSCR. ICG angiography repeatedly
dem on st rates both m ult ifocal ch oroidal hyperperm eabilit y an d hypoflu orescen t
areas suggest ive of focal ch oroidal vascular com prom ise in a pat ien t w ith CSCR.
Recen tly PDT h as been used to t reat su ch areas of hyperperm eabilit y in a case
w ith CSCR.
Cystoid Macular Edema
CME is th e accu m u lat ion of fluid (edem a) in th e in t racellu lar spaces of ou ter plexi-
form an d in n er n uclear layers of th e ret in a, cau sing Mü ller cell degen erat ion w ith
in t racellular vacu olat ion . CME w ith a postcat aract su rger y cau se is called Ir vin e-
Gass syn drom e. It is th e m ost com m on t ype. Oth er causes can be uveit is, diabet ic
m acu lopathy, AMD, ret in al vein occlu sion , ret in it is pigm en tosa (RP), vit reom acu-
lar t ract ion syn drom e, topical epin eph rin e, CNV, ret in al vascu lit is, ret in al telangi-
ect asias, in t raocu lar t um ors, n icot in ic acid, an d juven ile ret in osch isis.
Th ere are m any hypoth eses to describe th e m ech an ism leading to CME; h ow -
ever, th e m ost acceptable is th e on e involving in t raocular in flam m at ion , w h ich oc-
curs w h en CME com plicates uveit is or t ract ion /distor t ion of th e iris. In flam m ator y
m ediators gen erated from th e arach idon ic acid m et abolism , such as th e prosta-
glan din s an d leukot rien es, h ave been im plicated in th e p ath ogen esis (Fig. 9.9A,B).
Fig. 9.9 (A) Postoperative cys-

toid macular edema—color fundus
photo. (B) Postoperative cystoid
macular edem a—fundus fluoresein
angiography. B
Presentation
Pat ien t presen ts w ith gradu al loss of vision or m et am orph opsia an d a h istor y of a
cer t ain p recipit at ing factor. Biom icroscopy reveals ch aracterist ic cyst ic in tercellu-
lar sp aces in th e m acular region w ith dim in ish ed foveal ligh t reflex an d associated
foveal th icken ing. Eviden ce of in t raocular in flam m at ion like ciliar y flush , vit reou s
cells, n er ve fiber layer edem a, or papilloedem a m ay be presen t .
In Ir vin e- Gass syn drom e, sign s of com plicated cat aract su rger y m ay be p res-
en t , su ch as vit reous t ract ion , vit reou s loss, polym erase ch ain react ion , exch ange
of in t raocular len s (IOL), secon dar y IOL, old age, post–pen et rat ing keratoplast y,
post–YAG capsu lotom y, topical epin eph rin e, or latan oprost .
Ret in al telangiect asias, juven ile ret in osch isis, ph ototoxic injur y, Berlin edem a,
CNV, an d foveal cyst s
Management
FFA sh ow s an early an d m idph ase leak from th e ch oriocap illaries surroun ding fo-
vea. In th e late ph ase a t ypical petaloid or sp oke-w h eel pat tern of pooling of dye is
eviden t . An associated con dit ion can also be p icked up in FFA. OCT reveals th e cys-
t ic spaces presen t in th e layers of ret in a. It can also be u sed as a m on itoring device
becau se it can m ain t ain a serial foveal th ickn ess record of a pat ien t .
Sp on t an eou s resolut ion occu rs in 75% of postcataract pat ien t s w ith in a span of
6 w eeks. Cor t icosteroids h elp by in h ibit ing th e en zym e ph osph olipase an d th u s
con t rolling th e in flam m at ion in post uveit ic cau ses. It can be adm in istered by all
routes, su ch as topical, oral, posterior su bten on , an d in t ravit real. Non steroidal an -
t iinflam m ator y drugs (NSAIDs) in h ibit th e en zym e cyclooxygen ase an d can also be
u sed in th e preven t ion of CME. NSAIDs, especially ketorolac an d flu rbiprofen , can
be adm in istered topically for 3 to 4 m on th s. Carbon ic an hydrase in h ibitors su ch as
acetam ide are h elpfu l in con t rolling CME in pat ien ts of RP. Prolonged CME due to
BRVO respon ds w ell to laser ph otocoagu lat ion .
Th e role of p ars plan a vit rectom y is in dicated in CME related to uveit is. It is
h elpfu l in rem oving vit reou s st ran ds, in flam m ator y m ediators, ret ain ed len s frag-
m en ts, an d m alposit ion ed IOLs as w ell as for greater pen et rat ion of topical an d oral
cort icosteroids an d su ppressing th e an t igen -specific im m un e respon se. Th erapies
such as an t im etabolites, cyclodiath erm y, cr yoth erapy, an d ph otocoagu lat ion h ave
been t ried w ith lim ited success. Th e adven t of n ew m odes of steroid deliver y su ch
as posterior subten on depot an d in t ravit real inject ion of steroids are bet ter op-
t ion s available. Various st u dies (e.g., Italian diclofen ac st udy group ) h ave repor ted
th e su periorit y of ketorolac over oth er topical agen ts in t reat ing CME.
Age -Related Macular Degeneration
Exudative Age -Related Macular Degeneration

In 1995, th e In tern at ion al ARM Epidem iologic St udy Grou p redefin ed age-related
m acu lar degen erat ion (ARMD). Pat ien t s w ith m in im al to m oderate n on exudat ive
age-related ch anges at th e m acula w ere classified as age-related m acu lopathy
(ARM). Advan ced at rophy or th e presen ce of CNV m em bran es w as classified as
ARMD. Th e con dit ion is seen m ore com m on ly in w om en th an in m en an d is diag-
n osed in in dividuals over th e age of 50. Th e use of tobacco, obesit y, an d gen et ic fac-
tors are believed to be st rongly associated w ith th e con dit ion (Fig. 9.10A,B,C,D).
Presentation
Th e pat ien t u sually presen t s w ith a gradual, pain less loss of vision associated
w ith delayed dark adapt at ion , severe m et am orph opsia, an d field loss. Vision loss
is m ore rapid in exudat ive ARMD. A su dden loss of vision can be associated w ith
su bret in al h em orrh age or pigm en t epith elium det ach m en t . Th e Am sler grid an d
frequ en t slit-lam p biom icroscopy are th e best w ays to m on itor th e con dit ion (Fig.
9.10E,F,G,H).
Non exu dat ive ARMD, angioid st reaks, ch oroidal rupt u re, an d oth er causes leading
to CNV m em bran e
Management
FFA is an im p or tan t tool in diagn osis an d classificat ion (subfoveal, ju xtafoveal,
an d ext rafoveal) of CNV. ICG angiography provides a m ore specific diagn osis by
pinpoin t ing th e feeder vessel in th e ch oroidal vasculat ure. OCT is u sed as a tool
to follow u p after ph otodyn am ic th erapy (PDT).
A B
C D
E F
G H
Fig. 9.10 (A) Dry age -related macular degeneration (ARMD)—color fundus photo.
(B) Dry ARMD—FFA late phase. (C) Dry ARMD, same patient, other eye—color fundus
photo. (D) Dry ARMD, same patient, other eye—FFA late phase. (E) Wet ARMD—CNVM—
color fundus photo. (F) Wet ARMD—CNVM—midphase FFA. (G) Wet ARMD—CNVM—late
phase FFA. (H) Wet ARMD, same patient, other eye—venous phase FFA.
Th erm al laser p h otocoagu lat ion an d feeder vessel ph otocoagulat ion can be u sed
to t reat all kin ds of CNV m em bran es.
Tran spupillar y th erm oth erapy form s a t reat m en t m odalit y for occult CNV
m em bran es.
PDT using ph otosen sit izers (verteporfin , rost aporfin , m otexafin lu tet ium , tala-
porfin sodium , ATX-S10) is w idely used to t reat classic CNV. Target-receptor PDT
an d com bin at ion PDT h ave also been t ried.
An t iangiogen ic agen t s su ch as vascular en doth elial grow th factor (VEGF) in h ibi-
tors (pegaptan ib sodium , ran ibizum ab, bevacizum ab, VEGF t rap) form th e latest
adjuvan t in th e t reat m en t of CNV m em bran es.
Steroids like t riam cin olon e aceton ide, an ecor t ave acetate, an d im plan table cor-
t icosteroids h ave also been t ried.
Sm all in terfering RNA th erapy, pigm en t epith elium –derived factor in ducer, an d
m icrost ruct ure m odu lat ion are som e of th e t reat m en t m odalit ies u n der t rial.
Subm acu lar surgeries, m acu lar t ran slocat ion , an d RPE t ran splan tat ion are th e
su rgical opt ion s at variou s st ages of developm en t .
Stargardt Disease and Fundus Flavimaculatus
At roph ic m acu lar dyst rophy w ith flecks (i.e., St argardt disease) is th e m ost com -
m on t ype of juven ile m acu lar degen erat ion . Fu n dus flavim aculat us (FF) is a vari-
an t of St argardt disease w ith a sign ifican t differen ce in clin ical presen t at ion an d
progn osis. Alm ost all cases are determ in ed as autosom al recessive in th e pedigree
ch ar t . Rarely, cases h ave been iden t ified as au tosom al dom in an t . Th e gen e locu s
lies on ABC4R on 1p21–22. FF is also an autosom al recessive disease.
Presentation
Th ere is con siderable con fu sion related to th e term s Stargardt disease an d fundus
flavim aculat us. FF w as first described as a flecked fu n dus w ith yellow -w h ite ir-
regular lesion s th at exten ded to th e equ ator. St argardt disease occu rred as flecks
in th e posterior pole early in life w ith a p rogressive m acu lar dyst rophy. It is n ow
believed th at th ese t w o con dit ion s are t w o differen t allelic m an ifest at ion s of th e
sam e disease.
Th e sym ptom s are m ain ly bilateral, presen t ing in th e age grou p from 6 to 20
years. Th ere is associated color vision defect an d ph otop h obia. Disappearan ce of
th e foveal reflex form s th e first sign on oph th alm oscopy. Later, a gran u lar m ot tling
of th e m acula appears. An oval “sn ail-slim e” or “beaten bron ze” appearan ce of th e
fovea is th e h allm ark of Stargardt disease. Th e flecks m ay exten d to th e m idperiph -
er y an d m ay give a salt-an d-pepper app earan ce in th e region . Th e disk an d vessels
are gen erally n orm al. FF is usu ally diagn osed in ciden tally. Th e pat ien t can experi-
en ce a reduced cen t ral visu al acu it y an d m etam orph opsia in longst an ding cases.
FF is a bilateral disorder w ith ill-defin ed yellow ish w h ite flecks (“fish tail-like”)
exten ding from th e posterior pole to th e m idperiph er y. Th e fu n du s h as a verm ilion
t inge in m ost cases ( Fig. 9.11A,B).
Fig. 9.11 (A) Stargardt disease

fundus flavimaculatus—color fundus
photo. (B) Stargardt disease fundus
flavimaculatus—fundus fluorescein
angiography showing early hyper-
fluorescence.
Fu n dus albipu n ctat us, ret in it is pu n ct a albescen s, fam ilial dru sen , p at tern dyst ro-
phy, RP inversus, ARMD, h istosp ots
Management
FFA sh ow s th e m ost ch aracterist ic feat u re called silen t ch oroid. Th e eviden ce of
flecks over th e con t rast ing dark ch oroid w ith a n orm al m acu la is a t radem ark an -
giograph ic appearan ce of FF. ERG sh ow s a variable ph otopic resp on se an d n orm al
scotopic respon se. Elect ro-olfactogram (EOG) is subn orm al. Deu teran an d t ritan
defects are presen t . Progn osis is poor; h ow ever, visu al acuit y usu ally st abilizes at
6/60.
Fig. 9.12 Flecked retina.
Fundus Albipunctatus
Fu n du s albip un ct at u s is congen it al st at ion ar y n igh t blin dn ess w ith autosom al re-

cessive pat tern .
Presentation
Pat ien ts m ay be asym ptom at ic or h ave n igh t blin dn ess, u su ally st ar t ing in in fan cy.
Num erou s w h ite-yellow dotlike lesion s occu r in th e m idperiph eral fun du s at th e
level of th e RPE ( Fig. 9.12 ).
Ret in it is p un ct ate albescen ce (progressive, beh aves like RP), birdsh ot ch orioret i-
n it is, St argardt disease, fam ilial dru sen , Biet t i cr ystallin e ret in opathy, pat tern
dyst rophy, RP inversu s, m acu lar degen erat ion , pseu doexfoliat ion , cr ystallin e ret i-
n opathy, h istospots
Management
ERG sh ow s a prolonged t im e for dark adapt at ion to produ ce n orm al am plit u de du e
to th e delay in regen erat ion of rh odopsin . Vision an d fields rem ain n orm al.
Best Disease : Vitelliform Macular Dystrophy
Best disease is an au tosom al dom in an t (variable p en et ran ce) degen erat ive m acu -
lopathy w h erein a m u tat ion in th e best roph in gen e leads to lipofuscin accu m u la-
t ion in RPE cells.
Presentation
Pat ien ts m ay be asym ptom at ic in ch ildh ood an d progress to a decrease in vision
later in life. Fu n dus exam in at ion sh ow s a large, yellow, yolklike (vitelliform ), bilat-
eral, sym m et rical m acu lar lesion (p seu dohypopyon ). Vision m ay be bet ter th an th e
fun du s appearan ce. Th e yellow “yolk” even t ually breaks dow n , leaving a m ot tled
geograp h ical at rophy an d scarring (scram bled egg appearan ce).Vision deteriorates
in th is stage. Ch oroidal n ew vessels m ay be presen t .
The late scar stage is difficult to distinguish from m acular dystrophy or degeneration.
Management
Th e EOG is dim in ish ed, w h ereas th e ERG is n orm al. Th ere is n o kn ow n t reat m en t .
Cases sh ould be follow ed w ith an Am sler grid for early detect ion of CNV.
Pattern Dystrophy
Th is is an au tosom al dom in an t dyst rop hy of th e RPE du e to m u t at ion in th e pe-

riph erin gen e, w h ich presen t s in m idlife.
Presentation
Visual acu it y m ay be n orm al or m ildly decreased (~20/30), w ith n orm al color vi-
sion an d m ild m etam orph opsia. Oth er presen t ing sym ptom s in clude sym m et ric
bilateral hyperpigm en t at ion of th e RPE in th e m acula, but terfly dyst rophy, an d
adult-on set foveom acu lar vitelliform dyst rophy.
Drug-induced degenerations, angioid streaks, rubella retinopathy, myotonic dystrophy
Management
Th e progn osis is good. EOG is subn orm al. Fluorescein angiography sh ow s a block-
ing pat tern of th e RPE hyperpigm en t at ion . CNV an d geograph ical m acular at rophy
are th e com plicat ion s. No t reat m en t is kn ow n .
Cone Dystrophies
Goodm an et al classified variou s con e dyst roph ies in to seven con e dysfun ct ion
syn drom es. Sporadic cases are m ost com m on am ong th e various pat tern s of in -
h eritan ce seen in con e dyst roph ies. Au tosom al dom in an t is th e m ost com m on ly
establish ed pat tern an d carries a ver y grave progn osis. Th e con dit ion m ay also
presen t as autosom al recessive an d be X-lin ked.
Presentation
Th e sym ptom s are exactly th e reverse of RP an d appear gen erally in th e th ird de-
cade. Th e pat ien t m ost com m on ly presen t s w ith loss of vision an d ph otoph obia.
Th e pat ien t sees bet ter in even ing h ou rs an d prefers to w ear dark glasses. Color
vision is a problem begin n ing in th e early stages of th e con dit ion . Nyst agm us can
be seen in th e advan ced stage. Th e early stage of th e disease is m arked by a n orm al
fun du s or a gran ular appearan ce of th e m acu la. Late st ages classically resem ble a
bull’s-eye m acu lopathy. Few sign s m ay be congru en t w ith RP, th at is, th e presen ce
of bony spicules in th e m idperiph er y, vascular at ten u at ion , an d tem poral disk pal-
lor. A t apet al-like sh een is n oted arou n d th e parafoveal region in a colored ph oto-
grap h . Advan ced disease is ch aracterized by th e t ypical presen ce of RPE at rophy,
w h ich later develops in to a geograph ical at rophy.
Drug-in du ced ret in opathy, angioid st reaks, ru bella ret in opathy, an d m yoton ic ret i-
n opathy
Management
FFA fin dings dep en d on th e stage of th e disease an d it s severit y. A t ran sm ission
defect involving fovea is an oth er presen t at ion sim ilar to bull’s-eye m aculop athy.
Fun du s fin dings m ay also m im ic th ose of Stargardt disease or FF in th e advan ced
stages of th e disease. Ph otop ic respon se is subn orm al or alm ost un recordable. Se-
vere deuteron –t rit an defects are n oted. EOG an d dark adapt at ion are n ot reliable.
Progn osis depen ds on th e exten t of rod involvem en t an d m ode of in h eritan ce. No
t reat m en t is kn ow n .
Idiopathic Parafoveal Telangiectasia
In idiopath ic parafoveal telangiect asia th ere is clin ically apparen t ret in al telangiec-
tasia an d ectasia of th e capillar y bed, con fin ed to th e ju xtafoveal region of on e or
both eyes. Th is m ay result in visu al loss from capillar y in com peten ce an d exu da-
t ion . Histopath ologic eviden ce sh ow s th is is n ot a t ru e telangiectasia bu t rath er
con sists of st ru ct u ral abn orm alit ies sim ilar to diabet ic m icroangiopathy, w ith de-
posits of excess basem en t m em bran e w ith in th e ret in al capillaries ( Fig. 9.13A,B).
Presentation
On set is usu ally in th e fifth to sixth decade. Pat ien ts presen t w ith m ild blurring of
cen t ral visu al acu it y.
Group I: Un ilateral, congen ital parafoveal telangiectasia w ith vascu lar abn or-
m alit ies localized to th e tem poral h alf of th e m acula, leading to circin ate exu -
dates
Group II: Bilateral acquired idiopath ic telangiect asia
Group III: Bilateral idiop ath ic parafoveal telangiectasia w ith ret in al capillar y
obliterat ion On FFA, leakage is seen from th e telangiect at ic vessels.
Fig. 9.13 (A) Parafoveal telangiec-

tasia leakage—color fundus photo.
(B) Parafoveal telangiectasia—fun-
dus fluorescein angiography donut-
shaped leakage.
Diabet ic m acu lar edem a, m acular edem a from oth er cau ses, CNV
Management
Gen erally, on ly obser vat ion is required. Laser ph otocoagu lat ion an d in t ravit real
bevacizu m ab/ran ibizu m ab h ave been t ried.
Sickle Cell Retinopathy
Th is h ereditar y, gen et ically determ in ed, h em olyt ic an em ia occu rs alm ost exclu -
sively in blacks. HbSS (i.e., sickle cell disease) occu rs exclu sively in m ales.
Presentation
Oph th alm ic m an ifest at ion s in clude in t ravascular occlusion s on th e surface of th e
opt ic disk; ret in al ar ter y occlusion s; ch ron ic m acu lar ch anges (sickling m acu lopa-
thy); ch oroidal vascular occlusion s; n onproliferat ive sickle ret in opathy in cluding
ven ous tor t u osit y, salm on -patch h em orrh age, sch isis cavit y, an d th e black su n -
burst an d proliferat ive sickle ret in opathy w ith periph eral ar teriolar occlusion s;
ar teriolar-ven ular an astom osis; n eovascular proliferat ion ; vit reou s h em orrh age;
an d ret in al det ach m en t .
Management
Diath erm y, cr yoth erapy, xen on arc ph otocoagu lat ion , an d argon laser ph otoco-
agulat ion can be t ried to t reat th e proliferat ive sickle ret in opathy. Vit rectom y is
in dicated in con dit ion s such as n on resolving vit reou s h em orrh age an d t ract ion or
com bin ed ret in al detach m en ts.
Retinitis Pigmentosa
RP is a h ereditar y diffu se pigm en t ar y ret in al dyst rophy ch aracterized by th e ab-

sen ce of in flam m at ion , progressive field losses, an d abn orm al ERG. RP involves
ph otoreceptors, m ain ly th e rods. Sporadic cases are by far th e m ost com m on ( Fig.
9.14A,B).
Presentation
Presen tat ion is alw ays bilateral. Pat ien t s presen t m ain ly w ith nyctalop ia an d a
gradu al an d progressive loss of visual fields. Loss of cen t ral vision an d m et am or-
ph opsia are an in dicat ion of associated m acu lar involvem en t . A few pat ien t s also
experien ce episodic ligh t flash es. Th e classical t riad to diagn ose RP is arteriolar
at ten uat ion , bony spicule–like pigm en t at ion , an d w axy pallor of th e opt ic disk.
Fig. 9.14 (A) Retinitis pigmentosa

with maculopathy. (B) Retinitis pig-
m entosa—peripheral fundus shows
B bone spicule pigm ent hyperplasia.
RP can presen t at ypically as sector RP, pericen t ral RP, an d RP w ith exu dat ive vas-
culopathy. RP is associated w ith posterior su bcapsu lar cataract , op en -angle glau-
com a, m yop ia, keratocon u s, posterior vit reous det ach m en t , an d opt ic n er ve h ead
dru sen . System ic associat ion s of RP are as Bassen -Korn zw eig syn drom e, Refsum
disease, Ush er syn drom e, Kearn s-Sayre syn drom e, an d Bardet-Biedl syn drom e.
Ch oroidal dyst roph ies, vit am in A deficien cy, congen ital st at ion ar y n igh t blin dn ess,
ph en oth iazin e toxicit y, syp h ilis, congen it al ru bella, exu dat ive ret in al detach m en t ,
m yoton ic dyst rophy.
Management
FFA in dicates th e associated ch oroidal sclerosis an d in dicates th e progn osis. A de-
fect ive dark adapt at ion , EOG, an d scotopic respon se on ERG are seen . Color vision
is n orm al an d perim et r y sh ow s circu m feren t ial progressive field loss. Gen eral oph -
th alm ic care (i.e., refract ive stat us of p at ien t) sh ould be given . Associat ion s su ch as
cataract , glaucom a m acular edem a, an d epiret in al m em bran es sh ou ld be m an aged
on respect ive lin es. Psych ological cou n seling, gen et ic cou n seling, low vision aid,
an d vit am in an d n ut rit ion al supp lem en tat ion play im por tan t roles. Recen tly, ret i-
n al ch ip device, stem cells, an d gen e th erapy h ave been t ried.
Choroideremia
Ch oroiderem ia is a grou p of X-lin ked recessive disorders in w h ich th ere is a m ut a-

t ion in th e long arm of ch rom osom e X (Xq-21.2; CHM). Th is focu s involves m u ta-
t ion of th e CHM gen e, w h ich en codes for a protein geranylgeranyl t ran sferase Rab
escor t .
Presentation
Th e feat ures of th e disease are n igh t blin dn ess, con st rict ion of visual field, an d
ring scotom a, w ith severe visual loss occurring at later stages of life. Th e fu n du s
sh ow s patch es of ch oroidal an d ret in al pigm en t ar y at rophy. Th e involvem en t is at
th e level of ch oriocapillaries w ith involvem en t of large an d in term ediate ch oroidal
vessels in th e late stages w h en th ere is a clear visibilit y of sclera. A subn orm al EOG
an d severely abn orm al ph otopic ERG are ch aracterist ic fin dings.
RP, gyrate at rophy, albin ism
Management
Th e ch oroidal ph ase of th e FFA is ch aracterized by clear visualizat ion of th e filling
of m ediu m - an d large-caliber ch oroidal vessels. Th is pict u re presen ts du e to com -
plete absen ce of th e RPE an d an associated absen ce of ch oriocapillaries un der it .
Th ere is n o kn ow n t reat m en t . Gen et ic cou n seling h elps.
Gyrate Atrophy
Th is is an au tosom al recessive disorder w ith involvem en t of ch rom osom e 10 an d

involves th e m ut at ion of gen e coding for th e en zym e orn ith in e ketoacid am in o-
t ran sferase, w h ich is required for orn ith in e degradat ion . Th ere are elevated levels
of orn ith in e in th e body flu ids (i.e., plasm a, urin e, cerebrospin al flu id, an d aqu eou s
h um or).
Presentation
Th e m ain clin ical fin dings are n igh t blin dn ess associated w ith axial m yopia an d
ast igm at ism . Fun du s exam in at ion sh ow s lobular ch orioret in al degen erat ion in
th e region beh in d th e equ ator w ith vit reou s degen erat ion . Th ese later progress to
geograph ic at rophy w ith foveal involvem en t . In advan ced stage of th e disease, th e
lesion s convalesce to form w ider defects an d exten d in both an terior an d posterior
direct ion s. Flat ERG in th e later stages w ith su bn orm al EOG is seen .
Paving-ston e degen erat ion , ch oroiderem ia, h igh m yop e
Management
FFA in early st age of th e disease is ch aracterized by sh arply dem arcated m u lt iple,
circular w in dow defects, separated by st rip s of n orm al ret in a involving th e m id-
periph er y. Th e late ph ase is ch aracterized by fading flu orescen ce of th e ch oroidal
vessels an d m ild st ain ing of th e n orm al ret in a form ing th e m argin of th e lesion s.
Treat m en t involves redu ct ion of orn ith in e in th e diet w ith vit am in B6 in h igh doses.
First sym ptom s are seen in th e first or secon d decade. Visual acu it y becom es ver y
p oor by th e sixth decade of life w ith involvem en t of th e m acula.
Radiation Retinopathy
Irradiat ion to th e h ead in a tot al dose exceeding 3000 rads m ay lead to radiat ion
ret in opathy. It m ay follow plaqu e th erapy, brachyth erapy, or extern al-beam irra-
diat ion . Pat ien t s w ith preexist ing com p rom ised vascu lat u re such as th ose w ith
diabetes, hyper ten sion , or collagen vascu lar disease m ay develop it at even low
doses of radiat ion .
Presentation
Pat ien ts m ay be asym ptom at ic or h ave visu al loss delayed 6 m on th s to a year after
th erapy. Discrete foci of occluded capillaries; irregular dilatat ion of n eigh boring
m icrovascu lat u re an d vascular sh eath ing are seen at th e posterior fu n du s along
w ith cot ton -w ool spot s, m icroan eu r ysm s, n eovascularizat ion , exudates, an d m ac-
ular edem a. Disk edem a m ay also be seen .
Diabet ic ret in opathy, hyper ten sive ret in opathy, Eales disease, Coats disease, sickle
cell ret in opathy
Management
Macu lar edem a an d n eovascu larizat ion m ay be t reated w ith laser ph otocoagula-
t ion . System ic steroids are given for p apillopathy.
Solar Retinopathy
Foveom acu lar ret in it is, eclipse ret in opathy, solar ret in it is, follow ing direct/in -
direct view ing of th e solar eclip se. Secon dar y to RPE dam age cau sed by sh orter
w avelength s of u lt raviolet-A ligh t .
Presentation
Sym ptom s appear after 1 to 4 h ours of su ngazing an d in clude un ilateral or bilat-
eral decrease in visu al acu it y, m etam orph op sia, cen t ral/paracen t ral scotom a, an d
after-im age. Yellow -w h ite foveolar lesion s th at are replaced w ith a red foveolar
depression are seen su rrou n ded by a pigm en ted h alo. Vision im proves in 3 to 6
m on th s (usu ally to > 20/40), but m etam orph opsia an d scotom a m ay rem ain .
Blun t ocu lar t rau m a, RP
Management
Th ere is n o t reat m en t . Th e p at ien t is follow ed u p.
10 Surgical Retina
Clem ent K. Chan and Dariusz G. Tarasew icz
Developmental Anomalies and Degenerations
Cystoid Degeneration
Reticular Cystoid Degeneration

Ret icular cystoid degen erat ion is a t ype of periph eral ret in al cystoid degen erat ion
th at h igh ly correlates w ith age. It occurs in approxim ately 18% of adult pat ien t s
an d can be bilateral m ore th an 40% of th e t im e. It is n ot as com m on as t ypical cys-
toid degen erat ion . It can be radially orien ted an d often follow s th e cou rse of ret in al
vessels. Frequen tly, it is fou n d posterior to t ypical cystoid degen erat ion .
Presentation
It is asym ptom at ic. A st ip p led ap p earan ce to t h e in n er ret in al su r face is com -
m on . Ret icu lar cystoid d egen erat ion is t yp ically fou n d in t h e in fer ior tem p oral
qu ad ran t .
Oth er periph eral ret in al degen erat ion s
Management
Th orough oph th alm oscopic exam in at ion is requ ired. Man agem en t con sist s of ob-
ser vat ion on ly.
Typical Cystoid Degeneration

Typical cystoid degen erat ion is th e m ost com m on periph eral ret in al degen erat ion .
Spaces form in th e outer plexiform an d in n er n uclear layer an d coalesce to form
t u n n els. W h ere t u n n els h ave n ot form ed, ret in al “pillars” rem ain . Th ese fin dings
occur as early as age 1. It is fou n d in alm ost all eyes exam in ed. Typical cystoid de-
gen erat ion is m ore com m on in th e su perior an d tem poral qu adran ts.
Presentation
No sym ptom s are n ot able. Th e in n er ret in al su r face acqu ires a st ip p led ap p ear-
an ce. Pillars cau se d ep ression s on t h e su r face, w h ereas d om elike areas rep resen t
cystoid sp aces. Th e d egen erat ion alw ays begin s at t h e ora ser rat a an d sp read s
p oster iorly.
288
10 Surgical Retina 289
Management
Th orough oph th alm oscopic exam in at ion is requ ired. Man agem en t con sist s of ob-
ser vat ion on ly.
Meridional Folds, Complexes, and Other Peripheral Lesions

A m eridion al fold is a redu n dan cy of th e periph eral ret in a at th e border of th e ora
serrata. It is m ost com m on ly foun d in th e supran asal qu adran t an d is bilateral for
55% of th e cases. Its length can be variable. It h as been est im ated th at 26% of th e
popu lat ion h as m eridion al folds.
W h en a m eridion al fold is con t igu ou s w ith a den t ate process, it is called a m e-
ridion al com p lex. A m eridion al com plex can exten d to th e pars plan a ( Fig. 10.1A).
An en closed oral bay (EOB) is a sm all islan d of n onpigm en ted ciliar y body ep i-
th elium th at h as becom e isolated an d surroun ded by n eurosen sor y ret in a. Clin i-
cally, it m ay resem ble a ret in al break ( Fig. 10.1B).
Presentation
Both th e m eridion al folds an d th e EOB are in ciden tal fin dings in n orm al eyes an d
are u su ally n ot associated w ith any sym ptom s. Th e locat ion of m eridion al folds
m ay aid th eir diagn osis. Th ey are alw ays close to th e ora serrata. Th ey are alm ost
n ever associated w ith ret in al breaks.
A ret in al break is a differen t ial diagn osis for EOB. Scleral in den tat ion sh ow s grad-
ually slop ing edges un like th e sh arp an d abru pt ch ange in ret in al breaks. Also th e
color in th e oral bay correspon ds to th e adjacen t pars plan a.
Management
Careful oph th alm oscopy w ill differen t iate EOB from t ru e ret in al breaks. Th e color
w ith in th e EOB is th e sam e as th e pars plan a. A m eridion al fold is frequ en tly lo-
cated in th e sam e m eridian as an EOB. Un like ret in al breaks, th ese lesion s do n ot
progress rapidly over t im e. Th ere is n o role for prophylact ic t reat m en t .
A B
Fig. 10.1 (A) Meridional fold complex. ( B) Enclosed oral bay.

Pars Plana Cyst

Pars p lan a cyst s are convex, cystoid lesion s fou n d an terior to th e ora serrat a. Th ey
are often adjacen t to oral bays an d can be m ult iple. Th e exact et iology is u n kn ow n ,
but th ey are probably th e result of a degen erat ive p rocess. Microscopically th ey
represen t a separat ion of th e n onpigm en ted from th e pigm en ted epith elial cell
layers of th e pars plan a.
Presentation
Pars plan a cysts are asym ptom at ic. An au topsy series fou n d th eir presen ce in 16
to 18% of cases. Th ey are m ore frequen tly fou n d on th e tem poral side. Th ere is n o
associat ion w ith oth er eye diseases.
Differential diagnosis
Non e
Management
Th orough op h t h alm oscop ic exam in at ion reveals t h e cyst . No t reat m en t is in d i-
cated .
Pars Plana Pearls

Pars plan a pearls are st riking, glisten ing w h ite lesion s fou n d in th e far an terior
ret in al periph er y. Th ey are alw ays ben eath a den tate process an d represen t dr u-
sen -like deposit s on th e in n er surface of th e Bru ch m em bran e.
Presentation
Pars plan a pearls ser ve as good lan dm arks in th e far periph eral ret in a. Th ey are
alw ays located ben eath a den tate process. Th ey are drusen -like st ru ct u res on th e
Bruch m em bran e.
Management
Pars plan a pearls h ave been fou n d in 20% of au topsy subject s an d appear in all age
groups. Th ere are n o kn ow n associat ion s w ith oth er eye path ologies. Th orough
oph th alm oscopic exam in at ion is requ ired. No t reat m en t is in dicated.
Pavingstone Degeneration
Also com m on ly term ed cobblestone degenerat ion, th ese are discrete foci of ou ter
ret in al at rophy th at st art at th e ora an d progress posteriorly. It h as been est im ated
th at th ese lesion s occur as early as age 8. Histologically th ey are ch aracterized by
loss of rods, ret in al pigm en t epith elium (RPE), an d th e un derlying ch oriocapillaris
( Fig. 10.2 ).
Fig. 10.2 Pavingstone degenera-

tion.
Presentation
In addit ion to th e outer ret in al at rophy, th ese lesion s sh ow prom in en t ch oroidal
vessels an d are easy to see on in direct oph th alm oscopy. Th ey can h ave a yellow -
ish h ue, an d pigm en ted borders are com m on . Th ese lesion s ten d to clu ster on th e
in ferior ret in a.
Management
Th orough op h th alm oscopic exam in at ion is required. Th ese lesion s are n ot associ-
ated w ith any sym ptom s or risk for ret in al det ach m en t (RD). Man agem en t con sist s
of obser vat ion on ly; n o t reat m en t is in dicated.
Senile Tapetochoroidal Degeneration

Tapetoch oroidal degen erat ion represen t s a pron oun ced degree of depigm en t at ion
in th e periph eral ret in a. It is cau sed by a loss of pigm en t gran ules in th e RPE, usu -
ally m ore pron ou n ced w ith advan cing age.
Presentation
Exam in at ion reveals a circum feren t ial ban d run n ing from th e ora serrata to th e
equ ator.
Management
Tapetoch oroidal degen erat ion is foun d in 20%of pat ien t s older th an 40 an d is bilat-
eral in all cases. Th orough oph th alm oscopic exam in at ion is requ ired.
Lattice Degeneration, Retinal Tufts, and Retinoschisis
Lattice Degeneration
Lat t ice degeneration is a periph eral retin al pathology that carries a m oderate risk
for a retinal detach m en t. It h as been estim ated th at lat tice degen eration is foun d
in only 6 to 8% of the gen eral population . It is bilateral in up to 48% of n on selected
patients. Mult iple st udies h ave show n, h ow ever, that 20 to 30% of patients w ith
a retin al detachm ent h ave lat tice degen eration. Lat t ice degeneration h as been as-
sociated w ith m ultiple nam es, including sn ail-track degen erat ion an d retinal and
vitreous base excavation s, am ong others. Th ere is a h igh correlation bet w een lat tice
degen eration an d m yopia. No defin itive h ereditar y pat tern has been establish ed.
Presentation
A h allm ark feat u re of lat t ice is its variabilit y in presen t at ion . Classically it is de-
scribed as a patch of ret in al th in n ing w ith abn orm al overlying vit reou s. Vit reous
liquefact ion over a lat t ice lesion is com m on , an d vit reoret in al adh esion on it s bor-
ders is t aut an d st rong. W h ite lin es frequ en tly crisscross th e ret in al su rface of lat-
t ice degen erat ion . Perip h eral pigm en t ar y ch anges an d w h ite flecks are often fou n d
on th e posterior surface of th e lesion . Th e w h ite lin es corresp on d to sclerot ic ves-
sels, an d th e flecks are con sisten t w ith glial cells.
Progressive th in n ing of th e ret in al surface of lat t ice degen erat ion leads to form a-
t ion of at roph ic h oles. Th ese h oles are n ot associated w ith vit reous t ract ion . Al-
th ough m ild su bret in al fluid m ay accu m u late, asym ptom at ic at roph ic ret in al h oles
associated w ith lat t ice degen erat ion rarely lead to RD.
Lesion s of lat t ice degen erat ion are u su ally foun d bet w een th e 11 an d 1 o’clock
an d 5 an d 7 o’clock m eridian s for un kn ow n reason s. Th e dim en sion s of lat t ice
patch es are h igh ly variable, even w ith in th e sam e eye. Lat t ice lesion s are t ypically
asym ptom at ic.
Management
Myopia is a w ell establish ed risk factor for lat t ice degen erat ion . Lat t ice degen era-
t ion h as been lin ked to pigm en t dispersion syn drom e, alth ough th e reliabilit y of
th e associated st u dies h as been qu est ion ed becau se of th eir sm all sam ple sizes.
Myopia is sh ared by both con dit ion s, an d com m on et iological factors m ay con t rib-
ute to th e developm en t of both con dit ion s.
Lat t ice degen erat ion is best revealed th rough careful in direct oph th alm oscopy
or periph eral ret in al biom icroscopy w ith scleral depression . Sign ifican t debate ex-
ist s on th e t reat m en t of lat t ice degen erat ion . Th e presen ce of lat t ice degen erat ion
in creases th e risk of ret in al detach m en t from 0.01 to 0.5%.
Mu lt iple au th ors h ave poin ted to th e fut ilit y of prophylact ic t reat m en t of asym p-
tom at ic lat t ice degen erat ion . Even if at roph ic h oles are foun d in a patch of lat t ice,
th ey rarely result in RD. A ret rospect ive st u dy sh ow ed th at for eyes w ith RD, pro-
phylact ic t reat m en t of lat t ice degen erat ion in th e fellow eyes w as associated w ith
a risk reduct ion of RD from 5.9 to 1.8%. Previou s st u dies sh ow ed n o ben efit w ith
prophylact ic t reat m en t for eyes w ith greater th an –6 diopters of m yopia an d m yo-
pic eyes w ith m ore th an 6 clock h ours of lat t ice degen erat ion .
Close m on itoring of asym ptom at ic lat t ice lesion s is th e rout in e. Prophylact ic

t reat m en t is reser ved for th e follow ing sit uat ion s: sym ptom at ic lat t ice lesion s,
sign s of prom in en t vit reoret in al t ract ion , person al or fam ily h istor y of RD.
Retinal Tufts
Cystic Retinal Tufts

Th ese are discrete n odules of glial proliferat ion th at en t rap overlying vit reou s.
Th us th e bon d bet w een t uft an d vit reous is par t icu larly st rong. It is con sidered a
congen it al varian t . Approxim ately 5% of th e populat ion h as t u fts. Tufts h ave been
fou n d as early as in fan cy an d do n ot sh ow a par t icu lar age predilect ion .
Presentation
Cyst ic t u ft s can be located in all four qu adran ts. Th e average diam eter is from 0.1 to
1.0 m m . On clin ical exam th ey are u sually w h ite an d oval in appearan ce. Th ey are
elevated on th e ret in al su rface but n ot t ran slu cen t like a ret in al break. Becau se of
th e t igh t adh esion bet w een a ret in al t uft an d th e overlying vit reous, a ret in al flap
tear or an opercu lated break m ay develop at th e site of th e t uft during a posterior
vit reous detach m en t (PVD). Associated sym ptom s in clu de floaters, flash ing ligh ts,
“cu r tain effect s,” an d loss of vision .
Management
Careful in direct oph th alm oscopy or p eriph eral ret in al biom icroscopy w ith scleral
depression . Approxim ately 10% of RDs are origin ated from cyst ic ret in al t ufts.
How ever, because cyst ic ret in al t uft s are foun d in on ly 5% of th e gen eral popu la-
t ion , th e overall risk of a ret in al det ach m en t from a t u ft is calcu lated to be on ly
0.28%. Ow ing to th is low in ciden ce, rou t in e prophylact ic t reat m en t of asym ptom -
at ic t u ft s is n ot recom m en ded. If a ret in al tear is associated w ith a t u ft , laser or
cr yoth erapy is in dicated.
Noncystic Retinal Tufts

Th ese t u ft s are ver y th in aggregates of ret in al t issu e fou n d in clusters at th e vit re-
ous base. Th ey are less th an 0.1 m m in diam eter an d con sist of processes of Mü ller
cells.
Presentation
Non cyst ic t u ft s are fou n d as early as th e first decade of life. Th ey are foun d in 33%
of adu lts an d usually on th e n asal p eriph eral ret in a. Th ere are n o sym ptom s.
Management
Man agem en t con sist s of obser vat ion on ly, w ith carefu l in direct oph th alm oscopy
or p eriph eral ret in al biom icroscopy w ith scleral depression .
Zonular Traction Retinal Tuft

Zon ular t ract ion t u fts (ZTTs) are dist ingu ish able from cyst ic an d n on cyst ic t u ft s by
th eir greater size, closer locat ion to th e ora serrata, an d predilect ion for cau sing
sm all, full-th ickn ess ret in al h oles in th e far ret in al periph er y. ZTTs are cau sed by
abn orm al zon u lar con n ect ion s to th e periph eral ret in a. Th e result is a t aut t u ft of
t issue pulled an teriorly tow ard th e ciliar y body.
Presentation
ZTTs can be fu r th er subdivided in to ju xtabasal or in t rabasal t ufts. On ly in t rabasal
ZTTs h ave clin ical sign ifican ce. Ret in al tears occur in 4% of all ZTTs. Th ey are th e
cau se for 6% of ret in al tears in au topsy eyes. Associated tears are alm ost alw ays
in t rabasal (w ith in th e vit reou s base) lesion s. Even w h en RD occurs, it is localized
an d n onprogressive. Sym ptom s occu r on ly if com plicated by ret in al tears or RD.
Management
ZTTs occur th ree t im es m ore com m on ly in m en an d h ave a prevalen ce of 15% in
au topsy cases. Th ey are also bilateral in 15% of cases an d m ore com m on in th e
n asal ret in a. Th ey are best revealed th rough careful in direct oph th alm oscopy or
periph eral ret in al biom icroscopy w ith scleral depression . Laser or cr yoth erapy is
in dicated if th ere is eviden ce of a fu ll-th ickn ess ret in al break. Prophylact ic t reat-
m en t of ZTTs is n ot recom m en ded because associated ret in al breaks an d det ach -
m en ts are u su ally n onp rogressive.
Senile Retinoschisis
Sen ile ret in osch isis is a degen erat ive process in w h ich th e ret in a split s in to t w o
dist in ct layers at th e level of th e outer plexiform layer, in con t rast to juven ile ret i-
n osch isis, in w h ich th e ret in a split s at th e n er ve fiber layer. Dysfu n ct ion of Mü ller
cells likely con t ributes to th e form at ion of sen ile ret in osch isis. Th e ret in al sch isis
cavit y th at form s over t im e m ay resem ble a ret in al det ach m en t . Tw o form s of sch i-
sis h ave been described: t ypical degen erat ive an d ret icu lar degen erat ive.
Presentation
In t ypical degen erat ive ret in osch isis th ere are oval areas of ret in al sp lit t ing an d
fusiform elevat ion of th e in n er ret in al layer. Th e in n er layer con sist s of in tern al
lim it ing m em bran e, in n er p lexiform layer, an d ret in al vessels. It can h ave a tex-
t u red appearan ce, an d w h ite dot s can be seen on it s surface. Th e outer layer con -
sists of p or t ion s of ou ter plexiform , outer n u clear, an d ph otoreceptor com pon en t s.
It appears em pt y an d can h ave a beaten -m etal appearan ce. Ret in al breaks are n ot
ch aracterist ic for t ypical degen erat ive ret in osch isis. Posterior exten sion tow ard
th e m acula is un com m on .
Ret icular degen erat ive ret in osch isis h as m ore exten sive ret in al involvem en t .
The in n er ret in a can becom e ext rem ely th in , bu llou s, an d dom e-sh aped. It con sists
of on ly in tern al lim it ing m em bran e, rem n an t s of n er ve fiber layer, an d residu al
vessels th at appear susp en ded in m id-air du e to th e th in ret in al appearan ce. Th ere
is com plete loss of radial pillars. Typical cystoid degen erat ion is com m on ly seen
an terior to areas of ret icu lar degen erat ive ret in osch isis. Ret in osch isis can be ex-
acerbated by a posterior vit reous detach m en t . Th e sch isis cavit y m ay en large an d
som et im es progress tow ard th e m acu la. How ever, th e m acula is frequen tly spared,
an d th e rate of progression is u sually ver y slow.
Outer ret in al breaks are m ore com m on th an in n er ret in al breaks in ret icu lar
degen erat ive ret in osch isis. Th e appearan ce of dem arcat ion lin es in dicates th e de-
t ach m en t of th e ou ter ret in a of a ret in osch isis an d its even t ual conversion to a
full-th ickn ess RD. Th e rate of such a conversion is ver y slow. Th e n onprogressive
t ype of det ach m en t occurs m uch m ore frequ en tly th an th e progressive t ype. How -
ever, th e form at ion of in n er ret in al h oles along w ith th e preexist ing ou ter ret in al
h oles m ay lead to a rapid progression of a fu ll-th ickn ess RD th at requires prom pt
t reat m en t .
Sym ptom s occu r in case of ret in al breaks, ret in al detach m en t , or posterior ex-
tension tow ard th e m acu la.
Management
For au topsy eyes, t ypical degen erat ive ret in osch isis is seen in 1%of th e adult popu -
lat ion an d is bilateral in 33%of cases. Ret icu lar degen erat ive ret in osch isis is seen in
1.6% of adu lt s, an d bilateral on ly 16% of th e t im e. Both lesion s are com m on ly seen
in th e in ferotem poral qu adran t .
Man agem en t con sists of careful in direct oph th alm oscopy or periph eral ret in al
biom icroscopy w ith scleral depression .
Rout in e prophylact ic t reat m en t of m ost ret in osch isis is n ot n ecessar y ow ing
to th eir usual lack of progression . Th eir in discrim in ate t reat m en t m ay cause n ew
ret in al breaks, ret in al detach m en t , an d even p roliferat ive vit reoret in opathy. W ith
sign s of progression , t reat m en t m ay in clude laser, cr yoth erapy, scleral buckling,
an d vit rectom y, depen ding on th e locat ion an d exten t of th e path ology.
Posterior Vitreous Detachment
Presentation
Floaters (“cobw ebs,” “bugs,” “t adpoles,” or com m a-sh ap ed object s th at ch ange po-
sit ion w ith eye m ovem en t), blurred vision , flash es of ligh t , w h ich are m ore com -
m on in dim illu m in at ion an d are tem porally located, are th e m ain com plain t s of
th e pat ien t . On e or m ore discrete ligh t gray to black vit reou s op acit ies, often in th e
sh ape of a ring (Weiss ring) are su spen ded over th e opt ic disk. Th e opacit ies float
w ith in th e vit reous as th e eye m oves from side to side.
Vit rit is, m igrain e, vit reous h em orrh age, RD
Management
Com plete ocu lar exam in at ion , par t icularly a dilated ret in al exam in at ion , by using
in direct oph th alm oscopy an d scleral depression to r ule ou t a ret in al break an d
detach m en t is m an dator y. Th ere is n o sim ple t reat m en t for vit reous floaters. Using
th e Neo-dym ium -YAG laser to break up vit reou s floaters is a con t roversial pract ice
an d m ay be associated w ith th e risk of creat ing ret in al breaks an d RD. A vit rectom y
is reser ved on ly for ver y den se vit reous floaters.
Retinal Tears and Retinal Detachment
Th e posterior fun du s st r uct ures involved w ith th e developm en t an d m an agem en t

of a ret in al detach m en t in clu de th e n eu rosen sor y ret in a, ch oroid, RPE, pars plan a,
an d sclera. A rh egm atogen ou s RD is th e m ost com m on t ype of RD. It is cau sed by
on e or m ore ret in al breaks. Most ret in al breaks are located bet w een th e equator
an d th e ora serrata (28%) or n ear th e equator of th e globe (45%). Ch oroidal vortex
vein s are th e m ost visible lan dm arks adjacen t to th e equ ator. Periph eral ret in al le-
sion s (see Ch apter 9), in cluding 12% of sm all ret in al breaks, are frequ en tly located
close to th e ora serrat a, th e scalloped ju n ct ion bet w een th e n eu rosen sor y ret in a
an d th e pars plan a at th e vit reous base.
Th e distan ce bet w een th e equator an d th e ora m easures bet w een 5 an d 6 m m ,
an d it is sligh tly greater in th e tem poral th an in th e n asal ret in a. Th e pars plan a
con sists of an in n er n onpigm en ted an d an ou ter pigm en ted layer th at span s ~6
m m from th e posterior lim bus to th e ora serrata radially at th e an terior fu n dus. It
represen ts a su rgical “safe” zon e, th rough w h ich n eedles an d surgical in st ru m en ts
m ay be passed w ith out cau sing ocu lar dam ages. Rare pars plan a breaks in clude fo-
cal tears an d dialyses associated w ith cert ain con dit ion s (e.g., congen it al develop -
m en t , t raum a, aph akia, an d atopic derm at it is). On ly 15% of ret in al breaks develop
posterior to th e equator, in clu ding m acu lar h oles (1%), w h ich m ay in du ce a ret in al
detach m en t in h igh ly m yopic eyes.
In teract ion s bet w een cen t rifugal an d cen t rip et al forces across th e n eu rosen sor y
ret in a determ in e th e st at u s of ret in al at t ach m en t versu s detach m en t . Cen t rifugal
forces th at in du ce ret in al detach m en t in clude (1) vit reou s flu id en t r y in to th e sub-
ret in al space th rough ret in al breaks an d (2) vit reoret in al t ract ion al forces. Cen t rip-
et al forces th at prom ote ret in al at t ach m en t in clude (1) out w ard hydrostat ic vit re-
ous flu id pressu re on th e ret in al su rface, (2) m ucopolysacch aride adh esives an d
in terdigitat ing cellular processes bet w een th e ret in al ph otoreceptors an d th e RPE,
(3) ch oroidal on cot ic pressu re, an d (4) m etabolic pum p of th e RPE. An im balan ce
in favor of cen t rifugal forces th at leads to a ret in al detach m en t is m ost frequ en tly
cau sed by th e on set of ret in al break(s).
Presentation
Th e an n ual in ciden ce of rh egm atogen ous RD is close to 1 per 15,000 p eople, an d
th e prevalen ce is 0.3% of th e gen eral populat ion . Th e prevalen ce is in creased for
“h igh -risk” groups: 2% for aph akic pat ien ts, 2% for pat ien t s w ith asym ptom at ic
lat t ice degen erat ion , 5% for h igh ly m yopic pat ien t s, an d 10% for pat ien ts w ith vit-
reou s loss du ring cat aract ext ract ion .
Most ret in al breaks an d rh egm atogen ou s RDs develop spon t an eou sly. Th e ag-
ing p rocess gen erates vit reous syn eresis. Th e en suing fibrillar degen erat ion of th e
vit reous predisposes an acu te PVD an d associated ret in al breaks/RD. Th erefore,
th e m ost com m on age grou p for RD is bet w een 40 an d 70 years. Approxim ately 40
Fig. 10.3 Types of retinal break.

(A) flap tear; (B) atrophic retinal
hole; (C) operculated break; (D) reti-
nal dialysis.
to 50% of RDs are associated w ith m yopia, 30 to 40% are associated w ith aph akia
or p seu doph akia, an d 10 to 20% are associated w ith direct ocular t raum a. Close
to 60% of RDs occur in m ales, possibly because th ere are m ore m ale th an fem ale
m yopic p at ien t s. Abou t 15% of RDs even t ually develop in th e fellow eyes as w ell.
Bilateral RDs are m ore com m on in aph akic eyes (25 to 30%).
Gen et ic predisposit ion to RD in clu des Jew ish race an d fam ilial ten den cy (i.e.,
Jen sen disease). Th e black populat ion h as a low in ciden ce of RD.
Visual-field loss an d scotom as are com m on feat u res for all categories of ret i-
n al det ach m en t . Macu lar involvem en t leads to cen t ral vision loss. Th e locat ion of
th e ret in al detach m en t is in th e opposing quadran t(s) to th e visu al-field defect
ow ing to th e gen erally con t ralateral topograph ical correspon den ce bet w een th e
ret in a an d th e visu al field. An terior vit reou s pigm en t gran ules detected on slit-
lam p biom icroscopy con st it u te a reliable h allm ark sign (posit ive Sh afer sign ) for
th e presen ce of ret in al break(s). Vit reou s h em orrh age is frequen tly but n ot alw ays
associated w ith ret in al breaks/RD.
Th ere are several t ypes of ret in al breaks ( Fig. 10.3 ):
Flap tears: Th ese are also kn ow n as h orsesh oe tears du e to th eir U-sh aped m or-
ph ology. An terior vit reoret in al t ract ion on th e ret in al flaps con n ected to th e an -
terior m argin s of th e tears m akes th e flaps poin t an teriorly tow ard th e vit reou s
base. Sym ptom at ic flap tears are h igh risk. Su bret in al fluid ten ds to accum ulate
arou n d th e breaks, an d h en ce, breaks sh ou ld be t reated prom ptly. A gian t tear
span s 3 or m ore clock h ours an d u su ally h as a scrolled flap th at m ay roll over on
itself. It requ ires a vit rectom y as m an agem en t .
At rophic ret inal holes: Th ese lesion s are due to progressive ret in al th in n ing
w ith ou t associated vit reoret in al t ract ion . Th ey are often w ith in patch es of lat-
t ice degen erat ion . Most at roph ic h oles h ave a low risk an d do n ot requ ire pro-
phylact ic t reat m en t , par t icu larly w h en located in feriorly.
Operculated break s: Persisten t vit reoret in al t ract ion m ay lead to a com plete
separat ion of a free-float ing vit reou s opercu lu m from th e u n derlying break. It
ten ds to locate m ore posterior th an flap tears. It is in term ediate in risk (be-
t w een flap tears an d at roph ic h oles) for an RD. Su perior tem poral opercu lated
ret in al tears are m ore vuln erable th an in ferior n asal opercu lated tears for vision
loss. Treat m en t or obser vat ion is on a case-by-case basis.
Dialysis: Th is is a circum feren t ial ret in al tear by th e ora serrata. Un like oth er
breaks, vit reoret in al t ract ion is on th e posterior m argin of th e dialysis. It oc-
curs m ore com m on ly in th e you ng. Ret in al dialysis m ay be congen it al (m ostly
in th e in ferior tem poral qu adran t) or t raum at ic (usually in th e superior n asal
qu adran t). Ret in al dialysis carries m oderate to h igh risk for RD an d m ay requ ire
t reat m en t , in clu ding laser, cr yoth erapy, or scleral bu ckling/vit rectom y.
Acute : For acute ret in al breaks leading to rh egm atogen ou s RD in duced by PVD,
cardin al sym ptom s in clu de flash ing ligh t s, floaters, cobw ebs, an d a cur t ain ef-
fect . Th e m ech an ical dist urban ce of th e ret in a by vit reoret in al t ract ion is re-
spon sible for th ese ph otopic ph en om en a.
Ret in al det ach m en t s ( Fig. 10.4 ) are divided in to th ree t ypes: rh egm atogen ou s,
exu dat ive, an d t ract ion al ( Table 10.1 ).
Rhegm atogenous RD: Th ese are th e m ost com m on . Th ey involve ret in al breaks.
Exudat ive RD: Th ese are caused by excessive accum ulat ion of subret in al serous
flu id th at m ay sh ift w ith ch anges in body posit ion . Exam p les of con dit ion s as-
sociated w ith an exu dat ive RD in clude cen t ral serou s ch orioret in opathy, various
ch oroidal t um ors, an d diverse posterior in flam m ator y diseases.
Tract ional RD: Th ese are caused by vit reoret in al t ract ion (e.g., proliferat ive dia-
bet ic ret in opathy). W h ereas a su rgical in ter ven t ion is u su ally in dicated for a
rh egm atogen ous RD, it is often n ot requ ired for an exudat ive RD. A t ract ion al
RD involving th e m acu la requ ires su rger y.
A ret in osch isis m ay m im ic a ret in al detach m en t . Dist in ct ive feat ures of ret in os-
ch isis in clude a th in an d dom e-sh ap ed elevated in n er layer, absen ce of dem arca-
Fig. 10.4 View of a tum or associated with a retinal

detachm ent. The detached retina is denoted with blue
arrow.
Table 10.1 Di e rences betw een Types o f Retinal Detachm ent
Rhegm a-
Feature to geno us Exudative Tractio nal
1. Retinal break Yes No No primary break;

secondary break
can occur
2. Shifting uid No Yes No
3. Choroidal mass No May be No
present
4. Intraocular Low Varies Normal
pressure
5. Transillumination Normal Blocked trans- Norm al
illumination
If pigmented
choroidal
lesion is present
6. Retinal mobilit y Undulating folds Smoothly elevated, Taut retina
no folds
7. Extent of RD Extends to ora Gravit y dependent Does not extend
quickly to ora
8. Pigm ent in Mostly present No Present in trauma
vitreous cases
9. Subretinal uid Clear Turbid Clear
RD, ret in al det ach m en t .
t ion lin es, an d clu sters of ou ter oval ret in al h oles ( Fig. 10.5 ). It is often bilaterally
sym m et rical an d frequen tly localizes to th e in ferotem poral quadran t . If diagn osis
is u n cer tain , a laser spot test can be perform ed. A m ild ret in al laser u pt ake on th e
ou ter ret in al layer con firm s th e iden t it y of ret in osch isis. Most ret in osch isis lesion s
do n ot progress an d requ ire n o th erapy. Developm en t of both in n eran d outer-
layer h oles m ay conver t a ret in osch isis in to a rh egm atogen ous RD, w h ich requires
prom pt t reat m en t . A rare progression of a ret in osch isis close to th e m acula also
requ ires barrier laser th erapy, scleral bu ckling, or vit rectom y.
Fig. 10.5 Retinoschisis.

Management
Th e effect ive t reat m en t of a rh egm atogen ous RD requ ires th e iden t ificat ion an d lo-
calizat ion of all ret in al breaks ( Fig. 10.6 ). In direct oph th alm oscopy w ith scleral in -
den t at ion is an in dispen sable tool for accom p lish ing th is goal. An cillar y tech n iqu es
for revealing ret in al breaks in clude biom icroscopy an d ult rason ography. Historical
details of th e RD m ay provide clues of th e respon sible breaks. For in stan ce, an in -
ferior visual-field defect poin t s to a su perior tear an d RD. Rapid progression of th e
Fig. 10.6 Algorithm for localizing retinal break(s) associated with a retinal detach-
ment. RD, retinal detachm ent; SRF, subretinal fluid; VF, visual field.
Fig. 10.7 Final view of buckle with proper retinal indentation and positioning of the
tear. This internal/external conceptual illustration shows a cross section and the corre-
sponding surgeon’s view of the final configuration of a circumferentially placed sponge
exoplant (E). The cross section shows a portion of the globe without sclera (S) to en-
hance clarit y for the reader. The surgeon’s view is through the indirect ophthalmoscope
(O). Note the indented configuration in both views. The retina is reat tached and the
tear (T) is flat. Also note that the tear is properly positioned on the anterior slope of the
buckle, as indicated by the cot ton-tipped applicator (A). (Courtesy of Highlights of Ophthalmol-
ogy, “Retinal and Vitreoretinal Surgery: Mastering the Latest Techniques” English Edition, 2002. Editor-
in-Chief: Benjamin F. Boyd, MD., FACS; Co-Editor: Samuel Boyd, MD.)
RD suggests a large break, a p osterior break, or a su perior break. Previou s report s

also poin t out th e im port an ce of th e pat tern of su bret in al fluid dist ribu t ion in rela-
t ion to th e locat ion s of th e prim ar y ret in al breaks.
Th e closure of all ret in al breaks by in duct ion of firm ch orioret in al adh esion s
bet w een th e breaks an d un derlying RPE is th e key to successfu l m an agem en t of
a rh egm atogen ou s RD. Adh esive m odalit ies in clu de diath erm y, cr yoth erapy, an d
ph otocoagu lat ion ( Fig. 10.7 ).
Diathermy
Electrical current generates heat at the tip of the diatherm y probe, used for local-
izing retinal breaks. Diatherm y lesions on partial-thickness sclera induce strong
chorioretinal adhesions as w ell. Full-thickness treatm ent is avoided due to scleral
necrosis.
Cryothera py
Low -tem perat ure freezing at th e t ip of th e cr yoprobe allow s ret in al t reat m en t
th rough full-th ickn ess sclera w ith ou t scleral dam age. Direct m on itoring w ith in -
direct oph th alm oscopy for a w h it ish ice-ball su rroun ding th e ret in al break is th e
best m eth od.
Photocoagula tion
Precise ch orioret in al upt akes w ith laser ph otocoagulat ion can be applied on ly on
at t ach ed ret in a or areas of m in im al su bret in al fluid, lim it ing it s u t ilit y in RD repair.
Tran sscleral applicat ion of a diode laser sh ow s prom ising resu lts. Supplem en tal
ph otocoagu lat ion on th e buckle en h an ces resorpt ion of residual su bret in al flu id
adjacen t to ret in al breaks du ring or after scleral bu ckling. In t raocu lar inject ion of
gas before or after supplem en tal laser for t am pon ade of th e su perior ret in al breaks
on th e bu ckle m ay fur th er facilitate th e resolut ion of th e residu al subret in al fluid.
Sclera l Buckling
Th e th ree cardin al step s for successfu l scleral buckling in clu de (1) accu rate local-
izat ion of all ret in al breaks, (2) in duct ion of ch orioret in al adh esion s, an d (3) sup-
por t of ret in al breaks on th e buckle.
Localizat ion of breaks is at tain ed w ith extern al scleral m arks by diath erm y or
sim ilar devices u n der gu idan ce of in direct oph th alm oscopy. Ch orioret in al adh e-
sion s are ach ieved w ith cr yoth erapy, diath erm y, or ph otocoagu lat ion . Su ppor t of
ret in al breaks is accom plish ed w ith extern al scleral in den t at ion w ith don or t issues
or syn th et ic m aterials. Solid silicon e ru bber or sponges are th e m ost com m on ly
em ployed bu ckling elem en ts. Th ey are an ch ored in place w ith scleral su t ures or
scleral belt loops, placing th e breaks on th e an terior crest of th e buckle. Scleral
buckling tech n iqu es are broadly divided in to episcleral versus in t rascleral, en cir-
cling versu s segm en t al, an d drain age versus n on drain age. Ep iscleral tech n iques
h ave largely supp lan ted in t rascleral m eth ods in m odern scleral bu ckling. Table
10.2 outlin es th e algorith m s for ch oosing en circling versu s segm en t al buckling
procedu res. Su bret in al flu id drain age is perform ed th rough a ch oroidotom y u n der
a radial scleral cut-dow n w ith a tapered su t ure n eedle, diath erm y n eedle, 27- or
30-gauge n eedle, or en dolaser probe.
In dicat ion s for drain age in clu de (1) bu llou s RD w ith h igh ly elevated tears, (2)
p seu doph akic or h igh ly m yopic eyes w ith m ult iple periph eral breaks, (3) ch ron ic
RD w ith viscou s subret in al fluid an d cellu lar elem en t s, (4) eyes w ith poor ch oroi-
dal or RPE fu n ct ion , an d (5) severe glau com a or fresh cat aract w oun d n ot toleran t
of ocu lar hyper ten sion . Recurren t RD du e to drain age com plicat ion s occurs in 12%
of cases. Long-term successes of drain age an d n on drain age bu ckling procedu res
are equ ivalen t .
Table 10.2 Cho ice o f Buckling Pro ce dures
Segm ental versus Encircling
Few peripheral breaks Many peripheral breaks

Single break Extensive breaks
Mobile retina Rigid retina with folds
Clear vitreous Cloudy vitreous, vitreous strands and m em branes
Healthy sclera Thin and weak sclera
Good visualization Suboptimal visualization
Alterna tive Techniques

Altern at ive tech n iqu es in clu de pn eu m at ic ret in opexy, tem porar y orbit al balloon ,
an d prim ar y vit rectom y. Pn eum at ic ret in opexy involves cr yoth erapy or laser to
in duce ch orioret in al adh esion s an d inject ion of a sm all gas bubble to t reat lim ited
su perior ret in al breaks (w ith in 1 to 2 clock h ours ap ar t) of a sim ple RD. A tem -
porar y orbital balloon allow s t reat m en t of sim ple RDs w ith lim ited su perior or
in ferior breaks w ith ou t n eed of perm an en t buckling. Vit rectom y ach ieves direct
in tern al flat ten ing of com plex breaks an d RDs w h ile avoiding extern al bu ckling
elem en t s.
In t raoperat ive an d postoperat ive factors con t ribute to su rgical failure. In t raoper-
at ive causes in clu de (1) in adequate localizat ion , ch orioret in al adh esion , or su ppor t
of breaks; (2) m arked su bret in al or ch oroidal h em orrh age; (3) in adequate relief of
vit reoret in al/ch orioret in al t ract ion ; (4) com plicat ion s of subret in al flu id drain age
(in carcerat ion , perforat ion , h em orrh age); (5) in adverten t ret in al, ch oroidal, scleral
perforat ion s; an d (6) severe ch oroidal detach m en t . Postoperat ive causes in clude
(1) m arked exudat ive ret in al an d ch oroidal det ach m en t , (2) an terior segm en t isch -
em ia, (3) proliferat ive vit reoret in opathy, (4) ext r usion or in t rusion of bu ckling ele-
m en ts, an d (5) in fect ion s (episcleral, en doph th alm it is).
Num erou s advan ces in vit reoret in al su rgical tech n iqu es p rom ise m ore precise
an d qu icker surgical t reat m en ts for RD on an ou t pat ien t basis (Figs. 10.8 , 10.9 ,
Fig. 10.8 Retinal reat tachment with perfluorocarbon liquid in case of giant tear. In the
case of retinal detachment with a giant retinal tear, perfluorocarbon liquid (P) is injected
into the vitreous cavit y via a Chang cannula (N). Because the liquid has a specific gravit y
greater than water, it gravitates (blue arrow) to the posterior pole. This forces the sub-
retinal fluid (S, red arrows) out through the giant retinal tear and out of the eye via the
Chang cannula. The retina (R) is being forced to reat tach (green arrow) in this manner.
E, endoillum inator; I, infusion cannula. (Courtesy of Highlights of Ophthalmology, “Retinal and
Vitreoretinal Surgery: Mastering the Latest Techniques” English Edition, 2002. Editor-in-Chief: Benjamin
F. Boyd, MD, FACS; Co-Editor: Samuel Boyd, MD.)
Fig. 10.9 For surgical treatment of proliferative vitreo-retinopathy, perfluorocar-

bon liquid (L) m ay be injected. This will reveal any persistent retinal traction related
to epiretinal membranes (P) that must be rem oved. A vitreoretinal pic or Grieshaber
mini-diamond forceps (F) is used to remove such a m em brane (P). Note the subretinal
mem brane (S), endoilluminator (E), and infusion terminal (I). (Courtesy of Highlights of Oph-
thalmology, “Retinal and Vitreoretinal Surgery: Mastering the Latest Techniques” English Edition, 2002.
Editor-in-Chief: Benjam in F. Boyd, MD, FACS; Co-Editor: Sam uel Boyd, MD.)
Fig. 10.10 Air-fluid exchange and internal drainage of subretinal fluid (white arrow)
with an extrusion needle (A) is performed to flat ten the retina. E, endoilluminator. (Cour-
tesy of Highlights of Ophthalmology, “Retinal and Vitreoretinal Surgery: Mastering the Latest Techniques”
English Edition, 2002. Editor-in-Chief: Benjamin F. Boyd, MD, FACS; Co-Editor: Samuel Boyd, MD.)
Fig. 10.11 Combined m icrophakonit (700 µm cataract sur-

gery) and 25-gauge transconjunctival sutureless vitrectomy.
10.10 ). Pn eum at ic ret in opexy an d vit rectom y h ave becom e in creasingly p opu lar
tech n iques for repairing RD. Recen t advan ces in 25-gauge vit rectom y tech n ology
m ay allow it s applicat ion on cer tain sim ple RDs ( Fig. 10.11 ).
Scleral Buckle Procedure
Scleral buckling procedure is a t im e-h on ored surgical tech n ique first popu larized
by Cu stodis for repairing an RD. Su ccessful scleral bu ckling requires th e fu lfillm en t
of several im port an t criteria, in clu ding (1) th e accu rate assessm en t an d localiza-
t ion of all ret in al breaks an d vit reoret in al t ract ion , (2) appropriate applicat ion of
ret in opexy for all associated ret in al breaks, (3) precise p lacem en t of scleral bu ck-
ling elem en t s to sup por t th e u n derlying ret in al breaks an d relief of vit reoret in al
t ract ion , an d (4) proper drain age of subret in al flu id. Drain age of su bret in al flu id
is n ot alw ays n ecessar y, an d som e su rgeon s prefer th e n on drain age scleral bu ckle
procedu re.
Accurate Localization of Retinal Breaks

Th e con tour of an RD is frequ en tly determ in ed by th e locat ion s of its associated
ret in al breaks. Figure 10.12 depicts th e rules for predict ing th e locat ion (s) of th e
ret in al break(s) resp on sible for th e RD based on it s con tou r. Lin coff h as poin ted ou t
th at for RDs w ith an asym m et rical con tou r n ot crossing th e 12 o’clock m eridian ,
th e resp on sible break is fou n d w ith in 1.5 h ours from its h igh est level. In case th e
RD crosses th e 12:00 m eridian , th e prim ar y break(s) can be detected w ith in 1½
h ou rs from 12:00. Sch epen s an d Hilton h ave also in dicated a series of rules regard-
ing fin ding th e respon sible ret in al breaks: (1) th e prim ar y break is close to th e u p-
per m argin of th e RD for a su perior quadran t ic detach m en t , (2) th e prim ar y break
is close to 12:00 for a superior sym m et rical h em isph eric RD, (3) th e prim ar y break
Fig. 10.12 The distribution of the subretinal fluid associated with a retinal detachment
(RD) points to the likely location of the retinal break(s): (a) superior RD: break is near the
superior edge; (b) superior symmetrical hemispheric RD: break is close to 12:00; (c) in-
ferior quadrantic RD: break is near superior edge; (d) inferior hemispheric detachment:
break is by 6:00; (e) asym metrical inferior RD: break is close to side with higher level; (f)
superior RD crossing 12:00: break is within l½ clock hours from 12:00; (g) RD with a de-
marcation line: break is along meridian bisecting the demarcation. (Adapted from Agarwal
et al. Textbook of Ophthalmology (4 Vols.). New Delhi, India: Jaypee Brothers.)
is close to th e upper m argin or along th e m eridian bisect ing th e region of th e RD

for an in ferior quadran t ic RD, (4) th e prim ar y break is close to 6 o’clock for a sym -
m et rical in ferior h em isph eric RD, (5) th e break is close to th e u pper m argin for an
asym m et rical in ferior h em isph eric RD, (6) th e respon sible break is u su ally foun d
w ith in 12:00 to 2:00 m eridian s in cases of a com plete RD, (7) th e ret in al break is
along th e m eridian bisect ing th e dem arcat ing zon e for an RD w ith a dem arcat ion
lin e. Deviat ion s from th ese ru les can be expected in case of ch orioret in al scarring
an d t ract ion or reoperat ion s.
Color-Coded Diagram
A color-coded ch ar t w ith specific sym bols represen t ing variou s ret in al lesion s an d
t reat m en t m odalit ies allow s detailed docu m en tat ion of th e path ology of th e RD
an d it s m an agem en t in an organ ized m an n er. Figure 10.13 dem on st rates su ch a
ch ar t frequ en tly used along w ith scleral bu ckling tech n iqu es.
Fig. 10.13 A color-coded fundus chart with specific symbols allows the docum enta-
tion of the relevant retinal lesions associated with a retinal detachm ent, and the detailed
steps of the associated scleral buckling procedure. (Adapted from Agarwal et al. Textbook of
Ophthalmology (4 Vols.). New Delhi, India: Jaypee Brothers.)
Fig. 10.14 The parallax associated

with a bullous retinal detachment
tends to cause the m istaken localiza-
tion of the retinal break to be m ore
posterior than it should be. A com-
pensatory anterior adjustment dur-
ing the localization is necessary.
Parallax Associated w ith View ing

Paralla x is a frequen tly en coun tered ph en om en on w h en view ing an RD w ith bul-
lous subret in al flu id, w h ereby th e associated ret in al break appears m ore posterior
th an it s act u al locat ion . Th u s a com pen sator y an terior sh ift ing is n eeded to accu -
rately localize th e ret in al break ( Fig. 10.14 ).
Shafting during Localization

Figure 10.15 sh ow s th e ph en om en on of sh aft ing th at m istakes th e locat ion of th e
com pressing diath erm y sh aft on th e globe for th e t ip of th e probe. To avoid th is er-
ror, th e t ip of th e diath erm y probe is placed on th e extern al locat ion of th e ret in al
break first an d th en arch ed for w ard to p reven t exten sive con tact of th e sh aft of th e
probe w ith th e globe du ring localizat ion of th e ret in al break.
Exoplant Material
Soft an d h ard silicon e exoplan ts h ave w ith stood th e test of t im e to be du rable an d
safe bu ckling m aterial for su ppor t ing ret in al breaks in th e repair of an RD. Figure
10.16 sh ow ed details of soft silicon e sp onges an d h ard silicon e r ubber. Com m on ly
used silicon e sponges in clu de 3, 5, an d 7.5 m m diam eter sponges. Sponges m ay
also be t rim m ed to a precise dim en sion to cu stom ize th em for specific cases. Th ere
are n um erou s st yles of h ard silicon e ru bber w ith variable diam eters. More com -
m on st yles of solid silicon e im plan ts in clu de 20, 40, 41, 42, 220, 240, 276, 277, an d
287 ban ds or t ires. A silicon e t ire h as a groove along it s long axis to accom m odate
a silicon e ban d. Th e groove m ay be cen t rally located as in 20, 210, an d 277 t ires.
An off-cen tered groove (e.g., 276 t ire), allow s a m ore posterior bu ckling effect . Th e
m ajorit y of silicon e ru bbers are design ed for circu m feren t ial placem en t , w h ereas
m ost silicon e sponges are design ed for radial placem en t . How ever, circu m feren t ial
Fig. 10.15 (A) Mistaking the shaft for the tip of the diathermy probe may result in too
posterior a placem ent of the localizing diathermy mark. (B) This error may be avoided
with an anterior arching of the distal end of the probe, and indenting with only the tip
of the probe.
Fig. 10.16 Solid silicone is well tolerated by the eye and is the most commonly used
material for an implant or explant during scleral buckling in the modem era. (A) It is
available in (A) the form of soft sponges, or (B) solid rubber. A rubber silicone tire has
a groove along its long axis to accommodate an encircling band. (C) The groove may
be centrally located as in a 277 tire, or (D) off-centered as in a 276 tire associated with
a m ore posterior buckling effect. (E) A meridional silicone wedge allows the radial sup-
port of a retinal break.
sponges an d radial silicon e ru bbers (e.g., silicon e w edges) are available but u sed
less frequ en tly. A silicon e sponge w ith a h ollow in terior to accom m odate in sert ion
of a circum feren t ial ban d allow s a h igh er bu ckling effect correspon ding to th e loca-
t ion of it s placem en t (e.g., 507T).
Proper Suturing Technique

Spat u lated n eedles are u sed for an ch oring su t ures on th e sclera. To avoid in adver-
ten t scleral perforat ion , th e angle of th e n eedle m ust be relat ively parallel to th e
plan e of th e sclera ( Fig. 10.17 ).
Fig. 10.17 The angle of the spatulated needle m ust be parallel to the scleral surface
to avoid scleral perforation.
Fig. 10.18 A distance of 6 mm is required bet ween the t wo circumferentially placed

sutures to anchor a solid silicone rubber implant of 4 mm width and 1 mm thickness (1
mm + 4 mm + 1 mm = 6 mm).
Calculating the Distance of Suture Placement for Silicone Exoplant

In gen eral, th e cross-sect ion al circu m feren t ial dim en sion of th e silicon e exoplan t
is calcu lated to est im ate th e distan ce of su t ure placem en t , as sh ow n by th e follow -
ing exam ples:
Circum feren t ial im plan tat ion of solid silicon e ru bber w ith a 4-m m w idth an d
1 m m th ickn ess requ ires a sut u re distan ce of 6 m m (1 m m + 4 m m + 1 m m = 6
m m ) ( Fig. 10.18 ).
Th e size of a tear n eeds to be w ith in 4 m m to fit on th e an terior slope of a cir-
cum feren t ial bu ckle created by a 5-m m sponge (π x D/4 = 3.1416 × 5/4 = 3.927)
( Fig. 10.19 ).
Th e an ch oring of th e m at t ress su t u re corresp on ding to th e m eridian (s) of th e
ret in al break(s) m axim izes th e h eigh t of th e buckle for proper su pport of th e
break(s). Th e sut u re n eedle is placed at 1 to 2 m m from th e m argin s of th e sili-
con e ru bber ( Fig. 10.20 ).
Fig. 10.19 To fit on the anterior slope of the buckle created by a circumferentially
placed 5-mm sponge, the size of the tear needs to be within 4 mm (π × D/4 = 3.1416 ×
5/4 = 3.927).
Fig. 10.20 The anchoring suture is placed at 1 to 2 mm from the margins of the cir-
cumferential solid silicone implant corresponding to the m eridian of the retinal break to
maximize the support of the break.
Fig. 10.21 When placing a radial sponge for a large tear, the size of the sponge should
be 1 to 2 mm wider than the tear (e.g., 5-mm sponge for a 3-mm tear). The horizontal
distance bet ween the t wo radial bites of the double -armed suture should be 2 to 3
mm beyond the width of the sponge (e.g., 7 to 8 mm for a 5-mm sponge). The lat ter
distance equals the half circumference of the sponge and can be calculated with the
formula: Distance = ½ circumference = πD/2 = (3.416)(5)/2 = 7.85 mm. (Courtesy of Retina
Consultants Ltd., St. Louis, MO.)
Radial placem en t of a 5-m m sponge requires a su t ure distan ce of 7.85 or 8.0

m m . In gen eral, th e h orizon tal distan ce bet w een th e radial bites of th e double-
arm ed su t u re sh ould be 2 to 3 m m beyon d th e w idth of th e sponge (e.g., 7 to
8 m m for a 5-m m sponge). Th e precise calcu lat ion is h alf circu m feren ce of th e
radial sponge [½ (π diam eter) or 3.1416 × 5/2 = 7.85 or 8.0 m m ] ( Fig. 10.21 ).
For proper sup por t of a ret in al tear w ith a radial sponge, th e m at t ress sut ures
n eed to be placed from 1 to 2 m m an terior to th e an terior h orn s of th e tear an d
3 to 4 m m posterior to th e apex of th e tear. To ach ieve p roper su t u re ten sion , th e
assist an t h olds th e su t ure kn ot after th e surgeon m akes th e first double th row
of th e sut ure kn ot ( Fig. 10.22 ).
For a large ret in al tear, a 12-m m sponge or t w o 5-m m sponges are placed side
by side. Th e proper h orizon tal dist an ce bet w een th e t w o radial su t ure bites in
th e lat ter sit u at ion is calcu lated w ith th e follow ing form ula: 7.85 m m + 5 m m
– com pression factor = 11.5 m m ( Fig. 10.23 ).
Fig. 10.22 For a radial sponge to support a large tear, the anterior mat tress suture
should start at 2 mm anterior to the anterior horn of the tear, whereas the posterior
suture should extend 3 to 4 mm posterior to the apex of the tear. Achieving the proper
tension on the suture may also require the assistant to hold the suture knot after the
surgeon makes the first double throw of the suture knot.
Fig. 10.23 For a very large tear, a 12-mm sponge or t wo 5-m m sponges may be placed
side-by-side. The distance bet ween the t wo radial bites of the mat tress suture for t wo 5-
mm sponges placed side-by-side can be calculated with the following formulas: distance
across 2 sponges = 7.85 m m + 5 mm = 12.85 mm; 12.85 mm – compression factor =
11.5 m m. (Courtesy of Retina Consultants Ltd., St. Louis, MO)
Fig. 10.24 If a large staphyloma is present along the course of an encircling band, it
can be covered by a wider silicone groove-piece or tire under the band, which is then
sutured on the thicker sclera outside of the staphyloma. (Courtesy of Retina Consultants Ltd.,
St. Louis, MO.)
Anterior Shifting of Suture Tying for a Posterior Buckle

To ease t ying of th e sut u re kn ot for a radial bu ckle, th e su t u re kn ot can be sh ifted
from th e p osterior to th e an terior por t ion of th e m at t ress su t u re for t ying (Fig.
10.24 ).
Drainage of Subretinal Fluid

Drain age of su bret in al flu id can be accom plish ed (1) via a radial scleral in cision or
(2) un der a p ar t ial-th ickn ess t riangular-sh aped scleral flap . Th e scleral cut-dow n
is m ade by a n o. 64 or 69 Beaver blade. Diath erm y m ay be used to ach ieve h em o-
Fig. 10.25 A radial scleral drainage site is made with a 3- to 5-mm incision with a no. 64
or 69 Beaver blade. A 5–0 suture may be preplaced on the scleral edges and the edges
may be diathermized. A tapered suture needle, a sharp diathermy electrode, a 27- or 30-
gauge needle may be used for the transchoroidal penetration during drainage.
Fig. 10.26 A triangular scleral flap may also be made for a drainage site. A 5–0 nonab-
sorbable suture is preplaced on the apex of the scleral flap and the corresponding corner
of the scleral bed. A minimal radial cut-down through the rem aining thin scleral fibers of
the drainage bed allows a quick exposure of the choroid for drainage.
stasis. Th e ch oroid is p en et rated w ith a tapered n eedle (e.g., 6–0 silk C-1 n eedle),
sh arp diath erm y t ip, 27- or 30-gauge n eedle, or sh arp kn ife. Th e altern at ive is th e
use of laser delivered by an en dolaser probe or a laser in direct oph th alm oscope
( Figs. 10.25 an d 10.26 ).
Cot ton applicators are used to gen tly com press th e sclera adjacen t to th e drain -
age site (especially an terior to it) to en h an ce drain age of su bret in al flu id. Th e ap-
pearan ce of sm all pigm en t clum ps in th e exit ing flu id in dicates th e elim in at ion of
m ost of th e su bret in al fluid. In direct oph th alm oscopy is perform ed to in spect th e
com pleten ess of th e fluid drain age an d detect any com plicat ion s associated w ith
th e drain age site ( Fig. 10.27 ).
Fig. 10.27 Gentle traction of the

edges of the sclerotomy and mild
indentation of the sclera with a cot-
ton-tipped applicator allow further
drainage of the residual subretinal
fluid.
Conclusion
Adh eren ce to th e foregoing proper tech n iqu es for scleral buckling en h an ces th e
ch an ce of su ccessful repair of a ret in al det ach m en t . Despite th e in creasing popu -
larit y of altern at ive su rgical procedu res, scleral buckling rem ain s a reliable su rgical
tech n ique for repairing m ost prim ar y an d cert ain recurren t ret in al detach m en ts.
It s vit al role in ach ieving favorable an atom ical an d visu al outcom e for rep airing
ret in al detach m en t persist s in th e m odern era of vit reoret in al su rger y.
Choroidal Detachment
Serous Choroidal Detachment

Int raoperat ive or postoperat ive : Woun d leak, perforat ion of th e sclera from a su-
perior rect us, bridle su t u re, irit is, cyclodialysis cleft , leakage or excess filt rat ion
from a filtering bleb, or after laser ph otocoagu lat ion or cr yoth erapy
Traum at ic: Often associated w ith a rupt ured globe, rh egm atogen ous ret in al de-
tach m en t , or after scleral bu ckling repair or a detach m en t
Presentation
Pat ien ts presen t w ith decreased vision or are asym ptom at ic. Th ere m ay be m oder-
ate to severe pain . Decreased vision m ay occur if th e ch oroidal det ach m en t s (CDs)
are tou ch ing (“kissing ch oroidals”) or h em orrh agic. Sm ooth , bu llou s, orange-
brow n elevat ion of th e ret in a an d ch oroid usually exten ds 360 degrees aroun d th e
periph er y in a lobu lar con figu rat ion . Low in t raocular pressu re (IOP) (often less
th an 6 m m Hg), sh allow an terior ch am ber w ith m ild cell an d flare, an d posit ive
t ran sillu m in at ion s are associated fin dings.
Melan om a of th e ciliar y body an d RRD
Management
Check history : Gon ioscopy, fu n du s exam in at ion of both eyes, B-scan , an d ab-
sen ce of t ran sillu m in at ion con firm th e diagn osis. Cycloplegic, topical steroid,
an d surgical drain age of th e su prach oroidal flu id are th e t reat m en t opt ion s. Re-
pair th e un derlying problem .
W ound leak or leak y filtering bleb : Patch for 24 h ou rs, su t u re th e site, u se cya-
n oacr ylate glue, p lace a ban dage con tact len s on th e eye, or a com bin at ion of
th ese.
Cyclodialysis cleft : Laser th erapy, diath erm y, cr yoth erapy, or su t ure th e cleft to
close it .
Uveit is: Topical cycloplegic an d steroid as discu ssed previou sly
Inflam m atory disease : See th e specific en t it y.
Ret inal detachm ent : Surgical repair. Proliferat ive vit reoret in opathy after repair
is com m on .
Hemorrhagic or Expulsive Choroidal Detachment

In t raoperat ive or postoperat ive (from an terior disp lacem en t of th e ocular con ten ts
an d rupt u re of th e sh or t posterior ciliar y ar teries) is th e m ost com m on cau se.
Presentation
Sym ptom s are th e sam e as for serous ch oroidal detach m en t . Pain an d red eye m ay
be p resen t m ore often . High IOP (if detach m en t is large), sh allow an terior ch am ber
w ith m ild cell an d flare, an d absen ce of t ran sillum in at ion are feat u res in w h ich
h em orrh agic CD differs from serous CD.
Melan om a of th e ciliar y body an d RRD
Management
Ch eck the histor y. Confirm ation of th e diagnosis depen ds on h istor y, gon ioscopy,
fundus exam in ation , B-scan , and absen ce of transillum in ation .
An an terior vit rectom y an d drain age of th e ch oroidal det ach m en t is perform ed
for severe cases w ith ret in a or vit reous to th e w ou n d. Oth er w ise u se gen eral
t reat m en t .
Macular Hole
A m acular h ole is a roun d break involving th e layers of th e ret in a, eith er in a par-

t ial- or fu ll-th ickn ess m an n er, in th e m acu lar region . Th e lesion is fou n d m ore
com m on ly in w om en an d is predom in an t in th e fifth to seven th decades (Fig.
10.28 an d 10.29 ).
A B
Fig. 10.28 (A) Color fundus photograph showing a well-circumscribed full-thickness

macular hole with minimal surrounding fluid cuff. (B) Fundus fluorescein angiography of
the sam e patient showing a fading fluorescence in the late phase indicating the window
defect, the most com mon FFA finding in macular holes.
A B
Fig. 10.29 Optical coherence tomography showing (A) a full-thickness and (B) lamel-
lar macular hole.
Presentation
Slit-lam p biom icroscopy of a full-th ickn ess m acu lar h ole sh ow s a w ell-circum -
scribed pu n ch ed-ou t lesion th at predom in an tly involves th e fovea. A par t ial-th ick-
n ess m acu lar h ole can be an ou ter lam ellar h ole or an in n er lam ellar h ole, depen d-
ing on th e et iology beh in d it . It can be classified as follow s:
Fu ll-th ickn ess m acular h ole
Outer lam ellar h ole
In n er lam ellar h ole
Gass Classifica tion for Stages of Idiopa thic Macula r Hole
Stage 1A: Im pen ding h ole, foveal det ach m en t w ith yellow spot
Stage 1B: Im pen ding h ole, foveal det ach m en t w ith yellow h alo
Stage 2 : Full-th ickn ess h ole (cen t ral or eccen t ric) form at ion w ith ou t vit reofo-
veal detach m en t or a PVD
Stage 3 : Full-thickness hole w ith focal vitreofoveal detachm ent but absence of a PVD
Stage 4 : Fu ll-th ickn ess h ole w ith a PVD
Various t ypes of m acu lar h oles su ch as lam ellar m acular h ole, pseudoh ole, m yopic
m acu lar h ole, m icroh ole an d m acular h ole associated w ith epiret in al m em bran e
an d ret in al det ach m en t s, an d pseu dom acular h ole
Management
Observation
Th ere are few st udies describing stage 0 m acular hole, w h ich is a n orm al an d h ealthy
retinal m orphology w ith altered vitreoretinal in terface. Stage 0 m acular h ole is a
clinically silen t finding detected on optical coheren ce tom ography w here a para-
foveal posterior hyaloid separat ion is presen t an d a m in im ally reflective preretin al
band is obliquely inserted at one end of th e fovea. A sim ilar fin ding w h en foun d in
both th e eyes is associated w ith a sixfold rise in th e incidence of m acular h ole. Th us
such cases m ust be follow ed closely an d such patients m ust be coun seled.
Surgica l Management
Vit rectom y is th e m ain stay in th e m an agem en t of m acular h oles. Surger y for th e
t reat m en t of m acu lar h ole revolves aroun d t w o prin ciples: (1) to relieve all vit reo-
ret in al t ract ion by m et iculous rem oval of p osterior cor t ical vit reou s an d (2) to use
a tam pon ade to close th e h ole ( Fig. 10.30 ).
Fig. 10.30 “Apple peeling” technique for removal of the internal limiting mem brane
(ILM). (1A) The ILM is stained with indocyanine green (ICG) stain. The ILM is grasped with
the ILM forceps 500–700 µm above or below the fovea, and a thin strip is peeled radially
(arrow) almost to the fovea and released. (1B) This shows the extent of the initial peeled
flap of ILM. (1C) The exposed edge is then grasped at its midpoint and a parafoveal strip
of ILM is started with a circumferential movement (arrow) around the fovea. (2A) This
parafoveal circumferential rhexis is continued (arrow), releasing and regrasping as neces-
sary. (2B) The rhexis halfway around the fovea. (3A) The rhexis approaching a full circle
around the fovea as an out ward force vector (arrow) is then intentionally applied so that
the ILM strip expands out wardly in a continuous fashion. (3B) Regrasping as necessary,
this m aneuver is continued (arrow) until the macular ILM has been removed in a single
strip. (3C) The single -piece ILM strip ready for rem oval from the eye, avoiding the need
for multiple forceps removals and reinsertions. (4) A conceptual view of the microfor-
ceps holding the single-piece removed ILM strip as removed from the retina. Note the
unstained area of the retina from which this ILM strip was removed. Light is provided by
an endofiberoptic and infusion via a separate infusion port. (Courtesy of Highlights of Oph-
thalmology, “Retinal and Vitreoretinal Surgery: Mastering the Latest Techniques” English Edition, 2002.
Editor-in-Chief: Benjam in F. Boyd, MD, FACS; Co-Editor: Sam uel Boyd, MD.)
Idiopathic Epiretinal Membrane
Iw an off w as th e first to describe idiopath ic epiret in in 1865. It is com m on ly fou n d

in older age an d h as been called by variou s n am es:
Macu lar p ucker
Preret in al m acu lar fibrosis
Epiret in al fibrosis
Ep iret in al gliosis
Celloph an e m acu lopathy
Surface w rin kling ret in opathy
Most of th ese epiret in al m em bran es (ERMs) are idiopath ic an d are called pri-
m ar y epiret in al m em bran es. W h en associated w ith som e oth er ocu lar disorders,
th ey are called secon dar y ERM ( Fig. 10.31 ).
Presentation
Pat ien ts com plain of slow progressive loss of vision , m ild to severe m et am orph o-
psia, an d m on ocu lar diplopia. Com plain ts u su ally progress over years. Th ey m ay
give som e associated past or t reat m en t h istor y revealing th e cause for th e ERM.
Clin ical ch aracterist ics var y w ith th e grade of th e ERM. An early ERM presen ts
as an altered n orm al m acular text u re w ith a glistering sh in e on th e surface. It
is difficult to detect on oph th alm oscopic exam in at ion . A lit tle m ore con t ract ile
m em bran e presen ts as fin e ret in al st riae radiat ing from th e cen ter of th e ERM.
Fig. 10.31 (A) Fundus picture of

patient showing gray-white epi-
m acular membrane. Picture shows
distortion of macular retinal vessels.
(B) fundus fluorescein angiography
B (FFA) of the same patient.
Th e t ypical appearan ce of th e blood vessels h elps in th e diagn osis. Dilated ret in al

vein s an d vascular tor t uosit y w ith teth ering an d st raigh ten ing of th e vessels are
seen directed to th e cen ter of th e con t ract ing m em bran e. Foveal ectopia is a com -
m on fin ding. A w ide range of oth er associated fin dings are n oted, such as pun ct ate
h em orrh ages, m icroan eu r ysm s, profoun d vascu lar leakage an d ret in al edem a, soft
exu dates, an d ret in al tear. Macu lar pucker u sually develops after ret in al det ach -
m en ts. Th ey are opaqu e th ick m em bran es w ith associated fu ll-th ickn ess ret in al
folds.
Prim ar y cau ses of ERM: idiopath ic. Secon dar y cau ses of ERM: postocular in flam -
m at ion , uveit is, ret in al det ach m en t , ret in al vascu lar occlu sion s, m acu lar h oles,
post in t raocular su rgeries, post ret in al laser, cr yopexy.
Management
Vit rectom y is th e on ly t reat m en t for epiret in al m em bran es. After com plet ing th e
vit rectom y, posterior hyaloid th at is adh eren t to th e ret in a is rem oved. In t ravit real
t riam cin olon e can be u sed to facilit ate th is step. Th en th e m em bran e is peeled off
th e su rface of th e ret in a, p referably from th e cen ter to th e p eriph er y after fin ding
an edge of th e m em bran e. Recen tly in t ravit real stain s h ave been described. Tr ypan
blue an d in docyan in e green are used to stain th e epiret in al m em bran e an d in ter-
n al lim it ing m em bran e, resp ect ively.
11 Ocular Neoplasms
Soosan Jacob , Santosh Hanovar, and Am ar Agarw al
Iris Tumors and Nodules
Iris Nevus
Iris n evu s presen t s as a sm all (3 × 0.5m m ), w ell-defin ed spot on th e iris. It is com -
m on ly located in th e st rom a an d presen ts eith er as a t ypical lesion (i.e., w ell-de-
fin ed) or as a diffu se lesion . Th e lesion h as to be differen t iated from oth er pig-
m en ted lesion s. Cogan -Reese syn drom e is th e com m on associat ion .
Presentation
Susp icion for m align an t ch ange is h igh w h en th e n evu s in creases in size an d
ch anges color, in creases in vascularit y, rise in in t raocu lar pressu re (IOP), an d th ere
is pu pil peaking, ect ropion uveae, an d iris splin t ing. Histopath ological exam in a-
t ion of th e n evu s sh ow s proliferat ion of m elan ocytes, predom in an tly of spin dle
cells, th ough occasion ally den drit ic an d balloon cells m ay occu r.
Iris freckle, iris m elan om a, Lisch n odu les, ocular m elan ocytosis
Management
Man agem en t con sist s of reassu ran ce of th e pat ien t regarding th e ben ign n at u re of
th e lesion an d sim ple obser vat ion for m align an t ch ange after carefu l ph otography
an d clin ical docu m en tat ion .
Iris Pigment Epithelial Cyst

Iris pigm en t epith elial cyst is rare an d arises from th e epith elium bet w een layers
of pigm en t epith elium . It presen t s differen tly depen ding on w h eth er it is located
in th e epith eliu m or in th e st rom a.
Presentation
W h en it is epith elial in locat ion it can presen t as u n ilateral or bilateral as a solit ar y
or m u lt iple lesion s. It is usually asym ptom at ic an d obscures vision on ly w h en large.
It is a globu lar lesion located at th e papillar y border or th e m idzon e or th e iris root .
It is brow n w h en iris epith elium is involved or t ran sparen t w h en th e iridociliar y
epith eliu m is involved. It can get dislodged an d float in th e an terior ch am ber.
Lesion s located in th e st rom a are usu ally sm ooth , u n ilateral, solit ar y t ran slucen t
lesion s presen t ing in th e first year of life. W h en th ey in crease in size th ey lead to
secon dar y glaucom a, w h ich in t urn leads to corn eal decom pen sat ion an d pseudo-
hypopyon .
Iris an d ciliar y body m elan om a
322
11 Ocular Neoplasm s 323
Management
Th e n at ure of th e lesion can be ascert ain ed by eith er fin e-n eedle aspirat ion or
excision biopsy. If th e t u m or is large it can be t reated w ith argon laser ph otocoagu -
lat ion or by inject ing eth an ol in to th e lesion w h en it is recalcit ran t .
Iris Melanoma
Iris t u m ors con st it u te 8% of uveal t u m ors presen t ing in th e younger age grou p
(40 to 50 years) w ith a sligh t fem ale predilect ion . Th ey u su ally h ave a bet ter prog-
n osis th an oth er uveal m elan om as.
Presentation
Th e lesion can presen t as t ypical (spot), diffu sely grow ing (diffuse color ch ange),
or t apioca m elan om a (m ult iple su rface n odu les). Lesion s are m ostly asym ptom -
at ic, involving th e in ferior iris m ore com m on ly, bu t can presen t as a rise in IOP,
hyph em a, or cat aract . Risk factor for m align an t ch ange is th e sam e as th at for iris
n evus.
Iridocorn eal en doth elial (ICE) syn drom e, n evus, aden om a an d ciliar y body t u m or,
iris cyst , in t raocular foreign body (IOFB), juven ile xan th ogran u lom a, an d leiom y-
om a
Management
B-scan an d biopsy h elp to con firm th e diagn osis. Biopsy can be fin e n eedle asp ira-
t ion cytology or in cision al biopsy, eith er th rough a corn eal or a lim bal approach .
Th e lesion can be obser ved u n t il sym ptom at ic or over t m align an t ch anges occu r
for w h ich excision (iridectom y or iridocyclectom y), proton -beam radioth erapy, or
rarely en ucleat ion h ave to be u n der taken .
Th e progn osis is usu ally good w h en th e lesion is sm all w ith out ciliar y body or
ext rascleral exten sion .
Juvenile Xanthogranuloma
Juven ile xan th ogran u lom a is a rare idiopath ic gran ulom atou s disease of ch ildh ood
du e to proliferat ion of n on -Langerh an s h ist iocytes. It can presen t eith er as cu t an e-
ous lesion s or iris lesion s. Iris in filt rat ion is th e m ost com m on ocu lar m an ifesta-
t ion . It m ay also rarely involve th e orbit , eyelids, corn ea, episclera, ciliar y body,
ch oroid, an d opt ic disk.
Presentation
Cu tan eous lesion s presen t as yellow ish papu les w ith spon t an eou s regression . Iris
lesion s are eith er localized or diffu se. Th e pat ien t m ay presen t as h eteroch rom ia
of th e iris. Th e lesion s are usu ally associated w ith spon t an eou s hyph em a. An terior
uveit is an d glau com a are oth er less com m on presen t at ion s.
Ret in oblastom a, sarcoid, leukem ia, foreign body, m eduloepith eliom a
Management
Treat m en t opt ion s in clude local cor t icosteroids, irradiat ion , an d a com bin at ion of
both an d excision .
Leiomyoma (Uveal)
Leiom yom a is an ext rem ely rare ben ign lesion arising from th e sm ooth m uscle.
Th is is ver y sim ilar to am elan ot ic m elan om a, bu t gen erally it is n ot con fin ed to th e
in ferior iris on ly.
Presentation
Pat ien ts t ypically presen t w ith a localized, flat to m ildly elevated, ligh tly pigm en ted
or n onpigm en ted, vascular lesion in th e area of th e iris sph in cter. Th e lesion m ay
also occu r periph erally or in th e an terior ch am ber angle.
Am elan ot ic iris m elan om a, m et astat ic iris lesion s
Management
Fin e-n eedle aspirat ion cytology (FNAC) w ith elect ron m icroscopy an d im m un oh is-
toch em ist r y can diagn ose an d differen t iate th e con dit ion from oth ers. Th ere is n o
kn ow n m et astat ic p oten t ial.
Leukemic Iris Nodules

Leu kem ic iris n odules are m ore com m on in ch ron ic leu kem ias rath er th an in acu te
leukem ia. Iris in filt rat ion can presen t as iris n odules.
Presentation
Iris involvem en t m ay be u n ilateral or bilateral. It is a vascularized lesion . It m ay be
associated w ith an terior uveit is w ith or w ith out hypopyon . In filt rat ion can also
occur in to th e conju n ct iva, orbit , an d opt ic n er ve. Glau com a m ay be p resen t due
to blockage in th e filt rat ion from th e t rabecu lar m esh w ork. Leu kem ic ret in opathy
du e to eith er direct in filt rat ion or secon dar y to an em ia h as to be looked for.
Ret in oblastom a, uveit is, t rau m a
Management
Workup involving an on cologist is p referred. Treat m en t con sists of system ic ch e-
m oth erapy w ith localized radioth erapy. Topical steroids cau se quick bu t tem p o-
rar y resolu t ion . Cen t ral n er vou s system (CNS) evaluat ion is m ust .
Melanocytosis
Ocu lar m elan ocytosis is an un com m on , congen ital, prem align an t con dit ion . Nevu s
of Ot a is th e com m on varian t follow ed by th e lim ited derm al an d ocu lar form s.
Th is is m ore com m on in fem ales an d Asian s.
Presentation
Melan ocytosis presen ts as un ilateral hyperpigm en tat ion of th e face w ith ipsilat-
eral iris hyperch rom ia, iris m elan ocytosis, glau com a (10%), an d lid pigm en t at ion .
Th e con dit ion m ay be associated w ith pigm en tat ion of th e ext raocular skin w ith
ipsilateral uveal t ract an d m en ingeal involvem en t .
St u rge-Weber syn drom e, racial p igm en tat ion
Management
Becau se th e hyperpigm en ted area h as a predisposit ion to develop m align an t m ela-
n om a, regular close follow -u p ever y 6 to 12 m on th s is requ ired. Any suspiciou s
lesion sh ould be w orked up accordingly for m align an t m elan om a.
Brushfield Spots (Dow n Syndrome)

Br ush field spot s are seen in Dow n syn drom e. Th ey involve th e periph eral st rom a,
can be m u lt iple, an d th ey occu r as m u lt iple, pale, ben ign lesion s involving th e pe-
riph eral iris st rom a. Th e lesion s appear as pale lesion s. Th ese can also be seen in
n orm al in dividu als. Sarcoid n odu les, iris n evu s, or freckles m ust be ru led out . No
t reat m en t is required.
Lisch Nodules (Neurofibromatosis)

Lisch n odules are u su ally associated w ith n eurofibrom atosis. Th ey are sm all an d
m u lt iple. Th ey ap pear sim ilar to iris n evi, w h ich are ben ign an d bilateral. Th ey are
presen t in n eurofibrom atosis 1 in all pat ien t s after age 16 years. Th ey con sist of a
collect ion of glial cells an d m elan ocytes. Th ey n eed to be differen t iated from iris
n evus. Clin ical docum en t at ion an d follow -u p are required.
Ciliary Body Tumors
Ciliary Body Melanoma

Ciliar y body m elan om as con st it u te ~12% of all m align an cies an d u sually appear
bet w een th e ages of 50 an d 60 years.
Presentation
Clin ically th ey are asym ptom at ic, alth ough th ey can produce visual sym ptom s
occasion ally. Th ey are seen as ciliar y body m ass w ith sen t in el vessels overlying.
Th ey can exten d on to th e iris or globe. Len s sublu xat ion , angle-closu re glau com a,
spon tan eous hyph em a, secon dar y cat aract , an d an terior uveit is are com m on as-
sociat ion s.
Ciliary body m elanom as are well detected by ultrasound and biopsy using FNAC.
Ciliar y body aden om a, ciliar y body cyst , uveal effusion syn drom e, m eduloepith e-
liom a, leiom yom a, an d m et astat ic lesion s
Management
Th e t u m ors are am en able to excision w h en sm all. Radioth erapy, eith er as brachy-
th erapy or p roton beam th erapy, is an oth er opt ion . En ucleat ion is don e if oth er
m odalit ies are n ot possible. Progn osis is u su ally w orse.
Medulloepithelioma (of the Ciliary Epithelium)

Medulloepith eliom a arises from im m at u re epith elial cells of th e em br yon ic op-
t ic cu p an d are rare, slow -grow ing t u m ors th at presen t in ch ildren you nger th an
10 years of age, w ith n o sexual predilect ion . On e th ird are ben ign an d t w o th irds
are m align an t . Th ey can be eith er teratoid or n on teratoid. Th ey origin ate from
th e n onpigm en ted ciliar y epith eliu m predom in an tly involving th e iris an d ret in a,
th ough oth ers can also be involved. Metast asis is rare.
Presentation
Clin ically pat ien t s can presen t w ith red eye an d decreased vision or w ith an iris
m ass lesion w ith a ch ange of color. Tu m or m ay also be seen at th e opt ic disk. Neo-
vascu lar glaucom a, len s colobom a, or sublu xat ion an d cataract are th e com m on
com plicat ion s.
Persisten t hyperplast ic prim ar y vit reou s (PHPV), pars plan it is, en doph th alm it is,
congen it al glaucom a, ret in oblastom a, m align an t glau com a
Management
Ult rasoun d is useful in th e diagn osis. Sm all, w ell-defin ed lesion s of a ben ign n a-
t u re are t reated by iridocyclectom y, an d en ucleat ion is reser ved for t u m ors n ot
am en able to oth er t reat m en t m odalit ies.
Choroidal Tumors
Choroidal Nevus
Ch oroidal n evus is a ben ign m elan ocyt ic t u m or of th e ch oroid an d is fou n d in 5
to 30% of w h ite person s. Th ese are usually foun d in ciden t ally because th ey are
asym ptom at ic an d are th e com m on est of all in t raocu lar m align an cies.
Presentation
Clin ically th ey are asym ptom at ic but can p resen t w ith a decrease in vision . Lesion s
close to th e fovea can cau se vision loss. Th ey are sm all, gray-brow n , h om ogen eou s
lesion s. Th e lesion m ay be associated w ith lip ofu scin deposit s an d dru sen , or w ith
su bret in al flu id (SRF) is absen t . Th ese lesion s gain im port an ce in th e fact th at th ey
h ave to be differen t iated from m align an t m elan om a. Malign an t t ran sit ion is h er-
Fig. 11.1 Choroidal nevus, fundus

photograph shows a pigmented,
placoid, well-demarcated choroidal
mass with overlying drusen located
temporal to the fovea.
alded by th e presen ce of sym ptom at ic lesion s, w ith in creasing th ickn ess, lesion s
w ith lipofu scin pigm en t an d SRF, an d an in crease in IOP ( Fig. 11.1 ).
Ch oroidal m elan om a, congen it al hyper t rophy of ret in al pigm en t epith eliu m (RPE),
com bin ed h am ar tom a, hyperplasia of RPE, subret in al h em orrh age, ch oroidal h em -
angiom as, ch oroidal osteom a, an d m etast at ic t um or.
Management
Lesion m u st be invest igated w ith fu n dus fluorescein angiograp hy (FFA) an d B-scan
to ru le ou t oth er lesion s, especially m align an t m elan om a. Lesion s sh ould be closely
m on itored. Any ch anges in th e m orph ology of th e lesion s sh ou ld be alarm ing.
Choroidal Melanoma
Ch oroidal m elan om as con st it u te ~80% of uveal m elan om as presen t ing aroun d 50
to 60 years of age. Th ey are classified based on th e size of th e t um or as sm all (<10
m m ), m ediu m (10 to 15 m m ), an d large (>15).
Presentation
Th ey can presen t as a decrease in vision or field loss, or flash es of ligh t m ay be th e
presen t ing feat ures, bu t th ey are oth er w ise asym ptom at ic. Th ey can presen t as
an elevated su b-RPE m ass an d can be am elan ot ic. Lipofu scin deposit s, exu dat ive
ret in al det ach m en t , an d Bruch m em bran e ru pt ure w ith vit reous h em orrh age are
t ypical feat u res of th e lesion . Cert ain t u m ors are diffu se rath er th an being n odular.
Pat ien ts at t im es m ay com plain of blurr y vision , visual-field loss, floaters, ph otop-
sia, an d pain . Oth er associated fin dings can be ret in al detach m en t , angle-closu re
glaucom a, rubeosis iridis, vit reous an d subret in al h em orrh ages, an d len s su blu x-
at ion s ( Fig. 11.2 ).
Ch oroidal n evus, congen ital hyper t rophy of RPE, com bin ed h am ar tom a, hyper-
plasia of RPE, subret in al h em orrh age, ch oroidal h em angiom as, ch oroidal osteom a,
an d m et astat ic t u m or
Fig. 11.2 (A) Choroidal m ela-

noma, dome -shaped choroidal mass
with moderate intrinsic pigmenta-
tion and subretinal fluid (SRF). (B)
Choroidal melanoma, dome-shaped
choroidal m ass with mild intrinsic
pigmentation. (C) Choroidal m ela-
noma: wide -angle colored fundus
photograph shows a dark-pigmented,
dome -shaped choroidal mass with a
C smaller elevated nodule on its surface.
Management
Ch oroidal m elan om as can usu ally be p icked u p by u lt rasoun d as acoust ically h ol-
low, low in tern al reflect ivit y solid t u m ors associated w ith ch oroidal excavat ion .
Ext raglobular exten sion is to be ru led ou t by com pu ted tom ography/m agn et ic
reson an ce im aging (CT/MRI) along w ith a system ic w orkup for m et astasis. FNAC
is con t rain dicated.
Treat m en t guidelin es are given by ocu lar m elan om a st u dy. Tran s-pu pillar y th er-
m oth erapy can be t ried w h en th e lesion is aw ay from th e disk an d th e m acu la an d
w h en th e lesion is pigm en ted. Radioth erapy eith er as plaqu e or as proton beam
irradiat ion . Local resect ion can be used w h en th e t u m or is sm all an d th ere is n o
m etast asis. In advan ced cases orbital exen terat ion is th e opt ion .
Choroidal Metastasis
Ch oroidal m etast asis is th e m ost com m on form of in t raocu lar m align an cy in adu lts,
w ith m ore th an 85% of m etast at ic uveal n eoplasm s being ch oroidal. It usually m e-
tast asizes from breast can cer in w om en an d lung can cer in m en . Less com m on
sites of origin in clude th e prost ate, th e kidn ey, th e thyroid, an d th e gast roin test in al
t ract . Lym ph om as an d leukem ias m ay also m etast asize to th e eye an d th e orbit .
Metastases are usu ally bilateral, m u lt iple lesion s.
Presentation
Most pat ien t s are asym ptom at ic or m ay com plain of flash es, floaters, or m et am or-
ph opsia. Ch oroidal m etastases are u sually seen as solid, flat , plaqu elike, m ot tled,
yellow -brow n lesion s w ith or w ith ou t associated serou s ret in al det ach m en t (Fig.
11.3 ).
Ch oroidal h em angiom a, ch oroidal osteom a, exophyt ic ret in al cap illar y h em an -
giom a, oth er m etast at ic carcin om as, posterior sclerit is, h em orrh agic RPE det ach -
m en t , an d am elan ot ic ch oroidal m elan om a
A B
Fig. 11.3 (A) Choroidal metastasis, tallow pink smooth, dome -shaped juxtapapillary
choroidal mass. (B) Choroidal m etastasis. Fundus fluorescein angiography (FFA) shows
hypofluorescence in the arterial and early venous phases.
Management
A com bin ed approach in con su ltat ion w ith th e m edical on cologist an d radiat ion
th erapist is requ ired. Th ough ch em oth erapy m ay be in dicated in m any cases, ra-
diat ion th erapy in th e form of extern al beam radioth erapy is usually th e m ore
defin it ive t reat m en t . Early t reat m en t offers th e best h ope for preser ving vision .
Surger y is rarely required. If th e prim ar y is n ot readily iden t ifiable, a biopsy m ay
be required prior to t reat m en t .
Cavernous Hemangioma of the Choroid

Cavern ou s h em angiom a is a ben ign vascular h am artom a. Lesion s can be localized
or diffuse.
Presentation
Cavern ou s h em angiom a is congen it al an d asym ptom at ic u n t il adulth ood, at w h ich
t im e it can presen t w ith loss of vision . It can presen t eith er as an isolated, circum -
scribed lesion w ith ou t system ic associat ion or as a diffu se form along w ith oth er
system or ocu lar abn orm alit ies (St urge-Weber syn drom e). Circum scribed lesion s
are seen as an elevated, orange-red, ch oroidal m ass. Th e diffuse form is seen as a
deep red ch oroid, especially in th e posterior pole com pared w ith n orm al. Oth er
feat ures in clu de fibrous ch ange of RPE, tor t u ou s ret in al vessels, cyst ic ch ange, or
serou s det ach m en t of ret in a ( Fig. 11.4 ).
Ch oroidal n evus, congen ital hyper t rophy of RPE, com bin ed h am artom a, hyperpla-
sia of RPE, su bret in al h em orrh age, ch oroidal m elan om a, ch oroidal osteom a, an d
m etast at ic t u m or
Management
Ult rason ography is th e com m on invest igat ion an d sh ow s h igh in tern al reflect iv-
it y. Fu n dus flu orescein angiography (FFA) sh ow s early hyperflu orescen ce due to
in t ra lesion al vessels an d later hyperfluorescen ce of th e w h ole lesion . In docyan in e
green (ICG) h elps in delin eat ing th e lesion bet ter th an FFA. MRI h elps in diffuse
form . Brain an d system ic w orkup to r ule ou t h em angiom a in oth er sites is essen -
t ial. Histopath ology reveals cavern ou s vascular ch an n els (n orm al en doth elial cells
an d su ppor t ing sept a) an d capillaries like vessels in th e diffuse form .
Fig. 11.4 Choroidal hemangiom a,

dome -shaped, juxtapapillary, red-
dish-orange choroidal mass.
Treat m en t opt ion s range from ph otodyn am ic th erapy (PDT), TTT, an d irradia-
t ion . A n eurologist is to be con sulted w h en th ere is CNS involvem en t .
Choroidal Osteoma
Ch oroidal osteom a is foun d m ore com m on ly in w om en in th e secon d or th ird de-
cade of life. It m ay be bilateral in 25% of cases.
Presentation
Ch oroidal osteom a is seen as a w ell-dem arcated, relat ively flat (less th an 2 m m
th ick), yellow -w h ite lesion w ith clu m ps of black or brow n pigm en t on th e su rface,
gen erally n ear th e opt ic n er ve h ead. Th e ch oroidal osteom a im pedes th e circu -
lat ion to th e overlying ret in a an d m ay be associated w ith adjacen t RPE at rophy,
su bret in al n eovascularizat ion , su bret in al fluid, an d su bret in al h em orrh age. For t y
percen t of cases en large on long-term follow -u p. FFA sh ow s early, patchy hyper-
flu orescen ce w ith in ten se late stain ing. Ult rasou n d sh ow s in ten se reflect ivit y
from th e t um or w ith acou st ic sh adow ing of th e posterior orbit al con ten t s. CT scan
sh ow s a bon elike sign al ( Fig. 11.5 ).
Ch oroidal n evus, congen ital hyper t rophy of RPE, com bin ed h am artom a, hyperpla-
sia of RPE, su bret in al h em orrh age, ch oroidal m elan om a, ch oroidal osteom a, an d
m etast at ic t u m or
A
B
Fig. 11.5 (A) Choroidal osteoma: a

well-defined, yellow-orange, placoid jux-
tapapillary choroidal mass with scalloped
margins. (B) Choroidal osteoma: fundus
fluorescein angiography (FFA) shows dif-
fuse hypofluorescence in the arterial phase.
(C) Choroidal osteoma: fluorescence inten-
sifies diffusely in the late phase. C
Management
Man agem en t con sists of periodic obser vat ion for grow th an d su bret in al n eovascu -
larizat ion . Su bret in al n eovascularizat ion m ay be t reated w ith laser.
Retinal Pigment Epithelium Tumors
Congenital Hypertrophy of the Retinal Pigment Epithelium

Congen it al hyper t rophy of th e RPE is gen erally discovered during rout in e eye
screen ing an d h as an associat ion w ith fam ilial aden om atou s polyposis an d Gard-
n er syn drom e (in test in al polyposis, h am ar tom a of th e skeleton , an d m u lt iple soft
t issue t u m ors).
Presentation
Lesion s are gen erally seen in th e perip h er y bu t m ay occasion ally be seen n ear th e
disk. Th ey occu r as rou n d, w ell-circu m scribed, pigm en ted lesion s, eith er solit ar y
or m ult iple w ith flat or scalloped m argin s. Th ey h ave a m ore dist in ct border an d
are darker th an th e ch oroidal n evu s. Th ere m ay be depigm en ted h alos w ith in th e
lesion . Rarely, th ere are m u lt ip le pigm en ted lesion s in th e sam e area (bear-t rack
lesion s) ( Fig. 11.6 ).
Ch orioret in al scarring, ch oroidal m elan om a, ch oroidal n evu s, m elan ocytom as of
th e ch oroids, hyp erplasia of th e RPE, posth em orrh age h em osiderin deposits
Management
Screen ing is perform ed by a gast roen terologist an d is follow ed u p by periodic ocu -
lar screen ing.
Fig. 11.6 Congenital hypertrophy

of the retinal pigm ent epithelium,
fundus photograph of the deeply
pigmented, flat, well-circum scribed
retinal lesion with characteristic halo
and lacunae of depigmentation.
Fig. 11.7 Combined hamartoma of

the retinal pigm ent epithelium and
sensory retina, grayish juxtapapillary
placoid mass with variable pigmen-
tation and intrinsic vascularit y. Note
the altered configuration and drag-
ging of retinal vessels with partial
obscuration by fibroglial tissue.
Combined Hamartoma of the Retinal Pigment Epithelium

and the Retina
Th is rare, ben ign t u m or is form ed by a h am ar tom atou s overgrow th of several con -
st it uen ts su ch as th e RPE, vascu lar elem en t s, an d glial t issu e of th e ret in a to var y-
ing degrees.
Presentation
Pain less visu al loss is th e m ost com m on sym ptom . Ret in al vascu lar tor t uosit y,
hyperpigm en t at ion , elevat ion of th e lesion , an d an epiret in al m em bran e m ay be
fou n d. Macu lar fu n ct ion m ay be affected secon dar y to t ract ion al distort ion of th e
ret in a. Com bin ed h am ar tom as are m ost com m on ly located close to th e opt ic disk
follow ed by th e m acu la an d occu r least com m on ly in th e periph eral ret in a. It h as
been described to occur in n eu rofibrom atosis 1 ( Fig. 11.7 ).
Pigm en ted fu n dus t um ors, ret in oblastom a, ch oroidal m elan om a
Management
Angiography m ay sh ow vascu lar ch anges periph eral to th e lesion referred to as
“p seu doavascularit y.” Vit rectom y m ay be at tem pted in selected cases.
Retinal Tumors
Retinoblastoma
Ret in oblastom a is th e m ost com m on prim ar y ocular m align an cy of ch ildh ood,
w ith an in ciden ce of 1 in 15,000 live bir th s. It is cau sed by m u tat ion in th e long
arm of ch rom osom e 13q14, w h ich codes for th e RB1 gen e, a t u m or su ppressor
gen e. It affects th e p recu rsor cells th at form th e in n er an d outer ret in al cells, lead-
ing to th eir m align an t t ran sform at ion .
Th e m ode of in h eritan ce m ay be h eritable (germ -lin e m ut at ion ) or n on h erit able

(som at ic m ut at ion ). In h eritable m u tat ion on e allele of th e RB1 gen e is m u tated in
all cells of th e body. A “secon d h it” affect s th e secon d allele, leading to m align an t
t ran sform at ion . Th ese pat ien t s are predisposed to n on ocu lar t u m ors such as pin e-
alom a (t rilateral ret in oblastom a), osteosarcom a, an d m align an cies of th e brain an d
lung.
Presentation
In bilateral cases presen t at ion is usu ally arou n d age 1 year an d in un ilateral cases
2 years. Leu kocoria (w h ite pupillar y reflex, cat’s eye reflex) is th e com m on est sign .
St rabism u s, secon dar y glaucom a, proptosis, an d in flam m at ion secon dar y to t u m or
n ecrosis are oth er sign s th at can be seen . It can som et im es m im ic uveit is in ch il-
dren , presen t ing as a m asquerade syn drom e.
Ocu lar exam in at ion by scleral in den t at ion sh ow s a w h ite m ass w ith calcifica-
t ion . It m ay be in t raret in al or an en dophyt ic t um or project ing an d seeding in to th e
vit reous or m ay be exophyt ic w ith a subret in al m ass an d overlying ret in al det ach -
m en t .
Histopath ology sh ow s th e ch aracterist ic Flexn er-Win terstein er roset tes, Hom er
Wrigh t roset tes, an d fleu ret tes.
Management
A th orough system ic an d gen et ic evalu at ion is required. Ult rason ography an d CT
assess t um or size an d calcificat ion . MRI detect s opt ic n er ve, ext raocu lar exten -
sion , an d pin ealoblastom a. Prim ar y en ucleat ion is advised for un ilateral cases
w ith advan ced disease an d large t um ors, longstan ding ret in al detach m en ts, n eo-
vascu lar glau com a, or suspicion of opt ic n er ve invasion /ext rascleral exten sion . It
is also u sed in bilateral cases for th e m ore advan ced eye. Treat m en t m odalit ies
available in clu de ph otocoagu lat ion , ch em oth erapy, t ran sp upillar y th erm oth erapy,
cr yoth erapy for sm all t um ors an d extern al-beam radioth erapy (EBRT), an d p laqu e
brachyth erapy for m edium -sized t u m ors. EBRT m ay be associated w ith develop-
m en t of n on ocular t u m ors in th e irradiated field. Ch em oth erapy m ay be u sed prior
to oth er t reat m en t m odalit ies to decrease th e size of th e t um or or in pat ien ts w ith
system ic m etast asis.
Capillary Hemangioma
Th is is a ben ign h am ar tom a of th e ret in al (or opt ic disk) vasculat ure, con sist ing
of capillar y-like vessels. Th ough m ost com m on ly diagn osed in you ng adult s, it
m ay p resen t in any age. Isolated lesion s are n ot associated w ith any system ic in -
volvem en t , w h ereas von Hippel-Lin dau disease presen ts w ith bilateral/m u lt iple
t u m ors.
Presentation
Pat ien ts can be asym ptom at ic (diagn osed on fam ily screen ing) or m ay p resen t
w ith a decrease in visual acu it y. On exam in at ion it is seen as a red n odular le-
sion w ith tort uosit y an d dilatat ion of th e feeding ar ter y an d drain ing vein w ith or
w ith ou t exudat ion , exudat ive ret in al detach m en t , rubeosis/n eovascular glaucom a,
epiret in al m em bran es, t ract ion al ret in al detach m en t , an d vit reou s h em orrh age
( Fig. 11.8 ).
Fig. 11.8 (A) Retinal capillary hem-

angioma: montage of fundus photo-
graphs shows a well-defined, intense,
orange-red retinal lesion with prom i-
nent feeder and drainage vessels:
(B) Retinal capillary hemangioma,
higher magnification demonstrates
intense vascularit y of the lesion. (C)
Optic disc capillary hemangiom a:
fiery red vascular mass located over
the optic nerve head.
Coat s disease, racem ose h em angiom a, cavern ous h em angiom a, en doph th alm it is,
ret in oblastom a, ast rocytom as, fam ilial exudat ive vit reoret in opathy (FEVR), sickle
cell ret in opathy, p apilledem a, an d papillit is
Management
FFA reveals rapid sequen t ial filling of th e feeder arter y, h em angiom a, an d vein
w ith exten sive late leakage. Leakage in to th e vit reou s m ay m ake late im ages h azy.
System ic disease n eeds m u lt idiscip lin ar y care. Ocu lar diseases can be m an aged
w ith ph otocoagu lat ion (<3 m m ) w ith con fluen t burn s in cluding th e feeder vessels.
Cr yoth erapy, radioth erapy, an d excision are oth er m odalit ies of t reat m en t .
Fig. 11.9 Retinal cavernous hemangioma, fundus

photograph shows a grapelike cluster of blood-filled
secular spaces in the inner retinal layers.
Retinal Cavernous Hemangioma

Th is ben ign h am ar tom a of th e ret in al (or opt ic disk) vascu lat u re con sists of large-
caliber vessels. It is isolated bu t can be bilateral in fam ilial cases.
Presentation
Pat ien ts are u su ally asym ptom at ic or can presen t w ith a decrease in visual acu it y
or floaters. Exam in at ion reveals a cluster of in t raret in al blood-filled saccu les w ith
oth er w ise n orm al vasculat ure w ith or w ith ou t vit reou s h em orrh age (Fig. 11.9 ).
Coat s disease, racem ose h em angiom a, capillar y h em angiom a, en doph th alm it is,
ret in oblastom a, ast rocytom as, FEVR, sickle cell ret in opathy, papilledem a, an d
papillit is
Management
FFA reveals slow filling th at rem ain s hyperfluorescen t th rough ou t w ith out leak-
age. Treat m en t is u sually n on in ter ven t ion al an d is u sually n ot n ecessar y.
Fig. 11.10 Retinal arteriovenous

malformation: fundus photograph
shows abnormally dilated and tortu-
ous retinal vessels with surrounding
retinal edem a, hemorrhage, and fi-
brous proliferation.
Arteriovenous Malformation
Congen it al ret in al ar terioven ous m alform at ion (AVM) (racem ose h em angiom a) is
an an om alous ar ter y to vein an astom osis.
Presentation
AVM can presen t as a sm all, localized vascular com m u n icat ion n ear th e disk, or it
can presen t as a prom in en t t angle of large, tor t u ou s blood vessels th rough out m ost
of th e ret in a. It is u su ally asym ptom at ic or can presen t w ith a decrease in visu al
acuit y. It can presen t as par t of Wybu rn -Mason syn drom e ( Fig. 11.10 ).
Coats disease, racem ose h em angiom a, cap illar y h em angiom a, cavern ou s h em an -
giom a, en doph th alm it is, ret in oblastom a, ast rocytom as, FEVR, sickle cell ret in opa-
thy, papilledem a, an d pap illit is
Management
Th ere is n o effect ive t reat m en t , w h ereas in t racran ial AVM can be t reated by sur-
ger y, radioth erapy, an d em bolizat ion .
Melanocytoma
Melan ocytom a is a ben ign pigm en ted t um or. Opt ic n er ve m elan ocytom a is th ough t
to develop from den drit ic uveal m elan ocytes presen t in th e lam in a cribrosa.
Presentation
Tu m ors m ay grow deep in th e opt ic n er ve p aren chym a, in th e ju xt apapillar y ch o-
roid, or in th e ret in al n er ve fiber layer. Th e m ost com m on posit ion is on or adjacen t
to th e opt ic n er ve h ead. Th ey are gen erally sm all an d black an d m ay grow, but th ey
rarely t ran sform in to a m align an cy ( Fig. 11.11 ).
Ch oroidal m elan om a an d oth er pigm en ted t u m ors
Management
Th ere is n o t reat m en t at presen t to preven t or stop opt ic n er ve m elan ocytom a
grow th . Regular ocular exam in at ion s are don e ever y 6 to 12 m on th s w ith dilated
oph th alm oscopy an d visual-field exam in at ion s. Th e pat ien t sh ould be cou n seled
regarding th e risk of vision loss an d com pression -related vasculopathy an d sh ould
be m on itored for th e rare possibilit y of m align an t t ran sform at ion .
Fig. 11.11 Optic disc melanocy-

tom a: fundus photograph shows a
black lesion with irregular margins
straddling the inferior aspect of the
optic disk with subretinal fluid, exu-
dates, and macular edema.
12 Strabismus
Federico G. Velez , Noa Ela-Dalm an , and Arthur L. Rosenbaum
Pseudostrabismus
Pseudostrabism us is a false appearance of strabism us caused by facial character-

istics including a flat nasal bridge, eccentric position of the nose, w ide epicanthal
folds, w ide interpupilar distance, and a positive or negative kappa angle (Fig. 12.1 ).
Fig. 12.1 (A) Pseudoesotropia. (B)

Angle kappa. B
339
Presentation
In pat ien ts w ith pseu dost rabism us, th e corn eal reflex is cen tered an d sym m et ric
bilaterally, an d th ere is n o m an ifest ocular deviat ion on cover t test ing. Pat ien ts
w ith angle kappa h ave lateral (n egat ive angle kappa) or m edial (posit ive angle
kappa) desen t rat ion of th e corn eal reflex, bu t th ere is n o m an ifest deviat ion on
cover test ing.
Tru e h orizon t al or vert ical deviat ion , orbital an om alies, an d ret in al abn orm alit ies
su ch as m acular dragging in pat ien t s w ith ret in opathy of prem at urit y
Management
Th ere is n o t reat m en t , bu t follow -u p is in dicated.
Infantile Esotropia
In fan t ile esot ropia is a convergen t m isalign m en t of th e visual axes m an ifested by

age 6 m on th s ( Fig. 12.2 ).
Presentation
Th ere is a large-angle esot ropia associated w ith crossed fixat ion , lim it at ion to ab-
du ct ion , laten t nyst agm us, pu rsuit asym m et r y, dissociated ver t ical deviat ion , an d
in ferior oblique overact ion . Am blyopia is rare. Pat ien t s usu ally m an ifest a low de-
gree of hyperop ia.
Fig. 12.2 Infantile esotropia.

12 Strabism us 341
Sixth -n er ve palsy, fibrosis syn drom e, early accom m odat ive esot ropia, Duan e syn -
drom e
Management
Bilateral m edial rect u s m u scle recession is th e in it ial u su al t reat m en t for in fan t ile
esot ropia. Bilateral lateral rect us resect ion is used as a secon d procedu re for u n -
dercorrect ion .
Accommodative Esotropia
Convergen t deviat ion of th e visu al axes is associated w ith act ivat ion of th e ac-
com m odat ive reflex. Th e age of presen tat ion averages 30 m on th s, ranging from 6
m on th s to several years ( Fig. 12.3 ).
Fig. 12.3 (A) Refractive esotropia.

( B) High accommodative conver-
gence esotropia. B
Presentation
Th ere is a m oderate angle of esot ropic deviat ion averaging 20 to 30 prism diop-
ters. Th e con dit ion progresses from in term it ten t to con stan t esot ropia. Th e angle
of deviat ion at n ear an d at dist an ce is u su ally sim ilar u n less th ere is a h igh accom -
m odat ive convergen ce/accom m odat ion (AC/A) rat io w h ere th e n ear angle of devia-
t ion is larger. Th e average hyperopic correct ion is +4.00 diopters. Accom m odat ive
esot ropia is frequ en tly associated w ith am blyopia.
Types
Refract ive accom m odat ive esot ropia : High hyperopia an d n orm al accom m oda-
t ive convergen ce/accom m odat ion . Th e angles of deviat ion at n ear an d at dis-
tan ce are sim ilar.
High accom m odat ive convergence/accom m odat ion esot ropia : Low hyperopia
w ith a h igh accom m odat ive convergen ce/accom m odat ion . Th e angle of devia-
t ion at n ear is larger th an th e dist an ce deviat ion .
Part ially accom m odat ive esot ropia : Com bin at ion of refract ive an d n on accom -
m odat ive esot ropia. Hyperopic correct ion part ially corrects th e angle of devia-
t ion . Associated w ith a delay in correct ion of th e accom m odat ive th ere is an
accom m odat ive com pon en t .
Basic n on refract ive n on accom m odat ive esot ropia, in fan t ile esot ropia, acute eso-
t ropia associated w ith n eu rological disorders, cyclic esot ropia, spasm of th e n ear
syn kin et ic reflex, nyst agm us blockage syn drom e
Management
Man agem en t con sists of fu ll cycloplegic hyp eropic correct ion . Bifocals are pre-
scribed for pat ien ts w ith h igh accom m odat ive convergen ce/accom m odat ion if th e
eyes are st raigh t at distan ce. Su rger y correct s th e n on accom m odat ive or decom -
pen sated com pon en t of th e deviat ion .
Intermittent Exotropia
In term it ten t exot ropia is in term it ten t divergen t deviat ion of th e visu al a xes. It is
th e m ost com m on deviat ion seen in th e pediat ric popu lat ion (Fig. 12.4 ).
Presentation
On set is u su ally before age 5 years. Th e con dit ion is aggravated by fat igu e, sickn ess,
an d daydream ing. Pat ien t s frequen tly squin t to recover align m en t an d elim in ate
diplopia, esp ecially w ith exp osure to brigh t sun ligh t . At th e begin n ing it is usu ally
seen on ly at dist an ce, but it m ay progress to affect th e n ear deviat ion . Am blyopia
is rare. Pat ien t s u su ally m an ifest good stereop sis at n ear. Stereopsis at dist an ce can
decrease as a resu lt of poor con t rol.
12 Strabism us 343
Fig. 12.4 Intermit tent exotropia.
In term it ten t exot ropia can be divided in to differen t t ypes based on th e differ-
en ces bet w een th e n ear an d dist an ce angles of deviat ion :
Basic t ype : Th e n ear an d th e dist an ce angles of deviat ion are w ith in 10 prism
diopters.
Divergence excess: Th e distan ce angle of deviat ion is larger th an th e n ear angle
of deviat ion by 10 or m ore prism diopters. In pat ien ts w ith t ru e divergen ce
excess, th e differen ce rem ain s after m on ocu lar occlusion ; if th e differen ce dis-
appears, th e pat ien t h as sim ulated divergen ce excess.
Convergence insufficiency : Th e angle of exot ropia is 10 prism diopters or larger
at n ear th an at dist an ce.
Convergen ce in sufficien cy, dissociated h orizon tal deviat ion
Management
Correct ion of any refract ive error. Min u s len ses in du ce accom m odat ion . Patch ing
of th e n on deviat ing eye. Su rgical in dicat ion s in clu de deteriorat ion of con t rol an d
stereoacu it y, diplopia, an d visu al con fusion . Su rger y u sually con sists of bilateral
lateral rect us m u scle recession in pat ien t s w ith divergen ce excess, m edial rect u s
m u scle resect ion in convergen ce in su fficien cy, an d m edial rect u s m u scle resect ion
com bin ed w ith lateral rect us m uscle recession in th e basic t ype of in term it ten t
exot ropia.
Fig. 12.5 Constant exotropia.
Constant Exotropia
Con stan t exot ropia is usually associated w ith a decrease in visual acuit y, n eu ro-
logical disorder, disrupt ion of bin ocu lar vision , previou s st rabism us surger y, or
cran iofacial abn orm alit ies ( Fig. 12.5 ).
Presentation
In fan t ile exot ropia is a con st an t exodeviat ion presen t before age 6 m on th s, ch ar-
acterized by a large angle of deviat ion . Th is con dit ion is usually associated w ith
cerebral p alsy, n eu rological abn orm alit ies, or cran iofacial disorders. Sen sor y exo-
t ropia is th e resu lt of disru pt ion of bin ocularit y an d reduced visual acu it y in on e
eye. Con secu t ive or secon dar y exot ropia follow s previou s su rgical correct ion of an
exodeviat ion .
Cran ial th ird n er ve palsy, slipped m edial rect u s m u scle, Duan e syn drom e, deterio-
rat ion of an in term it ten t exot ropia
Management
Alw ays ru le ou t n eu rological disorders, cran iofacial syn drom es, ocular abn or-
m alit ies, or any abn orm alit ies in th e visu al path w ay associated w ith a decrease
in vision . Treat m en t of am blyopia an d correct ion of refract ive errors are requ ired.
Surger y con sist s of un ilateral or bilateral lateral rect us m u scle recession com bin ed
w ith m edial rect us m u scle resect ion , depen ding on th e angle of deviat ion .
Pattern Strabismus
Horizon tal ch ange of align m en t from th e m idlin e w h en th e eyes are m oved be-
t w een a 25-degree u pgaze, prim ar y posit ion an d a 25-degree dow ngaze. Th e in ci-
den ce of pat tern st rabism us is ~20%. Tw o prin ciples h ave been advan ced to exp lain
th e cause of th e A an d V pat tern s: (1) oblique m uscle dysfu n ct ion or (2) rect us
ext raocular m uscle overact ion or w eakn ess w ith ou t obliqu e m u scle dysfu n ct ion .
12 Strabism us 345
Fig. 12.6 (A) V pat tern. (B) A pat tern.
Presentation
An A pat tern is p resen t w h en th e eyes diverge 10 or m ore prism diopters bet w een
dow ngaze an d u pgaze. A V pat tern is presen t w h en th e eyes converge 15 or m ore
prism diopters bet w een u pgaze an d dow ngaze. Pat ien ts w ith pat tern st rabism u s
m ay presen t w ith an om alou s h ead post ure, ocular torsion , an d overact ion or un -
deract ion of th e obliqu e m u scles ( Fig. 12.6 ).
Cran iofacial abn orm alit ies, cyclovert ical m u scle w eakn ess or overact ion , h orizon -
tal rect u s m uscle overact ion , p seu do A an d V pat tern s in pat ien t s w ith accom m o-
dat ive esot rop ia, h eterotopic or un stable rect u s ext raocu lar m uscles
Management
Ten don offset t ing su rger y of th e h orizon t al rect u s m u scles is an effect ive operat ion
for collapsing A an d V pat tern st rabism us n ot associated w ith oblique m uscle dys-
fun ct ion w ith appropriate in dicat ion s. Th e m edial rect us m u scle is alw ays t ran s-
posed tow ard th e apex of th e pat tern (up for A pat tern an d dow n for V pat tern ).
Th e lateral rect us m u scle is t ran sposed in th e opposite direct ion . In th e presen ce
of obliqu e m uscle dysfun ct ion , appropriate obliqu e m uscle w eaken ing procedures
are in dicated.
Inferior Oblique Overaction
Un ilateral or bilateral over-elevat ion of th e addu cted eye is obser ved. Most cases
of in ferior obliqu e overact ion are prim ar y an d n ot associated w ith oth er m uscle
w eakn ess. Secon dar y in ferior obliqu e overact ion result s from w eakn ess of th e ip-
Fig. 12.7 Inferior oblique overaction.
silateral su perior oblique m u scle (an tagon ist m u scle) or th e con t ralateral su perior
rect us m u scle (yoke m uscle). Approxim ately 65% of pat ien t s w ith in fan t ile esot ro-
pia w ill develop in ferior oblique overact ion ( Fig. 12.7 ).
Presentation
Th e prim ar y an d secon dar y overreact ion s of th e in ferior obliqu e m u scles h ave dif-
feren t clin ical presen t at ion s. In prim ar y in ferior obliqu e overact ion , on set is usu -
ally after 1 year of age. It h as th e ten den cy to be bilateral an d sym m et ric. It is
frequ en tly associated w ith a V pat tern . Usu ally, th ere is n eith er ver t ical deviat ion
in th e prim ar y posit ion n or excyclotorsion , an d th e Bielsch ow sky h ead t ilt test is
n egat ive. In con t rast , secon dar y in ferior obliqu e m uscle overact ion is associated
w ith vert ical deviat ion in th e p rim ar y p osit ion an d excyclodeviat ion an d posit ive
Bielch ow sky h ead-t ilt test .
Secon dar y cau ses of in ferior obliqu e overact ion , h eterotopic an d u n st able rect us
ext raocu lar m uscles, orbital excyclotorsion , cran iosyn ostosis
Management
Th e t reat m en t of an overact ing in ferior obliqu e m u scle is a w eaken ing procedu re.
In ferior oblique recession is th e preferred surgical procedure.
Dissociated Strabismus Complex
Dissociated st rabism u s com plex is an in term it ten t or m an ifest deviat ion of th e

n on fixing eye. It is a dissociated m ovem en t th at violates th e Hering law ; as op-
posed to t rue hypert ropia, n o com pen sator y m ovem en t of th e fellow eye is seen
w h en th e fixing eye is covered. It m ay be ch aracterized by on e or m ore of th ese
com pon en ts: ver t ical, h orizon t al, an d torsion al ( Fig. 12.8 ).
12 Strabism us 347
Fig. 12.8 Dissociated vertical deviation.
Presentation
Th e m ost com m on clin ical presen t at ion is hyperdeviat ion , exodeviat ion , an d ex-
cyclodeviat ion of th e n on fixing eye. Th e deviat ion m ay be com itan t or in com itan t ,
especially if associated w ith oblique m u scle dysfu n ct ion . Su ppression an d visual
con fusion are com m on sen sorial abn orm alit ies. Oth er clin ical m an ifestat ion s m ay
in clu de nyst agm us, tor t icollis, an d oblique m uscle dysfun ct ion .
Obliqu e m uscle dysfun ct ion
Management
Su rgical in dicat ion s in clu de decom pen sat ion , in crease in m agn it ude an d fre-
qu en cy, an d tor t icollis. Bilateral surger y is alw ays recom m en ded u n less th ere is
st rong eviden ce th at th e pat ien t w ill n ever p refer fixat ion w ith th e n on fixing eye.
Large su perior rect us m uscle recession is th e procedu re of ch oice in pat ien ts w ith
com it an t deviat ion s. Obliqu e m uscle su rger y is in dicated in pat ien ts w ith in com i-
tan t deviat ion an d overact ing obliqu e m u scles.
Third-Nerve Palsy
Th ird-n er ve palsy con sists of isolated, m u lt iple, or com plete palsy or paresis of th e
st ru ct u res in n er vated by th e th ird cran ial n er ve
Presentation (Fig. 12.9)

Com plete th ird-n er ve palsy is ch aracterized by exot ropia, hypot ropia, an d ptosis
on th e side of th e affected n er ve. Th e clin ical presen t at ion of th e su perior th ird-
n er ve-division palsy is hypot ropia an d ptosis of th e side ipsilateral to th e affected
cran ial n er ve. In ferior th ird-n er ve-division p alsy presen t s w ith exot ropia, hyper-
t ropia, an d pu pillar y dilat ion . Th ird-n er ve palsy can presen t as an isolated palsy of
th e m edial rect u s m uscle, in ferior rect u s m uscle, sup erior rect us m u scle, in ferior
obliqu e m uscle, levator m uscle, an d p upillar y sph in cter.
C
Fig. 12.9 (A) Complete third-nerve palsy. (B) Superior branch palsy. (C) Isolated m e-
dial rectus palsy.
12 Strabism us 349
Th ird-n er ve palsy w ith pup illar y involvem en t can be a clin ical em ergen cy.
Workup is required to ru le ou t sup raten torial m ass or basilar an eur ysm . In cases
w ith ou t pupillar y involvem en t on e is required to rule ou t isch em ia, com pression ,
in flam m ator y process, n eu ropathy, an d m yon europathy.
In pat ien t s w ith congen it al th ird-n er ve palsy an d th ose w ith palsy secon dar y
to lesion s in th e cavern ous sin us, an eu r ysm s, m en ingiom a, an d t raum a can de-
velop aberran t in n er vat ion to th e ciliar y ganglia, levator m u scle, an d ext raocu lar
m uscles.
Rest rict ion , Graves oph th alm opathy, orbit al fract ure, fibrosis of th e ext raocular
m u scles, m yotoxicit y, m yasth en ia, ch ron ic progressive extern al oph th alm oplegia,
congen it al absen ce of th e ext raocu lar m u scles
Management
Cases secon dar y to diabetes, hyper ten sion , or m igrain e u su ally com pletely resolve
in 1 to 12 w eeks. Non su rgical t reat m en t in clu des occlusion , prism s, an d bot u lin u m
toxin . Su rgical t reat m en t depen ds on th e deviat ion in th e prim ar y posit ion an d
m u scle fun ct ion . Weaken ing of th e ip silateral lateral rect u s m uscle is n ecessar y in
cases of com plete th ird-n er ve palsy. Su rgical t reat m en t is ch allenging in cases w ith
com plete th ird-n er ve palsy or w h en several ext raocu lar m u scles are affected.
Fourth-Nerve Palsy (Superior Oblique Palsy)
Fou rth -n er ve palsy is a congen it al or acquired paralysis of th e su perior obliqu e

m u scle. Congen it al cases m ay be secon dar y to abn orm alit ies of th e ten don , in -
cluding laxit y, absen ce, or an om alous in sert ion . Acqu ired cau ses in clude t raum a,
com pression , isch em ia, in filt rat ion , an d h em orrh age. Tum ors sh ou ld alw ays be
su spected in p at ien ts w ith acqu ired bilateral su perior obliqu e palsy ( Fig. 12.10 ).
Fig. 12.10 (A) Unilateral superior oblique palsy. (Continued on page 350)
Fig. 12.10 (Continued) (B) Bilateral superior oblique palsy.
Presentation
Un ilateral su perior obliqu e palsy is ch aracterized by hypert ropia in addu ct ion ,
posit ive h ead-t ilt test , V p at tern , an d excyclot ropia. Oth er clin ical sign s in clu de
posit ive th ree-step test , large fusion al am plit udes, an d facial asym m et r y w ith
hypoplasia con t ralateral to th e side of th e palsy. Bilateral palsies are ch aracterized
by reverse hyp ert rop ia, posit ive h ead t ilt test , V pat tern , an d excyclot ropia larger
th an 10 degrees. Pat ien t s w ith sym m et rical bilateral su perior obliqu e palsy usu -
ally h ave lit tle or n o ver t ical deviat ion in th e prim ar y posit ion .
Skew deviat ion , Graves oph th alm opathy, an d prim ar y in ferior oblique overact ion
Management
Many pat ien ts are able to com pen sate for th e ver t ical deviat ion , especially th ose
w ith long-stan ding su perior obliqu e palsy. Surgical t reat m en t depen ds on th e
am ou n t of deviat ion in th e prim ar y posit ion , th e size of deviat ion in th e vert ical
plan e (upgaze an d dow ngaze), an d th e ipsilateral gaze (field of act ion aw ay from
th e affected superior oblique m uscle) an d degree of dysfun ct ion of th e sup erior
obliqu e m u scle. Pat ien t s w ith less th an 15 prism diopters of ver t ical deviat ion in
th e prim ar y posit ion can be t reated w ith su rger y on on e m u scle. In pat ien t s w ith
sign ifican t excyclotorsion , su perior oblique m uscle t ucking or an terior lateraliza-
t ion of th e an terior fibers of th e superior oblique ten don m ay be h elpfu l.
Sixth-Nerve Palsy (Lateral Rectus Muscle Palsy)
Sixth -n er ve palsy result s in paralysis of th e lateral rect us m u scle.
Presentation
In com it an t esot ropia is larger in th e abdu cted field of act ion of th e affected lateral
rect us m u scle. Et iology in cludes t rau m a, com pression , an d in fect ion . New born s
m ay presen t w ith a tem p orar y paralysis ( Fig. 12.11 ).
12 Strabism us 351
Fig. 12.11 Sixth-nerve palsy.
Duan e syn drom e, fibrosis of th e ext raocular m u scles, Möbiu s syn drom e, in flam -
m ator y disorders, m yasth en ia gravis, in fan t ile esot ropia, h orizon tal gaze palsy,
divergen ce palsy, an d previou s ext raocu lar m uscle surger y
Management
Obser ve for th e first 6 m on th s. Com plete resolu t ion is seen in up to 90% of cases
w ith in th e first 6 m on th s after on set . Cases secon dar y to m igrain e an d vascular le-
sion h ave th e ten den cy to recu r. Pediat ric pat ien t s w ith a persisten t p alsy beyon d
3 m on th s an d adult cases in w h om th e angle of deviat ion in creases an d th ose w h o
develop n eu rological deficits requ ire reevalu at ion . Ru le ou t gian t cell ar th rit is in
pat ien t s older th an 50 years.
Non su rgical t reat m en t in clu des occlusion , t reat m en t of am blyopia, prism , an d
bot ulin um toxin
Surgical approach depen ds on th e follow ing variables: residual fun ct ion of th e
lateral rect u s m uscle, ocu lar rot at ion s, saccades, forced duct ion an d forced gen era-
t ion test , deviat ion in p rim ar y posit ion , an om alous h ead post ure. Lateral rect us
m u scle resect ion in p at ien ts w ith residual lateral rect u s m u scle fu n ct ion an d ver-
t ical rect u s m uscle t ran sposit ion in pat ien ts w ith com plete lateral rect us m u scle
palsy are th e procedu res of ch oice in th ese pat ien ts. Many cases require ipsilateral
m edial rect us m u scle recession .
Duane Syndrome
Duan e syn drom e involves m isin n er vat ion of th e lateral rect us m u scle associated
w ith absen ce or hypoplasia of th e sixth cran ial n er ve. Most frequen tly fibers of th e
th ird cran ial n er ve going to th e m edial rect us m u scle are redirected to th e lateral
rect us m u scle. Th e lack of n orm al in n er vat ion from th e sixth n er ve to th e lateral
rect us m uscle resu lts in lim itat ion of abduct ion . Th e m isin n er vat ion of th e lateral
rect us m u scle w ith fibers from th e th ird cran ial n er ve result s in abn orm al con t rac-
t ion of th e lateral rect u s m uscle in at tem pted adduct ion ( Fig. 12.12 ).
Presentation
Duan e syn drom e occu rs m ore frequen tly in fem ales th an in m ales, an d it m ost
com m on ly involves th e left eye. Th e m ajorit y of pat ien t s w ith Duan e syn drom e
h ave st raigh t eyes. Bilateral involvem en t is seen in 20%of th e cases. Th e m ost ch ar-
acterist ic clin ical presen tat ion s are u n ilateral lim itat ion of abdu ct ion associated
w ith globe ret ract ion an d n arrow ing of th e palpebral fissure in at tem pted adduc-
Fig. 12.12 (A) Duane t ype 1. (B) Duane t ype 2. (C) Duane t ype 3. (D) Bilateral
Duane.
12 Strabism us 353
t ion . Oth er m an ifest at ion s in clude an om alou s h ead post ure, lim it at ion to addu c-
t ion , st rabism u s in th e prim ar y posit ion , an d an om alous vert ical m ovem en t s.
Classically th ree t ypes of Duan e syn drom e h ave been described. Type 1 is ch ar-
acterized by esot ropia in th e prim ar y posit ion w ith lim itat ion to abdu ct ion , globe
ret ract ion , an d palpebral fissu re n arrow ing in addu ct ion . Type II is ch aracterized
by exot ropia in th e prim ar y posit ion an d lim it at ion to adduct ion . Type III is ch arac-
terized by exot ropia or esot ropia w ith lim it at ion to abdu ct ion an d addu ct ion .
Duan e syn drom e m ay be associated w ith system ic m an ifest at ion s, in clu ding
h earing loss, Klippel-Feil syn drom e, Golden h ar syn drom e, Holt- Oram syn drom e,
at rial sept al defect , h an d an om alies, crocodile tears, an d cleft palate.
Sixth -n er ve palsy, in fan t ile esot ropia, fibrosis of th e ext raocu lar m u scles, congen i-
tal m isin n er vat ion syn drom es
Management
In dicat ion s for su rger y in clude st rabism u s in th e prim ar y posit ion , diplopia, an d
an om alous h ead post ure. Th e su rgical approach is based on th e align m en t in th e
prim ar y posit ion , lim itat ion to ocular rotat ion s, severit y of ret ract ion , an d th e
presen ce of an om alou s vert ical m ovem en ts. In gen eral, m edial rect us recession
an d ver t ical rect us m u scle t ran sposit ion s are th e procedu res of ch oice in pat ien ts
w ith esot rop ia in th e prim ar y p osit ion . Lateral rect u s m uscle recession is th e pro-
cedu re of ch oice in pat ien t s w ith exot ropia in th e prim ar y p osit ion . Y split t ing of
th e lateral rect u s m u scle m ay be added in pat ien ts w ith u p/dow n sh oot .
Brow n Syndrome
Brow n syn drom e is a congen ital or acquired lim itat ion to elevat ion in adduct ion .
Presentation
Th e p at ien t’s in abilit y to elevate th e eye in addu ct ion is w orse th an in th e m idlin e
or abdu ct ion . Th ere is a V pat tern , m in im al superior oblique overact ion , w iden ing
of th e palpebral fissure in addu ct ion , overdepression in addu ct ion (“dow n sh oot”
of th e affected eye in adduct ion ), an om alous h ead post u re, an d hypot ropia of th e
affected eye. Th e in abilit y to elevate th e eye in addu ct ion on forced du ct ion test ing
is con firm ator y of Brow n syn drom e. Pat ien ts w ith acquired Brow n syn drom e m ay
presen t w ith a t roch lear ten dern ess an d click ( Fig. 12.13 ).
Fig. 12.13 Brown syndrome.

Mon ocular elevat ion deficien cy, fibrosis of th e ext raocular m u scles, in ferior
obliqu e m u scle palsy, orbital floor fract ure, thyroid op h th alm opathy, an d h etero-
topic or dyn am ic displacem en t of th e rect u s m uscles
Management
Acquired Brow n syn drom e is frequen tly in flam m ator y. Spon tan eous resolut ion is
com m on . Medical t reat m en t in clu des local an d system ic cor t icosteroids. In dica-
t ion s for su rgical correct ion in clu de disrupt ion of th e bin ocu lar vision in a con -
gen it al Brow n syn drom e, an om alou s h ead post ure, hypot ropia in prim ar y posi-
t ion , diplopia, an d dow n sh oot in adduct ion . Ipsilateral superior obliqu e m u scle
w eaken ing is th e procedure of ch oice in Brow n syn drom e.
Monocular Elevation Deficiency
Mon ocular elevat ion deficien cy is a su pran u clear or in fran u clear lim it at ion to el-
evat ion frequ en tly associated w ith ptosis or pseu doptosis ( Fig. 12.14 ).
Fig. 12.14 (A) Monocular elevation deficiency. ( B) Supranuclear palsy.

12 Strabism us 355
Presentation
Lim itat ion to elevat ion in th e prim ar y posit ion an d lateral gaze is greater in u p-
gaze. Ptosis or pseudoptosis is p resen t in ~50% of th e cases. Pu pillar y abn orm ali-
t ies are u su ally seen in acqu ired cases. An an om alou s ch in elevat ion h ead post u re
is frequ en tly seen . Congen it al cau ses in clude supran uclear abn orm alit ies, su perior
rect us m u scle w eakn ess, an d in ferior rect u s m uscle rest rict ion . Acquired m on ocu -
lar elevat ion deficien cy m ay result from cen t ral n er vou s system t um or, in flam m a-
t ion , in fect ion , or st roke.
Brow n syn drom e, Du an e syn drom e, fibrosis of th e ext raocular m uscles, th ird-
n er ve palsy, Graves disease, m yasth en ia gravis, orbit al floor fract ure, m yosit is,
m yotoxicit y, cerebellar t u m ors
Management
Man agem en t con sist s of t reat m en t of am blyopia. Th e goal of surger y is align m en t
in th e prim ar y posit ion an d im provem en t of elevat ion . Th e surgical approach is
based on th e presen ce of rest rict ion , w eakn ess, or a com bin at ion of both factors
an d in clu des in ferior rect u s recession , superior rect u s m u scle resect ion , or h ori-
zon tal rect us m u scles t ran sposit ion .
Graves Ophthalmopathy
Proptosis, eyelid retraction, and extraocular m uscle m otility disorders associated w ith
hyperthyroidism or inflam m atory disease of the thyroid gland are characteristic of
Graves ophthalm opathy. Many patients are euthyroid at presentation (Fig. 12.15 ).
Presentation
Pat ien ts p resen t w ith proptosis, conjun ct ival inject ion , ch em osis an d lid edem a,
eyelid ret ract ion , an d lid lag on dow ngaze. Graves op h th alm opathy occurs m ore
frequ en tly in w om en (3:1), w ith t w o peaks, on e at age 40 an d on e at age 60. Re-
st rict ion to ocu lar m ovem en ts m ore com m on ly affects th e in ferior rect us m u scle,
Fig. 12.15 Graves disease.

follow ed by th e m edial rect us m u scle, superior rect u s m uscle, lateral rect u s m us-
cle, an d th e obliqu e m uscles. Orbit al im aging dem on st rates th e t ypical ext raocu lar
m u scle en largem en t sparing th e ten don s at th e m u scle in ser t ion .
Orbit al in flam m ator y disease, m yosit is, lym p h om a, sarcoidosis, am yloidosis, or-
bital t u m ors, vascu lar lesion s, h igh m yopia
Management
Treat m en t of th e hyper thyroidism . System ic steroids, im m un osuppressan ts, an d
radiat ion th erapy are u sed during th e acute ph ase of th e disease. St rabism us sur-
ger y sh ou ld be delayed u n t il th e in flam m ator y ph ase is resolved an d th e st rabis-
m u s is stable. Th e prim ar y su rgical approach in clu des recession of th e ext raocular
m u scle involved.
Myasthenia Gravis
Th is is an au toim m un e disorder, m ore com m on in w om en , ch aracterized by w eak-

n ess an d fat igabilit y of st riated m uscles ( Fig. 12.16 ).
Fig. 12.16 (A) Myasthenia gravis. (B) Myasthenia gravis post ice -pack test.
12 Strabism us 357
Presentation
Myasth en ia gravis can be congen it al, juven ile, or adu lt on set . Ocu lar m yasth en ia
is localized to th e ext raocular m uscles. System ic m yasth en ia involves oth er skel-
et al an d bulbar m uscu lat u re. Th e m ost com m on ocu lar m an ifest at ion is ptosis fol-
low ed by u n deract ion of th e ext raocu lar m uscles. Ph arm acological test ing w ith
an t ich olin esterases is th e stan dard diagn ost ic test for m yasth en ia.
Ch ron ic extern al oph th alm oplegia, m edicat ion s in terfering w ith n eu rom uscu lar
t ran sm ission , cen t ral n er vous system t um ors
Management
Man agem en t in cludes thym ectom y, oral an t ich olin esterases, steroids, prism , bot-
ulin um toxin , an d st rabism us su rger y.
Congenital Fibrosis of the Extraocular Muscles
Ptosis an d rest ricted lim it at ion of ocu lar rot at ion s secon dar y to m yopath ic an d
n eurogen ic abn orm alit ies lead to fibrosis of th e ext raocu lar m uscles. Th e con di-
t ion m ay in clu de on e or m u lt iple ext raocular m u scles. Gen e m ut at ion s h ave been
iden t ified for CFEOM t ype 1 (KIF21A gen e), CFEOM t ype 2 ( PHOX2A), an d CFEOM
t yp e 3 ( FEOM3 ) ( Fig. 12.17 ).
Presentation
Presen t at ion in clu des un ilateral or bilateral bleph aroptosis, fibrosis of th e ext ra-
ocu lar m u scles, ch in -u p post ure, lim it at ion to elevat ion an d h orizon tal ocular ro-
tat ion s, an d absen ce of Bell ph en om en on . Th e con dit ion is som et im es associated
w ith opt ic n er ve an d ret in al abn orm alit ies.
Fig. 12.17 Fibrosis syndrome.

Mon ocular elevat ion deficien cy, th ird-n er ve p alsy, Brow n syn drom e, ch ron ic pro-
gressive extern al oph th alm oplegia, Möbiu s syn drom e, m yasth en ia gravis, orbital
fract u re, at ypical Duan e syn drom e
Management
Man agem en t con sists of t reat m en t of am blyopia an d lu brican t s to avoid corn eal
dr yn ess du e to poor Bell’s ph en om en on an d eyelid closu re. St rabism us surger y
con sists of large recession of involved m u scle.
13 Neuro-Ophthalmology
Cranial Nerve Palsies
Isolated Oculomotor (Third) Nerve Palsy

Th e com m on causes of oculom otor n er ve palsy in clude n eoplasm s, t raum a, an eu -
r ysm s, isch em ic lesion s, an d oph th alm oplegic m igrain e. Ocu lom otor n er ve palsy
cou ld be congen it al du e to a h eredit ar y cause. Th e m ode of t ran sm ission cou ld be
eith er autosom al dom in an t or recessive.
Presentation
Total th ird-n er ve paresis m ay be cen t ral, sparing th e pupil, or periph eral w ith pu -
pillar y involvem en t . If th e pup il is spared, th e m ost likely cause is a vascular lesion .
If th e pu pil is involved, it is m ost likely du e to an an eu r ysm . Th e pat ien t h as a large
exot ropia w ith hypot ropia. A fixed, dilated pu pil is seen . On at tem pted addu ct ion ,
th e eye in tor ts as th e su perior obliqu e w ou ld be n orm al. On e can also get p ar t ial
paresis as th e th ird n er ve divides in to superior an d in ferior division . If th e su pe-
rior division of th e th ird n er ve is involved, gen erally oth er cran ial n er ves are also
involved. On e can get an isolated involvem en t of th e in ferior division of th e th ird
cran ial n er ve ( Figs. 13.1A,B,C).
Nuclea r Third-Nerve Pa resis
Th is is ext rem ely rare. Follow ing are th e im por t an t poin ts about th is lesion :
Each superior rect u s is in n er vated by th e con t ralateral th ird-n er ve n u cleu s; if
th ere w as n u clear th ird-n er ve palsy on on e side th en th ere w ould be a paresis
of th e con t ralateral su perior rect u s.
Both levator palpebrae su perioris are in n er vated by on e subn uclear st ruct u re,
th e cen t ral caudal n u cleu s. Th erefore, n u clear th ird-n er ve palsy leads to bilat-
eral ptosis.
Third-Nerve Fascicle Syndromes
In th ese cases th e th ird n er ve h as already left th e n u cleu s, so th e lesion s affect
on ly on e side. Th ere are various syn drom es th at can occur, depen ding on th e site
of lesion . Th ey are due gen erally to an isch em ic, in filt rat ive (t u m or) or rarely to an
in flam m ator y lesion .
Nothnagel Syndrome
In th is case th e lesion is in th e area of th e su perior cerebellar pedun cle. As th e le-
sion involves th e superior cerebellar pedu n cle th e pat ien t h as an ipsilateral th ird-
n er ve paresis w ith cerebellar at axia.
Benedikt Syndrome
In Ben edikt syn drom e th e lesion is in th e area of th e red n ucleus. Th is leads to
con t ralateral h em it rem or w ith ipsilateral th ird-n er ve paresis.
Cla ude Syndrome
Th is syn drom e h as feat u res of both Noth n agel an d Ben edikt syn drom es.
359
B
13 Neuro-Ophtham ology 361
Fig. 13.1 (A) Oculom otor nerve

nuclei. (B) Oculom otor nerve anat-
omy. (C) Syndromes of the oculo-
motor nerve. (D) Hutchinson pupil.
(E) Posterior communicating artery
aneurysm.
E
Weber Syndrome
In Weber syn drom e th e lesion is in th e area of th e cor t icospin al (pyram idal) t ract .
Th is leads to an ipsilateral th ird-n er ve paresis w ith con t ralateral h em iparesis.
Uncal Hernia tion Syndrome
As th e th ird cran ial n er ve goes tow ard th e cavern ous sin u s, it rests on th e edge of
th e ten torium cerebelli. A supraten torial space-occu pying m ass located anyw h ere
in or above th is cerebral h em isph ere m ay cau se a dow nw ard disp lacem en t an d
h ern iat ion of th e u n cus across th e ten torial edge, th ereby com pressing th e th ird
n er ve. Th is leads to a dilated an d fixed pu pil. Th is is called th e Hutch in son pu pil
an d is th e first in dicat ion th at altered con sciousn ess is du e to a sp ace-occupying
in t racran ial lesion ( Fig. 13.1D).
Posterior Communicating Artery Aneurysm
As th e th ird cran ial n er ve m oves tow ard th e cavern ou s sin us, it t ravels alongside
th e posterior com m un icat ing ar ter y. If th ere is an an eur ysm of th e posterior com -
m u n icat ing ar ter y it can lead to com p ression of th e th ird n er ve. Th is leads to an
isolated th ird-n er ve paresis w ith th e pu pil becom ing involved ( Fig. 13.1E).
Cavernous Sinus Syndrome
In cavern ou s sin us syn drom e, th ere w ould be th ird-n er ve paresis w ith involve-
m en t of oth er n er ves, such as cran ial n er ves IV, V, an d VI. Th ese p at ien ts h ave pain -
ful oph th alm oplegia, possibly du e to t raum a, n eoplasm s, an eu r ysm s, or in flam m a-
t ion s. Th is syn drom e can lead to aberran t regen erat ion of th e th ird cran ial n er ve.
Orbita l Syndrome
Proptosis can be an early sign . Th e fifth cran ial n er ve can also be involved, but th is
w ou ld involve on ly th e oph th alm ic division .
Pupil-Spa ring Isola ted Third-Nerve Pa resis
Th e pu pillom otor fibers t ravel in th e th ird n er ve in th e outer layers an d are th ere-
fore closer to th e n u t rien t blood su pply enveloping th e n er ve. Th is is th e reason
w hy th e pu pillom otor fibers are gen erally spared in isch em ic th ird-n er ve paresis
but are affected in com pressive lesion s su ch as t u m ors. Ocu lar m yasth en ia can
m im ic a pu pil-sparing th ird-n er ve palsy, so on e can perform th e Ten silon test to
differen t iate th e t w o.
Oth er causes of paralyt ic an d rest rict ive squ in t s
Management
Occlusion
On e can occlu de th e paret ic eye un t il th e h ealing occurs an d th e th ird-n er ve pa-
resis is cu red.
Medica l Trea tment
On e can give th e pat ien t m ult ivitam in inject ion s an d t ablet s an d t reat th e cause,
like diabetes or hyperten sion .
Surgica l Trea tment
Th e su rgical m an agem en t of a com plete th ird-n er ve paralysis is a difficult job. At
th e ver y best , th e surgeon w ill succeed on ly in m oving th e paret ic eye in to th e
prim ar y posit ion w ith ou t restoring adduct ion , elevat ion , or depression to a sig-
n ifican t degree. A ver y good m eth od to t reat th is con dit ion is to do a ten otom y of
th e lateral rect us an d th e su perior obliqu e com bin ed w ith a t ran sposit ion of th e
ver t ical rect i m uscles to th e in sert ion of th e m edial rect us m uscle. Even th ough th e
t reated eye w ill con t in u e to be im m obile, it w ill at least be cen tered, an d th is op-
erat ion sh ould be con sidered, especially in pat ien t s w h o fixate w ith th e paralyzed
eye. For th e ptosis on e sh ou ld perform a fron t alis m u scle sling operat ion . Th is can
be don e as a secon d step.
If th e pat ien t h as a part ial palsy w ith sligh t m edial rect us m ovem en t on e can
perform a m axim al recession of th e lateral rect us m u scle (at least 12 m m ) an d re-
sect ion of th e m edial rect u s (at least 7 m m ) w ith upw ard t ran sposit ion of th e ten -
don s in case of an associated hypot ropia. Th is m ay restore a sm all bu t usefu l field
of vision even th ough dou ble vision w ill persist in u pw ard an d dow nw ard gaze.
Isolated Trochlear (Fourth) Nerve Palsy

Th e t roch lear n er ve (fou rth cran ial n er ve) is th e th in n est an d also h as th e longest
in t racran ial cou rse (~75 m m ). Th is is th e on ly cran ial n er ve th at em erges from
th e dorsal aspect of th e brain stem . It is also th e on ly cran ial n er ve th at crosses
com pletely to th e op posite side. In oth er w ords, th e t roch lear n er ve arises from
th e con t ralateral n u cleu s.
Presentation
Depen ding on th e level of th e lesion , variou s syn drom es can occur as a result of
dam age to th e t roch lear n er ve. Th ey are as follow s ( Figs. 13.2A,B):
Nuclea r Fascicula r Syndrome
It is difficu lt to dist ingu ish bet w een n uclear an d fascicular lesion s because of th e
sh or t course of th e fascicles w ith in th e m idbrain . Th e n uclear fascicular syn drom e
cou ld be du e to h em orrh age t raum a or dem yelin at ion . It is seen w ith con t ralateral
Horn er syn drom e becau se th e sym path et ic path w ays descen d th rough th e dorso-
lateral tegm en t um of th e m idbrain adjacen t to th e t roch lear fascicles.
Suba rachnoid Spa ce Syndrome
As th e fou r th n er ve em erges from th e dorsal su rface of th e brain stem , it can be
inju red easily. W h en bilateral four th -n er ve palsies occu r, th e site of inju r y is likely
to be in th e an terior m edullar y velu m . Con t racou p forces t ran sm it ted to th e brain -
stem by th e free ten torial edge m ay injure th e n er ves at th is site. Oth er cau ses
cou ld be t um ors, such as pin ealom a or ten torial m en ingiom as.
If th e lesion is in th e cavern ous sin us, oth er cran ial n er ves in close associat ion w ith
th e four th cran ial n er ve can also be involved.
Orbita l Syndrome
In orbit al syn drom e oth er cran ial n er ves close to th e fou rth cran ial n er ve are also
involved. Oth er orbit al sign s su ch as proptosis, ch em osis, an d conju n ct ival injec-
t ion are also seen . Th is cou ld be due to t raum a, in flam m at ion , or t u m ors.
Isola ted Fourth-Nerve Pa lsy
Isolated fou rth -n er ve palsy could be du e to a congen ital cause or it could be ac-
qu ired. Th e feat u res of a four th -n er ve palsy are as follow s:
Hyperdeviat ion : Th e involved eye is h igh er as a result of th e w eakn ess of th e su -
perior obliqu e m uscle. On e sh ould perform th e Bielsch ow sky h ead-t ilt ing test
becau se w h en th e h ead is t ilted tow ard th e ipsilateral sh ou lder th e hyperdevia-
t ion becom es m ore obviou s.
B
Fig. 13.2 (A) Trochlear nerve anatomy. (B) Lesions of the trochlear nerve. (C) Biel-
schowsky’s head tilting test for R/L (right/left) hypertropia. (D) Bielschowsky’s head
tilting test for L/R (left/right) hypertropia. LIO, left inferior oblique; LSR, left superior
rectus; RIR, right superior rectus; RSO, right superior oblique.
Ocular m ovem ents: Depression is lim ited in adduct ion . In torsion is also lim ited.
Hom onym ou s vert ical diplopia occu rs on looking dow nw ard. Usually th e vision
is single as long as th e eyes look above th e h orizon t al plan e. Th e pat ien t espe-
cially n ot ices diplopia w h en w alking dow n th e st airs.
Abnorm al head post ure : To avoid diplopia, th e h ead t akes an abn orm al post u re
tow ard th e act ion of th e superior oblique m u scle (i.e., th e face is sligh tly t u rn ed
to th e opposite side, th e ch in is depressed, an d th e h ead is t ilted tow ard th e op-
posite sh oulder).
Checking Fourth-Nerve Function in the Set ting of a Third-Nerve Pa resis
Th e problem w ith ch ecking th e four th cran ial n er ve fu n ct ion if a pat ien t also h as
a th ird cran ial n er ve paresis is th at th e involved eye can n ot be adducted w ell be-
cause of th ird cran ial n er ve involvem en t . Because th e eye can n ot be adducted, on e
can n ot test th e ver t ical act ion (depression ) of th e su perior obliqu e m u scle.
To solve th is problem , first of all w e sh ould n ote a lim bal or conjun ct ival lan d-
m ark, such as a blood vessel or pter ygium . Th e pat ien t , on being asked to look
dow n , w ill n ot be able to do so as th e eye is abdu cted an d n ot adducted (becau se
of th ird-n er ve involvem en t). Bu t th e eye w ill in tor t as th e su perior oblique m u scle
w orks. We sh ould th en ch eck from th e conjun ct ival lan dm ark if th e eye is in tor t-
ing. If th e conju n ct ival lan dm ark is m oving, th e eye is in tor t ing, an d th at m ean s th e
fou r th n er ve is in t act .
Bielschowsky Hea d-Tilting Test
Th e Bielsch ow sky h ead-t ilt ing test can diagn ose w h ich m uscle is paralyzed. Let us
first look at a case of R/L hypert ropia in w h ich th e righ t eye is at a h igh er posit ion
th an th e left eye ( Figs. 13.2C,D).
R/L Hypertropia
If th e pat ien t h as an R/L hyper t ropia, th en it could m ean th at th e righ t eye is hy-
pert ropic, in w h ich case th e depressors are paralyzed like th e righ t in ferior rect u s
(RIR) or th e righ t superior oblique (RSO). It cou ld also m ean th at th e righ t eye is in
th e n orm al posit ion but th e left eye is hypot ropic. Th is could be du e to th e eleva-
tors of th e left eye being paralyzed like th e left in ferior obliqu e (LIO) an d th e left
su perior rect u s (LSR). Th is is th e first step of th e test . We h ave th u s n arrow ed dow n
th e diagn osis to fou r of th e ext raocu lar m u scles.
Now w e perform th e secon d step of th e test . In th is w e ask th e pat ien t to per-
form dext roversion or levoversion . Th is m ean s w e ask th e pat ien t to look to th e
righ t an d to th e left . If w e ask th e p at ien t to look to th e righ t , th e righ t eye cou ld
be h igh er th an th e left eye. If th e righ t eye is h igh er on dext roversion , th en it could
m ean th at th e RIR is involved or it cou ld m ean th at th e left eye is hypot ropic. Th is
w ou ld be due to an LIO paralysis. In levoversion if th e righ t eye is h igh er, it could be
du e to an RSO paralysis. Altern ately, it could m ean th at th e left eye is hyp ot ropic,
an d th is w ou ld be due to LSR paralysis. Th us w e h ave n arrow ed dow n th e m uscles
from fou r to t w o.
Fin ally, w e perform th e th ird step in w h ich w e t ilt th e pat ien t’s h ead to th e righ t
an d th en to th e left . If w e t ilt th e h ead to th e righ t , th e righ t eye w ill in tor t an d
th e left eye w ill extor t . Th is is becau se n er vou s im pu lses w ill be sen t from th e
sem icircu lar can als to keep th e eyes in a st raigh t posit ion . Rem em ber, the superiors
are intorters. So if th e righ t eye in tort s, it m ean s th e superiors in th at eye (RSR an d
RSO) w ork, an d if th e left eye extort s it m ean s th e in feriors of th at eye (LIO an d LIR)
w ork. W h en th is h appen s in th e righ t eye th e RIR w ill n ot be u sed at all because
it is an extor ter, an d in th e righ t eye extor t ion is n ot t aking place. But in th e left
eye, extor t ion w ill take place an d th e LIO an d LIR w ill w ork. Now as th e LIO is
paralyzed, on ly th e LIR act s in th at eye. As a balan ce is n ot be m ain t ain ed bet w een
th ese t w o m u scles, th e left eye m oves dow n as th e LIR is also a depressor. Th us, on e
can diagn ose a pat ien t w ith LIO paralysis.
If w e ask th e pat ien t to t ilt th e h ead to th e left , th e left eye w ill in tor t an d th e
righ t eye w ill extort . In th e righ t eye th e extor ters w ill be th e RIR an d righ t in ferior
obliqu e (RIO). Now th e RIR is paralyzed, an d so on ly th e RIO w ill w ork an d th e
righ t eye w ill m ove u pw ard.
Sim ilarly, w e can differen t iate bet w een th e RSO an d th e LSR in th e th ird step. If
w e t ilt th e h ead to th e righ t th e righ t eye w ill in tor t , an d th e m uscles th at w ill w ork
are th e RSO an d RSR. Becau se th e RSO is paralyzed on ly th e RSR w ill w ork, an d th e
righ t eye w ill m ove u pw ard.
If w e t ilt th e h ead to th e left , th e left eye w ill in tor t , an d th e m uscles th at w ill
w ork are th e LSR an d LSO. If th e LSR is paralyzed th e LSO w ill w ork an d th e left eye
w ill m ove dow n .
L/R Hypertropia
If w e n ow w ork on th e sam e prin ciple an d get th e m u scle involved in an L/R hyper-
t ropia. If th e pat ien t h as an L/R hyper t ropia, th en it in dicates th at th e depressors
are paralyzed like th e LIR or th e LSO. It cou ld also m ean th at th e left eye is in th e
n orm al p osit ion bu t th e righ t eye is hypot ropic. Th is could be du e to th e elevators
of th e righ t eye, n am ely, th e RIO an d th e RSR, being paralyzed. Th is is th e first step
of th e test . Of th e ext raocu lar m u scles w e h ave n arrow ed th e diagn osis of ext ra-
ocu lar m u scle paralysis to fou r m u scles, th e t w o depressors of th e righ t eye, or th e
t w o elevators of th e left eye.
Next , w e perform th e secon d step of th e test . In th is w e ask th e pat ien t to per-
form dext roversion or levoversion . Th is m ean s w e ask th e p at ien t to look to th e
righ t an d to th e left . If w e ask th e pat ien t to look to th e righ t , th e left eye cou ld be
h igh er th an th e righ t eye. If th e left eye is h igh er on dext roversion , th en it could
m ean th at th e LSO is involved or it cou ld m ean th at th e righ t eye is hypot ropic. Th is
w ou ld be due to an RSR paralysis. In levoversion if th e left eye is h igh er it cou ld be
due to an LIR paralysis. Altern ately, it could m ean th at th e righ t eye is hypot ropic,
w hich w ou ld be due to RIO paralysis. Th u s w e h ave n arrow ed dow n th e m uscles
from fou r to t w o.
Fin ally, w e perform th e th ird step, in w h ich w e t ilt th e pat ien t’s h ead to th e righ t
an d th en to th e left . If w e t ilt th e h ead to th e left , th e righ t eye w ill extort an d th e
left eye w ill in tort . Th is is becau se n er vou s im pulses w ill be sen t from th e sem icir-
cular can als to keep th e eyes in a st raigh t posit ion . Rem em ber th at th e superiors
are in tor ters. So if th e righ t eye extor t s, it m ean s th e in feriors in th at eye (LIO an d
LIR) w ork, an d if th e left eye in tor ts it m ean s th e su periors of th at eye (RSO an d
RSR) w ork. W h en th is h appen s, in th e left eye th e LSO an d LSR sh ou ld w ork. Now
th e LSO is paralyzed an d so can n ot w ork. Th erefore, on ly th e LSR w ill w ork to in -
tort th e eye. An d becau se th e balan ce w ill n ot be m ain t ain ed bet w een th ese t w o
m uscles, th e left eye w ill m ove u p given th at th e LSR is also an elevator. Th u s on e
can diagn ose th e case of LSO.
If we ask the patient to tilt the head to the right, the left eye w ill extort and the right
eye w ill intort. In the right eye the intorters w ill be the RSR and RSO. Since the RSR is
paralyzed, only the RSO w ill work. This w ill cause the right eye to m ove dow nward.
Sim ilarly, w e can differen t iate bet w een th e LIR an d th e RIO in th e th ird step . If
w e t ilt th e h ead to th e left , th e righ t eye w ill extor t an d th e m uscles th at w ill w ork
are th e RIO an d RIR. Becau se th e RIO is paralyzed on ly th e RIR w ill w ork to extor t
th e eye, th ereby m oving th e righ t eye dow nw ard.
If w e t ilt th e h ead to th e righ t , th e left eye w ill extort an d th e m u scles th at w ill
w ork are th e LIR an d LIO. If th e LIR is paralyzed th e LIO w ill w ork an d th e left eye
w ill m ove u p.
Prim ar y in ferior obliqu e over act ion
Management
Occlusion
W h en double vision is rest ricted to dow nw ard gaze as in fou rth -n er ve paralysis,
on e can occlu de th e low er th ird of th e spectacle len s in fron t of th e paret ic eye
w ith sem iopaqu e tape. Th is can be perform ed if th e m edical con dit ion is n ot suit-
able for su rger y.
Surgery
Depen ding on th e class of su perior oblique (SO) paralysis, th e su rgical t reat m en t is
based on th e class of paralysis as described by von Noorden ( Table 13.1 ).
Table 13.1 Treatm e nt o f Supe rio r Oblique Paralysis Based o n Class
Class o f
SO Paralysis Surgical Treatm ent
Class 1 Inferior oblique myectomy

Class 2 Superior oblique tuck (8–12 m m); recession of contralateral
inferior rectus as a secondary procedure
Class 3 Hypertropia of < 25 prism diopters; inferior oblique myectomy;
if there is hypertropia of < 25 prism diopters; inferior oblique
myectomy with superior oblique tuck
Class 4 As in class 3 plus recession of ipsilateral superior rectus or
contralateral inferior rectus
Class 5 Superior oblique tuck with recession of ipsilateral superior
rectus or recession of contralateral inferior rectus
Class 6 As in classes 1–5 but bilateral surgery
Class 7 Explore trochlea
A
Abducens (Sixth) Nerve Palsy

Th e abdu cen t (sixth cran ial) n er ve is a sm all, en t irely m otor n er ve th at supplies
th e lateral rect u s of th e eyeball ( Figs. 13.3A,B).
Fig. 13.3 (A) Nucleus of the abducent nerve and the brainstem syndromes. (B) Course
of the abducent nerve. (C) Lesions of the abducent nerve.
Presentation
In th e prim ar y posit ion th e eyeball is converged becau se of th e un opposed act ion
of th e m edial rect u s m uscle. Abduct ion is lim ited du e to w eakn ess of th e lateral
rect us m u scle. Un crossed h orizon tal diplopia occu rs, w h ich becom es w orse to-
w ard th e act ion of th e paralyzed m u scle. Th e face is t urn ed tow ard th e act ion of
th e paralyzed m u scle to m in im ize diplopia.
Lesions
Various lesion s of th e abducen t n er ve in it s cou rse can p rodu ce variou s syn drom es
as described in th e follow ing sect ion s ( Fig. 13.3C).
The Bra instem Syndrome
A brain stem lesion of th e sixth n er ve m ay also affect th e fifth , seven th , an d eigh th
cran ial n er ves an d also th e cerebellu m . Th e sixth -n er ve n u cleu s also h as con n ec-
t ion s via th e m edial longit u din al fasciculu s w ith th e th ird-n er ve n u cleu s, an d so a
lesion h ere produces a gaze palsy. Th ree syn drom es can occu r in th e brain stem :
1. Millard-Gubler syndrom e : In th is th e lesion is ven t ral an d involves th e facial
n er ve an d th e pyram idal t ract . Th us th ere is a sixth -n er ve paresis, ip silateral
seven th -n er ve p aresis, an d con t ralateral h em iparesis.
2 . Raym ond syndrom e : In th is syn drom e th e lesion involves on ly th e sixth cran ial
n er ve an d th e pyram idal t ract . Th u s th e pat ien t h as a sixth -n er ve paresis an d
con t ralateral h em iparesis.
3 . Foville syndrom e : In th is th e lesion is dorsally. Because th e lesion is dorsal th e
areas affected are th e m edial longit udin al fasciculus, th e pon t in e param edian
ret icu lar form at ion , th e fifth n er ve, an d th e sym path et ic n euron s. Th u s th e
pat ien t h as h orizon tal conjugate gaze palsy an d palsies of th e ipsilateral fifth ,
sixth , seven th , an d eigh th n er ves w ith ipsilateral Horn er syn drom e.
Subarachnoid Space Syndrome
Elevated in t racran ial p ressure m ay resu lt in dow nw ard displacem en t of th e brain -
stem , w ith st retch ing of th e sixth n er ve, w h ich is teth ered at its exit from th e pon s
an d in th e Dorello can al. Th is gives rise to n on localizing sixth -n er ve palsies of
raised in t racran ial pressu re. Th ir t y p ercen t of pat ien ts w ith pseudot um or cerebri
h ave sixth n er ve paresis, besides papilledem a an d it s visu al field ch anges.
Petrous Apex Syndrome
Th e sixth n er ve passes un der th e Gru ber ligam en t in th e Dorello can al. Th is m akes
th e n er ve liable to paralysis due to a lesion of th e pet rou s apex.
Gra denigo Syndrome
Graden igo syn drom e is due to a localized in flam m at ion or ext radu ral abscess of
th e pet rous apex follow ing com plicated ot it is m edia. Th is lesion can lead to th e
follow ing:
Sixth -n er ve palsy
Ipsilateral decreased h earing (eigh th -n er ve involvem en t)
Ipsilateral facial pain in th e dist ribu t ion of th e fifth n er ve
Ipsilateral facial paralysis
Pseudo-Gra denigo Syndrome
Th is syn drom e is seen in t w o con dit ion s:
Nasopharyngeal carcinom a : Th is m ay cau se serou s ot it is m edia due to obst ru c-
t ion of th e eu stach ian t u be, an d th e carcin om a m ay su bsequen tly invade th e
cavern ou s sin us, cau sing sixth -n er ve paresis.
Cerebellopont ine angle t um or: Th is m ay cau se sixth -n er ve paresis w ith de-

creased h earing (eigh th n er ve), seven th -n er ve palsy, fifth -n er ve p alsy, ata xia,
an d papilledem a.
In th is syn drom e oth er n er ves (e.g., th e th ird, four th , fifth n er ves) in th e cavern ou s
sin us are also involved.
Orbita l Syndrome
In th is syn drom e proptosis is an early sign , an d th e opt ic n er ve m ay app ear n or-
m al or dem on st rate at rophy or edem a. Th e oph th alm ic division of th e fifth n er ve
is involved. Th e th ird, four th , an d sixth n er ves are also involved. It occu rs du e to
traum a, t um ors, or in flam m at ion s.
Isola ted Sixth-Nerve Pa lsy
Isolated sixth -n er ve paralysis can also be seen in th e absen ce of any oth er asso-
ciated sign s .
Thyroid eye disease, m yasth en ia gravis, Du an e syn drom e t ype 1, spasm of th e n ear
reflex, m edial w all orbital blow -ou t fract u re w ith rest rict ive m yopathy, break in
fusion of a congen it al esoph oria
Management
Occlusion
On e can perform occlusion w h en dou ble vision is presen t in lateral gaze in pa-
t ien t s w ith m ild sixth -n er ve paresis.
Treatment of the Ca use
On e sh ould determ in e th e cau se an d t reat it .
Surgery
A m axim al recession -resect ion procedure su ffices in m ost in stan ces of in com plete
abdu cen s paralysis to restore a u sefu l field of single bin ocu lar vision an d to elim i-
n ate th e h ead t u rn . If th ere is a com plete p aralysis of th e lateral rect u s m uscle, on e
can perform a t ran sposit ion of th e superior an d in ferior rect us m u scles to th e in -
ser t ion of th e lateral rect u s m u scle. Th is is called th e Hu m m elsh eim operat ion . In
th is surger y, h alf of th e th ickn ess of th e su perior rect u s (SR) an d in ferior rect u s (IR)
are t ran sposed to th e in ser t ion of th e lateral rect u s (LR). Recession of th e m edial
rect us (MR) is also don e. In Jen sen’s operat ion also, th e t ran sposit ion is don e w ith
recession of th e m edial rect u s. In th is operat ion , th e LR is split as are th e SR an d
in ferior rect us (IR). Th en th e split por t ion s of th e SR an d IR are sut u red to th e split
por t ion s of th e LR ( Figs. 13.4 an d 13.5 ) .
Fig. 13.4 Hum melsheim operation. Half

the superior rectus (SR) and inferior rectus
(IR) are transposed to the area of the lateral
rectus (LR). This is also combined with a re -
cession of the MR.
Fig. 13.5 Jensen operation. The su-

perior rectus (SR), lateral rectus (LR),
and the inferior rectus (IR) are split.
The superior half of the split MR is su-
tured to one half of the split SR, and
the inferior half of the LR is sutured to
one half of the split IR. The MR is also
done.
Botulinum Toxin Injection

Tem porar y paralysis of an ext raocular m u scle can be u sed in conjun ct ion w ith th e
t ran sposit ion procedu res or in isolat ion . To determ in e th e st ate of recover y of th e
LR follow ing a sixth -n er ve palsy, a t iny dose of bot ulin um toxin is injected in to th e
belly of th e overact ing m edial rect us m uscle. Th is paralyzes th e m edial rect us an d
so th e h orizon tal forces on th e globe are m ore balan ced an d th e esot ropia reduced
or elim in ated.
Facial (Seventh) Nerve Palsy

Th e facial n er ve is th e seven th cran ial n er ve, an d it is both a m otor as w ell as a
sen sor y n er ve. Th e seven th cran ial n er ve h as th ree n u clei:
Main m otor nucleus : Lies in th e low er part of th e pon s. Th e part of th e n u cleu s
th at su pplies th e m u scles of th e upper part of th e face receives cor t icon u clear
fibers from both cerebral h em isph eres. Th e part of th e n ucleu s th at sup plies
th e m uscles of th e low er par t of th e face receives cort icon u clear fibers from th e
opposite cerebral h em isph ere on ly.
Parasym pathet ic nuclei: In clu de th e superior salivator y an d lacrim ator y n uclei.
Th e form er sup plies th e subm an dibu lar an d sublingu al glan ds an d th e lat ter th e
lacrim al glan d.
Sensory nucleus: Sit u ated in th e u pper par t of th e m edulla oblongat a
Presentation
Th e lesion s of th e facial n er ve are sh ow n in Figure 13.6 an d are described in th e
follow ing sect ion s.
Supra nuclear Lesion
If th e lesion is su pran u clear on ly th e low er h alf of th e face is involved, an d if it is
a low er m otor n eu ron lesion th e w h ole h alf of th e face is involved. Th is is because
th e u pper h alf of th e face h as a bilateral in n er vat ion .
Cerebellopontine Angle Tumor
Th e seven th n er ve m ay be affected by a cerebellopon t in e angle t u m or as it exit s
th e brain . Th e presen t ing sign s in clude :
Tot al ipsilateral facial w eakn ess (seven th -n er ve involvem en t)
Decreased tearing (lacrim at ion involved)
Hyperacusis (n er ve to stapedius involved)
Fig. 13.6 Lesions of the facial nerve: 1, supranuclear lesion; 2, cerebellopontine angle
tumor; 3, geniculate ganglionitis (Ramsay-Hunt syndrome); 4, isolate ipsilateral tear de-
ficiency; 5, lesion before nerve to stapedius; 6, lesion after nerve to stapedius; 7, lesion
after chorda t ympani nerve; 8, Bell palsy—isolated total ipsilateral facial palsy; 9, isolated
partial ipsilateral facial palsy.
Decreased t aste from th e an terior t w o th irds of th e tongu e (ch orda t ym pan i

n er ve involved)
Fifth , sixth , an d eigh th n er ve involved w ith cerebellar dysfu n ct ion s
Geniculate Ganglionitis
Gen iculate ganglion it is is kn ow n as th e Ram say-Hun t syn drom e an d is ch aracter-
ized by th e follow ing feat u res:
Sam e fin dings as in cerebellopon t in e-angle t u m ors except n o associated n euro-
logical deficits
Th ere m ay be zoster vesicles on th e t ym pan ic m em bran e, extern al au ditor y ca-
n al, or extern al ear.
Isola ted Ipsila tera l Tea r Deficiency
Isolated ipsilateral tear deficien cy occu rs in n asoph ar yngeal carcin om as, w h ich af-
fect th e vidian n er ve or th e pter ygopalat in e ganglion .
Lesion before Nerve to Sta pedius

Lacrim at ion is n orm al. Th e oth er fin dings are as follow s:
Hyperacusis (n er ve to stapediu s involved)
n er ve involved)
Total ipsilateral facial w eakn ess (seven th -n er ve involvem en t)
Lesion a fter Nerve to Sta pedius
n er ve involved)
Total ipsilateral facial w eakn ess (seven th -n er ve involvem en t)
Lesion a fter Chorda Tympa ni Nerve
On ly tot al ipsilateral facial w eakn ess (seven th -n er ve involvem en t)
Bell Palsy
On ly tot al ipsilateral facial w eakn ess (seven th -n er ve involvem en t)
Isola ted Pa rtia l Ipsilateral Fa cial Palsy
In th is con dit ion on ly cer tain bran ch es of th e seven th -n er ve are affected.
Parkin son ism , basal ganglia lesion
Management
Work up an d t reat according to cause.
Multiple Cranial-Nerve Palsies (Cavernous Sinus Syndrome and Orbital

Apex Syndrome)
Causes of m u lt iple cran ial-n er ve palsies can be t rau m at ic, vascu lar (in tern al ca-
rot id ar ter y an eu r ysm , posterior cerebral ar ter y an eu r ysm , direct or in direct ca-
rot id cavern ou s fist ulas, th rom bosis, etc.), n eop lasm s (m ay be prim ar y cran ial t u -
m ors or m et ast asis), in flam m at ion s, an d in fect ion s su ch as h erp es zoster, syph ilis,
t u bercu losis, m ucorm ycosis.
Presentation
Pat ien ts presen t w ith periorbital or epicran ial pain , ipsilateral ocular m otor n er ve
palsies, ocu losym path et ic paralysis, an d sen sor y loss in th e oph th alm ic an d m ax-
illar y division of th e t rigem in al n er ve. Orbital apex syn drom e occurs ow ing to a
sim ilar cau se at th e apex of th e orbit an d is dist ingu ish ed by involvem en t of th e
opt ic n er ve in it .
Management
Man agem en t is directed tow ard th e cau se.
Pupillary Abnormalities
W h en ligh t is sh on e in on e eye, both th e pu pils con st rict . Con st rict ion of th e pu pil
to w h ich ligh t is sh on e is called direct light reflex an d th at of th e oth er pupil is
called consensual (indirect) light reflex . If both pu pils are illu m in ated sim u lt an e-
ously, th e respon se su m m ates. Th is m ean s th e con st rict ion of each pu pil is greater
th an th e con st rict ion n oted w h en on ly on e pu pil is illum in ated. Rods an d con es
in it iate th e ligh t reflex ( Fig. 13.7 ).
Anisocoria
Un equ al size of p upils w ith differen ces in th e diam eter of 0.3 m m or m ore is re-
ferred to as anisocoria. Causes in clude th e follow ing:
Sym pathet ic paralysis: Miosis of th e affected pu pil. Degree of in equ alit y in -
creases in th e dark.
Parasym pathet ic paralysis: Mydriasis of th e affected pu pil. Degree of in equ alit y
in creases in ligh t .
Central anisocoria : Occurs in 20%of n orm al in dividu als. Norm al react ion is seen
w ith dim ligh t an d n ear t arget . Degree of in equ alit y also decreases in ligh t .
Fig. 13.7 Lesions of the pupil.

(Relative) Afferent Pupillary Defect

Com m on causes in clude opt ic n er ve involvem en ts (opt ic n eurit is, com pression of
opt ic n er ve, an terior isch em ic opt ic n eu ropathy, etc.), den se m edia opacit y, ret in al
cau ses (e.g., RD, cen t ral ret in al vein occlu sion or bran ch ret in al vein occlusion , cen -
t ral serou s ret in och oroidopathy, etc.) Also called Marcus Gun n p upil.
Presentation
Th e con dit ion can be detected by th e sw inging flash ligh t test . Th e test can be per-
form ed in corn eal opacit y, th ird-n er ve palsy, or an at ropin ised pup il as it is n ot
related to visu al acuit y. Sin ce relat ive afferen t pu pillar y defect occurs in con dit ion s
w h ere th ere is a relat ive decrease in th e con duct ion of th e opt ic n er ve as com pared
to n orm al, it is absen t in bilateral sym m et rical opt ic n er ve or ret in al lesion s.
To perform th e sw inging flash ligh t test th e exam in er sh in es a brigh t ligh t in to
th e n orm al eye w h ile th e pat ien t is fixing on a dist an ce t arget . Th e ligh t is kept 3 to
5 cm from th e eye just below th e visu al axis. Th e speed an d exten t of con st rict ion
are obser ved. A n orm al direct an d con sen su al ligh t reflex can be obser ved. Ligh t is
th en sh ifted briskly to th e con t ralateral eye. Each eye is illum in ated for ~1 secon d
an d pup illar y react ion is obser ved. Th e affected eye sh ow s paradoxical dilatat ion
in stead of con t ract ion .
Oth er pu pillar y abn orm alit ies
Horner Syndrome
Horn er syn drom e occurs in lesion s involving th e sym path et ic pupillom otor path -
w ay.
Presentation
Pat ien t s presen t w ith m iosis, n orm al pu pillar y react ion to ligh t , an d n ear, in creased
an isocoria in dim con dit ion s, ptosis, ip silateral an h idrosis (in lesion s proxim al to
carot id bifu rcat ion ), en op h th alm os, loss of ciliospin al reflex (in lesion s distal to th e
ciliospin al cen ter of Bu dge), hyp och rom ic an isocoria (eye w ith Horn er syn drom e
is ligh t in color) ( Fig. 13.8 ).
Cen t ral an isocoria
Management
Invest igat ion s in clu de th e follow ing ph arm acological tests:
Cocaine test : Cocain e (4%) is in st illed in both eyes. Th e Horn er pu pil w ill n ot
dilate (>0.8 m m an isocoria sign ifican t). Noradren alin reu ptake is blocked by co-
cain e cau sing dilatat ion . In Horn er syn drom e n o n oradren alin is p resen t at th e
n er ve en dings.
Hydroxyam phetam ine test : Hydroxyam ph etam in e (1%) is in st illed in both eyes.
In preganglion ic lesion s both pu pils w ill dilate an d in postganglion ic lesion s th e
Horn er pu pil w ill n ot dilate. Noradren alin e release is poten t iated by Paredrin e
(hydroxyam ph et am in e) causing dilat at ion . In postganglion ic lesion s n eu ron s
are dest royed so n o dilatat ion is seen .
Fig. 13.8 (A) Horner syndrom e. (B) Phar-

m acological tests to localize Horner syn-
drom e. B
Adrenaline test : Adren alin e 1:1000 is in st illed in both eyes. No dilatat ion is seen
in preganglion ic lesion s, bu t dilat at ion of a Horn er pu pil is presen t in postgan -
glion ic lesion s because adren alin e is n ot broken dow n becau se of th e absen ce
of m on oam in e oxidase (MAO) in h ibitor in dest royed n er ve en dings. Th is is du e
to “den er vat ion hypersen sit ivit y” to adren ergic n eu rot ran sm it ter.
Other investigations: Magnetic resonance im aging (MRI) of the brain/spinal cord/
orbit; com puted tom ography (CT) of the thorax; Doppler of the carotid; ear, nose,
and throat (ENT) assessm ent; chest x-ray. Treatm ent depends on the exact cause
and site of the lesion. The patient is referred to a suitable specialist.
Argyll-Robertson Pupil
Th is con dit ion is u su ally seen in pat ien ts suffering from syph ilis. It sh ow s a bi-
lateral an d asym m et rical involvem en t . Lesion s u sually affect th e n eu ron s of th e
pretectal area.
Presentation
Sign s in clu de bilateral, m iot ic, irregular pup il w ith asym m et rical respon se to ligh t
an d n ear st im ulu s for accom m odat ion . Ligh t reflex is absen t an d accom m odat ion
is ret ain ed. Th e m ain associated causes are tert iar y syph ilis, diabetes m ellit u s,
Wern icke en ceph alopathy, en ceph alit is, h eredit ar y n eu ropath ies, m idbrain t u-
m ors, h erpes zoster oph th alm icu s, an d ch ron ic alcoh olism .
Management
Man agem en t addresses th e u n derlying cau se.
Adie Pupil/Tonic Pupil

Adie ton ic pu pil result s from dam age to th e postganglion ic parasym path et ic n er ve
su pply to th e sp h in cter pu pillae. Th is is seen in young adult s an d is un ilateral in
80%.
Presentation
Sign s in clude a large, regu lar pu pil w ith poor ligh t react ion an d exaggerated, slow,
su st ain ed n ear respon se (ton ic) w ith ch aracterist ic verm iform m ovem en t s of th e
pupillar y border on slit lam p. Ligh t-n ear dissociat ion is p resen t . In ch ron ic cases,
th e pup il becom es poor an d poorly dilat ing.
Th is con dit ion is associated w ith dim in ish ed deep ten don reflexes (Adie syn -
drom e) an d patchy hypoh idrosis (Ross syn drom e).
Management
Invest igat ion s in clu de ph arm acological test s (e.g., in st illat ion of 0.125% pilocar-
pin e in both eyes). An Adie pu pil con st ricts, w h ereas th e n orm al pu pil does n ot
becau se of parasym path et ic den er vat ion supersen sit ivit y. Reassu re th e pat ien t
about any obvious an iscoria an d accom m odat ive dysfu n ct ion . Pilocarp in e (0.1%)
h elps in redu cing th e m ydriat ic blu rring. Glasses also h elp w ith accom m odat ive
dysfu n ct ion .
Fig. 13.9 Arterial loop. Color pho-

tograph shows a tortuous vessel
above the optic disk. (Courtesy of
Robin D. Hamilton, A.M. Hamilton)
Developmental Optic Nerve Anomalies
Prepapillary Vascular Loop

These blood vessels project from the optic disk into the vitreous and return to the
optic disk to continue their usual course; 80 to 90%are arterial in origin (Fig. 13.9 ).
Presentation
Prepapillar y vascu lar loop is an in ciden t al fin ding.
Optic Nerve Hypoplasia

Th is con dit ion is ch aracterized by a decreased n u m ber of opt ic n er ve axon s an d
n orm al m esoderm al an d glial supp or t ing t issue.
Presentation
Visual acu it y m ay range from 20/20 to percept ion of ligh t . Op h th alm oscopically
th e disk appears sm all an d pale. A peripapillar y h alo surroun ded by a pigm en t ring
(dou ble-ring sign ) is alw ays presen t .
De Morsier syn drom e is a com bin at ion of opt ic n er ve hypoplasia, absen ce of
sept um pellu cidu m , an d agen esis or par t ial developm en t of th e corpus callosum .
Coexisten t h em isph eric abn orm alit ies an d absen ce of pit uit ar y in fun dibulu m w ith
or w ith ou t post pit u it ar y ectopia m ay be presen t . Pit uitar y h orm on e deficien cy
m ay be p resen t .
Management
Man agem en t con sist s of n eu rological an d en docrin ological evalu at ion s w ith su p-
plem en tat ion of requ ired h orm on es.
Optic Nerve Pit

Opt ic n er ve pit is h ern iat ion of rudim en t ar y n eu roectoderm al t issu e in to a pocket-
like depression w ith in th e n er ve substan ce w ith u n kn ow n p ath ogen esis.
Presentation
A roun d or oval gray-w h ite or yellow ish depression is seen in th e opt ic disk, prefer-
ably at th e tem poral m argin . Associated visu al field defects m ay be presen t , m ost
com m on ly paracen t ral arcuate scotom a. Th ere is a 45% risk of serous ret in al de-
tach m en t .
Acquired depression in th e opt ic disc in n orm al ten sion glau com a pat ien t s, opt ic
n er ve colobom a, cen t ral serou s ret in opathy (CSR), age-related m acular degen era-
t ion , pigm en t ep ith eliu m det ach m en t
Management
Pat ien ts sh ou ld be given regular follow -u p. Laser p h otocoagu lat ion is p erform ed at
th e peripapillar y ret in a adjacen t to th e pit if serou s ret in al det ach m en t is presen t .
In tern al gas t am pon ade is recom m en ded for ret in al detach m en t .
Optic Disk Coloboma

Opt ic disk colobom a is a sp oradic or au tosom al dom in an t con dit ion arising from
fau lt y closure of th e em br yon ic opt ic cup.
Presentation
Visual acuit y is variably affected. Th ere is visu al-field defect depen ding on th e
n er ve fiber involvem en t . An en larged, w h ite, glisten ing, bow l-sh aped disk w ith
cen t ral excavat ion , w h ich m ay exten d to th e ret in a an d ch oroid. Microoph th alm ia
m ay presen t .
Associat ion s in clu de CHARGE syn drom e ( colobom a of iris or ret in och oroid,
h eart an om aly, ch oan al at resia, ret ardat ion , gen ital an om alies, e ar an om alies),
Golt z focal derm al hypoplasia, Walker-Warburg syn drom e, lin ear n evus sebaceous
syn drom e, an d Golden h ar syn drom e. Opt ic disk colobom a is rarely associated w ith
t ran ssph en oidal en ceph alocele ( Fig. 13.10A).
Management
Visual-field test ing m ust be don e. Obser vat ion is n eeded.
Morning Glory Syndrome

Morn ing glor y syn drom e is due to a fun n el-sh aped expan sion of th e distal por t ion
of th e opt ic stalk, w h ich is due to n orm al closure bu t abn orm al progression of clo-
su re of th e em br yon ic fissure in th e distal por t ion of th e opt ic st alk. It is sporadic
in occurren ce ( Fig. 13.10B).
A B
Fig. 13.10 (A) Coloboma of the optic disk. (B) Morning glory syndrome. (Courtesy of
Robin D. Hamilton, A.M. Hamilton)
Presentation
Visual acu it y m ay be variably affected. Oph th alm oscopy reveals an en larged, p in k
disk sit u ated in a fun n el-sh aped excavat ion w ith overlying glial t issu e. Ret in al
blood vessels are in creased in n u m ber, arise from th e disk periph er y, an d ru n an
abn orm ally st raigh t cou rse w ith acu te bran ch ing. Th e m acu la m ay be in corporated
in th e postglial excavat ion (m acular capt u re). Th ere is an in creased risk of ret in al
detach m en t . Associat ion s in clu de t ran ssph en oidal en ceph alocele, ipsilateral in -
t racran ial vascu lar hyp oplasia, hypopit u it arism , an d facial abn orm alit ies-hyper-
telorism , depressed n asal bridge, an d m idlin e u pper lip n otch cleft palate.
Management
Man agem en t con sists of regular follow -up an d pat ien t assu ran ce.
Tilted Disk
Tilted disk is bilateral, n on h ereditar y, presum ably du e to ect asia of th e in feron asal
fun du s.
Presentation
Pat ien ts p resen t w ith an obliqu ely placed disk w ith a posteriorly displaced in fero-
n asal port ion an d sit us inversu s of th e vessels. Bilateral h em ian opia th at does n ot
resp ect th e ver t ical m eridian is seen . Myopic ast igm at ism is p resen t . Th e con di-
t ion is associated w ith visu al-field defects. It m ay be associated w ith congen it al
su prasellar t u m or.
Management
Man agem en t con sists of correct ion of th e refract ive error an d pat ien t assu ran ce.
Optic Disk Pathology
Optic Neuritis
In flam m at ion of th e opt ic n er ve m ay be a ret robu lbar n eu rit is w ith n orm al opt ic
disk (m ost frequen tly m ult iple sclerosis, young adult s), papillit is w h ere th e disk is
sw ollen (m ost com m on ly in ch ildren ), or n eu roret in it is-associated in flam m at ion
of ret in al n er ve fibers w ith m acu lar st ar form at ion .
Dem yelin at ing opt ic n eu rit is is due to ph agocytosis of th e m yelin n er ve sh eath s
an d su bsequ en t lay-dow n of fibrou s plaqu es. It affect s th e w h ite m at ter t racts,
brain stem , an d spin al cord, bu t th e periph eral n er ves are spared.
Presentation
Presen tat ion is u su ally at age 20 to 30 years an d is subacute an d u n ilateral. Th ere
is decreased vision , pain exacerbated by eye m ovem en ts, an d t iny flash es of ligh t
(ph osph en es). Visu al acuit y usually varies bet w een 20/60 (6/18) to 20/200 (6/60)
in ret robulbar n eurit is an d papillit is. Afferen t pu pillar y defect , dysch rom atopsia
(n ot proport ion al to visu al loss), dim in ish ed ligh t an d brigh t n ess appreciat ion , an d
decreased con t rast sen sit ivit y are presen t . Th ere is diffuse depression of th e cen -
t ral 30 degrees of th e visu al field.
Dem yelin at ing diseases causing ocular involvem en t in clude th e follow ing:
Mult iple sclerosis: Most com m on . MRI sh ow s p eriven t ricu lar plaques.
Devic disease (neurom yelit ica opt ica) : Bilateral opt ic n eurit is w ith t ran sverse
m yelit is. Severe bilateral dim in ish ed visual acuit y w ith paraplegia.
Schilder disease : On set before 10 years an d death w ith in 1 to 2 years. Bilateral,
progressive opt ic n eurit is.
Com pressive opt ic lesion s, sarcoidosis, vascu lit is (e.g., system ic lu pus er yth em ato-
su s), syph ilis, an terior isch em ic opt ic n europathy (AION), Leber’s h ereditar y opt ic
n europathy (LHON), toxic or n ut rit ion al n eu ropath ies, post viral dem yelin at ion
Management
Presentation
Presen tat ion is u sually at age 20 to 30 years an d is su bacu te an d u n ilateral. Diag-
n osis is usu ally clin ical. Th ere is decreased vision , p ain exacerbated by eye m ove-
m en ts, an d t iny flash es. In th e case of an at ypical presen t at ion , progressive opt ic
n europ athy n eeds to be ru led ou t .
Invest igat ion s requ ired in clude MRI, ch est x-ray, serological tests an t in u clear
an t ibody (ANA), er yth rocyte sedim en t at ion rate, syph ilis, an t in eut roph il cytoplas-
m ic an t ibodies (ANCA), an d cerebrospin al fluid (CSF) an alysis. Typ ical w h ite m at-
ter plaques in MRI w ith oligoclon al ban ds in CSF suggest a dem yelin at ing cau se.
Treat m en t m odalit ies in clude in t raven ou s m ethylpredn isolon e 1 g daily for 3 days
follow ed by oral predn isolon e 1 m g/kg/day for 11 days, th en t apered over 3 days.
Th e resu lts of th e ONTT (opt ic n eu rit is t reat m en t t rial) sh ow th at th e above regi-
m en h asten s visual recover y, but does n ot affect long term visu al outcom es. In t ra-
m u scular in terferon β-1a at th e first episode delays th e developm en t of m u lt iple
sclerosis.
Band Optic Atrophy

Also called bow t ie at rophy, th is is a kin d of prim ar y opt ic at rophy seen w ith ch ias-
m al syn drom e w ith bitem poral h em ian opic field defects in th e ipsilateral eye, an d
opt ic t ract lesion s in th e con t ralateral eye.
Presentation
Opt ic disk pallor is seen prim arily at th e n asal an d tem poral p or t ion s of th e opt ic
disk. Sup erior an d in ferior port ion s are n ot involved because of sparing of th e su -
perior an d in ferior bu n dles of arcuate fibers.
Oth er causes of opt ic at rophy
Management
MRI an d n eu rological evaluat ion s are a m u st . Treat m en t is in collaborat ion w ith a
n eurologist an d n eu rosurgeon .
Papilledema
Papilledem a is bilateral disk sw elling due to raised in t racran ial ten sion (ICT),
w h ich is t ran sm it ted along th e subarach n oid space via n er ve sh eath s to block th e
axoplasm ic flow. Causes in clude in t racran ial t um ors, pseu dot u m or cerebri, sub-
du ral an d epidural h em atom as, subarach n oid h em orrh age, brain abscess, sagit tal
sin us th rom bosis, an d posterior fossa t u m ors (in ch ildren ).
Presentation
Pat ien ts presen t w ith t ran sien t visual obscu rat ion s of a few secon ds, w h ich m ay be
accen t u ated w ith post u re or st rain ing, h eadach e, n au sea, vom it ing, dou ble vision ,
an d decreased vision . Bilateral asym m et ric disk sw elling, disk hyperem ia w ith
dilated capillaries, dilated vein s, loss of spon t an eou s ven ous pu lsat ion s, splin ter
h em orrh ages, cot ton -w ool spots, obscurat ion of th e blood vessels en tering an d
leaving th e disk, an d con cen t ric ret in al folds (Paton lin e) form th e h allm arks of th e
acute papilledem a.
Elevat ion of th e disk w ith out h em orrh ages (ch am pagn e cork disks), decreased
ven ous dilat at ion , an d even t u al at rophy of th e disk w ith a decrease in th e disk el-
evat ion over a period of 6 w eeks is ch aracterist ic of ch ron ic papilledem a. En large-
m en t of a blin d spot is seen in field exam in at ion ( Fig. 13.11 ).
Fig. 13.11 Papilledema.

In t racran ial space-occu pying lesion s (t um ors, h em orrh age, abscess, etc.), in filt ra-
t ive (leukem ia, lym ph om a), in flam m ator y (opt ic n eurit is, sclerit is, etc.), gran ulo-
m atou s (sarcoidosis, t u bercu losis, etc.), an d vascu lar causes (AION, diabet ic papil-
lopathy, etc.) of disk sw elling an d pseudopapilledem a
Management
Fundus fluorescein angiography (FFA) show s leakage from dilated disk capillaries, and
late pooling of the dye is evident. Magnetic resonance venography is done to check
the cerebral sinuses and lum bar puncture for CSF analysis. Treatm ent is directed
tow ard the underlying cause; neurosurger y m ay be required. Regular ophthalm ic
follow -up w ith disk status, vision, color vision, and field changes is required.
Pseudopapilledema
An om alies resem ble papilledem a but are n ot du e to raised ICT. Th ey can in stead
be du e to disk drusen , t ilted disks, hyperopic disks, m yopic disks, an d m yelin ated
peripapillar y n er ve fibers. Disk drusen is autosom al dom in an t , bilateral lum py
disk w ith absen t cu p an d cen t rally em erging vessels w ith an abn orm al bran ch ing
pat tern . Visu al acuit y is u su ally n orm al bu t associated field defects are usually
presen t . Diagn osis is by autoflu orescen ce, B-scan , ult rason ography, an d CT scan .
Sim ilarly m yopic disks m ay be elevated n asally an d m ay leak on FFA. Hyperopic
disk is crow ded an d elevated an d th us can lead to con fu sion . Tilted disks are el-
evated su perotem porally ( Fig. 13.12 ).
Pseudotumor Cerebri
Pseu dot u m or cerebri is usually a ben ign , self-lim it ing disease of un kn ow n et iology
ch aracterized by sign s an d sym ptom s of raised in t racran ial pressu re w ith n orm al
CSF, pap illedem a, an d n orm al n er vous system im aging. It can be idiopath ic an d is
kn ow n to be associated w ith vit am in A in toxicat ion , tet racyclin e th erapy, steroid
w ith draw al, n alidixic acid, im pairm en t of cen t ral ven ous drain age, an d system ic
lupu s er yth em atosu s.
Fig. 13.12 Optic nerve head dru-

sen. (Courtesy of Robin D. Hamilton, AM
Hamilton)
Presentation
Presen t ing an d diagn ost ic feat ures of pseu do t u m or cerebri are h olocran ial h ead-
ach e, m on ocu lar or bin ocu lar blackout s of vision (du e to m icrocirculat ion s dis-
t u rban ce at th e tem poral lobe), sixth -n er ve palsy w ith diplopia, t in n it u s, papill-
edem a, arcu ate scotom as in th e n asal region , an d relat ive afferen t pu pillar y defect
(RAPD).
Brain t um ors (m ost com m on ly gliom a), ar terioven ous m alform at ion s, in fect iou s
diseases su ch as viral en ceph alit is, an d an om alous opt ic n er ve w ith h eadach e
Management
Asym ptom at ic p at ien ts sh ould be follow ed up regu larly. Carbon ic an hydrase in -
h ibitors (in h ibit th e CSF produ ct ion ), cor t icosteroids, oral hypoglycem ic agen ts,
lum boperiton eal sh u n t , an d opt ic n er ve sh eath decom pression (ONSD) are oth er
t reat m en t m odalit ies.
Arteritic Ischemic Optic Neuropathy (Giant Cell Arteritis)

Th is in farct ion of th e opt ic disk is associated w ith gian t cell arterit is (GCA). Tw en t y
percen t of cases do n ot h ave any system ic sign s of GCA at th e t im e of presen t at ion
(occult GCA).
Presentation
Th e pat ien t presen ts w ith sudden , profou n d, u n ilateral visual loss w ith periocu -
lar pain (preceded by t ran sien t visu al loss an d flash es of ligh t u n like n on arterit ic
isch em ic opt ic n europathy. Alt it u din al field defect is com m on in th ese pat ien ts.
Pat ien ts m ay experien ce associated sym ptom s su ch as h eadach e, n eck pain , jaw
pain w ith claudicat ion , scalp ten dern ess, fevers, w eigh t loss, an d m yalgias.
Th e age of th e pat ien t is m ore th an 55 years. Palp able an d ten der tem poral
ar ter y, elevated er yth rocyte sedim en t at ion rate, an d coexist ing an em ia are also
presen t .
Non ar terit ic isch em ic opt ic n eu ropathy, opt ic n eu rit is, opt ic n er ve com pression ,
opt ic n europathy, cen t ral ret in al vein occlu sion (CRVO).
Management
ESR, C-react ive protein (CRP), an d tot al blood cou n t-raised platelet s are th e blood
invest igat ion s requ ired to diagn ose an d follow up w ith th e pat ien t . Tem p oral ar-
ter y biopsy sh ow s gran ulom atou s in flam m at ion w ith a grossly n arrow lu m en . FFA
sh ow s severe hypoperfu sion of ch oroid.
Pat ien ts are t reated w ith in t raven ous m ethylpredn isolon e 1 g/day w ith 80 m g
oral predn isolon e for 3 days. After 3 days, 60-m g an d 50-m g oral dose each for 1
w eek follow ed by gradu al redu ct ion of 5 m g each w eek t ill 10 m g/day, w h ich is th e
m ain ten an ce dose. Azath ioprin e h as been t ried in pat ien ts in toleran t of steroids.
Progn osis is poor in spite of early st art of steroids, an d it w orsen s w ith t im e an d
su bsequen t episodes.
A B
Fig. 13.13 (A) Nonarteritic ischemic optic neuropathy (diabetic), color picture.
(B) Nonarteritic ischemic optic neuropathy (diabetic), fundus fluorescein angiography.
Nonarteritic Ischemic Optic Neuropathy

Th is n europathy occurs becau se of occlusion of th e sh or t posterior ciliar y ar teries.
It is m ost com m on in elderly pat ien ts, w ith st ruct ural crow ding of th e opt ic n er ve
h ead du e to a sm all or absen t physiological cu p. System ic hyper ten sion , hyperch o-
lesterolem ia, diabetes m ellit u s, hypercoagulat ion st ates, an d silden afil in take are
som e of th e predisposit ion s for th e con dit ion .
Presentation
Pat ien ts h ave a sudden un ilateral pain less visu al loss, par t icularly after aw aken -
ing, associated w ith color vision abn orm alit ies (propor t ion al to visu al loss) an d
in ferior alt it u din al field defects. Late stages p resen t w ith opt ic at rophy follow ing
ch ron ic vision loss.
Disk pallor w ith disk edem a (diffu se or sectoral) w ith peripapillar y splin ter
h em orrh ages is th e t ypical presen t at ion ( Fig. 13.13 ).
AION, opt ic n eu rit is, opt ic n er ve com pression , opt ic n europathy, an d CRVO
Management
Lipid profile, blood glu cose, com plete blood cou n t , an t in uclear an t ibody, flu ores-
cein t repon em a an t ibody (FTA)/ven ereal disease research laborator y test , ESR, an d
C-react ive protein (to rule ou t GCA) m ust be don e for all pat ien t s. FFA sh ow s early
disk hyperflu orescen ce, w h ich in creases in in ten sit y in th e late st ages. In term it-
ten t blocked fluorescen ce is seen ow ing to splin ter h em orrh ages. Treat m en t con -
sists of m an aging th e u n derlying m edical con dit ion . Progn osis is favorable w ith
m ain ten an ce of good visu al acu it y in m ost pat ien ts.
Toxic or Nutritional Optic Neuropathy

Th is con dit ion is also referred to as tobacco-alcoh ol am blyopia because it is preva-
len t in h eavy drin kers an d ch ron ic pip e or cigar sm okers w h o n eglect th eir diet .
Oth er et iologies in clude th iam in e an d vit am in B12 deficien cy, m eth an ol, eth am bu-
tol, ch loram ph en icol, ison iazide, rifam picin , lead, an d digit alis.
Presentation
Presen t at ion in cludes in sidious, progressive, bilateral visual im pairm en t w ith dys-
ch rom atopsia. Th e opt ic disk m ay be n orm al or tem poral pallor w ith splin ter h em -
orrh ages arou n d th e disk. Cen t rocecal scotom a (bet ter appreciated by red target
in stead of w h ite) is presen t .
Bilateral AION, hypoten sive sh ock, radiat ion inju r y, in filt rat ive opt ic n europathy,
Leber opt ic n europathy, an d bilateral com pressive opt ic n europathy
Management
Blood invest igat ion s m igh t reveal an associated pern icious an em ia an d vit am in
B12 deficien cy. Weekly inject ion s of 1000 un its of hydroxycobalam in for 10 w eeks
along w ith m ult ivitam in s are th e m ain stay of th e t reat m en t . Abstain from drin k-
ing an d sm oking. Progn osis is good w ith t reat m en t in th e early st ages.
Leber Optic Neuropathy

Leber opt ic n eu ropathy is a m atern ally t ran sm it ted t rait arising from p oin t m ut a-
t ion s (m ost com m on ly 11778 m ut at ion ) in m itoch on drial DNA. It t ypically affect s
m en (3:1) in th eir t w en t ies or th irt ies. It is u n ilateral in it ially w ith subsequ en t
involvem en t of th e oth er eye.
Presentation
Pat ien ts presen t w ith pain less un ilateral gradual loss of vision . Th e oth er eye is
involved su bsequen tly. Dysch rom atopsia is presen t . Th ere is a m ildly edem atou s,
hyperem ic disk w ith telangiect at ic vessels in th e peripapillar y ret in a. Th e late
stage presen t s w ith opt ic at rophy. Den se cen t ral or paracen t ral scotom a is seen .
Th e con dit ion m ay be associated w ith sp ast icit y an d gait dist urban ces ow ing to
th e in abilit y to detoxify cyan ide.
n ut rit ion al opt ic n europ athy, an d bilateral com pressive opt ic n europathy
Management
A detailed h istor y h elps in ru ling ou t oth er causes of opt ic n europ athy. CT scan
an d MRI scan h elp to rule ou t a com pressive lesion . Con sider a blood test to detect
m itoch on drial ch rom osom al m u t at ion s. Gen et ic coun seling h elps.
Compressive Optic Neuropathy

Com pressive opt ic n eu ropathy result s from com pression of th e pregen iculate por-
t ion of th e opt ic n er ve. Com m on causes in clude Graves thyroid oph th alm opathy,
m en ingiom as, orbit al space-occu pying lesion , pseu dot u m or, cran ioph ar yngiom a,
an d pit uitar y t u m ors.
Presentation
Visual acuit y, color vision , an d con t rast sen sit ivit y are u sually affected. In it ially
disk sw elling is presen t , follow ed by at rophy in th e late st ages. Afferen t pu pillar y
defect an d cen t ral an d arcu ate scotom as are com m on fin dings. Proptosis m ay or
m ay n ot be associated.
n ut rit ion al opt ic n europ athy, an d Leber opt ic n eu ropathy
Management
CT scan an d MRI of th e orbit an d brain are perform ed to n ote th e com pressive
lesion an d it s exten t . Treat m en t is directed tow ard th e u n derlying cau se of com -
pression .
Infiltrative Optic Neuropathy

In filt rat ion of th e opt ic n er ve can be by n eoplast ic or in flam m ator y cells from
lym p h om as, leu kem ias, sarcoidosis, syph ilis, t u berculosis (TB), fu ngal in fect ion , or
m etast asis from lu ng or breast carcin om a.
Presentation
Pat ien ts presen t w ith progressive, bilateral, severe vision loss. In it ially th e disk is
n orm al oph th alm oscopically but later m ay becom e sw ollen due to in filt rat ion at
th e opt ic n er ve h ead.
Bilateral AION, hypoten sive sh ock, radiat ion injur y, com pressive opt ic n europathy,
n ut rit ion al opt ic n europ athy, an d Leber opt ic n eu ropathy
Management
MRI of th e orbit an d brain , CSF an alysis, an d screen ing test s for gran u lom atou s
disorders an d blood-related disorders are required to con firm th e cau se. Palliat ive
radioth erapy for in filt rat ive n eoplasm s an d cor t icosteroids or an t im icrobials for
in filt rat ive or in fect iou s disorders are advocated form s of t reat m en t .
Radiation Optic Neuropathy

Th e n eu ropathy is usu ally delayed by 2 years after st an dard doses of radiat ion but
m ay be seen m any years after th e t reat m en t . Th e opt ic n er ve can be affected by
radiat ion to th e eye, orbit , sin us, n asoph ar yn x, or brain .
Presentation
Bilateral decreased visu al acu it y is th e presen t ing sym ptom .
Bilateral AION, hypoten sive sh ock, in filt rat ive opt ic n europathy, com pressive opt ic
n europ athy, n ut rit ion al opt ic n europathy, an d Leber opt ic n europ athy
Management
Th e h istor y is sign ifican t . CT an d MRI are don e to evalu ate th e opt ic n er ve. No
t reat m en t is available.
Tumors of Neural Origin
Optic Nerve Glioma

Low -grade, pilocyt ic ast rocytom a can involve opt ic n er ve an d ch iasm ; 30% are as-
sociated w ith n eurofibrom a t yp e 1 (NF-1) an d h ave a bet ter progn osis.
Presentation
Opt ic n er ve gliom a u su ally presen ts in th e first decade w ith gradu al visu al loss as-
sociated w ith proptosis (<3 m m , axial, n onpulsat ile, n on redu cible). St rabism u s an d
nyst agm u s can also be th e presen t ing com plain t s. Oth er feat u res in clude relat ive
afferen t papillar y defect , opt ic at rophy, opt ic disk sw elling, optociliar y sh un ts, an d
CRVO. In ch iasm al lesion s, hypoth alam ic an d pit uitar y dysfun ct ion sym ptom s an d
sym ptom s of in creased in t racran ial pressu re m ay be presen t . Opt ic n er ve gliom a
in adults is rare, bu t if presen t it ten ds to be aggressive an d fat al.
CT scan sh ow s a fusiform en largem en t of th e n er ve, kin king or irregular n er ve,
an d region s of low in ten sit y w ith in th e n er ve. Th ere is n o calcificat ion or hyper-
ostosis. MRI reveals hypoin ten se areas on T1-w eigh ted im ages an d hyperin ten se
areas on T2-w eigh ted im ages. It m ay sh ow in t racran ial exten sion .
Histology sh ow s spin dle-sh aped ast rocytes w ith deeply eosin oph ilic large cell
processes kn ow n as Rosen th al fibers w ith associated degen erat ive foci an d reac-
t ive hyperplasia of m en inges.
Oth er n eoplasm s (m en ingiom a, lym ph angiom a, h em angiom a, rh abdom yosar-
com a), acute eth m oidit is, hyp erthyroidism , cran iosten osis, an d t rau m a
Management
Th e t reat m en t lin e con sist s of obser vat ion for sm all t u m ors w ith good vision . Su r-
gical excision w ith globe preser vat ion is recom m en ded for large t um ors con fin ed
to th e orbit . Radioch em oth erapy is used for in t racran ial exten sion .
Neurofibroma and Schw annoma

NF is a ben ign t u m or d er ived from glial cells of p er ip h eral n er ves. En d on eu ral
cells an d a xon s m ay also be involved in NF. Sch w an n om as ten d to be sin gu lar an d
en cap su lated , w h ereas NFs are m ore likely to be m u lt ip le an d en cap su lated an d
are m ore likely to u n d ergo m align an t t ran sfor m at ion . Plexifor m NFs (an ot h er
t yp e) are alm ost alw ays associated w it h au tosom al d om in an t n eu rofibrom atosis
t yp e 1 (NF-1, von Recklin gh au sen NF) lin ked to ch rom osom e 17, w h ich u su ally
p resen t s in early ch ild h ood .
Presentation
Pat ien ts can presen t w ith a su bcut an eou s lu m p aroun d th e eye or w ith dou ble
vision , decreased vision , an d proptosis in cases w ith in t raorbit al t um or. Mult ip le
n eurofibrom as (NF-1) are associated w ith pigm en ted skin lesion s, osseou s lesion s,
café-au -lait spot s, S-sh aped deform it y of th e lid, Lisch n odu les in th e iris, an d hy-
pert rop hy of periocu lar t issu e, w h ich on palpat ion h as a “bag of w orm s” feeling.
Oth er n eoplasm s (opt ic n er ve gliom a, lym ph angiom a, h em angiom a, rh abdom yo-
sarcom a), acu te eth m oidit is, hyper thyroidism , cran iosten osis, an d t raum a
Management
Oph th alm ic w orkup in clu des a com p lete m edical h istor y, assessm en t of visual
acuit y, ocu lar m ovem en t s, displacem en t , exoph th alm om et r y, ton om et r y, dilated
fun du s exam in at ion , CT scan , MRI, an d u lt rason ography. Sym ptom at ic t u m ors
n eed excision .
Orbit al surger y sh ou ld n ot be don e becau se th e t u m or is related to im por tan t
orbit al st r uct u res.
Meningioma
Middle-aged fem ales are affected. Presen tat ion s var y w ith th e site of th e lesion .
Presentation
Opt ic n er ve sh eath m en ingiom as are ben ign p roliferat ion s of m en ingoepith elial
cells presen t ing w ith progressive m on ocular vision loss, opt ic at rophy, an d optocil-
iar y sh un t s. Tu bercu lum sellae m en ingiom a com presses th e ju n ct ion of th e opt ic
ch iasm w ith th e opt ic n er ve cau sing ipsilateral cen t ral scotom as w ith con t ralateral
jun ct ion al scotom a. Sph en oidal ridge t um ors w ill com press th e opt ic n er ve, an d
sim ilarly olfactor y lesion s w ill cau se com pression of th e opt ic n er ve an d loss of
th e sen se of sm ell.
CT sh ow s diffu se t ubular en largem en t of th e opt ic n er ve seat , t ram t rack or rail-
road sign , an d calcificat ion . MRI reveals isoin ten se or hyperin ten se lesion on T1
an d T2. CT or MRI w ith con t rast is m ore h elpful.
Oth er in t raocular t u m ors, m et astasis, m u cocele, an d lym ph om a
Management
Pat ien ts w ith progressive field loss requ ire t reat m en t by a n eu rosu rgeon . Treat-
m en t requires su rger y follow ed by radioth erapy.
Blepharospasm
Bleph arospasm is a t ype of facial dyston ia, m ain ly affect ing fem ales (2:1) an d as-
sociated w ith ton ic spasm s of orbicu laris ocu li ( Fig. 13.14 ).
Fig. 13.14 Blepharospasm.
Benign (Essential) Blepharospasm

Bleph arospasm can be a ver y disabling con dit ion in term s of vision an d social life .
Presentation
Bleph arospasm m ore com m on ly affects w om en in th e older age group. Th is is a
t ype of facial dyston ia in w h ich th ere is idiopath ic ton ic con t ract ion of th e orbi-
cularis oculi. If it is secon dar y to any ocular path ology (corn eal or conjun ct ival
foreign body, t rich iasis, bleph arit is, dr y eyes) th en it is called secon dar y bleph a-
rospasm . Th ere is a bilateral involu n t ar y lid closu re th at m ay be precipit ated by
st ress, fat igu e, or social in teract ion s. Th is is alw ays bilateral. It disappears during
sleep. Secon dar y ocular ch anges like ptosis or en t ropion can occu r.
Th is can be differen t iated from h em ifacial spasm , w h ich does n ot disappear dur-
ing sleep .
Hem ifacial spasm , facial m yokym ia, t rigem in al n euralgia, Parkin son disease, pro-
gressive supran uclear palsy, m u lt ip le sclerosis, st roke, Tou ret te syn drom e, an d
tardive dyskin esia
Management
Bot u lin u m toxin is given as m ult iple inject ion s on th e up per an d low er lid. Th e
effect gen erally last s for 3 m on th s. In cases of secon dar y bleph arospasm , t reat th e
un derlying cau se th at is precipitat ing th e bleph arospasm .
Oth er t reat m en t opt ion s are m edical (e.g., ben zodiazepin e) or su rgical (e.g., m y-
ectom y).
Hemifacial Spasm
Th is is a ton ic clon ic sp asm of th e m u scu lat u re th at occu rs even du ring sleep.
Presentation
Th e con dit ion u su ally affect s th e younger age grou p. It is th ough t to be caused by
irrit at ion of th e root of th e seven th cran ial n er ve by a com pressive lesion . MRI of
th e cerebellopon t in e angle sh ould be obtain ed to rule ou t t u m or.
Bleph arospasm , facial m yokym ia, t rigem in al n euralgia, Parkin son disease, pro-
gressive supran uclear palsy, m u lt ip le sclerosis, st roke, Tou ret te syn drom e, an d
tardive dyskin esia
Management
Man agem en t in clu des obser vat ion , bot ulin um toxin inject ion , or n eurosu rgical
decom pression of th e seven th n er ve (Jan n et t a procedure).
Myasthenia Gravis
Myasth en ia result s from dysfun ct ion of th e n eu rom uscu lar jun ct ion caused by au-
toim m un it y. It is a ch ron ic au toim m u n e disorder associated w ith a redu ced n u m -
ber of acet ylch olin e receptors n eurom u scular jun ct ion s result ing in w eakn ess an d
fat igabilit y of m uscle. Ocular m yasth en ia m ost com m on ly presen t s w ith diplopia,
ptosis, or both , w h ich are variable an d ch aracterist ically w orse tow ard th e en d of
th e day. Seru m an t ibodies to acet ylch olin e receptors are detected in 90% of th e
pat ien ts w ith gen eralized m yasth en ia, but on ly 50% w ill be detected in ocu lar m y-
asth en ia. Neon atal form s of m yasth en ia gravis occu r in 10 to 15% of ch ildren born
to m oth ers w ith m yasth en ia gravis becau se of th e placen tal t ran sfer of an t ibodies
to Ach (Acet ylch olin e) receptor.
The im pairm ent of the neurom uscular conduction causes weakness and fatigue of
the skeletal m usculature but not of cardiac and involuntary m uscles. The disease af-
fects fem ales tw ice as com m only as m ales and m ay be ocular, bulbar, or generalized.
Presentation
Myasth en ic sign s an d sym ptom s are variable an d ten d to w orsen w ith fat igue an d
st ress.
Fat igabilit y : Du ring test ing for lid fat igue, th e pat ien t is asked to look up w ith ou t
blin king at th e exam in er’s h an d for 1 to 2 m in utes. Lid fat igu e on prolonged
upgaze is perh aps th e m ost frequen tly elicited sign ( Fig. 13.15 ).
Peek sign : W h en th e pat ien t is asked to close th e lids gen tly, on e or both in ad-
ver ten tly open sligh tly or p eek.
Absent Bell’s Phenom enon : Th ere can an absen ce of Bell ph en om en on .
Cogan lid t w itch : After prolonged dow ngaze refixat ion to th e p rim ar y posit ion
result s in oversh oot ing of th e u pper lid.
Upper lid hop : Hop of th e upp er lid occu rs on looking to th e side.
Myasthenic ptosis: W hen unilateral is associated w ith contralateral lid retraction.
Oscillatory m ovem ent s: If on e eyelid is elevated m an u ally as th e pat ien t looks
up, th e fellow eyelid w ill sh ow fin e oscillator y m ovem en t s.
Ice pack test : Th e degree of ptosis im proves after th e ice pack is placed on th e
eyelid for 2 m in u tes. Th e test is n egat ive in n on m yasth en ic ptosis.
Fig. 13.15 Myasthenia gravis.
Diplopia : Th is is ver y frequ en tly ver t ical, alth ough any of th e m u scles can be
involved. Th e pupil is n ot involved. A pseu doin tern u clear oph th alm oplegia can
occur.
Saccadic abnorm alit ies : Abn orm alit ies su ch as hypom et ric large saccades,
hyperm et ric sm all saccades, qu iver m ovem en t s, an d hyperfast saccades can
occur.
Isolated or com bin ed palsies of th e th ird, four th , sixth , or seven th cran ial n er ves;
decom pen sated st rabism u s; thyroid disease; Eaton -Lam ber t m yasth en ic syn -
drom e; bot u lism ; ch ron ic progressive oph th alm oplegia; m yoton ic dyst rophy
Management
Tensilon test
In t raven ous inject ion of edroph on iu m is th e gold st an dard for th e diagn osis of oc-
ular m yasth en ia. Edroph on iu m is a sh or t-act ing an t ich olin esterase th at in creases
th e am ou n t of acet ylch olin e available at th e n eurom u scular ju n ct ion . In m yasth e-
n ia th is resu lts in t ran sien t im provem en t of sym ptom s an d sign s such as w eak-
n ess, ptosis, an d diplopia. Un com m on com plicat ion s in clude bradycardia, loss of
con sciousn ess, an d even death . Lacrim at ion , salivat ion , an d abdom in al cram ps are
m en t ion ed as com m on m in or side effects. Th e test sh ould be don e w ith a resu sci-
tat ion t rolley in h an d in case of sudden cardiorespirator y arrest .
Object ive baselin e m easu rem en t of ptosis or dip lopia w ith a Hess ch ar t sh ould
be t aken .
In t raven ous inject ion of at ropin e 0.3 m g is given to m in im ize m u scarin ic side
effect s.
In t raven ous dose of 0.2 m L con tain ing 2 m g of edroph on ium hydroch loride is
given . If defin it ive im provem en t is n oted th e test can be term in ated.
If n o respon se th en th e rem ain ing 0.8 m L of 8 m g is injected after 60 secon ds if
th ere is n o adverse react ion . Th e respon se lasts on ly for 5 m in utes.
Per verse react ion such as w orsen ing of th e st rabism u s or a paradoxical re-
spon se su ch as righ t hypert ropia becom ing a left hyper t ropia after th e inject ion
is con sidered posit ive by som e.
Neostigmine Test
In t ram u scu lar inject ion of n eost igm in e is u seful in ch ildren . Th e effect last s for
15 m in utes to p eak an d last s for on ly 30 m in utes.
Presen ce of acet ylch olin e receptor an t ibodies is vir t ually diagn ost ic of m yasth e-
n ia gravis.
On e lect rom yography, repet it ive st im ulat ion of a single m u scle fiber w ill sh ow
a decrem en tal respon se.
Sleep Test
Usefu l in n eon ates an d in fan t s. Th ere w ill be im provem en t after sleep.
Im aging th e ch est w ith CT or MRI for th e p resen ce of thym om a
Optica l Treatment
Becau se of th e variabilit y of sign s an d sym ptom s, it is difficu lt to t reat . For bin -
ocu lar diplopia occlusion of on e eye can h elp, but it forces th e pat ien t to view
m on ocularly.
Fresn el prism can be t ried if th e ocular deviat ion is stable for w eeks.
Cru tch glasses are h elpful in th e case of ptosis.
Medica l Trea tment
An t ich olin ergic drugs such as pyridost igm in e (60 m g) th ree t im es a day. On e
m u st be aw are of ch olin ergic crisis if too m u ch of pyridost igm in e is given .
Th e pat ien t sh ou ld be told to stop if bu lbar sym ptom s or gen eralized w eakn ess
occu rs.
Cor t icosteroids are used along w ith pyridost igm in e. Th e pat ien t sh ou ld be
m ain tain ed on steroids for m on th s before t apering slow ly t apering. W h en th e
pat ien t is m ain t ain ed on a low dose of steroids, th ere can be a relapse or un -
m asking of gen eralized m yasth en ia.
Im m un osupp ressan t azath iop rin e is effect ive again st m yasth en ia at a dose of 2
to 3 m g/kg/day.
Cyclosporin e A, plasm aph eresis, m ycoph en olate, an d in t raven ous gam m a glob-
ulin can also be u sed in gen eralized m yasth en ia.
Surgica l Trea tment
Thym ectom y is ver y effect ive for ocu lar m yasth en ia. Th e resu lts of thym ectom y
for gen eralized m yasth en ia are ver y favorable, w ith ~35%en tering com plete re-
m ission an d 50% im proving.
Eyelid surger y or ptosis an d eye m u scle surger y for diplopia are con sidered on ly
if it is st able for a few m on th s an d as a last resor t .
An t ibodies to acet ylch olin e receptors are th e et iology beh in d th e disorder.
Eye Movement Disorders
Nystagmus
Nyst agm us is a rhyth m ic to-an d-fro oscillat ion of th e eyes. In nyst agm u s, gen erally
th e m ovem en t in slow p h ase is in on e direct ion an d th e fast ph ase is in th e oppo-
site direct ion . Th e fast ph ase of nystagm u s is m ediated by th e saccadic system u n -
der all con dit ion s. On e or m ore of th e oth er system s w ill m ediate th e slow ph ase.
It is im port an t to rem em ber th at nystagm us is given its direct ion based on the fast
phase. Th is m ean s th at if w e say a nyst agm u s is to th e righ t , th e fast ph ase of th e
nyst agm us is to th e righ t . Bu t act u ally, th e im por t an t poin t of nystagm u s is th e
slow ph ase. So act u ally, nystagm us should be given its direct ion depending on the
slow phase—but this is not done. An abn orm alit y in th e slow ph ase is m ore sign ifi-
can t . But by conven t ion th e direct ion of nystagm u s is described by th e fast ph ase
( Fig. 13.16A). Th e n ull zon e is th e field of gaze in w h ich th e in ten sit y of nystagm u s
is m in im al, w h ereas in th e n eut ral zon e a reversal of direct ion of jerky nyst agm u s
occu rs an d any of several bidirect ion al w aveform s, pen du lar nyst agm us, or n o nys-
tagm us m ay be presen t .
Laten t nystagm u s is n ot n orm ally presen t w h en both eyes are open bu t is elic-
ited on covering eith er eye. In th e classic case th e nystagm u s ap pears on closing
on e eye. Bilateral jerky nystagm u s is seen w ith th e fast ph ase tow ard th e un cov-
ered eye. An oth er con dit ion , called m an ifest laten t nyst agm u s, occu rs in pat ien ts
w ith am blyopia or st rabism u s w h o, alth ough view ing w ith both eyes open , are
fixing m on ocu larly. Again th e fast ph ase is tow ard th e direct ion of th e in ten ded
view ing eye. Th e ph en om en on of laten t nyst agm us is par t icu larly eviden t w h en
th e visu al acuit ies of th e t w o eyes are un equal. Som et im es if on e eye h as ver y poor
vision , on covering th e bet ter eye, in stead of nyst agm us a conjugate deviat ion of
both eyes occu rs tow ard th e side of th e closed eye. Th is is called th e latent devia-
t ion of Kestenbaum . Th e cause of laten t nyst agm us is u n kn ow n . It cou ld be due to
lack of coordin at ion of th e supran uclear cen ters. It cou ld also be du e to th e fact th at
th e nystagm u s w as laten t bu t kept in ch eck by convergen ce so th at abolit ion of th e
im pu lse to bin ocu lar convergen ce allow ed it to becom e m an ifest .
Presentation
Types of Nystagmus
Pendular nystagm us: Th is con dit ion con sists of an u n dulator y m ovem en t of
equ al speed an d am plit ude in both direct ion s.
Jerk y nystagm us: Jerky nystagm u s dem on st rates a biph asic rhyth m w h erein a
slow m ovem en t in on e direct ion is follow ed by a rapid saccadic ret u rn to th e
origin al posit ion . Jerky nyst agm u s usually in creases in am p lit ude w ith gaze in
th e direct ion of th e fast com pon en t . Th is is called Alexander’s law .
Micronystagm us: Micronyst agm us a nystagm u s th at is subclin ical; it is in capa-
ble of being detected w ith ordin ar y clin ical tests becau se of it s ext rem ely sm all
am plit ude. Th e diagn osis is apparen t by th e fixat ion pat tern , w h ich sh ow s a
regu lar jerky t ype of nystagm u s w ith fast an d slow ph ases of ext rem ely sm all
am plit ude w ith in th e p arafoveal areas so th at it m ay be revealed on ly by a care-
ful exam in at ion w ith th e visu oscope or direct oph th alm oscope.
Fig. 13.16 (A) Types of nystagmus. (B) Miner’s nystagm us.

Fig. 13.16 (Continued) (C) Methods to treat nystagmus.
Gra des of Nystagmus

Nyst agm us is divided in to th ree grades:
Grade I: Jerky nyst agm us is eviden t on ly in th e direct ion of th e fast ph ase (i.e.,
on conjugate deviat ion to on e side).
Grade II: W h en in addit ion , it is eviden t in th e prim ar y posit ion .
Grade III: Eviden t in all p osit ion s of th e eyes.
Pathologica l Ocula r Nystagmus
Am aurot ic nystagm us: Nyst agm u s of p en dular or rarely jerky t ype m ay occur in
th ose w h o h ave been blin d for a long t im e. Th e nyst agm us is som et im es con -
stan t , an d at oth er t im es it appears on ly w h en at ten t ion is arou sed.
Am blyopic nystagm us: Th is is du e to a defect in cen t ral vision in both eyes,
w h ich preclu des th e n orm al developm en t of th e fixat ion reflex.
Spasm us nutans: In th is th e nyst agm us occurs w ith h ead n odding. It is also
called Dunkel syndrom e. It gen erally occu rs w ith in th e first year of life. Th e
cause appears to be difficu lt y in m ain tain ing fixat ion , w h ich is frequ en tly asso-
ciated w ith in adequate ligh t . Th ere is also in su fficien t con t rol due to in stabilit y
of th e m otor cort ical cen ters in early life.
Miner’s nystagm us : Th is is an acquired occupat ion al disease of th e n er vous sys-
tem w ith special m an ifest at ion s in th e ocu lar m otor app arat us, occurring in
w orkers in coal m in es ( Fig. 13.16B). Basically it is due to lack of illum in at ion .
In th e early laten t stage th ere is a m ild nyst agm us. Th e acute stage is ch aracter-
ized by t rem bling of th e h ead an d h an ds, w ith m arked nystagm u s, an d a pa-
th ogn om ic post ure of th e h ead being th row n back. Th e late psych opath ic st age
is ch aracterized by cram ps, h eadach es, t rem ors, an d in som n ia. Th e nyst agm us
is gen erally pen du lar in t yp e in th e prim ar y posit ion bu t frequen tly ch anges
to th e jerky t ype on lateral gaze. Th e t reat m en t of th is con dit ion is to give th e
pat ien t surface w ork an d im prove th e gen eral h ealth .
Vest ibular nystagm us : Th e sem icircu lar can als are th ree fin e t ubes arranged in
th e ear. Th e lateral sem icircular can al is t ilted up 30 degrees. Norm ally th e eyes
at rest are in th e prim ar y posit ion . Im pu lses go from each sem icircu lar can al
to th e respect ive vest ibular n u clei. From h ere, th e im pu lse goes to th e oppo-
site pon t in e gaze cen ter, w h ich in t urn con n ect s to th e sam e side sixth -n er ve
n ucleus an d opposite side th ird-n er ve n u cleu s. Th e im pu lses th u s reach th e m e-
dial an d lateral rect i an d th e eyes are balan ced an d in th e p rim ar y posit ion .
Cerebellar nystagm us : Th e exact m ech an ism of cerebellar nyst agm u s is n ot
kn ow n . W h en nystagm u s occurs it is opposite th at foun d in a vest ibu lar lesion .
In a righ t-sided vest ibular lesion , th e slow ph ase of th e nyst agm us is to th e righ t
an d th e fast p h ase to th e left . Th is m ean s th e nystagm u s is to th e left , in oth er
w ords opposite th e side of th e lesion . In cerebellar disease, th e fast ph ase of th e
nyst agm us is on th e sam e side of th e lesion . So, if th ere is a righ t-sided cerebel-
lar lesion , th e fast p h ase of th e nystagm u s is tow ard th e righ t side. Th is could
be du e to th e floccu lo-oculom otor path w ay, w h ich w orks in th e reverse of th e
vest ibular path w ay. Th e left vest ibu lar path w ay push es th e eyes to th e righ t ,
w h ereas th e left floccu lo-ocu lom otor p ath w ay from th e left cerebellu m push es
th e eyes to th e left .
Cent ral nystagm us: In cen t ral nyst agm us, th e nyst agm u s is of th e jerky t ype. It is
occasion ally presen t w h en th e eyes are at rest bu t u su ally develops on ly w h en
th ey are deviated to on e or th e oth er direct ion . Th e nystagm u s is sym m et rical.
Th is m ean s th at th e m ovem en t st art s at th e sam e angle of eccen t ricit y an d h as
approxim ately th e sam e excursion w h eth er th e gaze is directed to on e or th e
oth er side.
Volun t ar y eye m ovem en t s, dysm et ria, flut ter, opsoclon u s, m yoclon u s, spasm us
n ut an s, opt ic n er ve gliom a, superior obliqu e m yokym ia, m yasth en ia gravis (qu iv-
erlike m ovem en t s), ocular bobbing
Management
Man agem en t can con sist of t reat ing th e cau se, u se of prism s, or su rger y in w h ich
th e Faden operat ion is perform ed. Th e m eth ods to t reat nystagm u s are sh ow n in
Figure 13.16C. Th e t reat m en t can be gen eral, w h ere th e cau se is t reated, or spe-
cific, w h ich can be m edical or surgical. In m edical t reat m en t on e can im prove th e
visu al acu it y by using prism s, base ou t , to sim u late fusion al convergen ce. On e can
use prism s to elim in ate an om alous h ead post ures also. For a h ead t urn to th e left ,
th e n eut ral zon e is in dext roversion an d a p rism base ou t before th e righ t eye an d
base in before th e left eye w ill sh ift th e eyes conjugately along w ith th e n eut ral
zon e tow ard th e prim ar y posit ion . On e can also u se occlusion , in w h ich par t ial
occlusion of th e sou n d eye w ith a n eu t ral den sit y filter decreases visu al acuit y in
th e fixat ing eye to a level below th at of th e am blyopic eye bu t n ot dark en ough to

elicit th e nyst agm us. Su rgically on e can perform th e Faden operat ion , in w h ich th e
requ ired m u scle creat ing th e nyst agm us is sut ured to th e sclera at th e equator.
Internuclear Ophthalmoplegia
Lesion s affect ing th e path w ays by w h ich th e variou s ocu lar n u clei are lin ked to-
geth er [i.e., lesion s of th e m edial longit udin al fascicu lus (MLF) or m edial longit udi-
n al bu n dle] produ ces in tern uclear oph th alm oplegia. Th e MLF con n ects th e th ird-
n er ve an d th e sixth -n er ve n uclei. If a lesion occu rs in th is th ere is preven t ion of
th e h arm on iou s coordin at ion of th ese n u clei in p rodu cing conjugate m ovem en t s.
So on e eye carries ou t a volu n t ar y m ovem en t of gaze, w h ereas th e oth er eye does
n ot , th us leading to failure of th e conjugate m ovem en t (i.e., both eyes m oving in
th e sam e direct ion ). Th is leads to a m isalign m en t of th e eyes an d th us to diplop ia.
Th is feat ure differen t iates th e in tern uclear palsies from th e oth er supran uclear le-
sion s.
Depen ding on w h eth er th e lesion is un ilateral or bilateral, various cau ses of in -
tern uclear oph th alm oplegia are p resen t . Th e com m on cau ses are vascu lar lesion s
or m u lt iple sclerosis ( Fig. 13.17A).
Presentation
In tern uclear op h th alm oplegia (INO) m ay presen t as th ree t yp es as en um erated
below :
Type I: In th is t ype, th e lesion is n ear th e th ird cran ial n er ve n uclei, in clu ding
th e convergen ce area ( Fig. 13.17B). Essen t ially th ere is paralysis of both m edial
rect i. Th e im pu lses com ing from th e pon t in e gaze cen ter go to th e sixth -n er ve
an d th ird-n er ve n u clei. Becau se th e con n ect ion s to th e sixth -n er ve n u clei are
n ot affected n o dist urban ce is presen t in lateral rect us m ovem en ts. Th e eyes are
divergen t ow ing to bilateral involvem en t of th e m edial rect i an d th ere is loss of
convergen ce. It occu rs in hyper ten sive brain stem lesion s an d m u lt iple sclerosis.
Divergen ce m ay be com plicated by skew deviat ion of th e eyes in w h ich on e eye
m ay be u p an d ou t an d th e oth er eye looks dow n an d ou t . Th ere m ay be a see-
saw nystagm u s presen t in w h ich th e eyes jerk u p an d dow n altern ately.
Type II: In th is relat ively com m on variet y of INO, th e MLF is dam aged an d th e
m edial rect i fail to m ove syn ch ron ou sly w ith th e lateral rect i ( Fig. 13.17C) on
at tem pted lateral gaze to eith er side. Yet w h en each eye is tested alon e, th e m e-
dial rect i fu n ct ion is eviden t but in com plete. Test th is by covering th e abdu ct ing
eye an d m aking th e adduct ing eye follow th e finger. In t ype II INO convergen ce
is n orm al because th e convergen ce area is n ot affected. Th is occurs in m u lt iple
sclerosis, pon t in e gliom a, or en ceph alit is
Type III: Th e th ird variet y of INO occu rs in m u lt iple sclerosis. In th is t ype of
INO ( Figs. 13.17D,E), n on e of th e eye abdu cts com p letely, w h ereas addu ct ion
is com plete. Th e relay to th e sixth cran ial n er ve n uclei is affected on both sides.
If you test th e eye in dividually by closing th e oth er eye, th e eye w ou ld abdu ct ,
differen t iat ing th is from an in fran u clear lesion (sixth -n er ve palsy).
Fig. 13.17 (A) Causes for internuclear oph-

thalmoplegia (INO). (B) Type I INO. (C) Type
C II INO.
Myasth en ia gravis, orbit al disease, oth er supran uclear m ovem en t disorders
Management
Work up an d m an age according to th e cause.
Fig. 13.17 (Continued) (D) Type III

INO. (E) Eye m ovements. E
One -and-a-Half Syndrome

On e-an d-a-h alf syn drom e is also kn ow n as paralyt ic pont ine exotropia.
Presentation
In th e prim ar y posit ion th e eye th at is opposite th e side of th e lesion is exot ropic.
Th e eye on th e sam e side of th e lesion looks st raigh t ah ead. Th e lesion is in th e
pon t in e param edian ret icu lar form at ion (pon t in e gaze cen ter) or sixth -n er ve n u-
cleus an d ipsilateral m edial longit u din al fascicu lus (Fig. 13.18A). From th e figu re
on e w ill un derstan d th at on ly th e sixth n er ve on th e side opposite th e side of th e
lesion w ill w ork. Th e pat ien t is n ot able to gaze w ith eith er eye tow ard th e side of
th e lesion an d is n ot able to adduct th e eye on th e side of th e lesion ( Fig. 13.18B).
Th is is w hy th is is called on e-an d-a-h alf syn drom e, because on on e side gaze is
absen t an d on th e oth er side on ly h alf th e gaze m ovem en t is presen t .
Myasth en ia gravis, orbit al disease, oth er supran uclear m ovem en t disorders
Fig. 13.18 (A) One -and-a-half syndrome.

(B) One -and-a-half syndrome. MLF, medial
longitudinal fasciculus; PGC, pontine gaze
B center.
Management
Progressive Supranuclear Palsy

In progressive supran uclear palsy th ere is loss of n er ve cells, vascu lar degen era-
t ion , an d glial react ion s in th e basal ganglia an d m idbrain .
Presentation
Th e first m an ifest at ion of progressive su pran u clear palsy is an in abilit y to m ake
ver t ical saccades, par t icularly dow nw ard saccades. At th is poin t , th e p at ien ts bang
th eir sh in s, eat off on ly th e top par t of th eir plates, an d com p lain of being u n able
to read (th ey can n ot look dow n ). As th e disease progresses, h orizon tal fast m ove-
m en ts becom e involved as w ell. Even t u ally, occular m ovem en t s cease to be sm ooth
an d rapid. Pu rsuit m ovem en t s becom e ch aracterist ically cogw h eel.
Ch ron ic progressive extern al oph th alm oplegia, m yasth en ia gravis, brain stem le-
sion s, cavern ou s sin u s syn drom e
Management
No t reat m en t is available except su ppor t ive care.
Parinaud Syndrome
Th ere are several m an ifest at ion s of lesion s in th e collicular area. Th e sign s are
th ough t to be cau sed by pressure an d distor t ion of u n derlying st ru ct u res in th e
m idbrain an d n ot by dam age to specific path w ays t raversing th e colliculi. Th e gen -
eral n am e for th e clin ical pict u re produced is kn ow n as Parinaud syndrom e.
Presentation
Any com bin at ion of im paired u pw ard gaze, im paired dow nw ard gaze, pu pillar y
abn orm alit ies, or loss of accom m odat ion reflex can occu r. In gen eral, loss of u p-
w ard gaze associated w ith dilated pu pils th at are fixed to ligh t suggests a lesion
at th e level of th e su perior collicu lu s. Loss of dow nw ard gaze, n orm al p upillar y
react ion s to ligh t , an d loss of convergen ce suggest th at th e lesion is sligh tly low er
in th e area of th e in ferior colliculu s. It cou ld be du e to lesion s of th e pin eal glan d,
m u lt iple sclerosis, vascu lar diseases, or Wern icke en ceph alopathy.
A special t ype of nystagm u s, ret ractor y nyst agm us, is presen t . Th is is a ver y rare
sign of disease in th e collicular area an d con sist s of an inw ard an d ou t w ard m ove-
m en t of both eyes w h en th e pat ien t at tem pts to look upw ard. Presu m ably, it is
produ ced by all th e ext raocular m u scles act ing sim ult an eou sly—jerking th e globe
back in to th e orbit or at tem pted u pw ard gaze—in an at tem pt to overcom e th e in -
abilit y to look u pw ard.
Thyroid orbitopathy, cavern ous sin us syn drom e, m yasth en ia gravis
Management
Chronic Progressive External Ophthalmoplegia

Presentation
Th e clin ical feat u res are th e involvem en t of th e u pgaze an d th en th e lateral m ove-
m en ts an d m ay later be affected in all gaze result ing in a fixed globe. Becau se th e
m u scle involvem en t is sym m et rical, diplopia does n ot usu ally occu r. Th ere is also
slow ly p rogressive bilateral ptosis.
Kearn s-Sayre syn drom e is a m itoch on drial cytopathy in h erited from th e m oth er.
It is ch aracterized by pigm en t ar y ret in opathy w ith coarse gran ularit y. Con duct ion
defects of th e h ear t can occu r. Hear t block m ay result in sudden death . Oth er fea-
t u res are sh or t st at u re, m uscle w eakn ess, cerebellar ataxia, n eu rosen sor y deafn ess,
m en tal h an dicap, an d delayed pu ber t y.
Oth er causes of paralyt ic an d rest rict ive st rabism us, m yop ath ies, m yasth en ia gra-
vis, supran uclear palsies
Management
Treat th e associated con dit ion s. Use lu brican ts for th e exposure keratopathy an d
base-dow n prism s w ith in reading glasses if th e dow ngaze is rest ricted. Pacem aker
m ay be requ ired for th e cardiac con dit ion . In oculop h ar yngeal dyst rophy, dys-
ph agia, an d recu rren t asp irat ion s m ay w arran t cricoph ar yngeal su rger y. Gen et ic
cou n seling is n eeded.
14 Ophthalmic Pharmacology
Jam es M. Hill, Jean T. Jacob, Lori Vidal Denham , Blake A. Booth ,
Duncan A. Friedm an , Jeffery A. Hobden , Andrea T. Murina ,
Marie D. Acierno , Herbert E. Kaufm an , and Donald R. Bergsm a
Tw o gen eralizat ion s can be m ade abou t ph arm acological agen ts. First , all drugs
h ave m ore th an on e effect . Secon d, all drugs can be toxic. Cert ain ly, m ost drugs
h ave a p rim ar y m ech an ism of act ion , an d m any drugs are safe an d w ell tolerated.
How ever, ever yon e sh ould be aw are of th e possible u n in ten ded con sequ en ces of
any p h arm acological agen t . Alth ough m ost oph th alm ic drugs are adm in istered
th rough topical applicat ion , even w ith th is t ype of drug deliver y, both topical an d
system ic toxicit y can occur. Th is review h igh ligh ts th e m ost frequen t an d m ost
severe toxicit ies bu t does n ot give an exh aust ive list ing of all side effect s of th ese
oph th alm ic drugs. Th e gen eric n am e an d th e p rim ar y t rade n am e of th e dr ugs are
given . W h en approp riate, w e h ave based th e review on th e class of th e drug an d
h ave selected agen ts th at are m ost u sed to be in cluded in th is review. Fin ally, th is
review is n ot m ean t to be an exh au st ive review of all oph th alm ic drugs but out-
lin es th e m ost im por tan t an d m ost frequ en tly u sed agen t s.
Antibacterial Agents
Topical Antibacterial Agents

Treat m en t of ocu lar in fect ious diseases (Fig. 14.1 ) involves eith er topical adm in -
ist rat ion of an t im icrobial agen t s (for corn eal, conju n ct ival, an d lid m argin in fec-
tion s) or direct inject ion of th erapeu t ic agen ts in to th e eye it self (for posterior eye
in fect ion s).
Table 14.1 list s com m ercially available an t ibacterial agen t s u sed topically as
prophylaxis for surgical procedu res or for t reat ing corn eal u lcers, conju n ct ivit is, or
m argin al lid disease. Most of th ese an t ibiot ics are broad spect r um , covering both
gram -p osit ive an d gram -n egat ive path ogen s, yet som e are m ore act ive again st on e
grou p versus th e oth er. An t ibiot ics su ch as polym yxin B, w h ich is effect ive on ly
Fig. 14.1 Bacterial corneal ulcer

with hypopyon. (Courtesy of Nibaran
Gangopdhyay)
405
Table 14.1 To pical Antibacte rial Age nts
Trade Antibacterial
Nam e Agent Chem ical Class Fo rm ulatio n
Bleph-10 Sulfacetamide Sulfonamide 10% solution

sodium
Sulamyd 10% ointment
Generic Bacitracin Peptide 500 Units/g ointment
Generic Erythromycin Macrolide 0.5% ointment
Genoptic Gentamicin Aminoglycoside 0.3% solution
Garamycin 0.3% ointment
Tobrex Tobramycin Aminoglycoside 0.3% solution
0.3% ointment
Ciloxan Cipro oxacin Second-generation 0.3% solution
uoroquinolone 0.3% ointment
Ocu ox O oxacin Second-generation 0.3% solution
uoroquinolone
Quixin Levo oxacin Third-generation 0.5% solution
uoroquinolone
Zymar Gati oxacin Fourth-generation 0.3% solution
uoroquinolone
Vigamox Moxi oxacin Fourth-generation 0.5% solution
uoroquinolone
again st gram -n egat ive bacteria, are com bin ed w ith oth er an t ibiot ics to in crease
th e spect rum of coverage ( Table 14.2 ). Som e of th ese an t ibiot ics are form ulated as
aqueous drops or oin t m en t . Oth ers com bin e an t iin flam m ator y agen t s to reduce a
h ost in flam m ator y respon se th at can be as dam aging to ocular t issues as bacterial
proteases an d toxin s ( Table 14.3 ).
Table 14.2 Co m binatio ns o f Antibacterial and Antiin am m ato ry Drugs w ith

Po lym yxin B
Trade Antibacterial
Nam e Agen t(s) Chem ical Class Fo rm ulatio n
Polysporin Bacitracin Peptide 500 Units/g ointment

Neosporin Neomycin + Aminoglycoside + 3.5 mg/g ointm ent
ointment bacitracin peptide 400 Units/g ointm ent
Neosporin Neomycin + Am inoglycoside + 10,000 Units;
solution gramicidin peptide 1.75 mg/mL solution
0.025 mg/mL solution
Terramycin Oxytetracycline Tetracycline 5 mg/g ointment
Polytrim Trim ethoprim Diaminopyrimidine 1 m g/mL solution
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14 Ophthalm ic Pharm acology 407
Table 14.4 Inje ctable Antibacterial Agents
Antibacterial Trade Ro ute o f

Agent Chem ical Class Nam e Do sage Injectio n
Gentamicin Aminoglycoside Garamycin 20 mg/0.5 mL Subconjunctival

Am ikacin Am inoglycoside Amikin 250 µg/0.1 mL Intravitreal
Vancomycin Glycopeptide Vancocin 2 mg/0.2 mL Intravitreal
25 m g/0.5 m L Subconjunctival
Ceftazidim e Third-generation Fortraz 2.2 mg/0.1 mL Intravitreal
cephalosporin
Injectable Antibacterial Agents

Bacterial in fect ion s of th e posterior por t ion of th e eye (en dop h th alm it is) are
t reated w ith an t ibiot ics injected subconju n ct ivally or in t ravit really. Curren t an -
t ibiot ic t reat m en t regim en s are listed in Table 14.4 . Dosage recom m en dat ion s for
th ese an t ibiot ics sh ould n ot be exceeded to avoid in ducing ret in al toxicit y. Adm in -
ist rat ion of th ese an t ibiot ics is con t rain dicated w h ere th ere is a kn ow n hypersen -
sit ivit y to th ese agen ts.
Ocular Antivirals
More th an 45 years ago, Kaufm an first repor ted th e u se of idoxuridin e (Herp lex)
for th e t reat m en t of h erpes sim plex viru s (HSV) epith elial kerat it is ( Fig. 14.2 ).
Sin ce th en , 50 an t ivirals h ave been licen sed in th e Un ited St ates; 10 n ew an t ivirals
an d com bin at ion s h ave been in t rodu ced in th e last 10 years. Acyclovir (ACV) (Zo-
virax) h as been called th e “pen icillin of an t ivirals.” Th ere are th ree an t ivirals th at
h ave purin e st r uct ures sim ilar to ACV: valacyclovir (Valt rex), fam ciclovir (Fam vir),
an d gan ciclovir (Cytoven e, Vit raset). Idoxuridin e (Herp lex) an d t rifluorothym idin e
(Viropt ic) h ave ver y sim ilar pyrim idin e st ru ct u res. Viropt ic is th e drug of ch oice for
HSV pat ien ts. Table 14.5 is a sum m ar y of th e m ost im por tan t an t ivirals an d th eir
th erapeu t ic use in viral in fect ion s of th e eye.
Fig. 14.2 Herpes simplex viral

keratitis with large dendrite in late
untreated stage.
T
a O
D
H
C
V
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, c y t o m e g a l o v i r u s ; V Z
V , v a r i c e l l a z o s t e r v i r u s .
Table 14.6 An tifungal Agents to Treat Fungal Keratitis and Endo phthalm itis
Antifungal Chem ical Trade Ro ute o f

Agent Class Nam e Do sage Adm inistratio n
Natamycin Polyene Natacyn 5% suspension Topical

Amphotericin Polyene Fungizone 0.1–0.5% solution Topical
B 0.8–1.0 mg Subconjunctival
5 µg Intravitreal
Miconazole Imidazole Micatin 1% solution Topical
5–10 mg Subconjunctival
10 µg Intravitreal
Voriconazole Triazole Vfend 1% solution (made Topical
from IV solution;
dosing ranges from
hourly to t wice a
day as determined
by clinician.
200 mg t wice daily Oral
3–6 mg/kg every IV*
12 h
*Becau se of poten t ial side e ect s an d toxicit y, th e p ract it ion er sh ould con su lt the Physicians’ Desk
Reference for p ossible dosage adju st m en ts an d w arn ings.
Abbreviat ions: IV, in t ravenous.
Antifungal Agents
Th ere is on ly on e com m ercially available topical an t ifu ngal drug (Table 14.6 ): n a-
t am ycin (Nat acyn ). Th e rem ain ing an t ifungal agen t s in Table 14.6 m u st be extem -
poran eou sly com poun ded for topical, subconju n ct ival, or in t ravit real use. All of
th ese agen ts are effect ive again st yeast an d filam en tou s fu ngi. Because of poor
pen et rat ion , all of th ese an t ifungal agen t s adm in istered topically for corn eal in fec-
t ion s are dosed at h ou rly or 2-h our in ter vals for th e first 2 or 3 days. Th e dosing
sch edu le m ay be exten ded to a drop six to eigh t t im es a day, depen ding on h ow
th e in fect ion is resolving. Drug pen et rat ion in to corn eal t issu e im proves if th e epi-
th elium is absen t . Adverse react ion s to topical an t ifungal agen ts are lim ited to lo-
cal hypersen sit ivit y react ion s (e.g., conjun ct ival ch em osis an d hyperem ia, foreign
body sen sat ion ).
Antiparasite Agents
Exogen ou sly acqu ired parasite in fect ion s of th e eye are cau sed prim arily by am oeba
of th e gen u s Acantham oeba . Th ere are n o ch em oth erapeu t ic agen t s app roved by
th e Un ited States Food an d Drug Adm in ist rat ion specifically for th e t reat m en t of
Acantham oeba kerat it is. How ever, an over-th e-cou n ter arom at ic diam idin e avail-
able in th e Eu ropean Un ion , propam idin e iseth ion ate (Brolen e), h as been sh ow n to
be effect ive w h en com bin ed w ith th e an t ibacterial drug n eom ycin . Propam idin e
iseth ion ate is available as a 0.1% solut ion or as an oin t m en t (0.15%).
Antiinflammatories
An in flam m ator y respon se result s w h en cells are dam aged by m icrobes, p hysical
agen ts, or ch em ical agen t s an d can be ch aracterized by redn ess, pain , h eat , an d
sw elling ( Fig. 14.3 ).
Corticosteroids
Cor t icosteroids com m on ly u sed in oph th alm ic pract ice are sh ow n in Table 14.7 ,
an d are divided in to su bt ypes (sh ort-act ing, in term ediate-act ing, an d long-act ing)
based on th eir durat ion of act ion .
Fig. 14.3 Antiinflammatories are

given to suppress inflammation in
patients with uveitis.
Table 14.7 To pical Co rtico stero ids
Gen eric Nam e Co n cen - Trade Typical Adult

and Preparatio n tratio n Nam e Do sage
Dexam ethasone 0.1% Generic 1–2 drops every hour

sodium phosphate during the day and
solution every 2 h during the
night
Dexam ethasone 0.1% Maxidex Mild disease: 1–2 drops
ophthalm ic suspension 4–6 times per day.
or ointment Severe disease: apply
drops hourly
Fluorom etholone 0.1% Flarex 1–2 drops 2–4 times
acetate suspension daily
Fluorom etholone 0.1% FML and 1 drop 2–4 times daily
ophthalm ic suspension available
generically
0.25% FML Forte 1 drop 2–4 times daily
1
Fluorom etholone 0.1% FML S.O.P. 2 -inch ribbon into the
ophthalm ic ointment conjunctival sac 1–3
times/day
Loteprednol etabonate 0.5% Lotemax 1–2 drops 4 times daily
suspension
0.2% Alrex 1 drop 4 times daily
Medrysone ophthalmic 1% HMS 1 drop every 4 h
suspension
Prednisolone acetate 1% Pred Forte and 1–2 drops 2–4 times
ophthalmic suspension available daily
generically
1% Econopred Plus 2 drops 4 times daily
0.125% Econopred 2 drops 4 times daily
0.12% Pred Mild 1–2 drops 2–4 tim es
daily
Prednisolone sodium 1.0% In amase 1–2 drops up to every
phosphate ophthalm ic Forte and hour
solution available
generically
0.125% In amase Mild 1–2 drops up to every
and available hour
generically
Rimexolone 1% Vexol 1–2 drops 4 times daily
ophthalm ic suspension
Periocular inject ion s are also u sed to deliver cor t icosteroids w h en in flam m a-
tor y con dit ion s are u n respon sive to topical t reat m en ts. Th e m ain in dicat ion s for
periocular inject ion s are t reat m en t of in term ediate or posterior uveit is an d cys-
toid m acu lar edem a. Th e cor t icosteroids t ypically u sed are m ethylpredn isolon e
(Depo-Medrol), t riam cin olon e aceton ide (Ken alog-40), betam eth ason e (Celeston e
Solusp an ), dexam eth ason e (Decadron Ph osph ate), or available gen eric equivalen ts
as sh ow n in Table 14.8 .
Table 14.8 Inje ctable Co rtico stero ids
Generic Nam e and Pre paratio n Co ncentratio n Trade Nam e
Betamethasone sodium 6 mg/mL Celestone Soluspan

Phosphate and betamethasone
acetate injectable suspension
Dexamethasone sodium 4 mg/m L Decadron and
phosphate injection available generically
Methylprednisolone acetate 40 mg/mL and Depo-Medrol and
injectable suspension 80 mg/mL available generically
Triamcinolone acetonide 40 mg/mL Kenalog-40
injectable suspension
Topical Nonsteroidal Antiinflammatory Drugs (NSAIDs)

Topical NSAIDs cu rren tly u sed in oph th alm ology are sh ow n in Table 14.9 an d can
be classed as acet ic acids, propion ic acids, an d a prodrug ar ylacet ic acid.
Table 14.9 To pical No nstero idal Antiin am m ato ry Drugs
Generic Nam e Trade Do sing/

and Preparatio n Nam e Indicatio n Co ncentratio n
Brom fenac sodium Xibrom Postoperative in ammation One drop t wice

solution in patients who have a day/0.09%
undergone cataract
extraction
Diclofenac sodium Voltaren Postoperative in amm ation One drop four
solution following cataract times a day/
extraction and for the 0.1%
temporary relief of pain
and photophobia following
corneal refractive surgery
Flurbiprofen Ocufen Inhibition of interoperative One drop every
sodium solution and miosis ½ hour 2
generic h prior to
surgery/0.03%
Ketorolac Acular Postoperative in amm ation One drop four
trom ethamine and in patients who have times a day/
solution generic undergone cataract 0.5%
extraction and for relief of
ocular itching due to
seasonal allergic conjunctivitis
Acular PF Reduction of ocular pain and One drop four
photophobia following tim es a day/
incisional refractive surgery 0.5%
Acular LS For the reduction of ocular One drop four
pain and burning/stinging times a day/
following corneal refractive 0.4%
surgery
Nepafenac Nevanac For pain and in ammation One drop three
ophthalm ic associated with cataract times a day/
suspension surgery 0.1%
Drug Combinations (Corticosteroids/Antibiotics)
Th e com bin at ion t reat m en t s are m u lt iple-dose su spen sion s, solu t ion s, an d oin t-
m en ts for topical applicat ion th at in clu de cor t icosteroids com bin ed w ith an t ibiot-
ics su ch as gen tam icin , polym yxin B, n eom ycin , tobram ycin , an d sulfacetam ide.
Th e various cor t icosteroid/an t ibiot ic com bin at ion s are sh ow n in Table 14.10 .
Mydriatics and Cycloplegics
Mydriat ic an d cycloplegic agen ts h ave been used in th e pract ice of oph th alm ology
sin ce th e m id-n in eteen th cen t u r y. Trade n am es, st rength s, an d dosages are sh ow n
in Table 14.11 .
Glaucoma
Medicat ion s can preven t vision loss due to glau com a by slow ing or preven t ing
in t raocular pressu re (IOP)-related dam age to th e opt ic n er ve ( Fig. 14.4 ). Th e m a-
jorit y of glau com a agen ts w ere developed for th e m ore com m on , in sidiou s form
of th e disease, prim ar y open -angle glau com a (POAG). IOP plays an im por tan t role
in th e n europath ology of POAG. Prevalen ce an d in ciden ce of POAG in crease as IOP
in creases. Th erefore, if pat ien t s w ith in creased IOP or sign s of opt ic n er ve dam age
are star ted early on m edicat ion s th at decrease IOP, vision can often be preser ved
in th e long term . Seven classes of glaucom a drugs are u sed; th e n am es, adm in ist ra-
t ion , an d con cen t rat ion s are listed in Table 14.12 .
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Fig. 14.4 Cupping of the disk in

glaucoma.
Beta-Adrenergic Blocking Agents

Mechanism
A disru pt ion in th e physiological product ion of aqu eou s h u m or n at urally resu lts
in flu ct u at ion s in IOP. Th e m ajor site of aqueou s h u m or produ ct ion is in th e cili-
ar y body an d is part ially con t rolled by β-adren ergic receptors. Act ivat ion of th ese
receptors cau ses an in crease in th e product ion of aqu eous h u m or w ith in th e cili-
ar y body. A β-an tagon ist reduces th e form at ion of aqueous h um or result ing in a
low er IOP.
Indications
Th is agen t is u sed as a first-lin e t reat m en t of POAG. Prim ar y angle closure glau-
com a (PACG) is also an in dicat ion , bu t th e agen t s th at supp ress aqu eou s h um or for-
m at ion m ay be less effect ive in th is form of glau com a becau se th e ciliar y body m ay
be isch em ic, ren dering th e β-receptors n on fun ct ion al. Never th eless, β-an tagon ist s
are st ill in dicated in PAC because of th e redu ct ion in IOP-en h an cing relaxat ion of
th e an terior ch am ber angle.
Contraindications/Adverse Effects
Th e sam e β-adren ergic receptors in th e ciliar y body are fou n d in oth er organ s, an d
system ic side effect s m ay in clu de decreased h ear t rate, d ecreased blood p ressu re,
an d exacerbat ion of in t r in sic bron ch ial asth m a an d ch ron ic obst r u ct ive pu lm o-
n ar y disease du e to bron ch osp asm . Th is topical m edicat ion sh ou ld be u sed w ith
cau t ion in p at ien t s w ith cardiac or lu ng d isease. Bet a xolol is a cardioselect ive β-1-
adren ergic an t agon ist develop ed to avoid t h e p u lm on ar y com p licat ion of t im olol,
th e n on select ive classic top ical β-adren ergic an t agon ist . Th e select ive an t agon ist
m ay be as effect ive in low er ing IOP as t h e n on select ive, bu t p u lm on ar y sid e ef-
fect s h ave occasion ally been n oted. Th erefore, becau se of th e severit y of p u lm o-
n ar y com p licat ion s in th e u se of th is class of glau com a agen t , cau t ion sh ou ld be
u sed w h en con sid ering th is dr ug in th e p at ien t w ith excessive im p air m en t of
pu lm on ar y fu n ct ion . Also, t h e effect s of system ic m edicat ion s su ch as β-block-
ers, d igit alis, an d reserp in e can be en h an ced w it h th e u se of top ical β-ad ren ergic
an t agon ist s.
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Prostaglandin Analogues
Mechanism
Th e prostaglan din class of glau com a agen t s at tem pts to alter th e IOP by en h an cing
th e outflow of aqu eou s h um or. Th e classic route of aqu eou s outflow is th rough
th e t rabecu lar m esh w ork in to th e can al of Sch lem m . Th e prostaglan din s also take
advan tage of a sup plem en tal rou te of aqueous outflow th rough th e uveoscleral
path w ay by in creasing th e por t ion of aqu eou s th at th e path w ay n orm ally drain s.
Indications
Lat an oprost w as th e first syn th et ic prostaglan din an alogue th at form ed a n ew
class of drugs developed specifically for glau com a. A single daily dose h as been
sh ow n to be m ore effect ive in redu cing IOP th an t im olol 0.5% adm in istered t w ice
daily, establish ing th e prost aglan din class as a first-lin e t reat m en t for POAG. Th e
prostaglan din s are adm in istered topically on ly.
Th ere h ave been n o m ajor system ic toxic effects of topical prost aglan din an a-
logue use in glaucom a th us far, bu t th ese drugs are w ell kn ow n for som e com m on ,
un iqu e ocu lar effect s. Pat ien t s frequen tly acqu ire perm an en t darken ing of th e iris,
conju n ct ival hyperem ia, conju n ct ival flush ing, red eye, an d a hyper t rich osis of th e
eyelash es th at rem its w h en th e m edicat ion is stopped. Th ere are rare adverse ef-
fects in cluding cystoid m acu lar edem a, dam age to th e blood–ret in al barrier, an d a
periocu lar pigm en t at ion of cosm et ic con cern .
Carbonic Anhydrase Inhibitors

Mechanism
Like th e β-adren ergic blocking agen t s, th is class suppresses aqueou s h u m or pro-
du ct ion . Th e m ech an ism involves decreasing th e form at ion an d secret ion of aqu e-
ous at an in t racellu lar level. Carbon ic an hydrase in h ibitors (CAIs) are th e on ly class
of glau com a m edicat ion s th at ut ilize a system ic route of deliver y th rough oral
m edicat ion s. A topical form , dorzolam ide, is also available an d is m ore com m on ly
used th an th e system ic form .
Indications
Oral CAIs are u sually used to su pplem en t various topical agen t s w h en IOP is n ot
being adequ ately low ered. Becau se of th eir system ic effect s an d in abilit y to low er
IOP in depen den tly, CAIs are usu ally n ot u sed as a first-lin e or sole prim ar y t reat-
m en t of glau com a. A com bin at ion of dorzolam ide an d topical t im olol, a β-an t ago-
n ist , is available (Cosopt).
Th e p ossible adverse effect s of th e system ic CAIs, acetazolam ide, m eth azolam ide,
an d dich lorph en am ide, in clude paresth esias, an orexia, gast roin test in al dist u r-
ban ces, h eadach es, altered taste an d sm ell, sodium an d p otassiu m deplet ion , a
predisposit ion to form ren al calculi, an d rarely bon e m arrow sup pression . Th ese
side effect s are p ossible w ith th e topical CAIs bu t h ave a m uch low er in ciden ce.
With dorzolam ide an d brin zolam ide th e m ost com m on side effect is altered taste
sen sat ion .
Miotics
Mechanism
Also called parasym p ath om im et ic or ch olin ergic-st im ulat ing agen t s, th is class en -
h an ces ch olin ergic sign al to th e an terior ch am ber by eith er direct acet ylch olin e
receptor act ivat ion or in h ibit ion of acet ylch olin esterase act ivit y. Th is in creased
ch olin ergic act ivit y cau ses a st ate of m iosis th at low ers IOP by en h an cing aqueous
h um or outflow th rough th e t rabecu lar m esh w ork. A p ilocarpin e-in du ced m iosis
h as been sh ow n to directly in crease th e w idth of th e an terior ch am ber angle in
pat ien t s w ith a n arrow angle.
Indications
Miot ics are in dicated for both POAG an d PAC. Th e side-effect profile m akes m iot-
ics a rarely u sed glau com a agen t . Th is class of topical m edicat ion is also used to
con t rol accom m odat ive esot ropia.
System ic side effect s can on ly occu r at 5 to 10 t im es th e n orm al dose. Classic para-
sym path et ic syn drom e occu rs, in clu ding lacrim at ion , salivat ion , perspirat ion , n au -
sea, vom it ing, an d diarrh ea, bu t ver y rarely at prescribed dosages. Th e ocular side
effects are p roblem at ic ow ing to th e dim in ish ed vision w ith pupillar y con st rict ion
an d h eadach e from ciliar y m uscle spasm .
Sympathomimetics (Epinephrine)
Mechanism
Adren ergic st im u lat ion decreases IOP by im proving aqueous ou tflow th rough both
th e can al of Sch lem m an d th e uveoscleral path w ay.
Indication
Sym path om im et ics are in frequ en tly u sed in th e t reat m en t of POAG ow ing to th eir
poten t ially seriou s system ic side effect s. Th ey are also n ot in dicated for PAC be-
cau se of th e adverse effect of m ydriasis on th e an terior ch am ber angle.
Epin eph rin e m ay cau se cardiac arrhyth m ia or an in crease in system ic blood pres-
su re ow ing to it s system ic adren ergic st im ulat ion . Dipivefrin is an epin eph rin e
prodr ug th at causes few er system ic side effects.
Alpha2 Selective Agonists

Mechanism
Th e first drug in th is class, apraclon idin e, w as derived from clon idin e an d w as
in ten ded to select ively block th e α 2 adren ergic receptor. Brim on idin e w as th en
released an d sh ow n to be 23 to 32 tim es m ore select ive for α 2 receptors versu s
α 1 receptors th an ap raclon idin e. Act ivat ion of th e α 2 receptor is th ough t to h ave
a du al m ech an ism of decreasing aqueous produ ct ion an d in creasing uveoscleral
outflow.
Indications
Th e differen t con cen t rat ion s available for apraclon idin e h ave specific in dicat ion s. A
single-dose applicator of a 1% solut ion is available for sup pression of th e acu te IOP
spikes th at occur after laser t reat m en t s. A 0.5% con cen t rat ion is also available in a
m u lt idose bot tle for glau com a p at ien ts w h ose IOP is n ot adequ ately respon ding to
m axim ally tolerated th erapy. Ch ron ic use of apraclon idin e is lim ited by its adverse
effects an d tachyphyla xis. Th e h igh er α 2 receptor select ivit y of brim on idin e allow s
th is t ype of pressu re-low ering m edicat ion to be u sed on a ch ron ic basis.
Apraclon idin e h as been associated w ith tachyphylaxis or rapid physiological tol-
eran ce in u p to 48% of pat ien t s, ren dering it less u sefu l in th e ch ron ic form s of
glaucom a. Th e m ost con cern ing adverse effect s in clu de or th ost at ic hyp oten sion
an d vasovagal episodes. Th e topical applicat ion of apraclon idin e is associated w ith
m ild pupillar y dilat ion , w h iten ing of th e conjun ct iva, an d elevat ion of th e u pper
eyelid. Th e adverse effect profile of brim on idin e h as been m in im al but m ay in clude
oral dr yn ess, h eadach e, an d fat igue/drow sin ess. Brim on idin e is con t rain dicated in
in fan ts because of th e risk of severe hypoten sion an d apn ea. Both drugs m ay cau se
a local sen sit ivit y react ion , w ith apraclon idin e h aving a fairly h igh rate of con t act
derm at it is of th e lids an d conju n ct iva.
Diagnostic Agents
Oph th alm ology requ ires th e abilit y to see cer tain path ology an d m an ipulate th e
eye in cert ain circu m stan ces. Not all path ology is readily visible u n der direct slit
lam p exam in at ion . Th e eye is a ver y sen sit ive organ an d w ill n ot tolerate m an ip u-
lat ion w ith out appropriate an esth esia. An esth et ics are u sed to h elp w ith m an ip u-
lat ion an d cert ain dyes to visualize path ology ( Table 14.13 ).
Fluorescein
Flu orescein com es in m any form s, in clu ding topical drops (Flu ress), topical st rips,
oral form s, or in t raven ou s (IV) solu t ion ( Fig. 14.5 ). It appears as a red-orange p ig-
m en t un der n at ural ligh t , bu t w h en seen un der a blue filter, it t urn s a fluorescen t
green color. Th e m ost com m on use is th e topical form . It can also be top ically u sed
to evalu ate corn eal scarring or oth er dam age to th e corn eal epith elium . W h en th e
corn ea is dam aged, th e dye is able to pass across t igh t ju n ct ion s an d stain th e un -
derlying layers. Recen t pu blicat ion s also n ote th at it h as a diagn ost ic pu rpose in
evalu at ing fun ct ion ing of th e glan ds in th e eyelids. IV an d oral flu orescein is used
to evaluate ret in al path ology by direct fu n dus ph otography or con focal m icros-
copy. Flu orescein angiography allow s visualizat ion of th e vascu lat u re of th e ret in a
at differen t t im e in ter vals.
Flu orescein is u su ally adm in istered in com bin at ion w ith an esth et ic or by m oist-
en ed st rips. Slit-lam p exam in at ion provides bet ter diagn ost ic view ing w h en st rips
are u sed becau se th e con cen t rat ion of flu orescein delivered in m ost drops is too
h igh for discrim in at ing evalu at ion . IV fluorescein is usually injected at a dose of
500 m g. More recen t research n otes th at doses as low as 166 m g are effect ive for
evalu at ion w h en u sing con focal scan n ing laser im aging. Oral flu orescein m ay be a
possible diagn ost ic agen t as con focal im aging progresses, bu t curren t angiography
st ill requ ires th e u se of IV flu orescein .
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s t n a ts e g n oc eD
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Color Atlas of Ophthalm ology
sr ezili b a ts ll ec ts aM
sr ezili b a ts ll ec tsa m d n a
s e ni m a tsi hi t n a n oi t a ni b m oC
Fig. 14.5 Combined fundus fluorescein angiography and indocyanine green (ICG) an-
giography. It reveals a pigment epithelial detachment with pooling of dye with a notch,
and the ICG is suggestive of a focal hot spot in the area of the notch. (Courtesy of Dr. Manish
Nagpal)
Adverse effects are m in im al for th e topical form s of th e drug. More effect s are
p ossible w h en IV flu orescein is u sed. Nausea, vom it ing, dizzin ess, an d a bit ter taste
m ay resu lt during IV adm in ist rat ion . Many pat ien ts develop cough or dr y th roat ,
an d som e can develop u r t icaria, or localized in flam m at ion of th e inject ion site
from allergies. An aphylactoid react ion s, sickle cell crises, h em olyt ic an em ia, sei-
zure, m yocardial in farct ion , an d death s are all docu m en ted in th e literat ure, but
th eir occu rren ce is rare.
Indocyanine Green
An oth er poten t ial agen t used to evalu ate posterior com par t m en t path ology is ICG.
Mu ch like its closely related su bst an ce, flu orescein , th is dye is injected in t rave-
n ou sly to evaluate th e ret in a an d ch oroid. It h as a dist in ct advan tage over flu ores-
cein in th at it can bet ter diagn ose ch oroidal n eovascu larizat ion . Th is is due par tly
to th e fact th at ICG is h igh ly plasm a protein bou n d. Flu orescein , w h ich usu ally leaks
out of th e capillaries, can n ot defin e vasculat ure as w ell as ICG, w h ich stays w ith in
th e vessels. Oth er advan tages in clu de th e fact th at th e flu orescen t w avelength of
ICG places it in th e n ear in frared spect ru m . Th is factor allow s pen et rat ion of t is-
su es th at n orm ally absorb th e sh or ter w avelength fluorescen ce of flu orescein .
ICG is usu ally adm in istered in doses of 25 m g for proper im aging. Because of
advan ces in con focal oph th alm oscopy, adequ ate diagn ost ics can be obtain ed by
as lit tle as 5 m g of ICG. Adverse react ion s are rare bu t can in clude n au sea, vom it-
ing, an d gen eral discom for t . An aphylaxis h as been repor ted in cer tain cases. ICG
sh ould n ot be u sed in in dividuals w ith kn ow n allergy to th e su bst an ce or allergies
to iodide becau se m any preparat ion s of ICG con tain iodide. In in dividu als w ith al-
lergies to flu orescein , use of ICG m ay avoid serious react ion s.
Agents Used in the Diagnosis and Management of

Neuro-ophthalmological Conditions
Most pupillar y disorders of th e sym path et ic or parasym path et ic n er vou s sys-
tem are p h arm acologically diagn osed by u sing specific oph th alm ic drops ( Table
14.14 ).
Medications and Therapeutics for Dry Eye
An est im ated 7.1 m illion people in th e Un ited St ates over th e age of 40 are afflicted
w ith keratoconju n ct ivit is sicca (KCS, also kn ow n as dr y eye) ( Fig. 14.6 ). Dr y eye is
par t icularly prevalen t in w om en aged 50 years an d older, bu t in pat ien t s aged 75
an d older, th is prevalen ce is dim in ish ed. Table 14.15 provides a list ing of th e t ypes
of targeted an d palliat ive t reat m en t s available.
Fig. 14.6 Severe dry-eye syndrome with corneal neovascular-

ization.
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Table 14.15 The rape utic Optio ns fo r Dry Eye Syndro m e*
Pro duct Brand Nam e How Supplie d Do sing
I. Targeted therapies
Antibiotics:
Topical:
Bacitracin/ploymyxin Polysporin 7 5 mL bot tle 1–2 drops 2x
System ic: daily
Doxycycline Vibra-Tabs8 100 mg tablets 100 mg 2x daily
Immunomodulators:
Cyclosporine A Restasis2 Unit dose vials 1 drop 2x daily
(0.4 m L)
Mucolytic agents:
N-acet ylcysteine Mucomyst 10 10–20% drops, 1–2 drops up to
5 ml bot tle 4x daily
Corticosteroid & antibiotic
mixtures:
Loteprednol etabonate Zylet 7 2.5, 5, and 1–2 drops 4–6
(0.5%) and 10 mL bot tles hours
tobramycin (0.3%)
II. Palliative therapies
Corticosteroids:
Loteprednol etabonate Lotemax7 5 & 10 mL 1–2 drops 4x
(0.5%) dropper bot tle daily
(0.2%) Alrex7
Prednisolone acetate Pred Forte 2 5 & 10 mL 2 drops 4x daily
(0.1%) Omnipred 1 dropper bot tle
Flurometholone (0.25%) FML Forte 2 5, 10 & 15 mL 1 drop 2–4x
dropper bot tle, daily, ½ inch
ointment into cul de sac
1–3x daily
Rimexolone (1%) Vexol1 5 & 10 m L
dropper bot tle
Secretagogues:
Pilocarpine Salagen 11 5 mg tablets 1 daily
Therapeutic plug :
Hydroxypropyl cellulose Lacrisert 7 5 mg water- 1–2 rods per eye
soluble rod daily
Arti cial tears:
Low viscosit y— OPTIVE2 5 mL dropper or 1–2 drops as
unit dose needed
Refresh Plus2 Unit dose 1–2 drops as
needed
Refresh Tears2 Unit and 1–2 drops as
Multi-dose needed
Hypoosmotic— TheraTears 3
Unit and 1–2 drops as
m ulti-dose needed
Table 14.15 (con t in u ed)
Pro duct Brand Nam e How Supplie d Do sing
Moderate viscosit y— Refresh Dry Unit dose 1–2 drops as

Eye needed
Therapy2
Tear Naturale 5 mL dropper 1–2 drops as
Forte 1 bot tle needed
GenTeal 6
Unit dose, 1–2 drops as
preservative needed
free
Bion Tears 5
preservative needed
free
Ocucoat 7
preservative needed
free
High viscosit y— Systane Ultra 10 m L dropper
1
1–2 drops as
(best for nocturnal bot tle needed
application) Refresh Unit dose 1–2 drops as
Celluvisc 2
needed
Refresh Unit dose 1–2 drops as
Liquigel 2
needed
Blink Tears5 15 m L dropper 1–2 drops as
bot tle needed
Gel formulations— GenTeal Gel 6
Tube delivery ¼ inch into cul
(best for nocturanal system de sac
application) Tears Again 5
Tube delivery ¼ inch into cul
system de sac
Lubricating ointments— Refresh P.M. Tube 2
¼ inch into cul
(best for nocturnal de sac
application) Tears Tube ¼ inch into cul
Naturale de sac
PM1
Advanced Eye Tube ¼ inch into cul
Relief2 de sac
Systane Tube ¼ inch into cul
Night tim e 1
de sac
1
Alcon Laboratories, In c., For t Worth , TX USA: 2 Allergan , In c., Ir vin e, CA USA; 3 Advan ced Vision
Research , In c., Woburn , MA USA; 4 Abbot t Medical Opt ics, Abbot t Park, IL USA; 5 Cyn acon /Ocu Soft ,
In c., Richm ond, TX USA; 6 Novar t is Ph arm aceu t icals, St . Lou is, MO USA; 7 Bausch an d Lom b, In c.,
Roch ester, NY USA; 8 P zer Labs, In c., New York, NY USA; 9 Aton Ph arm aceu ticals, In c., Law ren ce-
ville, NJ USA; 10 Mead-Joh n son Laboratories, Evan sville, ID USA; an d 11 MGI PHARMA, In c., Bloom -
ington , MN USA.
Acknowledgments
Th is w ork w as m ade possible, in par t , by NEI-EY-006311 (JMH), Research to Pre-
ven t Blin dn ess Sen ior Scien t ific Invest igator Aw ard (JMH), EY02672 (HEK) an d LSU
Eye Cen ter Core Gran t EY02377. Th e Depart m en t of Oph th alm ology h as an un re-
st ricted gran t from Research to Preven t Blin dn ess, New York, NY an d fu n ds from
th e Lou isian a Eye Foun dat ion , New Orlean s.
15 Ocular Manifestations of Systemic Disease
Diabetes Mellitus
Diabetes m ellit u s (DM) is a ch ron ic disorder ch aracterized by persisten t hypergly-

cem ia presen t ing w ith varied m an ifestat ion s an d con sequ en tly result ing in m icro-
vascu lar an d m acrovascu lar com plicat ion s. Risk factors for diabet ic ret in opathy
in clu de du rat ion of DM, con t rol of blood glu cose, pu bert y an d t ype of DM, n e-
ph ropathy, hyp erten sion , pregn an cy, an d gen et ic factors ( Fig. 15.1 ).
Presentation
Nonproliferat ive diabet ic ret inopathy : Th is is th e earliest form ch aracterized by
m icroan eur ysm s, dot an d blot h em orrh ages, cot ton -w ool spot s, h ard exudates,
ven ous loops, ven ou s beading, an d in t raret in al m icrovascu lar abn orm alit y.
Proliferat ive diabet ic ret inopathy : Ch aracterized by th e proliferat ion of abn or-
m al n ew vessels eith er on th e opt ic disk or elsew h ere on th e surface of ret in a.
Diabet ic m acular edem a : Types in clu de focal, diffu se, isch em ic, an d m ixed.
Posterior subcapsular cataract s: Tran sien t sh ift in refract ion occu rs du e to len s
sw elling du e to osm ot ic gradien t form ed by sorbitol.
Glaucom a : Th ere is in creased risk of open -angle an d n eovascu lar glau com a.
Corneal neuropathic changes: It can presen t w ith decreased corn eal sen sat ion s
an d epith eliopathy.
Cranial-nerve palsies: Pupil-sparing th ird, four th , an d sixth cran ial n er ves can
be involved. Diabet ic papillit is, m u corm ycosis, an d orbital cellu lit is are also
com m on .
Management
Managem en t in cludes system ic cont rol of DM, correction of anem ia, and m ain te-
nan ce of blood pressure <130/80 m m Hg, fast ing blood sugar → 90 to 130 m g%,
post-pran dial blood sugar → 180 m g%, triglycerides <150 m g%, low -densit y lipo-
protein (LDL) <100 m g%, high -den sit y lipoprotein (HDL) >40 m g%, an d album inuria
<30 µg/m g.
Fig. 15.1 Diabetic retinopathy.
430
15 Ocular Manifestations of System ic Disease 431
Panretinal photocoagulation, focal and grid laser photocoagulation, pars plana

vitreous surgery, and pharm acotherapy w ith aldose reductase inhibitors, protein
kinase C-β inhibitors and intravitreal antivascular endothelial grow th factor (VEGF)
agents (bevacizum ab, ranibizum ab, etc.) are effective m odalities of treatm ent.
Acquired Immunodeficiency Syndrome
Acquired im m u n odeficien cy syn drom e (AIDS) is a poten t ially fat al m u lt isystem

syn drom e caused by h u m an im m u n odeficien cy virus (HIV), ch aracterized by pro-
fou n d disru pt ion of th e im m u n e system an d a propen sit y for variou s oppor t u n ist ic
in fect ion s an d n eoplasm s.
Presentation
Eyelids: Kaposi sarcom a, h erpes zoster oph th alm icu s, m u lt iple m olluscu m le-
sion s
Orbit : B-cell lym ph om a
Conjunct iva : Kaposi sarcom a, squ am ou s cell carcin om a
Cornea : Kerat it is due to m icrosporidium , h erpes sim p lex, h erp es zoster
Posterior segm ent : HIV ret in opathy, cytom egalovir us (CMV) ret in it is, toxoplas-
m osis, Candida ret in it is, progressive ou ter ret in al n ecrosis, pn eum ocyst is cari-
n ii pn eu m on ia ret in opathy, Histoplasm a capsulat um , cr yptococcal in fect ion
Neuro-ophthalm ic: Cran ial-n er ve palsies, pu pillar y abn orm alit ies, opt ic n euri-
t is, papilledem a, an d visu al-field defect s
Management
Diagn osis is con firm ed w ith en zym e-lin ked im m u n osorben t assay (ELISA) an d
Western blot test . Mon itoring is don e by com plete blood cou n t (CBC), CD4 cou n t ,
an d test s for secon dar y in fect ion s. High ly act ive an t iret roviral th erapy (HAART) is
th e recom m en ded t reat m en t , w h ich involves t w o n u cleoside reverse t ran script ase
in h ibitors w ith eith er a n on n ucleoside reverse t ran scriptase in h ibitor or on e or
t w o protease in h ibitors.
Nucleoside reverse t ranscriptase inhibitors : zidovu din e, lam ivudin e, an d zal-
citabin e
Protease inhibitors: Am pren avir, in din avir
Nonnucleoside reverse t ranscriptase inhibitors : efaviren z
Varicella Zoster Virus
Varicella zoster viru s (VZV) is on e of th e h erpesviruses kn ow n to in fect h u m an s.

It causes ch ickenpox in ch ildren an d both sh ingles an d posth erpet ic n eu ralgia in
adu lts. It is spread by airborn e droplet s as w ell as in fected secret ion s.
Presentation
After th e prim ar y in fect ion result ing in ch ickenpox, th e virus rem ain s dorm an t
w ith in th e body in th e t rigem in al an d dorsal root ganglia an d m ay becom e react i-
vated to produce h erp es zoster. Th e risk of zoster in creases w ith age an d im m u n o-
su ppression . System ic involvem en t m ay cause seriou s com plicat ion s.
Ocu lar involvem en t:
Acute conjun ct ivit is w ith or w ith out secon dar y bacterial in fect ion
Pseu doden drites an d corn eal u lcers
St rom al kerat it is
Uveit is, ret in it is, opt ic n eu rit is, oph th alm oplegia
Management
Man agem en t is in coordin at ion w ith th e n eu rologist , in fect ious disease specialist ,
an d derm atologist . Treat m en t is based on th e severit y, age, an d im m un e state. An -
t iviral m edicat ion s are th ough t to decrease th e du rat ion of sym ptom s an d th e like-
lih ood of posth erpet ic n euralgia w h en star ted w ith in 2 days of th e on set of rash .
Medicat ion s available in clu de acyclovir, valacyclovir, pen ciclovir, an d fam ciclovir.
Rheumatoid Arthritis
Rh eu m atoid arth rit is (RA) is an au toim m un e system ic disease ch aracterized by

asym m et rical, dest r uct ive, deform ing, in flam m ator y polyar th ropathy, in associa-
t ion w ith a spect ru m of ext raart icu lar m an ifest at ion s. Associat ion of HLA-DW 4/
DR4 occu rs in w h ite person s.
Presentation
Wom en are m ore frequ en tly affected th an m en (3:1). RA is com m on ly seen in th e
fou r th decade. Fever, m alaise, w eigh t loss, m orn ing st iffn ess, join t pain an d in -
flam m at ion , an d lim itat ion of m ovem en ts are th e m ain com plain ts. Uln ar devia-
t ion an d sw an n eck deform it y, bursal effusion , Baker cyst an d h allu x valgus are
seen . Ext raocular fin dings in clude superficial rh eum atoid n odu le, pericardit is an d
pleural effusion , Caplan syn drom e, an d Felt y syn drom e.
Oph th alm ic m an ifestat ion s in clude keratoconju n ct ivit is sicca, sclerit is, periph -
eral u lcerat ive kerat it is, an d acqu ired superior oblique ten don sh eath syn drom e.
Management
Laborator y invest igat ion s in clu de rh eu m at ic factor (RF), an t in uclear an t ibodies
(ANA), CBC, an d rh eum atology con su ltat ion . Non steroidal an t iin flam m ator y drugs
(NSAIDs), gold salts, D-pen cillam in e, hydroxych loroquin e, sulp h asalazin e, steroids,
an d cytotoxic agen ts are th e drugs u sed.
Systemic Lupus Erythematosis
System ic lu pus er yth em atosu s (SLE) is an au toim m un e, n on –organ -specific con -

n ect ive t issu e disease ch aracterized by n u m erous an t ibodies an d circulat ing im -
m u n e com plexes, w h ich m ediate w idespread vascu lit is an d t issu e dam age.
Presentation
Predom in an tly affects you ng w om en (9:1), frequ en tly in th e th ird or four th de-
cade. Com m on presen t ing sym ptom s in clu de fever, fat igu e, an d w eigh t loss.
Oph th alm ic feat u res in clude m adarosis, keratoconju n ct ivit is sicca, periph eral
ulcerat ive kerat it is, sclerit is, ret in al vascu lit is, an d opt ic n eu ropathy.
Ext raocular m an ifest at ion s in clude th e follow ing:
Mucocutaneous: Bu t terfly facial rash , discoid rash , vasculit is, alopecia, oral
ulcerat ion , Rayn aud ph en om en on
Musculoskeletal: Ar th rit is, m yosit is, ten don it is
Renal: Glom eru lon ep h rit is
Cardiovascular: Pericardit is, en docardit is, m yocardit is, ar terial an d ven ous
occlu sion s.
Pulm onary : Pleu risy, atelectasis
Hem atopoiet ic: An em ia, th rom bocytopen ia, lym ph open ia, leu copen ia
Ret iculoendothelial: Splen om egaly, lym ph aden opathy
Neurological: Polyn eurit is, cran ial-n er ve palsies, spin al cord lesion s, epi-
lepsy
Management
Laborator y invest igat ion s in clude CBC, ANA, dou ble st ran ded/single st ran ded
(DS/SS) DNA, SSA/SSB (Sjögren syn drom e), er yth rocyte sedim en tat ion rate (ESR),
an t iph osph olipid an t ibody, lupu s an t icoagulan t , an d a rh eu m atologist’s opin ion .
Th erapies in clu de an t im alarials, NSAIDs, steroids, an d cytotoxic agen t s.
Sjögren Syndrome
Sjögren syn drom e (SS) is ch aracterized by autoim m u n e in flam m at ion an d de-

st ru ct ion of lacrim al an d salivar y glan ds.
Prim ary SS: W h en it exist s in isolat ion
Secondary SS: W h en it is associated w ith oth er disease such as RA an d SLE
Presentation
Prim ar y SS affect s w om en m ore com m on ly th an m en . It presen ts in adu lt life w ith
grit t in ess of th e eyes an d dr yn ess of th e m outh . En largem en t of th e salivar y glan ds
w ith dim in ish ed salivar y flow rate an d a dr y fissu red tongue, dr y n asal passages,
dim in ish ed vagin al secret ion s, Rayn au d ph en om en on , an d cut an eou s vascu lit is
are th e feat u res of th e disorder.
Th e m ain ophthalm ic feat ures in clude keratoconjun ctivitis sicca and Adie pupil.
Management
Laborator y tests in clude CBC, rh eu m atoid factor, ANA, SSA/SSB, cr yoglobulin s,
circulat ing im m u n e com plexes, gam m aglobu lin s, an d an t ithyroid an t ibodies. Di-
agn osis can be con firm ed by salivar y glan d biopsy. Im por tan t th erapies in clude
system ic steroids an d cytotoxic agen ts.
Lyme Disease
Lym e disease is an in fect ion cau sed by a spiroch ete, Borrelia burgdorferi, t ran sm it-
ted th rough th e bite of a deer t ick, Ixodes dam m ini. It is m ost com m on ly rep or ted
from th e n or th eastern Un ited St ates.
Presentation
Follow ing are th e m an ifestat ion s of Lym e disease.
Early st age
Presen t s several days after th e bite
Er yth em a ch ron icu m m igran s
Con st it u t ion al sym ptom s, lym ph aden opathy
Neu rological an d cardiac com plicat ion s m ay follow w ith in 3 to 4 w eeks of
th e in it ial m an ifest at ion .
Late stage
Ch ron ic ar th rit is of th e large join ts
Polyn europathy, ch ron ic acroderm at it is
Oph th alm ic m an ifest at ion s in clu de th e follow ing:
Ph otoph obia, pain , periocular edem a, conjun ct ivit is
Kerat it is, an terior uveit is, in term ediate uveit is, opt ic n eu rit is, n eu roret in it is,
ocu lar m otor n er ve palsies
Periph eral m u lt ifocal ch oroidit is, ret in al periph lebit is
Management
Ch eck for a h istor y of t ick bite, exposure, an d th e ch aracterist ic rash . Perform a
th orough derm atological, n eu rological, an d ocu lar exam in at ion . Im m un ofluores-
cen ce an d ELISA m ust be perform ed. Western blot is con firm ator y. Th erapies in -
clude oral doxycyclin e an d in t raven ou s an t ibiot ics.
Leprosy
Leprosy is a ch ron ic gran ulom atou s in fect ion cau sed by an in t racellu lar acid-fast
bacillu s, Mycobacterium leprae .
Presentation
Leprom atou s leprosy is a gen eralized, m ult isystem in fect ion .
Sk in : W h en it exists in isolat ion
Nose : W h en it is associated w ith oth er disease such as RA an d SLE
Neurological: Sen sor y n europathy, au ton om ic n eu ropathy, an d m otor n eu -

ropathy
Tu bercu loid leprosy is rest ricted to th e skin an d periph eral n er ves.
Sk in : An n u lar, an esth et ic, hypopigm en ted lesion s w ith raised edges
Nerves: Th icken ing of cut an eou s sen sor y n er ves
Oph th alm ic feat ures in clude th e follow ing:
Madarosis, lagoph th alm os due to seven th -n er ve palsy, n eu rot ropic kerat it is
du e to t rigem in al n er ve involvem en t
An terior uveit is (gran u lom atous)
Management
Dapson e is th e drug of ch oice. Rifam picin an d clofazim in e con t ributes in m ult idrug
th erapy. Clofazim in e is n o longer available th rough m ost U.S. ph arm acies. Requ est s
for clofazim in e to t reat leprosy sh ould be directed to th e Nat ion al Han sen’s Disease
program (a division of th e U.S. Dept . of Health an d Hum an Ser vices), w h ich h olds
th e IND for th is in dicat ion . It can th us be obtain ed for u se in leprosy.
Leukemia
Leu kem ias are a grou p of n eoplast ic disorders ch aracterized by abn orm al p rolif-
erat ion of w h ite blood cells. Ocular involvem en t is m ore com m on ly seen in th e
acute th an in th e ch ron ic form .
Presentation
Acute leukem ia presen ts w ith con st it u t ion al sym ptom s, in clu ding fever, lym ph -
aden opathy, h epatosplen om egaly, epistaxis, an d easy bruisabilit y. Cen t ral n er vou s
system (CNS) involvem en t an d secon dar y in fect ion s are th e m ain con cern ( Fig.
15.2 ). Ocular m an ifestat ion s in clude th e follow ing:
Fig. 15.2 Color fundus photograph showing superficial and

deep retinal hemorrhages, and a preretinal boat-shaped hem -
orrhage associated with leukemia. (Courtesy of Stephen W. Wong,
MD, Philadelphia, PA)
Ret in opathy, in clu ding flam e-sh aped h em orrh ages, Roth spot s, cot ton -w ool
spot s, periph eral ret in al n eovascu larizat ion leu kem ic pigm en t epith eliop athy,
ch aracterized by a leopard sp ot fun du s
Orbit al involvem en t
Iris th icken ing, irit is, an d pseudohypopyon
Subconjun ct ival h em orrh age, hyph em a
Opt ic n europathy
Management
Bon e m arrow aspirat ion , biopsy w ith im m un ocytogen ic m arkers, com pu ted tom o-
grap h ic (CT) scan , an d lu m bar pu n ct u re are required for con firm ing th e diagn osis
an d exten t of th e disease. System ic ch em oth erapy, gen eral support ive m easu res,
an d ocu lar radioth erapy are th e m ain st ays of th e t reat m en t .
Lymphoma
Lym ph om a is a t ype of solid h em atological n eoplasm origin at ing from th e lym -

ph ocytes. Prim ar y in t raocular an d cen t ral n er vous system lym p h om a is a h igh ly
m align an t , diffuse, large B-cell lym ph om a ( Figs. 15.3 an d 15.4 ).
Presentation
In t raocu lar lym p h om as gen erally affect elderly pat ien t s an d are u n ilateral (20%) or
bilateral (80%). Tw o t ypes are recogn ized: vit reoret in al (n ot associated w ith sys-
tem ic involvem en t) an d uveal (associated w ith system ic involvem en t).
Ocu lar feat ures
Ch ron ic an terior uveit is
In term ediate uveit is
Fig. 15.3 Color fundus photograph of a patient with

intraocular lymphoma showing hemorrhagic retinal
vasculitis. (Courtesy of Debra A. Goldstein, MD, Chicago, IL)
A B
Fig. 15.4 (A) Color and (B) red-free fundus photographs of a patient with central ner-
vous system lymphoma that resulted in central retinal artery occlusion secondary to
optic nerve compression. (Courtesy of Lawrence J. Ulanski, MD, Chicago, IL)
Posterior segm en t
Mu lt ifocal, large, yellow ish , subret in al pigm en t epith elium (RPE) in filt rates.
Diffuse ret in al in filt rates, vascu lar sh eath ing, an d occlu sion
CNS feat ures
Solit ar y or m u lt iple in t racran ial n odu les
Diffuse m en ingeal or periven t ricular lesion s
Localized in t radu ral spin al m asses
Management
Diagn osis of ocular lym ph om a is con firm ed w ith aqu eou s an d vit reou s biopsy an d
cytology. CT, m agn et ic reson an ce im aging (MRI), an d lu m bar p un ct ure h elp in as-
cer t ain ing th e diagn osis of CNS lym ph om a. Lym ph om a is t reated w ith h igh -dose
extern al beam radioth erapy to th e eyes, w h ole-brain radioth erapy, system ic or in -
t rath ecal ch em oth erapy, an d in t rath ecal m eth ot rexate.
Pregnancy
Pregn an cy is associated w ith m atern al h orm on al, m et abolic, h em atological, car-

diovascu lar, an d im m u n ologic alterat ion s th at can affect th e ocular t issues.
Presentation
Ret in al an d ch oroidal disorders arising in pregn an cy
Preeclam psia an d eclam psia
Ret in opathy—focal or gen eralized ret in al ar teriolar n arrow ing
Ch oroidopathy—serou s ret in al det ach m en t s or yellow RPE lesion s
Cor t ical blin dn ess
Ret in al ar terial an d ven ou s occlusion s
Cen t ral serou s ch orioret in opathy
Dissem in ated in t ravascu lar coagulop athy (DIC)
Th rom bot ic th rom bocytopen ic purpu ra (TTP)
Am n iot ic flu id em bolism

Uveal m elan om a
Worsen ed preexist ing con dit ion s
Diabet ic ret in op athy (progression can be dim in ish ed by bet ter m etabolic con -
t rol before pregn an cy)
Diabet ic m acu lar edem a (pregn an t w om en w ith diabet ic m acular edem a sh ould
n ot be t reated during pregn an cy becau se of th e h igh rate of spon tan eous im -
provem en t post par t um )
Management
Treat m en t is aim ed at th e path ological st ate an d is don e in coordin at ion w ith th e
pat ien t’s obstet rician . A close w atch m ust be kept on th e ret in opath ies because
spon tan eous regression can occur w ith t im e. Proliferat ive diabet ic ret in opathy
m ay be required to be t reated to preven t vit reous h em orrh age during labor.
Albinism
Albin ism is a gen et ically determ in ed h eterogen eou s group of disorders involving
hypop igm en tat ion of th e eyes or skin due to a deficien cy of t yrosin ase, w h ich m e-
diates th e conversion of t yrosin e to m elan in ( Fig. 15.5 ).
A B
Fig. 15.5 (A) Albinotic patient. (B) Transillumination seen through the iris in the same
patient.
Presentation
Oculocuta neous Albinism
Tyrosinase-negat ive : Th ese albin os are in capable of syn th esizing m elan in an d
h ave blon d h air an d ver y pale skin .
Iris is diaph an ou s an d t ran slu cen t , giving rise to a “pin k-eyed” appearan ce.
Fu n dus
Lack of pigm en t w ith con spicu ou sly large ch oroidal vessels
Hypoplasia of vessels form ing th e perim acu lar arcades
Foveal an d opt ic n er ve hypoplasia m ay be presen t .
Refract ive errors are com m on .
Nyst agm us
Th e ch iasm h as a decreased n um ber of u n crossed n er ved fibers.
Tyrosinase-posit ive : Th ese albin os can syn th esize variable am oun ts of m elan in
an d var y in com plexion from ver y fair to alm ost n orm al.
Iris m ay be blu e or dark brow n .
Fu n dus sh ow s variables hypopigm en t at ion .
Visual acuit y is u su ally im paired ow ing to foveal hypoplasia.
Associated syndrom es: Ch édiak-Higash i an d Herm an sky-Pu dlak syn drom es can
be associated w ith oculocut an eou s albin ism .
Ch édiak-Higash i syn drom e is associated w ith w h ite cell abn orm alit ies re-
su lt ing in pyogen ic in fect ion s, lym ph aden opathy, an d death .
Herm an sky-Pu dlak syn drom e is a lysosom al storage disease of th e ret iculo-
en doth elial system ch aracterized by easy bru ising due to platelet dysfu n c-
t ion .
Ocula r Albinism
Th e eyes are predom in an tly affected, w ith less eviden t skin an d h air involvem en t .
In h eritan ce is XL or less com m on ly AR. Fem ale carriers are asym ptom at ic an d h ave
n orm al vision , alth ough th ey m ay sh ow part ial iris t ran slu cen cy, m acu lar st ip-
pling, an d scat tered areas of depigm en tat ion an d gran u larit y in th e m idperip h er y.
Affected m ales m an ifest hypopigm en ted iris an d fun du s.
Management
Tyrosin ase test , elect roret in ography, h em atological tests are h elpfu l in th e diagn o-
sis. Dark glasses, low vision aids, gen et ic coun seling are h elpfu l.
Marfan Syndrome
Marfan syn drom e is a w idespread disorder of con n ect ive t issu es associated w ith
m u tat ion of th e fibrillin gen e or ch rom osom e 15q. It is th e m ost com m on cause of
h eritable ectopia len t is. Marfan syn drom e is au tosom al dom in an t , an d th e preva-
len ce is 5/100,000 ( Fig. 15.6 ).
Presentation
Oph th alm ic feat ures
Ectopia len t is, hypoplasia of dilator pu pillae, angle an om aly, m yopia, an d
ret in al detach m en t
Len s dislocat ion occurs in ~80% of pat ien ts w ith Marfan syn drom e an d is
usually bilateral, sym m et rical, an d su perotem poral.
Microsph eroph akia, keratocon u s, an d corn ea plan a
Muscu loskeletal feat u res
Tall, th in st at u re, scoliosis, in creased arm span
Arach n odact yly, m ild join t hyperm obilit y
Narrow an d h igh -arch ed palate
Cardiovascu lar feat ures
Dilat at ion of th e ascen ding aor ta leading to aor t ic in com peten ce an d h ear t
failu re
Mit ral valve disease an d aor t ic dissect ion
Skin
St riae, fragilit y, an d easy bruising
Fig. 15.6 Long, thin, and slender fingers in a patient with Marfan syndrom e.
Management
Evalu at ion by a cardiologist is often n ecessar y. Ru le ou t oth er cau ses of len s sub-
lu xat ion . Th e sublu xat ion is t reated according to th e grade an d visual sym ptom s.
Homocystinuria
Hom ocyst in u ria is cau sed by deficien cy of cystath ion in e β-syn th et ase leading
to accu m ulat ion of h om ocyst in e an d m eth ion in e. It is th e secon d m ost com m on
cau se of ectopia len t is.
Presentation
Oph th alm ic feat ures
Ectopia len t is, w h ich is u su ally bilateral, sym m et rical, in feron asal, an d pres-
en t in n early 90% of pat ien ts. Deficien t zon u lar in tegrit y secon dar y to th e
en zym at ic defect h as been im plicated as th e prim ar y cau se of len s disp lace-
m en t ( Fig. 15.7 ).
Myopia, ret in al detach m en t , ret in al vein occlu sion s, ret in al arter y occlu -
sion s
Oth er feat ures
Fair skin w ith coarse h air, osteoporosis, m en tal retardat ion , seizu re disorder,
m arfan oid h abit u s, an d poor circu lat ion
Th rom boem bolic even t s con st it ute th e m ajor th reat to su r vival, especially
follow ing gen eral an esth esia.
Sodium n it ropru sside test an d u rin e ch rom atography h elp in con firm ing th e
diagn osis.
Management
IQ test ing an d special n eeds program s an d sch ooling m ay be required. Lim it m e-
th ion in e in take an d in crease cystein e in take. Oral pyridoxin e reduces h om ocyst in e
an d m eth ion in e levels. Rule ou t oth er cau ses of sublu xat ion an d t reat it if th e pa-
t ien t is sym ptom at ic.
Fig. 15.7 Inferior subluxation seen in a

patient with hom ocystinuria.
Weill-Marchesani Syndrome
Weill-March esan i syn drom e is a rare au tosom al, recessive, system ic, con n ect ive
t issue disorder, ch aracterized by sh or t st at ure, brachydact yly, an d st iff join t s. Pen -
et ran ce is variable an d con sanguin it y is often presen t .
Presentation
Ocu lar feat ures
Ectopia len t is, bilateral an d in ferior, occurs in ~50% of cases du ring th e teen
years or early t w en t ies.
Microsph eroph akia is th e m ost p rom in en t feat ure of th is syn drom e.
Secon dar y angle-closure glau com a due to p upillar y block
Lenticular m yopia, asym m etrical axial lengths, presenile vitreous liquefaction
Oth er feat u res
Sh or t st at u re, brachyceph aly, lim ited join t m obilit y, w ell-develop ed m uscu -
lar appearan ce, an d n orm al in telligen ce
Management
Radiography of th e m et acarpals is im por tan t . Treat angle-closu re glau com a w ith
m ydriat ics an d laser iridotom y. Th e fellow eye m u st be kept on m iot ics to preven t
a sim ilar occu rren ce. Rule ou t oth er causes of len s sublu xat ion an d t reat it if th e
pat ien t is sym ptom at ic.
Pseudoxanthoma Elasticum
Pseudoxan th om a elast icum (PXE) is a rare gen et ic disorder ch aracterized by pro-

gressive calcificat ion an d fragm en tat ion of elast ic fibers in th e skin , ret in a, an d
cardiovascu lar system , w h ich is referred to as elastorrhexia.
Presentation
Ocu lar feat ures
Angioid st reaks of th e ret in a, w h ich are slate gray to reddish brow n cu r vilin -
ear ban ds radiat ing from th e opt ic disk. Th ey resu lt from calcificat ion of th e
elast ic fibers in th e Bru ch m em bran e of th e ret in a. Th ey are presen t in 85%of
pat ien ts w ith PXE.
Fibrovascular ingrow th in th e ret in a m ay lead to ret in al h em orrh age.
Developm en t of subret in al n eovascular m em bran e can cau se loss of cen t ral
vision .
Yellow ish speckled m ot tling described as peau’d’orange in seen in th e tem -
poral qu adran t of th e ret in a.
Cu tan eous fin dings
Plaques are seen on th e lateral part of th e n eck an d involve th e an tecubital
fossa.
Cardiovascular fin dings
Hyperten sion , coron ar y in farct ion , m it ral valve prolapse
Oth er fin dings
Gast roin test in al bleeding, perip h eral vascu lar diseases
Management
Skin biopsy is diagn ost ic. Fu n dus flu orescein angiography (FFA) an d opt ical co-
h eren ce tom ography (OCT) h elp in determ in ing u n derlying ch oroidal n eovascu-
lar m em bran e if th e pat ien t com plain s of m et am orp h opsia. Pat ien ts sh ou ld avoid
h eavy lift ing, st rain ing, an d h ead t rau m a. Treat m en t of th e ch oroidal n eovascu lar
m em bran e eith er w ith laser ph otocoagulat ion or w ith in t ravit real an t i-VEGF in -
ject ion is recom m en ded.
Phakomatoses
Neurofibromatosis (von Recklinghausen Disease)

Neurofibrom atosis 1 (NF-1) is by far m ore com m on , associated w ith skin , n er vou s
system , an d bon e an d join t m an ifest at ion s. Pat ien ts w ith NF-2 h ave few derm a-
tological fin dings, but th ey h ave a h igh in ciden ce of m en ingiom as an d acou st ic
n eurom as. Prevalen ce is est im ated to be ~1/3000 ( Fig. 15.8 ).
Presentation
Cutaneous involvem ent : Hyperpigm en t at ion , hypom elan ot ic m acu les, cut an e-
ous n eurofibrom as
CNS involvem ent : Sim ple m egalen ceph aly, hydroceph alus, vascu lar occlusion s,
du ral ectasia, absen ce of th e sph en oid w ing, lam bdoidal su t ure defect , seizures,
learn ing disabilit ies, em ot ion al/beh avioral dist u rban ces, gliom as, an d m en in -
giom as
Skeletal involvem ent : Progressive kyph oscoliosis, plexiform n eu rofibrom as,
lyt ic m etaphyseal, diaphyseal defect s, an d sh ort stat u re
Visceral involvem ent : Neu rofibrom as of th e gast roin test in al t ract an d ph eoch ro-
m ocytom as
Ophthalm ic involvem ent : Lisch n odu les, m ult iple n odules, n evi, plexiform n eu-
rofibrom as of th e eyelid, congen ital glau com a, p rom in en t corn eal n er ves, h am -
ar tom as of th e ch oroid, ast rocyt ic h am ar tom as (w h ite t um ors involving th e op-
t ic n er ve), com bin ed h am artom as, ret in al capillar y h em angiom as. Absen ce of
th e greater w ing of th e sph en oid bon e m ay lead to p ulsat ile proptosis, ch oroidal
m elan om as, an d opt ic n er ve gliom as
Fig. 15.8 Lisch nodules.

Management
Skin biopsy is diagn ost ic. Radiograph s of th e m et aphyseal join ts an d n euroim aging
are don e to ru le out associated lesion s.
Tuberous Sclerosis (Bourneville Disease)

Tu berous sclerosis is an au tosom al dom in an t disease w ith in com plete pen et ran ce.
It is a rare, m u lt isystem disorder w ith h am ar tom as in th e brain an d on oth er vital
organ s such as th e kidn eys, h ear t , eyes, lu ngs, an d skin ( Fig. 15.9 ).
Presentation
Th e cu t an eou s feat u res in clude aden om a sebaceu m (angiofibrom a, w h ich appears
on th e n ose an d ch eeks in a bu t terfly dist ribut ion ), u ngu la or su bu ngu al fibrom as,
hypom elan ic m acu les called ash leaf spots, café-au-lait spot s, lu m bosacral sh agreen
patch es, an d foreh ead plaques. Oth er feat u res in clude CNS involvem en t in th e form
of su bepen dym al n odules an d cor t ical/subcor t ical t u bers, learn ing difficu lt ies, sei-
zu res, ren al angiom yolipom ata, cardiac rh abdom yom a, an d p ulm on ar y fibrosis.
Ocu lar involvem en t in clu des th e follow ing:
Ret in al ast rocyt ic h am ar tom as, w h ich appear as a grayish or yellow ish w h ite
lesion , 1 to 2 disk diam eters on th e ret in a. Th ese can calcify an d m ay be seen on
a CT scan .
Gian t drusen of th e opt ic n er ve h ead
Angiofibrom as of th e eyelids, colobom as, an d p apilledem a m ay be seen .
Management
A m ult idisciplin ar y team approach is requ ired w ith p eriodic m on itoring for in ter-
n al t um ors. Progn osis is gen erally p oor, w ith death by th e secon d or th ird decade.
Fig. 15.9 Bourneville disease spot

in a patient with suspected tuberous
sclerosis.
Sturge -Weber Syndrome (Encephalofacial Cavernous

Hemangiomatosis)
Th is n eu rocu tan eous disorder com prises angiom as involving th e leptom en inges
(leptom en ingeal angiom as) an d skin of th e face, t yp ically in th e op h th alm ic (V1)
an d m axillar y (V2) dist ribu t ion s of th e t rigem in al n er ve (n evu s flam m eu s or p or t-
w in e st ain ). It h as n o defin ite in h erit an ce p at tern .
Presentation
Classificat ion u ses th e Roach scale:
Type I: Both facial an d leptom en ingeal angiom as; m ay h ave glau com a
Type II: Facial angiom a alon e (n o CNS involvem en t); m ay h ave glau com a
Type III: Isolated leptom en ingeal angiom as; usually n o glau com a
Cran ial h em angiom as, cerebral calcificat ion , m en t al retardat ion , an d seizures
m ay also be seen .
Ocular involvem ent m ay consist of glaucom a in 30 to 71%, w h ich m ay develop
early or in adulth ood. Conjun ctival or episcleral hem angiom as, h eteroch rom ia of
th e iris, an d ch oroidal h em angiom as m ay also be seen . Tom ato-catsup color of th e
fundus m ay be seen ipsilateral to the n evus flam m eus. Ch oroidal hem angiom as
m ay lead to RPE degen erat ion, fibrous m etaplasia, cyst ic retin al degen erat ion, an d
retinal detach m en t. Retinal vascular tort uosit y, iris h eteroch rom ia, optic disk colo-
bom a, an d cataracts m ay also be seen in th ese patien ts.
Management
System ic evalu at ion sh ou ld be don e by an in tern ist . Treat m en t in clu des yearly
exam in at ion s, looking for opt ic n er ve dam age (w ith m easurem en t of in t raocular
pressure an d visual fields) an d corn eal diam eter an d refract ive ch anges in ch il-
dren . Ch oroidal lesion s m ay be t reated w h en in dicated w ith ph otocoagulat ion , dia-
th erm y, cr yoth erapy, an d local irradiat ion .
Von Hippel-Lindau Syndrome (Retinocerebellar Capillary

Hemangiomatosis)
Von Hippel-Lin dau Syn drom e is a ben ign capillar y h am artom a w ith au tosom al
dom in an t in h erit an ce w ith variable pen et ran ce.
Presentation
Hem angioblastom as in th e cerebellum an d oth er organ s of th e body along w ith
cyst s of th e pan creas an d kidn eys, ph eoch rom ocytom a, an d hypern eph rom a m ay
be seen . Ocu lar involvem en t in cludes th e follow ing:
Ret in al capillar y h em angiom as, 1 to 3 disk diam eters in size an d supplied by a
feeder ar ter y, m ay be seen .
Ch oroidal m ass (t u m or/m et ast asis)
Ret in al telangiect asis
Ret in al m acroan eur ysm s
Leakage from th ese vessels an d h em angiom as m ay lead to ret in al exu dates, fi-
broglial ban ds, t ract ion ret in al detach m en t , an d vit reou s h em orrh age.
Management
Van illylm an delic acid levels in u rin e an d im aging st udies are requ ired w ith peri-
odic evaluat ion .
Wyburn-Mason Syndrome (Racemose Hemangiomatosis)

Ar terioven ou s m alform at ion s (AVMs) are seen in th e CNS an d th e ret in a.
Presentation
Neu rological sym ptom s m ay be seen depen ding on th e locat ion an d severit y.
Classifica tion of Retina l Arteriovenous Anastomoses (Archer et a l)
Group I: Sm all arteriole-ven u le an astom oses, w h ich m ay be su btle an d difficu lt
to detect clin ically. Th ese vessels are usually isolated to a sector or quadran t of
th e ret in a, an d th ey often involve th e m acula.
Group II: Represen t s direct ar ter y-to-vein com m un icat ion w ith ou t in ter ven ing
capillar y or ar teriolar elem en t s. Th is group m ay represen t an exaggerated form
of th e abn orm alit ies in cluded in group I, an d it is likew ise geograph ically seg-
m en ted w ith in th e fu n du s.
Group III: In cludes m alform at ion s ch aracterized by m arkedly convolu ted, di-
lated, an d tor t uou s ret in al vessels exten ding th rough out th e en t ire fun du s,
m aking it vir t ually im possible to differen t iate bet w een ar terial com pon en t s
an d ven ous com pon en t s. Th ese eyes are u sually severely vision im paired, w h ich
gen erally leads to earlier diagn osis in ch ildh ood. Pat ien t s in th is group are at
h igh er risk for system ic vascu lar involvem en t .
Management
Refer for n eurological evalu at ion , an d perform rout in e, periodic oph th alm ic ex-
am in at ion s.
Ataxia-Telangiectasia (Louis-Bar Syndrome)

Presentation
Th is is an au tosom al recessive disorder w ith m u lt isystem involvem en t in th e form
of progressive n eu rological im p airm en t , cerebellar at axia, variable im m un odefi-
cien cy w ith suscept ibilit y to sin opulm on ar y in fect ion s, im paired organ m at ura-
t ion , x-ray hypersen sit ivit y, ocu lar an d cut an eou s telangiect asia, an d a predisposi-
t ion to m align an cy.
Bu lbar conjun ct ival telangiect asias along w ith ocu lom otor sign s are diagn ost i-
cally im por tan t . Saccadic im balan ce m ay also be seen along w ith squin t an d nys-
tagm u s.
Management
Laborator y st udies for detect ing h u m oral or cellular im m u n ologic defect s, im ag-
ing st u dies, elect ro-ocu lography, an d ocular evalu at ion are requ ired. Th e pat ien t
sh ould be referred to an in tern ist for t reat m en t of in fect ion s.
16 Contact Lenses
Kenneth M. Daniels
Corneal Edema
Corn eal edem a is th e leakage of fluid in to th e corn ea du e to a ch ange in th e en -

doth elial p um p m ech an ism leading to fluid in flu x in to th e st rom al layer. Corn eal
st rom a, m ade of hydroph ilic glycosam in e glycan s an d glycop rotein s (GAGS) can
absorb a t rem en dous am oun t of w ater. Flu id leakage du e to defect s in th e en do-
th elium m ore so th an th e epith eliu m leads to fluid in t u m escen ce w ith subsequ en t
disru pt ion to th e regularit y of th e collagen fibrils seen as eith er st riae, folds, or
cen t ral corn eal clouding ( Fig. 16.1 ).
A B
C D
Fig. 16.1 (A) Central corneal striae. (B) Corneal folds. (C) Retroillumination of corneal
folds. (D) Parrellapiped image of folds. (Continued on page 448)
447
E F
Fig. 16.1 (Continued) (E) 4+ (severe) corneal edema. (F) Clinically significant corneal
edema.
Presentation
St riae are recogn ized as fin e, m esh like, grayish w h ite ver t ical lin es th at do n ot
in tercon n ect . Th ey m ay appear sim ilar to w h ite bran ch ing n eural fibrils. Con t act
len s–in duced hypoxia leads to a ch ange in th e h om eostat ic balan ce of th e corn ea
an d th e det u rgen ce or u pt ake of flu id in to th e corn eal st rom a. Th e su btle in crease
in flu id in duces sw elling of collagen fibrils leading to th e appearan ce of th e fin e,
m esh like ch aracter of st riae. Th e st riae w ith con t act len s corn eal edem a ten d to
appear sligh tly m ore obliqu e th an th e Vogt vert ical st riae associated w ith kerato-
con us. Th ey are graded as in Table 16.1 .
Folds are seen as black, ver t ical criss-crossing creases th at represen t a buckling
of th e corn ea at th e posterior st rom a–Descem et layer. Both fin dings are best ob-
ser ved in direct focal illu m in at ion u sing a m oderately angled p arallelepiped.
Table 16.1 Grading Co rneal Striae
Grade Finding
Grade 0 No striae
Grade 1 One to t wo faint lines
Grade 2 Two to six lines
Grade 3 Greater than six lines without folds
16 Contact Lenses 449
Table 16.2 Co rneal Edem a Levels
Co rneal
Sw elling Signs Relatio nships Level
< 2% Undetectable edema Unknown Benign

2 to 5% Early stages of striae Implies chronic hypoxia Safe
> 5% Vertical striae observed Chronic hypoxia Caution
> 8% Posterior folds and striae Acute edema Danger
> 20% Loss of corneal Pathological Pathological
transparency, folds,
striae
Cen t ral corn eal clouding or h aze occurs at th e corn eal apex as a w h iten ed region
th rough out th e st rom al depth , w h ich w ill bu lge. Th e cen t ral corn ea w ill exh ibit se-
vere ep ith elial th in n ing w ith su bsequen t redu ct ion in vision an d len s in toleran ce.
Cen t ral corn eal clou ding is best visu alized w ith sclerot ic scat ter an d/or m oderate
angle parallelepiped ( Table 16.2 ).
In th e in it ial stages, pat ien t s w ill gen erally h ave n o sym ptom s oth er th an a m i-
n or to m oderate level of len s aw aren ess, glare sen sit ivit y, an d h alos. Visu al acuit y
w ill be sligh tly to m oderately reduced an d possibly n otable keratom et ric–topo-
grap h ic distor t ion w ill be presen t . Pachym et r y w ill be sh ow ing a m ild to m oderate
in crease from baselin e. As th e corn eal edem a progresses, th ere w ill be a m arked
visu al distor t ion appreciated by th e pat ien t as w ell as n ot iceable cloudin ess or
h aze to vision . If th e pat ien ts en ter th e severe state, th en th ere w ill a m u ch defin ed
decrease in vision an d in creased ocu lar discom for t accom p an ied by a variable
level of “red eye” an d ocu lar congest ion . Th is m ay act ually m im ic a form of iri-
docyclit is. If epith elial breaks subsequ en tly occu r, th ere is a h igh er poten t ial for
oppor t un ist ic in fect ion .
Tigh t len s syn drom e, corn eal w arpage, corn eal u lcer, oth er cau ses of con tact len s–
related discom fort , an d redn ess. Rule ou t oth er corn eal degen erat ion s an d dyst ro-
ph ies su ch as keratocon u s, pellucid, posterior p olym orph ous dyst rop hy, or Fuch s
en doth elial dyst rophy, as w ell as postoperat ive edem a, acutely elevated in t raocu -
lar pressure (IOP), or iridocyclit is.
Management
Discon t in ue w earing len ses un t il resolu t ion . Clin ical aftercare an d pat ien t educa-
t ion n eed to be m ore aggressive to m on itor for m ore advan ced sign s of edem a an d
possible corn eal irregularit ies via biom icroscopy, pachym et r y, topography, en do-
th elial cell cou n t , an d refract ion . En doth elial cell cou n t w ill be n ecessar y on ly if
on e suspects en doth elial cell disease or loss. Pachym et r y of th e n orm al corn eal
th ickn ess m easu res ~0.55 m m cen t rally, progressing in n at u ral th ickn ess to ~0.8
m m in th e corn eal periph er y. Result an t corn eal edem a associated w ith variou s
disease st ates w ill be su spect w ith th e cen t ral corn eal th ickn ess above 0.6 m m .
Serial m easu res w ill assist in th e determ in at ion of t reat m en t su ccess.
If th e corn eal edem a is less th an 5%(caut ion ), th e pat ien t requ ires a refit to h igh
w ater con ten t hydrogel or silicon e hydrogel m aterial. High w ater con ten t m aterials
th at m ain tain proper hydrat ion are crit ical, an d th erefore a grou p 2 n on ion ic, h igh
w ater m aterial is suggested. A refit to a h igh diffu sion coefficien t rigid gas per-
m eable len s is also h igh ly advisable. Both gas perm eable an d soft len s m odalit ies
sh ould be st rictly p rescribed for a daily w ear sch edule; exten ded w ear sh ou ld be
forbidden . If th e edem a is greater th an 5%, but less th an 8% (m oderate to danger-
ous), th e sam e con tact len s refit approach is pu rsued w ith th e in ten t of in t rodu c-
ing m edicin al in ter ven t ion based on subject ive fin dings, su ch as reduced visu al
acuit y, clin ically sign ifican t corn eal h aze, an d pachym et r y.
Fin ally, if th e edem a is 9%or m ore (path ological), len ses n eed to be discon t in ued
w ith th e adm in ist rat ion of eith er or both hyper ton ics an d steroids to draw corn eal
fluids an teriorly out of th e st rom a. At th is st age, corn eal irregularit ies such as m i-
crocyst ic eru pt ion s, a decrease in epith elial th ickn ess, an d an in crease in st rom al
th ickn ess w ill be n oted. Len ses sh ou ld n ot be refit u n t il m icrocyst s part ially re-
solve an d th e edem a is redu ced. W h en refit , th e pat ien t sh ould lim it th e h ours of
w ear an d be rest ricted to daily w ear h igh dK w ater con ten t len ses.
Hyp erosm ot ic agen t s su ch as sodium ch loride 2 to 5%drops or oin t m en t are th e
first n on m edicin al t reat m en t . Steroid agen ts su ch as predn isolon e 1% w ith a taper
to fluorom eth olon e or lotepredn ol 1% w ill be of assist an ce in a m ore rapid redu c-
t ion of appreciable corn eal h aze from edem a. Caut ion sh ould be taken if th ere
is bacterial or viral co-disease or if th e pat ien t m ay be a steroid respon der. Also,
ow ing to th e su perficial corn eal t reat m en t , a steroid of a ph osph ate agen t , low er
corn eal–an terior ch am ber pen et rat ion is m ore appropriate th an an acet ate prepa-
rat ion . In gen eral, based on th e level of corn eal edem a, t reat m en t result s m ay be
seen w ith in 24 to 48 h ou rs of len s discon t in uan ce alon e, but resolut ion in relat ion
to severit y w ill take p lace over 2 days to 2 w eeks. As a n ote, if th e corn eal edem a
is related to a disease state, such as pseudoph akic bullou s keratopathy, a ban dage
con tact len s sh ould be con sidered for pain con t rol as w ell as allow ing th e dosing
of steroid as n ecessar y.
Contact Lens–Induced Papillary Conjunctivitis or

Giant Papillary Conjunctivitis
Con tact len s–in du ced papillar y conjun ct ivit is (CLPC) w as first described as a “con -
tact-related spring cat arrh ” or vern al keratoconjun ct ivit is (VKC). Later, gian t papil-
lar y conju n ct ivit is (GPC) received it s n am e from Allan sm ith et al, w h o described
th e palp ebral conju n ct ival lid form at ion s as an elevat ion w ith a cen t ral core vascu -
larizat ion , u n like a follicle, w h ich is a flu id-filled cyst form at ion .
Presentation
CLPC is a ch ron ic in flam m ator y respon se isolated to th e su perior lid in relat ion
to eith er m ech an ical or an t igen ic respon se, foun d m ore so w ith longer-du rat ion -
w ear hydrogel len ses th an w ith gas-perm eable len ses. (With longer du rat ion w ear
hydrogel len ses [con t in uous, 14 to 30 days] > exten ded [3–7 days] > flexible [1–3
days] > daily w ear [1 day] > single use or daily disposable len ses.) It is described
as a cut an eou s basoph ilic hypersen sit ivit y to surface protein s. It is con sidered th at
th e com bin at ion of th e irritat ive m ech an ics in du ced by surface deposit s as w ell as
th e an t igen ic respon se of th e den at u red surface protein s w ith in th e dep osits lead
to th e in flam m ator y resp on se an d th e developm en t of th e papillae. Mech an ical
irrit at ion of th e conju n ct iva n ot on ly occu rs w ith con t act len ses bu t can also occur
in isolated areas associated w ith su t u res. Also, th e in t roduct ion of silicon e hydro-
gel con t act len s m aterials, an d th e related con t in u ou s w ear, h as given a rebirth to
an in creasing in ciden ce of CLPC. Th is is related to a h igh er m odulus of elast icit y
(1.1 to 1.2 m egapascals) m aking th e len s st iffer th an hydrogel len ses. Th e rigidit y
of th e m aterial en courages m ech an ical irrit at ion by ru bbing again st th e su perior
palp ebral conju n ct iva, producing a local respon se.
Papillae are m orph ologically differen t from follicles an d less severe com pared
w ith an acu te, hypersen sit ive follicu lar or cobbleston e ap pearan ce of a vern al con -
jun ct ivit is. GPC or CLPC is best obser ved using a w h ite, diffuse ligh t on low m ag-
n ificat ion . Papillae, w h ich are space-occu pying elevat ion s, w ill ten d to grab th e
len s u pon th e blin k an d h old it in a sligh tly su perior, decen tered posit ion w h ile
im peding th e dow nw ard t ran slat ion of th e len s. Because of th e con stan t irrit at ion
of th e palpebral conju n ct iva, a react ive m u cous disch arge w ill in crease, leading to
addit ion al len s su rface deposit ing.
Th e palpebral conju n ct iva can be described as sm ooth or sat in , un iform or n on -
un iform . Un der low m agn ificat ion , w ith or w ith ou t NaFl stain ing, a variable level
of hyp erem ia w ill appear w ith an in creasing degree of edem a. Th e papillae w ill be
of var ying sizes, from 0.5 m m an d greater. With th e in st illat ion of NaFl, dist in ct
crevices can be visu alized bet w een each papillae, w h ich w ill assist in th e delin ea-
t ion of th e severit y of th e pap illar y react ion .
In th e earliest stages th e pat ien t is gen erally asym ptom at ic yet com plain s of fre-
qu en t deposit ing of len ses an d variable vision . As th e con dit ion progresses, th e
pat ien t st ar t s to fin d th at clean ing th e len ses becom es som ew h at fu t ile, an d th ere
is a sligh t to m ore puru len t m ucou s disch arge. Th is m ay or m ay n ot be sym m et ric
to both eyes an d is often bilateral, asym m et ric in presen tat ion . As th e disch arge
an d th e vision depreciate, so does th e pat ien t’s len s-w earing t im e. Th e pat ien t w ill
also fin d th at th e len s ten ds to decen ter sign ifican tly an d m ay even com plain of a
greater len s aw aren ess. Most often th e pat ien t w ill presen t w ith a self-diagn osis
of a “com m on conjun ct ivit is or red eye” an d m ay h ave self-t reated w ith over-th e-
cou n ter vasocon st rict ive agen ts or been t reated by th e prim ar y care physician for
a “garden variet y” bacterial conjun ct ivit is w ith out respon se to an t ibiot ics. Th ere
m ay be som e ten dern ess to t act ile m an ipulat ion of th e lid bu t n o sign ifican t pain ,
n or do system ic sym ptom s suggest bacterial or viral conju n ct ivit is.
CLPC can be st aged in to four levels of severit y. Stage 1 dem on st rates n o an a-
tom ical sign s, an d on ly m in or sym ptom s of m u cous disch arge an d itch ing. Stage
2 exh ibit s p apillar y en largem en t to 0.5 m m but less th an 1 m m , m ucou s st rain s,
hyperem ia, an d an in crease in len s deposits. Th e pat ien t w ill describe itch in ess,
disch arge, len s aw aren ess, an d blu rred acu it y. Stage 3 sh ow s papillae greater th an
1 m m , in creased m u cu s, len s aw aren ess, hyperem ia, edem a, an d len s decen t rat ion
su periorly. Th e pat ien t w ill describe m oderate to severe sym ptom s w ith decreased
w ear t im e, frequ en t len s deposit ing, an d in creased len s m ovem en t an d blu r. Stage
4 dem on st rates pap illae larger th an 1 m m , w h ich h ave a m u sh room sh ape accom -
pan ied by severe sym ptom s an d sign s ( Fig. 16.2 ).
A B
Fig. 16.2 (A) Papillary formation right eye (ocular dextrose, OD). (B) Nonpapillary
satin left eye (ocular sinistras, OS). (C) Grade 1 contact lens–induced papillary conjunc-
tivitis (CLPC) marginal papillary formation with hyperemia Pre -Tx OD. (D) Grade 3 CLPC
with isolated giant papillary formation with hyperemia Pre-Tx OS. (E) Grade 1 CLPC with
hyperem ia post-Tx OD 2 weeks: significant decrease in hyperemia and early papillary
formation.
Fig. 16.2 (Continued) (F) Grade 3 CLPC with hyperemia post-Tx OS 2 weeks: signifi-
cant decrease in hyperem ia and early papillary form ation. (G) Zonal diagram of the su-
perior everted lid.
Involves iden t ifying th e un derlying cu lprit of m ech an ics, protein den at urat ion ,
an t igen ic-related respon se, or environ m en t al in flu en ces giving rise to sim ilar fin d-
ings an d sym ptom s exacerbated by th e use of con t act len ses, yet th ey are n ot th e
cau se. By h istor y alon e, th e et iology an d differen t ial can be m ade. If th ere is hy-
drogel con tact len s w ear, exten ded m ore th an daily; if th ere is a h istor y of cat aract
or corn eal procedures w ith su t u res; or if th e pat ien t h as a sign ifican t h istor y of
season al or vern al allergy, th e h istor y is th e t ru e stor y. Treat m en t w ill th us follow
th e scen ario of th e h istor y.
CLPC m ay also presen t w ith sim ilar sym ptom s t ypical of a variet y of conjun ct i-
vit is, in cluding bacterial, viral, vern al, atopic, or m ech an ically in du ced by su t u res
postoperat ively. Th e h allm ark differen t ial of CLPC is a m ore rapid on set w ith in -
creased m u cous disch arge an d th e in abilit y to properly m ain tain th e con t act len s
on th e eye, u su ally exh ibited by excessive len s m ovem en t du e to th e m ech an ical
in fluen ce of th e en larged pappilae. Follicles of variable size are seen in a hyper-
em ic conjun ct iva, in ferior m ore so th an sup erior (un like CLPC seen in th e su perior
palp ebral conju n ct iva) w ith t ran slu cen t , avascular fluid–lym p h oid accu m ulat ion
an d are accom pan ied by system ic fin dings as in ph ar yngoconjun ct ival fever (PCF)
or epidem ic keratoconjun ct ivit is (EKC), as w ell as ru ling out ch lam ydial disease.
Th e Academ y of Op h th alm ology h as presen ted an excellen t differen t ial form at for
conju n ct ivit is as seen in Table 16.3 .
Table 16.3 Typical Clinical Signs o f Co njunctivitis
Typical Clinical Signs o f Co njun ctivitis
Type o f Co njunctivitis Clinical Signs
Allergic/immunologic
Seasonal allergic Bilateral; conjunctival injection, chem osis, watery
discharge, mild m ucous discharge
Vernal Bilateral; giant papillary hypertrophy of superior

tarsal conjunctiva, bulbar conjunctival injection,
conjunctival scarring, watery and mucoid discharge,
limbal Trantas dots, limbal “papillae,” corneal
epithelial erosions, corneal neovascularization and
scarring, corneal vernal plaque/shield ulcer
Atopic Bilateral; eczematoid blepharitis; eyelid thickening,

scarring; lash loss; papillary hypertrophy of superior
and inferior tarsal conjunctiva; conjunctival scarring;
watery or mucoid discharge; boggy edem a; corneal
neovascularization, ulcers, and scarring; punctate
epithelial keratitis; keratoconus; subcapsular cataract
Giant papillary Lateralit y associated with contact lens wear pat tern;
papillary hypertrophy of superior tarsal conjunctiva,
mucoid discharge; in severe cases: lid swelling, ptosis
Mechanical/irritative
Superior lim bic Bilateral superior bulbar injection, laxit y, edem a, and
keratoconjunctivitis keratinization; superior corneal punctate
(SLK) epitheliopathy and laments
Contact lens-related SLK Injection of superior bulbar conjunctiva, epithelial

thickening of limbus with neovascularization and/or
extension of conjunctival epithelium onto superior
cornea; papillary hypertrophy of tarsal conjunctivitis
is variable
Floppy eyelid syndrome Upper eyelid edema; upper eyelid easily everted,
som etimes by simple elevation or lifting of lid; di use
papillary reaction of superior tarsal conjunctiva;
punctate epithelial keratopathy; pannus; bilateral
often asym metric
Pediculosis palpebrarum Unilateral or bilateral follicular conjunctivitis; adult lice

(Pthirus pubis) at the base of the eyelashes, nits (eggs) adherent
to the eyelash shafts, blood-tinged debris on the
eyelashes and eyelids
Medication-induced Lateralit y based on drug use; conjunctival injection,

keratoconjunctivitis inferior fornix conjunctival follicles; distinctive signs:
contact dermatitis of eyelids with erythema, scaling
in some cases
Table 16.3 (Continued) Typical Clinical Signs o f Co njunctivitis
Viral
Adenoviral Abrupt onset; unilateral or bilateral; varies in severit y;
bulbar conjunctival injection, watery discharge,
follicular reaction of inferior tarsal conjunctiva,
chemosis
Distinctive signs: preauricular lymphadenopathy,
petechial and subconjunctival hemorrhage, corneal
epithelial defect, multifocal epithelial punctate
keratitis evolving to anterior stromal keratitis,
mem brane/pseudom em brane formation, eyelid
ecchymosis
Herpes simplex virus Unilateral: bulbar conjunctival injection, watery

discharge, mild follicular reaction of conjunctiva; may
have palpable preauricular node
Distinctive signs: vesicular rash or ulceration of eyelids,
pleom orphic or dendritic epithelial keratitis of cornea
or conjunctiva
Molluscum contagiosum Typically unilateral but can be bilateral: m ild to

severe follicular reaction, punctate epithelial keratitis;
may have corneal pannus, especially if longstanding
Distinctive signs: single or multiple shiny, dom e-shaped
umbilicated lesion(s) of the eyelid skin or margin
Bacterial
Nongonococcal Unilateral: bulbar conjunctival injection, purulent or
m ucopurulent discharge
Gonococcal Unilateral or bilateral: marked eyelid edem a, marked

bulbar conjunctival injection, marked purulent
discharge, preauricular lymphadenopathy
Important sign to detect: corneal in ltrate
Chlamydial Unilateral or bilateral

Neonate/infant Eyelid edema, bulbar conjunctival injection, discharge
may be purulent or mucopurulent, no follicles
Adult Bulbar conjunctival injection, follicular reaction of

tarsal conjunctiva, mucoid discharge, corneal
pannus, punctate epithelial keratitis, preauricular
lymphadenopathy
Immune -mediated Distinctive sign: bulbar conjunctival follicles

Ocular cicatricial Bilateral: bulbar conjunctival injection, papillary
pemphigoid conjunctivitis, conjunctival subepithelial
brosis and keratinization, conjunctival scarring
beginning in the fornices, punctal stenosis and
keratinization, progressive conjunctival shrinkage,
symblepharon, entropion, trichiasis, corneal ulcers,
neovascularization, and scarring
(continued on page 456)

Table 16.3 (Continued) Typical Clinical Signs o f Co njunctivitis
graft-versus-host Bilateral; conjunctival injection, chemosis,

disease pseudomembranous conjunctivitis,
keratoconjunctivitis sicca, superior lim bic
keratoconjunctivitis, cicatricial eyelid disease,
episcleritis, corneal epithelial sloughing, limbal stem
cell failure, calcareous corneal degeneration; rare
intraocular involvem ent
Neoplastic
Sebaceous gland Unilateral: intense bulbar conjunctival injection,
carcinoma conjunctival scarring; corneal epithelial invasion may
occur
Eyelids may exhibit a hard nodular, nonm obile mass
of the tarsal plate with yellowish discoloration; may
appear as a subconjunctival, m ultilobulated yellow
mass, may resem ble a chalazion
Note: Typical clin ical sign s m ay n ot be p resen t in all cases. Dist in ct ive signs are m ost u sefu l in
m aking a clinical diagn osis, bu t m ay occu r u n com m on ly. In all en t it ies, lateralit y m ay var y and
m ay be asym m et rical.
Source : Matoba AY. Preferred Pract ice Pat tern s, Conjun ct ivit is. San Fran cisco: Am erican Acad em y
of Op h th alm ology; 2003. Available at: h t t p ://w w w.aao.org/aao/edu cat ion /librar y/p pp /u p load/
Conjun ct ivit is_.p df. (Accessed 12–08–2008). Reprinted w ith p erm ission .
Management
As w ith any poten t ial an t igen ic–allergic respon se, on e m u st first rem ove th e in i-
t iat ing st im u lus. Th e sim p le rem oval an d discon t in u an ce of th e len s are th e easi-
est t reat m en t , bu t for som e pat ien ts th e m ost t rau m at ic. Th erefore a com bin at ion
th erapy is suggested based on th e level of disease. It is im port an t to n ote th at th e
use of vasocon st rict ive agen ts, an t ibiot ics, an d/or an t ivirals th at h ave been star ted
prior to presen tat ion can be safely discon t in u ed. How ever, if th ere is con cern abou t
bacterial coin fect ion , m ain t ain th e appropriate level of an t ibiot ics such as four th -
gen erat ion fluoroquin olon e w h en involving con tact len s.
The m ost expedient treatm ent is the sim ple discontinuation of w earing contact
lenses and converting to eyeglass w ear until the condition im proves. How ever, in
m any cases, the patient m ay not have eyeglasses or m ay not be tolerant of the alter-
native. Therefore, the use of soft daily disposable lenses w ith a high m oisture content
in conjunction w ith a steroidal antiinflam m atory serves as the best overall therapy.
Stage 1 CLPC requires m inim al intervention, such as refitting the patient w ith a
frequent replacem ent or disposable lens. In this situation, the simple conversion to a
“daily disposable–single use lens” is the m ost appropriate. Another option would be to
continue conventional, disposable, or frequent replacem ent lenses but change the care
product to a peroxide-based system and possibly add an enzym atic cleaning solution.
In an unpu blish ed st u dy by K. Dan iels, daily disposable len ses dem on st rated a
m ore rapid resolu t ion of p at ien t sym ptom s w ith grade 2 to 4 CLPC w ith ou t th e u se
of m edicin als follow ed by 1-w eek an d 2-w eek disposable len ses, respect ively. Th is
suggest s th at th e sim ple u se of daily disposable–single use len ses m igh t be th e
m ost appropriate single or adjuvan t t reat m en t for CLPC.
St age 2 CLPC requ ires len s replacem en t , frequen t irrigat ion w ith lu bricat ing
drops to rid m ucus, lid hygien e to avoid lid w ipers epith eliopathy, an d possibly
a prescript ion for a m ast cell st abilizer such as such as lodoxam ide (Alom ide, Al-
lergan , Ir vin e, CA), crom olyn (Crolom , Bausch & Lom b, Roch ester, NY) (Opt icrom ,
Allergan , Ir vin e, CA), n edocrom il (Alocril, Allergan ), pem irolast (Alam ast , Vist akon
Ph arm aceu t icals, Jacksonville, FL), or olopatadin e (Pataday or Patan ol, Alcon Labo-
ratories, For t Wor th , TX) for sh ort-term to ch ron ic th erapy.
Stage 3 CLPC requires a discontinuation of lenses for a short tim e w hile prescribing
a m ast cell stabilizer or low -concentration steroid such as prednisolone 1%. Lenses can
be refit to daily disposable or short-term frequent replacem ent lenses w ith peroxide
care products until resolution of the papillae to a w hitened cap called hypertrophy.
St age 4 CLPC requires com plete discon t in u at ion of len ses an d m ore aggressive
steroid in ter ven t ion u n t il resolu t ion . Up on resolut ion , frequ en t replacem en t or
disposable len ses sh ould be fit ted u sing a peroxide-based product system .
In gen eral, w h en u t ilizing steroids for t reat m en t , it is h igh ly suggested to adju -
vantly treat the patient w ith single-use daily disposable lenses, w hich w ill satisfy
the patient’s needs w hile allow ing for a bandage lens–drug delivery efficiency. The
steroid (prednisolone 1%) should be aggressively dosed for the first 1 to 2 w eeks at
four tim es a day and then taper slow ly over the next 2 to 3 w eeks. As one tapers
the prednisolone dow n to t w ice a day, start the addition of a soft steroid such as
loteprednol either 1%or 0.2%for 1 to 2 weeks or until resolution of clinical findings.
Long-term m aintenance is m ost appropriate w ith shorter-term , frequently replaced
hydrogel lenses of 2 to 4 w eeks of nonionic, high-w ater-content m aterials and per-
oxide cleaning or a conversion to gas perm eable high Dk–plasm a-treated designs.
Also con sider th e long-term u se of an t ih ist am in e–m ast cell st abilizer for long-
term m ain ten an ce. Addit ion ally, if on e w as to be con ser vat ive w ith long-term m e-
dicin als, con sider t w ice-a-day to th ree-t im es-a-day u se of physiologically based
w et t ing drops such as vit am in A drops (ViVa, Corn eal Scien ces, Gaith ersbu rg, MD)
or elect rolyte-balan ced form ulas such as Th era–Tears (Advan ced Vision Research ,
Boston , MA) or Sooth e (Bau sch & Lom b, Roch ester, NY; Alm ira Scien ces, Atlan t a,
GA). Th ere is also suggest ion th at cyclosporin e drops (Rest asis, Allergan , Ir vin e, CA)
m ay also be h elpful in a t w ice-a-day dosage for long-term con t rol of ocular in flam -
m ator y respon se as w ell as being a veh icle carrier h elpfu l in m ain tain ing a h ealthy
ocu lar m ucin surface to avoid m ech an ical irritat ion from th e len s m aterial.
Vascularization
Vascularizat ion is con sidered to be th e gen eral form at ion an d exten sion of capil-
laries th at h ad n ot previously existed w ith in th e avascu lar corn ea. Neovasculariza-
t ion is th e form at ion of n ew vessels as an exten sion or sh u n ts to preexist ing vas-
cularized areas of th e avascular corn ea. To differen t iate fur th er is to classify form s
of redn ess an d vascu larizat ion by locat ion . Lim bal engorgem en t or hyperem ia is
th e disten sion of lim bal blood vessels in th e absen ce of n ew vessel grow th . Vessel
ingrow th or pen et rat ion is n ot n eovascularizat ion , bu t sim ply an exten sion of a
vessel inw ard tow ard th e cen t ral corn ea. Pan n u s, w h ich is h igh ly vascularized, is
exten sion of conju n ct ival t issue overlapping th e clear avascular corn ea seen as an
an atom ical varian ce or in duced by t rau m a to th e eye.
Ch ron ic hypoxia is th e u n derlying con dit ion th at in it iates th e vascu larizat ion
respon se. Hypoxia cau ses lactate acidosis, w h ich decreases th e in tegrit y of th e epi-
th elium an d st rom al soften ing. Th is yields an oppor t un it y for vessel ingrow th . Du e
to hyp oxia, an early ph ase of vascu larizat ion occu rs in ducing a release of in flam -
m ator y m ediators. Th is st im ulates addit ion al vessel grow th called an angiogenic
response. Tigh t len ses, lim bal com pression , an d/or t rau m a m ay also st im ulate a
vascu lar respon se, w h ich w ill in crease th e release of in flam m ator y an d vasost im u -
lator y m ediators. Th ere are several p ossibilit ies th at en cou rage vascu larizat ion un -
der hypoxic con dit ion s: (1) vasost im u lat ion an d in flam m at ion , (2) t igh t len s syn -
drom e, (3) lim bal-plexal com pression , (4) solut ion sen sit ivit y, an d (5) vasogen ic
resp on se to t raum a ( Fig. 16.3 ). Th e m ain cau ses of vascularizat ion associated w ith
con tact len s w ear are t igh t len s (edge su ct ion ) cen t ral corn eal edem a (hyp oxia),
solut ion toxicit y, m ech an ical abrasion , len s dam age an d m ech an ical st im u lu s as-
sociat ion w ith irrit at ion , surface dep osit ion , an d poorly fit t ing len ses.
A B C
D E F
G H I
Fig. 16.3 (A) Limbal congestion–hyperemia. (B) Com bination of vasodilatation

(corkscrew vessels)–vasoproliferation–vasolimbal congestion. (C) Severe lim bal vas-
cularization congestion with early neovascularization. (D) Vessel penetration with
early pannus. (E) 4+ superior lim bal neovascularization leading toward the central
cornea and papillary zone encroachment. (F) Intracorneal hemorrhage from neovas-
cularization post-LASIK on an extended-wear contact lens wearer. (G) Sectoral pan-
nus associated with overwear. (H) Sectoral pannus with corneal decompensation.
(I) Sectoral pannus with corneal decompensation–placido topographic image.
Presentation
Vascular respon ses to ch ron ic hyp oxia can var y from m in or to severe based on th e
associat ion w ith possible m icrobial in filt rat ion . Som e form of corn eal vascular-
izat ion occurs in ~34% of cases associated w ith hydrogel len s u se versu s 2% w ith
n on len s w earers, w ith 98% of th e vascularizat ion occu rring w ith in th e superficial
st rom a. Th e pat ien t is oth er w ise asym ptom at ic oth er th an n ot ing an apparen t hy-
perem ia or lim bal engorgem en t , w h ich app ears to th e pat ien t as a “ch ron ic red
eye” w h en w earing con tact len ses. Th is is sim ply an engorgem en t of th e m argin al
arcade capillaries. Th ese vessels h ave a st raigh t prot uberan ce w ith a defin ed loop
at th e en d. Hyperem ic episcleral lim bal vessels are differen t iated from n eovessels
th at exten d for w ard w ith leaflike fron ds th at in terdigitate.
Vascu larizat ion appears sim ilar to a m esh like plexal grow th in th e m idepith e-
lium project ing tow ard th e corn ea like sm all, lin ear spikes an d bran ch es called
fronds (sim ilar to th e vein s w ith in a leaf). Th ere is gen erally n o sym ptom atology
associated w ith th e fin dings. Th ese are differen t iated from n orm al lim bal vessels
th at “loop” back tow ard th e lim bu s. Low -grade (grade 1) vessels w ill ten d to m i-
grate inw ard to approxim ately 0.4 to 0.6 m m (daily w ear) to 1.4 m m (exten ded
w ear). Grade 2 w ill be grade 1 vessels th at w ill ten d to m igrate tow ard th e pupil
w ith ou t p assing in to th e pupillar y zon e. Th e m ost severe, grade 3, w ill pen et rate
th e pu pillar y region . It is im por tan t to ph otodocum en t th e vessels an d determ in e
th e locat ion on th e lim bus, depth (su perficial or deep), degree of p en et rat ion , an d
severit y defin ed as th e depth of pen et rat ion (an d th e advan cem en t of grow th to-
w ard th e papillar y region ).
Vascularizat ion an d n eovascu larizat ion are differen t iated from lim bal vessels
th at m ay be dilatated by t rau m a, in fect ion , in flam m at ion , t um or, conjun ct ival in -
grow th or pter ygiu m , or postoperat ive com plicat ion . Differen t iat ion m ust also be
m ade from vascu larized pan n us, w h ich is th e ingrow th of vessels an d con n ect ive
w ith in th e epith eliu m . Th e differen t iat ion of vessel depth is im port an t . Su perficial
vessels or vascularizat ion in it iates from th e lim bal capillar y arcade, an d th e ves-
sels are m ore tor t u ou s an d of sm aller caliber th an deep st rom al vessels, w h ich
em erge from w ith in th e lim bal m idst rom al region an d are larger in caliber, h ave
abr upt en d bulbs, an d m ay disru pt th e regu larit y of th e lim bal corn ea. Oth er fac-
tors th at m ay be associated w ith con t act len s–related vascularizat ion are dr y eyes
(keratoconjun ct ivit is sicca) or ocular su rface disease an d oth er diseases, su ch as
bleph arit is, acn e rosacea, Sjögren syn drom e, an d im m u n e dysfun ct ion , as w ell as
in terst it ial kerat it is, h erpes kerat it is, t u bercu losis, m easles, syph ilis, an d th e pos-
sibilit y of am in o acid deficien cies.
Because of th e p oten t ial of n on –con t act len s–related con cern s associated w ith
corn eal vascularizat ion , biom icroscop ic exam sh ould in clude direct illu m in at ion
an d ret roillu m in at ion , part icu larly of th e lim bal periph eral vessel arcades. In gen -
eral, superficial vessels w ill em erge in to th e an terior st rom a an d appear as single
or m ult ip le (pan n u s) tor t uous vessels un der low m agn ificat ion , yet deeper st rom al
vessels course th rough th e corn ea as m ore lin ear vessels th at arborize. Lip id de-
posit ion appears as yellow -w h ite opacit ies at th e leading edge or surrou n ding th e
st rom al vessels. Obser vat ion of lipid exu dates su rrou n ding an act ively engorged
vessel(s) sh ould raise th e con cern of a possible iris-angle carcin om a requiring dili-
gen t gon ioscopic exam in at ion . If th ere is a conju n ct ival grow th closely ju xt aposed
to th e lim bu s adjacen t to th e corn eal vascularizat ion , conju n ct ival carcin om a m ay
be suspected.
Management
Th e pat ien t w ith lim bal hyp erem ia or vessel engorgem en t ten ds to self-t reat w ith
over-th e-cou n ter vasocon st rict ive agen t s. Th ese pat ien ts w ill fin ally presen t st at-
ing th at even w ith th ese agen t s th eir len ses feel dr y an d th eir eyes are red. In th is
sit u at ion , sim ply an d forcefu lly tell th e pat ien t to discon t in u e drops th at “get th e
red ou t .” Th ese drop s ten d to yield reboun d congest ion as w ell as a m ild m ydriasis
an d slow ing of accom m odat ion du e to th e sym path om im et ic effects. In th is sit u-
at ion , it is best to discon t in u e th e len ses for a sh or t period—2 to 3 days—an d pre-
scribe a soft steroid to rid any low -level in flam m ator y com pon en t s.
In conju n ct ion , im plem en t physiologically based w et t ing drops su ch as vit am in
A (ViVA, Corn eal Scien ces, Gaith ersbu rg, MD), Th era-Tears (Advan ced Vision Re-
search , Boston , MA), Bion Tears (Alcon Laboratories, For t Wor th , TX), or Sooth e
(Bau sch & Lom b, Roch ester, NY, Alm ira Scien ces, Atlan ta, GA). In addit ion to topical
su pplem en t at ion , con sider th e u se of om ega 3-6 com bin at ion s for th eir in h eren t
an t iin flam m ator y an d m u cin com plem en tar y abilit ies. Also, on e sh ou ld con sider
agen ts su ch as Syst an e (Alcon Laboratories, Fort Wor th , TX) or En dura (Allergan ,
Ir vin e, TX) for topical su rface t reat m en t .
After th e discon t in uan ce of th e con tact len s u se an d after vessel regression ,
th ere w ill be gh ost vessels or ch an n els th at develop. Th ese m u st be w ell docu -
m en ted to differen t iate th en from gh ost vessels th at m ay be associated w ith an
in terst it ial kerat it is.
On ce th e lim bal hyperem ia is ten ded to, a refit to con tact len ses sh ou ld be com -
pleted w ith th e ph ilosophy of h igh w etabilit y, m oderate m odu lu s, an d h igh oxygen
perm eabilit y. Th e pat ien t can be refit to n on ion ic, h igh -w ater-con ten t hydrogel,
silicon e hydrogel of a low er m odu lu s. If th e m odu lus is h igh , th ere ten ds to be st iff-
n ess to th e len s th at can in du ce conju n ct ival irritat ion an d repeat of conju n ct ival
hyperem ia. In addit ion , on e sh ou ld con sider a h igh diffu sion coefficien t of a rigid
gas p erm eable len s w ith su fficien t axial edge clearan ce to en h an ce th e flu id–tear
ch an n el an d avoid corn eal an d lim bal edge in flu en ces. Th ese len ses sh ould also in -
corporate an app ropriate plasm a t reat m en t for w et abilit y. Care produ cts an d len s
sch edu les sh ould be revisited, an d pat ien t educat ion n eeds to be com preh en sive.
In th e even t of m ore pron ou n ced an d progressive vascu larizat ion , such as n eo-
vascu larizat ion , on e m u st be con cern ed abou t th e fragilit y of th ese vessels.
It is n ot un com m on to obser ve an in t racorn eal h em orrh age as a direct sequ ela
of n eovascu larizat ion . If a h em orrh age occu rs, it w ill app ear as a “red spot on th e
corn ea” sim ilar to a “petech ial conjun ct ival h em orrh age” located at th e proxim al
en d of a vascular fron d. Th ese h em orrh ages sh ould be ph otograph ed an d m on i-
tored for spread. Th e pat ien t sh ou ld refrain from any agen t th at h as an an t icoagu-
lan t effect su ch as aspirin , Plavix, w arfarin , as w ell as n eut raceu t icals th at h ave an
an t ith rom bot ic ch aracter. Addit ion ally, th ese n eed to be w ell docum en ted as w ell
as th e n eovascu larizat ion in th e even t th e pat ien t decides to pursu e in t racorn eal
refract ive surgical procedu res in w h ich in t raoperat ive corn eal h em orrh age could
be sign ifican t .
Essen t ially, th e t reat m en t for any vascular respon se w ith in th e corn ea from con -
tact len ses is th e sam e, an d th e varian ce is th e un derlying cau se an d in ter ven t ion .
On ce th e pat ien t h as been properly refit , edu cat ion an d m on itoring are th e keys to
avoidan ce of recu rren ce. Addit ion ally, as part of th e t reat m en t gu idelin e, on e m ust
clin ically m on itor for issu es th at m ay in duce ocu lar irrit at ion an d vascu larizat ion
oth er th an con t act len ses, such as acn e rosacea, system ic m edicat ion s, an d con -
dit ion s th at in clu de con n ect ive t issu e disorders, autoim m u n e an d in flam m ator y
disease, im m u n ocom prom ised disorders, vascular st im u lator y disease su ch as kid-
n ey problem s an d diabetes, keratoconjun ct ivit is sicca, an d associated h orm on ally
related disorders of th e eye, as w ell as environ m en tal irritan t s, use of diu ret ics
(m edicin al or as beverage–alcoh ol an d caffein e), an d sm oking.
Subepithelial Infiltrates
Su bepith elial in filt rates (SEIs) are an in flam m ator y react ion secon dar y to ch ron ic
hypoxia or an acu te react ion th at th reaten s th e avascular corn ea an d an terior
segm en t . Th is acute react ion in duces an in flam m ator y react ion th at en courages
an aggregat ion or accu m u lat ion of cellu lar com pon en ts, su ch as p olym orph on u -
clear cells, to m igrate th rough th e avascular corn eato an d set tle w ith in th e sub-
epith eliu m adjacen t to Bow m an’s layer or basem en t m em bran e. Th is occurs in ~2%
of len s w earers regardless of w ear an d replacem en t sch edule. Th e et iology of SEI,
w h en associated w ith con t act len ses, is ch ron ic hypoxia, prolonged edem a, an im -
m u n e resp on se, solut ion toxem ia, m ech an ical irrit at ion –foreign debris, or a local
in fect ion or th e exotoxin s from bacteriu m residen t in th e ocular flora. Th ey are
th ough t to represen t a delayed hypersen sit ivit y im m u n e respon se to viral an t igen s
in th e corn eal st rom a.
Corn eal in filt rates are aggregates of gray or w h ite m igrat ing in flam m ator y cells
arising from n orm ally t ran sparen t corn eal t issue. Th e in flam m ator y respon se
st im ulates th e m igrat ion from th e lim bal vascu lat u re or from th e tears as a re-
spon se to t issue dam age or a secon dar y ch em ot act ic react ion associated w ith an
environ m en t al an t igen ic act ivit y or toxin s, con t act len s solut ion s, or from m icro-
bial organ ism s th em selves. In filt rates are defin ed as polym orph on uclear leu ko-
cytes (n eu t roph ils) but m ay also con t ain lym ph ocytes an d m acroph ages. In con -
tact len s w ear, in filt rates are m ost often sterile (n on in fect ious) but can also be
in fect iou s ( Fig. 16.4 ).
A B C
D E F
Fig. 16.4 (A) Subepithelial infiltrate with limbitis secondary to contact lens wear.
(B) Subepithelial infiltrate (SEI) secondary to extended-wear contact lenses pretreat-
ment. (C) Epithelial compromise with migration of SEI forward through epithelium.
(D) Subepithelial Infiltrate secondary to extended-wear contact lenses post treatment
with steroid. (E) Adenovirus with infiltrates. (F) Viral Infiltrates with secondary scarring.
Fig. 16.4 (Continued) (G) Viral infiltrates

with secondary scarring topography.
Presentation
Th ere is an in it ial an d p ron ou n ced lim bal–vascu lar resp on se in th e area of th e ocu -
lar in su lt . Su bsequen tly th ere is a release of m ediators from th e lim bal p lexu s. Th e
cellular or h u m oral com pon en ts w ill m igrate in to an d th rough th e corn eal t issu e
leading to th e accu m ulat ion of cells th at w ill appear as discrete w h ite-gray subepi-
th elial p ockets or opacit ies. SEIs are seen as h azy gray, circum scribed in filt rates, at
an in t ra- or su bepith eliu m (at th e surface of Bow m an’s layer w ith ou t in filt rat ion
in to th e st rom a; th erefore, n o scarring) or w ith an terior st rom al level w ith in fil-
t rat ion , th u s w ith a poten t ial for scarring. Th ey w ill be predom in an tly u n ilateral
an d con cen t rated focally or diffuse w ith a preferen ce to th e lim bal an d paracen t ral
areas of th e corn ea. Adjacen t to th e SEI m ay be an area of localized conjun ct ival
inject ion at th e lim bal ju n ct ure of m ild to m oderate severit y. If th ere is sign ifican t
corn eal vascu larizat ion of any form , th ere w ill be th e poten t ial for a h igh er in ci-
den ce of subep ith elial in filt rates. As SEI m igrates for w ard, th ere m ay be a subtle
epith elial com prom ise or break th at w ill st ain .
Th e p at ien t m ay h ave subtle sym ptom s ranging from m ild to m oderate. With in -
flam m at ion of any form th ere w ill be an associated hyperem ia localized to th e area
of occu rren ce, localized edem a, an d a variable degree of discom fort . Th e pat ien t
m ay also exp erien ce a m ild to m oderate level of lacrim at ion an d ph otoph obia an d
a decrease in visu al acuit y based on th e locat ion of th e in filt rates, irrit at ion , an d/or
foreign body sen sat ion .
If th ere is an oth er an terior ch am ber react ion , on e m u st differen t iate bet w een an
in filt rat ive kerat it is associated w ith several oth er an terior segm en t path ologies.
Th ese m ay also h ave a system ic relat ion sh ip th at n eeds to be looked for. Th ese
in clu de episclerit is, m argin al ulcer, irit is, aden ovirus or EKC, or keratoconjun ct i-
vit is. Addit ion ally, quiet n on in flam m ator y opacit ies m ay be in act u alit y a su btle
asym ptom at ic in filt rate or a sim ple scar. Histor y in th is case w ill assist in th e dif-
feren t ial. If th ere is sign ifican t corn eal vascu larizat ion of any form , th ere w ill be
th e poten t ial for a h igh er in ciden ce of subep ith elial in filt rates.
NaFl w ill be an im port an t differen t ial in dist inguish ing bet w een a scar, u lcer, or
SEI. Scars an d SEI w ill n ot stain , bu t u lcerat ion w ith an epith elial defect w ill. Th is
w ill allow a differen t ial bet w een SEI an d m icrobial epith elial defects. Upon t reat-
m en t , th e in filt rates m ay m igrate for w ard an d disru pt th e epith elial surface, cau s-
ing a top ograph ic irregu larit y an d p ossibly a n egat ive stain ing su perficial pun ct ate
keratopathy. To en su re n o epith elial com prom ise an d to ru le ou t an early stage of
m asquerader such as a h erpet ic lesion (den drit ic) Pseudom onas, Acantham oeba ,
or Fusarium , th e u se of rose bengal or lissam in e green w ill st ain early bu lbs of a
den drit ic lesion an d detail devitalizat ion of t issue m u ch m ore readily th an sodium
flu orescein .
If t h e SEI is associated w it h an ad en ovir u s, t h ere w ill be system ic an d con st i-
t u t ion al fin d in gs su ch as feve r, m alaise, let h argy, m yop at hy (m u scle w eakn ess),
p er iau r icu lar lym p h ad en op at hy, an d /or t h e p rese n ce of a follicu lar conju n ct i-
vit is. If t h e SEI is associated w it h a ke ratoconju n ct ivit is, t h e clin ician sh ou ld
con sid er t h e p ossibilit y of a t ran sm it t able d isease. An exam p le w ou ld be a ch la-
m yd ial in fect ion if t h e re is a seve re follicu lar conju n ct ivit is an d h istor y of u ro-
gen it al in fect ion . Th e p at ie n t sh ou ld be refer red to an in te r n ist , p ar t icu larly in
p ed iat r ic cases.
An terior segm en t fin dings associated w ith SEI m ay also be fou n d w ith an
episclerit is, w h ich m ay h ave a relat ion sh ip to a con n ect ive or collagen t issue dis-
order (rh eu m atoid). In flam m ator y con dit ion s, su ch as a rh eu m atologic disorder,
in flam m ator y bow el disease, or sacroidosis m ay h ave an associated irit is, w h ich
presen t s w ith an acute red eye, discom for t or pain , m iosis, decrease in IOP, an d
a decrease in acuit y. Even th ough corn eal su bepith elial in filt rates are con sidered
a rep resen t at ion of a low -grade im m u n e respon se to bacterial exotoxin s, su bepi-
th elial in filt rates can com plem en t oth er vasost im ulator y resp on ses as seen w ith
corn eal vascularizat ion , atopic or viral disease, as w ell as postsurgical causes such
as p ostLASIK.
Scars an d u lcers can easily m asqu erade as an in filt rate becau se of th eir sim ilar
appearan ce of h azy, gray opaqueing, n on t ran slucen t corn ea, an d locat ion at th e
su bepith elial an terior st rom al level. Pat ien t s w ith corn eal scars w ill h ave a posit ive
h istor y an d w ill n ot respon d to any th erapy. Also, m argin al u lcers can be m istaken
for in filt rates barring th e h istor y. Ulcers w ill h ave a m ore rapid on set an d n ot ice-
able inject ion an d decreased com fort . Ulcers w ill ten d to be located cen t rally. In -
filt rates m ay be diffuse an d cen t ral; h ow ever, m ore t ypically th ey are lim bal. SEIs
w ill appear less den se th an m argin al u lcerat ion s an d dem on st rate a lesser an terior
ch am ber an d conjun ct ival react ion . For a differen t ial of u lcers versu s in filt rate see
Table 16.4 .
Management
In th e m ost basic t reat m en t form at it w ou ld be appropriate to discon t in ue con -
tact len s u se u n t il resolu t ion . Len s w ear sh ould n ot be resu m ed u n t il all sign s an d
sym ptom s are com pletely resolved. Medicat ion is usu ally un n ecessar y in m ost
cases of in filt rat ive kerat it is (IK), w ith palliat ive use of preser vat ive-free ocu lar
lubrican ts. Th e use of a hyperosm ot ic agen t is prescribed, su ch as NaCl 5% pre-
scribed fou r t im es a day is m ore th an su fficien t if vision is n ot affected an d th ere
is a lim ited vascular resp on se. If th ere is a greater vascu lar respon se an d vision is
decreased a m ore aggressive approach w ith a steroid su ch as predn isolon e 1%four
t im es a day for 1 w eek w ith a slow taper to a soft steroid (lotepredn ol 1%) is h igh ly
recom m en ded. Prophylact ic use of an t ibiot ics to preven t secon dar y in fect ion or
an t ibiot ic/steroid com bin at ion drops to m it igate th e in flam m ator y respon se is
som et im es ben eficial. Such topicals w ould in clu de tobram ycin w ith dexam eth a-
son e or lotepredn ol (Tobradex, Alcon Laboratories) or Zylet (Bau sch & Lom b) fou r
t im es a day for 5 to 7 days an d slow t ap er. Cau t ion on ce again for steroid respon se
an d a corn eal toxic keratop athy to tobram ycin .
Because m any cases of recu rren t IK are secon dar y to exotoxin s released by lid
m argin bacteria ( Staphylococcus an d St reptococcus), it is w ise to recom m en d lid
hygien e in th ese cases an d to lim it len s w ear to daily w ear com plem en ted by a
Table 16.4 Di e rential o f Ulcers versus In ltrate
Ulcer Infiltrate
Epidemiology: relatively rare Epidemiology: relatively com mon; usually the

result of hypoxia
Represents active bacterial Represents m igration of in ammatory white
infection blood cells from the lim bal vasculature and
precorneal tear lm
Generally causes signi cant pain Pain is mild to moderate; rarely marked
Tends to be central rather than Tends to be peripheral because of proximit y
peripheral (Staphylococcus, to the cellular in ammatory mechanisms
exotoxin “peripheral ulcers” released from the limbal blood vessels
are toxic/in am matory
epithelial defects)
Usually a solitary lesion Can be multiple lesions
Size of the uorescein epithelial Size of the uorescein epithelial staining
staining defect closely mirrors defect is usually much sm aller than the
the underlying stromal lesion underlying stromal lesion; in any situation
where there is a strom al in ammation, it is
a real challenge for the overlying epithelial
cells to remain physiologically intact, which
explains why there can be some uorescein
staining even in these strom al in am matory
responses
There is alm ost invariably a Secondary anterior chamber reaction is rarely
cellular in am matory response elicited
in the anterior chamber
Pat tern of bulbar conjunctival The pat tern of bulbar conjunctival injection is
injection is usually generalized usually sectored and proximally associated
rather than sectoral with the in ltrate; even if there is 360-
degree injection, the vascular injection
pat tern is skewed toward the sector nearer
the in ltrate, particularly if it is peripherally
located
Possible tear lake debris Tear lake is clear
Treatment options: There are t wo therapeutic approaches:
Aggressive use of a If diagnosis is clear: Treat with antibiotic/
topical uoroquinolone steroid combination such as tobramycin
with uoroquinolone or with dexam ethasone, or tobramycin with
polysporin ointment at loteprednol, one drop every 2 h for 2 days,
bedtime and daily follow- and then modify and taper according to
up until good control is circumstances
achieved If diagnosis is unclear: Treat with a
Forti ed tobramycin or uoroquinolone every 1 to 2 h and follow
gentamicin (for gram- up in 24 hours; if it is an ulcer, there may be
negative) and forti ed no or minimal improvement in 24 h; if the
cephazolin or bacitracin defect is an in ltrate, it will be the same or
(for gram-positive); worse the following day; at day 1 follow-
therapeutic cycloplegia up, the conservative antibiotic therapy can
with 5% hom atropine be continued for another day, or if your
or 0.25% scopolamine is diagnostic decision is now in ltrate, then
usually wise add loteprednol four tim es a day while
continuing the antibiotic
peroxide care system . Lid t reat m en t w ould in clu de stan dard lid scru bs w ith n on ir-
rit at ive agen ts, doxycyclin e 20 m g, 50 m g by m outh , or up to a 100 m g for bleph a-
rit is or m in ocyclin e w ith lid clean sing (Cleeravu e–M, Ston ebridge Ph arm a, Du luth ,
GA) an d/or possibly cyclosporin e A drops—Restasis t w ice a day if th ere is a h istor y
of ch ron ic rosacea.
Th e m ajor con cern is w h eth er th e in filt rate is act ually a n on in fect iou s sterile
ulcer or con tact len s–related periph eral u lcer (CLPU). If su spicious of u lcers, p re-
scribe an t ibiot ics on ly. Refrain from steroid u se, cu lt ure w h en possible, an d t reat
w ith flu oroqu in olon e an t ibiot ics. In th is case, th e in it ial u se of a steroid w ou ld be
con t rain dicated un t il after a sh or t course of a poten t an t ibiot ic su ch as m oxifloxa-
cin (Vigam ox, Alcon Laboratories) or gat ifloxacin (Zym ar, Allergan ). In th e case of
in filt rates, th ere w ill be n o respon se to an t ibiot ics. If th ere is an u lcer, th ere w ill be
a favorable respon se to an t ibiot ics, w h ich can be follow ed by th e in t roduct ion of
steroids after th e loading dose h as redu ced th e bacterial burden . As an added com -
m en t for pain con t rol w ith corn eal u lcerat ion , am ple cycloplegia u sing h om at ro-
pin e 2 to 5%or a m ore frequ en t dose of cyclopen tolate 1%w ill in m any cases suffice
w ith ou t th e n eed for steroid ut ilizat ion . Oral an algesia for pain can be in t rodu ced
using basic acet am in oph en or ibuprofen or both as n eeded.
Th e progn osis of t reat ing in filt rates is h igh ly favorable w ith sym ptom s an d fin d-
ings dissipat ing in a sh ort cou rse of a few days. In filt rates th at are den ser an d m ore
cen t ralized, su ch as w ith an aden ovirus, w ill t ake longer to resolve an d m ay h ave a
profoun d effect on vision requ iring longer-term care an d slow t apering of m edica-
t ion s, part icu larly w h en u sing steroidal th erapy from h ard to soft steroid topicals.
Contact Lens-Related Acute Red Eye
Con tact len s-related red eye (CLARE) is an acu te, n on specific, n on ulcerat ive sterile
keratoconju n ct ivit is h as in flam m ator y associat ion w ith th e adh eren ce of debris
from exogen ou s m at ter, m et abolic by-p rodu cts, or vest iges of bacterial debris an d
exotoxin s th at in duce th e recruit m en t of in flam m ator y cells. Th e exotoxin s are
from th e breakdow n of t rapped debris or devitalized bacteria w ith in th e closed
eye environ m en t . Presu m pt ively, th e greater risk is bacterial in filt rat ion an d colo-
n izat ion by Staphylococcus an d Pseudom onas th at m ay lead to CLPU; th ere is sug-
gest ion th at som e pat ien ts h ave h igh er levels of gram -n egat ive con tam in at ion .
Con tact len s acute red eyes (CLAREs) h ave a variet y of cau ses. CLARE cou ld be
con sidered an in flam m ator y con dit ion associated w ith hypoxia, toxic effect s from
post-len s tear debris, m ech an ical irrit at ion from a poorly fit t ing len s, dehydrat ion
of th e len s du ring sleep, solut ion hypersen sit ivit y or toxicit y, or a react ion to bac-
terial toxin s. Du e to poten t ial hypoxic con dit ion s associated w ith len s u se, cellular
glu cose convert s to lact ate. In addit ion , lactate diffu sing in to th e st rom a in creases
th e osm olarit y, leading to m et abolic acidosis w ith resu ltan t corn eal edem a. A
decrease in n orm al corn eal m etabolism com p rom ises corn eal t issue leading to
CLARE. W h ite blood cells m igrate from th e lim bal vasculat ure an d form in filt rates
in th e periph eral corn ea.
Causes of Acute Red Eye

Th e con t act len s relat ion sh ip is via len s-in duced m ech an ical factors or from len s
deposit s th at lead to inju r y or m icrot rau m a to th e corn ea. Microt rau m a en cou r-
ages th e m igrat ion an d in filt rat ion of in flam m ator y cellu lar con st it u en t s. In th e
case of len s deposits, th ese ser ve as an an t igen et ic sou rce th at t riggers an im m u n e
respon se leading to in filt rates. Th e casu al relat ion sh ips are eith er (1) t igh t len s
syn drom e, (2) tear-film deficien cy/dr y eye [i.e., con t act len s–in du ced dr y-eye
(CLIDE)], (3) bacterial conju n ct ivit is, (4) in flam m ator y react ion to debris on th e
back su rface debris (m et abolic an d/or exogen ous debris stagn an t bet w een th e
len s an d corn eal su rface), (5) m ech an ical irrit at ion /abrasion , (6) solu t ion toxem ia/
hypersen sit ivit y, or (7) irrit at ion to len s deposits. Th e in ciden ce of corn eal fin dings
w ith 30-day con t in uou s-w ear silicon e hydrogels h as been foun d at an occurren ce
rate of 10% for CLPU an d 29% for CLARE ( Fig. 16.5 ).
Fig. 16.5 (A) Contact lens acute

red eye (CLARE) secondary to a small
foreign body. (B) CLARE secondary
to a small foreign body. (C) Sectoral
CLARE—tight lens syndrome—differen-
tial diagnosis episcleritis. (D) CLARE
D secondary to solution toxemia.
E F
Fig. 16.5 (Continued) (E) CLARE—tight lens syndrome. (F) CLARE—tight lens syn-
drom e—CLIDE.
Presentation
CLARE h as a gen eric appearan ce of a n on descript “red eye” directly associated w ith
th e u se of con t act len s exten ded w ear m ore often th an w ith daily w ear. Th e acu te
react ion could be obser ved as a n on specific red eye w ith lim bal hyperem ia, con -
jun ct ival inject ion , corn eal in filt rates, an d a possible corn eal edem a th at is lim bal
m ore so th an cen t ral. Upon len s rem oval, th e pat ien t m ay experien ce a greater
level of ocular discom for t an d m ay exh ibit pu n ctate keratopathy eviden ce w ith
posit ive fluorescein st ain ing associated m ostly w ith t rapped debris, a prim ar y
cau se for th e in duced in flam m ator y con dit ion .
Th e pat ien t w ill describe a “garden variet y” red eye w ith un ilateral, som etim es
bilateral, variable levels of discom fort or pain , redn ess, epiph oria, ph otoph obia, and
disch arge described as w ater y to m ucopurulen t. The am ount of vision reduction ,
pain , and discharge w ill assist in th e different ial diagn osis an d un derlying et iology.
If th e con dit ion is contact lens related, a histor y of exten ded or con tin uous len s
use w ill have a h igher in cidence th an daily w ear reusable m ore so th an single-use
len ses. If a contact len s patient presen ts w ith an ARE (acute red eye), it is im portan t
in the h istor y to determ in e th e w ear m odalit y of th e len s. Con tinuous an d exten ded
w ear schedules w ill dem on strate a h igher incidence of CLARE th an daily w ear or
single use lens w ear sch edules. It should be assum ed th at all con tact len s w earers
m ay h ave th e presen ce of m icrobial kerat itis an d ulcer, unt il proven oth er w ise. Th is
is im portan t in clin ical m an agem en t, for m any patients ten d to self-treat or h ave
been treated in appropriately for a “garden variet y conjunct ivitis” by a prim ar y care
physician (PCP). Because of th e potent ial of a potent ially devastating ulcer, such as
Acantham oeba , Pseudom onas, or Fusarium , it is im portant to stress to PCPs th at if a
“red eye–contact lens” patient presents to th eir office, they should defer treatm en t
and seek a con sult w ith an ophth alm ologist or optom etric physician.
As st ated, an acu te red eye presen t s as a garden variet y of red eye th at h as a dist in ct
ch aracterist ic of rapid on set w h en related to con t act len s, w ith th e h igh poten t ial
of being ulcerat ive, bu t can also m im ic or be directly related to m any oth er form s
of ocu lar disorders su ch as bacterial, viral, allergic, or ch lam ydial in fect ion s. If th e
pat ien t is n ot a con tact len s w earer, th is is n ot CLARE bu t is m ore probably a bacte-
rial conju n ct ivit is. Th e differen t ial of th e CLARE pat ien t , due to th e con t act len s
associat ion , is alw ays u lcer first un t il proven oth er w ise. On ce proven oth er w ise, by
cult u re or by an t ibiot ic t reat m en t , CLARE w ill rem ain as a red eye given the inflam -
m ator y nature. The inflam m at ion cou ld also be an u n derlying irit is in absen ce of
an an terior ch am ber react ion an d n orm al pu pils. If th ere is a sectoral com pon en t
to th e CLARE, th en con sider a con tact len s periph eral ulcer (CLPU), episclerit is,
su perior lim bic kerat it is (ru le out thyroid disease), vascu larized lim bic kerat it is,
or ocu lar su rface in flam m at ion associated w ith a pingueculae or pter ygiu m . Th ese
are an atom ically obviou s.
Management
In m any cases, th e pat ien t self-t reat s w ith over-th e-coun ter vasocon st rict ive lubri-
can t drop s w ith ou t relief. In m any oth er cases, th e pat ien t w ill presen t to a PCP for
a garden variet y conju n ct ivit is th at is first t reated w ith an t ibiot ics. Precau t ion ar y
care is requ ired. In m any in stan ces, a n on oph th alm ic–n on optom et ric provider m ay
h ave star ted t reat m en t , th ereby disgu ising a possible et iology of CLARE. As such ,
th e pat ien t m ay h ave already been t reated w ith a sulfacet am ide 10% op h th alm ic
preparat ion th at does n ot allow th e con dit ion to resolve an d in fact w orsen s th e
con dit ion if th e pat ien t h as su lfa drug sen sit ivit y. Or in oth er cases, th e pat ien t m ay
h ave been given a variet y of eith er am in oglycosides, flu oroqu in olon es, m acrolide,
or an t iallergy m edicat ion s, som e h aving an effect or n o effect at all.
In m any cases th e sim ple discon t in uan ce of th e con t act len s an d use of glasses
for a few days is sat isfactor y. If th e con dit ion resolves w ith th is m ode of t reat m en t ,
it suggests a sim ple m aterial an d w ear con dit ion issue th at n eeds to be addressed.
If th e pat ien t rein t roduces, or rech allenges th e u se of th e sam e m aterial an d w ear
sch edu le (i.e., exten ded-w ear or con t in u ou s-w ear m odalit y), an d th e con dit ion re-
m an ifest s, th e rech allenge defin es th e n eed to readdress len s u se by refit t ing th e
pat ien t w ith a n ew m aterial, w ear sch edu le, an d care p rodu ct .
Treat m en t som et im es determ in es th e differen t ial diagn osis in th e absen ce of
corn eal fin dings. As th e caveat w ou ld suggest , “do n o h arm ,” th erefore it is best to
t reat th e eye w ith an t ibiot ics, an d if n eeded for cycloplegia, for a m in im um of 24 to
48 h ou rs prior to th e in t rodu ct ion of a steroid to avoid a possible exacerbat ion of
an u n derlying ulcerat ive or h erpet ic: viral, fungal, or protozoan en t it y. Aggressive
an t ibiot ic th erapy sh ou ld be th e first course of th erapy w h en m aking th e assum p-
t ion of ulcer, an d a flu oroqu in olon e sh ould be in t roduced. Moxifloxacin (Vigam ox,
Alcon Laboratories) or gat ifloxacin (Zym ar, Allergan ) sh ould be th e first ch oice;
h ow ever, th ird-gen erat ion flu oroqu in olon es w ill su ffice. If th ere is som e resolu -
t ion w ith th e an t ibiot ic, th en th e CLARE w as n ot in flam m ator y but in fect iou s. If
th ere is m in im al to n o respon se to an t ibiot ics, th en a steroid, such as predn isolon e
1% fou r t im es a day, to rid th e in flam m ator y com pon en t of CLARE can be in t ro-
du ced safely, after th e loading dose of an t ibiot ic redu ces th e bacterial load.
After th e successfu l resolu t ion of CLARE, th e pat ien t sh ould be refit w ith a n on -
ion ic, h igh -w ater-con ten t , deposit-resistan ce len s or a n on ion ic silicon e hydrogel
len s m aterial w ith th e rest rict ion to daily w ear u se an d n o exten ded or con t in u -
ous w ear. Peroxide-based care produ ct s are recom m en ded w ith vigorous rubbing
to clean se debris an d con t am in an ts. Also con sider gas-perm eable len ses th at w ill
allow for n ot on ly an appropriate h igh oxygen perm eabilit y bu t also a flat ter or
hyperbolic periph eral cu r ve an d edge design th at facilit ates su fficien t tear pu m p
an d exch ange.
Contact Lens-Induced Dry Eye
Som et im es described as th e m in im al sicca syn drom e, con tact len s–in duced dr y
eye forces a borderlin e keratoconju n ct ivit is sicca pat ien t in to a full m an ifest at ion
of sym ptom s an d fin dings associated directly w ith a fu lly m an ifested dr y eye w ith
th e in t rodu ct ion of a con tact len s on to th e ocu lar su rface. Th e len s act s as an obst a-
cle an d com pet itor w ith th e n at ural tear film , leading to in sult th at w ill ju st ifiably
cau se a react ion by th e eye leading to th e ch ange in its n at u ral tear film physiology
an d m etabolism . Th is w ill lead to in toleran ce, in flam m at ion , an d a com prom ise of
th e ocu lar su rface.
Causes of Contact Lens-Induced Dry Eye

Th e n orm al tear-film environ m en t is at t acked by th e in t roduct ion of a con t act
len s. In it ially, th ere is a reflexive in crease in tear product ion . How ever, over t im e
tear produ ct ion w ill “fat igu e” th e system , decreasing th e effor t s of th e lacrim al
system an d in creasing th e poten t ial for con t act len s deposits, m icrobial in fect ion ,
an d corn eal in filt rat ion , corn eal edem a, an d u lt im ately p at ien t dissat isfact ion an d
in toleran ce to con tact len ses.
Th e in t roduct ion of th e con t act len s to th e ocular surface w ill disru pt th e h o-
m eostat ic balan ce of th e tear film , requiring a n ew balan ce to be establish ed be-
t w een th e pre-len s ocular tear film an d th e post-len s ocu lar tear film –precorn eal
tear film . As deposit s or su rface film accum u lates, blin king com presses th e tear
film an d rem oves th e lipid-con t am in ated, hydroph obic m u cu s an d debris from
th e len s–tear su rface. Th e in tegrit y of th e precorn eal an d len s tear film is directly
propor t ion al to th e abilit y to m ain t ain proper con tact len s w et tabilit y an d len s
su rface hydrat ion .
If th e lipid layer is poor, th e evaporat ive process in creases, leading to a greater
loss of aqueous an d th e in duct ion of a for w ard osm ot ic draw across th e con t act
len s surface leading to len s dehydrat ion an d corn eal desiccat ion . W ith len s de-
hydrat ion , th e hydroph ilic len s w ill steepen , m ech an ically pu lling on a w eaken ed
ep ith elial su rface, allow ing for corn eal com prom ise visualized as cen t ral corn eal
epith elial desiccat ion an d/or cell jun ct u re split t ing or separat ion .
Also, w h en th e ocular su rface becom es “u nprotected,“ th ere is th e developm en t
of n eu ron al hyposen sit ivit y associated w ith hypoxia an d th e barrier effect created
by th e con t act len s in terface. As th e con tact len s develops a su bst an t ial dehydra-
t ion it w ill ten d to vau lt aw ay from th e ocu lar su rface, leaving an exposed gap
bet w een th e post-len s surface an d th e corn eal su rface. Th e gap h ow ever is n ot
flu id filled an d leaves th e ocu lar surface u nprotected, leading to com prom ise an d
dessicat ion of th e epith eliu m an d aberrat ion to n eural regulat ion an d biofeedback
to th e lid st ru ct ure ( Fig. 16.6 ).
C D
Fig. 16.6 (A) Classic appearance of contact lens acute red eye (CLIDE) in patient exhib-
iting circumlimbal injection, marginal erythema, conjunctival injection, and immobile
lenses. (B) Minim al lacrimal lake as demonstrated by lissam ine green. (C) Disrupted tear
film spread with subsequent paracentral punctate keratopathy associated with CLIDE.
(D) Lissamine green staining of the conjunctiva in a CLIDE patient.
Presentation
Th e pat ien t w ill presen t w ith a CLARE-t ype ap pearan ce th at h ad been som ew h at
ch ron ic. Th e eyes w ill be described as feeling t ired, dr y, an d irritated an d alw ays
red, par t icularly later in th e day. It w ou ld also be n oted th at pat ien t s h ave difficu lt
len s rem oval an d describe a feeling of relief u pon len s rem oval. Often th e pat ien t
w ill proceed w ith vigorou s eye rubbing after len s rem oval. In som e cases of dif-
ficu lt len s rem oval, th e eye feels overly sen sit ive an d presen t s w ith an in crease in
inject ion du e to th e in adver ten t rem oval of su perficial epith elial t issu e an d n eu ro-
n al exp osure due to epith elial com prom ise during len s w ear associated w ith len s
dr yn ess an d bin ding. Su pplem en tat ion w ith topical drops su ch as lubrican t s m ay
or m ay n ot ben efit th e pat ien t , leading to self-lim itat ion of len s w ear or even dis-
con t in u an ce.
Th e clin ician w ill obser ve a CLARE-t ype red eye w ith ou t in fect ion th at appears
to be ch ron ic. Th ere m aybe a m ild to m oderate corn eal stain ing presen t due to len s
vau lt . Th e len s m ay appear som ew h at im m obile, suggest ing an in duced t igh t len s
syn drom e or sm all petech ial h em orrh ages on th e conju n ct iva ju xtaposed to th e
len s edge or on th e paralim bal conjun ct iva. Th ere is n eith er apparen t disch arge
n or follicu lar n or papillar y react ion . How ever, in th e long-term sufficien t debris
an d len s su rface dr yn ess can in duce a CLPC react ion . Tear film spreading abn or-
m alit ies w ill be seen w ith th e u se of various diagn ost ic test ing su ch as Sch irm er
st rips, lissam in e green , assessm en t of th e tear break-u p t im e, sodiu m flu orescein
stain ing of th e corn ea an d conjun ct iva, assessm en t of th e lacrim al lake–m argin al
tear volum e as a variet y of test .
Con t act len s–in duced edem a, w arpage, over w ear, oth er causes of redn ess an d dis-
com for t associated w ith con tact len s u se. CLIDE is directly related to oth er ocu lar
con dit ion s affect ing th e lacrim al–ocular su rface balan ce in cluding eyelid an d glan -
du lar dysfu n ct ion or disease, poor blin king m ech an ism su ch as lagoph th alm os,
floppy lid, an d/or dysfun ct ion of th e lacrim al an d m eibom ian glan ds. Oth er as-
sociat ion s to CLIDE an d dr y eye in clude Sjögren syn drom e, au toim m un e disease,
rh eu m atoid disorders, an d m edicat ion s, especially an t ih ist am in es, an t idepres-
san t s, an d oral con t racept ives.
Management
Th e t reat m en t for dr y eye an d CLIDE is to relieve th e un derlying problem by first
iden t ifying th e por t ion of th e tear film th at is dysfun ct ion al. On ce iden t ified, th e
t reat m en t sh ou ld be biased to com plem en t th e len s w ith m in im al com plexit y to
th e pat ien t . W ith th e u se of ocu lar lubrican ts as a su pplem en t or st im u lan t , th e
eye becom es “subject ively com for table,” bu t lit tle is kn ow n in regard to th eir long-
term effect on th e various tear film st ruct ures an d corn eal physiology. W ith re-
spect to su pplem en t in teract ion w ith th e m aterial an d th e m aterial’s in teract ion
w ith th e eye, th e con t act len s design an d m aterial are th e key long-term com for t
an d physiological balan ce in th e poten t ial su ccess of th e con t act len s pat ien t .
To ach ieve th e proper tear-film balan ce, th e CLIDE p at ien t m u st be t reated as a
n orm al dr y-eye pat ien t . Follow ing a flow ch ar t of t reat m en t such as proper tear
an d n ut rit ion al supplem en tat ion is th e first step. Th e select ion of supplem en ta-
t ion an d/or m edicin al t reat m en t is crit ical. I prefer to t r y to defin e m edicin al care
by th e determ in at ion of dr y eye as a “w h ite” or “red” dr y eye. If th e pat ien t pres-
en ts sym ptom at ically an d object ively as a dr y eye yet h as a w h ite, n on in flam ed
conju n ct iva, th e use of goblet cell–m ucin en h an cers in conju n ct ion w ith lacrim al
glan d st im u lus (e.g., cyclosporin e) w ould be con sidered appropriate. If th e pat ien t
appears w ith a red, in flam ed dr y eye, th en th e in ter ven t ion w ith steroids w ould be
deem ed m ore ap propriate. Th is m ay also be com plem en ted w ith th e use of n u t ri-
t ion al su pplem en t at ion of om ega 3 an d 6 essen t ial oils (fish an d seed oil sou rces),
w h ich h ave a n at ural n on steroidal an t iin flam m ator y effect . In addit ion , tear an d
len s rehydrat ion is w ell accom plish ed by using an t ioxidan t or elect rolyte-balan ced
tear su pplem en t s. At th e sam e t im e, a clin ical decision m ust be m ade to refrain
from con tact len s u se or lim it it during th e in it ial stages of th erapy. Pun ctal oc-
clusion sh ould be reser ved for long-term len s com for t m ain ten an ce un t il posit ive
result s are est ablish ed w ith topical an d oral th erapies.
Material select ion is crit ical in th e t reat m en t of th e CLIDE pat ien t . Alw ays con -
sider RGP len ses first for borderlin e dr y eye pat ien ts. A deficien t or u n st able tear
film requires h igh oxygen perm eabilit y an d a len s w ith low surface react ivit y th at
m oves adequ ately to m in im ize th e risk of com plicat ion s. Also con sider th e rech al-
lenge of hydrogel-based grou p 2 m aterials of h igh -w ater, n on ion ic ch aracter such
as h ioxifilcon an d ph osph at ylch olin e. As n oted, silicon e hydrogel m aterials m ay
be appropriate for oxygen en h an cem en t bu t are n ot prom ising w h en t reat ing a
defin ed CLIDE pat ien t . Silicon e hydrogels w ou ld be h igh ly desirable on ce th e CLIDE
p at ien t h as been t reated an d th e eye h as resu m ed a feasible level of com for t an d
p roper tear film balan ce.
Superior Epithelial Arcuate Lesions
Th e separat ion of th e corn eal epith elium , occurring m ore so at th e superior lim bal
m argin , is know n as superior epith elial accurate lim bal split (SEALS), or a sim ilar
form of epith elial split ting th at follow s th e corn eal-lim bal border aroun d the cornea
is know n as int ra-epithelial split ting (ILES) . It is ch aracterized by an obser vat ion of
an arcuate staining ju xtaposed to the lim bus. Th e pat tern of th e full-th ickness cor-
neal epithelial separation usually occurs in th e lim bal corn ea covered by th e upper
eyelid, w ithin 2 to 3 m m of th e superior lim bus in th e 10- and 2-o’clock region.
Th e m ain cause for th is en t it y is con sidered to in clude m ech an ical irrit at ion an d
dehydrat ion of th e len s su rface. Th e et iology of SEALS an d/or ILES is con sidered
to be eith er a m ech an ical pull or st ress on th e epith elial ju n ct ures du e to a t igh t
len s or len s dehydrat ion or a m ech an ical ch afing w ith in th e paralim bal corn ea.
As for SEALS, sup erior m ech an ical ch afing m ay be th e resu lt of inw ard pressure
of th e upper lid at th e su perior in t ralim bus associated w ith len s design , rigidit y,
an d surface ch aracterist ics. In com bin at ion th e excessive “frict ion al” p ressure an d
abrasive sh ear force on th e epith elial surface in du ce a separat ion of th e epith elial
cell jun ct u res. New m aterials, first-gen erat ion silicon e hydrogels, w h ich possess a
st iffer elast ic m odu lu s, m ay cau se an in crease in th e sh earing force n ot on ly on th e
corn ea, but also m ay in duce a sim ilar force on th e conju n ct iva leading to a pseu do-
im pression ring called a conju n ct ival flap.
Th e con cern s w ith SEALS or ILES are th e provocat ion of an in flam m ator y an d/
or bacterial in filt rat ive respon se. Addit ion ally, excessive sh earing forces m ay lead
to a degradat ion of lim bal stem cells require for corn eal repair m ech an ism s ( Fig.
16.7 ).
Presentation
Presen tat ion is gen erally asym ptom at ic oth er th an m ild, obser vable inject ion to
th e eye, par t icu larly upon len s rem oval. Th e pat ien t m ay also com plain of a m ild to
m oderate len s aw aren ess, difficu lt len s rem oval, burn ing, or itch ing sen sat ion . Th e
pat ien t m ay describe a sligh tly greater len s in toleran ce to stan dard low m odu lu s
2-hydroxyethyl m eth acr ylate-based len ses as com pared w ith h igh er m odu lus sili-
con e–hydrogel com bin ed polym ers.
Upon biom icroscopic exam , th e gen erally asym ptom at ic p at ien t m ay presen t
w ith conjun ct ival inject ion ju xt aposed to th e affected area. In th e case of SEALS,
th e conju n ct ival inject ion w ou ld appear m ore st rictly bet w een 10 o’clock an d 2
o’clock versu s a 360-degree-t ype epith elial split t ing, w h ich w ou ld app ear m ore as
th e CLARE-t ype eye associated w ith a t igh t len s syn drom e. Su bsequen tly, sign ifi-
can t paralim bal stain ing appears in an arc abou t th e in t ralim bal corn ea.
Fig. 16.7 (A) Classic circumlimbal

epithelial split ting with tear-film ir-
regularit y. (B) Classic circumlimbal
superior epithelial arcuate limbal
split ting (SEALS) due to a tight lens
syndrome with tear-film disruption
and conjunctival congestion [con-
tact lens acute red eye (CLARE)]. (C)
Classic SEALS due to a tight lens syn-
drome with tear film disruption and
conjunctival congestion (CLARE).
Th is is sim ply related to th e h istor y of len s use or th e lack th ereof. If th ere is n o h is-
tor y of len s u se, th en on e n eeds to evalu ate th e localized conjun ct ival respon se to
determ in e th e level of inject ion . A su perior lim bic kerat it is (SLK) m ay be an in dica-
tor of su bsequ en t thyroid disease. If th e conju n ct ival respon se is tem poral or n asal,
th en determ in e in flam m ator y con cern s of vascu larized lim bic kerat it is, episcleri-
t is, phylcten ulit is, pin qu ecu lit is, pter ygium , an d/or early m argin al degen erat ion s.
For t un ately, SEALS or ILES, are m ore con sisten t w ith con t act len s use, exten ded
w ear m ore so th an daily w ear. If su perior lim bal inject ion occu rs, part icu larly bi-
lateral, con sider oth er issues su ch as thyroid disorder or corn eal disorder.
Management
Sim p le len s discon t in u an ce is th e m ost appropriate, allow ing th e lesion to recover
w ith in a few days to several w eeks. If th e SEALS or split is m in or, th e sim ple dis-
con t in u an ce of th e len s is prop er based on th e pat ien t’s abilit y to fun ct ion w ith
spectacles. As a precau t ion again st bacterial in filt rat ion , a sh or t course of topical
an t ibiot ics m ay be ben eficial. In th is scen ario, a sim ple refit to a daily disposable
len s w ith adequ ate an d frequ en t rehydrat ion is deem ed appropriate. Th e refit of
len s sh ould n ot on ly reevaluate th e hydrat ion abilit y of th e len s but th e len s’ rela-
t ion to th e corn eal topography an d asp h ericit y or corn eal con tou r. Th erefore, th e
len s design sh ou ld com plem en t th e an atom y suggest ing a flat ter base cur ve or
h igh er eccen t ricit y value. In conjun ct ion , sh or t-term pu n ctal p lugs can be con sid-
ered an am ple adjuvan t th erapy to en h an ce epith elial h ealing an d m ain t ain len s
an d corn eal hydrat ion .
If th ere is a low -grade conjun ct ival respon se, a break w ith in th e epith elium
cou ld be th e p oten t ial opp or t u n it y for bacterial con t am in at ion an d/or in flam m a-
tor y respon ses. As such , th e level of th e con dit ion w ill w arran t th e level of th erapy.
Again , discon t in u an ce is ap propriate, yet th e addit ion of a soft steroid w ith m ild
an t ibiot ics su ch as tobram ycin /lotepredn ol (Zylet , Bausch & Lom b) fou r t im es a day
for 5 to 7 days w ith th e addit ion of a bacit racin oin t m en t during sleeping h ou rs is
con sidered prophylact ically safe.
If th ere is a greater (m ild–m oderate–severe) ocu lar inject ion an d sign ifican t
split t ing, com plete discon t in u an ce of th e len s is required w ith a m ore aggressive
th erapy ut ilizing a com bin at ion steroid-an t ibiot ic drop an d oin t m en t or a separate
flu oroqu in olon e drop an d oin t m en t w ith a secon dar y steroid su ch as p redn iso-
lon e acet ate if th e con dit ion h as associated in filt rates or m ay lead to an u lcerat ive
keratopathy.
On ce th e con dit ion is resolved, th e pat ien t can be refit to a low er elast ic m odulu s
len s, avoiding exten ded or con t in u ou s w ear or be refit to a h igh Dk gas perm eable
len s.
Dellen-Epithelial Hyperplasia
Dellen are focal areas of corn eal th in n ing t ypically located, usu ally at th e 3- to
9-o’clock lim bu s du e to dehydrat ion of th e region . Th ese form at ion s are th e ac-
t ive state of m ech an ical irritat ion of a sector or por t ion of th e corn ea ju xtaposed
to th e edge of th e gas perm eable con tact len s. Th ey are con sidered a t ran sien t
form of corn eal degen erat ion th at resu lts from st rom al dehydrat ion secon dar y to
poor w et t ing by th e tear film seen n ot on ly w ith con t act len s w ear but also p ost-
su rgically (e.g., cataract , LASIK, st rabism u s). It represen t s a localized th in n ing of
th e corn eal an d scleral t issu e. Epith elial hyperplasia is m ore related to th e ch ron ic
dehydrat ion an d m ech an ical ch afing of th e gas perm eable len s in a specific ju xt a-
posed area of th e corn ea.
Th e et iology of th e dellen is local dehydrat ion . It is th ough t th at breaks in th e oil
layer of th e tear film preven t th e proper w et t ing of th e t issue, leading to devital-
izing dehydrat ion of th e epith elial cells. Du e to th e lack of proper oil an d aqueou s
A B
Fig. 16.8 (A) Corneal dellen secondary to gas permeable lens wear. (B) Epithelial hy-
perplasia secondary to gas permeable lens wear.
flu ids in th e locat ion , th ere is a greater ten den cy for len s bin ding an d m ech an i-
cal ch afing. Mech an ical in flu en ces are in du ced by th e eyelids, w h ich are u n able
to follow th e ch anging con tou r of th e len s edge forcing it to lift or bore in to th e
epith eliu m . If th e len s edge creates a gap, th en th ere is a su bsequen t lack of w et-
t ing an d hydrat ion of th e corn eal t issu e in w h ich m u cin s are n ot spread over th e
epith eliu m , resu lt ing in an area of n onw et t ing resu lts. Fur th er dehydrat ion eith er
even t u ally causes corn eoscleral th in n ing to occur, w ith th e form at ion of a dellen
or long-term local epith elial hyperplasia ( Fig. 16.8 ).
Presentation
Pat ien ts w ith corn eal dellen m ay be asym ptom at ic but often report m ild ocu lar
discom fort or a foreign body sen sat ion . Th e pat ien t m ay describe a sectoral red-
n ess to th e eye w ith len s aw aren ess over t im e. Th ere m ay be in creased epiph oria
as w ell as n ot iceable len s decen t rat ion . Su bsequen tly, th e pat ien t m ay describe dr y
eye–t ype sym ptom s an d in creasing len s in toleran ce. Addit ion ally, th e pat ien t m ay
exh ibit an in com plete or errat ic blin k reflex w h en ut ilizing len ses.
Dellen appear as pale–kerat in ized w h ite “saucerlike” depression s w ith in , usu-
ally th e 3- to 9-o’clock area in t ralim bal ju xt aposed to th e edge of th e len ses. Rarely
are th ese lesion s larger th an 2 m m . If th e overlying epith elium is in t ake, th ey w ill
n ot st ain . Th ey m ay h ave a sligh tly vascularized su rface w ith adjacen t sectoral con -
jun ct ival inject ion . Flu orescein m ay appear to “gu t ter” an d surrou n d th e base of
th e dellen . Th e st ain ing, at or n ear th e 3- an d 9-o’clock posit ion s, appears on or
adjacen t to th e lim bu s of rigid len s w earers. Breaks in th e epith elium m ay allow for
periph eral bacterial con t am in at ion leading to a kerat it is or u lcerat ion or in flam -
m ator y in filt rat ion . Becau se of th e localized dehydrat ion , subsequ en t cell desicca-
t ion occu rs an d n ecrosis of th e epith elial cells. Th is leads to epith elial hyperplasia
sim ilar to corn eal scarring. Epith elial hyp erplasia is obser ved as a m ou n d of t issue
th at becom es eroded an d irritated by th e len s edge due to ch ron ic dehydrat ion of
t issue exposu re.
Several p oten t ial differen t ials n eed to be con sidered w h en obser ving a dellen or
epith elial hyperplasia. If th ere is sign ifican t sectoral conjun ct ival inject ion , th en
on e sh ou ld also con sider vascularized lim bic kerat it is, episclerit is, ph lycten u lit is,
pin qu ecu lit is, pter ygiu m , or early m argin al degen erat ion s su ch as Terrien’s. Th e
m ost im por tan t differen t ial w ould be m argin al ulcerat ion , part icu larly if epith e-
lium com prom ise is obser ved. If th e adjacen t conju n ct iva is quiet , th en on e m ay
con sider ru ling ou t a preexist ing scar or postoperat ive scar from cataract or ex-
t raocu lar m uscle surger y, filtering blebs, an d sclerit is, obviously differen t iated by
h istor y.
Management
Th erapy focuses on th e proper reepith elializat ion an d rehydrat ing th e corn ea.
Copious lubricat ion ever y 1 or 2 h ours an d blan d oin t m en t at n igh t are recom -
m en ded. In par t icu lar, n u t rien t-based th erapy is m ost appropriate ut ilizing an
elect rolyte-balan ced or an t ioxidan t-based drop. In addit ion , a dem ulcen t su ch as
Syst an e (Alcon Laboratories) or En du ra (Allergan ), w h ich form s a n et w ork gel-like
con sisten cy on th e ocu lar su rface an d acts as a tem porar y ban dage en h an cing epi-
th elial h ealing an d revitalizing th e m icrovilli an d surface glycocalyx. In it ial t reat-
m en t w ill dem on st rate efficacy in 48 to 72 h ours, bu t rein sult occurs easily. Topical
cyclosporin e can also be con sidered in sh ort- or long-term care.
Topical an t ibiot ics are u n n ecessar y, except in ext rem e cases involving sign ifi-
can t epith elial com prom ise to preven t in fect ion an d ulcerat ion . In th ese cases, th e
in flam m ator y com pon en t is raised, an d th erefore a com bin at ion tobram ycin w ith
dexam eth ason e or lotepredn ol m ay be appropriate in a sh or t cou rse of 7 days at
fou r t im es a day.
On ce th ere is proper resolu t ion , th e gas perm eable len ses can be refit w ith a dili-
gen t reevaluat ion of th e p eriph eral corn eal an atom y versu s th e periph eral cu r ve
an d edge profile of th e len s. Addit ion ally, blin king exercises en couraging th e pa-
t ien t to force a com plete lid closu re, on ce ever y 2 or 3 secon ds, are essen t ial. Also,
con t in u e proper lubricat ion w ith rew et t ing drops com plem en t ar y to th e m aterial
of ch oice. Con sider h igh er dK m aterials w ith plasm a t reat m en t for en h an ced w et-
tabilit y.
Ep ith elial hyp erplasia m ay resolve follow ing discon t in u at ion of len s w ear. W h en
poor resolut ion occurs an d is an obst ru ct ion to len s refit t ing an d PTK excim er laser
procedu re m ay be con sidered to flat ten th e area an d elevate th e devitalized cells.
Contact Lens-Induced Corneal Warpage Syndrome
Th e syn drom e of “corn eal w arpage” im plies th at th e con t act len s h as iat rogen ically
in duced an atom ical ch anges to th e corn eal surface. Th e w arpage is visu alized as
an irregular ret in oscopic reflex, distort ion to topography, an d keratom et ric m ires,
in duced irregu lar corn eal ast igm at ism , an d obser vable distor t ion s to th e corn eal
su rface u pon len s rem oval.
Con t act len s–in du ced corn eal w ar p age is m ore frequ en tly associated w ith
polym ethyl m eth acr ylate (PMMA), m ore so t h an gas-p er m eable len ses; h ow ever,
~27% of rep or ted cases of cor n eal w ar p age h ave been at t r ibu ted to hyd rogel len s
w ear. Corn eal con tou r–in du ced irregu lar it ies are con sid ered th e resu lt of p rob-
able m ech an ical deform at ion , ch ron ic m et abolic in su lt , or a com bin at ion of both .
Ad dit ion ally, w ar page is seen as secon dar y to len s bin ding or st agn at ion du e to
len s d ehydrat ion or a CLIDE syn d rom e. Also, som e solu t ion in teract ion s m ay ten d
to in du ce len s im m obilit y du e to th e level of solu t ion viscosit y leading to len s
bin d ing.
More com m on ly, h ard PMMA or rigid gas-perm eable len ses are recogn ized for
th e in duct ion of corn eal w arpage or iat rogen ically in duced resh aping of th e cor-
n ea w h en im properly fit or align ed to th e corn eal su rface. Th e corn eal epith eliu m ,
h aving a ver y “p last ic” or “m oldable” ch aracter, is easily resh aped by th e h igh
m odu lus or st iffn ess of th e len s, w h ich m olds th e corn ea to th e sh ape of th e len s
cur vat ure(s) un in ten t ion ally. Th is can be in ten t ion ally accom plish ed in a process
called orthokeratology.
Rigid con tact len s–in du ced corn eal w arpage is easily docum en ted an d m on i-
tored via obser vat ion of topograph ic abn orm alit ies. Th ere w ill be in du ced cen t ral
irregu lar ast igm at ism , in ferior steepen ing or sm ile-like pat tern (pseu dokeratoco-
n us-like im ages), su perior flat ten ing, loss of radial sym m et r y, h igh ly irregular pat-
tern s w ith loss of surface sym m et r y, or an im pression of a dem arcat ion con tou r
lin e con sisten t w ith th e len s edge. In du ced w arpage w ith hydrogels are con sidered
m ore t ran sien t; h ow ever, gas perm eable m ay be t ran sien t to p erm an en t .
Hydrogel-in duced deform at ion s are related to a w eaken ed epith elium du e to
lesser th an opt im al oxygen t ran sm issibilit y th rough various hydrogel len s m ateri-
als. Subsequ en tly, a m isalign ed, t igh t , u lt rath in len s or ext rem e varian ts in elast ic
m odu lus of len ses can in duce hydroph ilic corn eal w arp age syn drom e. In a posit ive
effect , a h igh elast ic m odu lu s len s w orn in reverse can act u ally in du ce a subtle flat-
ten ing–or th okeratological effect to th e corn ea of 0.50 to 1 diopter ( Fig. 16.9 ).
A B
C D
Fig. 16.9 (A) Corneal wrinkling (convolutions) induced by ultrathin hydrogel lenses.
(B) Hydrogel corneal warpage as seen with sodium fluroscein stainage. (C) Corneal im -
pression ring induced by orthokeratologically fit lens. (D) Irregular keratometric mires
by induced corneal warpage from a rigid gas permeable lens.
Presentation
Pat ien ts w ill com plain of poor or distor ted vision w ith len ses w ith su bsequen t
reduct ion in visual acu it y. Th ey w ill ten d to u t ilize m ore rew et t ing drops th at ten d
to assist in clearing th e vision m om en t arily, yet do n ot resolve th e visu al decre-
m en t . Th e u n in ten t ion al m olding or w arpage of th e corn ea leads to subject ive vi-
su al distort ion an d com plain t s of postlen s rem oval spectacle blu r or in abilit y to
w ear glasses an d object ive m easu res of regression of pow er if th e len s is fit flat an d
reduct ion of ast igm at ism can be m ade. In m any cases, par t icularly w ith hydrogel
len ses, su btle distor t ion s can n ot be obser ved by biom icroscopy bu t m ay be m ore
obviou s on topography an d aberrom et r y. In th ese cases, h istor y of p oor vision or
n on opt im al vision is th e on ly descriptor by th e pat ien t . Th is w ill often occur w h en
on e is refit t ing from older conven t ion al design s to frequ en t-replacem en t , th in n er
len s design s th at m ay lack th e proper align m en t to th e corn eal topography.
Fig. 16.9 (Continued) (E) Topography of warpage—RGP-induced corneal warpage at

present and at 1 week lens discontinuance.
Clin ical sign s of corn eal w arpage are first docum en ted by redu ced, su bject ive
an d object ive, visu al acu it y w ith con t act len ses an d subsequ en tly persisten t acu -
it y reduct ion w ith spectacles. Th e visual redu ct ion m ay be su btle (20/20 m in us)
or pron ou n ced. On ce th e len ses are rem oved for an adequate period, th e p at ien t’s
m an ifest refract ion ret u rn s to n orm al w ith con sisten t im provem en t in acuit y m ea-
sures. During th e exam in at ion , ret in oscopy reflexes w ill appear “w arped” or yield a
“scissor” reflex often seen in keratocon u s. Keratom et r y an d topograph ic m easures
dem on st rate varian t levels of irregu larit ies. Par t icu larly, descript ive st at ist ics w ith
topography w ill be h igh ly variable as com pared w ith prefit m easures, som et im es
m im icking a disease st ate.
Histor y of len s use an d subject ive com plain t s w ill defin e th e con cern . How ever,
a spect acle blur from len s-in du ced w arp age occurs eith er due to th e m ech an i-
cal distor t ion or from corn eal edem a w ith corn eal h aze. If th ere is a w eaken ed
en doth eliu m , part icularly w ith th e exten ded-w ear pat ien t or an older in dividu al,
corn eal edem a m ay occur secon dar y to im bibem en t of flu id th rough en doth elial
cell jun ct ion s an d m ay lead to associated cen t ral corn eal st riae, folds, or clouding.
Th erefore, en doth elial polym egath ism , polym orph ism , an d reduced en doth elial
cell cou n t m ay be th e u n derlying cau se.
Advan ced or u n con t rolled glaucom a w ith sign ifican t corn eal edem a can also
cau se corn eal w arpage or distor t ion s, part icu larly w ith a low er th an n orm al (540
µm ) cen t ral corn eal th ickn ess. On ce th e IOP is con t rolled, th e distort ion of th e cor-
n ea dissipates an d refract ive recover y occu rs rapidly.
High ly ast igm at ic, n on diseased, corn eal an atom y m ay also dem on st rate topo-
graph ic irregularit ies. Th e irregu lar corn eal ast igm at ism m ay h ave a decen tered
corn eal cap or a h igh asym m et r y of sup erior versus in ferior, tem poral versus n asal,
sect ion s of th e corn ea. In th ese cases, a con tact len s is ver y difficult to cen ter on
th e u n u su al lan dscape of th e corn eal topography an d w h en don e so, it w ill h ave
a h igh er propen sit y to decen ter tow ards th e steeper m eridian an d bin d, cau sing
in duced m olding to th e corn ea. Redu ced vision in th ese pat ien ts is n ot alw ays in -
du ced by th e con t act len s, bu t w ill h ave an en h an ced ap preciat ion for visu al aber-
rat ion s an d m ay also h ave a m eridion al am blyopia.
Scarring or oth er path ologies th at in duce m ech an ical distor t ion s to th e corn ea
can also m im ic corn eal w arpage. In part icular pter ygium , pulling across th e cor-
n eal su rface, h as a m ore global th an localized effect , an d pan n u s or aberran t con -
jun ct ival t issue m ay in du ce sim ilar m ech an ical distor t ion s.
Ect act ic corn eal degen erat ion s such as p ellu cid or keratocon u s m ay n ot h ave
been determ in ed at th e in it ial len s fit t ing of th e pat ien t m any years prior. Form e
fruste keratocon us (absen ce of classic an atom ical fin dings) is often m issed in th e
in it ial fit if topography an d aberrom et r y are n ot properly ut ilized. Du e to th e in -
creased in terest in refract ive surgical procedures, m any of th ese pat ien ts are being
discovered in preoperat ive care an d ju stly den ied th e procedure. Literat u re sug-
gest s th at PMMA an d gas-perm eable len ses at on e t im e in duced keratocon us. In
fact , th e len ses did n ot in du ce th e disorder, bu t in stead cau sed a “pseudokerato-
con ic” distort ion of th e corn ea, w h ich , in som e cases, is perm an en t . Pseu dokera-
tocon u s in du ced by con t act len ses w arping of th e corn ea produ ces a pat tern th at
m im ics keratocon us eviden ced in a localized area of in ferior corn eal steepen ing.
Upon len s discon t in uan ce th e in du ced steepen ing w ill regress to a n orm al “bow
t ie” or “h ourglass-sh aped” topography w ith sym m et r y, m u ch un like a t ru e kera-
tocon ic eye.
Management
Diagn osis by h istor y, refract ion , ret in oscopy, an d corn eal im aging is crit ical. Serial
m easu res after len s discon t in u an ce w ill determ in e th e severit y, t ran sien ce, or p er-
m an en cy an d th e fin al en d poin t prior to refit or su rgical procedu re. If con cern s of
corn eal edem a arise, serial pachym et r y is a requ ired m easu re.
If th ere is sign ifican t corn eal edem a, len s discon t in u an ce is recom m en ded w ith
th e in corporat ion of hyperosm ot ic agen t s w ith repeat pachym et r y t ill th e pat ien t
dem on st rates refract ive recover y an d stable cen t ral corn eal th ickn ess (CCT) m ea-
su res.
If th e corn eal distortion s are in duced by hydrogel soft len ses, th e sim ple discon-
tin uance for a few days and refit to a slightly thicker or interm ediate elast ic m odu-
lus, high-w ater, high -oxygen perm eabilit y, an d param eter-stable lens is appropri-
ate. Th ese w ould include a group 2 len s such as Proclear (CooperVision, Fairport,
NY), Extrem e Water (Hydrogel Vision Corp., Sarasota, FL) or a low er elastic m odulus
silicon e hydrogel. In these cases, the in duced distortion is tran sisent an d resolves in
a few h ours. Mon itor w ith topography an d aberrom et r y un til stabilit y of refract ion
and vision occurs prior to com pleting a refit ting of the contact lenses.
In t reat ing corn eal deform it y or w arpage in duced by gas-perm eable len ses, on e
cou ld take several approach es. Sim ply discon t in ue con t act len ses an d rever t to
eyeglass w ear for several days to w eeks u n t il th e stabilit y of th e corn ea can be
determ in ed. In th e m ajorit y of cases, th is is n ot an at t ract ive opt ion for th e pat ien t
becau se vision w ill dyn am ically ch ange an d th e pat ien t likely does n ot h ave a pair
of glasses to w ear du ring th is period. Or first obser ve th e len s on eye, th en rem ove
an d perform a dr y refract ion , p achym et r y, an d topograph ic or aberrom et r y m ea-
su res. Th ese m easu res w ill be repeated several t im es in th e “reh abilit at ion pro-
cess” u n t il st abilit y of m easu res occu rs. Defer cycloplegia u n t il resolu t ion after sev-
eral serial m easu res. Du ring th e “recover y” or “corn eal reh abilitat ion ” period, refit
th e pat ien t w ith hydrogel soft len ses to th eir best ach ievable acu it y. On aftercare,
repeat diagn ost ic m easu res an d refit w ith soft len ses according to th e resu lt s. Con -
t in u e th is process u n t il corn eal an d refract ive stabilit y is ach ieved. A crit ical poin t
in pat ien t care is to w arn th e pat ien t of th e dyn am ic ch ange to vision an d th e frus-
t rat ion th at w ill probably pursu e. Pat ien t reassuran ce is vital, en couraging th em to
im m ediately ret u rn for con t in ued care if th e vision becom es u n acceptable.
St abilizat ion of th e corn ea is defin ed as th e poin t th at th e m an ifest refract ion
is con sisten t t w o to th ree t im es w ith in a –0.50 diopters sph ere an d cylin der, w ith
axis w ith in 10 to 15 degrees of origin al m an ifest , proper sym m et r y to th e topogra-
phy an d p achym et r y w ith in n orm al ranges of prefit est im ates or m easu res. Not ing
th at , based on th e exten t an d severit y of corn eal w arpage, 20/20 (6/6) m ay n o
longer be ach ievable by th e pat ien t . On ce st able, th e p at ien t can be refit to any len s
th at is deem ed app ropriate, be it soft hydrogel or gas perm eable or orth okeratol-
ogy or a refract ive surgical procedure.
If con sidering or th okeratology or refract ive su rger y, even cataract procedu res on
pat ien ts w h o h ave w orn gas-perm eable len ses for any exten t of t im e, a “corn eal
reh abilit at ion ” sh ou ld be in corp orated in to th e prefit or preoperat ive care. Th ere
is n o single r ule for p reoperat ive care. Som e m igh t suggest th at corn eal stabilit y
is ach ieved bet w een 8 an d 20 w eeks, or even u p to 6 m on th s. In m y experien ce it
is suggested th at approxim ately 1 to 2 w eeks’ discon t in u an ce of gas perm eable
len ses for ever y year of w ear be obser ved prior to a refract ive su rgical procedure,
m ore t im e if th e pat ien t h ad w orn PMMA len ses on ly.
Contact Lens Deposits
Deposit s are th e accu m ulat ion of debris on th e surface. Th e variou s t ypes of de-
posit accu m u late based on th e m aterial ch em ist r y ch aracterist ics su ch as w ater
con ten t , ion ic versu s n on ion ic surface t reat m en ts, or physiological in teract ion s of
th e con st it u en t s of th e tear film or th e abilit y of th e lids to spread th e tear film
an d clean se th e con t act len s surface. Deposit s are m u lt ifactorial w ith a variet y of
t ypes ( Fig. 16.10 ).
A B C
D E
F G
Fig. 16.10 (A) Mucin balls and surface film silicone hydrogel lenses. (B) Mucin balls
and surface film silicone hydrogel lenses. (C) Severe lipid–protein –m ucous surface film
on soft hydrogel lenses. (D) Severe lipid protein calcium deposits—lens calculi. (E) Se-
vere protein film rigid gas permeable (RGP). (F) Severe nonwet ting secondary to surface
film RGP. (G) Filming of a prosthetic eye.
Presentation
Th e un derlying problem is th e im proper clearan ce of m et abolic an d/or exogen ou s
debris th at accum u lates on or pen et rates w ith in th e len s m aterial. Th is accum ula-
t ion of debris an d w aste is due to poor tear ch em ist r y, in appropriate or in com p lete
clean ing by th e len s care product , or a poor lid in teract ion to spread th e tear film
an d clean se th e len s su rface. Th is leads to deleteriou s in teract ion s w ith th e pal-
pebral conjun ct iva of th e su perior eyelid th at leads to a m ech an ical an d an t igen ic
resp on se causing CLPC.
Protein s in th e tear film are com p osed of organ ic am in o acids of variou s elec-
t rolytes. Oxidat ion by h eat ing or ult raviolet exposu re en courages th e den at uring
(ch em ical t ran sform at ion ) or alterat ion of th e protein s, th e m ost predom in an t
being lysozym e, w h ich h as a st rong an t im icrobial ch aracterist ic. W h en th e pro-
tein s ch ange th eir ch aracter, th ey n ow becom e foreign to th e system , in it iat ing
an autoim m u n e hypersen sit ivit y respon se. An t ibodies are st im u lated leading to
su bsequen t in flam m at ion , inject ion or er yth em a, an d pruisit is. As th e deposit s ac-
cum u late, th ey becom e “space occupying” n odu les on th e back surface th at lead to
localized im pression s on th e corn eal epith elial su rface, seen as discrete pu n ct ate
stain ing. Also, th e den at ured protein s can lead to a cytotoxic respon se an d a gen er-
alized su perficial pu n ctate kerat it is. Th e su rface protein s can also act as n u t rit ion
to n orm al flora, w h ereas m ucu s can en capsulate an d n ur t ure th e colony. As such ,
th e flora rep licate in to a larger colony, w h ich is beyon d th e n orm al balan ce an d
defen ses of th e ocu lar surface. If th ere is a break in th e ep ith eliu m , a sequela of
in filt rat ion w ith resu lt an t m icrobial kerat it is or ulcerat ion m ay occu r.
Th e pat ien t , in m any cases, is asym ptom at ic oth er th an describing variable dr y-
n ess, itch , redn ess, an d visual con cern s w ith th e len ses. In th e m ajorit y of cases,
w h en frequ en t replacem en t len s m odalit ies are properly u sed, th e deposits do n ot
becom e sign ifican t en ough to cause a n ot iceable p roblem . Rarely, in few pat ien ts,
len s deposits form qu ickly du e to th eir poor tear ch em ist r y. In frequ en t-replace-
m en t len ses, len ses used less th an 30 days, deposit s ten d to create a “film ” th at
clean s off easily w ith th e prop er care product , yet reaccum u lates m ore rapidly
each day of len s w ear u n t il it is discarded.
In pat ien ts w h ere deposits becom e excessive, th e len s w ill in teract aggressively
again st th e lid, leading to in flam m at ion such as CLPC. Th e pat ien t w ill on ce again
be som ew h at asym ptom at ic oth er th an describing a len s th at frequ en tly decen ters
or even dislodges. Th ere w ill also be a “conju n ct ivit is t ype” disch arge an d redn ess
w ith ou t discom for t . In th ese cases, ver y often , th e PCP is con sulted an d w ill pre-
scribe an an t ibiot ic in appropriately (see CLPC an d ARE).
In oth er cases, th e p at ien t w ill presen t w ith an acute red eye, w ith discom fort to
pain , copiou s w ater y or pur ulen t disch arge, w ith possible effects on visu al abilit y.
Th is w ou ld im ply a secon dar y bacterial con tam in at ion du e to a con t act len s–as-
sociated n ut rien t- an d p rotein -rich biofilm . Th e biofilm allow s bacteria, even n or-
m al flora, to “feast” an d in crease colon izat ion beyon d levels in w h ich th e eye can
defen d it self. Also, w ith th e lack of tear lysozym e, th e bacterium h as a great abilit y
to popu late an d invade. In th ese cases of u su al abuse to th e con tact len s an d lack
of proper care, th e corn ea is also com prom ised, allow ing for a vast oppor t un it y for
bacterial in filt rat ion an d su bsequen t u lcerat ion of th e corn ea. Th e m ost com m on
are Staphylococcus epiderm idis an d aureus, follow ed by Pseudom onas aeruginosa .
As m icroorgan ism s spread, th ey w ill eith er im m ediately at tack th e corn ea or
serpigin ou sly spread over th e len s su rface. Th ese are gray, black, brow n , or w h ite
grow th s in th e len s m at rix. Fu ngi or yeast w ill appear in a filam en t ar y pat tern ,
obser ved as h aving a cen t ral den sit y an d t ran slucen t fron ds prot r uding from th e
core. Most com m on ly, surface m icroorgan ism s are associated w ith con tam in at ion
from variou s n on sterile w ater sources, su ch as pools, recreat ion al w aters (pon ds,
rivers, lakes), con tam in ated tap w ater, n onp reser ved salin e, or, as m ore recen tly
seen , in effect ive con t act len s care produ cts. Cat ast roph ic in fect ion s related to th e
acan th am eoba (a protozoa) an d fusarium (a fu ngal in fect ion ) h ave been cited in an
outbreak of cases w orldw ide in 2005 th rough 2006.
Protein Deposits
Th e m ost com m on t ype of deposit is protein based origin at ing from tear com -
pon en ts of album in , globu lin , an d lysozym e. Th e deposit is ch aracterized by it s
opaque, th ick, den se, w h ite film w ith st riat ion s, fou n d on hydrogel len ses, p re-
dom in an tly on th e an terior su rface versus gas perm eables th at accum ulate th ese
deposit s on both surface an d par t icu larly in th e perip h eral cur ves. Protein u ptake
occurs w ith in m om en t s upon len s in ser t ion an d varies to th e t im e of m at rix sat u-
rat ion . Sat u rat ion of th e m at rix is h igh ly depen den t on th e polym er ch aracterist ic.
Ion ic len ses w ill ten d to absorb an d bin d a great am ou n t of protein , n on ion ic as
w ell as plasm a or surface-t reated len ses ten d to retard th e absorpt ion .
Lipid Deposits
Lipids are a com pon en t of th e tear film arising from th e m eibom ian glan ds to pre-
ven t tear evaporat ion . W h en lipids accum u late on th e len s surface th ey h ave a
sm eared, greasy, w h it ish appearan ce. In dividu als w ith dietar y or m etabolic defi-
cien cy in potassiu m , h ave dr y eye, use diu ret ics or alcoh ol, h ave poor tear osm olar-
it y, or h ave h igh er-fat diet ten d to be m ore pron e to th ese form s of deposits.
Lens Ca lculi or Jelly Bumps
Com bin at ion deposits of lipid an d protein s accu m ulate as m ore discrete, rou n d
bum ps an d localized “droplets” th at bore in to th e len s m at rix. As a com bin ed de-
posit , variou s m in erals such as calcium from th e tear film m ay also be par t of th e
com posit ion . Th ese t ypes of deposits are m ost com m on in ion ic, h igh -w ater-con -
ten t len ses u sed in a con t in uous or exten ded-w ear m odalit y.
Mucin Ba lls
Most syn onym ou s w ith silicon e hydrogel len ses, th ese are recogn ized as sm all,
roun d, discrete par t icles or plugs seen bet w een th e con tact len s an d corn eal su r-
face. Th ey are com posed of a com bin at ion of m u cin , tear p rotein s, an d lipids. Th e
appearan ce w ill var y in size from 10 to 20 µm in diam eter an d is t ypically t ran s-
paren t , sim ilar to th e appearan ce of m icrocyst s. Th ey are differen t iated from m i-
crocysts by th eir im pregn at ion of th e len s surface, versu s m icrocyst s, w h ich are
in t raepith elial. Mu cin balls m ay accu m ulate an d clu m p ; th u s th ey m ay n ot m ove
as th e len s m oves, appearing t rapp ed again st th e corn eal su rface. Upon len s re-
m oval or su bsequ en t blin king, th ey w ill dislodge, leaving an in den tat ion in th e
corn eal su rface th at also appears sim ilar to discrete pu n ctate st ain ing associated
w ith back surface debris.
Exogenous Deposits
Th ese t ypes of deposits can be from foreign bodies su ch as ru st , cosm et ics, pain t ,
lot ion s an d cream s, an d oth er form s of airborn e debris. Th ese deposit s w ill be dis-
crete an d obvious upon obser vat ion . Rust or m et allic foreign bodies appear rou n d,
sm all, an d brow n -red th at h ave becom e em bedded in th e len s su rface. Cosm et ics
ten d to spread across th e len s surface in a vor tex m an n er, w h ereas pain t (an d som e
oil-based cosm et ics) ten d to variably “spot” th e surface. In gen eral, exogen ou s de-
bris is u su ally airborn e or physically im paled on to th e len s surface an d w ill quickly
bin d w ith ou t pat ien t sym ptom s.
Medica tions
Medicat ion s can also create deposits or len s discolorat ion . Th is is due to th e w ater
solubilit y of hydroph ilic soft len ses th at allow for th e ease of absorpt ion by th e by-
produ cts th at circu late in to th e tear film . An t ibiot ics su ch as ph en azopyridin e or
n it rofu ran toin (u rin ar y in fect ion ) w ill discolor len ses to a yellow or pin k. Ph enyl-
eph rin e, propin e (prodrug), an d ep in eph rin e w ill appear as brow n -black deposit s
on th e len s su rface (aden och rom e st ain ing), w h ich can also be foun d on th e con -
jun ct iva an d lid m argin . Tet racyclin e, used for derm atological con cern s or upp er
resp irator y in fect ion , m ay be obser ved as grayish brow n dots. Rifam p in u sed in th e
t reat m en t of t ubercu losis–m en ingococcal in fect ion s cau ses orange-pin k tear ex-
cret ion th at absorbs in to th e len s. Iodin e an d sulfa-based drugs such as sulfasala-
zin e (t reat m en t for u lcerat ive colit is an d Croh n disease) as w ell as stool soften ers
su ch as ph en olp h th alein w ill yield a yellow discolorat ion of th e len s. Th ese are eas-
ily defin ed by m edical h istor y. Any supp ression of th e lacrim al or exocrin e system
w ill allow for in creased len s deposit ing.
Management
Th e t reat m en t is sim ple bu t th e h ardest th ing for m any pat ien ts to com plete. It is
called proper len s care an d hygien e. Th e sim plificat ion of care system s is a dou ble-
edged sw ord. On th e posit ive side, it is easier, less cum bersom e, an d m ore user
frien dly w ith good effect iven ess in m icrobial ch allenge m ult i-item test ing. On th e
n egat ive, sim p licit y is follow ed by com placen cy an d w orsen ed by th e com m odit i-
zat ion of con t acts th rough th ird-par t y ven dors. Th e gen eral pu blic does n ot look at
con tacts as a m edical device th at requires proper care un t il som eth ing goes w rong.
Th e best t reat m en t for deposits is good educat ion an d in st ru ct ion s on th e proper
use of th e recom m en ded an d p rescribed len s care product . In recen t st u dies, per-
oxide, act ing as a solven t clean ser an d h igh efficaciou s an t im icrobial solut ion , h as
been sh ow n to be th e m ost efficien t for clean sing an d m icrobial reduct ion . Mult i-
purpose solu t ion s, w h ich at on e t im e recom m en ded n o rub, h ave sin ce gon e back
to “ru b.” Ru bbing can redu ce th e bacterial load by 90% an d pu rges th e len s su rface
of th e protein –lipid accum ulat ion .
17 Ophthalmic Instruments and Diagnostic Tests
Sam uel Boyd and Am ar Agarw al
Cover Testing
Cover/ Uncover Tests
Cover Test
Th e cover test is don e to con firm th e presen ce of a m an ifest squ in t . Th e pat ien t is
asked to look at th e fixat ion target (a flash ligh t sh ou ld n ever be u sed as a fixat ion
target because it fails to con t rol accom m odat ion —an accom m odat ive fixat ion t ar-
get h eld at 33 cm is used for n ear an d th e Sn ellen 6/9 visu al acu it y sym bol is u sed
for dist an ce fixat ion ). Th e apparen tly fixat ing eye is th en covered an d th e beh av-
ior of th e un covered eye is n oted. Each eye is tested in t u rn for n ear (33 cm ) an d
distan ce (6 m ). It is perform ed in all n in e posit ion s of gaze. Head post u re sh ould
be st raigh t . Th e test is perform ed w ith an d w ith out eyeglasses. In th e presen ce of
squ in t , th e un covered eye m oves to take up fixat ion . If th ere is n o m ovem en t of th e
un covered eye, th at eye is th en covered an d th e oth er eye obser ved. No m ovem en t
of eith er eye is seen in p seu dosqu in t .
Uncover Test
Cover th e apparen tly fixing eye an d obser ve th e oth er eye (apparen tly deviat ing
eye). If m ovem en t is seen , th en th ere is h eterotopia.
Cover/ Uncover Test

Th is test tells abou t th e presen ce an d t ype of h eteroph oria. Th e pat ien t looks at
th e fixat ion target an d on e eye is covered w ith an occlu der. Th e occluder is th en
rem oved, an d th e eye u n der cover is obser ved. Th e fin dings var y depen ding on th e
diagn osis:
In a person w ith n orm al vision , covering eith er eye w ill n ot produ ce any m ove-
m en t of th e oth er eye. On rem oving th e occluder, th ere is n o m ovem en t of th e
un covered eye, w h ich con t in u es to look st raigh t ah ead.
In h eteroph oria, th e eye u n der cover w ill deviate in th e direct ion of th e h etero-
ph oric posit ion . On u n covering, it w ill m ove in th e opposite direct ion to rees-
tablish bin ocular fixat ion . Th e opposite eye con t in u es to m ain tain fixat ion an d
m akes n o m ovem en t . Th us on ly on e eye m oves in case of a h eteroph oria.
In h eterot rop ia, on covering th e fixat ing eye, th e op posite eye, p rovid ed it is
able to do so, w ill m ake a m ovem en t from th e h eterot rop ic p osit ion to t ake
u p fixat ion , an d th e covered eye w ill m ake a corresp on ding m ovem en t in ac-
cordan ce w ith t h e Hering law. On u n cover ing th e for m erly fixat ing eye, it w ill
eith er m ove again to t ake u p fixat ion or m ay con t in u e to rem ain d eviated de-
486
17 Ophthalm ic Instrum ents and Diagnostic Test s 487
pen d ing on w h et h er it is a u n ilateral or an altern ate h eterot rop ia. On e can also
m ake ou t th e fixat ion p at tern , th at is, w h eth er th ere is st rong fixat ion p refer-
en ce for on e eye, free alter n at ion (form erly d eviated eye con t in u es to m ain t ain
fixat ion in defin itely), w eak altern at ion (form erly deviated eye m ain t ain s fixa-
t ion for som e t im e, su ch as u n t il a blin k), or eccen t r ic fixat ion (on covering th e
fixat ing eye, th e deviated eye m akes n o m ovem en t or an in com plete m ove-
m en t) is p resen t .
Prism Bar Cover Test

Th is is an object ive m eth od of m easu ring th e deviat ion s. Apply th e follow ing rule:
th e apex of th e prism sh ou ld poin t tow ard th e deviat ion :
Esodeviat ions: Place th e p rism base out .
Exodeviat ions: Place th e prism base in .
Right hypert ropia : Place th e prism base dow n in fron t of th e righ t eye.
Right hypot ropia : Place th e prism base up in fron t of th e righ t eye.
Com binat ion of vert ical and horizontal deviat ion : Place h orizon tal prism s in
fron t of on e eye an d ver t ical prism s in fron t of th e oth er eye.
Alternate Cover Test

In th is test , th e pat ien t looks at th e fixat ion t arget w ith both eyes open , an d th e oc-
cluder is altern ately m oved bet w een th e t w o eyes to produ ce m axim al dissociat ion
of th e t w o eyes. Th is preven t s fu sion bet w een th e t w o eyes an d decom pen sates
any laten t squin t . Th e pat ien t sh ould n ot be allow ed to regain fu sion w h ile th e
cover is being t ran sferred. It can be u sed to diagn ose a laten t squ in t of even 2 de-
grees an d sm all degrees of h eterot ropia. It also differen t iates con com it an t squ in t
from paralyt ic squ in t .
Rod Tests
Maddox Rod Test

Th e pat ien t is asked to fix on a poin t ligh t in th e cen ter of th e Maddox t angen t scale
at a dist an ce of 6 m . A red Maddox rod (w h ich con sists of m any glass rods of red
color set togeth er in a m et allic disk) is placed in fron t of on e eye w ith th e axis of
th e rod at a righ t angle to th e axis of deviat ion . Th e Maddox rod convert s th e poin t
ligh t im age in to a lin e. Th us th e pat ien t w ill see a poin t ligh t w ith on e eye an d a red
lin e w ith th e oth er. Due to dissim ilar im ages of th e t w o eyes, fusion is broken an d
h eteroph oria becom es m an ifest . Th e n um ber on th e Maddox t angen t scale w h ere
th e red lin e falls w ill be th e am oun t of h eteroph oria in degrees (Fig. 17.1 ).
Fig. 17.1 Maddox rod test.
Double Maddox Rod Test

Th is test h elps in detect ing an d m easu ring cyclodeviat ion s. Place a red Maddox rod
ver t ically in fron t of th e pat ien t’s righ t eye an d a w h ite Maddox rod also ver t ically
in fron t of th e oth er eye in a t rial fram e. Th e axes of th e Maddox rod(s) are rotated
un t il th e t w o lin es seen by th e pat ien t are parallel. Th e degrees of cyclodeviat ion
an d direct ion are m easu red from th e t rial fram e w ith excyclodeviat ion h aving out-
w ard rot at ion an d in cyclodeviat ion s h aving inw ard rot at ion s.
Maddox Wing Test

Th e Maddox w ing is an in st rum en t by w h ich th e am oun t of h eteroph oria for n ear
(at a dist an ce of 33 cm ) can be m easu red. It is based on th e p rin ciple of dissocia-
t ion of fusion by dissim ilar objects. It h as t w o slit h oles in th e eyepiece. Th e fields
th at are exposed to each eye are separated by a diaph ragm in su ch a w ay th at th ey
glide tangen t ially in to each oth er. Th e righ t eye sees a w h ite arrow poin t ing ver t i-
cally upw ard an d a red arrow poin t ing h orizon t ally to th e left . Th e left eye sees a
h orizon tal row of figures in w h ite an d a vert ical row in red. Th ese are calibrated in
diopters of deviat ion . Th e arrow p oin t ing to th e h orizon tal row of figures an d th e
arrow poin t ing to th e vert ical row are both at zero in th e absen ce of a squin t or in
th e presen ce of squin t w ith a h arm on iou s abn orm al ret in al correspon den ce.
Clin ically im por t an t poin ts are as follow s:
Th e Maddox w ing sh ould be h eld poin t ing 15 degrees in feriorly, as for reading.
It is im p or tan t to do th e test w ith an d w ith out correct ion for refract ive errors.
Th e abilit y of th e pat ien t to give an an sw er on th e Maddox w ing does n ot m ean
th e pat ien t h as n orm al bin ocu lar vision becau se th e pat ien t can h ave abn orm al
ret in al correspon den ce (ARC) or rapid alterat ion .
Stereopsis and Fusional Testing
Stereopsis
Stereopsis is th e visual appreciat ion of th ree dim en sion s during bin ocular vision .
It is a fu n ct ion of spat ial disparit y an d arises w h en h orizon tally disparate ret in al
elem en t s are st im u lated sim ultan eou sly. Th e fu sion of th ese disparate ret in al im -
ages w ill result in a single visu al im pression perceived in depth , provided th e fu sed
im age lies w ith in th e Pan u m area of bin ocular single vision .
Tests for Stereopsis

Titmus Test
Th e t it m us test con sists of a th ree-dim en sion al Polaroid vectograph —t w o plates in
th e form of a booklet , w h ich h as to be view ed th rough Polaroid spect acles. On th e
righ t th ere is a large fly an d on th e left a series of circles an d an im als. Th e w orking
distan ce is 16 in ch es (40 cm ).
Fly Test
Th e fly test is for gross stereop sis (degree of disparit y is 3000 secon ds of arc). Th e
fly sh ou ld appear solid an d th e su bject sh ould be able to p ick up on e of th e w ings
of th e fly. On invert ing th e book, th e t argets w ill appear to recede. If th e fly appears
as a flat ph otograph , th e subject is n ot appreciat ing stereoscopic vision .
Circles Test
Th e circles test m easu res fin e stereopsis (degree of disparit y is 800 to 40 secon ds
of arc). Th ere are n in e squ ares, each of w h ich con t ain s four circles. On e of th e cir-
cles in each squ are w ill appear for w ard of th e plan e of referen ce in th e presen ce of
n orm al stereopsis. Th e subject th at perceives th e circle to be sh ifted off to th e side
is n ot appreciat ing stereoscopic vision but is using m on ocu lar clues in stead.
TNO Test (degree of dispa rit y is 480 to 15 seconds of a rc)
Th e TNO test con sist s of seven plates to be view ed w ith red-green spectacles. Each
plate h as variou s sh apes created by ran dom dot s in com plem en t ar y colors. Som e
sh apes are visible w ith out glasses, w h ereas oth ers can be appreciated in th e pres-
en ce of stereopsis on ly. It h as n o m on ocular clu es.
La ng Test (degree of dispa rit y is 1200 to 600 seconds of a rc)
Th e t arget s are seen altern ately by each eye th rough th e built-in cylin drical len s
system ; h en ce th ere is n o n eed for special spect acles.
Frisby Test (degree of disparit y is 600 to 15 seconds of a rc)
Th ere are th ree t ran sparen t p lates of var ying th ickn ess. On th e su rface of each
plate th ere are prin ted four squ ares of sm all ran dom sh apes. On e of th e squ ares
con tain s a h idden circle in w h ich th e ran dom sh apes are prin ted on th e reverse of
th e plate. Th e su bject m ust iden t ify th is h idden circle.
Base -Out Prism

Th is is a quick an d sim ple m eth od using a 20 diopter base-ou t prism to detect
bin ocular single vision (BSV).
Synoptophore
All t ypes of h eteroph orias an d h eterot rop ias can be m easured accu rately w ith a
syn optop h ore (both object ive an d subject ive angle of squ in t). In th e syn optoph ore
th e rays of ligh t from th e target h it a m irror an d th en p ass th rough a convex len s
of + 6.5 diopters to reach th e eye. Th us th e im age is seen beh in d th e m irror, for ex-
am ple, at a dist an ce of 6 m , w h ich w ill be equal to th e focal length of th e len s. Th u s
th e syn optoph ore im ages are seen at a dist an ce an d n ot n ear. Th is is becau se w e do
n ot w an t th e pat ien t to use h is or h er accom m odat ion . Th e tech n ique for u sing th e
syn optop h ore is as follow s ( Fig. 17.2 ):
In terpupillar y distan ce (IPD) is ch ecked an d adjusted.
Angle kappa is m easu red.
Sim ult an eou s m acular percept ion is tested for.
We u se th e sim u ltan eou s param acular percept ion slides. Both th e object ive an d
su bject ive angles of squin t are ch ecked in all n in e cardin al p osit ion s of gaze (on e
is th e prim ar y posit ion an d th e oth er eigh t are 15 degrees from th e prim ar y posi-
t ion ).
To test th e object ive angle, on e arm of th e syn optoph ore is fixed at zero degrees.
Th e oth er arm is m oved un t il th ere is n o m ovem en t of th e eyes w h en th e tester
altern ately sw itch es on an d off th e ligh t s of th e t w o arm s. Th e poin t w h ere th e
eyes do n ot m ove is th e object ive angle.
To test th e su bject ive angle, on e arm of th e syn optoph ore is fixed at zero de-
grees. Th e pat ien t is sh ow n slides of a lion an d it s cage. Th e slide of th e cage is kept
in th e arm th at is fixed. Th e p at ien t is asked to m ove th e oth er arm (con t ain ing th e
slide of th e lion ) so as to put th e lion in th e cage. Th e angle at w h ich th is is don e is
th e su bject ive angle of squin t .
Fig. 17.2 Synaptophore

To detect abn orm al ret in al correspon den ce (ARC) w ith th e syn optop h ore, th e
object ive angle (OA) an d th e su bject ive angle (SA) of squ in t are first determ in ed,
w hich gives th e angle of an om aly (AOA).
AOA = OA – SA
In th e case of n orm al ret in al correspon den ce (NRC), th e SA is equ al to th e OA,
an d th e AOA w ill be zero. In un h arm on iou s ARC, th e su bject ive angle w ill be less
th an th e object ive angle, an d th e differen ce bet w een th e t w o sh ou ld be at least 5
degrees or m ore. In th e case of h arm on ious ARC, th e su bject ive angle w ill be zero.
Th us th e angle of an om aly in a h arm on iou s ARC w ill be equ al to th e object ive
angle.
Fusion : For test ing fu sion , t w o slides are used: on e is th at of a rabbit w ith out a
tail an d th e oth er is th at of a rabbit w ith ou t ears. Th e t w o slides are kept in each
arm of th e syn optoph ore an d th e arm s are fixed at th e angle of squ in t . If th e
pat ien t sees both th e ears an d th e tail, th en fu sion is presen t . If th e pat ien t sees
eith er th e tail or th e ears, fusion is absen t .
Stereopsis: Stereop sis can be tested w ith slides con tain ing parat roopers w ith a
plan e in th e backgrou n d. Th e pat ien t sh ou ld be able to tell w h eth er th e para-
t roopers are in fron t of th e plan e or n ot , w h ich in dicates good stereopsis.
After im ages: After im ages can also be don e.
Visual-Field Testing
Th e visu al field is th e por t ion of sp ace th at is visible to th e fixat ion eye. Visual-field
exam in at ion is th e exam in at ion of th e fun ct ion of th e visu al system in th e field
an d n ot on ly th e determ in at ion of th e lim its of th e field. Th e differen ce th resh old
is th e sm allest m easu rable differen ce in lum in an ce bet w een a st im ulu s an d th e
backgroun d ( Fig. 17.3 ).
Automated Static Perimetry

Th e differen t test s in autoperim et r y are as follow s:
Suprathreshold test : Th is test is u sed as a screen ing device for severe or m oder-
ate defect s.
Threshold related st rategy : Th e act ual th resh old is determ in ed at a sm all n u m -
ber of p oin t s an d th ey are u sed to ext rapolate th e h ill of vision .
Threshold test ing: Th resh old test ing is th e stan dard of care for glau com a m an -
agem en t . Many p oin t s are tested an d th ere are differen t st rategies used to ac-
curately defin e th e visu al field. Th ese tests can be long an d th e pat ien t s can
becom e fat igu ed.
SITA: Becau se fu ll th resh old can be t im e con su m ing, sh or ter algorith m s h ave
been developed. Th e Sw edish In teract ive Th resh olding Algorith m (SITA) uses
m ath em at ical m odeling an d presen t un derstan ding of th e visu al field to in -
crease accu racy w h ile speeding up th e test .
Short-w avelength autom ated perim etry (SW AP) : Th is select ively tests th e sh or t-
w avelength path w ay by presen t ing a blue t arget on a yellow backgroun d. SWAP
m ay be able to sh ow defect s 1 to 3 years before st an dard tech n iques.
Fig. 17.3 Humphrey autoperimetric 30–2 test.
Th e various field defects seen in glaucom a are gen eralized depression , baring of
th e blin d spot , isolated p aracen t ral scotom a, Seidel scotom a, Bjerru m scotom a or
arcuate scotom a, dou ble arcuate scotom a, Ron n e n asal step (w h ich respects th e
h orizon tal m idlin e), tem poral w edge defect , p eriph eral breakth rough , alt it udin al
defect , cen t ral an d tem poral islan ds, an d split fixat ion .
Automated Corneal Topography
Im aging tech n iqu es of th e corn ea are developing rapidly, m ain ly because of con t in -
uou s advan ces in refract ive an d cat aract su rger y. It is crucial to u n derst an d th e sig-
n ifican ce of n ew im aging tech n iques an d th e relevan t prin ciples of corn eal opt ics.
Th e discu ssion of th e m ost com m on clin ical m eth od of Placido-based corn eal to-
pography em ph asizes im por tan t con cept s of it s clin ical in terpret at ion (Fig. 17.4 ).
Fig. 17.4 (A) Astigmatic corneal topography. (B) Orbscan topography of an eye with
keratoconus.
Optical Properties of the Cornea

Several con cepts are u sed to ch aracterize opt ical proper t ies of th e corn ea, such as
cur vat ure, sh ape, local su rface, p ow er, expressed as refract ion in diopters, th ick-
n ess, an d th ree-dim en sion al st ru ct u re. Th e keratom et ric value is a con cept in h er-
ited from keratom et r y an d is calculated sim ply from radii of cu r vat u re as follow s:
K = refract ive in dex of 337.5/radiu s of cur vat u re
Th e in tact cen t ral corn eal th ickn ess of ~560 µm is con sidered en ough to en su re
long-term m ech an ical stabilit y of th e corn ea. Th e periph eral th ickn ess ( ~600 µm )
is cer t ain ly clin ically im por tan t in som e refract ive procedures su ch as in t racorn eal
rings, ast igm at ic keratotom y, an d cat aract surger y. W ith th e advan ces in corn eal
im aging an d w idesp read refract ive su rgeries, corn eal beh avior w ill likely be bet ter
un derstood. Corn eal topography in st ru m en ts u sed in clin ical pract ice m ost often
are based on Placido reflect ive im age an alysis. Th is m eth od of im aging of th e an -
terior corn eal surface uses th e an alysis of reflected im ages of m u lt iple con cen t ric
rings projected on th e corn ea.
Interpretation of Topographic Maps

Ever y m ap h as a color scale th at assign s par t icu lar color to a cer tain keratom et ric
diopt ric range. Never base an in terpret at ion on color alon e. Th e value in kerato-
m et ric diopter is crucial in th e clin ical in terpret at ion of th e m ap an d h as to be
looked at w ith th e in terpret at ion of ever y m ap. Absolute m aps h ave a preset color
scale w ith th e sam e diopt ric step s an d diopt ric m in im um an d m axim u m assign ed
to th e sam e colors for par t icu lar in st ru m en t .
Norm alized m aps h ave differen t color scales assign ed to each m ap based on in -
st ru m en t soft w are th at iden t ifies th e act u al m in im al an d m axim al keratom et ric
diopt ric valu e of a par t icu lar corn ea. Th e diopt ric range assign ed to each color is
gen erally sm aller com pared w ith th e absolu te m ap, an d, con sequ en tly, m aps sh ow
m ore det ailed descript ion of th e su rface. Th e disadvan tage is th at th e colors of t w o
differen t m aps can n ot be com pared directly an d h ave to be in terpreted based on
th e keratom et ric valu es of th eir differen t color scales.
Specular Microscopy
Th is is an im port an t par t of th e preoperat ive evaluat ion , especially if corn eal gut-
tata or oth er sign s of a low en doth elial cell coun t are fou n d. It is clin ically an d
m edical-legally im por t an t to docu m en t a low en doth elial cell coun t before LASIK,
rath er th an w orr y later w h eth er LASIK caused it . It m u st be don e before any ex-
am in at ion th at m igh t rough en or dr y th e corn eal su rface. Alth ough en doth elial
cell ch anges follow ing lam ellar surger y (in cluding LASIK) h ave n ot been reported
clin ically, th ey h ave been seen experim en t ally w h en excim er ablat ion of greater
th an 90% depth w as ach ieved. Th is depth is of cou rse en t irely con t rain dicated in
pat ien t s, bu t t w o cases of corn eal perforat ion du ring LASIK h ave recen tly been
reported, an d on e w ou ld assu m e th at oth er cases w ith deep ablat ion m ust also
exist ( Fig. 17.5 ).
Fig. 17.5 A normal specular microscopy.
Corneal Pachymetry
St u dy of th e cen t ral corn ea w ith ult rasoun d pachym et r y is fu n dam en tal. Th e sur-
face of th e u lt rason ic pachym eter p robe is w iped w ith alcoh ol an d does n ot n eed to
be sterilized. Th is in st ru m en t is used to take a ver y carefu l an d accurate reading of
th e th ickn ess of th e cen t ral corn ea. Th e pachym eter probe is placed on th e cen ter
of th e corn ea, perpen dicu larly, to determ in e corn eal th ickn ess. Th e pachym eter
h as a con sole displaying th e corn eal th ickn ess reading ( Fig. 17.6 ).
Clinical Importance
Ideally, w e m ust preser ve a m in im um of 250 µm in th e posterior st rom al bed.
Oth er invest igators con sider th at preser vat ion of a m in im u m of 50% of th e preop-
erat ive cen t ral pachym et r y is essen t ial. Th is is referred to as th e Barraquer law of
corneal thickness. Th is is par t icu larly im por t an t in th e t reat m en t of h igh refract ive
errors w ith th e excim er laser an d in “en h an cem en t” procedures. Un less sufficien t
corn eal st rom a rem ain s, as determ in ed by Barraqu er’s basic prin ciple, th ere is an
in creased risk of developing corn eal ect asia. It is gen erally est im ated th at 10 µm of
ablat ion corrects on e diopter of m yopia.
Fig. 17.6 Illustration of a corneal

pachymetry in a patient undergoing
LASIK. P, ultrasound pachymeter;
A, flap depth in case of LASIK; B,
amount of strom a ablated for cor-
rection of refractive error; C, resid-
ual bed thickness. (From Boyd, BF. Pre -
operative evaluation and considerations.
In: Atlas of Refractive Surgery. Highlights
of Ophthalm ology, 2000, 45. Courtesy
Jaypee Highlights Medical Publishers Inc.,
Panama.)
A-Scan Ultrasonography
Ocu lar biom et r y m u st be perform ed prior to cat aract su rger y. Biom et r y is th e dis-
cip lin e in ch arge of th e physical param eters of th e eyeball an d in clu des t w o fu n -
dam en tal explorat ion s, keratom et r y an d th e a xial length m easurem en t of th e eye.
Usu ally, in clin ical pract ice, th e term biom et ry refers to th e lat ter an d con siders
keratom et r y to be a separate procedure.
Th ere is n o qu est ion th at w h en w ell selected an d properly don e th e m odern
m eth ods of part ial coh eren ce in terferom et r y (opt ical coh eren ce biom et r y), th e
pen t acam (Ocu lu s, In c., Lyn nw ood, WA) an d th e advan ce con t act an d im m ersion
ult rason ography afford u s th e best w ay of ach ieving th e desired postoperat ive re-
fract ion . Determ in at ion of in t raocular len s pow er th rough m ean ingful keratom et-
ric readings an d a xial length m easu rem en ts h as becom e th e stan dard of care. Th is
is a ch allenging tech n iqu e to obt ain good visual resu lts an d pat ien t sat isfact ion
( Fig. 17.7 ).
Fig. 17.7 Determination of intraocular lens power in patients with normal axial length
(normal eyes)—mechanism of how ultrasonography measures distances and determines
axial length. The use of ultrasonography to calculate the intraocular lens power takes
into account the variants that may occur in the axial diameter of the eye and the curva-
ture of the cornea. The ultrasound probe (P) has a piezoelectric crystal that electrically
emits and receives high-frequency sound waves. The sound waves travel through the
eye until they are reflected back by any structure that stands perpendicularly in their
way (represented by arrows). These arrows show how the sound waves travel through
the ocular globe and return to contact the probe tip. Knowing the speed of the sound
waves and based on the time it takes for the sound waves to travel back to the probe
(arrows), the distance can be calculated. The speed of the ultrasound waves (arrows) is
higher through a dense lens (C) than through a clear one. Soft-tipped transductors (P)
are recomm ended to avoid errors when touching the corneal surface (S). The ultraso-
nography equipment computer can automatically multiply the time by the velocit y of
sound to obtain the axial length. Calculations of intraocular lens power are based on pro-
gram s such as SRK-II, SRK-T, Holladay, or Binkhorst, am ong others, installed in the com-
puter. (From Boyd, BF. IOLpower calculation in normal cases. In: The art and science of cataract surgery.
Highlights of Ophthalmology, 2001, 41. Courtesy Jaypee Highlights Medical Publishers Inc., Panam a.)
Fig. 17.8 Mode B-scan showing

retinochoroidal colobom a.
B-Scan Ultrasound
Lin ear A scan s are sum m ated in th e B-scan . Th is gives a t w o-dim en sion al cross-
sect ion al im age of th e eye an d orbit . It is of great valu e in evaluat ing th e posterior
segm en t in eyes w ith opaque m edia (Fig. 17.8 ).
Potential Acuity Meter Testing
Th e Poten t ial Acu it y Meter (PAM; Marco Oph th alm ic In c., Jacksonville, FL) is an
in st r um en t th at at tach es to a slit lam p. It ser ves as a vir t ual pin h ole by project ing a
regu lar Sn ellen visual acuit y ch ar t th rough a ver y t iny aerial p in h ole aper t u re ~0.1
m m in diam eter. Th e ligh t carr ying th e im age of th e visu al acu it y ch art n arrow s to
a fin e 0.1-m m beam an d is directed th rough clearer areas in cataracts (or corn eal
disease), allow ing th e pat ien t to read th e visual acu it y ch ar t as if th e cat aract or
corn eal disease w ere n ot th ere. Th e PAM is taken from it s st an d an d placed directly
on to th e slit lam p in th e sam e m an n er as th e detach able t ype of Goldm an n ton om -
eter. Th e exam in at ion takes from 2 to 5 m in utes per eye, depen ding on th e den sit y
of th e cat aract ( Fig. 17.9 ).
As poin ted ou t by Guyton , for th e PAM to w ork adequ ately, th ere m ust be som e
sm all h ole in th e cat aract for th e ligh t beam to pass th rough . You m ay fin d su ch a
h ole even in cat aract s th at h ave m edia clou ding of up to 20/200 an d bet ter. W h en
you fin d it , th en you can avoid th e ligh t scat tering produ ced by th e op acit ies. It is
th is ligh t scat tering th at w ash es ou t th e ret in al im age an d decreases vision beh in d
cataracts. By project ing th e im age of th e visual acuit y ch art th rough on e t iny area,
w e avoid th e scat tering effect , an d th e pat ien t can see th e ch art .
Fig. 17.9 Potential Acuit y Meter

Guyton Test. C, cornea; R, retina.
(From Boyd, BF. Preoperative evaluations.
In: The art and science of cataract surgery.
Highlights of Ophthalmology, 2001, 16.
Courtesy Jaypee Highlights Medical Pub-
lishers Inc., Panama.)
Color Vision Tests (Ishihara Plates,

Depth Perception Tests)
People w ith n orm al con es an d ligh t sen sit ive pigm en t (t rich rom asy) are able to
see all th e differen t colors an d su btle m ixt ures of th em by u sing con es sen sit ive
to on e of th ree w avelength s of ligh t—red, green , an d blu e. A m ild color deficien cy
is presen t w h en on e or m ore of th e th ree con es’ ligh t sen sit ive pigm en t s are n ot
qu ite righ t an d th eir peak sen sit ivit y is sh ifted (an om alou s t rich rom asy—in clu des
protan om aly an d deu teran om aly). A m ore severe color deficien cy is presen t w h en
on e or m ore of th e con es’ ligh t sen sit ive p igm en ts is really w rong (dich rom asy—in -
cludes prot an opia an d deuteran opia).
Th e Ish ih ara test , for exam ple, is com posed of a series of colored cards on w h ich
n um bers or lin es of equal sh ade can be read by a person w ith n orm al color vision
but n ot by som eon e w ith defect ive color vision . It m akes use of th e peculiarit y th at
in red-green blin dn ess, blu e an d yellow appear rem arkably brigh t com pared w ith
red an d green . Th e color p lates are en cased in a specially design ed album -t ype
book for ease of h an dling. Th e set in clu des fou r special plates for test s to deter-
m in e th e kin d an d degree of defect in color blin dn ess. Th is color blin dn ess test is
accepted by leading au th orit ies ( Fig. 17.10 ).
Fig. 17.10 Ishihara plate.

Fluorescein Angiography
Flu orescein angiography can be a ver y u sefu l procedure for assessing ret in al dis-
ease by delin eat ing areas of involvem en t , gu iding t reat m en t , an d form u lat ing a
progn osis for ch anges in th e pat ien t’s vision .
To in terpret fluorescein angiograph ic im ages, kn ow ledge of ret in al/ch oroidal
an atom y an d circulat ion is essen t ial. Arterial an d ven ou s circu lat ion differen ces,
as w ell as th e ret in a’s barriers again st th e passage of sodium fluorescein (NaFl)
dye, in cluding th e ret in al pigm en t epith eliu m (RPE) (ou ter blood–ret in al barrier)
an d th e ret in al vascu lar en doth elium (in n er blood–ret in al barrier), m ust be un -
derstood. Kn ow ledge of fu n dus path ophysiology an d an atom y h as been greatly
en h an ced by research using flu orescein angiograp hy ( Fig. 17.11 ).
For purposes of angiogram in terpretat ion , th e sen sor y ret in a can be divided in to
vascu lar an d avascu lar por t ion s. Th e vascu lar port ion is com posed of th e in tern al
lim it ing m em bran e (ILM), n er ve fiber layer (NFL), ganglion cell layer (GC), in n er
plexiform layer (IPL), an d in n er n uclear layer (INL). Th ese por t ion s of th e ret in a
receive direct m etabolic support from ret in al blood vessels.
Fig. 17.11 (A) Fundus photo of neovascularization.

(B) Fluorescein angiography of neovascularization.
The sensory retina’s avascular portion consists of the outer plexiform layer (OPL),
the outer nuclear layer (ONL), and rod and cone photoreceptor cells. These structures
receive m etabolic support from the choroidal vessels via the pigm ent epithelial cells.
Optical Coherence Tomography
Optical coherence tom ography (OCT) is an im aging m odalit y that perform s high-reso-
lution, m icrom eter-scale cross sections of the eye and other biological structures.
At th is t im e th e tech n ology is an alogou s to B-scan ult rasoun d. Its ut ilit y to th e
clin ician lies in th e abilit y to accurately m ake n on invasive an atom ical m easure-
m en ts in vivo ( Fig. 17.12 ). Prior to th e developm en t of OCT, th e m ain stays of clin ical
exam in at ion for m any ret in al diseases w ere slit-lam p biom icroscopy, fun du s ph o-
tography, fluorescein angiography, an d in docyan in e green angiography. Alth ough
th ese diagn ost ic tech n iques rem ain essen t ial to diagn osis an d m an agem en t , OCT
h as em erged as an invalu able diagn ost ic tool as w ell, providing quan t itat ive an d
qualit at ive in form at ion th at w as previou sly un at t ain able.
In addit ion to presen t ing a clearer pict ure of th e path ophysiology of disease,
OCT h as been ext rem ely u seful in determ in ing respon se to th erapy. Mult ip le st u d-
ies, for exam ple, h ave u sed OCT to exam in e ocu lar respon se follow ing m acu lar
t reat m en ts determ in ing th at th ere is an in it ial in crease in su bret in al an d in t raret i-
n al flu id, follow ed by a gradual declin e over th e n ext days.
Lasers In Ophthalmology
Laser Peripheral Iridectomy

Becau se of th e coagu lat ing effect of argon laser ligh t , iridectom y perform ed by
argon laser offers advan t ages over in cision al iridectom y or n eodym ium :yt t rium -
alu m in u m -garn et (Nd:YAG) laser iridectom y in pat ien t s predisposed to bleeding
con dit ion s, such as th ose taking an t icoagulan t s or w ith kn ow n blood-clot t ing dis-
orders. Th e laser iridectom y is perform ed as an office p rocedu re in a closed eye—a
con siderable advan t age over surgical iridectom y (Fig. 17.13 ).
Fig. 17.12 Optical coherence tomography of a macular hole.

Fig. 17.13 Iridectomy with argon

laser—opening a narrow angle in
chronic, narrow angle glaucoma. A
laser iridectomy is the procedure of
choice for narrow angle glaucoma ex-
cept in cases such as (A) where the pe -
ripheral iris lies too close to the cornea
for treatment. Laser applications (D)
are placed in the midstroma area of
the iris to open the angle. These non-
perforative laser applications cause
heat, which in turn causes shrinkage
of the iris collagen fibres in the direc-
tion of the arrow. The iris sphincter
muscle (S) and the laser beam (L)
are shown in (B), shrinkage from la-
ser applications (D) has opened the
angle to an acceptable position (C).
A peripheral laser iridectomy is then
executed. The normal iris location is
shown on dot ted line (N). The angle
is now sufficiently open for laser tra-
beculoplast y if indicated. Laser beam
(L) is shown producing burns (E) in
the now visible trabeculum. (From
Boyd, BF, Lunt z, M. Acute and chronic angle
closure. In: Innovations in the glaucomas.
Highlights of Ophthalmology, 2002, 277.
Courtesy Jaypee Highlights Medical Publish-
ers Inc., Panama.)
It is an effective w ay of producing an open ing in th e iris but should n ot be used

w hile the eye is congested or in flam ed. Clear m edia are essent ial. Th e eye is prepared
w ith topical an esth esia. The surgeon sh ould have com fortable arm supports.
Th e Abrah am tech n ique is h igh ly u seful an d effect ive. Th ese bu rn s im m ediately
cause iris con t ract ion an d put th e iris cr ypt on st retch . Oth er surgeon s fin d th at th e
st retch burn is gen erally u n n ecessar y if th e Abrah am con tact len s is used.
In th e m ajorit y of cases, an iridectom y is ach ieved at th e first session . As p en -
et rat ion of th e iris st rom a reach es th e pigm en ted epith elium of th e iris, bu rst s of
p igm en t appear in th e an terior ch am ber (“sm oke sign als”). Pow er is th en reduced,
an d fu r th er burn s are applied u n t il a m ush room clou d of aqueous an d pigm en t
balloon s th rough th e iridectom y, in dicat ing pen et rat ion of th e iris.
Argon Laser Trabeculoplasty

Th e Glaucom a Laser Trial, a m ajor prospect ive, ran dom ized st udy, con cluded th at
laser t rabeculoplast y as an in it ial t reat m en t for op en -angle glaucom a is as safe
an d as effect ive as m edical t reat m en t . In som e cases, it m ay be m ore appropriate
as in it ial th erapy. Th ese cases in clude (1) pat ien ts w h o can n ot or w ill n ot com p ly
w ith prescribed m edical th erapy, (2) areas of th e w orld w h ere adequ ate m edical
t reat m en t is un feasible because of povert y.
In all cases, to be su ccessful, th e angle does h ave to be open , th e m edia m u st be
clear, an d on e m u st h ave access to th e t rabecular m esh w ork. Jam es B. Wise, M.D.,
w h o developed argon laser t rabecu loplast y (argon laser t rabecu loplast y), h as ob-
ser ved th at populat ion group s of ph akic pat ien ts do bet ter th an aph akic. It ap pears
th at aph akia does in terfere w ith respon se to th e laser, probably by th e in fluen ce
of vit reous in th e an terior ch am ber an d th e t rabecu lar m esh w ork. In terest ingly
en ough , p seu doph akic pat ien t s resp on d to th e laser ver y sim ilarly th an ph akic pa-
t ien t s. Th at is, th e presen ce of th e posterior ch am ber len s im plan t keeping th e vit-
reou s out of th e an terior ch am ber greatly im p roves th e respon se to th e laser. Eyes
w ith an terior ch am ber len ses an d glaucom a u sually sh ow a p oor laser respon se
du e to uveit is an d t rabecu lar dam age from th e len s ( Fig. 17.14 ).
Fig. 17.14 Applying laser burns correctly in argon laser trabeculoplast y. Cross section to
the left; cornea (E), Schlemm canal (C), scleral spur (S), Schwalbe line (G), anterior corneo-
scleral meshwork (A), pigmented band (P), and uveal meshwork (U). Proper placement of
the 50 µm laser burn (L) is shown at the posterior margin of the pigmented band (P). To
the right is a gonioscopic view with iris (I) below. Properly placed 50 µm laser burn at the
posterior pigment band (P) shown at (1). Another burn is shown at (2) along the posterior
margin of the pigment band (P). An oversized burn is shown at (3), spanning the entire
pigment band. A slightly misplaced burn is shown at (4) in the middle of the pigment
band. A seriously misplaced burn into the uveal meshwork (5). (From Boyd, BF, Luntz, M. Argon
laser trabeculoplast y. In: Innovations in the glaucomas. Highlights of Ophthalmology, 2002, 149. Courtesy
Jaypee Highlights Medical Publishers Inc., Panama.)
Retinal Laser Photocoagulation

Many sign ifican t applicat ion s h ave been discovered for clin ical use of th e laser in
oph th alm ology. Th e un ique feat u res of th e eye allow ing in t raocular t ran sm ission
an d select ive absorpt ion of ligh t en ergy accoun t for a w ide range of laser t reat-
m en ts.
Essen t ially, t reat m en t m ay be categorized in to five gen eral effect s: (1) in du ct ion
of ch orioret in al bu rn s or ret in al scar th at lead to (possible ph arm acological) n eu-
t ralizat ion of isch em ia-in duced ret in al n eovascu larizat ion ; (2) redu ct ion in ret in al
vascu lar perm eabilit y via direct vascular closu re or un kn ow n m ech an ism s; (3) ab-
lat ion of un desired t issue su ch as ch oroidal n eovascu larizat ion , t u m ors, abn orm al
n at ive vessels, or aqu eou s-produ cing t issu e; (4) in du ct ion of a ch orioret in al scar
th at m ay ser ve as a barrier to exten sion of subret in al fluid; an d (5) lysis of t rac-
t ion -in ducing or m edia-opacifying t issu es (Fig. 17.15 ).
Th e tech n iques of laser ph otocoagu lat ion in clu de th e follow ing broad catego-
ries: scat ter t reat m en t , focal t reat m en t , ablat ive t reat m en t , dem arcat ing t reat-
m en t , an d cut t ing t reat m en t .
Focal Macular Treatment

Macu lar edem a occurs from a variet y of disease m ech an ism s, bu t th e feat u re com -
m on to each is in creased ret in al vascular perm eabilit y. Con t ribut ing factors in -
clude ret in al isch em ia, in flam m at ion , an d t ract ion . Som e en t it ies in th e first of
Fig. 17.15 Argon laser treatm ent

of a retinal tear (T). (From Cortez, R.
Managem ent of retinal detachment. In:
Retinal and vitreoretinal surgery. Highlights
of Ophthalmology, 2002, 402. Courtesy
Jaypee Highlights Medical Publishers Inc.,
Panama.)
th ese categories are resp on sive to focal laser t reat m en t: clin ically sign ifican t dia-
bet ic m acular edem a an d bran ch vein occlu sion -associated m acu lar edem a. Macu -
lar edem a du e to cen t ral vein occlusion m ay resolve after t reat m en t , but th is is n ot
usually accom pan ied by a visu al ben efit .
Nd:YAG Laser Posterior Capsulotomy

Posterior capsu le opacificat ion (PCO) is curren tly th e m ost frequ en t postoperat ive
com plicat ion in cat aract su rger y.
Th e u se of hypoton ic solu t ion an d preser vat ive free lidocain e are being invest i-
gated in th e preven t ion of PCO. St udies w ith in vivo sp ecular m icroscopy suggest
th at a hydrop h ilic acr ylic in t raocu lar len s w ou ld h ave h igh er biocom pat ibilit y th an
th e hydroph obic acr ylic len s, con t rar y to som e p reviou s st udies, w h ich affirm ed
th at hydroph obic acr ylic len s h ad h igh er biocom pat ibilit y.
Opacificat ion of the posterior capsule is an in adequ ate term becau se it is n ot th e
capsule th at opacifies bu t an opaque m em bran e th at grow s, origin at ing from th e
epith elial cells th at w ere ret ain ed, w h ich proliferate an d m igrate on th e posterior
capsule ( Fig. 17.16 ).
Th e pearls of Elsch n ig origin ate from th e superim p osed epith elial cells of th e
flexible w ing of th e an terior capsu le an d m igrate to th e posterior capsu le.
Opacificat ion of th e posterior capsu le is t reated w ith Nd:YAG laser, w h ich is a
ph otodisru ptor laser. In oth er w ords, it u ses a h igh p ulse of ion izing elect rom ag-
n et ic en ergy to break th e t issue.
To ach ieve th e open ing of th e capsu le, begin from th e periph er y at 12 o’clock
an d go to 6 o’clock w ith a size larger th an th e pupil (un dilated). Make th e in cision
going from 3 o’clock to 9 o’clock u sing th e Abrah am len s. Fin ally clean th e residues
to avoid leaving float ing fragm en t s.
Fig. 17.16 Nd:YAG laser posterior capsulotomy.

Cryoprobes
Cr yoprobes are used for perip h eral ret in opexy in pat ien ts w ith periph erally lo-
cated ret in al breaks an d tears, in th e t reat m en t of ret in al t u m ors an d ret in al vas-
cular m alform at ion s. Th ey h ave also been u sed for perform ing cyclocr yoth erapy in
en d-st age glaucom as.
Th e t ip of th e probe is placed over th e globe at th e edge of th e break w h ile visu -
alizing w ith th e in direct oph th alm oscope. Th e freezing is th en applied un t il th ere
is w h iten ing of th e ret in a (freezing of th e vit reous sh ou ld be avoided). On ce th e t ip
h as th aw ed, it is rem oved an d th e n ext ap plicat ion is m ade ( Fig. 17.17 ).
Fig. 17.17 Cryoprobe.

Index
Note: Page n u m bers follow ed by f an d t in dicate figu res an d t ables, respect ively.
A prim ar y, 191
Ablepharon, differen tial diagn osis, 51 An em ia, 261. See also Sickle cell anem ia
Abscess An esth et ics, topical, an d n eurot ropic
differen t ial d iagn osis, 47 keratopathy, 155
orbital, 72f, 95f Angioedem a, 69, 72
su bp eriosteal, 71–73 Angioid st reaks, 270–271, 271f
presen tat ion , 71 causes, 270
Acantham oeba kerat itis, 147–149, 148f differen t ial d iagn osis, 21, 271, 272, 276,
differen t ial d iagn osis, 146, 149, 192 281, 282
Accom m odat ive esot rop ia, 341–342, 341f m an agem en t , 271
h igh , 341f, 342 presen t at ion , 270–271
part ially, 342 Angiom a, facial, 130
Acetam ide, for cen t ral ret in al arter y occlu sion , Angle kappa, 339, 339f, 340
268 An iridia, 135
Acet ic acid bu rn , 5–7 an d len s su blu xat ion , 13
Acid bu rn s, 5–7 An kylosing spondylit is, ocular involvem en t
Acn e rosacea, 100–101 in , 194
differen t ial d iagn osis, 192 An terior basem en t m em bran e dyst rop hy, an d
ep isclerit is in , 123 recu rren t corn eal erosion , 156
Acroch ord on , 52 An terior ch am ber cleavage syndrom e, 192, 258
Act in ic keratosis, 52–53, 53f An terior ch am ber dysgen esis syn drom es, 157
differen t ial d iagn osis, 52, 54, 62 An terior corneal dyst rophy(ies), 158–161
Act inom yces can alicu lit is, 65 An terior em br yotoxon, 157, 158
Acu te m u lt ifocal p osterior p lacoid p igm en t An terior sclerit is, 124
ep ith eliop athy, 204–205, 205f An terior segm en t t ransplan tat ion , for anterior
Acu te m yeloid leu kem ia, orbit al involvem en t st aphylom a, 189–191, 190f
in , 94 An terior uveit is, 10
Acu te ret in al n ecrosis syn drom e, 209 Aph akia, and ret in al detach m en t , 296–297
Aden oid cyst ic carcin om a, lacrim al glan d, Apocrine hydrocystom a, 57, 58f
98, 99f Apon eurosis repair, for ptosis, 38
Aden om a “Apple peeling” techn iqu e, for rem oval of
ciliar y body, 325 in tern al lim it ing m em bran e, 318,
pleom orp h ic, lacrim al glan d, 97–98, 97f 319f
Aden oviral conju n ct ivit is, 101–103, 102f Arcu s juven ilis, 173
Age-related m acular degen eration , 276–278, Arcu s senilis, 173, 174f
277f differen t ial d iagn osis, 157, 158
differen t ial d iagn osis, 269, 272, 274, 276, ARM. See Age-related m aculopathy
279 ARMD. See Age-related m acular degen erat ion
exu dat ive, 276, 277f Ar teriosclerosis, an d m acroan eur ysm , 269
an d vit reou s h em orrh age, 270 Ar terioven ou s m alform at ion , 90
m an agem en t , 276–278 cavern ous sin us, 128
n on exudat ive, 276, 277f ret in al, 337, 337f
presen t at ion , 276 Ar terioven ou s n icking, 260
risk factors for, 276 Ar tificial tears, 114–115
Age-related m acu lop athy, 276 Aspergillus kerat it is, 149, 150
Albinism , 285 Ast rocytom a, retin al, 335, 336, 337
Alkali burn s, 5–7 Ath erosclerosis, an d ocu lar isch em ic
differen t ial d iagn osis, 112 syn drom es, 268–269
an d recu rren t corn eal erosion , 156 Atop ic keratoconju n ct ivit is, 45, 110, 112, 153
Alkapton uria, blue sclera in , 134 Atopy
Allergic conjun ct ivit is, 45 ep isclerit is in , 123
Allergy, 47 an d filam en tar y kerat it is, 154
an d eyelid ed em a, 47, 69, 72 an d keratocon u s, 171
Alph a- Cor, 188 At rial sept al d efect , 353
Am aurosis fugax, 267, 268 At rop h ic bu lbi, 136
Am blyopia, vit reou s h em orrhage an d, 19 At rop h ic retin al h oles, 297, 297f
Am iodaron e, corn eal vert icillata cau sed by, Autoim m u ne disorders, 110. See also speci c
180, 181f disorder
Am m on ium hydroxide burn s, 5–7 Avellin o dyst rop hy, 161
AMPPPE. See Acute m ult ifocal posterior AVM. See Ar terioven ous m alform at ion
placoid p igm en t ep ith eliopathy Axen feld an om aly syndrom e, 135
Am yloid degen erat ion Axen feld Reiger syn drom e, 157, 158
conju n ct ival, 120
corn eal, p olym orph ic, 178, 179f B
Am yloidosis Bacillus
differen t ial d iagn osis, 57, 128, 191, 192 corn eal u lcer cau sed by, 146
orbital, 128 trau m at ic en doph thalm it is caused by, 213
539
540 Index
Bacterial corn eal ulcer, 146–147, 146f ch em ical, 5–7, 6f, 64, 110, 112, 113, 136,
Bacterial in fect ion 155, 192
differen t ial d iagn osis, 192 an d sym bleph aron , 116
endogenous en dophth alm itis caused by, 215 th erm al, 6, 112, 192
traum at ic en doph th alm it is caused by, 213
Bacterial keratit is, 9, 143, 147, 149 C
Ballet sign , 76t Calcific ban d keratop athy, 176–177, 177f
Ban d keratop athy, 176–177, 177f Calcium hydroxide bu rn s, 5–7
Bardet-Biedl syn drom e, an d retin it is Can alicular lacerat ions, 2, 3f
pigm en tosa, 285 Can aliculit is, 65, 66f, 68
Basal cell carcin om a, 59–61, 60f Candida albicans, n eon at al en dogen ous
differen t ial d iagn osis, 51, 53, 54, 56, 62, 64 en doph th alm it is cau sed by, 215
Bassen -Korn zw eig synd rom e, an d ret in it is Candida kerat it is, 149, 149f
pigm en tosa, 285 Can did iasis, ocu lar involvem en t in , 201
Bear-t rack lesion s, 332 Can d lew ax d rip p ings, 199
Behçet syn drom e, ocu lar involvem en t in , Cap illar y h em angiom a, 81–84, 84f, 85t
212–213, 212f d ifferen tial d iagn osis, 59, 86
Berlin edem a, 275 opt ic disk, 334, 335f
Best disease, 280–281 ret in al, 334–335, 335f
Biet t i’s cr yst alline retin op athy, 280 d ifferen tial d iagn osis, 329, 335, 336, 337
Birdsh ot ch orioret in it is, 280 exop hyt ic, 329
Birdsh ot ret in och oroidop athy, 206–207.207f p resen tat ion , 334
Birth t raum a, corn eal involvem en t in , 166, Capsular ten sion ring, 220
170, 192 Carbogen th erapy, for cen t ral ret in al arter y
Bleeding disorders, an d hyp h em a, 15 occlu sion , 268
Bleph aritis, 100–101 Cardiac failure, h igh -out put , 84
differen t ial d iagn osis, 46, 63, 64 Carot id cavern ou s fist ula, 90–93, 92f,
infect iou s, 100, 100f 127–129, 128f
pem p h igoid an d, 112 differen t ial d iagn osis, 30, 72
seborrh eic, 100 h igh -flow, 90, 93
st aphylococcal, 100, 106 low -flow, 90, 92f, 93
an d trich iasis, 64 post t rau m at ic, 31–32, 31f, 90, 93
Bleph arop him osis syn d rom e, 33, 38, 48, 48f, traum at ic, 31–32, 31f, 90, 93
49 Cast roviejo square graft , 186f
Bleph aroptosis, 48 Cat aract
Bleph arospasm an d d egen erat ive m yop ia, 272
in congen ital glaucom a, 134–135 hyperm at ure, an d len s sublu xat ion , 13
differen t ial d iagn osis, 35 m at u re, 222–223, 223f–226f
Blood vessels, dilated, 125–126, 126f m iot ic pu p il, 220–221, 221f
in St u rge-Weber syn drom e, 126–127, 127f posterior polar, 218–219, 218f
Blow -ou t fract u res, orbit al, 26–28, 27f posterior su bcap su lar, an d ret in it is
Blue sclera pigm en tosa, 285
causes, 134 sen ile, 226–227, 226f–229f
in osteogen esis im p erfect a, 133–134, 134f differen t ial d iagn osis, 13
Bon e m arrow t ran sp lan tat ion , an d su blu xated, 219–220, 219f
conju n ct ivalization of corn ea, 136 traum at ic, 11–13, 12f
Boston sign , 76t Cataract su rger y. See also In t raocular len s
Brain t um or su rger y, an d n eu rotrop ic corn eal edem a after, 236–237
keratopathy, 155 an d cystoid m acu lar edem a, 238–239, 239f,
Bran ch ret in al arter y occlu sion , 265–266 274, 275f
differen t ial d iagn osis, 19, 266 elevated in t raocu lar p ressu re after, 238
Bran ch ret in al vein occlu sion , 265f en doph th alm it is after, 240–242, 241f
differen t ial d iagn osis, 261, 263, 269 hypotony after, 237–238
BRAO. See Bran ch ret inal ar ter y occlusion in t raop erat ive com p licat ion s, 234–236
Breast can cer, orbit al m et astases, 94 iridodialysis in , 235, 235f
Bron ch ial carcin om a posterior cap su lar ru pt u re in , 234–235,
orbital m etast ases, 94 235f
ret in op athy secon dar y to, 217 postop erat ive com p licat ion s, 236–243
Brow n -McLean syn d rom e, 236 ret in al d etach m en t after, 239–240, 240f
Brow n syn drom e, 353–354, 353f sph in cter-involving tech n ique, 221, 221f
Brucellosis, 101 sph in cter-sparing tech n ique, 221
Bruch m em bran e, defect s, 20 su b 1 m m 700-m icrom eter
Brush field spot s, 325 (Microp h akon it), 218f, 219
BRVO. See Bran ch ret in al vein occlu sion w oun d leak after, 237–238
Bu llae, subepith elial, Fuch s end oth elial Cavern ous h em angiom a, 85t, 90, 91f
dyst rophy an d, 168f differen t ial d iagn osis, 81, 335, 336, 337
Bu llous keratop athy iris, an d hyp h em a, 15
ap h akic, 167, 169, 192 ret in al, 335, 336, 336f, 337
differen t ial d iagn osis, 167, 169, 192 Cavern ous sin us
pseu dop h akic, 167, 169, 192 arterioven ou s m alform at ion , 128
Bu ph th alm os th rom bosis, 71–73
in congen ital glau com a, 134–135, 135f differen t ial d iagn osis, 69, 76, 78, 93, 128
differen t ial d iagn osis, 157, 158 presen t at ion , 71
Bu rn(s) Cavern ous sin us syndrom e, 128
acid, 5–7 CCF. See Carot id cavern ou s fist u la
alkali, 5–7, 112, 156 Cellu lit is
Index 541
acu te st reptococcal, 69 precip itat ing factors, 273

orbital, 69, 71–73, 73t presen tat ion , 273
adjacen t in fect ion an d , 71 Ch oroid
differen t ial d iagn osis, 30, 47, 66, 69, 73t, at rophy, age-related, 272
76, 78, 87, 128 cavern ous h em angiom a, 330–331, 330f
m an agem en t, 73 dyst roph ies, 285
post t rau m at ic, 71 h em angiom a, 327, 328, 329
presen tat ion , 71 m elan ocytom a, 332
sin u s-related, 71 m elan om a, 132, 327–329, 328f
w arning signs w ith , 73 am elan ot ic, differen t ial diagn osis, 329
presept al, 69–71 differen t ial d iagn osis, 216, 327, 328,
w ith allergic react ion , 70f 330–333, 338
w ith bacterial infect ion, 70f m an agem en t, 329
w ith dacr yocyst it is, 67f presen tat ion , 327–329, 328f
differen t ial d iagn osis, 47 vit reou s h em orrh age in , 18
w ith fungal infect ion, 70f m et astat ic t u m or, 329–330, 329f
Cen t ral clou dy dyst rophy of Fran çois differen t ial d iagn osis, 327–331
(corn eal), 165, 165f, 175 n eovascularizat ion
Cen t ral cr ystallin e dyst rop hy of Sch nyder, an d cystoid m acu lar edem a, 274
164, 164f, 175 differen t ial d iagn osis, 21, 275
Cen t ral ret inal ar ter y occlu sion , 266–268, n evu s, 326–327, 327f
267f differen t ial d iagn osis, 328, 330, 331, 332
differen t ial d iagn osis, 19, 266 osteom a, 327, 331–332, 331f
w ith du rat ion of on set less th an 24 h ours, differen t ial d iagn osis, 327–331
268 ru pt u re
m an agem en t, 268 differen t ial d iagn osis, 276
Cen t ral ret inal vein occlu sion , 264, 265f traum at ic, 20–21, 20f
differen t ial d iagn osis, 261, 263, 264, 269 t um ors, 326–332
m an agem en t, 264 Ch oroidal det ach m en t , 316–317
presen tat ion , 264, 265f expulsive, 317
Cen t ral serou s ch orioret in opathy, 273–274, h em orrh agic, 317
273f serou s, 316
differen t ial d iagn osis, 274 in t raop erat ive, 316
ep idem iology, 273 postop erat ive, 316
m an agem en t, 274 trau m at ic, 316
precip itat ing factors, 273 Ch oroiderem ia, 285
presen tat ion , 273 differen t ial d iagn osis, 286
Cen t ral serou s ret in op athy, 263 Ch oroidit is
CFEOM. See Ext raocular m uscle(s), congenit al m u lt ifocal, 203
fibrosis serp igin ou s, 205–206, 206f
Chalazion , 45–46, 45f Ch rom ic acid bu rn , 5–7
bleph arit is an d, 100 Ch rom osom al deletion /du plication , 135
differen t ial d iagn osis, 46, 51, 62, 63, 69, 72 Cicatrizing disorders, 111–116
Chalcosis, 24 Ciliar y body
Chan dler syn drom e, 167, 258 aden om a, 325
Chem ical burn s, 5–7, 6f cyst , 325
an d conju n ct ivalizat ion of corn ea, 136 m elan om a, 325–326
differen t ial d iagn osis, 110, 112, 113, 136, differen t ial d iagn osis, 322
192 vit reou s h em orrh age in , 18
an d dist ich iasis, 64 m et astat ic lesion s, 325
an d n eurot ropic keratop athy, 155 t um or, 323, 325–326
Chem osis, 138–140 CIN. See Corn eal in t raepith elial n eoplasia
in acu te bacterial conjun ct ivit is, 105 Cleft p alate, 353
causes, 138t Cleidocran ial dysp lasia, blu e sclera in , 134
m assive, 139f CME. See Cystoid m acular edem a
m ild, 139f CNV, 283
Chen ey syndrom e, blue sclera in , 134 CNV m em bran es, 276–278
Cherr y-red spot , 267 Coat s disease, 286, 335, 336, 337
Chickenpox, 143 Cobbleston e degen erat ion , 290–291, 291f
Child abuse, t raum at ic vit reou s h em orrhage Cockayn e syn d rom e, an d recu rren t corn eal
in , 18 erosion , 156
Chlam ydia t rach om a, 106–109 Cogan m icrocyst ic edem a, 158–159, 159f
Chlam ydia t rachom at is, 106, 150 Cogan -Reese syn d rom e, 258, 322
Ch loroqu in e, corneal vert icillata cau sed by, Cogan syn drom e, 150
180 Collagen vascu lar disease
Chorioret inal scarring, 332 differen t ial d iagn osis, 143, 172, 261
Chorioret init is perip h eral u lcerat ive keratit is in , 182
syp h ilit ic, 198 Colobom a(s), 50–51, 50f, 219, 219f
Toxoplasm a, 200–202, 201f congen it al, 201
t uberculous, 201 syn drom es associated w ith , 51
vit iligin ou s, 206–207.207f Com m ot io ret in ae, 19–20, 19f
Ch orioret inop athy, cen t ral serou s, 273–274, Con e dyst rop h ies, 281–282
273f differen t ial d iagn osis, 282
differen t ial d iagn osis, 274 gen et ics, 281
ep idem iology, 273 m an agem en t, 282
m an agem en t, 274 presen tat ion , 282
542 Index
Congen ital an om aly(ies), corn eal, 157–158 differen t ial d iagn osis, 175
Congen ital h ereditar y corneal dyst rop hy, 192 ch em ical bu rn . See Ch em ical bu rn s
Conju n ct iva congen it al an om alies, 157–158
am yloid degen erat ion , 120 conju n ct ivalization , 136, 136f
edem a, 138. See also Ch em osis degen erat ion , 173–180
foreign body, 5f polym orp h ic am yloid , 178, 179f
lacerat ion , 4–5, 5f Salzm an n n od u lar, 177–178, 177f
lip oid/lip id d egen erat ion , 120–121, 121f sph eroidal, 178
m elan om a, 132–133, 132f den drite
m et ap lasia, in ectrop ion , 137, 137f in EBV in fect ion , 145
m et astases to, 133 in HSV kerat it is, 141f
n eop lasm s in HZV in fect ion , 144f
differen t ial d iagn osis, 118, 120 dep osits in , 173–180
w ith secon dar y h em orrh age, 3 dyst rophy. See Corn eal dyst rop hy(ies)
pigm en ted lesion s, 129–133 ectat ic disorders, 170–173
reten t ion cyst , 121–122, 121f edem a
differen t ial d iagn osis, 118 after cataract ext ract ion , 236–237
salm on p atch in , 79, 79f in congen ital h ered itar y en doth elial
Conju n ct ivit is, 101–111 dyst rop hy, 170, 170f
acu te bacterial, 105–106, 106f Fuchs en dothelial dyst rophy an d, 167,
acu te h em orrh agic, 101, 103–104, 103f 168f
aden oviral, 101–103, 102f in p osterior p olym orph ous dyst rophy,
differen t ial d iagn osis, 112 169, 169f
adu lt in clu sion , 106, 107t–108t, 109 en larged n er ves in , 191
differen t ial d iagn osis, 112 differen t ial d iagn osis, 163
allergic, d ifferen t ial diagn osis, 45 en largem en t , in congen ital glau com a,
atop ic, differen t ial diagn osis, 111 134–135
bacterial, associated w ith eyelid edem a, erosion s, trau m at ic, differen tial d iagn osis,
105, 106f 159, 160
ch lam ydial (in clu sion ), 106, 107t–108t, 109 farin ata, d ifferen t ial d iagn osis, 178
differen t ial d iagn osis, 112 Fleck dyst rophy, 164–165
ch ron ic, 109–110 differen t ial d iagn osis, 166
w ith bleph arit is, 100 foreign body, 8–9, 8f
differen t ial d iagn osis, 62, 63 differen t ial d iagn osis, 7
differen t ial d iagn osis, 3, 66, 101, 114, 124, Fu ch s en dothelial dyst rop hy, 167, 168f
125 differen t ial d iagn osis, 167, 169, 191, 192
gian t papillar y, 110–111, 111f graft reject ion , 188, 188f
differen t ial d iagn osis, 45 gu t tae, 167, 168f
gon ococcal, 105–106 differen t ial d iagn osis, 160, 178
m u cop u ru len t , in Steven s-Joh n son hydrops
syn drom e, 113 in keratocon u s, 171
n eon atal, ch lam ydial, 106 in keratoglobu s, 171, 172f
pseu dom em bran ou s, in Steven s-Joh n son in fect ion s, 141–150
syn drom e, 113 in flam m at ion , 150–153
recu rren t , 109–110 differen t ial d iagn osis, 192
toxic, differen tial diagn osis, 192 inju r y, an d recu rren t corneal erosion , 156
vern al, 110–111, 111f keloid, 175–176, 176f
differen t ial d iagn osis, 111, 192 lacerat ion , 9–10, 9f
viral, 101, 104 lip oid/lip id d egen erat ion , 120–121, 121f
associated w ith eyelid edem a, 69, 72 n eovascu larizat ion , in Steven s-Joh n son
Con n ect ive t issu e disease (CTD), perip h eral syn drom e, 113
u lcerat ive kerat it is in , 182 perforat ion
Con stan t exot rop ia, 344, 344f differen t ial d iagn osis, 7
Con t act derm at it is, d ifferen tial d iagn osis, in Steven s-Joh n son syn drom e, 113
69, 72 recu rren t erosion , 156
Con t act len ses scarring
clean ing solu tion s, an d kerat it is, 147, 149 cen t ral, 164
corn eal scarring cau sed by, differen t ial in HSV kerat it is, 141f
diagn osis, 171 in terst it ial kerat it is an d , 150, 151f
for dr y eye, 115 in Steven s-Joh n son syn drom e, 113
gian t p ap illar y conju n ct ivit is of, 110–111 su rface d isord ers, 150–153
over w ear, keratit is cau sed by, 154 th in n ing. See also Keratocon u s; Pellu cid
differen t ial d iagn osis, 152 m argin al d egen erat ion (corn ea)
an d recu rren t corn eal erosion , 156 perip h eral, 180–185
u se, differen t ial diagn osis, 192 u lcer, 7
Con t recou p inju r y(ies), 11, 19 from Acantham oeba, 147, 148f
Convergen ce in su fficien cy, 343 bacterial, 146–147, 146f
Corn ea differen t ial d iagn osis, 7, 143
abrasion s, 7–8, 7f fu ngal, 149, 149f
differen t ial d iagn osis, 9, 10, 105 h erpet ic, d ifferen t ial diagn osis, 192
recu rren t , 109–110 h erpet ic den d rit ic, 104f
cen t ral clou dy dystrop hy of Fran çois, 165, im m u n e-m ediated , 146, 147, 153
165f in fect iou s, differen t ial diagn osis, 153
differen t ial d iagn osis, 175 m argin al
cen t ral cr ystallin e dyst rop hy of Sch nyder, in HSV in fect ion , 142
164, 164f st aphylococcal, 177, 182
Index 543
sterile, 146 in parasit ic in festat ion , 121, 122

n eurot ropic p ost t rau m atic in clu sion , 121
in HSV in fect ion , 142, 143 d erm oid. See Derm oid cyst(s)
in HZV in fect ion , 144f d uctal, 57–59
in Steven s-Joh nson syn d rom e, 113 foveal, 275
t rach om a, 107f of iris, 323
viral, differen t ial diagn osis, 146 of Moll, 57, 58f
Corn ea gu t tata, 167 p ars p lan a, 290
Corn eal dyst rophy(ies), 158–170 sebaceou s, 57, 58f
an terior, 158–161 of Zeis, 57–59
cen t ral clou dy dystrop hy of Fran çois, 165, Cyst in osis, differen t ial diagn osis, 160
165f Cystoid m acular edem a, 274–276, 275f
d ifferen t ial d iagn osis, 175 after cataract su rger y, 238–239, 239f, 274,
cen t ral cr ystallin e dyst rop hy of Sch nyder, 275f
164, 164f causes, 274
d ifferen t ial d iagn osis, 175 differen t ial d iagn osis, 263, 274, 275
congen it al h eredit ar y, differen t ial diagn osis, m an agem en t, 275–276
192 path ogen esis, 274
congen it al h eredit ar y en d oth elial, 170, 170f postcat aract surger y, 238–239, 239f, 274,
d ifferen t ial d iagn osis, 166, 169 275f
Fleck, 164–165 presen tat ion , 275
d ifferen t ial d iagn osis, 166 Cytom egaloviru s (CMV)
Fu ch s en doth elial, 167, 168f congen it al in fect ion , 208
d ifferen t ial d iagn osis, 167, 169, 191, 192 in im m u n osu pp ressed p at ien t , 208, 209f
granular, 161, 162f in clu sion ch orioret init is, d ifferen t ial
d ifferen t ial d iagn osis, 161, 163 diagn osis, 201
lat t ice, 162–163, 162f ret in itis caused by, 208, 208f
d ifferen t ial d iagn osis, 178
p osterior, 167–170 D
p osterior polym orph ou s, 169, 169f Dacr yoaden it is, 78, 97
d ifferen t ial d iagn osis, 166, 170, 191, 258 Dacr yocyst itis, 66–67, 67f
p re-Descem et , 165–166 ch ron ic, differen t ial diagn osis, 65
st rom al, 161–166 differen t ial d iagn osis, 47, 68, 110
congen it al h eredit ar y, 166 Dalyr ym p le sign , 76t
differen t ial d iagn osis, 170 Degen erat ive m yopia, 272, 272f
d ifferen t ial d iagn osis, 164 Derm atobu llou s d isord ers, differen t ial
p osterior am orp h ou s, 166 diagn osis, 112
Corn eal elastosis, 178 Derm atoch alasis, 42–43, 42f
Corn eal in t raep ith elial n eop lasia, differen t ial differen t ial d iagn osis, 45, 47
diagn osis, 159 Derm oid cyst(s), 50, 80–81, 82f–83f
Corn eal su rger y, 185–191 deep, 80–81, 82f–83f
Corn eal vert icillata, 180, 181f differen t ial d iagn osis, 84, 90, 93, 94, 118,
Corynebacterium diphtheriae, conjun ct ivit is 192
cau sed by, 105 ru pt u red , differen t ial d iagn osis, 87
d ifferen t ial d iagn osis, 112 su perficial, 80–81, 82f
Cou p injur y(ies), 11 Descem et m em bran e
Cow en sign, 76t breaks, keratoconu s an d, 171
Coxsackievirus A24, conju n ct ivit is cau sed by, ru pt u re, 134
102, 103 tears, differen t ial diagnosis, 192
Cranial n er ve(s) Descem et st ripp ing en doth elial keratoplast y,
III. See Third-n er ve palsy 187, 187f
IV. See Fou rth -n er ve p alsy Diabetes m ellit u s. See also Maculopathy,
VI. See Sixth -n er ve p alsy diabet ic
CRAO. See Cen t ral ret in al arter y occlu sion an d n eu rot ropic keratopathy, 155
Crocodile sh agreen , differen t ial diagn osis, 175 Diabet ic ret in opathy, 261–264
Crocodile tears, 353 differen t ial d iagn osis, 261, 263, 264, 269,
Croh n’s disease (CD), ocu lar involvem en t in, 286
195 m an agem en t, 263–264, 263f
Crouzon disease, an d lens sublu xat ion , 13 n onp roliferat ive, 261, 262f
CRVO. See Cen t ral ret in al vein occlu sion differen t ial d iagn osis, 263
Cr yoth erapy, for ret in al det ach m en t, 302 presen tat ion , 261
Cr ystallin e retin opathy, d ifferen tial d iagn osis, proliferat ive, 261, 262f
280 differen t ial d iagn osis, 263
CSCR. See Cen t ral serou s ch orioret in op athy w ith preret in al h em orrh age, 262f–263f
CSR. See Cen t ral serous ret in opathy an d vit reou s h em orrh age, 270
CTR. See Capsular ten sion ring Diath erm y, for ret in al det ach m en t , 301
Cushing syn drom e, an d cen t ral serous Diffu se corn eoscleral kerat it is, 190
ch orioret in opathy, 273 Dilated vessels, 125–126, 126f
Cutan eous h orn , differen t ial diagn osis, 53, 54 in St u rge-Weber syn drom e, 126–127, 127f
Cu tis laxa, blue sclera in, 134 Dim m er kerat it is, 101
Cyclit is, ch ron ic, 200 Dissociated st rabism u s com p lex, 346–347,
Cylin drom a, differen t ial diagn osis, 58 347f
Cyst(s) Distich iasis, 64, 65f
ciliary body, 325 differen t ial d iagn osis, 35, 110
conjun ct ival pem p h igoid an d, 112
lym ph at ic, 121, 121f Distract ion test , 33
544 Index
Divergen ce excess, 343 d ifferen tial d iagn osis, 283

Doh lm an keratoprosth esis, 188, 189f orbit al, 27
Dow n syn drom e ret in al, post t rau m at ic, 21–22
Brush field sp ot s in , 325 su bconju n ct ival. See Ch em osis
corn eal involvem en t in , d ifferen tial Eh lers-Dan los syn d rom e
diagn osis, 192 an d angioid st reaks, 270
an d keratocon u s, 171 blue sclera in , 134
Drug(s), an d hyp h em a, 15 an d d egen erat ive m yop ia, 272
Drug abu se, differen t ial d iagn osis, 263 an d len s su blu xat ion , 13
Drug-in d u ced degen erat ion s, differen t ial Eikenella can aliculit is, 66f
diagn osis, 281 Elsch n ig spot s, 260–261
Drug-ind uced ret in op athy, differen t ial Em boli, in bran ch ret in al arter y occlu sion ,
diagn osis, 282 265–266
Dru sen En ceph alocele, 84
fam ilial, differen t ial diagn osis, 279, 280 En closed oral bay, 289, 289f
opt ic n er ve h ead , an d ret in itis p igm en tosa, En dop h th alm it is, 213–216
285 after cataract surger y, 240–242, 241f
Dr y eye Candida, 215
causes, 114 differen t ial d iagn osis, 326, 335, 336, 337
differen t ial d iagn osis, 101 en dogen ou s, 215–216
Dr y-eye syn d rom e, 114–116, 114f postop erat ive, 214–215, 214f, 240–242,
an d filam en tar y kerat it is, 154 241f
DSEK. See Descem et st ripping en dothelial post t rau m at ic, 12f
keratoplast y sequ elae, differen t ial diagn osis, 136
Du an e syn drom e, 351–353, 352f traum at ic, 213–214
bilateral, 352, 352f En doth elial keratop last y, 187, 187f
system ic association s, 353 En doth eliitis, in HSV in fect ion , 142
t ypes, 352f, 353 En op h th alm os, ru pt u red globe an d, 16
Du ral sin u s arterioven ou s fist u la, differen t ial Enterobacter, corn eal u lcer caused by, 146
diagn osis, 128 En teroviru s grou p 70, conju n ct ivit is cau sed
Dysthyroid orbitopathy, 74–77, 74f–75f by, 102, 103
differen tial d iagn osis, 93, 128 En t rop ion , 35, 36f
m an agem ent , 76–77 cicatricial, 35, 36f
congen it al, 35
E differen t ial d iagn osis, 48, 49, 64
Eales disease involu t ion al, 35, 36f
differen tial d iagn osis, 286 spasm odic, 109, 110f
an d vit reou s h em orrh age, 270 EOB. See En closed oral bay
EBMD. See Epith elial basem en t m em bran e Epiblep h aron , 48–49, 49f
dyst rophy d ifferen tial d iagn osis, 35, 49, 64
Eccrin e hydrocystom a, 57, 58f Epican th al fold s, syn drom es associated w ith ,
differen tial d iagn osis, 57 49
Eclip se ret in op athy, 287 Epican th u s, 49–50, 50f
Ectop ia len t is, 251 Epican th u s inversu s, 49
congen it al, an d len s su blu xat ion , 13 in blep h aroph im osis syn drom e, 48, 48f
differen tial d iagn osis, 13 Epican th u s p alp ebralis, 49
Ect rop ion , 33–35, 34f Epican th u s su p erciliaris, 49
in bleph aroph im osis syn drom e, 48, 48f Epican th u s tarsalis, 49, 50f
cicat ricial, 33–35, 34f Epid erm olysis bu llosa, 112
congen ital, 33, 35 Epin eph rin e, top ical, an d cystoid m acu lar
conju n ct ival m etaplasia in , 137, 137f edem a, 274
differen tial d iagn osis, 45 Epip h ora, 2
involut ion al, 33, 34f in congen ital glau com a, 134–135
m ech anical, 33 Epiret in al m em bran e
paralyt ic, 33–35, 34f d ifferen tial d iagn osis, 23, 321
Eczem a, differen t ial d iagn osis, 47 id iopath ic, 320–321, 320f
Edem a. See also Angioedem a; Cystoid m acu lar p rim ar y, 320
edem a secon dar y, 320
Cogan m icrocyst ic, 158–159, 159f Episcleral ven ou s pressu re, elevated, 252–253
conju n ct ival, 138. See also Ch em osis Episclerit is, 123–124, 123f–124f
corn eal differen t ial d iagn osis, 3, 125
after cataract ext ract ion , 236–237 n odular, 123, 124f
in congenit al heredit ar y en doth elial in Steven s-Joh n son syn drom e, 113
dyst rophy, 170, 170f Ep ith elial basem en t m em bran e dyst rop hy,
Fu ch s en doth elial dyst rophy an d, 167, 158–159, 159f
168f differen t ial d iagn osis, 160, 161
in posterior polym orph ou s dyst rop hy, Ep ith eliom a, 112
169, 169f Ep stein -Barr viru s (EBV), in fect ion
eyelid, 47, 47f corn eal, 145, 145f, 150, 152
in acu te bacterial conju n ct ivit is, 105, 106f differen t ial d iagn osis, 101, 152
allergy an d, 47, 69, 72 ERM. See Ep iretin al m em bran e
in thyroid eye disease, 47, 47f Er ysip elas, 69, 72
in viral conju n ctivit is, 69, 72 Er yth em a m u lt iform e
m acular differen t ial d iagn osis, 112, 136
clin ically sign ifican t , 261, 262f an d sym bleph aron , 116
diabet ic, 283 Er yth em a m u lt iform e m ajor, 113
Index 545
Er yth em a m u lt iform e m in or, 113 isolated, 30

Er yth roderm a, congen ital ich thyosiform , 112 Francesch et t i syn drom e, 51
Esot rop ia Front alis-sling procedure, for ptosis, 38
accom m odat ive, 341–342, 341f Fu ch s en dothelial dyst rop hy, 167, 168f
refract ive, 341f, 342 differen t ial d iagn osis, 167, 169, 191, 192
in fan t ile, 340–341, 340f Fuchs h eteroch rom at ic iridocyclit is, 197
Ew ing sarcom a, m etast atic, 94 Fuchs h eteroch rom ic iridocyclit is, 197
Exoph th alm os Fuchs spot , 272
post t rau m at ic, 31–32, 31f Fuchs superficial m argin al kerat it is, 184
pu lsat ing, 127–128 Fun dus albipun ctat us, 280, 280f
causes, 31 differen t ial d iagn osis, 279
post t rau m at ic, 31–32, 31f Fun dus flavim aculat us, 278–279, 279f
ru pt u red globe an d , 16 differen t ial d iagn osis, 279
in thyroid eye d isease, 73t m an agem en t, 279
Exot rop ia presen tat ion , 278, 279f
con secu t ive, 344 Fungal in fect ion
con st an t , 344, 344f differen t ial d iagn osis, 192
in fan t ile, 344 en dogen ou s en dophth alm it is caused by,
in term it ten t , 342–343, 343f 215
secon dar y, 344 postop erat ive en doph th alm it is caused by,
sen sor y, 344 214
Exp osu re kerat itis, 183 trau m at ic en doph thalm it is caused by, 213
Exposu re keratopathy, 153–154 Fungal kerat it is, 149–150, 149f
Ext raocu lar m u scle(s). See also speci c m uscle differen t ial d iagn osis, 9, 143, 146, 147
congen it al fibrosis, 357–358, 357f Furrow degen erat ion , 185
Eyelid (s) differen t ial d iagn osis, 183, 184
bacterial in fect ion , 69 Fusarium keratit is, 149, 150
colobom a, 50–51, 50f
deform it ies, in Steven s-Joh n son syn drom e, G
113 Gardn er syn drom e, 332
dist ract ion test , 33 Gast roesoph ageal reflu x disease, and cen t ral
edem a, 47, 47f serou s chorioret in opathy, 273
in acu te bacterial conjun ct ivit is, 105, 106f Gastroin test in al m align an cy, orbit al
allergic, 47, 69, 72 m et astases, 94
in thyroid eye d isease, 47, 47f Gen t am icin , in t raocu lar inject ion , 267
in viral conjun ct ivit is, 69, 72 Gh ost vessels, 150, 151f
inw ard rotat ion . See En t ropion differen t ial d iagn osis, 163
lacerat ion , 1–2, 1f, 3f Gian t cell arterit is, 268–269
ou t w ard rotat ion (eversion ). See Ectrop ion Gifford sign , 76t
papillom a, 51, 51f Glau com a
ret raction , 43, 43f acu te, 3
t um ors, 51–57, 59–64 acu te angle, 7
u pp er angle-closu re, 254–257
abn orm ally low p osit ion . See Ptosis acu te, 254–255, 254f, 255f
cicat ricial ch anges, 43 ch ron ic, 256, 256f
scarring, 43 w ith pupillar y block, 254
w ithout pupillar y block, 254
F angle recession , 13
Fabr y disease, corn eal vert icillata in , 180, 181f congen it al, 127, 134
Fam ilial aden om atou s p olyp osis, 332 differen t ial d iagn osis, 68, 166, 170, 192,
Fam ilial exu dat ive vit reoret in op athy, 335, 326
336, 337 gh ost-cell, vit reou s h em orrh age an d , 18–19
Ferr y lin e, 179 len s-in du ced, 250–251
FF. See Fun dus flavim aculat us len s p art icle, 250
Fibrin collaret tes, 100, 100f m align an t , 258–259
Filam en tar y keratit is, 154–155, 154f differen t ial d iagn osis, 326
Fleck dyst rop hy (corn eal), 164–165 n eovascular, 256–257
differen t ial d iagn osis, 166 n orm al-ten sion (low -ten sion ), 246–248,
Fleisch er ring, 179, 179f 246f–247f
keratocon u s an d , 171 op en -angle
Fleu ret tes, 334 an d d egen erat ive m yop ia, 272
Flexn er-Win terstein er roset tes, 334 prim ar y, 244, 245f
Flop py eyelid syn drom e, 44–45, 44f an d ret in it is pigm en tosa, 285
an d keratocon u s, 171 secon dar y, 248, 249f
Foreign body ph acoan ap hylact ic, 251
conju n ct ival, 5f, 129, 130 ph acolyt ic, 251
corn eal, 7, 8–9, 8f ph acom orp h ic, 12, 251
differen t ial d iagn osis, 7, 9, 129, 130, 133, pigm en tar y, an d degen erat ive m yopia, 272
323 in p osterior p olym orph ou s dyst rophy, 169
in t raocu lar, 9, 23–25, 24f, 217, 323 post t rau m at ic, 12
int raorbit al, 28–29, 29f in sarcoidosis, 198
an d recu rren t corn eal erosion , 156 uveit ic, 251–252, 252f, 253f
Fourth -n er ve palsy, 349–350, 349f–350f differen t ial d iagn osis, 15
Foveal cysts, 275 in uveit is-glau com a-hyp h em a syn drom e,
Foveom acu lar ret in it is, 287 196
Fract ure(s), orbit al, 26–28 uveit is related to, 196
546 Index
Globe, rupt u red, 16–17, 17f an d trich iasis, 64

differen t ial d iagn osis, 30 Herring’s law, 43
Golden h ar syn d rom e, 51, 353 High accom m odat ive esot ropia, 341f, 342
Goldzieh er sign , 76t Histoplasm osis
Grapevin es, in adu lt toxop lasm osis, 201 ocu lar
Graves’ disease, 74–77 differen t ial d iagn osis, 21, 201, 272
in filt rat ive (severe), 74 presu m ed, 202–203, 202f
n on in filt rat ive (m ild), 74 vit reou s h em orrh age in , 18
op h th alm ic m an ifest at ion s, 76t Histospot s, 202f
Graves oph th alm op athy, 355–356, 355f. See differen t ial d iagn osis, 279, 280
also Thyroid eye disease; Thyroid- Hollen h orst p laqu es, 265
related op h th alm op athy Holt-Oram syn d rom e, 353
Grid laser, for diabet ic ret in op athy, 263, 263f Hom er Wrigh t roset tes, 334
Griffith sign , 76t Hom ocyst in u ria, 135
Grön blad-St ran dberg synd rom e, an d angioid an d len s su blu xat ion , 13, 14
st reaks, 270 Hord eolu m , 46–47, 46f
Guillain -Barré syn drom e, Miller-Fish er bleph arit is an d, 100
varian t , eyelid ret ract ion in , 43 differen t ial d iagn osis, 46, 47, 66
Gyrate at rop hy extern al, 46–47, 46f
differen t ial d iagn osis, 272, 285, 286 in tern al, 46–47
gen et ics, 286 Hu dson -Stäh li lin e, 179
m an agem en t, 286 Hu rler syn drom e, 267
presen tat ion , 286 Hyalin e Pillat scleral plaque, 140, 140f
Hydroch loric acid bu rn , 5–7
H Hydroxych loroquin e, corn eal ver ticillat a
Haab st riae, 134–135 cau sed by, 180
Haem ophilus in uen zae conju n ct ivit is, 105 Hyperbaric oxygen , for cent ral ret inal ar ter y
Ham artom a, com bin ed, of ret in al p igm en t occlu sion , 268
ep ith eliu m an d ret in a, 333, 333f Hyperlipoprotein em ia, 173, 175
differen t ial d iagn osis, 327, 328, 330, 331, Hyperlysinem ia, and len s sublu xat ion , 13
333 Hyperten sion, 267
Han d an om alies, 353 an d angioid st reaks, 270
Hassall-Hen le bodies, 167 an d cen t ral serou s ch orioret in opathy, 273
Hearing loss, 353 an d hyp h em a, 15
Helm in th in fect ion , 150 an d m acroan eu r ysm , 269
Hem angiom a. See also Cap illar y h em angiom a; Hyp erten sive ret in opathy, 260–261, 260f
Cavern ous h em angiom a differen t ial d iagn osis, 263, 264, 286
ch oroidal, 327, 328, 329 Hyperthyroidism , 355–356. See also Graves’
exophyt ic ret in al, 329 disease; Graves oph th alm opathy
racem ose, 335, 336, 337 Hyphem a
Hem op h ilia, an d hyp h em a, 15 differen t ial d iagn osis, 13
Hem orrh age grad ing system , 15, 15f
opt ic n er ve sh eath , 26 post t rau m at ic, 10f
orbital, 26 sp on tan eou s, cau ses, 15
ret robu lbar, 29–30, 30f traum at ic, 14–16, 15f
ru pt u red globe an d , 16 in uveit is-glau com a-hyp h em a syn drom e,
su bconju n ct ival, 3–4, 4f 196
su bret in al, 327, 328, 330, 331 Hypoparathyroidism , 192
vit reou s. See Vitreou s h em orrh age Hypopyon, in fu ngal in fect ion , 149, 149f
Hem osiderosis bu lbi, vit reou s h em orrh age Hypotony, after cataract su rger y, 237–238
an d, 19
Hep atolen t icu lar degen erat ion , 180 I
Herp es sim p lex Ich thyosis, 33
ch orioret in it is, 201 differen t ial d iagn osis, 166, 191
keratit is, 150, 151f, 167, 192 In born errors of m etabolism , corn eal
keratoconju n ctivit is, 104–105, 104f involvem en t in , 192
Herp es sim p lex viru s In clusion conjun ct ivitis, 192
acu te ret in al n ecrosis syn drom e cau sed In dom eth acin , corneal vert icillata caused
by, 209 by, 180
infect ion In fan t ile esot ropia, 340–341, 340f
corn eal, 141–143, 141f In fan t ile exot ropia, 344
differen t ial d iagn osis, 142, 147 In fect ion(s)
in it ial, 141 bacterial, eyelid, 69
recu rren ce, 142 an d conju n ct ivalizat ion of corn ea, 136
differen t ial d iagn osis, 101, 143, 191 corn eal, 141–150
an d in terstit ial kerat it is, 150, 151f differen t ial d iagn osis, 84, 192
an d recu rren t corn eal erosion , 156 orbital, 69–99
skin lesion s in , 104f In fect ious keratit is, 182, 183
an d n eurot ropic keratop athy, 155 In fect ious m onon ucleosis, 145
Herp es zoster. See also Varicella-zoster viru s w ith eye involvem en t , differen t ial
infect ion , 101, 191 diagn osis, 101
corn eal, 143–145, 144f, 150 In ferior obliqu e m u scle, palsy, isolated, 348
differen t ial d iagn osis, 147 In ferior oblique overact ion , 345–346, 346f
kerat it is, 192 In flam m at ion
an d n eurot ropic keratop athy, 143, 144f, 155 ch ron ic, an d len s su blu xat ion , 13
Herp es zoster op h th alm icu s, 143–145, 144f corn eal, 150–153
Index 547
differen t ial d iagn osis, 192 Juven ile rh eum atoid arthrit is
d ifferen t ial d iagn osis, 135 ocu lar involvem en t in , 196–197
in traocu lar, 194–217 pau ciar t icu lar on set , 197
lacrim al glan d, 97 polyar t icu lar on set , 197
orbital, 71–80 system ic on set , 197
differen t ial d iagn osis, 73t t ype 1, 197
an d sym bleph aron , 116 t ype 2, 197
In flam m ator y bow el d isease (IBD), ocu lar uveit is in , 196–197
involvem en t in , 195 Juven ile xan th ogran ulom a, 135, 216t, 217
In sect bite, differen t ial diagn osis, 69, 72 differen t ial d iagn osis, 323
In term it ten t exot ropia, 342–343, 343f an d hyp h em a, 15
basic t ype, 343 iris lesion s, 323–324
convergen ce insu fficien cy, 343
d ivergen ce excess, 343 K
In tern al lim it ing m em bran e, rem oval, “ap ple Kap osi sarcom a, w ith secon dar y h em orrh age,
peeling” tech n iqu e, 318, 319f 3
In terst it ial kerat it is, 150–151, 151f Kap p a angle, 339, 339f, 340
In t raarterial fibrin olysis, for cen t ral ret in al Kasabach -Merrit t syndrom e, 84
arter y occlu sion , 268 Kayser-Fleischer ring, 24, 180
In traocu lar len s Kearn s-Sayre syn drom e, an d ret in it is
d islocated, tem p orar y h apt ic p igm en tosa, 285
extern alizat ion , 233–234, 234f Keloid, corn eal, 175–176, 176f
foldable, im p lan t at ion , 227, 229f d ifferen tial d iagn osis, 177
glued , 229–233, 230f–233f Kerat it is
opacificat ion , 242–243, 242f Acantham oeba, 147–149, 148f
torn , 236, 236f d ifferen tial d iagn osis, 146, 149, 192
In traocu lar p ressu re, elevated , after cataract Aspergillus, 149, 150
su rger y, 238 bacterial, 9, 143, 147, 149
IOL. See In t raocu lar len s Candida, 149, 149f
Irid ocorn eal en doth elial syn drom e, 258 con t act len s clean ing solu t ion s an d , 147,
d ifferen t ial d iagn osis, 169, 323 149
Irid ocyclit is con t act len s over w ear, 152, 154
Fuch s h eteroch rom ic, 197 d en drit ic in t raepithelial
h erpet ic, 104–105 d ifferen t ial d iagn osis, 142
Irid od ialysis, p ost t rau m at ic, 12f in HSV in fect ion , 141, 141f, 142
Irid od on esis, 13 d iffu se corn eoscleral, 190
Iris d im m er, 101
at rop hy d isciform , h erp et ic, 104–105
in HSV kerat it is, 141f exp osu re, 183
progressive, 258 filam en tar y, 154–155, 154f
cavern ou s h em angiom a, an d hyp h em a, 15 Fu ch s su p erficial m argin al, 184
cyst , 323 fu ngal, 149–150, 149f
freckle, d ifferen t ial diagn osis, 322 d ifferen t ial d iagn osis, 9, 143, 146, 147
juvenile xan thogran ulom a an d, 323–324 Fusarium , 149, 150
leukem ic n odu les, 324 h erpes sim plex, 150, 151f, 167, 192
m elanom a, 323 h erpes zoster, 192
differen t ial d iagn osis, 322 h erpet ic
m et astat ic lesion s, differen t ial diagnosis, d en d rit ic, 141, 141f, 142
324 in t raep ith elial, 141f, 142
n evus, 322 st rom al, 141f, 142
p igm en t epith elial cyst , 322–323 im m u n e st rom al, in HSV in fect ion , 141,
t u m ors an d n odu les, 322–325 141f, 142–143
Iris bom bé, in sarcoidosis, 198 in fect iou s, 182, 183
Iris sph in cter tear, 11, 11f in terst it ial, 150–151, 151f
Irit is h erpet ic, 104–105
d ifferen t ial d iagn osis, 3 n ecrotizing st rom al
in Steven s-Joh n son syn drom e, 113 in HSV in fect ion , 142
syphilit ic, 198 in HZV in fect ion , 144f
t raum at ic, 10–11, 10f n eurot ropic, in HSV in fect ion , 142, 143
Irit is n odosa, in syph ilis, 198 n um m ular, 143, 145
Irit is papu losa, in syph ilis, 198 Paecilom yces, 149, 150
Irit is roseat a, in syp h ilis, 198 p un ctate, in viral in fect ion , 141–142, 143,
Iron dep osit s, 179 145
conju n ct ival, differen t ial diagn osis, 129, 130 st ap hylococcal m argin al, 146
Iron lin es, 179–180, 179f st riate, after cat aract ext ract ion , 237f
Ir vin e-Gass syn drom e, 274, 275 st rom al
Isch em ic opt ic n eu ropathy, differen t ial d ifferen tial d iagn osis, 142, 145
d iagn osis, 261, 267 in EBV in fect ion , 145, 145f
su p erficial pu n ct ate, w ith ch em ical bu rn , 5
J su perior lim bic, 45
Jacob-Agar w al sling surger y, 37f, 38–41, Thygeson superficial pun ctate, 152, 152f
39f–41f u lcerat ive
Jellin ek sign , 76t d ifferen tial d iagn osis, 6, 182, 184
Juven ile ret in osch isis p erip h eral, 182–184, 183f, 184
an d cystoid m acu lar edem a, 274 u lt raviolet (UV), 6
d ifferen t ial d iagn osis, 275 viral, 149, 150
548 Index
Keratoacan th om a, 53–54, 53f w ith broken capsule an d in flam m at ion ,

differen t ial d iagn osis, 59, 62, 64 14
Keratoconju n ct ivit is in to vit reou s, 14
atop ic, 110 su blu xat ion , 13–14, 14f
differen t ial d iagn osis, 45, 112, 153 asym ptom at ic, 14
ep idem ic, 101–103 w ith cataract , 14
differen t ial d iagn osis, 105, 152 w ith h igh un correct able ast igm at ism , 14
ep idem ic h em orrh agic, 103–104, 103f w ith m on ocular diplopia, 14
h erp es sim p lex, 104–105, 104f Len t igo m align a, 63f, 64
ph lycten u lar, 192 Lep rosy
su perior lim bic, 122–123, 122f differen t ial d iagn osis, 150, 191, 192
differen t ial d iagn osis, 63, 192 an d n eu rot ropic keratop athy, 155
an d filam en tar y kerat it is, 154 Leu kem ia
vern al, 110, 146 differen t ial d iagn osis, 78, 94, 323
sh ield u lcer in , 152–153, 153f an d hyp h em a, 15
Keratoconju n ct ivit is sicca, 192 in t raocu lar, 216, 216t
Keratocon u s, 170–171, 171f iris n odu les in , 324
differen t ial d iagn osis, 172, 191 orbital involvem en t in , 96f
an d ret in it is pigm en tosa, 285 Leu kocorn ea, 192–193
Keratoglobu s, 171–172, 172f Levator m u scle
Keratopathy fu n ct ion , m easu rem en t , 36
ban d, 176–177, 177f palsy, isolated, 348
bullous Lim bal follicles, 118
ap h akic, 167, 169, 192 Lim bal girdle of Vogt , 174, 174f
differen t ial d iagn osis, 167, 169, 192 Lipid degen erat ion of corn ea an d conjun ctiva,
pseu dop h akic, 167, 169, 192 120–121, 121f
calcific ban d, 176–177, 177f Lipid s, seru m , abn orm alit ies. See also
exposure, 153–154 Hyperlipoprotein em ia
n europaralyt ic, 153–154 an d m acroan eu r ysm , 269
n eurot ropic, 143, 144f, 155–156, 155f Lipoprotein em ia, 164
in HZV in fect ion , 143, 144f, 155 Lisch n odules
prim ar y lipid , 175, 175f differen t ial d iagn osis, 322
Keratoplast y in n eu rofibrom atosis, 325
Descem et st rip p ing en d oth elial, 187, 187f Low e syn d rom e, 135, 175
graft reject ion in , 188, 188f, 192 corn eal involvem en t in , 192
lam ellar, 186f, 187 Low ey sign , 76t
pen et rat ing, 185, 186f Lym e d isease, an d in terst it ial kerat it is, 150,
graft reject ion in , 188, 188f 151
Keratop rosth esis, 188, 189f Lym ph angiom a, 85–87, 85t, 86f
Kh odadou st lin e, 188, 188f differen t ial d iagn osis, 84, 93
Kidn ey, m align an cy of, orbital m etast ases, 94 Lym ph ogran u lom a ven ereu m (LGV), 106
Klipp el-Feil syn drom e, 353 Lym ph oid hyp erp lasia, 133
Kn ies’ sign , 76t Lym ph om a(s)
Koch er sign , 76t differen t ial d iagn osis, 59, 90
in t raocu lar, 216, 216t
L orbital, 79–80, 79f
Lacerat ion (s)
can alicu lar, 2, 3f M
conju n ct ival, 4–5, 5f Macroaneur ysm , 269–270
corn eal, 9–10, 9f differen t ial d iagn osis, 263
eyelid, 1–2, 1f, 3f Macular degen erat ion
Lacrim al glan d(s) age-related. See Age-related m acular
accessor y, 121 degen erat ion
aden oid cyst ic carcin om a, 98, 99f differen t ial d iagn osis, 280
en largem en t , 97–99 juven ile, 278
in flam m at ion , 97 Macu lar dyst rop hy, 163, 163f
t um ors, 97–98 differen t ial d iagn osis, 161
ben ign m ixed, 97–98, 97f vitelliform , 280–281
Lacrim al sac t u m or, 68 Macular edem a
Lacrim al system clin ically sign ifican t , 261, 262f
disord ers, 65–68 diabet ic, 283
traum a, 2, 3f differen t ial d iagn osis, 283
Lagop h th alm ia, 154 Macular h em orrhage, 267
Lam ellar keratop last y, 186f, 187 Macular h ole, 267, 296, 317–318, 317f, 318f
Lateral rect us m u scle palsy, 350–351, 351f differen t ial d iagn osis, 274, 318
Lat tice corn eal dyst rop hy, 162–163, 162f idiopath ic, 23
differen t ial d iagn osis, 178 m an agem en t
Lat tice degen erat ion , 292–293 by obser vat ion , 318
Leber congen ital hereditar y opt ic n eu rop athy, su rgical, 318, 319f
an d keratocon u s, 171 st ages, Gass classificat ion, 318
Leiom yom a traum at ic, 22–23, 23f
differen t ial d iagn osis, 323, 325 Macular ret in al vein occlusion, 265f
uveal, 324 Maculopathy
Len s age-related. See Age-related m acu lopathy
dislocat ion , 13–14 diabet ic, 261, 262f
asym ptom at ic, 14 an d cystoid m acu lar edem a, 274
Index 549
differen t ial d iagn osis, 263, 269 ch oroidal, 329–330, 329f

Madarosis d ifferen tial d iagn osis, 327–331
w ith bleph arit is, 100 ciliar y body, 325
w ith m align an cy, 59 conju n ct ival, 133
Maffu cci syn drom e, 84 d ifferen tial d iagn osis, 80, 324
Malign an t m elan om a. See Melan om a of iris, 324
Man dibu lofacial dysostosis. See Fran cesch et t i orbital, 94, 96f
syn d rom e MEW DS. See Mult iple evan escen t w h ite dot
Man n is lin e, 179 syn d rom e
Map-d ot-fingerprin t corn eal dyst rop hy, Microan eu r ysm s, 261
158–159, 159f Microcorn ea, 157
Marcu s- Gu n n jaw -w in king syn drom e, 38 Microhyp h em a, 14–16
Marfan syn d rom e, 135 t raum at ic, 10
blue sclera in , 134 Microp an n u s, 192
and d egen erat ive m yop ia, 272 Microp h akon it, 218f, 219
an d keratocon u s, 171 Microp h th alm os, 50
and len s su blu xat ion , 13, 14 d ifferen tial d iagn osis, 157, 158
Margin to lid reflex, 36 Migrain e, 295
Masqu erade syn d rom es, 216–217, 216t Möbiu s sign , 76t
Medial rect us m uscle palsy, isolated , 348, 348f Mollu scu m con tagiosu m , 54, 54f
Medu lloep ith eliom a d ifferen tial d iagn osis, 51, 192
of ciliar y ep ith eliu m , 326 Mon ocu lar elevat ion deficien cy, 354–355,
d ifferen tial d iagn osis, 323, 325, 326 354f
Meesm an n dyst rop hy, 160, 160f Mooren u lcer, 180–182, 182f
d ifferen tial d iagn osis, 159, 161 d ifferen tial d iagn osis, 183
Megalocorn ea, 157 MRD. See Margin to lid reflex
Meibom ian glan d dysfu n ct ion , p em p h igoid Mu cocele, 93–94, 95f
an d , 112 d ifferen tial d iagn osis, 65, 68, 81
Meibom it is, w ith blep h arit is, 100 eth m oidal, 65, 68
Melan ocytom a(s), 338, 338f Mu colip idosis, corn eal involvem en t in , 192
choroidal, 332 Mu cop olysacch arid osis, corn eal involvem en t
Melan ocytosis in , 166, 170, 192
ocu lar, 129–130, 129f, 324–325 Mu corm ycosis, 128
differen tial d iagn osis, 322 Mu lt iple en d ocrin e n eoplasia, t yp e IIb, 191
ocu lod erm al, 130, 130f Mu lt ip le evan escen t w h ite d ot syn drom e,
differen tial d iagn osis, 129 203–204, 203f–204f
Melan om a Mum ps, corn eal involvem en t in, 150
am elan otic Myasth en ia gravis, 356–357, 356f
ch oroidal, 329 Myopia
differen tial d iagn osis, 324, 329 degen erat ive, 272, 272f
choroidal, 132, 327–329, 328f ocu lar associat ion s, 272
am elan ot ic, 329 system ic associat ion s, 272
differen tial d iagn osis, 216, 327–333, 338 h igh , 272, 272f
m an agem en t , 329 “blu e sclera” in , 134
presen tat ion , 327–329, 328f differen t ial d iagn osis, 21, 286
vit reou s h em orrh age in , 18 an d len s su blu xat ion , 13
ciliar y body, 325–326 an d t rau m at ic ret in al detach m en t , 21
differen tial d iagn osis, 322 an d ret in al d etach m en t , 296
vit reou s h em orrh age in , 18 an d ret in it is pigm en tosa, 285
conju n ct ival, 132–133, 132f Myopic sh ift , in in fan ts, vit reou s h em orrh age
differen tial d iagn osis, 131 an d, 19
cutan eou s, 63–64, 63f Myosit is, 78
ret in op athy secon dar y to, 217 Myoton ic dyst rop hy, 281, 285
d ifferen tial d iagn osis, 52, 56, 59, 129, 130 Myoton ic ret inop athy, 282
d ilated episcleral sen t in el vessels in , 125
of iris, 323 N
n odular, 64 Nanoph th alm os, 157
orbital m etast ases, 94 Naproxen , corn eal vert icillata caused by, 180
p ingu ecu la an d , 118f Nasolacrim al duct obst ruct ion, 67–68, 67f
sup erficial spreading, 64 Neoplasm (s), 322–338. See also Tum or(s)
su r veillan ce, w ith m elan ocytosis, 325 conju n ct ival, w ith secon dar y h em orrh age, 3
Melan osis Neovascu larizat ion
conju n ct ival ben ign ep ith elial, 131–132, ch oroidal
131f an d cystoid m acu lar edem a, 274
d ifferen tial d iagn osis, 133 differen t ial d iagn osis, 21, 275
p rim ar y acqu ired corn eal, in Steven s-Joh n son syn d rom e,
differen tial d iagn osis, 129, 130 113
m align an t poten t ial, 132 in diabet ic ret in op athy, 261
Melan osis bu lbi, 140 differen t ial d iagn osis, 263
Men ingiom a, 81 in ocu lar isch em ic syn drom e, 268, 269f
Men kes syn drom e, blu e sclera in , 134 Neovascu lar m em bran es, su bret in al, 21
Meridion al com p lex, 289 Ner ve fiber layer, m yelin ated, 266
Meridion al folds, 289, 289f Neu rilem m om a, 81
Metabolic d isease, 135 Neu roblastom a
Metallosis bu lbi, 23 differen t ial d iagn osis, 84
Metastat ic d isease m et astat ic, 87, 94
550 Index
Neu rofibrom a, 58–59, 59f Orbital h em orrh age, 26

plexiform , 86 Orbital in flam m ator y pseu dot u m or, 77–78,
Neu rofibrom atosis, 135 78f, 97
differen t ial d iagn osis, 191 Orbital varices, 85t, 93
Lisch n odules in , 325 Osteitis deform an s, an d angioid st reaks, 270
Neu roparalyt ic keratop athy, 153–154 Osteogen esis im p erfect a
Neu roret in it is blue sclera in , 133–134, 134f
differen t ial d iagn osis, 263 d ifferen tial d iagn osis, 191
syp h ilit ic, 198 Osteom a
Neu rot ropic keratopathy, 143, 144f, 155–156, ch oroidal, 327–332, 331f
155f d ifferen tial d iagn osis, 94, 327–331
Nevu s (pl., n evi), 54–56, 55f Osteom yelit is, of p aran asal sin u ses, 69, 72
ch oroidal, 326–327, 327f
differen t ial d iagn osis, 328, 330, 331, 332 P
com p ou n d, 54–56, 55f, 131, 132 Paecilom yces kerat it is, 149, 150
differen t ial d iagn osis, 52, 59, 64, 129, 130, Paget disease, an d angioid st reaks, 270, 271
133, 323 Palpebral fissure, 36
int raderm al, 54–56, 55f in blep h aroph im osis syn drom e, 48, 48f
of iris, 322 Pan n us, 191–192, 192f
jun ct ional, 54–56, 55f, 131, 132 d ifferen tial d iagn osis, 118
m align an t , sign s, 132, 133 Pan uveitis, 269
m align an t poten t ial, 131, 132 Papillae, 110
st raw berr y, 81 Papilledem a, 264, 335, 336, 337
Nevu s flam m eu s, 84 Papillit is, 264, 335, 336, 337
Nevu s of Ot a, 56, 56f, 324–325 Papillom a
differen t ial d iagn osis, 129 d ifferen tial d iagn osis, 54, 133
Nicolas-Favre disease, 106 eyelid, 51, 51f
Nicot inic acid, an d cystoid m acular edem a, p ostsurgical, 119f
274 Paracentesis, an terior ch am ber, 268
Nigh t blin dn ess, congen it al st at ion ar y, 280 Parafoveal telangiectasia
differen t ial d iagn osis, 285 d ifferen tial d iagn osis, 263, 269, 283
Nit ric acid burn, 5–7 id iopath ic, 282–283, 283f
Nod u le(s), of iris, 322–325 m an agem ent , 283
Nonproliferat ive diabet ic ret in op athy. See p resen tat ion, 282
Diabet ic ret inopathy, n onproliferat ive Paran eoplast ic syn drom e, 217
Non steroidal an t i-in flam m ator y drugs Parasit ic in fest at ion , conjun ct ival cysts in ,
(NSAIDs), an d su bconju n ct ival 121, 122
h em orrh age, 3–4 Parat rachom a, 106, 107t
NPDR. See Diabet ic ret in op athy, Parin aud syn drom e, eyelid ret ract ion in, 43
n onp roliferat ive Pars plan a, 296
breaks, 296
O Pars plan a cyst , 290
OAG. See Glau com a, open -angle Pars plan a pearls, 290
Ocu lar isch em ic syn drom e, 268–269, 269f Pars plan it is, 200
differen t ial d iagn osis, 263, 264, 269 d ifferen tial d iagn osis, 326
Ocu lar m assage, 268 Par t ially accom m odat ive esot ropia, 342
Ocu lar vein varicosit ies, 125–126 Patau syn drom e, corn eal involvem en t in , 192
Ocu loau ricu lovertebral dysp lasia. See Pat tern dyst rophy
Golden h ar syndrom e d ifferen tial d iagn osis, 279, 280, 281
Oculocerebroren al syndrom e, 135, 175 gen et ics, 281
Oculogen it al syndrom e, 107t–108t m an agem ent , 281
OIP. See Orbital in flam m ator y pseudot u m or p resen tat ion , 281
On chocerciasis, 150 Pat tern st rabism us, 344–345, 345f
Open-angle glaucom a. See Glaucom a, open - A pat tern , 344–345, 345f
angle V p at tern , 344–345, 345f
Oph th alm ic arter y occlusion , 266–268 Pavingstone degen erat ion , 290–291, 291f
Opt ic disk d ifferen tial d iagn osis, 286
capillar y h em angiom a, 334, 335f PDR. See Diabet ic ret in opathy, proliferative
m elanocytom a, 338, 338f Pellagra, 192
Opt ic n er ve Pellu cid m argin al degenerat ion (cornea),
avu lsion , 26 172–173, 173f
gliom a, 81, 86 at yp ical, 184
t ran sect ion , 26 d ifferen tial d iagn osis, 171, 184
Opt ic n er ve sheath, h em orrh age, 26 Pem ph igoid
Opt ic n europathy, trau m at ic, 25–26, 25f cicat ricial, 63, 192
Ora serrata, 296 d ifferen tial d iagn osis, 63, 110, 113, 192
Orbit ocu lar, an d d ist ich iasis, 64
foreign body in , 28–29, 29f ocu lar cicat ricial, 111–113, 112f
fract u res, 26–28 an d sym bleph aron , 116
in fect ion s, 69–71 Pem ph igus, ocular, 191
in flam m at ion , 71–78 Penet rat ing keratoplast y, 185, 186f
differen tial d iagn osis, 73t graft reject ion in, 188, 188f
n eoplasm s, 79–94 Perioptic n eurit is, 78
t raum a, 26–32 Persisten t hyperplastic prim ar y vit reous, 326
Orbital balloon , tem p orar y, 303 Peters an om aly, 135, 190
Orbital em physem a, 26 PEX. See Pseudoexfoliat ion syn drom e
Index 551
Ph acoan aphylact ic uveit is, 12 differen t ial d iagn osis, 280

Ph acod on esis, 13 Pseu d ogeron toxin , 173
Ph aco m ach in e Pseudom onas aeruginosa, corn eal u lcer cau sed
an d con n ection of air p u m p to in fu sion by, 146
bot tle, 226f Pseu d oph akia, an d retin al detach m en t , 297
prin ciples, 223, 223f Pseu d opter ygiu m , 120
su rge p h en om en on Pseu doptosis, 38
m ech an ism of, 224f w ith m on ocu lar elevat ion deficien cy,
preven tion , 225f 354–355, 354f
Ph acom atoses, 135 Pseu dostrabism u s, 339–340, 339f
Ph ar yngoconju n ct ival fever, 101–103 Pseu d ot rach om a, 112
Ph en oth iazin es, corn eal vert icillata cau sed Pseu d ot u m or, orbital, 73t
by, 180 differen t ial d iagn osis, 76, 80, 86, 87, 93,
Ph en oth iazin e toxicit y, 285 94, 128
Ph lycten u lar keratoconju n ct ivitis, 192 in flam m ator y, 77–78, 78f, 97
Ph lycten u le, 118 Pseu d ovascu larit y, 333
Ph lycten u losis, 177 Pseu doxan th om a elast icu m
Ph otocoagu lat ion an d angioid st reaks, 270, 271
an d recu rren t corn eal erosion , 156 d ifferen t ial d iagn osis, 21
for ret in al detach m en t , 302 Psoriatic arth rit is, ocu lar involvem en t in ,
Ph otoph obia, in congen ital glau com a, 195
134–135 Psych op h arm acological m ed ication s, an d
Ph ototoxic inju r y, 275 cen t ral serous ch orioret in opathy, 273
PHPV. See Persisten t hyp erplast ic p rim ar y Pter ygium (pl., pter ygia), 118–120, 119f
vit reou s d ifferen t ial d iagn osis, 118, 133
Ph th isis bu lbi, 136, 191 Ptosis, 36–41
Picorn aviru s conju n ct ivit is, 101, 103–104, ap on eu rot ic, 37f, 38
103f m an agem en t, 38
Pigm en t at ion s, conju n ct ival, 131–132, 131f bilateral, 37f
causes, 131 in bleph arop h im osis syn drom e, 48, 48f
Pigm en ted dep osits, 129, 130, 133 congen it al, 36, 37f
Pillat scleral p laqu e, 140, 140f m an agem en t, 38
Pingu ecu la, 117–118, 117f, 118f con t ralateral, w ith ip silateral lid ret ract ion ,
differen t ial d iagn osis, 120 43
Plasm in ogen act ivator in h ibitor 1, an d cen t ral d erm atoch alasis an d, 42–43, 42f
serous ch orioret in op athy, 274 m echanical, 38, 59, 59f
Pleom orp h ic aden om a, lacrim al glan d , 97–98, w ith m on ocu lar elevat ion deficien cy,
97f 354–355, 354f
Pn eum at ic ret in opexy, 303 m yogen ic, 38
Poch in sign , 76t n eurogenic, 38
POHS. See Presum ed ocular h istoplasm osis overcorrect ion , 43
syn drom e p resen tat ion , 36–38
Polyarterit is nodosa, sclerit is in , 124 Pu p illar y block, w ith len s su blu xat ion , 14
Polym orph ic am yloid degen erat ion , 178, 179f Pup illar y sp h in cter, p alsy, isolated, 348
Por t-w in e stain, 126, 127f PVD. See Posterior vit reous det ach m en t
Posn er-Sch lossm an syn drom e, 196 Pykn odysostosis, blu e sclera in , 134
Posterior am orp h ou s st rom al dyst rop hy, 166 Pyocele, 93
Posterior em br yotoxon , 157, 158 Pyogen ic gran u lom a, 45f
Posterior sclerit is, 78, 124, 125
differen t ial d iagn osis, 329 Q
Posterior vit reous detach m ent , and vit reous Qu in in e toxicit y, 267
h em orrh age, 270
Pot assium hydroxide burn s, 5–7 R
Pre-Descem et dystrophy, 165–166 Racial pigm en tat ion, 325
Pregn an cy, an d cen t ral serou s Radiat ion exposure, 112, 113
ch orioretin op athy, 273 Radiat ion ret in opathy, 286–287
Preser vatives, top ical, an d n eu rot ropic d ifferen t ial d iagn osis, 261, 263, 264, 286
keratopathy, 155 p resen tat ion , 286
Presu m ed ocu lar h istop lasm osis syn drom e, Radiat ion th erapy
202–203, 202f an d conju n ct ivalizat ion of corn ea, 136,
Prim ar y lip id keratopathy, 175, 175f 136f
Progeria, blu e sclera in , 134 an d n eu rot rop ic keratopathy, 155
Proliferat ive d iabet ic ret in op athy. See Diabet ic RCE. See Recurren t corn eal erosion
ret in op athy, p roliferat ive React ive lym ph oid hyp erplasia, 80
Proliferat ive ret in op athy, vit reou s h em orrh age Recu rren t corn eal erosion , 156
an d, 18, 19 Red eye
Propionibacterium acnes, p ostop erat ive causes, 3, 126
en doph th alm it is cau sed by, 214, 241 d ifferen t ial d iagn osis, 126
Proptosis Refractive accom m odat ive esot ropia, 341f,
in Graves oph th alm opathy, 355, 355f 342
in thyroid eye d isease, 43, 43f, 355, 355f Refsu m disease
Prost aglan din (s), top ical, 64 d ifferen t ial d iagn osis, 191
Prost ate can cer, orbital m etastases, 94 an d ret in it is pigm en tosa, 285
Proteus, corn eal u lcer caused by, 146 Reis-Bü cklers dyst rop hy, 160–161, 161f
Pseu d oesot ropia, 49, 339f d ifferen t ial d iagn osis, 159
Pseu d oexfoliat ion syn drom e, 249–250, 250f an d recu rren t corn eal erosion , 156
552 Index
Reiter syn d rom e, ocu lar involvem en t in , hyperplasia, differen tial diagn osis, 327, 328,
194–195 330, 331, 332
ReNu w ith Moist u reLoc, 149 in farct ion s, 260–261
Ret icu lar d egen erat ive ret in osch isis, 294–295 p osth em orrh age h em osid erin dep osits,
Ret in a d ifferen tial d iagn osis, 332
con t u sion , 266 t u m ors, 332–333
cystoid degen erat ion , 288–289 Ret in al tears, 296–305
ret icu lar, 288 flap , 297, 297f
t yp ical, 288–289 h orsesh oe, 297, 297f
degen erat ion s, 288–291 Ret in al telangiectasis
develop m en t al an om alies, 288–291 an d cystoid m acu lar edem a, 274
edem a, p ost t rau m atic, 21–22 differen t ial d iagn osis, 275
en closed oral bay, 289, 289f Ret in al t u fts, 293–294
equ ator, 296 cyst ic, 293
exophyt ic capillar y h em angiom a, n on cyst ic, 293–294
differen t ial d iagn osis, 329 zon ular t raction , 294
flecked, 280, 280f. See also Fu n du s Ret in al vein occlu sion , an d cystoid m acu lar
flavim aculat us; Stargardt disease edem a, 274
h em orrh ages, post t rau m at ic, 21–22 Ret in it is
m ed ical, 260–287 acu te, d ifferen t ial diagn osis, 264
m eridion al com p lex, 289 cytom egaloviru s, 208, 208f
m eridion al fold s, 289, 289f differen t ial d iagn osis, 266
n ecrosis Ret in it is p igm en tosa, 284–285, 284f
acu te, d ifferen t ial diagn osis, 21 an d cystoid m acu lar edem a, 274
post t rau m at ic, 21–22 differen t ial d iagn osis, 285
su rgical, 288–321 m an agem en t, 285
tears, post t raum at ic, 21–22 ocu lar associat ion s, 285
t um ors, 333–337 presen tat ion , 284–285
Ret in al breaks system ic associat ion s, 285
acu te p resen tat ion , 298 Ret in it is pu n ct a albescen s, 279, 280
id en t ificat ion , 300–301, 300f Ret in oblastom a, 216t, 333–334
localizat ion , 300–301, 300f, 305–306, 306f associated m align an cies, 334
op ercu lated , 297f, 298 differen t ial d iagn osis, 201, 216, 323, 324,
t ypes, 297–298, 297f 326, 333, 335, 336, 337
Ret in al cap illar y h em angiom a, diabet ic, 269 ep idem iology, 333
Ret in al det ach m en t , 296–305 gen et ics, 333–334
acu te p resen tat ion , 298 m an agem en t , 334
after cataract su rger y, 239–240, 240f presen t at ion , 334
ap h akic, 239–240, 240f vit reou s h em orrh age w ith , 18
causes, 21 Retin op athy
cen t rifugal forces an d, 296 cen t ral serou s, 263
differen t ial d iagn osis, 19, 274, 295, t um or-associated, 217
298–299 Retin op athy of p rem at u rit y, an d degen erat ive
exu dat ive m yop ia, 272
ch aracterist ics, 299t Retin op exy, pn eu m at ic, 305
differen t ial d iagn osis, 285, 298, 299t Retin osch isis
localized serou s. See Cen t ral serou s differen t ial d iagn osis, 298–299, 299f
ch orioret in op athy ret icu lar degen erat ive, 294–295
m an agem en t, 300–305 sen ile, 294–295
ou t p at ien t tech n iqu es, 303–305, 303f, Rh abd om yosarcom a, 87–89, 88f–89f
304f, 305f differen t ial d iagn osis, 59, 78, 94
su rgical failu re, 303 Rh egm atogen ou s ret in al det ach m en t ,
path ology, color-coded diagram , 306, 239–240, 296
307f ch aracterist ics, 299t
post trau m at ic, 21–22, 22f differen t ial d iagn osis, 298, 299t
rh egm atogen ou s, 239–240, 296 ep idem iology, 296
ch aracterist ics, 299t m an agem en t , 300–301, 301f
differen tial d iagn osis, 298, 299t presen t at ion , 296–298
epidem iology, 296 risk factors for, 296–297
m an agem ent , 300–301, 301f Rh eu m atoid arth rit is
presen tat ion , 296–298 ep iscleritis in , 123
risk factors for, 296–297 perip h eral u lcerat ive kerat it is in , 182–184,
t ract ion al 183f
ch aracterist ics, 299t sclerit is in , 124
differen tial d iagn osis, 298, 299t Rh exis
in proliferat ive ret in opathy, 261, 263f in m at u re cat aract , 222–223, 223f–226f
Ret in al dialysis, 297f, 298 in sen ile cataract , 226–227, 226f–229f
an d ret in al detach m en t , 21, 22f Rieger an om aly syn drom e, 135
Ret in al pigm en t degen erat ion , secon dar y, in Riesm an sign , 76t
syph ilis, 198 Rosacea
Ret in al pigm en t ep ith eliu m differen t ial d iagn osis, 182, 183
congen it al hypert rop hy, 332, 332f ocu lar, 100–101, 100f
differen tial d iagn osis, 327, 328, 330, RPE. See Ret in al p igm en t ep ith eliu m
331, 332 RP inversu s, differen t ial diagn osis, 279, 280
h em orrh agic det ach m en t , differen t ial RRD. See Rh egm atogen ou s ret in al
diagn osis, 329 det ach m en t
Index 553
Rubella, 135 Sen sor y exot rop ia, 344

congen it al, 285 Serp igin ou s ch oroidit is, 205–206, 206f
Rubella ret in opathy, 281, 282 Serrat ia corn eal u lcer, 146
Rubeola, corn eal involvem en t in , 150 Seton im plan t at ion , 252, 253f
Rubeosis iridis Sh afer sign , 297
an d hyp h em a, 15 Sh aken baby syn drom e, 18
vit reou s h em orrh age an d, 18 Sh ield ulcer, 146, 152–153, 153f
Sh ingles, 143
S Sickle cell an em ia, an d angioid st reaks, 270,
Salzm an n n od u lar degen erat ion , 177–178, 271
177f Sickle cell ret in op athy, 283–284
Sarcoid differen t ial d iagn osis, 263, 286, 335, 336,
at ypical lym p h oid in filt rate, 57 337
differen t ial d iagn osis, 57, 323 m an agem en t , 284
Sarcoidosis, 97 presen t at ion , 283
cardiovascular involvem en t in , 199 proliferat ive, an d vit reou s h em orrh age, 270
differen t ial d iagn osis, 80, 112, 150, 263 Siderosis, 23–24
ocu lar involvem en t in , 198–199 Siegrist st reaks, 261
pu lm on ar y involvem en t in , 199 Silden afil cit rate, an d cen t ral serou s
vit reou s h em orrh age w ith , 18 ch orioret in opathy, 273
Satellite lesion , in adu lt toxop lasm osis, 201 Sin u sit is, an d m u cocele, 93–94, 95f
Scarring Sixth-n er ve palsy, 350–351, 351f
ch orioret in al, 332 Sjögren syn drom e, differen t ial diagn osis, 112
corn eal Skin t ag. See Acroch ordon
cen t ral, 164 Sling surger y
in HSV kerat it is, 141f conven t ion al, 37f
from infect ion, 177 Jacob-Agar w al, 37f, 38–41, 39f–41f
in terst it ial kerat it is an d , 150, 151f Sn ap -back test , 33
in Steven s-Joh n son syn d rom e, 113 Sn ellen sign , 76t
st rom al, 192 Sodiu m hydroxid e bu rn s, 5–7
of lid m argin Solar ret in it is, 287
an d dist ich iasis, 64 Solar ret in opathy, 287
an d trich iasis, 64 Sp h eroidal corn eal d egen erat ion , 178
u pp er eyelid, 43 Sp h ingolip idoses, 267
Sch nyder corn eal dyst rophy. See Cen t ral SPK. See Superficial pun ct ate kerat it is
cr ystallin e dystrophy of Sch nyder Squ am ou s cell carcin om a, 61–62, 61f
Sch w an n om a, 90 conju n ct ival, 137
Sch w ar t z-Jam p el syn drom e, 217 cyst ic, 61f
Scleral bu ckling differen t ial d iagn osis, 51–54, 59, 64, 113,
en circling, 302, 302t 133, 137
proced u re, 305–316 n odu lar, 62
color-coded d iagram , 306, 307f plaqu elike, 62
drain age of su bret in al flu id, 314–315, u lcerat ing, 62
314f, 315f St ap hylococcal hyp ersen sit ivit y, 152
exoplan t m aterial, 308–310, 309f St ap hylococcal m argin al u lcer, 177, 182
localizat ion of ret in al breaks, 305–306, Staphylococcus
306f coagu lase-n egat ive, p ostoperat ive
parallax associated w ith view ing, 308, en doph th alm it is cau sed by, 241
308f corn eal u lcer cau sed by, 146, 177, 182
sh aft ing du ring localizat ion , 308, 309f trau m at ic en doph thalm it is caused by,
su t u re p lacem en t for silicon e exp lan t , 213
calculat ing distance of, 310f, Staphylococcus aureus
311–312, 311f–313f blep h arit is cau sed by, 106
su t u re t ying, an terior sh ift ing, 314, 314f h ordeolu m cau sed by, 46–47, 46f
su t u ring tech n iqu e, 310, 310f postop erat ive en doph th alm it is caused by,
for ret inal detachm en t , 302 214, 241
segm en t al, 302, 302t trau m at ic en doph thalm it is caused by, 213
Scleral ectasia, 140 Staphylococcus epiderm idis, postoperat ive
Sclerit is, 124–125, 125f en doph th alm it is cau sed by, 214
an terior, 124 St ap hylom a(s)
differen t ial d iagn osis, 124, 329 an terior, an terior segm en t t ran sp lan tat ion
n ecrot izing, 124 for, 189–191, 190f
posterior, 78, 124, 125, 329 congen it al, 272
Sclerocorn ea, 158 posterior, w ith degen erat ive m yop ia, 272
differen t ial d iagn osis, 157, 192 scleral, 134–135, 135f
Sclerom alacia, 140 differen t ial d iagn osis, 140
Search ligh t in fog, in adu lt toxop lasm osis, 201 St argardt disease, 278–279, 279f
Sebaceou s cell carcin om a, 62–63, 62f differen t ial d iagn osis, 279, 280
differen t ial d iagn osis, 46, 101, 133 m an agem en t, 279
Sebaceou s hydrocystom a, 57, 58f presen tat ion , 278, 279f
Seborrh eic blep h arit is, 100 Stellw ag sign , 76t
Seborrh eic keratosis, 52, 52f Steroid s
differen t ial d iagn osis, 53, 54, 64 exogen ou s, an d cen tral serous
Sen ile ret in osch isis, 294–295 ch orioretin opathy, 273, 274
differen t ial d iagn osis, 21 injection s, 267
Sen ile tapetoch oroidal degen erat ion , 291 an d su bconju n ct ival h em orrh age, 3
554 Index
Steven s-Joh n son syn drom e, 113 Tem porar y orbital balloon, 303
differen t ial d iagn osis, 136, 192 Tenon it is, 78
an d dist ich iasis, 64 Terrien m arginal degen erat ion , 119f, 184–185,
an d sym bleph aron , 116 184f
an d trich iasis, 64 differen t ial d iagn osis, 172, 183, 185
St ickler syn d rom e, an d degen erat ive m yop ia, Th erm al bu rn (s), 6, 112, 192
272 Th ird-n er ve p alsy, 347–349
Stocker lin e, 179 com p lete, 348, 348f
St rabism u s, 339–358 congen it al, 349
pat tern , 344–345, 345f differen t ial d iagn osis, 349
St raw berr y n evu s, 81 in ferior, 348, 348f
St reptococcus m an agem en t, 349
corn eal ulcer caused by, 146 pu p illar y involvem en t, 348, 349
grou p B, n eon at al en dogen ou s secon dar y, 349
en doph th alm it is cau sed by, 215 su perior bran ch , 348, 348f
postoperat ive end oph th alm it is cau sed by, Th rom bosis, cavern ou s sin u s, 71–73
214, 241 differen t ial d iagn osis, 69, 76, 78, 93, 128
traum at ic en doph th alm it is caused by, 213 presen tat ion , 71
St reptococcus pyogenes conju n ct ivit is, 105 Thygeson su perficial pu n ct ate kerat it is, 152,
St riate kerat itis, after cataract extract ion , 237f 152f
St riate m elan okeratosis, corn eal vert icillata Thyroid eye disease, 74–77, 74f–75f
in , 180, 181f differen t ial d iagn osis, 78, 93, 128
St ring of p earls app earan ce, 215 dilated vessels in , 126
St u rge-Weber syn d rom e, 135, 330 exoph th alm os in , 73t
differen t ial d iagn osis, 325 eyelid edem a in , 47, 47f
dilated episcleral vessels in , 126–127, 127f eyelid ret ract ion in, 43, 43f
Su bconju n ct ival h em orrh age, 3–4, 4f m an agem en t, 76–77
Su bretin al h em orrh age, 327, 328, 330, 331 proptosis in , 43, 43f
Su ker sign , 76t Thyroid-related op h th alm opathy, 74–77, 74f–
Su lfite oxidase deficien cy, an d len s 75f. See also Graves oph th alm opathy;
su blu xat ion , 13 Thyroid eye disease
Su lfu ric acid bu rn , 5–7 differen t ial d iagn osis, 78, 93
Su lfu rou s acid bu rn , 5–7 in filt rat ive (severe), 74
Su p erficial p u n ctate keratitis, w ith ch em ical m an agem en t , 76–77
burn , 5 n on in filt rat ive (m ild), 74
Su p erior lid crease, 36 Wern er classificat ion , 74
Su p erior lim bic keratoconju n ctivit is, 122–123, Tilosis, w ith blep h arit is, 100
122f Tolosa-Hun t syn drom e, 77, 128
differen t ial d iagn osis, 63, 192 Toxic epiderm al n ecrolysis, 112, 113
an d filam en tar y kerat it is, 154 Toxocariasis, 201
Su p erior obliqu e p alsy Toxoplasm osis
acqu ired, 349 adu lt , 201
bilateral, 349–350, 350f congen it al, 200
congen it al, 349 ocu lar involvem en t in , 200–202
u nilateral, 349f, 350 Trach om a
Su perior rect u s m uscle palsy, isolated, 348 Chlam ydia, 106–109, 107f, 107t–108t
Su pranu clear p alsy, 354f differen t ial d iagn osis, 110, 112, 192
Sym bleph aron , 116–117, 116f an d sym bleph aron , 116
in Steven s-Joh n son syn d rom e, 113 an d dist ich iasis, 64
Sym pathet ic oph th alm ia, 213 an d trich iasis, 64
Syph ilis Trau m a, 1–32
acqu ired an terior segm en t , 1–17
an d in terstit ial kerat it is, 150, 151 an d bacterial corn eal u lcer, 146
an d len s su blu xat ion , 13 carot id cavern ou s fist u la caused by, 31–32,
ocu lar involvem en t in , 198 31f, 90, 93
congen it al cat aract cau sed by, 11–13, 12f
an d in terstit ial kerat it is, 150, 151, 151f cerebral, in p ed iatric pat ien t , 201
ocu lar involvem en t in , 198 corn eal involvem en t in , 192
differen t ial d iagn osis, 285 differen t ial d iagn osis, 69, 72, 324
vit reou s h em orrh age in , 18 en doph th alm it is cau sed by, 213–214
Syringom a, 57–58 an d fu ngal kerat itis, 149
System ic lupus er yth em atosus lacrim al system , 2, 3f
an d cen t ral serous ch orioret in op athy, 273 an d len s su blu xat ion , 13
perip h eral u lcerat ive keratit is in , 183f opt ic n er ve, 25–26, 25f
sclerit is in , 124 orbital, 26–32
pen et rat ing, 21
T perforat ing, 16–17, 17f
Taches de bougie, 199 posterior segm en t , 17–26
Tapetoch oroidal degen erat ion , sen ile, 291 an d ret in al d etach m en t , 297
Tay-Sach s disease, 267 an d sym bleph aron , 116
Tear deficien cy to t rigem in al ner ve, and n eurot ropic
an d filam en tar y kerat it is, 154 keratopathy, 155
in Steven s-Joh n son syn d rom e, 113 an d vit reou s h em orrh age, 270
Tears, art ificial, 114–115 Trich iasis, 64, 65f
Telecan thus, in bleph aroph im osis syndrom e, w ith bleph arit is, 100
48, 48f differen t ial d iagn osis, 35, 110
Index 555
pem p h igoid an d, 112 glau com a-related, 196

in Steven s-Joh n son syn d rom e, 113 gran ulom atous, 198–199
Trisom y 13, 135 HLA-B27-associated , 194–195
Trisom y 15, 135 in in flam m ator y bow el disease, 195
Trisom y 21, 49. See also Dow n syn drom e in term ed iate, 200
Tuberculosis in juven ile rh eu m atoid ar th rit is, 196–197
ch orioretin it is in , 201 p h acoan ap hylact ic, 12
an d in terstit ial kerat it is, 150, 151 p h acolyt ic, 196
ocu lar involvem en t in , 199, 199t p osterior, 200–209
Tum or(s) in psoriat ic ar th rit is, 195
an d bilateral superior obliqu e palsy, 349 in Reiter syn drom e, 194–195
ch oroidal, 326–332 in sarcoidosis, 198–199
differen t ial d iagn osis, 274 t u bercu lou s, 199, 199t
ciliar y body, 323, 325–326 in uveit is-glau com a-hyp h em a synd rom e,
eyelid, 51–57, 59–64 196
fu n dal, p igm en ted , 333 vit reous h em orrh age w ith, 18
in t raocu lar Uveit is-glau com a-hyp h em a syn d rom e, 196
an d cystoid m acu lar edem a, 274
an d vit reou s h em orrh age, 270 V
of iris, 322–325 Valsalva ret in opathy, 263
lacrim al glan d, 97–98 Varicella-zoster virus. See also Herpes zoster
orbital, 69–99 acu te ret in al n ecrosis syn d rom e cau sed
differen t ial d iagn osis, 128 by, 209
ret in al, 333–337 in fect ion , 105
an d ret in al d etach m en t , 298f Vascular lesion s, orbit al, 85t, 90. See also
ret in al p igm en t epith eliu m , 332–333 Cap illar y h em angiom a; Cavern ou s
Turn er syndrom e, 135 h em angiom a; Lym ph angiom a;
Type A p erson alit y, and cen t ral serou s Orbital varices
ch orioretin op athy, 273 Vasculit is
d ifferen tial d iagn osis, 269
U ret in al, an d cystoid m acu lar edem a, 274
Ulcer Verruca, 58
corn eal VH. See Vit reous h em orrhage
from Acantham oeba, 147, 148f Viral in fect ion , corn eal, 146
bacterial, 146–147, 146f Viral kerat it is, 149, 150
differen t ial d iagn osis, 7, 143, 146, 153, Vision loss, w ith ret in al breaks/ret in al
192 d etach m en t , 297
fu ngal, 149, 149f Vitam in A d eficien cy, 285
h erp et ic, 192 Vitam in B deficien cy, 192
den d rit ic, 104f Vitelliform m acu lar dyst rop hy, 280–281
im m u n e-m ediated , 146, 147, 153 Vit iligin ou s ch orioret in it is, 206–207.207f
in fect iou s, 153 Vit rectom y
m argin al for ep iret in al m em bran e, 321
in HSV in fect ion , 142 25-gauge t ransconjun ct ival, 305, 305f
st aphylococcal, 177, 182 p n eu m at ic, 305
sterile, 146 p rim ar y, 303
n eu rot ropic an d recu rren t corn eal erosion , 156
in HSV in fect ion , 142, 143 Vit reom acu lar t ract ion syn drom e, an d cystoid
in HZV in fect ion , 144f m acular edem a, 274
in Steven s-Joh n son syn d rom e, 113 Vit reoret in al adh eren ce, focal, an d t rau m at ic
trach om a, 107f ret in al detachm en t , 21
viral, 146 Vit reou s d etach m en t , p osterior, 295–296
den drit ic d ifferen tial d iagn osis, 295
differen t ial d iagn osis, 3 an d retin it is pigm en tosa, 285
h erp et ic, 104f Vit reou s h em orrh age, 270
h erp et ic am blyop ia an d, 19
den d rit ic, 104f cau ses, 270
differen t ial d iagn osis, 7 diabet ic ret in op athy an d, 270
Ulcerat ive colit is, ocu lar involvem en t in , 195 differen t ial d iagn osis, 18, 270, 295
Ulcerat ive kerat it is Eales disease an d , 270
differen t ial d iagn osis, 6, 182, 184 an d exu dat ive age-related m acu lar
perip h eral, 182–184, 183f degen erat ion , 270
Ultraviolet (UV) kerat it is, 6 an d gh ost-cell glau com a, 18–19
Ush er syn drom e, an d ret in it is p igm en tosa, an d h em osiderosis bu lbi, 19
285 in h istoplasm osis, 18
Uveal effu sion syn drom e, 325 m an agem en t, 270
Uveit is in m elan om a, 18
acu te an terior n ongran u lom atou s, 194–197 posterior vit reou s detach m en t an d , 270
in an kylosing sp ondylit is, 194 post t rau m at ic, 17–19, 18f, 270
an terior, 10 presen tat ion , 270
in Beh çet syn drom e, 212–213, 212f an d p roliferat ive ret in opathy, 18, 19
ch ron ic n ongran u lom atou s, 196–197 proliferat ive sickle cell ret in op athy an d, 270
congen it al syp h ilit ic, 198 w ith ret in al breaks/ret in al detach m ent , 297
an d cystoid m acu lar edem a, 274, 276 w ith ret in oblastom a, 18
differen t ial d iagn osis, 124, 125, 324 sp on tan eou s, 18
disord ers m asqu erading as, 216–217, 216t in syp h ilis, 18
556 Index
Vit reou s h em orrh age (cont inued) Wegen er gran u lom atosis, sclerit is in ,
traum at ic, 17–19, 18f, 270 124
in ch ild abu se, 18 Weill-March esan i syn drom e, 135
Vit rit is an d len s su blu xat ion , 13
in ad ult toxoplasm osis, 201 Weiss ring, 295
differen t ial d iagn osis, 295 Wet sn ow, in adu lt toxop lasm osis, 201
Vogt-Koyan agi-Harada syn drom e, 210, 210f, W h ite dot syn drom e, 203–204
211f Wilder sign , 76t
Vogt lim bal gird le, 174, 174f Wou nd leak, after cat aract su rger y, 237–
Vogt st riae, keratocon u s an d, 171 238
Von Grafe sign , 76t
von Hipp el-Lin dau disease, 334 X
Vossius ring, 11 Xan th elasm a, 57, 58
W Z
Warfarin , an d su bconjunct ival hem orrh age, 3 ZTTs. See Ret in al t uft s, zon ular t raction

Color Atlas of Ophthalmology - Amar Agrawal & Soosan Jacob

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The Quick-Reference Manual

Amar Agarw al, MS, FRCS, FRCOphth

Soosan Jacob, MS, FRCS, DNB, MNAMS

Brian S. Boxer Wach ler, MD

Chapter 14 Oph th alm ic Ph arm acology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 405

Ronald R. Krueger, MD, MSE

W h at is an oph th alm ologist? On e w h o h as gradu ated from m edical sch ool an d is

Am ar Agarw al, MS, FRCS, FRCOphth

Amar Agarw al, MS, FRCS, FRCOphth

Soosan Jacob, MS, FRCS, DNB, MNAMS

Am ar Agarw al, MS, FRCS, FRCOphth

Athiya Agarw al, MD, FRSH, DO

Sam uel Boyd, MD

Clem ent K. Chan, MD

Kim berly P. Co ckerham , MD, FACS

Kenneth M. Daniels, OD, FAAO

Duncan A. Friedm an, MD, MPH

David R. Hardte n, MD, FACS

Jam es M. Hill, PhD

Jeffery A. Ho bde n, PhD

Jean T. Jaco b, PhD

So o san Jaco b, MS, FRCS, DNB, MNAMS

Herbert E. Kaufm an, MD

Natalia Kram arevsk y, MD

Dhivya Asho k Kum ar, MD

Man de ep Lam ba, MBBS, DNB, FERC

Pao lo Lan zetta, MD

Lo uis E. Pro bst, MD

Carlo Sbo rgia, MD

Ivan R. Schw ab, MD, FACS

Cristina Sim ó n-Castellví

Guillerm o Sim ó n-Castellví

Jo sé María Sim ó n-Castellví

Sarabel Sim ó n-Castellví

Jo sé María Sim ó n-To r

Giuseppe Sm aldo ne, MD

Dariusz G. Tarasew icz, MD, PhD

Fede rico G. Velez, MD

Christo pher I. Zo um alan, MD

Anterior Segment Trauma

Fig. 1.1 Eyelid laceration involving the eyelid margin with

su spected. Tetan u s prophylaxis an d baselin e serology for h u m an im m un odefi-

Lacrimal System Trauma

Fig. 1.2 (A) Lacrimal system trauma

Fig. 1.3 Subconjunctival hemorrhage. A bright red

Fig. 1.5 (A) A moderate chem i-

Fig. 1.6 Corneal defect stained

Corneal Foreign Body

Fig. 1.7 Corneal foreign body.

Fig. 1.8 Penetrating corneal laceration with iris prolapse.

am in e 0.25%) an d an an t ibiot ic (e.g., frequ en t polym yxin B/bacit racin oin t m en t

Fig. 1.9 Post traumatic hyphema

Iris Sphincter Tear

Fig. 1.10 Sphincter tear. (Courtesy of Amar Agarwal, Dr.

Fig. 1.11 (A) Cataract and iridodialysis secondary to severe

Fig. 1.12 Lens subluxation and zonular disruption.

Fig. 1.13 Post traum atic hyphema. Grade II hyphemas have

Fig. 1.14 Perforating ocular trauma.

Posterior Segment Trauma

Posttraumatic Vitreous Hemorrhage

Fig. 1.15 Post traum atic vitreous

Fig. 1.16 Peripheral comm otio