EDITOR

Douglas J. Rhee, MD
Associate Chief, Operations and Practice Development
Massachusetts Eye and Ear In rmary
Associate Professor
Harvard Medical School
Boston, Massachusetts

SERIES EDITOR
Christopher J. Rapuano, MD
Director and Attending Surgeon, Cornea Service
Co-Director, Refractive Surgery Department
Wills Eye Institute
Professor of Ophthalmology
Jefferson Medical College of Thomas Jefferson University
Philadelphia, Pennsylvania
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Glaucoma / edi or, Douglas J. Rhee. – 2nd ed.
p. ; cm. – (Color a las & synopsis o clinical oph halmology-Wills Eye Ins i u e)
Rev. ed. o : Glaucoma : color a las and synopsis o clinical oph halmology / Douglas J. Rhee.
New York : McGraw-Hill, Medical Pub. Division, c2003.
Includes bibliographical re erences and index.
ISBN 978-1-60913-337-5 (alk. paper)
I. Rhee, Douglas J. II. Rhee, Douglas J. Glaucoma. III. Series: Color a las and synopsis o clinical
oph halmology series.
[DNLM: 1. Glaucoma–A lases. WW 17]
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o my lovely wi e, ina, I dedica e my con ribu ions o you and am so gra e ul or your pa ience and suppor .
o my daugh ers, Ashley and Alyssa, I dedica e his book wi h my hopes or your u ure happiness and success.
o my a her and mo her, Dennis and Serena Rhee, in apprecia ion or your endless love, sacrif ce, suppor ,
and dedica ion, I hank you. o Susan Rhee or your unders anding and kindness.
Finally, o all my amilies—Rhee, Chang, Kim, Chomakos, and Joseph.
 Con ribu ors
Augus o Azuara-Blanco, MD, PhD Francisco Fan es, MD
Consul an Oph halmic Surgeon Associa e Pro essor o Oph halmology
Aberdeen Royal In rmary Bascom Palmer Eye Ins i u e
Honorary Clinical Senior Lec urer Universi y o Miami School o Medicine
Universi y o Aberdeen Miami, Florida
Aberdeen, Uni ed Kingdom
a hryn B. Freidl, MD
Oscar V. Beaujon-Balbi, MD Florida Eye Specialis s
Clinica Luis Razet i and Me ropoli an Cen er o Jacksonville, Florida
Oph halmology
JoAnn A. Giaconi, MD
Francisco Risquez Hospi al
Heal h Sciences Assis an Pro essor o
Caracas, Venezuela
Oph halmology
Oscar Beaujon-Rubin, MD Jules S ein Eye Ins i u e
Clinica Luis Razet i and Me ropoli an Cen er o David Ge en School o Medicine, UCLA
Oph halmology Chie o Oph halmology
Francisco Risquez Hospi al Ve erans Adminis ra ion o Los Angeles
Caracas, Venezuela Los Angeles, Cali ornia
José I. Belda, MD, PhD, FEBO Rober J. Goule III, MD
Chairman, Depar men o Oph halmology Boling Vision Cen er
Hospi al de orrevieja Elkhar , Indiana
Alican e, Spain
Shelly R. Gup a, MD
Nicole Beni ah, MD Assis an Pro essor o Oph halmology
Priva e Prac ice Glaucoma Division
Cali ornia Ohio S a e Universi y
Havener Eye Ins i u e
Ronald Buggage, MD
Columbus, Ohio
Chie Scien i c Of cer
Novagali Pharma Alon Harris, MS, PhD, FARVO
Evry, France Lois Le z er Pro essor o Oph halmology
Pro essor o Cellular and In egra ive Physiology
Gabriel Chong, MD
Direc or, Glaucoma Research and Diagnos ic
Glaucoma Specialis
Cen er
Raleigh Oph halmology
Indiana Universi y School o Medicine
Raleigh, Nor h Carolina
Indianapolis, Indiana
Mary Jude Cox, MD
Ribhi Hazin, MD
Eye Physicians
Gradua e S uden
Voorhees, New Jersey
Harvard School o Public Heal h
Syril Dorairaj, MD Bos on, Massachuset s
Einhorn Clinical Research Cen er
Malik Y. ahook, MD
New York Eye and Ear In rmary
Associa e Pro essor
New York, New York
Chie o Glaucoma Service
Universi y o Colorado Hospi al Eye Cen er
Denver, Colorado

vi
 CO NTRIBUTO RS vii

L. Jay a z, MD Jona han S. Myers, MD

Pro essor o Oph halmology Associa e At ending Surgeon
Je erson Medical College Wills Eye Ins i u e
Direc or o Glaucoma Service Philadelphia, Pennsylvania
Wills Eye Ins i u e
Jamie E. Nicholl†
Philadelphia, Pennsylvania
Oph halmic Pho ographer
Ashley G. Lesley, MD Wills Eye Hospi al
Glaucoma Specialis Philadelphia, Pennsylvania
Visalia Eye Cen er Paul F. Palmberg, MD, PhD
Visalia, Cali ornia Pro essor o Oph halmology
Chris opher ai-Shun Leung, BMedSc, MSc, Bascom Palmer Eye Ins i u e
MB ChB, MD, MRCS, FHK M, FH COph h Universi y o Miami School o Medicine
Associa e Pro essor Miami, Florida
Depar men o Oph halmology and Visual George N. Papaliodis, MD
Sciences Direc or, Ocular Immunology and Uvei is Service
T e Chinese Universi y o Hong Kong Assis an Pro essor o Oph halmology
Hong Kong Eye Hospi al, Hong Kong Harvard Medical School
Richard A. Lewis, MD Massachuset s Eye and Ear In rmary
Consul an in Glaucoma Bos on, Massachuset s
Sacramen o, Cali ornia Sung Chul Park, MD
Jef rey M. Liebmann, MD Einhorn Clinical Research Cen er
Einhorn Clinical Research Cen er New York Eye and Ear In rmary
New York Eye and Ear In rmary New York, New York
Depar men o Oph halmology Depar men o Oph halmology
New York Universi y School o Medicine New York Medical College
New York, New York Valhalla, New York

Michele C. Lim, MD Louis R. Pasquale, MD, FARVO

Associa e Pro essor o Oph halmology
Vice Chair and Medical Direc or
Universi y o Cali ornia Davis Heal h Sys em Eye
Cen er
Sacramen o, Cali ornia
Direc or, Glaucoma Service
Massachuset s Eye and Ear In rmary
Associa e Pro essor o Oph halmology
Harvard Medical School
Bos on, Massachuset s

Shan Lin, MD omas D. Pa rianakos, DO

Associa e Pro essor o Clinical Oph halmology
Universi y o Cali ornia, San Francisco
San Francisco, Cali ornia
imberly V. Miller, MD
Assis an Pro essor o Oph halmology
UPMC Eye Cen er
Pit sburgh, Pennsylvania
Marlene R. Mos er, MD
Pro essor o Clinical Oph halmology
Wills Eye Hospi al
Je erson Medical College
T omas Je erson Universi y
Philadelphia, Pennsylvania
Direc or o Glaucoma Services
John H. S roger Jr. Hospi al o Cook Coun y
Universi y o Chicago Hospi al
Chicago, Illinois
Rober Ri ch, MD
Shelley and S even Einhorn Dis inguished Chair
Pro essor o Oph halmology
Surgeon Direc or and Chie , Glaucoma Services
Depar men o Oph halmology
Einhorn Clinical Research Cen er
New York Eye and Ear In rmary
New York, New York
New York Medical College
Valhalla, New York
†Deceased
 viii CO NT RIBUTO RS
Deepam Rusia, MD
Glaucoma Research and Diagnos ic Cen er
Eugene and Marilyn Glick Eye Ins i u e
Indiana Universi y School o Medicine
Indianapolis, Indiana
onrad Schargel, MD, PhD, FEBO
Glaucoma and Neurooph halmology Depar men
Hospi al de orrevieja
Allican e, Spain
onrad W. Schargel, MD, PhD
Chairman, Oph halmology Depar men (Re ired)
Hospi al Cesar Rodriguez Rodriguez
Puer o La Cruz, Edo Anzoa egui, Argen ina
Joel S. Schuman, MD, FACS
Eye and Ear Founda ion Pro essor and
Chairman
Depar men o Oph halmology
Universi y o Pit sburgh School o Medicine
Direc or, UPMC Eye Cen er
Pro essor o Bioengineering
Eye and Ear Ins i u e
Pit sburgh, Pennsylvania
Geof rey P. Schwar z, MD
Ins ruc or, Glaucoma Service
Wills Eye Ins i u e
Je erson Medical College
T omas Je erson Universi y
Philadelphia, Pennsylvania
Louis W. Schwar z, MD
Clinical Associa e Pro essor
Je erson Medical College
T omas Je erson Universi y
Philadelphia, Pennsylvania
Rajesh . Shet y, MD
Florida Eye Specialis s
Jacksonville, Florida
Bren Siesky, PhD
Assis an Direc or, Ocular Vascular Research
Cen er
Vascular Reading Cen er
Glaucoma Research and Diagnos ic Cen er
Eugene and Marilyn Glick Eye Ins i u e
Indiana Universi y School o Medicine
Indianapolis, Indiana
Ar hur J. Si , MD
Assis an Pro essor
Depar men o Oph halmology
Mayo Clinic
Roches er, Minneso a
George L. Spae h, MD
Direc or, Glaucoma Research Cen er
Esposi o Research Pro essor
Wills Eye Ins i u e
Je erson Medical Cen er
Philadelphia, Pennsylvania
Angela V. uralba, MD
Ins ruc or in Oph halmology
Harvard Medical School
Massachuset s Eye and Ear In rmary
Bos on VA Heal hcare Sys em
Bos on, Massachuset s
ara A. Uhler, MD
Assis an Pro essor o Oph halmology
Je erson Medical College
T omas Je erson Universi y
Direc or o Residen Educa ion
Wills Eye Residency Program a Je erson
Philadelphia, Pennsylvania
Zinaria Y. Williams, MD
Vice Presiden and Associa e Medical Direc or
Cline Davis & Mann
New York, New York

he beau y o he a las/ synopsis concep
is he power ul combina ion o illus ra-
ive pho ographs and a summary approach
o he ex . Oph halmology is a very visual
discipline ha lends i sel nicely o clinical
pho ographs. Al hough he seven oph halmic
subspecial ies in his series—Cornea, Re ina,
Glaucoma, Oculoplas ics, Neurooph halmology,
Pedia rics, and Uvei is—use varying levels o
visual recogni ion, a rela ively s andard orma
or he ex is used or all volumes.
Abou he Series
T e goal o he series is o provide an up- o-
da e clinical overview o he major areas o
oph halmology or s uden s, residen s, and
prac i ioners in all o he heal h care pro es-
sions. T e abundance o large, excellen qual-
i y pho ographs and concise, ou line- orm ex
will help achieve ha objec ive.
Chris opher J. Rapuano, MD
Series Edi or
ix
 Acknowledgmen s

I would like o hank he many au hors and con ribu ors who par icipa ed in his endeavor.
I believe ha he diversi y o represen a ions is one o he s reng hs o his ex .

x

C olor A las & Synopsis o Clinical
Oph halmology—Wills Eye Ins i u e—
Glaucoma at emp s o cover as many o he
glaucoma syndromes as possible. No condi ion
appears iden ical in all cases. T ere ore, many
di eren represen a ive images are presen ed
or he more common condi ions in an at emp
o re ec he diversi y o presen a ions.
Pre ace
In his second edi ion, we have added several
new chap ers o encompass he evolving ech-
niques and echnologies o he surgical man-
agemen o glaucoma as well as enhanced he
sec ion on acu e angle closure. Fur hermore,
several new gures appear hroughou he
book. I hope ha you will nd his a las o be
a use ul re erence and an aid o your clinical
endeavors.
Douglas J. Rhee, MD
Edi or
xi
 Con en s
Con ribu ors vi
Abou he Series ix
Acknowledgmen s x
Pre ace xi

SEC ION I Glaucoma Diagnosis

Ch a pt er 1 In roduc ion o Glaucoma Diagnosis 2

Douglas J. Rhee

Ch a pt er 2 Basics o Aqueous Flow and he Op ic Nerve 4

Ar hur J. Si and Douglas J. Rhee
Aqueous Flow 4
Impor ance o In raocular Pressure 4
Brie Summary o Aqueous Physiology and IOP 4
Measuremen o Aqueous Humor Dynamics 5
Op ic Nerve 13

Ch a pt er 3 onome ry 14
Rajesh K. Shet y
Goldmann Applana ion onome er 14
Schiö z onome er 16
Perkins onome er 18
ono-Pen 19
Pneumo onome er 20
Dynamic Con our onome ry 21

Ch a pt er 4 Gonioscopy 22
Oscar V. Beaujon-Balbi and Oscar Beaujon-Rubin
Direc Gonioscopy 22
Indirec Gonioscopy 25
Es ima ing he An erior Chamber Dep h 28
echnique 30
Elemen s o he Angle Ana omy 32
Iden i ca ion o Angle S ruc ures 34
Classi ca ion o he Angle 38

xii
 CO NTENTS xiii

Pigmen Deposi ion and Gonioscopy 40

Error Fac ors on Gonioscopy 44
Use o Gonioscopy in rauma 46

Ch a pt er 5 An erior Segmen Imaging 48

Sung Chul Park, Syril Dorairaj, Je rey M. Liebmann, and Rober Ri ch
Angle-closure Glaucoma 49
Rela ive Pupillary Block 49
Pla eau Iris 50
Phacomorphic Glaucoma 50
Malignan Glaucoma 50
Pseudophakic Pupillary Block 51
Pseudophakic Malignan Glaucoma 51
Open-angle Glaucoma 56
Pigmen Dispersion Syndrome and Pigmen ary Glaucoma 56
Ex olia ion Syndrome 56
O her Condi ions 58
Cyclodialysis Cle 58
Iridociliary Cys 58
Ciliary Body umors 58
Iridoschisis 58
Surgery and Glaucoma 61
Fil ering Bleb 61
Glaucoma Drainage Implan s 61

Ch a pt er 6 Op ic Nerve Imaging 64
T omas D. Pa rianakos
S ereopho ography 64
Con ocal Scanning Laser Oph halmoscopy 67
Scanning Laser Polarime ry 71
Op ical Coherence omography 75

Ch a pt er 7 Evalua ion o he Op ic Nerve and Nerve Fiber Layer 82

Zinaria Y. Williams, Kimberly V. Miller, and Joel S. Schuman
Func ional es s 82
Au oma ed Perime ry 84
Swedish In erac ive T reshold Algori hms 85
Glaucoma Progression Analysis 88
Visual Field Index 89
Shor -waveleng h Au oma ed Perime ry 90
Frequency-doubling echnology 92
Mul i ocal Elec rore inography 95
Visually Evoked Cor ical Po en ial 97
 xiv CO NTENTS

S ruc ural es s 99
Pho ography 99
Scanning Laser Polarime ry 102
Con ocal Scanning Laser Oph halmoscopy 105
Op ical Coherence omography 110
Re inal T ickness Analyzer 119

Ch a pt er 8 Psychophysical es ing 120

Douglas J. Rhee, ara A. Uhler, and L. Jay Ka z
Purpose o es 121
Descrip ion 122
Common Op ic Nerve Visual Fields Found in Pa ien s wi h Glaucoma 125
Newer Psychophysical es ing: Frequency-doubling Perime ry
and Shor -wave Au oma ed Perime ry 132

Ch a pt er 9 Blood Flow in Glaucoma 136

Alon Harris, Bren Siesky, and Deepam Rusia
Scanning Laser Oph halmoscope Angiography 138
Color Doppler Imaging 142
Measuremen o Ocular Pulsa ion 144
Con ocal Scanning Laser Doppler Flowme ry 146
Spec ral Re inal Oxime ry 148
Doppler Ocular Coherence omography 149

SEC ION II Clinical Syndromes

Ch a pt er 10 In roduc ion o Clinical Syndromes 150

Douglas J. Rhee

Ch a pt er 11 Developmen al Glaucomas (Congeni al Glaucomas) 152

Oscar V. Beaujon-Balbi, Oscar Beaujon-Rubin, and Douglas J. Rhee
Primary Congeni al Glaucoma 153
Glaucoma Associa ed wi h Congeni al Anomalies 162
Aniridia 162
Axen eld’s Anomaly 164
Rieger’s Anomaly 164
Rieger’s Syndrome 164
Pe er’s Anomaly 164
Mar an Syndrome 167
Microspherophakia 169
S urge–Weber Syndrome (Encephalo rigeminal Angioma osis) 170
Neuro broma osis (Von Recklinghausen’s Disease and Bila eral Acous ic
Neuro broma osis) 172
 CO NTENTS xv

Ch a pt er 12 Primary Open-angle Glaucoma 174

George L. Spae h, Shelly R. Gup a, and Rober J. Goule III
De ni ion 174
Epidemiology 177
Pa hophysiology 178
His ory 180
Clinical Examina ion 182
Special es s 190
rea men 191

Ch a pt er 13 Secondary Open-angle Glaucoma 196

Jona han S. Myers
Pigmen Dispersion Syndrome 196
Ex olia ion Syndrome 200
S eroid-responsive Glaucoma 204

Ch a pt er 14 Uvei ic Glaucomas 208

Nicole Beni ah, Ronald Buggage, and George N. Papaliodis
Epidemiology 209
E iology 209
Open-angle Mechanisms 210
Closed-angle Mechanisms 214
Speci c En i ies 227
Fuchs’ He erochromic Iridocycli is 227
Glaucoma ocycli ic Crisis (Posner–Schlossman Syndrome) 230
Herpe ic Kera ouvei is 232
Syphili ic In ers i ial Kera i is 235
Juvenile Idiopa hic Ar hri is 237
Lens-induced Uvei is and Glaucoma 240
Sarcoidosis 243

Ch a pt er 15 Lens-associa ed Open-angle Glaucomas 246

Michele C. Lim and Ashley G. Lesley
Lens Pro ein or Phacoly ic Glaucoma 247
Lens Par icle Glaucoma 250
Lens-associa ed Uvei is (Phacoanaphylaxis) 253
Phacomorphic Glaucoma 256

Ch a pt er 16 rauma ic Glaucoma 258

Angela V. uralba and Mary Jude Cox
rauma ic Hyphema 258
Angle Recession 266
Cyclodialysis Cle 268
 xvi CO NTENTS

Ch a pt er 17 Primary Acu e Angle-closure and Chronic Angle-closure

Glaucoma 270
Chris opher Kai-Shun Leung
Primary Acu e Angle Closure 276
Chronic Angle–closure Glaucoma 277
Pla eau Iris 277

Ch a pt er 18 Secondary Angle-closure Glaucoma 282

Douglas J. Rhee and Jamie E. Nicholl
Neovascular Glaucoma 282
Iridocorneal Syndromes 285
Aqueous Misdirec ion Syndrome (Malignan Glaucoma) 289

Ch a pt er 19 Glaucoma Secondary o Eleva ed Venous Pressure 292

Douglas J. Rhee, Ribhi Hazin, and Louis R. Pasquale
Caro id-Cavernous Fis ula 292
S urge–Weber Syndrome 296
Idiopa hic Eleva ed Episcleral Venous Pressure 299
O her Causes o Eleva ed Episcleral Venous Pressure 300

SEC ION III Glaucoma Managemen

Ch a pt er 20 In roduc ion o Glaucoma Managemen 302

Douglas J. Rhee

Ch a pt er 21 Medical Managemen 304

Malik Y. Kahook and Douglas J. Rhee
Descrip ion and Physiology 304
Alpha Agonis s 306
Be a-blockers 308
Carbonic Anhydrase Inhibi ors 309
Hyperosmolar Agen s 311
Mio ics 311
Pros aglandins 313
Sympa homime ic Agen s 315
Combina ion Agen s 316
echnique o Drop Ins illa ion 317
 CO NTENTS xvii

Ch a pt er 22 Laser rabeculoplas y 320

L. Jay Ka z and Ka hryn B. Freidl
Indica ions 320
Argon Laser rabeculoplas y 321
Selec ive Laser rabeculoplas y 324
Economics 327

Ch a pt er 23 Deep Sclerec omy Surgery or Glaucoma 328

Konrad Schargel, José I. Belda, and Konrad W. Schargel
S udies 328
Surgical echnique 331
Pos opera ive Care 343
Conclusion 344

Ch a pt er 24 rabeculec omy and Ex-PRESS Mini Glaucoma Shun 350

Marlene R. Mos er and Augus o Azuara-Blanco
rabeculec omy 350
Surgical echnique 352
Pos opera ive Care 363
Complica ions 365
Ex-PRESS Mini Glaucoma Shun 373
Surgical echnique 374

Ch a pt er 25 Glaucoma Drainage Devices 376

JoAnn A. Giaconi and Marlene R. Mos er
Descrip ion 376
Surgical echnique 379
Pos opera ive Care 386
Complica ions 386

Ch a pt er 26 Schlemm’s Canal-based Surgery 392

Richard A. Lewis
Indica ions 392
Canaloplas y 392
rabec ome 397
iS en 399

Ch a pt er 27 Cyclodes ruc ive Procedures or Glaucoma 402

Shan Lin, Geo rey P. Schwar z, and Louis W. Schwar z
Indica ions 402
Con raindica ions 403
echniques 404
 xviii CO NTENTS

Noncon ac ransscleral CPC 404

Con ac ransscleral CPC 406
Cyclocryo herapy 407
ranspupillary CPC 408
Endoscopic Cyclopho ocoagula ion 409
Pos procedure Care 411
Complica ions 411

Ch a pt er 28 La e Complica ions o Glaucoma Surgery 412

Gabriel Chong, Francisco Fan es, and Paul F. Palmberg
Hypo ony 413
Bleb Leaks 419
Gian Blebs 424
T e Failing Bleb: Encapsula ion 430
Circum eren ial Bleb 432
ube Shun Erosions 434

Index 437
 C H AP T ER
Introduction to Glaucoma Diagnosis
T he term glaucoma is rom the Greek
glaukos, which means “watery blue.”
It is irst mentioned in the Hippocratic
Aphorisms around 400 BC. However, it was
considered a disease o the crystalline lens
or several hundred years ollowing. “ he
scienti ic history o glaucoma began the day
on which cataracts were put in their correct
place” (Albert erson, 1867–1935, French
ophthalmologist). he correct anatomic
location o cataracts is credited to Pierre
Brisseau (1631–1717) in 1707. Elevation
o intraocular pressure as a sign o glaucoma
was irst mentioned in Breviary (1622) by
Richard Banister (1570–1626, English oph-
thalmologist). Discovery o the ophthalmo-
scope in 1851 by Hermann von Helmholtz
(1821–1894, German ophthalmologist)
and its subsequent use by Edward Jaeger
(1818–1884) led to the belie that the optic
nerve was also involved. Cupping o the
optic nerve as a sign o glaucoma was con-
irmed by anatomist Heinrich Muller in the
late 1850s. Von Grae e is credited as hav-
ing irst described contraction o the visual
ield and paracentral de ects in glaucoma in
1856.
2
Douglas J. Rhee
In recent history, glaucoma had been def ned by
having an intraocular pressure (IOP) above 21
mm Hg (i.e., more than two standard deviations
above the mean IOP rom a population-based
survey o IOP). Later research indicated that the
majority o people with IOPs above 21 mm Hg
do not develop glaucomatous visual f eld loss.
Additionally, up to 40% o patients with docu-
mented glaucomatous visual f eld loss never
achieve IOPs higher than 21 mm Hg. Our mod-
ern concept o primary open-angle glaucoma
(POAG) is a description o the constellation o
signs requently seen in “glaucoma” that incor-
porate IOP, optic nerve appearance, and charac-
teristic visual f eld changes. T e hallmark o the
diagnosis o glaucoma is progressive change in
the optic nerve or visual f eld, or both, over time.
Many glaucoma specialists believe that POAG
probably represents many diseases with a f nal
common pathway. Our most recent genetic
investigations indicate that POAG is polygenic
in which minor alterations o numerous genes
contribute to create the syndrome. All mende-
lianly inherited genetic mutations account or
less than 10% o all POAGs. Our def nition o
glaucoma will no doubt continue to evolve as
our understanding o the disease increases.

A more modern def nition or glaucoma is as
ollows: a pathologic condition in which there
is a progressive loss o ganglion cell axons
causing visual f eld damage that is related to
IOP. Currently, we look to evaluate the ollow-
ing components when making the diagnosis
o glaucoma: history, presence or absence o
risk actors, IOP, optic nerve examination, and
visual f eld testing.
T is section covers the various methods or
obtaining the in ormation used or both diag-
nosing glaucoma and ollowing the adequacy
o treatment.
Introduction to Glaucoma Diagnosis 3
BIBLIOGRAPH Y
Allingham RR, Liu Y, Rhee DJ. T e genetics o primary
open-angle glaucoma: A review. Exp Eye Res. 2009;
88:837–844.
Blodi FC. Development o our concept o glaucoma.
In: Basic and Clinical Science Course, Section 10. San
Francisco, CA: American Academy o Ophthalmo-
logy; 1996.
Kron eld PC. Glaucoma. In: Albert DM, Edwards DD, eds
History of Ophthalmology. Cambridge, MA: Blackwell
Science; 1996:203–223.
Mikelberg FS, Drance SM. Glaucomatous visual f eld
de ects. In: Ritch R, Shields MB, Krupin , eds.
T e Glaucomas. St. Louis, MO: Mosby-Year Book;
1996:523–537.
Sommer A, ielsch JM, Katz J, et al. Relationship between
intraocular pressure and primary open angle glaucoma
among white and black Americans: T e Baltimore eye
survey. Arch Ophthalmol. 1991;109:1090–1095.
 C H AP T ER
Basics o Aqueous
Flow and he Op ic Nerve
AQ UEO US FLO W
IMPORTANCE OF
INTRAOCULAR PRESSURE
H aving a basic unders anding o he physi-
ology o he eye is help ul o under-
s anding he pa hophysiology, diagnosis, and
managemen o glaucoma. Many clinicians
and scien is s now believe ha several ac ors
are involved in he pa hogenesis o glaucoma,
such as apop osis, al ered blood ow o he
op ic nerve, and possible au oimmune reac-
ions. However, in raocular pressure (IOP)
remains one o he mos impor an risk ac-
ors or he disease syndromes. Addi ionally,
lowering o he IOP is he only rigorously
proven rea men or glaucoma. Al hough we
have some unders anding o he physiology o
IOP, we do no ye ully unders and how he
eye regula es IOP a he cellular and molecu-
lar level. Wi h each passing year, more is learn
abou his physiologic process. Someday, we
may have he answer o wha many pa ien s
have asked—wha causes glaucoma?
4
Arthur J. Sit and Douglas J. Rhee
BRIEF SUMMARY OF
AQUEOUS PHYSIOLO GY
AND IOP
A queous is ormed in he ciliary processes
(pars plica a region o he re ina) (Fig.
2-1). T e epi helial cells o he inner nonpig-
men ed layer are el o be he si e o aqueous
produc ion (Fig. 2-2). Aqueous is produced
by a combina ion o ac ive secre ion, ul raf l ra-
ion, and di usion. Many o he IOP-lowering
agen s work by decreasing aqueous secre ion in
he ciliary body.
Aqueous hen ows hrough he pupil and
in o he an erior chamber nourishing he lens,
cornea, and iris (Fig. 2-3). Aqueous drains
hrough he an erior chamber angle, which
con ains he rabecular meshwork ( M) and
ciliary body ace (Fig. 2-4).
Be ween 80% and 90% o aqueous ou ow is
hrough he M— he so-called conven ional
pa hway—wi h he remaining 10% o 20%
hrough he ciliary body ace— he so-called
uveoscleral or al erna ive pa hway. T e M is

hough o be he region where regula ion o
aqueous humor ou ow akes place. Wi hin
he M, especially under condi ions o ele-
va ed IOP, he jux acanalicular area appears
o have he highes resis ance o ou ow
(Fig. 2-5).
IOP is physiologically de ermined by he ra e
o aqueous produc ion in he ciliary body,
resis ance o ou ow hrough he conven ional
ou ow rac ( M and Schlemm’s canal),
resis ance o ou ow hrough he unconven-
ional ou ow rac (uveoscleral ou ow), and
episcleral venous pressure. In he Goldmann
equa ion [P0 = (F/ C) + Pv], P represen s he
IOP, F is he ra e o aqueous orma ion, and
C is he acili y o ou ow, which roughly cor-
responds o he inverse o he o al resis ance
o ou ow. As one can imagine, eleva ions o
episcleral venous pressure can resul in an ele-
va ed IOP (Fig. 2-6).
MEASUREMENT OF
AQUEOUS HUMOR
DYNAMICS
A s discussed above, he basis o IOP can
be described by he Goldmann equa ion.
As mos research sugges s ha he amoun o
uveoscleral ou ow is rela ively insensi ive o
changes in pressure,1 he Goldmann equa ion
can be modif ed as: P = (F − U)/ C + Pv, where
P represen s he IOP, F is he ra e o aqueous
orma ion, U is he ra e o aqueous ou ow
hrough he pressure insensi ive uveoscleral
pa hway, and C is he ou ow acili y. Each
o hese parame ers can be measured excep
U which mus be calcula ed rom IOP and he
remaining variables.
Ou ow acili y is measured clinically using
onography. T e concep o onography
involves placing a weigh ed onome er on
he sur ace o he eye, causing an eleva ion
in IOP, and measuring he ra e a which IOP
Aqueous Flow 5
re urns o i s baseline value over a f xed ime
in erval (usually 2 or 4 minu es).2 Di eren
devices can be used or onography measure-
men s, including weigh ed pneuma onom-
e ers or elec ronic Schio z onome ers. T ese
devices share he charac eris ic o being able
o record IOP con inuously over he measure-
men in erval, ei her on a paper char or elec-
ronically (Fig. 2-7). egardless o he device,
all share he same limi a ions including he
assump ion ha aqueous humor produc ion
ra e, episcleral venous pressure, and ou ow
acili y are cons an during he measuremen
in erval.3 In normal individuals, ou ow acil-
i y is ypically be ween 0.23 and 0.33 µL/
min/ mm Hg.4
Aqueous humor produc ion ra e is measured
using uoropho ome ry.5,6 Wi h his ech-
nique, a uorescein depo is es ablished in
he cornea using eye drops. Over ime, he
uorescein is removed rom he cornea as i
di uses in o he an erior chamber and is car-
ried away by aqueous humor ow. T e ra e o
uorescein removal can be es ima ed by using
a uoropho ome er o measure he change
over ime o uorescence in he cornea and
an erior chamber (Fig. 2-8). T is in orma ion
can hen be used o calcula e he ra e o aque-
ous humor ow, assuming ha di usion in o
he pos erior segmen is minimal (around 10%
or less). Using his echnique, he mean aque-
ous humor produc ion ra e is ypically ound
o range rom 2.2 o 3.1 µL per minu e.7
Episcleral venous pressure is curren ly he
mos di cul parame er o measure in aqueous
humor dynamics. Non-invasive measuremen
involves iden i ying an episcleral vein, plac-
ing a clear pressurized membrane on he vein,
increasing he pressure wi hin he chamber,
and observing he response. T e amoun o
pressure required o produce a response, such
as compression, can be used o es ima e he epi-
scleral venous pressure (Fig. 2-9). T e correc
amoun o compression, however, is subjec ive,
 6 2 BASICS O F AQ UEO US FLO W AND THE OPTIC NERVE
leading o uncer ain y in he measuremen o
episcleral venous pressure. Normal values
repor ed or mean episcleral venous pressure
have ranged rom 7 o 14 mm Hg.8–10
EFE ENCES
1. Alm A, Nilsson SF. Uveoscleral ou ow–a review. Exp
Eye Res. 2009;88(4):760–768.
2. Gran WM. onographic me hod or measuring he
acili y and ra e o aqueous ow in human eyes. Arch
Ophthal. 1950;44(2):204–214.
3. Brubaker F. Goldmann’s equa ion and clinical
measures o aqueous dynamics. Exp Eye Res. 2004;
78(3):633–637.
4. Becker B. onography in he diagnosis o simple
(open angle) glaucoma. Trans Am Acad Ophthalmol
Otolaryngol. 1961;65:156–162.
5. Jones F, Maurice DM. New me hods o measuring
he ra e o aqueous ow in man wi h uorescein. Exp
Eye Res. 1966;5(3):208–220.
6. McLaren JW, Brubaker F. A wo-dimensional scan-
ning ocular uoropho ome er. Invest Ophthalmol Vis
Sci. 1985;26(2):144–152.
7. McLaren JW. Measuremen o aqueous humor ow.
Exp Eye Res. 2009;88(4):641–647.
8. Phelps CD, Armaly MF. Measuremen o episcleral
venous pressure. Am J Ophthalmol. 1978;85(1):35–42.
9. oris CB, Yablonski ME, Wang YL, e al. Aqueous
humor dynamics in he aging human eye. Am J
Ophthalmol. 1999;127(4):407–412.
10. Zeimer C, Gieser DK, Wilensky J , e al. A prac ical
venomanome er. Measuremen o episcleral venous
pressure and assessmen o he normal range. Arch
Ophthalmol. 1983;101(9):1447–1449.
BIBLIOG APH Y
Bill A. T e drainage o aqueous humor. Invest Ophthalmol
Vis Sci. 1975;14:1–3.
Bill A, Phillips CI. Uveoscleral drainage o aqueous
humour in human eyes. Exp Eye Res. 1971;12:
275–281.
Gran WM. Fur her s udies on acili y o ow hrough
he rabecular meshwork. Arch Ophthalmol. 1958;60:
523–533.
Maepea O, Bill A. Pressures in he jux acanalicular is-
sue and Schlemm’s canal in monkeys. Exp Eye Res.
1992;54:879–883.
Maepea O, Bill A. T e pressures in he episcleral veins,
Schlemm’s canal and rabecular meshwork in mon-
keys: E ec s o changes in in raocular pressure. Exp
Eye Res. 1989;49:645–663.
Moses R , Grodzki WJ, E heridge EL, e al. Schlemm’s
canal: T e e ec o in raocular pressure. Invest
Ophthalmol Vis Sci. 1981;20:61–68.
Pederson JE, Gaas erland DE, MacLellan HM.
Uveoscleral aqueous ou ow in he rhesus monkey:
Impor ance o uveal reabsorp ion. Invest Ophthalmol
Vis Sci. 1977;16:1008–1017.
Seiler , Wollensak J. T e resis ance o he rabecular
meshwork o aqueous humor ou ow. Graefes Arch
Clin Exp Ophthalmol. 1985;223:88–91.
 Aqueous Flow 7

A B

FIGURE 2-1. Gross dissection o a cadaver eye.

A. An erior segmen rom a cadaver eye. B. Same
an erior segmen urned over, looking rom behind
he lens. T e lens is s ill suppor ed by he zonular
f bers ex ending rom he ciliary body processes.
C. Higher-magnif ca ion view o he ciliary body
process. T is region is called he pars plica a. T e
C pars plana is peripheral o he pars plica a.

FIGURE 2-2. Hema oxylin and eosin (H&E)–s ained sec ion o he ciliary body. T e mul iple olds help increase
he overall sur ace area.
 8 2 BASICS O F AQ UEO US FLO W AND THE OPTIC NERVE

FIGURE 2-3. Route o aqueous f ow. Schema ic diagram showing he rou e o aqueous rom he ciliary body
o he ou ow rac . (From Rhee DJ, Budenz DL. Acu e angle-closure glaucoma. In: A las o O ce Procedures.
Philadelphia, PA: Saunders; 2000, 3( 2) :267–279.)

FIGURE 2-4. H&E-s ained sec ion o he an erior chamber angle showing he ou ow rac s o he eye.
T e conven ional ou ow pa hway consis s o seven layers o M beams (corneoscleral and uveal M) , he
jux acanalicular region, Schlemm’s canal, collec ing channels, and episcleral veins. T e uveoscleral pa hway
consis s o he uveal ace, wi h ow even ually moving in o he choroidal space. T is pa hway is no well
unders ood. T ere is evidence o show ha he aqueous drains ou he vor ex veins and hrough he scleral wall.
 Aqueous Flow 9

FIGURE 2-5. Con ocal microscopy o the juxtacanalicular region o TM. Green ( uorescein labeled)
indica es s aining or a nonspecif c secre ed ma ricellular pro ein. Red ( exas red labeled) indica es s aining or
smoo h muscle ac in ( wi hin he M endo helial cells) , whereas he blue (DAPI) s ains or nuclear ma erial.
T ese smaller, bubble-shaped nuclei correspond o he cells o he inner wall o Schlemm’s canal, whereas he
elonga ed nuclei correspond o he M endo helial cells. One can see ha he uveal and corneoscleral M
consis s o endo helial cell–lined beams, whereas he jux acanalicular region is an amorphous area o ex racellular
ma rix and M endo helial cells.
 10 2 BASICS O F AQ UEO US FLOW AND THE O PTIC NERVE

A B

D
C

FIGURE 2-6. Extreme example o neglected

bilateral carotid cavernous sinus stula. A–C. T e
chronic eleva ion o venous pressure has resul ed in
dila ion o all he downs ream venous channels wi h
lid involvemen . T e pa ien had eleva ed IOP wi h
modera e generalized cupping in he righ op ic nerve
E (D) and in erior no ch in he le op ic nerve (E).
 Aqueous Flow 11

A
B

FIGURE 2-7. Measurement o acility using tonography. A. Elec ronic Schio z onome er recording o a
paper char ( V. Mueller & Company, Chicago, IL) . B. Digi al Schio z onome er recording o compu er ( Mayo
Clinic, Roches er, MN) . C. Per orming Schio z onography on a subjec . D. Paper char recording o IOP over a
4-minu e in erval. T e slow decay in pressure is recorded as an increase in he Schio z scale. A curve is manually
drawn hrough he da a o de ermine he ra e o IOP decay. E. Recording o IOP and curve f t ing o da a rom a
4-minu e onography measuremen , using a digi al Schio z onome er. T e scale is reversed compared wi h he
paper char .
 12 2 BASICS O F AQ UEO US FLOW AND THE O PTIC NERVE

B
A
FIGURE 2-8. Measurement o aqueous humor production rate. A. Scanning wo-dimensional an erior
segmen uoropho ome er ( Mayo Clinic, Roches er, MN) . B. Per orming uoropho ome ry on a subjec o
measure uorescein concen ra ion in he cornea and an erior chamber.

FIGURE 2-9. Measurement o episcleral

venous pressure. A. Manually opera ed episcleral
venomanome er (Eye ech L d., Mor on Grove, IL) .
B. Sequence o images showing compression o an
episcleral vein ( be ween arrows) during he process o
venomanome ry. T e amoun o pressure required o
crea e an appropria e compression is used o es ima e
episcleral venous pressure. T is can be per ormed
subjec ively wi h direc visualiza ion, or objec ively
A using so ware o de ermine he amoun o compression
rom baseline (Mayo Clinic, Roches er, MN) .
 Optic Nerve 13

Wi hou rea men , an eleva ed IOP can resul

O P IC N ERVE
T he op ic nerve consis s o all he axons
rom he ganglion cell layer o he re ina.
T e op icnerve is he si e o damage in glaucoma
(Figs. 2-10 and 2-11). Func ionally, op ic
nerve damage causes visual f eld changes.
in progressive loss o he visual f eld, even u-
ally leading o blindness. T e various pat erns
o op ic nerve change and means o measur-
ing he unc ional s a us o he op ic nerve
are described in more de ail in subsequen
chap ers.

FIGURE 2-10. Pho ograph o he op ic nerve showing an in erior no ch rom a pa ien wi h glaucoma. No e he
rela ive absence o pallor. (Cour esy o L. Jay Ka z, MD, Wills Eye Hospi al, Philadelphia, PA.)

A B
FIGURE 2-11. H&E stained histopathologic section o optic nerves. A. Normal op ic nerve. B. Op ic nerve
rom advanced glaucoma ( bean po cup). (Cour esy o Ralph J. Eagle, MD, Wills Eye Hospi al, Philadelphia, PA.)
 C H AP T ER
T onome ry is he measuremen o he in ra-
ocular pressure ( he pressure wi hin he
eye). Mos o he ins rumen s used in onom-
e ry rely on de orming an area o he cornea
wi h a small amoun o orce ha is used o
calcula e he in raocular pressure. onome ers
can be divided in o ypes ha applana e, or
f at en, he cornea and hose ha inden i . T e
accuracy o ei her ype o onome er assumes
ha all eyes have a similar ocular rigidi y, cor-
neal hickness, and ocular blood f ow.
GOLDMANN APPLANATION
TONOMETER
A pplana ion onome ry is based on he
Imber –Fick law ha he in raocular
pressure is equal o he amoun o orce needed
o f at en a spherical sur ace divided by he
applana ed area. Goldmann applana ion, he
gold s andard and he mos commonly used
orm o onome ry, was in roduced in 1954.
T is device can be used only in pa ien s sea ed
a he sli lamp. T e cornea is viewed hrough
a prisma ic doubling device in he cen er o a
cone-shaped head ha is obliquely illumina ed
14
onome ry
Rajesh K. Shet y
wi h a cobal blue ligh (Fig. 3-1). While he
pa ien ’s head is held s eady, he applana ion
head is gen ly placed agains a f uorescein-
s ained, anes he ized cornea (Fig. 3-2). T e
examiner sees a spli image o he ear lm
meniscus around he onome er head. T ese
f uorescein rings jus overlap when he pres-
sure a he head equals he in raocular pres-
sure. T e gradua ed dial on he side measures
he orce in grams and is conver ed o millime-
ers o mercury by mul iplying by 10.
Wi h a circular applana ion sur ace o 3.06 mm
in diame er, he sur ace ension o he ear lm
coun erac s he orce needed o overcome he
rigidi y o he cornea, allowing he amoun o
orce applied o equal he in raocular pressure.
T e ip f at ens he cornea less han 0.2 mm,
displaces 0.5 µL o aqueous, increases he in ra-
ocular pressure by 3%, and provides a reliable
measuremen o ±0.5 mm Hg. In corneas wi h
high as igma ism (grea er han 3 diop ers), he
f at es corneal meridian should be placed a
45 degrees o he axis o he cone. T is can
be done simply by placing he red line on he
onome er ip a he same axis o he minus (or
f at es ) cylinder o he eye.
 Goldmann Applanation Tonometer 15

A
B

FIGURE 3-1. Goldmann tonometer. Example o a

Goldmann onome er moun ed on a Haag-S rei sli
lamp. A. T e red lines seen on he cone can be aligned
o he axis o nega ive cylinder in pa ien s wi h high
as igma ism. B, C. Cobal blue illumina ion o he
onome er ip allows or visualiza ion o he f uorescein
C
con aining ear lm.

B
FIGURE 3-2. Applanation technique. A. An individual demons ra ing blepharospasm on at emp ed
applana ion. B. Success ul con ac be ween he onome er ip and he cornea, wi h he examiner demons ra ing
proper echnique o placing suppor ing rac ion only on he orbi al rims, no on he globe i sel .
 16 3 TO NO METRY
SCHIÖTZ TONOMETER
I n roduced in 1905, he Schiö z onome er
is he classic inden a ion onome er, and
requires he pa ien o be supine (Fig. 3-3).
As opposed o applana ion onome ry, he
amoun o inden a ion o he cornea by
he Schiö z onome er is propor iona e o he
in raocular pressure. T is de orma ion, how-
ever, crea es an unpredic able and rela ively
large in raocular volume displacemen . T e
16.5-g Schiö z onome er has a base weigh
o 5.5 g ha is at ached o he plunger. T is
weigh may be increased o 7.5, 10, or 15 g
or higher ocular pressures. T e calibra ed
oo pla e o he onome er is placed gen ly on
he anes he ized cornea, and he ree ver ical
movemen o he at ached plunger de ermines
he scale reading. o es ima e in raocular pres-
sure, conversion ables are available based
on empirical da a rom bo h human cadaver
eyes and in vivo s udies. T e ables assume
a s andard ocular rigidi y such ha in eyes
wi h al ered scleral rigidi y (e.g., a er re inal
de achmen surgery), he Schiö z measure-
men may no be accura e.
 Schiötz Tonometer 17

A
FIGURE 3-3. Schiötz tonometer. A. Image o he Schiö z onome er wi h he 7.5- and 10-g weigh s shown.
B. Schiö z inden a ion onome ry can be used only on pa ien s in a supine posi ion.
 18 3 TO NO METRY

PERKINS TONOMETER source is bat ery powered, and he ins rumen

can be used in ei her a ver ical or a supine posi-

T his handheld Goldmann- ype applana-

ion onome er is especially use ul in
in an s and children (Fig. 3-4). T e ligh
ion. T e orce o corneal applana ion varies by
ro a ing a calibra ed dial wi h he same conical
measuring device as he Goldmann onome er.

FIGURE 3 4. Perkins tonometer. Perkins onome ry is commonly used in he examina ion o in an s under
anes hesia.
 Tono-Pen 19

TONO -PEN rans erred o a surrounding sleeve so ha he

cen ral pla e measures only he in raocular

T he handheld ono-Pen© (Men or Oph-

halmics, San a Barbara, CA) can mea-
sure in raocular pressure in ei her a sea ed or
a supine pa ien (Fig. 3-5). T is echnique is
especially use ul in pa ien s wi h scarred or
edema ous corneas, hose unable o be exam-
ined a a sli lamp, or pedia ric pa ien s. Wi h
a Mackay–Marg- ype onome er such as he
ono-Pen, he e ec s o corneal rigidi y are
pressure. A microprocessor in he ono-Pen
ha is connec ed o a s rain-gauge rans-
ducer measures he orce o he 1.02-mm–
diame er cen ral pla e as i applana es he
corneal sur ace. Measuremen s o our o en
readings will give a nal readou wi h a vari-
abili y be ween he lowes and highes accep -
able readings o less han 5%, 10%, 20%, or
grea er han 20%.

B
FIGURE 3 5. Tono Pen ©. A. T e ono-Pen XL is a handheld device ha does no require a sli lamp. B. Proper
placemen o he ono-Pen is 90 degrees perpendicular o he sur ace o he cornea. T e small diame er o he
ono-Pen makes i also use ul in children.
 20 3 TO NO METRY

PNEUMOTONOMETER needed o overcome corneal rigidi y. T e cen-

ral sensing area is a silas ic diaphragm ha

T his handheld device can also be used

wi hou a sli lamp, wi h he pa ien
sea ed or supine, and on eyes wi h irregular
corneal sur aces (Fig. 3-6). Like he ono-
Pen, his Mackay–Marg- ype onome er has
i s sensing sur ace in he cen er, wi h an adja-
cen surrounding rim ha rans ers he orce
caps an air- lled plunger. When his f exible
diaphragm is applied o he cornea, gas escape
rom he plunger is impeded, allowing he air
pressure o rise un il i balances he in raocular
pressure. An elec ronic ransducer measures
he air pressure in he chamber.

A
FIGURE 3-6. Pneumotonometer. A. T e Pneumo onome er readou includes a paper racing wi h he average
in raocular pressure in mm Hg which demons ra es he rela ionship o he pa ien ’s pulse. B. T e ip mus be held
perpendicular o he cornea wi h he ngers no exer ing orce on he globe.

DYNAMIC CONTOUR
TONOMETRY
D ynamic con our onome er (DC ) or
Pascal onome er is a novel device or
he noninvasive measuremen o in raocular
pressure (Fig. 3-7). T e concave pressure-
sensing ip (10.5 mm radius o curva ure) is
sligh ly f at er han ha o he average human
cornea. Since he con our o he 7-mm rans-
ducer head ma ches ha o he cornea, here is
minimal dis or ion o he cornea. T e 1.7-mm
piezoresis ive pressure sensor a he cen er o
he concavi y measures he in raocular pres-
sure a he cornea 100 imes per second wi h
less han 1 g o apposi ional orce.
FIGURE 3-7. DCT. Dynamic con our onome ry is a novel echnique ha may be less inf uenced by he
s ruc ural charac eris ics o he eye.
Dynamic Contour Tonometry 21
Published s udies sugges ha DC may be
less dependen han applana ion onome ry
on cen ral corneal hickness, corneal curva-
ure, as igma ism, an erior chamber dep h, and
axial leng h. IOP measured by DC correla es
wi h Goldmann applana ion onome ry, how-
ever, DC may have signi can ly higher read-
ings. Variabili y be ween observers and wi h
he same observer over ime may be less wi h
his device han wi h applana ion onome ry.
I is possible o measure bo h he dias olic and
he sys olic in raocular pressures and de er-
mine he di erence be ween he wo, ha
is, he ocular pulse ampli ude. Ocular pulse
ampli ude is an indirec measure o choroidal
per usion and may have a role in he pa ho-
physiology o glaucoma.
 C H AP ER
4
G onioscopy is an examina ion o grea
impor ance or he evalua ion, diagnosis,
and rea men o he pa ien wi h glaucoma. I s
main purpose is visualiza ion o he conf gu-
ra ion o he an erior chamber angle. Under
normal circums ances, he s ruc ures o he
an erior angle canno be seen direc ly hrough
he cornea because o he op ical phenomenon
known as o al in ernal re ec ion. Brie y, his
phenomenon re ers o op ical physics whereby
ligh ha is re ec ed rom he an erior chamber
angle is ben in ernally wi hin he cornea a he
cornea–air in er ace. T e gonioscopy lens (or
goniolens) elimina es his e ec by placing he
lens–air in er ace a a di eren angle, making i
possible o observe he ligh re ec ed rom he
s ruc ures o he angle.
Gonioscopy can be direc or indirec , depend-
ing on he lens employed, wi h a magnif ca ion
o 15 o 20 imes normal.
DIRECT GONIOSCOPY
Direc gonioscopy re ers o a “direc ” view
o he angle. A lens is used o overcome he
in ernal re ec ance o ligh in he cornea o
allow visualiza ion o he angle. T us, one
22
Gonioscopy
Oscar V. Beaujon-Balbi and Oscar Beaujon-Rubin
would examine he nasal angle by looking in
he same direc ion; his is in con radis inc ion
o looking a a mirror o examine he angle
opposi e o he mirror.
T e Koeppe lens is an example o a direc
gonioscopy ins rumen (Fig. 4-1). I requires
a magnif ca ion device (microscope) and a
separa e ligh source.
T e pa ien needs o be in a supine
posi ion.
Direc gonioscopy lenses are o en used in
he opera ing room or angle surgery such as
gonio omy or ab interno rabeculec omy.
Advantages
Direc gonioscopy is use ul in pa ien s
wi h nys agmus and irregular corneas.
Direc gonioscopy is use ul or examina-
ion in children a he o ce under opical
anes hesia. I necessary hey can be seda ed as
usual, and he Koeppe lens allows examina-
ion o bo h he angle and he pos erior pole.
Direc gonioscopy allows a wide and pan-
oramic evalua ion o he angle ha enables
comparison be ween he di eren sec ors and
be ween bo h eyes i wo lenses are placed
simul aneously.
 Direct Gonioscopy 23

Direc gonioscopy allows re roillumina- I is echnically more di cul o per orm.

ion, which is o grea impor ance in di eren- I requires a separa e ligh source and mag-
ia ing congeni al and acquired abnormali ies nif ca ion device (microscopy) wi h less op ic
o he angle (Fig. 4-2). quali y han he examina ion made a he sli
lamp (Fig. 4-3).
Disadvantages
Direc gonioscopy requires ha he pa ien
be in he supine posi ion.

A B
FIGURE 4-1. Direct gonioscopy instruments. A. Direc gonioscopy. B. Koeppe lens.

FIGURE 4-2. Retroillumination. Re roillumina ion wi h he Koeppe lens.

 24 4 GO NIO SCO PY

FIGURE 4-3 Barkan’s device. Barkan’s op ical and

illumina ion device.

INDIRECT GONIOSCOPY
T e angle is visualized wi h a lens ha has
one or more mirrors, allowing he evalua-
ion o he s ruc ures opposi e o he mirror
employed. For evalua ing he nasal quadran ,
he mirror is placed emporally, bu he supe-
rior and in erior orien a ion o he image is
main ained. T e examina ion is per ormed
using he sli lamp.
Since he in roduc ion o Goldmann’s
indirec concep wi h he one-mirror gonio-
lens, mul iple lenses have been developed
(Table 4-1).
Lenses are available wi h wo mirrors
ha enable examina ion o all quadran s
wi h ro a ion o 90 degrees o he lens.
O her lenses, wi h our mirrors, allow eval-
ua ion o he en ire angle wi hou ro a ion.
T e Goldmann and similar lenses have a
con ac sur ace wi h a curva ure radius and
diame er higher han he cornea, requiring
he use o a viscous coupling subs ance.
Zeiss and similar lenses do no require any
coupling subs ance, because he radius o
curva ure is similar o ha o he an erior
cornea. T ese lenses also have a smaller
con ac sur ace diame er, and he ear f lm
f lls he cornea–lens space (Fig. 4-4).
TABLE 4-1. Charac eris ics o Goniolenses
Corneal Radius
Lens Diameter (mm) (mm)
Goldmann, three mirrors 12 7.4
Goldmann, one mirror 12 7.4
Zeiss, three mirrors 11 7.7
Zeiss, our mirrors 9 7.85
Allen T orpe 10 8.15
Sussman OS4M 9 —
Indirect Gonioscopy 25
Proper selec ion o he ype o goniolens
is crucial o per orm an e ec ive gonioscopy.
o aid his selec ion, some aspec s should be
considered. Wi hou using a goniolens, he
an erior chamber dep h can f rs be es ima ed
using he Van Herick–Sha er me hod. I a
wide-open angle is suspec ed, any lens could
be employed because here is no elemen ha
would preven visualiza ion o he angle
(Fig. 4-5).
I a shallow angle is suspec ed, i is pre er-
able o use a one- or wo-mirror Goldmann or
a Zeiss lens. T e mirrors o hese goniolenses
are higher and closer o he cen er, enabling
visualiza ion o he s ruc ures ha would be
o herwise occluded by he an erior displace-
men o he lens–iris diaphragm.
o bet er explain his concep , see
Figure 4-6. Imagine an observer s and-
ing a poin A who wan s o see a house
ha is placed behind a hill. T e hill in his
example resembles he iris convexi y. o
solve he problem, he observer could go
o a higher poin , B, ha enables him o
see he house, or move closer o he cen er
( op o he hill), poin A′, or even bet er,
go o poin B′, which allows comple e
observa ion o he house and surrounding
elemen s.
Peripheral Distance to Mirror
Curve (mm) Center (mm) Height (mm)
3 7 12
1.5 3 17
3.5 7 20
— 5 12
— 5 7
— — 15
 26 4 GO NIO SCO PY

B
A

FIGURE 4-4. Types o goniolenses. A. Indirec

gonioscopy using a Goldmann one-mirror lens.
B. Zeiss our-mirror ype o indirec goniolens, which
C uses a handle. C. Sussman our-mirror ype o indirec
goniolens, which is handheld.

FIGURE 4-5. Diagram o an open angle conf guration. T is f gure shows ha wi h an open angle, you can
view any objec in a re ec ive mirror, no mat er he heigh or dis ance rom he cen er, because you do no have
any in er erence.
 Indirect Gonioscopy 27

FIGURE 4-6. Observer and obstacle. T is f gure shows ha when here is an obs ruc ion (in his example, he
hill; wi h gonioscopy, he convex iris o a narrow angle) , i is bet er o be higher and closer o he cen er. T is is
analogous o using a goniolens whose mirrors are higher and closer o he cen er.
 28 4 GO NIO SCO PY
ESTIMATING THE
ANTERIOR CH AMBER
DEPTH
Be ore evalua ing he an erior chamber
angle conf gura ion, he Van Herick–Sha er
echnique is used o es ima e he an erior
angle dep h. T e procedure is per ormed
while evalua ing he pa ien wi h he sli lamp.
Wi h he hinnes sli beam possible, he cor-
nea is illumina ed perpendicularly near he
emporal limbus (crea ing an op ical sec ion)
and viewed a 50 o 60 degrees rom he sli
incidence. o es ima e he an erior chamber
dep h, he ra io be ween he cornea–iris dis-
ance and corneal hickness is observed.
I he separa ion o he cornea–iris is
more han 50% o he corneal hickness,
he an erior chamber is mos likely
deep wi h a wide-angle conf gura ion
(Fig. 4-7A,B). On he o her hand, i i is
less han 50%, a narrow angle is suspec ed
(Fig. 4-7C,D).
T e angle can be graded as ollows:
Grade 0 (closed)—when he iris is con-
ac ing he corneal endo helium
Grade I—when he space be ween he
iris and cornea is less han 25% o corneal
hickness
Grade II—when he space is 25%
Grade III—when i is 25% o 50%
Grade IV—when i is higher han 50%
T is echnique does no subs i u e goni-
oscopy, bu i can be o grea help in es ima -
ing he ampli ude o he an erior chamber,
especially in pa ien s wi h opaci ies or cloudy
corneas.
 Estimating the Anterior Chamber Depth 29

D
FIGURE 4-7. Van Herick’s technique or angle depth estimation. A. Schema ic showing proper placemen
o he sli beam; magnif ed view shows ha he dep h o he an erior chamber (AC) ( black) is grea er han
50% o he corneal sli beam (whi e) , es ima ing a wide angle. B. Demons ra ion o he preceding placemen
in a live pa ien . In his example, he AC dep h is approxima ely 90% o he corneal sli beam. C. Schema ic
showing proper placemen o he sli beam; magnif ed view shows ha he AC dep h ( black) is less han 50% o
he corneal sli beam (whi e) , es ima ing a narrow angle. D. Demons ra ion o he preceding placemen in a live
pa ien . In his example, he AC dep h is approxima ely 10% o 15% o he corneal sli beam.
 30 4 GO NIO SCO PY

TECHNIQUE o evalua e he superior and in erior

A drop o anes he ic is adminis ered o
bo h eyes, and he examina ion is per ormed
a he sli lamp. Depending on he lens
employed, a viscous coupling subs ance may
be required. T e goniolens mus be placed
gen ly on he eye, while rying o avoid dis or-
ion o he in raocular elemen s (Figs. 4-8
and 4-9). o ob ain a good view o he angle,
he incidence o he ligh beam mus be per-
pendicular o he mirror o he goniolens.
Some adjus men s on he sli lamp have o
be made as he evalua ion is per ormed:
angles, he pa ien is asked o look a he
ligh source.
o evalua e he nasal and emporal
angles, he illumina ion source is inclined
orward and he goniolens is shi ed
sligh ly downward, and he pa ien is asked
o look o he side being evalua ed.
T ese simple echnical de ails are vi al o
enable evalua ion o narrow angles and o
iden i y he di eren elemen s o he angle,
especially Schwalbe’s line.

A B

C FIGURE 4-8 Goldmann goniolens. Placing o a

Goldmann one-mirror– ype goniolens.
 echnique 31

FIGURE 4-9. Zeiss goniolens. Placing o a Zeiss- ype goniolens.

 32 4 GO NIO SCO PY
ELEMENTS OF THE ANGLE
ANATOMY
In Figure 4-10, he di eren elemen s
o be iden if ed during gonioscopy are illus-
ra ed. T e angle ex ends rom he las iris
old o Schwalbe’s line. T e angle s ruc ures
can be divided in o wo groups:
A f xed por ion ha includes Schwalbe’s
line, he rabecular meshwork, and he
scleral spur
A mobile por ion ha includes he
an erior-superior ace o he ciliary body
and he iris inser ion, wi h i s las old
T e examiner mus make a general inspec-
ion in order o iden i y some impor an aspec s:
Iris plane— his can be planar on wide
angles and very convex on narrow angles.
Las iris old and i s dis ance rom
Schwalbe’s line— hese wo elemen s are
used o es ima e he ampli ude o he angle.
T e superior angle por ion is generally nar-
rower han he o her por ions.
Iris roo — his represen s he inser-
ion on he ciliary body. I is he hinnes
por ion, and he mos easily displaced
wi h eleva ion o he pos erior chamber
A B
FIGURE 4-10. Angle structure elements. A. Schwalbe’s line (S) , scleral spur (E) , and ciliary body (C) .
B. Angle s ruc ure elemen s in a human cadaver eye. S.L., Schwalbe’s line; S.S., scleral spur.
pressure. In myopic eyes, he iris is larger
and hinner, wi h numerous cryp s, and is
usually inser ed more pos erior on he cili-
ary body. On he o her hand, in hyperopic
eyes, he iris ends o be hicker and i s
inser ion is more an erior han in emme-
ropic eyes, resul ing in a narrower angle
conf gura ion.
Iris nodules, cys s, nevi, or oreign bod-
ies (Fig. 4-11).
Mos o he ime, i is easy o iden i y
one elemen , making i possible o discern
he o hers. For example, Figure 4-12 shows
Schlemm’s canal f lled wi h blood. T is is a
requen observa ion in pa ien s wi h low
in raocular pressure or hose wi h increased
pressure in he episcleral veins. An elevation
in episcleral venous pressure can occur when he
examiner presses he lens oo s rongly on o
he sclera during gonioscopy.
Pa hologically, i can occur in cases o
secondary glaucoma such as hose caused by
increased episcleral venous pressure, S urge–
Weber syndrome, caro id cavernous f s ula,
or in some cases o iridocycli is in which he
venous pressure o conges ive eyes could be
eleva ed, causing a passage o he blood rom
he venous sys em o Schlemm’s canal.
 Elements o the Angle Anatomy 33

A B

FIGURE 4-11. Iris cyst. A. Sli -lamp pho ograph

showing an iris mass in eriorly. B. Sli beam showing
he same. C. Gonioscopic view o he in erior angle
showing he cys ic mass. T e cys ic na ure o his mass
C was conf rmed by ul rasound biomicroscopy ( UBM)
(no shown) .

FIGURE 4-12. Schlemm’s canal. Schlemm’s canal f lled wi h blood (arrow) .

 34 4 GO NIO SCO PY
IDENTIFICATION OF ANGLE
STRUCTURES
Schwalbe’s line represen s he end o
Desceme ’s membrane and marks he an e-
rior limi o he angle. In some pa ien s,
Schwalbe’s line can be hickened wi h pro ru-
sion o he an erior chamber, which has been
ermed pos erior embryo oxon (Fig. 4-13).
Pigmen requen ly deposi s around
Schwalbe’s line, because i marks he ransi-
ion be ween he corneal and scleral curva-
ures. T is change o curva ure produces a
s ep ha acili a es deposi ion o pigmen
and o her ma erials. I Schwalbe’s line is no
visible, i can easily be localized by aiming a
hin sli beam on he angle. T is beam gives
re exes on bo h he an erior and he pos erior
sur aces o he cornea. T e inner re ec ion
line corresponds o he pos erior sur ace o
he cornea and is con iguous wi h he angle
s ruc ures and iris sur ace. T e ou er,
corresponding o he an erior cornea, ends
jus where Schwalbe’s line is loca ed, a he
poin where bo h ligh beams are joined
(Figs. 4-10A and 4-14). T is maneuver is
very impor an or evalua ing a narrow angle
and di eren ia ing i rom a closed angle
(Fig. 4-15).
In Figure 4-16, he ollowing elemen s
can be observed:
rabecular meshwork ha ex ends rom
he scleral spur o Schwalbe’s line
Schlemm’s canal, si ua ed jus an erior
o he scleral spur. T e scleral spur has a
grayish appearance in young pa ien s and
becomes more pigmen ed wi h age. In
he f gure, a pigmen band is eviden , cor-
responding o he pigmen ed rabecular
meshwork.
Scleral spur, a whi er line jus in erior o
he rabecular meshwork. T is serves as he
inser ion o he longi udinal por ion o he
ciliary muscle.
Las iris old, and an erior inser ion
o he ciliary body. T e posi ion where i
is inser ed de ermines in major par he
ampli ude o he chamber angle. T e vis-
ible por ion o he ciliary body ex ends
rom i s inser ion o he scleral spur and
is covered an eriorly by rabecular mesh-
work, ermed uveal meshwork.
Some imes, his issue is ormed o wide
and mul iple bands ha inser on he scleral
spur, bu hey may also ex end over he ra-
becular meshwork. T e las ones are called
iris processes (Fig. 4-17). Iris processes can
overpass he rabecular meshwork, coa ing
he angle, and inser on o Schwalbe’s line, rep-
resen ing a minor mani es a ion o abnormal
embryologic developmen (Fig. 4-18).
I is impor an o avoid con using iris
processes wi h peripheral an erior synechiae,
which are produc s o adherence o he
peripheral iris o any o he angle s ruc ures,
mos o en Schwalbe’s line or he peripheral
cornea. Peripheral an erior synechiae are
observed as en s passing over he angle
(Fig. 4-19B).
 Identifcation o Angle Structures 35

A B
FIGURE 4-13. Schwalbe’s line. A. Pos erior embryo oxon ( arrow) . B. Schwalbe’s line in gonioscopy ( arrow) .

A B
FIGURE 4-14. Schwalbe’s line. A. Schwalbe’s line localiza ion using he edges o he corneal sli beam.
T e di eren beam re exes are shown; “b” corresponds o an erior cornea and “a” o pos erior cornea.
B. Gonioscopic view demons ra ing ha Schwalbe’s line is loca ed where he an erior and pos erior ligh re exes
o he corneal sli beam converge. S.L., Schwalbe’s line ( arrow) . (A, Reproduced wi h permission rom Beaujon-
Rubin O, ed. Glaucoma Primario: Diagnos ico & ra amien o. Caracas, Venezuela: Venezuelan Socie y o
Oph halmology; 1983.)

FIGURE 4-15. Schwalbe’s line. Schwalbe’s line localiza ion using he corneal sli beams in a narrow angle.
 36 4 GO NIO SCO PY

FIGURE 4-16. Gonioscopy. Open angle. .M., rabecular meshwork; S.S., scleral spur.

FIGURE 4-17. Iris processes. Gonioscopic view o he an erior chamber angle demons ra ing iris processes
(I.P.; arrow) .
 Identifcation o Angle Structures 37

FIGURE 4-18. Iris processes. Iris processes inser ing on o Schwalbe’s line ( arrow) .

A B
FIGURE 4-19. Peripheral anterior synechiae. Examples o peripheral an erior synechiae ( arrow) .
 38 4 GO NIO SCO PY

CLASSIFICATION Grade II—20 degrees; angle closure is

OF THE ANGLE possible.
Grade I—10 degrees; angle closure is
Among he objec ives o gonioscopy is o likely.
de ermine he ampli ude o he angle, and Sli —angle is less han 10 degrees;
o de ermine whe her he glaucoma is o he angle closure is more likely.
open-angle or closed-angle ype. Each ype
Closed—iris is s uck o he meshwork
has a di eren epidemiology, physiopa hol-
(Fig. 4-21).
ogy, rea men , and preven ion.
Spae h’s classif ca ion adds de ail regarding
Sha er’s classif ca ion (Fig. 4-20) es i-
peripheral iris and he e ec s o inden a ion
ma es he angle ampli ude be ween he
on he conf gura ion o he angle (Fig. 4-22).
las iris old and he rabecular meshwork–
Schwalbe’s line, as ollows:
Grade IV—45 degrees
Grade III—30 degrees

FIGURE 4-20. Sha er ’s classif cation. Diagram o Sha er’s classif ca ion o angle ampli ude.
 Classifcation o the Angle 39

FIGURE 4-21. Narrow angle. Aspec o he narrow angle on gonioscopy. No e he marked convexi y o he iris,
some imes re erred o as iris bowing. T e angle s ruc ures are di cul o visualize.

B
FIGURE 4-22. Spaeth’s classif cation. Spae h’s classif ca ion, which provides addi ional in orma ion and de ail.
(Cour esy o Dr. George L. Spae h, Wills Eye Hospi al, Philadelphia, PA.)
 40 4 GO NIO SCO PY
PIGMENT DEPOSITION
AND GONIOSCOPY
T e amoun o pigmen deposi ion in
he angle varies widely among individuals.
Some imes he pat ern can serve as a diagnos-
ic ool o de ermine he underlying mecha-
nism. Some examples are described nex .
Pigmentary Glaucoma
In his condi ion, a highly dense band
o brown pigmen is deposi ed on he ra-
becular meshwork in a homogeneous ashion
(Fig. 4-23A). T is pigmen is observed
on he pos erior lens capsule and on he cor-
neal endo helium (Krukenberg’s spindle)
(Fig. 4-23B).
Lens Pseudoexfoliation
T is en i y occurs when ma erial o amor-
phous subs ance is deposi ed on and an erior
o Schwalbe’s line in an undula ing pat ern
known as Sampaolesi’s sign (Fig. 4-24).
T e ma erial also deposi s on lens zonule
and can be observed during gonioscopy
(Fig. 4-25).
Uveitis
In cases o uvei is, irregular areas o pig-
men deposi s can be observed, giving an
appearance o a “dir y” angle (Fig. 4-26).
Angle-closure Glaucoma
In cases o angle-closure glaucoma, a
pa chy area o pigmen may be observed on
any angle s ruc ure, an indica ion ha he
iris was s uck a ha place bu a permanen
adherence did no develop. T e presence o
pa chy pigmen and a narrow angle can be
an indica ion o a previous episode o acu e
angle-closure glaucoma (Fig. 4-27).
Vasculariza ion is usually absen on he
angle. Some imes, small branches o he cili-
ary body’s ar erial circle can be observed.
T ese branches are usually covered by he
uveal meshwork and orm a circum eren ial
serpiginous pat ern or can be observed radi-
ally oward he iris sphinc er.
In neovascular glaucoma, abnormal vessels
cross over he ciliary body and arborize he
rabecular meshwork. Con rac ion o myo-
f brils o he f broblas s ha accompany he
abnormal vessels causes peripheral an erior
synechiae and angle closure (Fig. 4-28).
 Pigment Deposition and Gonioscopy 41

A B
FIGURE 4-23. Pigmentar y glaucoma. A. Pigmen ary deposi ion on he rabecular meshwork (arrow) in an
eye wi h pigmen dispersion syndrome. B. Pigmen ary deposi ion on he pos erior lens capsule ( Zen meyer line,
arrow) in an eye wi h pigmen dispersion syndrome.

FIGURE 4-24. Lens pseudoex oliation. Sampaolesi’s sign ( arrow) .

FIGURE 4-25. Lens pseudoex oliation. Deposi o pseudoex olia ion ma erial on he lens zonule (arrow) .
 42 4 GO NIO SCO PY

FIGURE 4-26. Uveitis. Irregular pigmen deposi s on he angle in a pa ien wi h uvei is (arrow) .

FIGURE 4-27. Angle closure glaucoma. Pigmen pa ches ormed a er angle-closure crisis ( arrow) .
 Pigment Deposition and Gonioscopy 43

A B
FIGURE 4-28. Neovascular glaucoma. A. Fibrovascular membrane over he angle ( arrow) . A his s age, he
angle is open bu occluded. T ere is marked corneal edema, giving a hazy view. B. Diagram o a f brovascular
membrane growing over he angle and causing peripheral an erior synechiae rom con rac ion in neovascular
glaucoma.
 44 4 GO NIO SCO PY
ERROR FACTORS
ON GONIOSCOPY
When per orming gonioscopy, he exam-
iner mus be aware ha some maneuvers al er
he precision o he procedure. T e gonios-
copy lens can deepen he ampli ude o he
angle i oo much pressure is applied o he
sclera by orcing a uid movemen oward he
angle (Fig. 4-29).
T is inden a ion gonioscopy is invaluable
in evalua ing angle-closure glaucoma, espe-
cially in di eren ia ing iris apposi ion rom
FIGURE 4-29. Error actors in gonioscopy. Placing obliquely direc ed pressure on he sclera.
real synechiae. For his maneuver, he Zeiss-
ype gonioscopy lens is recommended.
T e procedure, which is called dynamic
gonioscopy, employs he mechanical e ec
on aqueous humor ha ollows he cor-
neal inden a ion, enabling he examiner
o al er he rela ive posi ion o he iris in
a dynamic way. T is maneuver helps o
dis inguish narrow rom closed angles and
o de ermine he risk o closure. An excess
o pressure produces olds on Desceme ’s
membrane ha makes evalua ion o he
angle di cul (Fig. 4-30).
 Error Factors on Gonioscopy 45

A B

FIGURE 4-30. Dynamic gonioscopy. A. Schema ic demons ra ing dynamic, compression, or inden a ion
gonioscopy. B. Dynamic gonioscopy demons ra ing peripheral an erior synechia orma ion (C.A., closed angle)
and chronic angle-closure glaucoma in a pa ien wi h narrow angles. Par o he angle is s ill open (O.A., open
angle) .
 46 4 GO NIO SCO PY

USE OF GONIOSCOPY covered by he longi udinal por ion o he

IN TRAUMA ciliary muscle (Fig. 4-33).

Cyclodialysis
Contusion Trauma
Cyclodialysis is a comple ed dehiscence
When he cornea is hi , a wave o uid
o he ciliary body rom he sclera, opening a
abrup ly orms. T is wave moves oward he
communica ion pa hway o he suprachoroi-
angle because he iris–lens diaphragm ac s
dal space (Fig. 4-34).
as a valve, preven ing he uid rom going in
a pos erior direc ion. T is uid movemen T ese gonioscopic pat erns can be ound
can harm he s ruc ures o he angle, crea ing in he same pa ien and are requen ly accom-
acu e lesions ha are rela ed o rauma in en- panied by hyphema.
si y (Fig. 4-31).
Iridodialysis
Separa ion o he iris inser ion rom he
Iridodialysis occurs when here is sepa-
scleral spur, ermed iridodialysis, causes one
ra ion o he iris inser ion rom he scleral
o hese lesions (Fig. 4-32).
spur.
Angle Recession
Angle recession occurs when he ciliary
body is separa ed, leaving he ex ernal wall

FIGURE 4-31. Contusion trauma. Diagram o blun rauma o he eye.

 Use o Gonioscopy in rauma 47

FIGURE 4-32. Iridodialysis. T e iris roo (arrow) has allen, exposing he underlying ciliary body processes.

A B
FIGURE 4-33. Angle recession a er trauma. A. Ex ensive angle recession a er rauma. In his example,
he normal angle inser ion is no visible, which could ool an examiner in o hinking ha he angle is normal.
B. Angle recession a er rauma. In his example, here is a smaller degree o angle recession and he border
be ween he recessed angle and he normal angle is seen. A.R., angle recession; S.S., scleral spur; .M., rabecular
meshwork.

FIGURE 4-34. Cyclodialysis. T e ciliary body is comple ely de ached, exposing he underlying sclera (arrow).
 C H AP T ER

An erior Segmen Imaging

Sung Chul Park, Syril Dorairaj, Jef rey M. Liebmann, and Robert Ritch

A n erior segmen ul rasound biomi-

croscopy (UBM) uses high- requency
ransducers (35 o 75 MHz) o provide in
vivo imaging o he an erior segmen wi h an
axial resolu ion o 30 o 70 µm and pene ra-
ion dep h o 2 o 7 mm. T e s ruc ures sur-
rounding he pos erior chamber, hidden rom
clinical observa ion, can be imaged and heir
ana omic rela ionships assessed.
UBM has been used o inves iga e bo h he
normal s ruc ure and disease mechanisms and
pa hophysiology in many areas o oph halmol-
ogy, including glaucoma, cornea, lens, congen-
i al abnormali ies, e ec s and complica ions o
surgical procedures, an erior segmen rauma,
cys s and umors, and uvei is. S udies using
UBM were ini ially primarily quali a ive, bu
quan i a ive s udies have become increas-
ingly common.1 T ree-dimensional analysis o
UBM images is s ill in i s in ancy.
An erior segmen op ical coherence omo-
graphy (AS-OC ) uses ligh ins ead o
ul rasound and ransmi s signals o shor er
waveleng h (1,310 nm), which produce
images o higher resolu ion (axial resolu ion
o 15 µm) han UBM.2 I enables noncon ac
imaging because he re rac ive index be ween
air and issue is much less han he acous ic
impedance be ween hem. Dynamic rela ion-
ships be ween he iris, angle wall, and lens
can be assessed hrough real- ime limbus- o-
limbus cross-sec ional images because o i s
as er scan speed, reducing eye movemen
ar i ac s. AS-OC also provides sof ware or
au oma ic measuremen o various cornea
and an erior chamber parame ers (Fig. 5-1).
However, he ciliary body is rarely visualized
owing o he pigmen ed pos erior layer o he
iris, which blocks ligh pene ra ion. AS-OC
has been used similarly o UBM, bu , because
o i s charac eris ics, has been more use ul in
quan i a ive analysis o he an erior cham-
ber and in corneal disease or surgery, such as
kera oplas y.
T ese imaging devices do no replace con-
ven ional sli -lamp biomicroscopy or gonios-
copy, bu supplemen and augmen clinical
prac ice and provide invaluable research ools.
Charac eris ics o UBM and AS-OC are
compared in Table 5-1.

48

TABLE 5-1. Characteristics o UBM and AS-OC
UBM
Signal source Ultrasound
Resolution (µm) 30–70
Tissue penetration Up to 7 mm, ciliary body visualized
Image width (mm) 4–7
Tissue contact Yes (needs fuid coupling medium)
Image acquisition time Slower
Quantitative analysis Manual
ANGLE-CLOSURE
GLAUCOMA
B ecause o i s abili y o image he ciliary
body, pos erior chamber, iris–lens rela-
ionships, and angle s ruc ures simul aneously,
UBM is ideally sui ed o he s udy o angle clo-
sure. Signi can correla ions have been ound
be ween angle measuremen s by AS-OC ,
UBM, and gonioscopy.3,4 When assessing a
narrow angle or occludabili y, gonioscopy in a
comple ely darkened room, using he smalles
square o ligh or a sli beam o avoid s imu-
la ing he pupillary ligh re ex, is o u mos
impor ance. T e e ec o ambien ligh on
he angle con gura ion is well illus ra ed by
per orming UBM under illumina ed and dark-
ened condi ions (Fig. 5-2).
Because mos o he impor an an erior cham-
ber angle parame ers or quan i a ive mea-
suremen are based on he iden i ca ion o
he scleral spur, reliable documen a ion o he
angle dimensions using UBM or AS-OC is
here ore dependen on i s precise and repea -
able localiza ion. In a UBM or AS-OC
image, he scleral spur can be seen as he
innermos poin o he line separa ing he cili-
ary body and he sclera a i s poin o con ac
wi h he an erior chamber. Al hough i canno
be visualized wi h UBM or AS-OC , he ra-
becular meshwork is loca ed direc ly an erior
Angle-Closure Glaucoma 49
AS-OCT
In rared light
15
Ciliary body rarely visualized
15–16
No
Faster
Automatic
o his s ruc ure and pos erior o Schwalbe’s
line (Fig. 5-3).
Cornea and angle s ruc ures are less dis or ed
during AS-OC because o i s noncon ac
na ure, avoiding ar i ac s induced by inadver-
en pressure on he cornea during gonioscopy
or on limbal issues wi h he eye cup during
UBM. Di eren ia ion o apposi ional and
synechial angle closure in eyes wi h irido ra-
becular con ac by inden ion AS-OC adds o
i s clinical u ili y in he evalua ion o pa ien s
wi h angle closure.5 An erior chamber dep h
and volume measured using AS-OC may be
use ul parame ers or de ec ing individuals a
risk o developing primary angle closure.
Angle closure can be classi ed by he si e o
he ana omic s ruc ure or orce causing iris
apposi ion o he rabecular meshwork. T ese
are de ned as block origina ing a he level o
he iris (pupillary block), ciliary body (pla-
eau iris), lens (phacomorphic glaucoma),
and orces pos erior o he lens (malignan
glaucoma).
ELATIVE PUPILLA YBLOCK
Rela ive pupillary block is responsible or over
90% o he angle closure in Caucasian popula-
ions. In pupillary block, resis ance o aqueous
ow rom he pos erior o he an erior chamber
hrough he pupil and he resul ing increased
 50 5 ANTERIO R SEGMENT IMAGING
aqueous pressure in he pos erior chamber
orces he iris an eriorly (Fig. 5-4A), causing
an erior iris bowing and angle narrowing. An
an eriorly convex con gura ion o he en ire
iris can be imaged using AS-OC (Fig. 5-5).
Pupillary block may be absolu e, i he iris
is comple ely bound o he lens by pos e-
rior synechiae, bu mos of en is a unc ional
block, ermed relative pupillary block. Rela ive
pupillary block usually causes no symp oms.
However, i i is su cien o cause apposi-
ional closure o a por ion o he angle wi hou
eleva ing in raocular pressure (IOP), periph-
eral an erior synechiae may gradually orm
and lead o chronic angle closure (Fig. 5-6).
I he pupillary block becomes absolu e, he
pressure in he pos erior chamber increases
and pushes he peripheral iris ar her orward
o cover he rabecular meshwork and close
he angle wi h an ensuing rise o IOP (acu e
angle closure) (Fig. 5-7).
Laser irido omy elimina es he pressure di -
eren ial be ween he an erior and pos erior
chambers and relieves he iris convexi y. T is
resul s in several changes in an erior segmen
ana omy. T e iris assumes a a or planar con-
gura ion (Fig. 5-4B), and he iridocorneal
angle widens. T e region o iridolen icular
con ac ac ually increases, as aqueous ows
hrough he irido omy ra her han he pupil-
lary space.
PLATEAU I IS
In pla eau iris, he ciliary processes are ei her
large or an eriorly si ua ed, or bo h, so ha
he ciliary sulcus is obli era ed and he cili-
ary body suppor s he iris agains he ra-
becular meshwork. T e an erior chamber is
usually o medium dep h and he iris sur ace
only sligh ly convex. Argon laser peripheral
iridoplas y con rac s and compresses he
peripheral iris, pulling i away rom he rabecu-
lar meshwork (Fig. 5-8).6 Al hough large or
an eriorly si ua ed ciliary processes are rarely
visualized by AS-OC , i can be used o con rm
a clinical suspicion o pla eau iris con gura ion
(Fig. 5-9).7
PHACOMO PHIC GLAUCOMA
Lens enlargemen may cause shallowing o he
an erior chamber and precipi a e acu e angle
closure by orcing he iris and ciliary body an e-
riorly. Mio ic herapy increases he lens axial
leng h and causes i o move an eriorly, which
ur her shallows he an erior chamber, and may
paradoxically worsen he si ua ion (Fig. 5-10).
AS-OC is use ul in his condi ion, because
an erior chamber dep h, iris con gura ion, and
angle s ruc ures can be evalua ed a a glance.
MALIGNANT GLAUCOMA
Malignan (ciliary block) glaucoma is a mul i-
ac orial disease in which he ollowing com-
ponen s may play varying roles: (1) previous
acu e or chronic angle closure, (2) shallow
an erior chamber, (3) orward lens move-
men , (4) pupillary block by he lens or vi re-
ous, (5) zonular laxi y, (6) an erior ro a ion
or swelling o he ciliary body, or bo h, (7)
hickening o he an erior hyaloid membrane,
(8) vi reous expansion, and (9) pos erior aque-
ous displacemen in o or behind he vi reous.
UBM reveals a shallow supraciliary de ach-
men , no eviden on rou ine B-scan or clini-
cal examina ion. T is e usion appears o be
he cause o he an erior ro a ion o he ciliary
body. Aqueous humor is secre ed pos erior o
he lens (pos erior aqueous displacemen ),
increasing vi reous pressure, pushing he lens–
iris diaphragm orward, and causing angle clo-
sure and shallowing o he an erior chamber
(Fig. 5-11). Al hough changes in he shape
or posi ion o he ciliary body canno be accu-
ra ely assessed, an an eriorly displaced iris–lens
diaphragm and shallow an erior chamber are
well demons ra ed using AS-OC (Fig. 5-12).
 Angle-Closure Glaucoma 51

PSEUDOPHAKIC lens implan a ion. Forward displacemen o

PUPILLA YBLOCK
An erior chamber in amma ion af er ca arac
ex rac ion can lead o pos erior synechiae be-
ween he iris and a pos erior chamber in ra-
ocular lens, producing absolu e pupillary block
and angle closure. An erior chamber lenses can
also underlie pupillary block (Fig. 5-13).
PSEUDOPHAKIC MALIGNANT
GLAUCOMA
Malignan glaucoma may occur af er ca arac
surgery wi h pos erior chamber in raocular
he vi reous in o apposi ion wi h he iris and
ciliary body, possibly associa ed wi h hicken-
ing o he an erior hyaloid, has been proposed
as he mechanism o accoun or he pos e-
rior diversion o aqueous ow. UBM reveals
marked an erior displacemen o he in raocu-
lar lens (Fig. 5-14). Nd:YAG laser an erior
hyaloido omy may be cura ive.

A B
FIGURE 5-1. Measurement o cornea and anterior chamber parameters using AS OCT. Corneal thickness,
corneal radius o curvature, anterior chamber depth and volume, pupil diameter, and distance between scleral
spurs (A), as well as anterior chamber angle parameters such as AOD500 (angle opening distance at 500 µm
rom the scleral spur) , ISA500 (trabecular–iris space area at 500 µm rom the scleral spur), and IA500
(trabecular–iris angle at 500 µm rom the scleral spur) (B) can be measured using AS-OC .

A B
FIGURE 5-2. Ef ect o ambient light on angle con guration. A. Under light conditions the angle is open.
Aqueous has access to the trabecular meshwork (arrows) . B. In the dark, the angle is capable o occlusion
(arrows) .
 52 5 ANTERIO R SEGMENT IMAGING

A B
FIGURE 5-3. Anatomy o normal eye. UBM (A) and AS-OC (B) o normal eye showing anterior chamber
(AC) , cornea (C) , ciliary body (CB), iris (I) , lens capsule (LC) , posterior chamber (PC) , sclera (S) , scleral spur
( black arrow), Schwalbe’s line ( vertical white arrow) , and angle recess ( horizontal or oblique white arrows) .

A B
FIGURE 5-4. Iris con guration be ore and a er laser iridotomy (UBM). Convex iris conf guration
(arrowheads) be ore (A) and planar conf guration a er (B) laser iridotomy in an eye with relative pupillary block.

FIGURE 5-5. Iris con guration with relative pupillar y block (AS OCT). Convex conf guration o entire iris
(arrowheads) is visualized in one rame.
 Angle-Closure Glaucoma 53

FIGURE 5-6. Peripheral anterior synechiae (arrows) (UBM).

FIGURE 5-7. Iris con guration with absolute pupillar y block (AS OCT). Extremely convex iris with absolute
pupillary block caused by 360-degree posterior synechiae ( arrows) .

A B
FIGURE 5-8. Plateau iris syndrome (UBM). A. In plateau iris syndrome, the angle remains closed (arrowhead)
a er laser iridotomy because the ciliary processes are large and anteriorly positioned. T e ciliary sulcus is absent
(asterisk) . B. Following peripheral iridoplasty, the appositional angle closure is relieved.
 54 5 ANTERIO R SEGMENT IMAGING

FIGURE 5-9. Plateau iris syndrome (AS OCT). Plateau conf guration o the iris is prominent, although the
ciliary body is not visualized.

FIGURE 5-10. Phacomorphic glaucoma (UBM). T e intumescent lens (L and arrowheads) pushes the iris (I)
and ciliary body into the angle.

FIGURE 5-11. Malignant glaucoma (UBM). Malignant glaucoma can result rom aqueous misdirection or
rom annular ciliary body detachment. T e ciliary body (CB) is rotated anteriorly (white arrow) . Fluid is visible in
the supraciliary space. S, sclera; I, iris.
 Angle-Closure Glaucoma 55

FIGURE 5-12. Malignant glaucoma (AS OCT). Anteriorly pushed iris and lens result in angle closure with
shallow anterior chamber. Ciliary body is not visualized.

A B
FIGURE 5-13. Pseudophakic pupillar y block. A. T is eye shows peripheral anterior ( black arrows) and
posterior synechiae ( white arrows) , resulting in an iris bombé conf guration. B. A er laser iridotomy, the iris
conf guration is at while the peripheral anterior synechiae ( black arrows) still hold the iris root to the trabecular
meshwork.

FIGURE 5-14. Pseudophakic malignant glauoma. Peripheral iridocorneal touch (white arrow) with angle
closure is visible (scleral spur at black arrow) . T e haptic is visible beneath the iris (arrowhead) .
 56 5 ANTERIO R SEGMENT IMAGING
OPEN-ANGLE GLAUCOMA
PIGMENT DISPE SION SYND OME
AND PIGMENTA YGLAUCOMA
T e angle is widely open. T e midperipheral
iris charac eris ically assumes a concave con-
gura ion (reverse pupillary block), bringing
he iris in o con ac wi h he an erior zonular
bundles, and iridolen icular con ac is grea er
han normal. T e lat er is hough o preven
equilibra ion o aqueous be ween he wo
chambers, leading o grea er pressure in he
an erior chamber han in he pos erior cham-
ber. T e concave con gura ion is accen ua ed
by accommoda ion.
When blinking is inhibi ed, he iris assumes
a convex con gura ion which is immedia ely
reversed upon blinking, sugges ing ha he
ac o blinking ac s as a mechanical pump o
push aqueous rom he pos erior o he an e-
rior chamber. Laser irido omy elimina es he
pressure di eren ial be ween he an erior and
pos erior chambers and relieves he iris con-
cavi y. T e iris assumes a a or planar con gu-
ra ion and he ex en o iridolen icular con ac
is decreased (Fig. 5-15).8
EXFOLIATION SYND OME
Ex olia ion ma erial may be de ec ed clinically
earlies on he ciliary processes and zonules.
UBM can demons ra e various degrees o
zonular disrup ion (Fig. 5-16).9
 Open-Angle Glaucoma 57

FIGURE 5-15. Pigment dispersion syndrome.

AS-OC (A) and UBM (B) showing concave iris
conf guration. C. Planar iris conf guration a er laser
C iridotomy.

A B
FIGURE 5-16. Ex oliation syndrome. A. Normal zonules. B. Deposited ex oliation material produces a di use
patchy granular appearance to the zonules ( arrow) .
 58 5 ANTERIO R SEGMENT IMAGING

OTHER CONDITIONS bu poorly demarca ed in AS-OC images

CYCLODIALYSIS CLEFT
Cyclodialysis is a separa ion o he longi u-
dinal muscles o he ciliary body rom he
scleral spur. In blun ocular rauma, UBM or
AS-OC should be kep in mind or evalua -
ing any rauma-associa ed change in ocular
ana omy. A cyclodialysis clef is of en accom-
panied by a supraciliary e usion (Fig. 5-17).
I IDOCILIA YCYST
A si ua ion similar o pla eau iris is of en pres-
en wi h he cys s unc ioning similarly o
enlarged or an eriorly posi ioned ciliary pro-
cesses. T ese are easily diagnosed wi h UBM
(Fig. 5-18).
CILIA YBODYTUMO S
UBM can be used o di eren ia e solid rom
cys ic lesions o he iris and ciliary body. T e
dimensions o umors can be measured and
he ex en o which hey do or do no invade
he iris roo and ciliary ace de ermined
(Fig. 5-19). AS-OC is rarely help ul in his
condi ion.
I IDOSCHISIS
Iridoschisis is a separa ion o he an erior and
pos erior iris s romal layers. Angle closure may
occur (Fig. 5-20).

A B
FIGURE 5-17. Cyclodialysis cle . UBM (A) and AS-OC (B) reveal the separation between the longitudinal
muscle o the ciliary body and the scleral spur (arrows) . Note the supraciliary e usion ( asterisks) .
 Other Conditions 59

A B
FIGURE 5-18. Iridociliar y cysts. Iridociliary cysts ( asterisks) in UBM (A) and AS-OC (B) images are
characterized by an echolucent lumen. T e angle is ocally closed (arrows) .

FIGURE 5-19. Ciliar y body melanoma. In this eye with ciliary body melanoma (asterisk) , the angle is ocally
closed ( arrows) .
 60 5 ANTERIO R SEGMENT IMAGING

FIGURE 5-20. Iridoschisis. Extensive stromal separation ( arrowhead) reaches the cornea and compromises
aqueous out ow ( vertical arrow) .

SURGERY AND GLAUCOMA
FILTE ING BLEB
Success ul blebs have a di use, spongy appear-
ance. Blebs ollowing surgery wi h adjunc ive
an ime aboli es are of en hin walled and cys-
ic. Encapsula ed blebs end o be eleva ed and
localized, wi h or wi hou prominen vessels.
Failed blebs are of en a and may be vascu-
larized. T e clinical appearance o a bleb, how-
ever, is no always an accura e predic or o
unc ional s a us.
T e UBM appearance o a unc ioning bleb
shows a uid rack rom he an erior cham-
ber, hrough he in ernal os ium, benea h he
scleral ap, and in o he subconjunc ival space
(Fig. 5-21).10 Accura e localiza ion o he si e
o obs ruc ion o uid ow can be acili a ed
by UBM. Eyes wi h a blebs have no evidence
o subconjunc ival l ra ion and demons ra e
blockage o ow a he level o he episclera
(Fig. 5-22). enon cys s demons ra e hick
wall o conjunc iva and enon wi h or wi h-
ou a pa en scleral ap (Fig. 5-23). Needling
procedures may normalize IOP in eyes wi h
encapsula ed blebs.
T e noncon ac na ure o AS-OC provides
a sa er me hod o evalua e he l ering bleb
morphology wi hou dis or ion o bleb archi-
ec ure or risk o in ec ion, especially in he
early pos opera ive period.11 Due o i s higher
resolu ion han UBM, AS-OC can assess he
super cial layers o he l ering bleb in more
de ail (Fig. 5-24).
GLAUCOMA DR INAGE IMPLANTS
T e posi ion, pa ency, and course o drain-
age ubes can be ascer ained using UBM or
Surgery and Glaucoma 61
AS-OC (Fig. 5-25). UBM would be espe-
cially help ul in assessing he ube inser ed
in o he ciliary sulcus. T e ana omic rela ion-
ships can be assessed, as can compression o
he ube a he scleral en ry si e.
REFERENCES
1. Ishikawa H, Liebmann JM, Ri ch R. Quan i a ive
assessmen o he an erior segmen using ul ra-
sound biomicroscopy. Curr Opin Ophthalmol. 2000;
11(2):133–139.
2. Radhakrishnan S, Rollins AM, Ro h JE, e al. Real-
ime op ical coherence omography o he an e-
rior segmen a 1310 nm. Arch Ophthalmol. 2001;
119(8):1179–1185.
3. Wirbelauer C, Karandish A, Häberle H, e al.
Noncon ac goniome ry wi h op ical coherence
omography. Arch Ophthalmol.2005;123(2):179–185.
4. Dada , Siho a R, Gadia R, e al. Comparison o an e-
rior segmen op ical coherence omography and ul ra-
sound biomicroscopy or assessmen o he an erior
segmen . JCataract Re act Surg. 2007;33(5):837–840.
5. Pra a S, Dorairaj S, De Moraes CGV, e al.
Inden a ion sli lamp-adap ed op ical coherence
omography echnique or an erior chamber angle
assessmen . Arch Ophthalmol. 2010;128(5):646–647.
6. Ri ch R. Argon laser peripheral iridoplas y: An over-
view. J Glaucoma. 1992;1:206–213.
7. Parc C, Laloum J, Bergès O. Comparison o op ical
coherence omography and ul rasound biomicros-
copy or de ec ion o pla eau iris. JFr Ophtalmol. 2010;
33(4):266.e1–266.e3.
8. Pavlin CJ, Macken P, rope G, e al. Ul rasound bio-
microscopic ea ures o pigmen ary glaucoma. Can J
Ophthalmol. 1994;29(4):187–192.
9. Sbei y Z, Dorairaj SK, Reddy S, e al. Ul rasound bio-
microscopy o zonular ana omy in unila eral ex olia-
ion syndrome. Acta Ophthalmol. 2008;86:565–568.
10. Yamamo o , Sakuma , Ki azawa Y. An ul rasound
biomicroscopic s udy o l ering blebs af er mi omy-
cin C rabeculec omy. Ophthalmology. 1995;102(12):
1770–1776.
11. Leung CK, Yick DW, Kwong YY, e al. Analysis o
bleb morphology af er rabeculec omy wi h Visan e
an erior segmen op ical coherence omography. Br
J Ophthalmol. 2007;91(3):340–344.
 62 5 ANTERIO R SEGMENT IMAGING

FIGURE 5-21. Functioning ltering bleb. T e internal ostium (I) , intrascleral uid pathway (asterisk) , and
scleral ap (S) are seen. T e bleb (B) is moderately elevated and homogeneously spongy with uid-f lled spaces.
C, cornea; Cb, ciliary body; PI, peripheral iridectomy.

FIGURE 5-22. Failed bleb. T e internal ostium (I) and intrascleral uid pathway are patent, but the scleral
pathway or aqueous is closed ( arrow) .

A B
FIGURE 5-23. Bleb encapsulation. enon cyst wall ( ) is thick due to f broblastic proli eration. T e
intrascleral uid pathway is patent (A, asterisk) or closed (B). Cb, ciliary body; I, internal ostium; PI, peripheral
iridectomy; S, scleral ap.
 Surgery and Glaucoma 63

A B

FIGURE 5-24. Filtering blebs (AS OCT) .

Moderately (A) and highly (B) elevated unctioning
f ltering blebs. C. A ailing f ltering bleb with
C encapsulation.

A B
FIGURE 5-25. Glaucoma drainage implant. UBM (A) and AS-OC (B) showing the path o the tube rom
the anterior chamber.
 C H AP T ER
6
Op ic Nerve Imaging
M aking he diagnosis o glaucoma requires
he presence o op ic nerve head (ONH)
damage and/ or charac eris ic visual f eld
changes. While au oma ed and s a ic perime ry
provides an excellen aid in documen ing unc-
ional visual f eld loss rom glaucoma, as much
as 40% o he re inal nerve f ber layer ( NFL)
may be los be ore changes are de ec ed on s an-
dard whi e-on-whi e visual f eld es ing.1 T e
need or more sensi ive, reproducible, and accu-
ra e de ec ion o glaucoma ous damage has led
o he developmen o various imaging devices
or he ONH and NFL. o bes u ilize hese
new echnologies, i is essen ial ha physicians
amiliarize hemselves wi h he in orma ion he
es s provide and he s reng hs and weaknesses
o each modali y (Table 6-1).
EFE ENCE
1. Quigley HA, Addicks EM, Green W . Op ic nerve dam-
age in human glaucoma. Arch Ophthalmol. 1982;100:135.
64
T omas D. Patrianakos
STEREOPHOTO GRAPHY
A ssessmen o changes in he ONH and
NFL over ime can be evalua ed by
using color s ereopho ographs (Fig. 6-1).
T is me hod is he mos widely used imaging
echnique and is considered he gold s andard
or documen a ion o glaucoma ous op ic
neuropa hy (GON).1
S ereopho ographs can be produced by ak-
ing wo pho ographs in sequence, ei her
by manually reposi ioning he camera or
by using a sliding carriage adap er (Allen
separa or). Al erna ively, hey can be pro-
duced by aking wo pho ographs simul-
aneously wi h wo cameras ha u ilize
he indirec oph halmoscopic principle
(Donaldson s ereoscopic undus camera) or
a win-prism separa or. Simul aneous ONH
pho os have demons ra ed he bes reproduc-
ibili y over ime.2 ecen ly, al erna ion icker
 Stereophotography 65

TABLE 6-1. Principles and Clinical Parameters o Various Optic Nerve Imaging Devices
Device Principles Clinical Parameters Measured
Stereophotography Simultaneous photographs taken Subjective interpretation o ONH
with two cameras or two separate and RNFL anatomy (pallor, disc
photographs o same nerve at dif erent hemorrhages, peripapillary atrophy)
angles
HRT CSLO Optic disc tomography
GDx SLP/ bire ringence RNFL thickness
OCT Inter erometry Optic disc tomography and RNFL
thickness
ONH, optic nerve head; RNFL, retinal nerve ber layer; CSLO, con ocal scanning laser ophthalmoscopy; SLP, scanning laser
polarimetry.

o monoscopic op ic disc images over ime EFE ENCES

(digi al s ereochronoscopy) has also proven 1. Fingere M, Medeiros FA, Susanna , e al. Five rules
o be a sensi ive me hod or de ec ing GON o evalua e he op ic disc and re inal nerve f ber layer
or glaucoma. Optometry. 2005;76:661–668.
changes. Newer image processing and regis ra-
2. robe JD, Glaser JS, Cassady J, e al. Nonglaucoma ous
ion permi s accura e alignmen o ON pho o- excava ion o he op ic disc. Arch Ophthalmol. 1980;98:
graphs and acili a es image comparison wi h 1046.
de ec ion o changes in vessel posi ion, color, 3. Berger JW, Pa el , S one R , e al. Compu erized
and o her cues or con our change.3 s ereochronoscopy and al era ion icker o de ec op ic
nerve head con our change. Ophthalmology. 2000;107:
T e s reng hs o ONH s ereopho ographs 1316–1320.
include he abili y o documen parame ers 4. akamo o , Schwar z B. eproducibili y o pho-
ogramme ric op ic disc cup measuremen s. Invest
ha canno be quan if ed, such as disc hemor-
Ophthalmol Vis Sci. 1985;26:814.
rhages, peripapillary a rophy, and pallor. T ey
also allow he clinician o assess he impac o
o her nonglaucoma ous processes ha may
in uence unc ional es ing. However, while
s ereopho ographs provide excellen docu-
men a ion o he ONH, heir in erpre a ion
remains subjec ive and can here ore have
increased variabili y and limi ed use ulness
over ime.4 Addi ionally, media opaci ies, such
as ca arac s, or a poorly ocused pho ograph
can inhibi op imal analysis.
 66 6 O PTIC NERVE IMAGING

FIGURE 6-1. Stereophotography uses two simultaneous disc photos. With the use o a special viewer,
stereovision is achieved. Stereophotography is use ul to assess optic nerve changes over time. (Courtesy o
ara A. Uhler, MD.)

CONFO CAL SCANNING
LASER OPHTH ALMOSCOPY
H eidelberg re ina omography (H ;
Heidelberg Engineering GmbH,
Heidelberg, Germany) is a ype o con ocal
scanning laser oph halmoscopy (CSLO) used
o provide a s ruc ural quan i a ive measure-
men o he ONH (Fig. 6-2A). A 670-nm
diode laser is aimed hrough a pinhole on o
he re ina. T e ligh re ec ed passes hrough
a second pinhole in o a de ec or, which rans-
ers he maximum in ensi y o ligh a a given
poin o crea e an image. A series o 16 o 64
wo-dimensional (2D) sequen ial scans, each
measuring 384 × 384 pixels, is used o crea e
a hree-dimensional (3D) color-coded opo-
graphic image (Fig. 6-2B).
T e re ec ivi y image (Fig. 6-2C) produced is
used by he echnician o draw a con our line
around he inner border o he scleral ring,
which is ur her separa ed in o six sec ors.
T is line remains s andard or all u ure exams
and is used o calcula e a series o parame ers.
T e H 3.0 reduces echnician dependence
by producing au oma ed resul s o he con-
our line as described by Swindale e al.1 T e
graphic analysis (Fig. 6-2D) corresponds o
all six sec ors in he re ec ivi y image.
A re erence plane def ned as 50 µm below he
sur ace o he re ina along 6 degrees o he
con our line in he emporal in erior region
is designa ed as he cu o be ween he neuro-
re inal rim (all s ruc ures above he re erence
plane) and he cup (all s ruc ures below he
re erence plane).
A er he da a is recons ruc ed, 12 s ereo-
me ric parame ers (Fig. 6-2E) are au oma i-
cally ob ained. According o univaria e and
mul ivaria e analysis rom he ocular hyper-
ension rea men s udy (OH S), he mos
predic ive parame ers or glaucoma de ec ion
wi h he H were he ones independen o
Con ocal Scanning Laser Ophthalmoscopy 67
he re erence plane (mean heigh con our,
rim area, and mean cup dep h).2 opography
s andard devia ion is used as a measuremen
o image quali y and reliabili y, wi h any-
hing grea er han 30 o 40 µm considered
unreliable.
Moorf eld’s regression analysis (Fig. 6-2D)
uses linear analysis be ween op ic disc area
and neurore inal rim area in bo h global and
localized segmen s o classi y he nerve as
“wi hin normal limi s,” “borderline,” or “ou -
side normal limi s” based on comparison
o an age- and e hnici y-specif c norma ive
da abase.
Progression o GON on he H can be eval-
ua ed wi h even - or rend- ype analysis so -
ware. opographical change analysis (Fig. 6-3)
is an even - ype analysis ha ocuses on he
di erence be ween sur ace heigh measure-
men s o ollow-up images o hose o a base-
line image. Changes ha are consis en are
highligh ed in a color-coded overlay on op
o he re ec ivi y image wi h red represen -
ing areas o s a is ically signif can worsen-
ing. rend- ype analysis assesses he ra e o
change over ime in one o he s ereome ric
indices, such as rim area, in order o documen
progression.
Several s udies have conf rmed he abili y
o H o dis inguish be ween heal hy and
glaucoma ous eyes wi h a range o specif ci y
rom 75% o 95% and sensi ivi y rom 51% o
97%.3–5 eproducibili y is enhanced when a
leas hree scans are combined.6
Among advan ages o he H is he com-
pany’s commi men o assure older so ware
remains compa ible wi h newer machines,
hereby allowing or longer da a series on
individuals. Addi ional benef s include good
image quali y hrough undila ed pupils and he
capabili y o assess glaucoma ous progression
by comparing sequen ial scans, using ei her
he glaucoma change probabili y analysis or
 68 6 O PTIC NERVE IMAGING
opographic change analysis so ware.7,8 A dis-
advan age o he older H 2.0 model is he
requiremen or an opera or-dependen ou -
line o he disc margin. An addi ional limi a-
ion is he s andardized re erence plane used
o calcula e many o he parame ers as his
may no accura ely represen he rue NFL
hickness in all individuals. T ese concerns
have been addressed wi h he newer H
3.0 model. In addi ion, he H 3.0 so ware
con ains a larger e hnici y-specif c norma ive
da abase.
EFE ENCES
1. Swindale NV, S jepanovic G, Chin A, e al. Au oma ed
analysis o normal and glaucoma ous op ic nerve
head opography images. Invest Ophthalmol Vis Sci.
2000;41:1730–1742.
2. Zangwill LM, Weinreb N, Beiser JA, e al. Baseline
opographic op ic disc measuremen s associa ed wi h
he developmen o primary open-angle glaucoma: T e
Con ocal Scanning Laser Oph halmoscopy Ancillary
S udy o he Ocular Hyper ension rea men S udy.
Arch Ophthalmol. 2005;123:1188–1197.
3. Harasymowycz PJ, Papama heakis DG, Fansi AK.
Validi y o screening or glaucoma ous op ic nerve
damage using con ocal scanning laser oph halmos-
copy (Heidelberg e ina omograph II) in high-
risk popula ions: A pilo s udy. Ophthalmology.
2007;112:2164–2171.
4. eus NJ, deGraa M, Lemij HG. Accuracy o GDx
VCC, H I and clinical assessmen o s ereoscopic
op ic nerve head pho ographs or diagnosing glau-
coma. Br J Ophthalmol. 2007;91:313.
5. Miglior S, Guareschi M, Albe E, e al. De ec ion o glau-
coma ous visual f eld changes using he Moorf elds
regression analysis o he Heidelberg re ina omogra-
phy. Am J Ophthalmol. 2003;136:26–33.
6. Weinreb N, Lusky M, Morsman D. E ec o repe i-
ive imaging on opographic measuremen s o he
op ic nerve head. Arch Ophthalmol. 1993;111:
636–638.
7. Chauhan BC, Blanchard JW, LeBlanc P. echnique
or de ec ing serial opographic changes in he op ic
disc and peripapillary re ina using scanning laser
omography. Invest Ophthalmol Vis Sci. 2000;41:
775–782.
8. Bowd C, Balasubramanian M, Weinreb N. Per ormance
o con ocal scanning laser omography opographic
Change Analysis ( CA) or assessing glaucoma-
ous progression. Invest Ophthalmol Vis Sci. 2009;50:
691–701.
 Con ocal Scanning Laser Ophthalmoscopy 69

S te re ome tric a na lys is ONH Norma l ra nge

Dis k a re a 2.111 mm 2 1.69 – 2.82

2
Cup a re a 0.430 mm 0.26 – 1.27
Rim a re a 1.681 mm 2 1.20 – 1.78
Cup volume 0.100 cmm 0.01– 0.49
Rim volume 0.487 cmm 0.27– 0.49
Cup/dis k a re a ra tio 0.204 0.16– 0.47
Line a r/dis k a re a ra tio 0.451 0.36– 0.80
Me a m cup de a th 0.207 mm 0.14 – 0.38
Ma ximum cup de a th 0.735 mm 0.46– 0.90
Cup s ha pe me a s ure -0.304 -0.27– -0.09
He ight va ra tion contour 0.472 mm 0.30– 0.47
C Me a n RNFL thichkne s s 0.291 mm 0.18 – 0.31
RNFL cros s s e ctiona l a re a 1.499 mm 2 0.95– 1.61
Re fe re nce he ight 0.486 mm
E Topogra phy s td dev. 14 µm

FIGURE 6-2. A. HR o a healthy-appearing optic nerve. B. Color-coded topographic image o a healthy-

appearing optic nerve. C. Ref ectivity image separated into six sectors within a contour line. D. Graphic analysis
corresponding to all six sectors o the ref ectivity image with Moor eld’s regression analysis classi cation.
E. Stereometric analysis parameters indicating an accurate scan with a topography standard deviation <40 µm.
(Courtesy o David Hillman, MD.)
 70 6 O PTIC NERVE IMAGING

FIGURE 6-3. HR progression analysis demonstrating worsening o GON on topographic analysis with areas
o red/ yellow in the ref ectivity image represents statistically signi cant worsening. rend analysis o certain
stereometric parameters over time additionally documents deterioration o the ONH. (Courtesy o David
Hillman, MD.)

SCANNING LASER
POLARIMETRY
T he glaucoma diagnosis (GDx) es (Laser
Diagnos ics echnologies, San Diego,
CA) is he pro o ypical scanning laser polarim-
e ry (SLP), which uses he bire ringence prop-
er ies o he NFL o measure i s hickness. A
780-nm diode laser passes hrough an orderly
arrangemen o axons and micro ubules sur-
rounding he ONH. As ligh passes hrough
he NFL i undergoes a change in polariza-
ion re erred o as re arda ion. T e degree o
change in polariza ion is propor ional o he
hickness o he NFL and is de ec ed by a
buil -in ellipsome er. T ese changes are hen
rans ormed o a opographical map o NFL
hickness measuremen s and given a numeric
value by he GDx so ware using an assumed
cons an bire ringence value.1 However, bire-
ringence is no cons an around he ONH in
all individuals and hus NFL values may be
alsely repor ed. Addi ionally, he abundance
o bire ringen issues in he eye can con ami-
na e he ac ual values o he NFL. Newer
GDx models con ain a variable corneal com-
pensa or (GDx-VCC) (Zeiss Medi ec, Dublin,
CA) (Fig. 6-4A) which elimina es he re arda-
ion con ribu ed by he cornea. However, his
is based on he macula as an in ernal re erence
and can be in uenced by macular pa hologies.
T e la es version o he ins rumen uses indi-
vidualized an erior segmen compensa ion or
eyes wi h low signal- o-noise ra io called an
enhanced corneal compensa or (ECC).2
A undus image (Fig. 6-4B) is used by he
opera or o manually def ne he ONH. A
calcula ion circle wi h a f xed band measuring
0.4 mm wide (inner diame er 2.4 mm; ou er
diame er 3.2 mm) is au oma ically aligned
around he disc based on illumina ion pat erns
and highligh s he area where he NFL
measuremen s will be derived. Image quali y
and reliabili y o he es can be quan if ed
Scanning Laser Polarimetry 71
by he appearance o he undus image. Even
illumina ion, good ocus, and proper disc/
ellipse cen ra ion are essen ial or a reliable
scan. Addi ionally, each eye scanned is
assigned a quali y number or q value (scale 0
o 10; 10 = per ec scan), which can also be
used o gauge he quali y o he es . In general,
any hing wi h a q value ≥7 is accep able or
in erpre a ion.
T e NFL hickness map (Fig. 6-4C) is
a color-coded image corresponding o he
hickness o he NFL. Brigh red or yellow
colors represen areas o hick NFL and are
normally seen in an hourglass dis ribu ion
superiorly and in eriorly. Blue colors rela e
o areas o hin NFL and are normally seen
nasally and emporally. GON is charac erized
by increasing blue colors o he superior/ in e-
rior por ion o he ONH.
T e devia ion map (Fig. 6-4D) reveals he
loca ion and magni ude o NFL de ec s over
he en ire hickness o he map and how much
hey devia e rom he race- and age-ma ched
norma ive da abase. T ese are color coded
and assigned a rela ive s a is ical signif cance
based on probabili y o normali y.
T e SNI ( emporal, superior, nasal, in e-
rior, emporal) graph (Fig. 6-4E) maps ou he
pa ien ’s NFL modula ion curve and super-
imposes i on a norma ive NFL modula ion
curve. Normal NFL modula ion should ol-
low a sinusoidal pat ern (double hump a he
superior and in erior por ion) wi h at ening
o he humps represen ing NFL loss consis-
en wi h glaucoma.
T e ac ual numeric values calcula ed o rep-
resen he NFL hickness are lis ed in he
SNI parame ers (Fig. 6-4F). T ese values
are also color coded and assigned a rela ive
s a is ical signif cance based on he probabili y
o normali y. S udies have demons ra ed ha
he nerve f ber indica or (NFI) correla es bes
wi h he exis ence o glaucoma.3 I represen s
 72 6 O PTIC NERVE IMAGING
a global value based on he en ire NFL hick-
ness [range 1 (normal) o 100 (glaucoma)].
Values ≥50 are highly sugges ive o glaucoma.
However, his number canno be he sole
de erminan or he diagnosis o glaucoma, as
he NFI can be wi hin he normal range when
here is only a ocal NFL de ec . In such
cases, he devia ion map would exhibi he
color-coded local damage. O her impor an
parame ers include he in erior normalized
area and he SNI average, bo h o which
have been highly correla ed wi h he de ec ion
o glaucoma.4
Progression in he GDx is documen ed using
guided progression analysis (Fig. 6-5). T is
even analysis echnique compares he re ar-
da ion pat erns o ollow-up scans o a baseline
and assesses he variabili y o he individ-
ual pa ien o ha observed in a ma ched
popula ion.
T e GDx-VCC has been shown o have good
diagnos ic accuracy, wi h an overall sensi ivi y
ranging rom 80% o 92% and specif ci y rom
66% o 98%.5–7
S reng hs o he GDx include a large race-
and age-ma ched norma ive da abase and
he abili y o ob ain rapid da a on NFL
measuremen s hrough an undila ed pupil. A
limi a ion o older GDx devices, f xed corneal
compensa ion, has been improved wi h he
newer VCC. However, s udies have ound ha
even wi h VCC, a ypical bire ringence pa -
erns can occur rom ar i ac in roduced by
he device’s at emp o compensa e or poor
noise- o-signal ra io.8 T e newer ECC model
hopes o correc he shor comings o he pre-
vious uni s.
EFE ENCES
1. Sehi M, Guaque a DC, Feuer WJ, e al. Scanning laser
polarime ry wi h variable and enhanced corneal com-
pensa ion in normal and glaucoma ous eyes. Am J
Ophthalmol. 2007;143:272–279.
2. Medeiros FA, Bowd C, Zangwill LM, e al. De ec ion
o glaucoma using scanning laser polarime ry wi h
enhanced corneal compensa ion. Invest Ophthalmol
Vis Sci. 2007;48:3146–3153.
3. Medeiros FA, Zangwill LM, Bowd C, e al.
Comparison o he GDx VCC scanning laser polarim-
e er, H II con ocal scanning laser oph halmoscope,
and s ra us OC op ical coherence omograph or
he de ec ion o glaucoma. Arch Ophthalmol. 2004;
122:827–837.
4. Funaki S, Shirakashi M, Yaoeda K, e al. Specif ci y
and sensi ivi y o glaucoma de ec ion in he Japanese
popula ion using scanning laser polarime ry. Br J
Ophthalmol. 2002;86:70–74.
5. Colen P, Lemij HG. Sensi ivi y and specif ci y o
GDx: Clinical judgmen o scanning laser polarime -
ric analysis o s andard prin ou s versus he number. J
Glaucoma. 2003;12:129–133.
6. Poinoosawmy D, an JCH, Bunce C, e al. T e abil-
i y o he GDX nerve f ber analyser neural ne work o
diagnose glaucoma. Graefes Arch Clin Exp Ophthalmol.
2001;239:122–127.
7. Medeiros FA, Zangwill LM, Bowd C, e al. Use o
progressive glaucoma ous op ic disc changes as he
re erence s andard or evalua ion o diagnos ic es s
in glaucoma. Am J Opthalmol. 2005;139:1010–1018.
8. Bagga H, Greenf eld DS, Feuer WJ, e al. Quan i a ive
assessmen o a ypical bire ringence images using
scanning laser polarime ry wi h variable corneal com-
pensa ion. Am J Ophthalmol. 2005;139:437–446.
 Scanning Laser Polarimetry 73

A B

D
C
FIGURE 6-4. A. GDx with VCC o a glaucoma suspect demonstrating slight in erior RNFL loss in the le
eye. B. Fundus image with the calculation circle. C. RNFL thickness map demonstrating bright red or yellow
colors representing areas o thick RNFL normally seen in an hourglass distribution superiorly and in eriorly.
D. Deviation map with color-coded areas exempli ying RNFL deviation rom a race- and age-matched normative
database.

(continued)
 74 6 O PTIC NERVE IMAGING

TS NIT OD OS
Pa ra me te rs Actua l va l. Actua l va l.

TS NIT ave ra ge 51.3 49.3

S upe rior ave ra ge 65.9 66.0

Infe rior ave ra ge 54.1 50.1

TS NIT s td. dev. 19.6 18.5

Inte r-eye s ymme try 0.87

NFI 15 21

p> = 5% p<5% p<2% p<1% p<0.5%

E
F
FIGURE 6-4. ( Continued) E. SNI graph showing slight in erior RNFL thinning superimposed on a
normative RNFL modulation curve. F. SNI parameters that are color coded and assigned a relative statistical
signi cance based on the probability o normality.

FIGURE 6-5. Progression o GON with GDx-guided progression and trend analysis comparing ollow-up scans
to a baseline scan. T e deviation map shows ocal thinning o the superior and in erior RNFL which is urther
exempli ed by color-coded di erence rom baseline scans. SNI parameters also demonstrate worsening o
glaucoma by increasing NFI values and decreasing RNFL values over time.

OPTICAL COHERENCE
TOMO GRAPHY
O p ical coherence omography (OC )
provides high-resolu ion real- ime cross-
sec ional imaging o he re ina and ONH. I
spli s a low-coherence, near-in rared 810-nm
diode laser in o wo arms, wi h one beam
aimed a he re ina and he o her a re erence
mirror. T e echo ime delay and in ensi y o
back-re ec ed ligh rom he re ina crea e an
in er erence pat ern which is analyzed by a
pho ode ec or. Di erences in re inal layers
are illus ra ed in an image due o he unique
ime delay o he re ec ions rom various is-
sue componen s.
T e OC is unique among nerve f ber analy-
sis equipmen in ha i con ains a varie y o
quan i a ive analysis pro ocols used o evalu-
a e bo h op ic nerve and re inal pa hology.
Glaucoma de ec ion can be moni ored wi h
he NFL analysis, ONH analysis, and macu-
lar hickness analysis scans; however, s udies
have shown ha NFL analysis provides he
bes discrimina ion be ween normal and glau-
coma ous eyes.1,2
Curren ly, here are several commercially
available OC s, each o ering i s own unique
benef s. Carl Zeiss Medi ec (Dublin, CA)
has developed he wo mos commonly used
OC s. T e hird-genera ion S ra us OC is a
ime domain sys em ( D-OC ) ha scans a
an average ra e o 400 axial scans per second
and provides an 10 micron axial image reso-
lu ion. I s major def ciency lies in i s inabili y
o au oma ically regis er ollow-up compari-
son o baseline scans and ensure ha measure-
men s are ob ained a he same loca ion or
analysis and de ec ion o change. T e our h-
genera ion Cirrus OC is a spec ral domain
sys em (SD-OC ) ha provides higher-speed
and higher-resolu ion scans allowing or he
crea ion o 3D da ase s. I allows or scanning
speeds o 55,000 axial scans per second wi h
Optical Coherence Tomography 75
axial resolu ions o 5 o 6 µm. In addi ion,
i permi s regis ra ion o scan rom session
o session o ensure he same measuremen
loca ion and allows arbi rary analysis o 3D
da ase s.
Fas NFL analysis scanning (Fig. 6-6A)
involves he acquisi ion o hree circular scans
wi h a 3.4-mm diame er cen ered on he
ONH. Charac eris ic changes in re ec ivi y
observed a he inner and ou er re inal bound-
aries are au oma ically conver ed o quan-
i a ive NFL hickness da a by a compu er
algori hm. T e SNI image (Fig. 6-6B) pro-
vides comparison o he pa ien ’s NFL hick-
ness (black line) o an age-rela ed norma ive
da abase. T is is ur her displayed as a circular
diagram o 12 clock-hour sec ions, 30 degrees
each, and color coded o ma ch a norma ive
da abase. Various measuremen s are hen
lis ed in he abular Da a Sec ion (Fig. 6-6C),
he mos signif can o which or glaucoma are
Smax/ Imax (maximum hickness o superior/
in erior quadran ), Smax/ avg or Imax/ avg
(maximum hickness o superior or in erior/
average hickness o emp quadran ), Smax,
Imax, and Avg. T ickness.2,3 Finally, scan
image and video image o ONH (Fig. 6-6D)
should be evalua ed o de ermine he accuracy
o he es . In general, signal s reng hs o ≥7 are
considered accep able or in erpre a ion.
ONH analysis (Fig. 6-7) enables assessmen
and measuremen o he ONH. D-OC ac-
quires six radial cross-sec ional scans around
ONH, while SD-OC scans a 6 × 6 mm
cubed area cen ered on he ONH. Changes
in re ec ivi y are used o de ec he an erior
sur ace o he NFL and he re inal pigmen
epi helium ( PE). T ese markers are hen
used o measure all ea ures o disc ana omy.
Several s udies have shown ha he highes
per orming ONH parame ers, such as he C:D
ra io and in egra ed rim volume, have diagnos-
ic accuracies equivalen o he bes NFL
parame ers.4–6
 76 6 O PTIC NERVE IMAGING
T e macular hickness analysis scan
(Fig. 6-8) also de ec s he NFL and PE
wi h six radial cross-sec ional images o he
macula ( D-OC ) or a cube scan (SD-
OC ). A color-coded map is crea ed, illus ra -
ing he re inal hickness or specif c regions o
he macula. Images ob ained provide in orma-
ion abou issue s ruc ures in all layers o he
re ina.
T e SD-OC con ains rend analysis so ware
o assess progression (Fig. 6-9) by plot ing a
linear regression o NFL hickness agains
age. Even -based guided progression analysis
also compares he NFL hickness pat erns
o ollow-up scans o a baseline scan and indi-
ca es hinning as possible loss (yellow) or
likely loss (red).
T e OC has been shown o have good diag-
nos ic accuracy in de ec ing GON, wi h an
overall sensi ivi y ranging rom 61% o -84%
and specif ci y rom 85% o 100%.7,8
Advan ages o OC include i s abili y o pro-
vide various quan i a ive analysis pro ocols
ha can be combined o o er more in orma-
ion o he clinician. Newer SD-OC s allow
or higher-resolu ion images comparable o
conven ional his opa hology. Limi a ions o
he D-OC include he need or a dila ed
pupil in some pa ien s, as well as a lack o
e hnici y-specif c norma ive da abase and
progression analysis so ware. T e newer
SD-OC s (Fig. 6-10) have improved image
quali y while also o ering progression analy-
sis so ware and he abili y o au oma ically
regis er ollow-up comparison o baseline
scans.
EFE ENCES
1. Medeiros FA, Zangwill LM, Bowd C, e al. Evalua ion
o re inal nerve f ber layer, op ic nerve head, and
macular hickness measuremen s or glaucoma de ec-
ion using op ical coherence omography. Am J Oph-
thalomol. 2005;139:44–55.
2. Wolls ein G, Ishikawa H, Wang J, e al. Comparison o
hree op ical coherence omography scanning areas or
de ec ion o glaucoma ous damage. Am J Ophthalmol.
2005;139:39–43.
3. Bourne , Medeiros FA, Bowd C, e al. Comparabili y
o re inal nerve f ber layer hickness measuremen s o
op ical coherence omography ins rumen s. Invest
Ophthalmol Vis Sci. 2005;46:1280–1285.
4. Leung CK, Chan WM, Hui YL, e al. Analysis o re i-
nal nerve f ber layer and op ic nerve head in glaucoma
wi h di eren re erence plane o se s, using op ical
coherence omography. Invest Ophthalmol Vis Sci.
2005;46:891–899.
5. Leung CK, Chan WM, Hui YL, e al. Comparison o
macular and peripapillary measuremen s or he de ec-
ion o glaucoma: An op ical coherence omography
s udy. Ophthalmology. 2005;112:391–400.
6. Manassakorn A, Nouri-Mahdavi K, Caprioli J, e al.
Comparison o re inal nerve f ber layer hickness and
op ic disc algori hms wi h op ical coherence omogra-
phy o de ec glaucoma. Am J Ophthalmol. 2006;141:
105–115.
7. Chang , O’ ese KJ, Dudenz DL, e al. Sensi ivi y
and specif ci y o ime-domain versus spec ral-domain
op ical coherence omography in diagnosing early
o modera e glaucoma. Ophthalmology. 2009;116:
2294–2299.
8. Budenz DL, Michael A, Ka z J, e al. Sensi ivi y and
specif ci y o he S ra us OC or perime ric glau-
coma. Ophthalmology. 2005;112:3–9.
 Optical Coherence Tomography 77

FIGURE 6-6. A. Stratus D-OC RNFL analysis

scan demonstrating RNFL thinning consistent with
glaucoma in the right eye and a normal-appearing le
eye. B. SNI image comparing the patient’s NFL
thickness ( black line) to an age-related normative
database with corresponding color-coded circular
diagrams. C. abular data with various color-coded
RNFL measurements indicating thinning o the
superior and in erior RNFL in the right eye. D. Video
image o ONH with signal strength = 8, indicating an
D
accurate scan.
 78 6 O PTIC NERVE IMAGING

FIGURE 6-7. Stratus D-OC ONH analysis scan demonstrating topographic analysis and measurements o
the optic nerve.
 Optical Coherence Tomography 79

FIGURE 6-8. Stratus D-OC macular thickness analysis scan demonstrating the RNFL and RPE o the macula.
 80 6 O PTIC NERVE IMAGING

FIGURE 6-9. Cirrus SD-OC RNFL imaging scan o a patient with glaucoma demonstrating classic thinning o
the superior RNFL in both eyes.
 Optical Coherence Tomography 81

FIGURE 6-10. Cirrus SD-OC progression analysis so ware demonstrating no evidence o progression over
time on both event- and trend-based analysis.
 C H AP T ER

7
Evalua ion o he Op ic Nerve
and Nerve Fiber Layer
Zinaria Y. Williams, Kimberly V. Miller, and Joel S. Schuman

G laucoma is a common cause o blindness

worldwide. I may occur in any age group,
bu is especially common af er 40 years o
age. Eleva ed in raocular pressure is he mos
impor an causal risk ac or or glaucoma, bu
high in raocular pressure is no necessary or
glaucoma ous damage o occur. T e physi-
cal impac o glaucoma ous op ic neuropa hy
includes an irreversible loss o re inal ganglion
cells ha is clinically mani es ed as op ic nerve
head cupping and localized or di use de ec s
o he re inal nerve ber layer (NFL). Because
glaucoma ous damage is irreversible bu
largely preven able, early and accura e diagno-
sis is impor an .
FUNCTIONAL TESTS
E valua ion o he op ic nerve and NFL
includes examina ions ha es heir
s ruc ure and unc ion. Glaucoma ous re inal
ganglion cell loss resul s s ruc urally in NFL
and op ic nerve de ec s, and unc ionally in
visual eld changes ha can be assessed by
au oma ed perime ry and elec rophysiologic
es ing. Glaucoma ous visual eld de ec s
include localized paracen ral sco omas, arcu-
a e de ec s, nasal s eps, and more uncommonly
emporal de ec s (Fig. 7-1). T e mos com-
mon loca ion o visual eld de ec s rela ed o
glaucoma is wi hin an arcua e area commonly
re erred o as Bjerrum’s region, which ex ends
rom he blind spo o he median raphe.

82
 Functional Tests 83

FIGURE 7-1. Visual f eld de ec s. A. Arcua e de ec in Bjerrum’s region. B. Double arcua e de ec .

C. Paracen ral sco omas. D. emporal wedge de ec . (Adap ed wi h permission rom Eps ein DL. Chandler
and Gran ’s Glaucoma. 4 h ed. Bal imore, MD: Williams & Wilkins; 1997.)
 84 7 EVALUATION O F THE O PTIC NERVE AND NERVE FIBER LAYER

AUTOMATED PE IMET Y examina ion, has been he gold s andard or

A u oma ed perime ers es he visual eld

by presen ing s a ic s imuli o cons an
size and varying ligh in ensi y a speci c loca-
ions or a shor period o ime while record-
ing he pa ien ’s response a each loca ion.
T e Humphrey eld analyzer (HFA) 24–2
s andard achroma ic ull hreshold examina-
ion (Humphrey Sys ems, Dublin, CA) uses a
whi e s imulus wi h whi e background illumi-
na ion; similar programs are presen on o her
au oma ed perime ers. S andard achroma ic
au oma ed perime ry, along wi h clinical
ollowing glaucoma; however, his early au o-
ma ed es ing s ra egy is ime-consuming,
of en resul ing in pa ien a igue and pa ien
errors. Advances in au oma ed perime ry have
aimed a reducing es ing ime and a devel-
oping s ra egies or earlier de ec ion o visual
damage in glaucoma. Glaucoma hemi eld
es ing is a s ra egy ha compares speci ed
regions o he visual eld above and below
he horizon al midline (Fig. 7-2). T is es
is presen in he sof ware o mos au oma ed
perime ers.

FIGURE 7-2. Glaucoma hemif eld es ing. Superior visual f eld zones used in he glaucoma hemif eld es .
Each zone is compared wi h i s mirror zone below he horizon al meridian. (Adap ed wi h permission rom
Eps ein DL. Chandler and Gran ’s Glaucoma. 4 h ed. Bal imore, MD: Williams & Wilkins; 1997.)

SWEDISH INTER CTIVE
TH ESHOLD ALGO ITHMS
S wedish in erac ive hreshold algori hms
(SI As; Humphrey Sys ems, Dublin, CA)
are a amily o es algori hms developed o
signi can ly reduce he es ing ime wi hou a
reduc ion in da a quali y1 (Figs. 7-3 and 7-4).
How Sita Works
SI A uses in orma ion gained hrough-
ou he program o de ermine he hreshold
s ra egy or adjacen poin s. SI A measures
he response ime o each pa ien and uses he
in orma ion o se he pace o he es .
T ese SI A s ra egies are as and accom-
plish he same or bet er es quali y as he
ull hreshold program.2 Average es ime is
approxima ely 5 o 7 minu es per eye wi h
SI A S andard.
T ere is also a SI A as s ra egy, which
requires approxima ely 50% less ime han
Functional Tests 85
SI A S andard; however, here is a signi can
radeo in sensi ivi y or he reduc ion in es -
ing ime.
When To Use Sita
SI A is quickly becoming he gold s an-
dard or clinical glaucoma care.
ACKNOWLEDGMEN S
Elec rophysiologic es ing in orma ion in his chap er was
con ribu ed by Erich Sut er, PhD, a Smi h-Ket lewell,
San Francisco, and Elec ro-Diagnos ic Imaging, San
Ma eo, CA. Suppor ed in par by NIH 29-EY11006,
01-EY13178, and O1-EY11289.
EFE ENCES
1. Beng sson B, Heijl A, Olsson J, e al. A new genera ion
o algori hms or compu erized hreshold perime ry,
SI A. Ac a Oph halmol Scand. 1997;75:368–375.
2. Beng sson B, Heijl A, Olsson J. Evalua ion o a new
hreshold visual eld s ra egy, SI A, in normal sub-
jec s. Ac a Oph halmol Scand. 1998;76:165–169.
 86 7 EVALUATION O F THE O PTIC NERVE AND NERVE FIBER LAYER

B
FIGURE 7-3. SITA, normal eye. A. Normal op ic nerve head (ONH) pho ograph. B. Normal SI A visual f eld.
 Functional Tests 87

B
FIGURE 7-4. SITA, glaucoma ous eye. A. ONH pho ograph o an eye wi h glaucoma B. SI A visual f eld
showing a superior arcua e sco oma and an in erior nasal s ep.
 88 7 EVALUATION O F THE O PTIC NERVE AND NERVE FIBER LAYER
GLAUCOMA P OG ESSION
ANALYSIS
G laucoma progression analysis (GPA) is
one analy ic me hod in a sui e o pro-
gression evalua ion echniques called guided
progression analysis (known as GPA as well,
Carl Zeiss Medi ec, Dublin, CA). Guided pro-
gression analysis is an even -based analysis
ha uses he Humphrey visual eld pat ern
devia ion, and is included in he HFA sof ware
package (Carl Zeiss Medi ec, Dublin, CA)
(Fig. 7-5).
How GPA Works
GPA is based on he visual eld endpoin
rom he Early Mani es Glaucoma rial
(EMG ).1
GPA maps compare each poin on a s an-
dard 24–2 visual eld o he average o wo
baseline es s.
T e sof ware iden i es each poin as
s able, de eriora ing, or improving. When
hree es poin s are signi can ly progress-
ing (p < 0.05) in he same loca ion over wo
consecu ive es s, he GPA ags “possible
FIGURE 7-5. GPA, glaucoma ous eye. GPA showing devia ion rom baseline and likely progression near f xa ion.
progression.” “Likely progression” is agged
when hree es poin s are signi can ly pro-
gressing in hree consecu ive es s.
When to Use GPA
GPA can be used in ollowing all glaucoma
pa ien s and glaucoma suspec s.
Limitations
Because i is based on pat ern devia ion,
severely depressed elds canno be analyzed
wi h he GPA. Poin s ha are depressed
beyond he range o he GPA analysis sof -
ware are iden i ed wi h an “x.” Such poin s
are no eligible o be iden i ed by GPA as
progressing, due o heir being ou o range;
here ore, even i progression is occurring a
an “x”-labeled poin , GPA will no demon-
s ra e progression a ha poin . Because i is
an even -based analysis, very slow generalized
progression also may no be iden i ed.
EFE ENCE
1. Leske MC, Heijl A, Hyman L, e al. Early Mani es
Glaucoma rial: Design and baseline da a. Oph hal-
mology. 1999;106(11):2144–2153.

VISUAL FIELD INDEX
T he visual eld index (VFI), ano her com-
ponen o he GPA sui e, is an es ima e
o he ra e o glaucoma progression based on
he mean devia ion o he visual eld, and is
included in he HFA sof ware package (Carl
Zeiss Medi ec, Dublin, CA).
How VFI Works
o avoid he e ec o ca arac on implied
glaucoma progression, each poin on he pa -
ern devia ion map is scored as a percen .
Poin s wi h normal sensi ivi y are given
a score o 100% and poin s wi h absolu e
de ec s are given a score o 0%. Poin s wi h
depressed sensi ivi y (p < 0.05) receive a score
calcula ed by a ormula ha includes o al
devia ion and age-correc ed normal hreshold.
T e cen er o he visual eld is hen
weigh ed more highly.
FIGURE 7-6. VFI, glaucoma ous eye. VFI showing progression over ime and projec ed progression.
Functional Tests 89
T e resul ing percen age is displayed on a
graph, and he HFA calcula es a linear regres-
sion which de ermines signi cance and es i-
ma es overall ra e o progression1 (Fig. 7-6).
T e VFI projec s he predic ed u ure
visual loss using his linear regression and ra e
o progression.
When to Use VFI
VFI can be used in ollowing all glaucoma
pa ien s and glaucoma suspec s.
Limitations
VFI may no iden i y localized progres-
sion, and may be subjec o variabili y in
pa ien es reliabili y.
EFE ENCE
1. Beng sson B, Heijl A. A visual eld index or calcula-
ion o glaucoma ra e o progression. Am J Oph halmol.
2008;145(2):343–353.
 90 7 EVALUATION O F THE O PTIC NERVE AND NERVE FIBER LAYER
SHO T-WAVELENGTH
AUTOMATED PE IMET Y
S hor -waveleng h au oma ed perime ry
(SWAP) has bet er sensi ivi y han s an-
dard au oma ed perime ry or glaucoma diag-
nosis a early s ages o damage1,2 (Fig. 7-7).
SWAP is now available using he SI A s ra egy
(SI A-SWAP), which reduces es ing ime.
How SWAP Works
A care ully chosen waveleng h o blue ligh
is used as he s imulus, and a speci c color
and brigh ness o yellow ligh is used or he
background illumina ion. SWAP isola es and
measures blue-yellow ganglion cell unc ion.
SWAP is hough o presen earlier diagnosis
because blue-yellow ganglion cells are selec-
ively damaged in early glaucoma; however,
he increased sensi ivi y o his es over s an-
dard achroma ic perime ry may be a resul o
he reduc ion in he redundancy o he visual
sys em achieved hrough he use o a blue ar-
ge ligh on a yellow background.3–5
When to Use SWAP
SWAP es ing can be used on pa ien s
suspec ed o having glaucoma who have one
or more risk ac ors or glaucoma wi h normal
achroma ic perime ry.
SWAP es ing is appropria e or glaucoma
and ocular hyper ensive pa ien s wi h mild o
modera e eld loss.
Limitations
Pa ien s having signi can nuclear scle-
ro ic ca arac s or advanced achroma ic visual
eld loss may no be good candida es or
blue-yellow es ing, because nuclear sclero ic
ca arac con ounds SWAP and he dynamic
range o SWAP is exceeded wi h modera e
o high degrees o achroma ic visual eld
loss.
One obs acle o he in erpre a ion o
SWAP elds is he presence o grea er
long- erm variabili y in normal subjec s,
which makes di eren ia ion be ween ran-
dom varia ions and rue progression more
di cul .6,7
EFE ENCES
1. Johnson CA, Adams AJ, Casson EJ, e al. Progression
o early glaucoma ous visual eld loss as de ec ed by
blue-on-yellow and s andard whi e-on-whi e perim-
e ry. Arch Oph halmol. 1993;111:651–656.
2. Sample PA, aylor JD, Mar inez GA, e al. Shor -
waveleng h color visual elds in glaucoma suspec s a
risk. Am J Oph halmol. 1993;115:225–233.
3. Heron G, Adams AJ, Hus ed . Cen ral visual elds
or shor waveleng h sensi ive pa hways in glaucoma
and ocular hyper ension. Inves Oph halmol Vis Sci.
1988;29:64–72.
4. Sample PA, Boyn on M, Weinreb N. Isola ing he
color vision loss in primary open angle glaucoma. Am J
Oph halmol. 1988;106:686–691.
5. Har WM Jr, Silverman SE, rick GL, e al.
Glaucoma ous visual eld damage: Luminance and
color-con ras sensi ivi ies. Inves Oph halmol Vis Sci.
1990;31:359–367.
6. Kwon YH, Park HJ, Jap A, e al. es -re es variabili y
o blue-on-yellow perime ry is grea er han whi e-on-
whi e perime ry in normal subjec s. Am J Oph halmol.
1998;126:29–36.
7. Blumen hal AZ, Sample PA, Zangwill L, e al.
Comparison o long- erm variabili y or s andard and
shor -waveleng h au oma ed perime ry in s able glau-
coma pa ien s. Am J Oph halmol. 2000;129:309–313.
 Functional Tests 91

B
FIGURE 7-7. SWAP, normal eye. A. Normal ONH pho ograph. B. Normal SWAP visual f eld.
 92 7 EVALUATION O F THE O PTIC NERVE AND NERVE FIBER LAYER
F EQUENCY-DOUBLING
TECHNOLOGY
F requency-doubling echnology (FD )
perime ry (Welch Allyn, Skanea eles,
NY, and Carl Zeiss Medi ec, Dublin, CA) can
serve as an e ec ive ini ial visual eld evalua-
ion or de ec ion o glaucoma ous visual eld
loss. T is small, able op uni is por able and
can be easily used in he o ce or a o -si e
loca ions.
How FDT Works
T e requency-doubling illusion is he
phenomenon ha resul s when low–spa ial-
requency gra ing pat erns o black and whi e
bars undergo rapid coun erphase icker, cre-
a ing he percep ion ha wice as many bars
are visible o he pa ien as are ac ually pres-
en in he s imulus.
Evidence has sugges ed ha here is a
selec ively more rapid dea h o larger ganglion
cells (M-cells) ha projec o he magnocel-
lular layers o he la eral genicula e body han
o o her cell ypes in glaucoma.1–3 T is small
subse o ganglion cells demons ra es a non-
linear response o he requency-doubling
s imuli delivered by FD .
T is ins rumen de ec s glaucoma ous
visual eld loss wi h grea er han 90% sensi-
ivi y and speci ci y when compared o he
HFA s andard achroma ic perime ry as he
gold s andard4 (Figs. 7-8 and 7-9).
When to Use FDT
FD is a good es or general glaucoma
screening because i is quick, inexpensive,
easily adminis ered, and highly sensi ive and
speci c.
I is e ec ive in de ec ing visual eld loss
in glaucoma.
FD can de ec neurologic diseases,
including an erior ischemic op ic neuropa hy,
pseudo umor cerebri, and compressive op ic
neuropa hies.
Limitations
T e s andard version o FD shows 19
spo s, each sub ending 10 degrees o visual
arc, in he N-30 glaucoma es ing program.
T e number o spo s is signi can ly ewer,
wi h each spo covering a larger area, com-
pared wi h he s andard 24–2 Humphrey
visual eld program. In he 24–2 s andard,
here are 54 spo s, each covering 4 degrees o
arc.
T e Ma rix FD device (Carl Zeiss
Medi ec, Inc., Dublin, CA) incorpora es more
es spo s, wi h each spo covering a smaller
visual arc, in order o increase spa ial resolu-
ion wi h his device.
EFE ENCES
1. Quigley HA, Dunkelberger G , Baginski A, e al.
Chronic human glaucoma causes selec ively grea er
loss o large op ic nerve bers. Oph halmology. 1988;
95:357–363.
2. Dondona L, Hendrickson A, Quigley HA. Selec ive
e ec s o experimen al glaucoma on axonal rans-
por by re inal ganglion cells o he dorsal la -
eral genicula e nucleus. Inves Oph halmol Vis Sci.
1991;32:1593–1599.
3. Cha urvedi N, Hedley-Why e E , Dreyer EB. La eral
genicula e nucleus in glaucoma. Am J Oph halmol.
1993;116:182–188.
4. Quigley HA. Iden i ca ion o glaucoma-rela ed
visual eld abnormali y wi h he screening pro ocol
o requency doubling echnology. Am J Oph halmol.
1998;125:819–829.
 Functional Tests 93

FIGURE 7-8. FDT, normal eye.

 94 7 EVALUATION O F THE O PTIC NERVE AND NERVE FIBER LAYER

FIGURE 7-9. FDT, glaucoma ous eye. FD o he same glaucoma ous eye shown in Figure 7 4. T e righ eye
shows a superior and in erior s ep de ec .

MULTIFOCAL
ELECT O ETINOGR PHY
E lec rore inography objec ively es ab-
lishes he loss o re inal unc ion. In mul-
i ocal elec rore inography (m E G), ocal
responses are ob ained rom a large number o
re inal pa ches, and opographic maps o dys-
unc ional areas are derived.
How mfERG Works
All ocal areas are independen ly and
concurren ly s imula ed as he E G signal is
derived rom he cornea by means o a con-
ac lens elec rode. T e special ma hema ical
scheme o mul i ocal s imula ion permi s pre-
cise ex rac ion o he ocal response con ribu-
ions rom he single E G signal. No pa ien
response is required.
Using he visual evoked response imaging
sys em (VE IS; Elec ro-Diagnos ic Imaging,
San Ma eo, CA), he s imulus may consis o
up o several hundred ocal s imuli.
ypically, 103 hexagonal pa ches displayed
on a video moni or s imula e he cen ral
50 degrees o he pa ien ’s visual eld
(Fig. 7-10).
Functional Tests 95
In mos cases, he ocal s imula ion con-
sis s o pseudorandomly presen ed ashes.
T e ocal elec rophysiologic response sig-
nals are organized and displayed opographi-
cally o produce unc ional maps o he re ina,
similar o hose o visual eld es ing.
When to Use mfERG
Al hough mos o he E G response
origina es in he ou er layers o he re ina
(pho orecep ors, bipolar cells), he m E G
can be used o objec ively measure ganglion
cell unc ion. A por ion o he response signal
origina es rom ganglion cell bers in he
vicini y o he op ic nerve head. T is compo-
nen is diminished in glaucoma pa ien s.
T is echnique does no require pupil
dila ion.
Special paradigms are being developed
and es ed ha enhance, isola e, and map his
response componen .
Limitations
Curren ly, he m E G is used experimen-
ally and is no included in common clinical
prac ice.
 96 7 EVALUATION O F THE O PTIC NERVE AND NERVE FIBER LAYER

Multifoca l ERG

Monitor S cre e n 103 s ca le d e le me nts

~50°

Conta ct Le ns
Ele ctrode

Amplifie r
100k ga in 10-300 Hz

Compute r
fa s t M tra ns form

103 foca l 1 µv
ERGs
100 ms

fove a l pe a k blind s pot

Re s pons e
De ns itie s

FIGURE 7-10. m ERG. Schema ic display o he m E G showing s imulus array, he response race array, and
hree- and wo-dimensional plo s. (Cour esy o Erich Sut er, PhD, Elec ro-Diagnos ic Imaging, San Ma eo, CA.)

VISUALLYEVOKED
CO TICAL POTENTIAL
T he visually evoked cor ical po en ial
(VECP, also abbrevia ed VEP or VE or
visually evoked response) is an elec rical sig-
nal genera ed by he occipi al visual cor ex in
response o s imula ion o he re ina by ei her
ligh ashes or pat erned s imuli (Fig. 7-11).
Pat ern VEP is now pre erred over ash VEP
or he evalua ion o he visual pa hways,
owing o i s enhanced sensi ivi y in de ec ing
axonal conduc ion de ec s.
How VEP Works
T e VEP measures he elec rical response
o he brain’s visual cor ex o pat erned or
ashed s imuli.
T e VE po en ial is measured be ween
elec rodes on he scalp. One elec rode, which
measures he response i sel , can be placed
over or la eral o he ex ernal occipi al pro u-
berance (or inion; see Fig. 7-11B), loca ed
close o he primary visual cor ex. Ano her
elec rode is placed a a re erence loca ion. T e
nal elec rode is used or grounding.
Functional Tests 97
When to Use VEP
T e VEP is primarily used o iden i y
visual loss secondary o diseases o he op ic
nerve and an erior visual pa hways.
T e mul i ocal echnique described in he
previous sec ion can also be applied o he
cor ical response (mul i ocal visually evoked
cor ical po en ial, m VEP). In his ins ance,
s imulus arrays are usually con gured in a
dar board pat ern whereby each sec or con-
ains a con ras reversing checkerboard s imu-
lus. T e di cul y wi h his me hod is ha a
reduc ion or absence o local responses is due
in par o he convolu ed cor ical ana omy
and does no always re ec loss o unc ion.
However, unila eral local loss o unc ion
can be demons ra ed by comparison o he
response maps rom he wo eyes. ecen
s udies have shown correla ions be ween he
VEP and visual eld de ec s.
Limitations
Similar o he m E G, much more work
remains o be done wi h m VEP prior o gen-
eral clinical adop ion o his echnique.
 98 7 EVALUATION O F THE O PTIC NERVE AND NERVE FIBER LAYER

A B
FIGURE 7-11. m VEP. A. S imulus and response array o a normal m VEP. (A, B, Cour esy o Erich Sut er,
PhD, Elec ro-Diagnos ic Imaging, San Ma eo, CA.) B. Diagram o elec rode placemen s above and la eral o he
inion.

STRUCTURAL TESTS
G laucoma can be measured hrough he
assessmen o op ic nerve head cupping,
re inal NFL de ec s, and possibly macular
hickness. T ese are among he reliable signs
o glaucoma and i s progression.
T e developmen o noninvasive, objec ive
echniques ha measure re inal s ruc ures
mos likely o su er glaucoma ous damage
aids in he diagnosis o glaucoma and in he
moni oring o progressive glaucoma ous dam-
age. S ereoscopic and NFL pho ography are
among he simples echnologies ha can be
used or assessing glaucoma ous s ruc ural
damage; however, new compu erized image
analysis echniques have been developed or
more objec ive and quan i a ive measuremen s
o he re inal NFL and op ic nerve head.
PHOTOGR PHY
S ereoscopic op ic nerve head pho ogra-
phy is one o he mos widely used op ic
nerve head imaging echnologies. NFL pho-
ography, more di cul and less requen ly
used han op ic nerve head pho ography,
permi s ex ended evalua ion o he NFL ol-
lowing a pa ien examina ion. Speci c re i-
nal abnormali ies associa ed wi h glaucoma
include ocal and di use NFL hinning. NFL
losses in glaucoma correla e wi h visual eld
abnormali ies.
How Stereoscopic Photography Works
S ereo images can be produced using
sequen ial (consecu ive) or simul aneous
pho ographic echniques.
Sequen ial s ereoscopic pho ography cap-
ures wo consecu ive images using a manual
shif o he camera joys ick.
Simul aneous s ereoscopic pho ography
cap ures ins an aneous s ereo images wi h a
single exposure o produce a spli - rame image
Structural Tests 99
o wo images on one or wo 35-mm slides,
depending on he sys em used (Fig. 7-12).
T e images can be viewed s ereoscopically
using a specialized viewing appara us.
When to Use Stereoscopic Photography
S ereoscopic op ic nerve head pho ogra-
phy should be used whenever available every
1 o 2 years o evalua e glaucoma suspec s and
glaucoma pa ien s or progressive disease.
Limitations
S ereoscopic op ic nerve head pho og-
raphy does no o er an objec ive sys em or
in erpre a ion o he op ic nerve.
How NFL Photography Works
T e NFL is composed o he axons rom he
ganglion cells, neuroglia, and as rocy es. Axons
o he ganglion cells ravel oward he op ic
nerve in an organized ashion (Fig. 7-13).
T e NFL is bes observed using a red- ree,
blue or green ligh . Green or blue waveleng hs
are highly absorbed by he re inal pigmen
epi helium and choroid, while he axon
bundles re ec he ligh and appear as silvery
s ria ions (Figs. 7-14 and 7-15).
When to Use NFL Photography
NFL examina ion is use ul in dis inguish-
ing be ween glaucoma suspec s and rue glau-
coma damage.
De ec s in he NFL may precede
op ic nerve head and visual eld changes.
T ere ore, correla ing NFL appearance wi h
visual elds is an objec ive way o con rming
a subjec ive nding in au oma ed perime ry.
Limitations
Media opaci ies such as ca arac , poorly
ocused pho ographs, and poor con ras
because o a ligh ly pigmen ed undus are
among he ac ors ha can cause di cul y in
evalua ing or pho ographing he NFL.
 100 7 EVALUATIO N O F THE O PTIC NERVE AND NERVE FIBER LAYER

A B
FIGURE 7-12. S ereoscopic pho ography. A. S ereo pho ograph o normal eye. B. S ereo pho ograph o an
op ic nerve; however, supranasally, here is a NFL de ec .

FIGURE 7-13. NFL represen a ion. Lower drawing represen s he opography o he NFL where he dis al
ganglion cell axons projec o he peripheral area o he op ic disc rim. ( eprin ed wi h permission rom Schuman
JS. Imaging in Glaucoma. T oro are, NJ: SLACK; 1997.)
 Structural Tests 101

FIGURE 7-14. NFL pho ograph, normal eye.

FIGURE 7-15. Color NFL pho ograph, normal eye.

 102 7 EVALUATIO N O F THE O PTIC NERVE AND NERVE FIBER LAYER

SCANNING LASE POLA IMET Y e arda ion da a a 65,536 individual re i-

S canning laser polarime ry (SLP) derives

peripapillary NFL hickness rom mea-
suremen s o o al ocular bire ringence.
nal loca ions (256 by 256 pixels) covering 15
degrees are ob ained rom circular band o 1.5
o 2.5 disc diame ers concen ric o he disc.
Each pixel is quali a ively illus ra ed as yellow
How SLP Works and whi e or high re arda ion and dark blue
or low re arda ion.
T e GDx (Laser Diagnos ic echnologies,
San Diego, CA) akes advan age o he prop- When to Use SLP
er ies o he in erac ion o polarized ligh wi h
SLP may be use ul in de ec ing glaucoma
bire ringen issue o measure he hickness o
and ollowing i s progression.
he NFL. T is echnique is based on he prin-
ciple ha he bire ringence o he NFL causes Limitations
a change in he s a e o he polarized ligh ,
T e cornea and lens represen signi can
known as re arda ion. T is re arda ion is lin-
sources o bire ringence ha may al er he
early rela ed o he hickness and op ical prop-
re ardance and lead o inaccura e measure-
er ies o he NFL1 (Figs. 7-16 and 7-17).
men s o NFL hickness, even wi h he new-
T e 780-nm near-in rared diode polar- es sof ware.
ized ligh source is ocused on o one poin
T e magni ude o re arda ion represen s
o he re ina. T e polarized ligh pene ra es
a rela ive, ra her han absolu e, measure o
he NFL and is par ially re ec ed back rom
NFL hickness. Con ounding o SLP NFL
i s deeper layers. T e polarized s a e o he
hickness measuremen s by nonre inal (e.g.,
re ec ed ligh is analyzed digi ally. In he
corneal and len icular) bire ringence is an
earlies model, a xed compensa ion device
obs acle o he widespread u ili y o his
neu ralized he average an erior segmen
echnology.
bire ringence.
In subsequen models o he GDx, how-
EFE ENCE
ever, variable corneal compensa ion (VCC)
1. Weinreb N, Shakiba S, Zangwill L. Scanning laser
and enhanced corneal compensa ion (ECC) polarime ry o measure he nerve ber layer o
were developed o improve he signal- o-noise normal and glaucoma ous eyes. Am J Oph halmol.
ra io and improve he usabili y o he da a. 1995;119:627–636.
 Structural Tests 103

FIGURE 7-16. SLP, normal eye. SLP o a normal eye showing he CSLO image in he upper le corner and
he bire ringence represen a ion in he upper righ . T e re ardance (NFL hickness) da a are shown in he
middle le panel. T e subjec eye is shown in dark blue, while he 95% conf dence in erval or he normal range
is illus ra ed in ligh blue. T e “Devia ion rom Normal” char is shown in he middle righ panel. Nerve f ber
analysis parame ers are displayed a he bot om.
 104 7 EVALUATIO N O F THE O PTIC NERVE AND NERVE FIBER LAYER

FIGURE 7 17. SLP, glaucoma ous eye. SLP o he same glaucoma ous eye shown in Figures 7-4 and 20-9.
No e he general reduc ion in re arda ion illus ra ed in he “Nerve Fiber Layer” graph, and he devia ion rom
normal represen ed in he middle righ panel. NFL parame ers ha are borderline or ou side o normal limi s
are highligh ed.

CONFOCAL SCANNING
LASE OPHTHALMOSCOPY
C on ocal scanning laser oph halmoscopy
(CSLO) is a me hod or acquiring and
analyzing real- ime hree-dimensional opo-
graphic images o he op ic nerve head.
How CSLO Works
T e Heidelberg re ina omograph (H ;
Heidelberg Engineering GmbH, Heidelberg,
Germany) is he only curren ly available con-
ocal scanning laser oph halmoscope.
I uses a con ocal scanning sys em based
on he principle o spo illumina ion and spo
de ec ion. In his sys em, one spo on he
re ina or op ic nerve head is illumina ed a a
ime, allowing only ligh origina ing rom he
illumina ed area o pass hrough he aper ure
while scat ered ligh and issue planes ha are
ou o ocus do no . T us, areas ha do no lie
close o he plane o ocus are no illumina ed
and are no seen. T is allows or high-con ras
images. In addi ion, layer-by-layer ( omo-
graphic) imaging wi hin he re ina and op ic
nerve head is possible.
H uses a diode laser o 670 nm o scan
and analyze he pos erior segmen . A hree-
dimensional image is ob ained rom a series
o op ical sec ions a 16 o 64 consecu ive
ocal planes. T e in orma ion is displayed
in wo images—a opographic image and a
re ec ivi y image (Figs. 7-18 and 7-19). T e
opographic image consis s o 256 × 256 or
384 × 384 pixel elemen s, each o which is a
measuremen o heigh a i s corresponding
loca ion. T e op ical ransverse resolu ion is
approxima ely 10 µm, whereas he longi udi-
nal resolu ion is abou 300 µm.
In curren clinical prac ice, hree scans o
each eye are aken and hen averaged o crea e
a mean opographic image, and compared o
a norma ive da abase in an analysis called he
Structural Tests 105
Moor eld’s regression analysis (MR )
(Fig. 7-20). A second way o analyzing he
da a ob ained is he glaucoma probabili y
score (GPS), which has he advan age o
being independen o an opera or-drawn
con our line1 (Fig. 7-21).
Images can be ob ained hrough undi-
la ed pupil, bu dila ion will improve image
quali y in pa ien s wi h small pupils and
ca arac s.2 eproducibili y is bes in undi-
la ed eyes.3
opographic change analysis ( CA) pro-
vides progression analysis capabili ies wi h
he con ocal scanning laser. Por ions o he
opographic image which have less volume
han baseline are highligh ed in red, whereas
hose which have gained volume are high-
ligh ed in green (Fig. 7-22).
When to Use CSLO
CSLO may be use ul in de ec ing glau-
coma and ollowing i s progression.
Limitations
CSLO op ic nerve head measuremen s
require a re erence plane o calcula e many
o he parame ers: cup area, cup- o-disc
ra io, cup volume, rim area, rim volume,
re inal NFL hickness, and re inal NFL cross-
sec ional area. T e re erence plane used by
he curren sof ware may change over ime,
especially in pa ien s wi h glaucoma who
have changing opography.3 T is varia ion
can lead o inaccura e measuremen s. T e
user is required o de ne he op ic nerve head
border. Cup shape, cup volume below he sur-
ace, mean cup dep h, maximum cup dep h,
and disc area are he parame ers ha are inde-
penden o he re erence plane.
Misalignmen be ween he pa ien and
he scanner in he horizon al plane is also a
po en ial source o signi can variabili y.
 106 7 EVALUATIO N O F THE O PTIC NERVE AND NERVE FIBER LAYER

EFE ENCES by re inal hickness mapping. Oph halmology. 1998;

1. Alencar LM, Bowd C, Weinreb N, e al. Comparison
o H -3 glaucoma probabili y score and subjec ive
s ereopho ograph assessmen or predic ion o pro-
gression in glaucoma. Inves Oph halmol Vis Sci. 2008;
49(5):1898–1906.
2. Zeimer , Asrani S, Zou S, e al. Quan i a ive de ec-
ion o glaucoma ous damage a he pos erior pole
105:224–231.
3. Mikelberg F, Wijsman K, Schulzer M. eproduc-
ibili y o opographic parame ers ob ained wi h he
Heidelberg re ina omograph. J Glaucoma. 1993;2:
101–103.

B
FIGURE 7-18. CSLO, normal eye. CSLO o a normal eye using he H I. A. opographic image (le ) and
re ec ivi y image ( righ ) showing he ONH image and con our graph. In he graph, he whi e line represen s
he re erence plane a which here is a heigh o zero. T e red line represen s he heigh o he re erence line
be ween he cup and disk. T e green line is he re inal heigh o he subjec eye a he con our line showing he
ypical double-hump ea ure a he superior and in erior poles. B. opographic image wi h he cup represen ed
in red, he sloping neural issue in blue, and he rim in green. T e ONH parame ers and subjec classif ca ion are
lis ed on he righ . T e classif ca ion number or he H I is de ermined by an au oma ed algori hm devised by
Frederick Mikelberg based on he ONH and re inal parame ers. T e classif ca ion number or H II is derived
rom an algori hm developed by Wolls ein e al. a Moorf elds Eye Hospi al.
 Structural Tests 107

B
FIGURE 7-19. CSLO, glaucoma ous eye. CSLO o he same glaucoma ous eye shown in Figures 7 4, 7 9, and
7 17. A. opographic and re ec ivi y images. B. ONH analysis and measured parame ers.
 108 7 EVALUATIO N O F THE O PTIC NERVE AND NERVE FIBER LAYER

FIGURE 7 20. Moorf eld’s regression analysis prin ou , glaucoma ous eye. Signal s reng h and disc size
described a op o page. Applica ion o he MR represen ed as red X, yellow “!,” and green check over. Con our
line represen ed by green line circum eren ial o op ic nerve.
 Structural Tests 109

FIGURE 7-21. GPS, glaucoma ous eye.

FIGURE 7-22. TCA. Progression shown by increasing area o red signal in he in ero emporal neurore inal rim.
 110 7 EVALUATIO N O F THE O PTIC NERVE AND NERVE FIBER LAYER
OPTICAL COHE ENCE
TOMOGR PHY
O p ical coherence omography (OC )
compu es NFL hickness measuremen s
rom high-resolu ion cross-sec ional images o
he re ina. Newer spec ral-domain OC ech-
nology has signi can ly improved he resolu-
ion, scan densi y, and use ulness o OC in
de ec ing glaucoma, and several machines
now include progression analysis in he sof -
ware package.
How OCT Works
OC uses low-coherence ligh in an in er-
erome er o per orm high-resolu ion imag-
ing. T e opera ion o OC is analogous o
ul rasound B-mode imaging or radar excep
ha ligh is used ra her han acous ic or radio
waves. ime-domain OC measuremen s o
dis ance and micros ruc ure are per ormed by
measuring he ime o igh o ligh re ec ed
rom di eren micros ruc ural ea ures wi hin
he eye. A-scans are collec ed pixel-by-pixel,
wi h a vibra ing mirror on he re erence
arm providing he loca ion in space o each
re ec ed pixel. Spec ral-domain OC mea-
sures he en ire A-scan simul aneously, and
uses requency in orma ion in he backre ec-
ion o iden i y he spa ial loca ion o each
pixel. A sequence o longi udinal measure-
men s (i.e., A-scans) is used o cons ruc a
alse-color opographic image o issue micro-
sec ions ha appears remarkably similar o
his ologic sec ions (Figs. 7-23 and 7-24).
T e original S ra us ime-domain OC
has a longi udinal-axial resolu ion o abou
10 µm and a ransverse resolu ion o approxi-
ma ely 20 µm. Scan speed in commercial
uni s is 400 A-scans per second. Newer spec-
ral-domain OC s scan a he ra e o 25,000
o 55,000 A-scans per second, have an axial
resolu ion o 5 µm and a ransverse resolu-
ion o 15 µm.1
Commercially available SD-OC s in
he US a he ime o his wri ing include
Biop igen, Inc., Durham, NC; Cirrus, Carl
Zeiss Medi ec; Spec ralis, Heidelberg
Engineering, Heidelberg, Germany; Spec ral
OC / SLO, Opko, Miami, FL; SOC
Copernicus H , Op opol echnology SA,
Zabia, Poland; Vue, Op ovue, Fremon ,
CA; 3D OC 2000, opcon, Oakland, NJ.
T e as er scanning a orded by SD-OC
allows hree-dimensional image cap ure,
grea er scanning densi y, higher reproducibil-
i y, and may improve sensi ivi y and speci c-
i y in disease and progression de ec ion.
In he clinical evalua ion o glaucoma,
Cirrus OC provides a 6 mm × 6 mm cube o
da a as well as he 3.4-mm circle pat ern car-
ried over rom S ra us echnology (Fig. 7-25).
Spec ralis OC adds eye racking or ease
o image acquisi ion (Fig. 7-26). Vue
OC also includes an NFL hickness map,
bu addi ionally can direc ly image he gan-
glion cell complex in he macula2 (Fig. 7-27).
Bo h Cirrus OC and Vue OC include
progression analysis sof ware (Figs. 7-28
to 7-31).
Unlike CSLO, OC does no require a
re erence plane. T e NFL hickness is an
absolu e cross-sec ional measuremen . T e
re rac ive s a e or he axial leng h o he eye
does no a ec axial OC measuremen s.
OC measuremen s o NFL hickness are no
dependen on issue bire ringence.
When to Use OCT
OC is use ul in de ec ing glaucoma and
ollowing i s progression.
Limitations
Media opaci ies may limi he abili y o
per orm OC ,3 hough he spec ral-domain
OC has shown improvemen rom pas
echnology.
 Structural Tests 111

EFE ENCES op ical coherence omography. Oph halmology.

1. Sung K , Kim JS, Wolls ein G, e al. Imaging o he
re inal nerve bre layer wi h spec ral domain op ical
coherence omography or glaucoma diagnosis. Br J
Oph halmol. 2010.
2. an O, Chopra V, Lu A , e al. De ec ion o macular
ganglion cell loss in glaucoma by Fourier-domain
2009;116(12):2305–2314.e1–2.
3. Swanson E, Izat , Hee M, e al. In vivo re inal imag-
ing by op ical coherence omography. Op Let .
1993;18:1864–1866.

FIGURE 7-23. OCT, normal eye. OC peripapillary circular scan o a normal eye. NFL hickness is graphed
below he scan image. Average overall NFL hickness is shown in he righ middle panel. NFL hickness by
quadran and clock hour is displayed on he bot om righ . T e bot om le image shows a video rame o he
undus during scanning.
 112 7 EVALUATIO N O F THE O PTIC NERVE AND NERVE FIBER LAYER

FIGURE 7-24. OCT, glaucoma ous eye. OC peripapillary circular scan o he same glaucoma ous eye shown
in Figures 7 4, 7 9, 7 17, and 7 19. No e he considerably hinner NFL hickness measuremen s.
 Structural Tests 113

FIGURE 7-25. Cirrus spec ral domain OCT, glaucoma ous eyes. OC o peripapillary re inal NFL showing
generalized and superior and in erior hinness in bo h eyes.
 114 7 EVALUATIO N O F THE O PTIC NERVE AND NERVE FIBER LAYER

FIGURE 7-26. Spec ralis spec ral domain OCT, glaucoma ous eye. OC o peripapillary re inal nerve f ber
laying showing ocal de ec in ero emporally.
 Structural Tests 115

FIGURE 7-27. RTVue spec ral domain OCT, glaucoma ous eye. OC o macular ganglion cell complex in
glaucoma ous eye showing ocal hinness in ero emporally in he le eye.

FIGURE 7-28. Cirrus spec ral domain OCT progression analysis. OC GPA showing progression
superiorly, in eriorly, and overall in a glaucoma ous eye.
 116 7 EVALUATIO N O F THE O PTIC NERVE AND NERVE FIBER LAYER

FIGURE 7-29. OCT, normal eye. OC macular scan o a normal eye. T e macula has a ring o issue
surrounding he ovea ha is hicker han he ovea cen ralis, as can be seen in he macular hickness map. T e
map is displayed in alse color, and he quan i a ive da a are shown o i s righ .
 Structural Tests 117

FIGURE 7-30. OCT, normal eye. OC ONH scan o a normal eye. T is scan prof le illus ra es he op ic disc
physical charac eris ics.
 118 7 EVALUATIO N O F THE O PTIC NERVE AND NERVE FIBER LAYER

FIGURE 7-31. OCT, glaucoma ous eye. OC macular scan o ano her glaucoma ous eye showing macular
hinning.
 Structural Tests 119

ETINAL THICKNESS ANALYZE o re inal ganglion cells reside in he macula,

R e inal hickness analyzer ( A; alia

echnology, Mevasere Zion, Israel)
compu es macular hickness and displays he
measuremen in wo- and hree-dimensional
images.
and re inal ganglion cells are signi can ly
hicker han heir axons (which comprise he
NFL), macular hickness may be a good mea-
sure o glaucoma. A and OC may bo h be
used or macular hickness assessmen .

When to Use RTA

How RTA Works
A can be use ul in de ec ing glaucoma
e inal hickness mapping wi h he A
and ollowing i s progression.
uses a green, 540-nm HeNe laser sli beam
o image he re ina. T e dis ance be ween Limitations
he laser’s in ersec ion wi h he vi reo-re inal
A requires a 5-mm pupil size and is
in er ace and he re ina–re inal pigmen epi-
limi ed by eyes wi h numerous oa ers or sig-
helial in er ace is direc ly propor ional o he
ni can media opaci y.
re inal hickness (Fig. 7-32).
Because o he shor er waveleng h o he
Nine scans wi h nine separa e xa ion
ligh used or A, his echnology is more
arge s are aken. T e composi ions o hese
sensi ive o nuclear sclero ic ca arac han
scans cover he cen ral 20 degrees (measuring
OC , CSLO, or SLP.
6 × 6 mm) o he undus.
Axial leng h and re rac ive error are
Unlike OC and SLP, which measure he
needed o conver values in o absolu e hick-
NFL, or CLSO or OC , which measure op ic
ness values.
nerve head con our, A measures macular
hickness. Because he highes concen ra ions

FIGURE 7-32. Re inal hickness mapping. Normal eye analyzed wi h he A. T e wo- and hree-
dimensional images are shown in alse color.
 C H AP T ER
8
Psychophysical es ing
T he broad erm psychophysical testing
re ers o he subjec ive es ing o vision
o an eye. In clinical erms or he glaucoma
pa ien , his involves perime ry o assess he
peripheral vision o an eye. Because he pa ho-
physiology o glaucoma af ec s he peripheral
vision be ore af ec ing cen ral acui y, here are
bo h diagnos ic and herapeu ic bene s o
assessing he pa ien ’s visual eld. I is impor-
an o no e ha usage o he erm peripheral
vision does no necessarily mean ar periphery.
In ac , mos glaucoma ous visual eld de ec s
occur paracen rally (wi hin 24 degrees o xa-
ion). Our use o he erm peripheral re ers o
any hing beyond cen ral xa ion (i.e., grea er
han he cen ral 5 o 10 degrees).
T e goal o his chap er is o show represen-
a ive visual eld pat erns or glaucoma ra her
han provide a comprehensive discussion o
perime ry. T ere are several ex s dedica ed
solely o he ex ended descrip ion o perim-
e ry and a lases dedica ed o jus perime ric
ndings.
120
Douglas J. Rhee, Tara A. Uhler, and L. Jay Katz
BIBLIOGRAPH Y
Anderson DR, Pa ella VM. Automated Static Perimetry.
2nd ed. S . Louis, MO: Mosby; 1999.
Aulhorn E, Harms H. Early visual eld de ec s in glau-
coma. In: Leydhecker W, ed. Glaucoma Symposium,
Tutzing Castle, Basel, Switzerland: S. Karger; 1966.
1967:151–186.
Budenz DL. Atlas of Visual Fields. Philadelphia, PA:
Lippincot -Raven; 1997.
Drance SM, Wheeler C, Pa ullo M. T e use o s a ic
perime ry in he early de ec ion o glaucoma. Can J
Ophthalmol. 1967;2:249–258.
Harwer h RS, Car er-Dawson L, Shen F, e al. Ganglion
cell losses underlying visual eld de ec s rom
experimen al glaucoma. Invest Ophthalmol Vis Sci.
1999;40:2242–2250.
Heijl A. Au oma ic perime ry in glaucoma visual eld
screening. A clinical s udy. Graefes Arch Clin Exp
Ophthalmol. 1976;200:21–37.
Heijl A, Lundqvis L. T e loca ion o earlies glauco-
ma ous visual eld de ec ed documen ed by au o-
ma ic perime ry. Doc Ophthalmol Proceed Series.
1983;35:153–158.
Ka z J, ielsch JM, Quigley HA, e al. Au oma ed perim-
e ry de ec s visual eld loss be ore manual Goldmann
perime ry. Ophthalmology. 1995;102:21–26.
Lynn JR. Examina ion o he visual eld in glaucoma.
Invest Ophthalmol. 1969;8:76–84.
Quigley HA, Dunlelberger GR, Green WR. Re inal gan-
glion cell a rophy correla ed wi h au oma ed perim-
e ry in human eyes wi h glaucoma. Am J Ophthalmol.
1989;107:453–464.

PURPOSE
PU
U RP
P O SE
E OF ES
ES
DIAGNOSIS
As par o he ini ial evalua ion o a pa ien
suspec ed o having glaucoma, au oma ed
monochroma ic visual eld es ing is an
impor an aspec o he diagnos ic de ermina-
ion o glaucoma ous op ic nerve damage.
Visual eld abnormali ies have localizing
value or lesions along he en ire visual rac ,
which ex ends rom he re ina o he occipi al
lobes. Glaucoma ous visual eld de ec s are
hose ha are ypically ound wi h lesions
localizing o he op ic nerve.
I is impor an o no e ha he presence
o a so-called op ic nerve eld (i.e., de ec s
ha localize o he op ic nerve) is no solely
diagnos ic o glaucoma. T is mus occur in he
presence o a charac eris ic op ic nerve appear-
ance (covered in Chap er 6) and his ory.
O her ndings, such as in raocular pres-
sure, gonioscopic appearance, and an erior
segmen ndings, may help ca egorize he
speci c ype o glaucoma. All op ic neuropa-
hies (e.g., an erior ischemic op ic neuropa-
hies, compressive op ic neuropa hies, e c.)
demons ra e “op ic nerve” visual elds.
Purpose of Test 121
I is also cri ical o no e ha he
absence o an op ic nerve eld does no
exclude he diagnosis o glaucoma. Al hough
in he year 2002 au oma ed achroma ic s a ic
visual eld (AASVF) es ing is he gold s an-
dard or he evalua ion o op ic nerve unc ion,
i s hreshold o sensi ivi y o de ec ganglion
cell loss is s ill limi ed. Clinicopa hologic and
experimen al evidence indica es ha he earli-
es visual eld de ec de ec able by AASVF
corresponds o approxima ely 40% ganglion
cell loss.
MANAGEMENT
AASVF es ing along wi h serial op ic
nerve evalua ions remains he gold s andard
or he moni oring o glaucoma.
T e goal (or arge ) in raocular pressure
is he herapeu ic range a which we modi y
he ocular physiology in an at emp o pro ec
he op ic nerve rom ur her baro raumas.
However, he de ermina ion o he arge
pressure is empirical, meaning ha we es i-
ma e wha he goal should be. AASVF and
serial op ic nerve evalua ions are he ways
in which we de ermine i ha empirically
derived pressure range is ac ually ef ec ive a
pro ec ing he op ic nerve.
 122 8 PSYCHO PH YSICAL TESTING
DESCRIP
D ES
SC R I P IIO
ON
Perime ric es ing at emp s o de ermine
he visual hreshold a a par icular loca ion in
he visual eld. T e visual hreshold is de ned
as he minimum level o ligh ha can be per-
ceived a a given loca ion in he visual eld;
his concep is also ermed retinal sensitivity.
T is is a dif eren concep rom he low-
es level o pho ic energy ha will s imu-
la e a pho orecep or cell or area o re ina.
Perime ric es ing relies on he pa ien o
subjec ively de ermine wha he or she can
see. T ere ore, he visual hreshold is subjec
o some level o cogni ive and in rare inal
processing, hence he name psychophysical
es ing.
T e visual hreshold is highes in he
ovea, which is de ned as he cen er o he
visual eld. As he eld ex ends peripherally,
he sensi ivi y decreases. T e hree-dimen-
sional represen a ion o his is o en called he
“hill o vision” (Fig. 8-1). T e visual eld
or one eye ex ends 60 degrees superiorly,
60 degrees nasally, 75 degrees in eriorly, and
100 degrees emporally.
T ere are wo main me hods o perime ry:
s a ic and kine ic (Fig. 8-2).
His orically, various orms o kine ic
es ing were developed rs , and hese are
generally per ormed manually. Brie y, a
visual s imulus o known size and in ensi y
is brough rom he ar periphery, where
i would no be expec ed o be perceived,
oward he cen er. A some poin , i will
move in o an area where i is perceived;
his is he visual hreshold a ha loca ion.
T is process is con inued wi h varying
s imuli o size and in ensi ies o give a
opographic map o he hill o vision. T e
Goldmann visual eld at emp s o map he
en ire visual eld (Fig. 8-3).
S a ic visual eld es ing presen s visual
s imuli in varying sizes and in ensi ies a
xed loca ions. Al hough here are many
dif eren s ra egies or de ermining he
visual hreshold, mos use he ollowing
basic principle.
T e examiner begins by presen -
ing s imuli o higher in ensi y and, in
measured s eps, presen s s imuli o
lower in ensi y un il he pa ien no
longer perceives he s imulus. T en he
es is usually rechecked by presen -
ing a gradually increasing in ensi y o
s imulus in smaller incremen s un il he
pa ien again can perceive he s imulus.
T a in ensi y o ligh is de ned as he
visual hreshold in ha region o he
visual eld. Generally, s a ic visual eld
es ing is au oma ed and presen s whi e-
colored s imuli on a whi e background,
hence he name AASVF es ing.
T ere are many makers o AASVF
machines, among hem are Humphrey
(Allergan, Irvine, CA), Oc opus
(Fig. 8-4), and Dicon. In our prac ice,
we pre er he Humphrey machine.
Various es ing algori hms have been
developed, such as ull hreshold, FAS PAC,
S A PAC, and Swedish in erac ive hreshold
algori hm (SI A), among o hers. T ey vary
wi h regard o leng h o es ime and sligh ly
wi h regard o dep h o eld de ec .
 Description 123

Blind s pot
Fove a

FIGURE 8-1. T e “hill o vision.” A hree dimensional represen a ion o he visual hreshold in various
loca ions wi hin he visual f eld o a normal eye. (From Haley MJ, ed. T e Field Analyzer Primer. 2nd ed. San
Leandro, CA: Humphrey Ins rumen s; 1987:4. Fig. 1.)

FIGURE 8-2. Comparison o static and kinetic perimetr y. Slopes and sco omas are shown bet er by s a ic
han by kine ic perime ry. A. Al hough he normal visual f eld wi h i s gradual slope and absence o abnormal
sco omas is well ou lined by kine ic es ing, he presence o f eld de ec s makes his me hod less precise han
s a ic es ing. B. T e a emporal slope migh yield a response a any poin be ween 40 degrees and 12 degrees
i he es objec were op imum or es ing ha zone. Nasally, he bes chosen kine ic es migh be repor ed
anywhere be ween 25 degrees and 7 degrees, and i would miss he rela ive sco oma be ween 7 degrees and
12 degrees. C. When he slope is s eep, kine ic perime ry usually ou lines he de ec well wi h a ew well chosen
es objec s, bu he choice is o en arbi rary and may ail o reveal he ac ual s eepness o he slope. S a ic
es s elucida e well he a slopes and small sco omas in D and bo h kinds o slope in E. (From Leydhecker
W. Glaucoma. Symposium, u zing Cas le, held in Connec ion wi h he 20 h In erna ional Congress o
Oph halmology, Munich, Augus 1966; 1967:151–186. Wi h permission rom S. Karger AG, Basel, Swi zerland.)
 124 8 PSYCHO PH YSICAL TESTING

FIGURE 8-3. Goldmann visual f eld testing. Goldmann visual f eld es o he righ eye showing superior nasal
s ep and arcua e de ec .

FIGURE 8-4. Oc opus AASVF es wi h corresponding op ic nerve pho ograph and Heidelberg re inal
omography (HR ) scan.
 Common Optic Nerve Visual Fields Found in Patients with Glaucoma 125

raphe. Ganglion cells loca ed superiorly

CO
C O MMO
M M O N O P IC C N ERVE
ER
RVE o he horizon al raphe arc superiorly and
VISUAL
V ISUU AL FFIELDS
I EL
L D S FO
F O UN
ND deliver heir bers o he supero emporal
IIN
N PA
PA IEN
IE
EN S W WII H aspec o he op ic nerve.
GLAUCO
G LA
AU CO O MA
MA T e converse is rue or ganglion cells
loca ed emporal o he op ic nerve and
T e ana omic loca ion o he de ec in in erior o he horizon al raphe.
glaucoma is in he op ic nerve, wi h ocal De ec s in he op ic nerve af ec ing bers
spo s wi hin he lamina cribrosa. On he eld rom he area emporal o he op ic nerve
es , he visual de ec s mani es in rela ively produce bo h nasal s ep de ec s and arcua e
speci c pat erns because o he ana omy o de ec s.
he re inal nerve ber layer (RNFL). T e
RNFL is composed primarily o he axons T e nasal s ep de ec (Figs. 8-7 and
rom he ganglion cells projec ing hrough he 8-8) ge s i s name no only rom he nasal
op ic nerve o he la eral genicula e nucleus loca ion o he eld de ec bu also rom
(Fig. 8-5). he ac ha he de ec respec s he hori-
zon al median. T e horizon al raphe is he
Axons rom ganglion cells loca ed nasal o ana omic basis or his appearance.
he op ic disc ravel s raigh in o op ic disc;
de ec s in he op ic nerve af ec ing bers rom T e arcua e de ec ge s i s name rom
his region produce a emporal wedge de ec he appearance o he de ec (Fig. 8-9).
(Fig. 8-6). Nasal s ep and arcua e de ec s are ar more
common han emporal wedge de ec s.
Axons rom ganglion cells loca ed
emporal o he op ic nerve arc in o he As glaucoma progresses, mul iple
op ic nerve. A line ha in ersec s he ovea de ec s can presen (Fig. 8-10) in a single
and he op ic nerve de nes he horizon al eye (Fig. 8-11).
 126 8 PSYCHO PH YSICAL TESTING

FIGURE 8-5. Glaucomatous damage to nerve bundles and location o resulting visual abnormalities.
Damage a he lower pole o he op ic disc causes abnormali ies in he visual f eld as shown ( le eye) . (From
Anderson DR, Pa ella VM. Au oma ed S a ic Perime ry. 2nd ed. S . Louis, MO: Mosby; 1999:51. Fig. 4-4.)
 Common Optic Nerve Visual Fields Found in Patients with Glaucoma 127

B C

FIGURE 8-6. emporal wedge. A. Humphrey AASVF es ing. B. Corresponding op ic nerve pho ograph
showing some nasal hinning. C. Corresponding HR scan.
128
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 Common Optic Nerve Visual Fields Found in Patients with Glaucoma 129

B C
FIGURE 8-8. More advanced superior nasal step de ect. A. Humphrey AASVF es ing. B. Corresponding
op ic nerve pho ograph showing more advanced in ero emporal hinning. C. Corresponding HR scan.
 130 8 PSYCHO PH YSICAL TESTING

B C
FIGURE 8-9. Arcuate de ect. A. Humphrey AASVF es ing. B. Corresponding op ic nerve pho ograph
showing some nasal hinning. C. Corresponding HR I scan.
 Common Optic Nerve Visual Fields Found in Patients with Glaucoma 131

FIGURE 8-10. AASVF test rom a Humphrey machine demonstrating a combination o de ects. T ere are
bo h superior and in erior nasal s eps wi h bo h in erior and superior arcua e de ec s. T e in erior arcua e is more
prominen han he superior arcua e.

FIGURE 8-11. Summary o charac eris ic glaucoma ous visual f eld de ec s ha localize o he re inal nerve
f ber layer.
 132 8 PSYCHO PH YSICAL TESTING

o projec o he magnocellular layers o he

N EWER
EW
WE R PSYCH
P SY
YCHH O PH
PH YYSICAL
SIC
C AL la eral genicula e nucleus, while SWAP iso-
ES IN
INGG:: FREQ
F RE
E Q UEN
U EN C YY-- la es bis ra i ed blue-yellow ganglion cells
DO
D O UBLINN G PE
PERIME
E R IM
M E RY Y ha are sensi ive o blue s imuli ha are
AN
A ND S
SHH O R -W
-WAVE
WAVE hough o projec o he parvocellular layers
AU
AU O MA A ED
E D PERIME
PEE R I M E RY Y o he la eral genicula e nucleus.
T ese have an increased sensi ivi y o
Bo h requency-doubling perime ry de ec ion ha may be rela ed by isola ing a
(FDP) and shor -wave au oma ed perime ry subpopula ion o ganglion cells; here is a
(SWAP) rely on isola ing a subpopula ion o decreased unc ional reserve (Figs. 8-12
ganglion cells. to 8-14).
FDP isola es a subse o large-diame er
ganglion cells, called My cells, ha are hough

FIGURE 8-12. Schematic o unctional reserve. Yellow bar schema ically represen s he ull undamaged
number o ganglion cells. T e blue bars schema ically represen he level o ganglion cells in which symp oma ic
dys unc ion would occur. T us, he di erences as represen ed by he red and green brackets represen he
unc ional reserve.
 Newer Psychophysical Testing: Frequency-doubling Perimetry and Short-wave Automated Perimetry 133

B
FIGURE 8-13. Visual elds rom he same pa ien using he 24–2 es ing s ra egies o he (A) Humphrey
f eld analyzer ( Zeiss) wi h he SI A FAS s ra egy, (B) 24–2 SI A SWAP, and (C) 24–2 FDP. (D) T e
corresponding op ic nerve.
(continued)
 134 8 PSYCHO PH YSICAL TESTING

C D
FIGURE 8-13. ( Continued)
 Newer Psychophysical Testing: Frequency-doubling Perimetry and Short-wave Automated Perimetry 135

FIGURE 8-14. Example o abnormal HVF and

FDP. Corresponding achroma ic visual f eld (A), FDP
visual f eld (B), and corresponding op ic nerve (C). B
 C H AP T ER

9
Blood Flow in Glaucoma
Alon Harris, Brent Siesky, and Deepam Rusia

E leva ed in raocular pressure (IOP) is asso-

cia ed wi h glaucoma progression and is
presen ly he only major risk ac or approved
or rea men in pa ien s wi h primary open
angle glaucoma (POAG). However, despi e
success ully mee ing heir arge ed IOP, many
pa ien s con inue o experience glaucoma pro-
gression, indica ing ha o her risk ac ors are
involved.
Vascular risk ac ors, including sys emic blood
pressure, ocular per usion pressure, disc
hemorrhage, and migraine, have been linked
o glaucoma in popula ion-based s udies.
Addi ionally, clinical rials have demons ra ed
decreased ocular blood f ow and dys unc-
ional au oregula ion o blood f ow in pa ien s
wi h glaucoma. A consensus was ormed a he
2009 World Glaucoma Associa ion Mee ing
ha vascular dysregula ion may con ribu e
o he pa hogenesis o glaucoma and ha he
rela ionship be ween glaucoma, ocular blood
f ow, and per usion pressure should be ur her
inves iga ed.
Various s udies have demons ra ed abnor-
mal blood f ow in glaucoma pa ien s in he
re ina, op ic nerve, re robulbar vessels, and
he choroid. No single device is curren ly
able o assess blood f ow in all o hese areas,
so a mul i-ins rumen approach is necessary
o evalua e he various ocular vascular beds
(Fig. 9-1).
BIBLIOGRAPH Y
Harris A, Kagemann L, Ehrlich R, e al. Measuring and
in erpre ing ocular blood f ow and me abolism in glau-
coma. Can J Ophthalmol. 2008;43:329–336.
Weinreb RN, Harris A, eds. Ocular Blood Flow in
Glaucoma: T e 6th Consensus Report of the World
Glaucoma Association. Ams erdam, T e Ne herlands:
Kugler Publica ions; 2009.

136
 Blood Flowin Glaucoma 137

S LO – IC G
P OBF

S LO -
F lu o r e s c e in

H R F \ LDF
S RO

C DI Do p p le r O C T

FIGURE 9-1. Instruments used to measure hemodynamics. Dif erent technologies measure hemodynamics
in speci c ocular tissue beds.
 138 9 BLO O D FLO W IN GLAUCO MA

Description
SCA
SCAN
AN N IN
I N G LA
LASER
ASERR
Fluorescein dye is used in conjunc ion
OP
PHH H ALMOSCO
AL M O SC
C O PE
E wi h a low-pene ra ing laser beam requency
AN
NGGIO
IO
OGGRAPH
RAPH HY o op imize visualiza ion o re inal vessels.
High clari y allows isola ion o individual re i-

S canning laser oph halmoscope (SLO)

angiography builds upon f uorescein angi-
ography, one o he rs modern measuremen
nal vessels in bo h he superior and in erior
re ina (Fig. 9-3). AVP represen s he amoun
o ime be ween he rs appearance o dye in
echniques o ga her empirical da a abou he a re inal ar ery and i s appearance in he asso-
re ina. T e SLO overcomes many o he limi- cia ed vein (Fig. 9-4).
a ions o radi ional pho ographic or video
AVP allows localiza ion o measuremen s
angiography echniques by replacing he
o a speci c re inal quadran , and i has been
incandescen ligh source wi h a low-power
shown o be sensi ive o small changes in
argon laser beam o achieve bet er pene ra ion
re inal blood f ow. Mean dye veloci y, ano her
hrough lens and corneal opaci ies (Fig. 9-2).
parame er measured by SLO angiography,
T e laser beam requency is chosen according
evalua es he average speed o blood f owing
o proper ies o he injec ed dye, ei her f uores-
hrough large re inal vessels by measuring he
cein or indocyanine green. As dye en ers he
amoun o ime he dye akes o ravel be ween
eye, he ref ec ed ligh exi s he pupil where
wo loca ions on he same re inal ar ery. T e
a de ec or measures he in ensi y o he ligh
mean ransi ime (M ), a hird quan i able
in real ime. T is crea es a video signal, which
parame er, measures he amoun o ime ha
passes hrough a video imer and is direc ed
blood spends in he re inal vascula ure.
oward a digi al recorder. T e videos are hen
analyzed o -line o ob ain measuremen s
such as ar eriovenous passage ime (AVP) and
mean dye veloci y.

FLUORESCEIN SLO
ANGIO GRAPHY

Purpose
Evalua ion o re inal hemodynamics
 Scanning Laser Ophthalmoscope Angiography 139

FIGURE 9-2. SLO angiography. T e SLO can use either uorescein or indocyanine green dye to look at retinal
or choroidal vessels.

FIGURE 9-3. Fluorescein SLO angiography. Fluorescein SLO angiography provides high-clarity visualization
o retinal vessels.

FIGURE 9-4. AVP time. AVP time equals the time dif erences in dye arrival between the isolated retinal artery
and adjacent retinal vein.
 140 9 BLO O D FLO W IN GLAUCO MA

INDO CYANINE GREEN wo areas adjacen o he op ic disk and

SLO ANGIOGRAPHY our areas surrounding he macula, each
25 × 25 pixels, are selec ed (Fig. 9-5). Area
Purpose dilu ion analysis (Fig. 9-6) measures he
brigh ness o hese six areas and de ermines
Evalua ion o choroidal hemodynamics
he ime required o reach prede ned levels o
Description brigh ness (10% and 63%). Nex , he six areas
are compared o each o her o de ermine heir
Indocyanine green (ICG) dye is used in
rela ive brigh ness. Rela ive comparisons are
conjunc ion wi h a higher-pene ra ing laser
possible because here is no need o com-
beam requency han ha o he f uorescein dye
pensa e or varia ions in op ics, lens opaci y,
o op imize visualiza ion o he choroidal vas-
or movemen as all da a are being collec ed
cula ure. ICG is used or evalua ion o choroi-
hrough an iden ical op ical sys em wi h all
dal vessels because i has an increased a ni y
six areas lmed simul aneously.
or plasma pro eins, reducing leakage o he dye
rom choroidal vessels in o surrounding issue.
 Scanning Laser Ophthalmoscope Angiography 141

FIGURE 9-5. Indocyanine green SLO angiography. Indocyanine green SLO angiography allows analysis o six
areas o the choroid: two areas near the optic disk, and our areas centered around the macula.

FIGURE 9-6. Area dilution analysis. Area dilution analysis measures the brightness o an area to determine
the time required to reach prede ned levels o brightness ( 10% and 63%) . It also allows relative brightness
comparisons to be made between the six areas.
 142 9 BLO O D FLO W IN GLAUCO MA
CO
O LO
OR D
DOO PPLER
P P LE
ER
IMAGIN
IM
M AG
GIN G
PURPOSE
Evalua ion o re robulbar vessels, speci -
cally he oph halmic, cen ral re inal, and pos-
erior ciliary ar eries.
DESCRIPTION
Color Doppler imaging (CDI) is an
ul rasound echnique ha combines wo-
dimensional s ruc ural ul rasound imaging
(B-scan) wi h superimposed color represen-
a ion o blood f ow veloci y, which is derived
rom he Doppler shi o sound waves as hey
are ref ec ed rom moving red blood cells.
Measuremen s are generally aken wi h he
pa ien in a reclined posi ion using a mul-
i unc ional probe (Fig. 9-7). T e veloci y
da a are graphed agains ime, and he peak
and rough are iden i ed as he peak sys olic
(PSV) and end dias olic (EDV) veloci ies
(Fig. 9-8). Pourcelo ’s resis ive index (RI) is
a measure o downs ream resis ance ha is
derived rom he PSV and EDV.
CDI has been shown o be reliable and
reproducible, and i provides a noninvasive
me hod o evalua e blood f ow. Addi ionally,
in vi ro and in vivo s udies have con rmed
he meaning ulness o CDI parame ers,
including RI. Despi e hese advan ages,
CDI su ers rom some limi a ions. For
example, because i canno evalua e vessel
diame er, CDI is unable o assess volume -
ric blood f ow. Addi ionally, here is no
universal me hodology or recording CDI
measuremen s.
BIBLIOGRAPH Y
Ehrlich R, Harris A, Siesky BA, e al. Repea abili y o
re robulbar blood f ow veloci y measured using color
Doppler imaging in he Indianapolis glaucoma pro-
gression s udy. J Glaucoma. 2011;20(9):540–547.
 Color Doppler Imaging 143

FIGURE 9-7. CDI. CDI is per ormed by placing a single, multi unction probe (usually 5 to 7 MHz) over the
closed eye.

A B
FIGURE 9-8. CDI. A. Speci c retrobulbar vessels can be chosen with the CDI. T ese include the ophthalmic,
central retinal, and posterior ciliary arteries. B. T e PSV and EDV are taken rom the peak and trough o the
velocity plot. Pourcelot’s RI can then be calculated rom these two values.
 144 9 BLO O D FLO W IN GLAUCO MA

(Fig. 9-9). T is me hodology yields several

ME
MEASUREMEN
E ASUU R EM
M ENN OF ou pu parame ers, including ocular pulse
O CULAR
C U LA
AR PULSA
P U LS
SA IO N ampli ude, IOP wave orms, and a calcula ed
value or POBF (Fig. 9-10).
PURPOSE T e choroidal circula ion, which rep-
resen s 80% o o al ocular blood f ow, is
Es ima ion o choroidal blood f ow hough o be he main componen o POBF.
However, al hough s udies have ound an
DESCRIPTION associa ion be ween POBF measuremen s
and ocular blood f ow, his assump ion has ye
Blood f ow o he eye varies during he o be con rmed. Addi ionally, con ounding
cardiac cycle. A large rush o blood en ers he ac ors, such as scleral rigidi y, may inf uence
ocular circula ion during sys ole and f ows in measuremen s.
a a reduced ra e during dias ole. Dynamic
measuremen o IOP has been u ilized o BIBLIOGRAPH Y
measure he pulsa ile componen o ocular
Zion IB, Harris A, Siesky B, e al. Pulsa ile ocular blood
blood f ow. A modi ed pneumo onome er f ow: Rela ionship wi h f ow veloci ies in vessels
or a dynamic con our onome er is used o supplying he re ina and choroid. Br J Ophthalmol.
assess pulsa ile ocular blood f ow (POBF) 2007;91:882–884.
 Measurement of Ocular Pulsation 145

A B
FIGURE 9-9. POBF. A. T e POBF device provides real-time measurement o IOP approximately 200 times
per second. B. T e POBF tonometer is placed on the cornea to record the amplitude o the IOP pulse wave.

FIGURE 9-10. POBF. T e IOP pulse wave is thought to correlate primarily with systolic choroidal blood ow.
 146 9 BLO O D FLO W IN GLAUCO MA
CON
CONFOCAL
N FO
O C AL
L SCANNING
SCA
AN NII NG
G Doppler-shi ed spec ra (which signi y he
LASER
LAS
SER DOPPLER
DO PPLLER
FLOWME
FLO
O WME RYRY
PURPOSE
o assess capillary per usion in he re ina,
op ic nerve head, and choroid
DESCRIPTION
Laser Doppler f owme ry (LDF) u ilizes
a laser Doppler device coupled wi h a modi-
ed undus camera and a compu er sys em o
evalua e re inal capillary blood f ow. Con ocal
scanning LDF is a me hod ha combines
LDF wi h a scanning laser omograph, which
enhances he abili y o he sys em o exclude
choroidal blood f ow when measuring re inal
capillaries.
T e Heidelberg re inal f owme er (HRF)
is a commonly u ilized con ocal scanning
LDF device (Fig. 9-11). T e HRF employs
a 790-nm laser o scan each pixel wi hin he
arge ed area o he re ina. T e Doppler shi
a each poin is hen calcula ed by a compu er
via a as Fourier rans orma ion. Using his
me hod, volume ric blood f ow can hen be
FIGURE 9-11. HRF. T e HRF uses the principles o retinal LDF.
calcula ed by using he requencies in he
veloci ies o blood cells) oge her wi h he
signal ampli ude a each requency (which
signi y he propor ion o he blood cells a
each veloci y) (Fig. 9-12).
Several me hods have been developed
o analyze HRF da a. Under he de aul
set ing, a 10 × 10 pixel area is used o
produce mean values o veloci y and vol-
ume; however, his me hod resul s in poor
reproducibili y because o varia ions in mea-
suremen s when he selec ed area is sligh ly
changed.
An al erna ive echnique developed by
Harris e al. uses a larger arge ed area and
analyzes each individual pixel (Fig. 9-13).
A his ogram o he blood f ow values is hen
crea ed o iden i y he dis ribu ion o blood
f ow wi hin he re ina, including per used and
avascular issue. A signi can limi a ion o
bo h LDF and con ocal scanning LDF is ha
volume ric blood f ow is measured in rela ive,
no absolu e, uni s.
BIBLIOGRAPH Y
Zion IB, Harris A, Moore D, e al. In erobserver repea -
abili y o Heidelberg re inal f owme ry using pixel-by-
pixel analysis. J Glaucoma. 2009;18:280–283.
 Confocal Scanning Laser Doppler Flowmetry 147

FIGURE 9-12. HRF. T e amplitudes o Doppler-shi ed requencies caused by moving blood cells are used to
create a ow map o the peripapillary retina and optic disk.

FIGURE 9-13. HRF. Pointwise analysis o the HRF ow maps provides a more robust interpretation o ow
map by providing a description o the varying degrees o per used and avascular tissues.
 148 9 BLO O D FLO W IN GLAUCO MA

requency a which oxygena ed hemoglobin

SPE
SPEC
E C RAL
R AL
L RE
E IINAL
N AL
L ref ec ion is maximized when compared wi h
OXIME
OX
XI ME
E RYY deoxygena ed hemoglobin ref ec ion. T e
op ical densi y, or absorbance o ligh , is hen
PURPOSE de ermined or he re inal vascula ure using
an algori hm ha ollows he pa h o ref ec ed
Evalua ion o oxygen ension in he re ina ligh along vessels. A ra io be ween he op ical
and op ic nerve head densi ies o wo o he images is used o de er-
mine he oxygen sa ura ion.
DESCRIPTION T is novel echnique can assis inves i-
ga ors in s udying me abolic changes ha
Spec ral re inal oxime ry (SRO) involves may be associa ed wi h glaucoma e iology.
he measuremen o he oxygen sa ura ion However, curren ly here is a lack o su -
o hemoglobin in re inal vessels (Fig. 9-14). cien ly valida ed da a, limi ing he use o
A s andard digi al undus pho ograph is re inal oxime ry a his ime.
per ormed, and our equivalen images are
recorded and l ered o isola e par icular re- BIBLIOGRAPH Y
quencies. T e requencies o in eres include Harris A, Dinn R, Kagemann L, e al. A review o me hods
he requency a which oxygena ed and deoxy- or human re inal oxime ry. Ophthalmic Surg Lasers
gena ed hemoglobin ref ec iden ically and he Imaging. 2003;34(2):152–164.

FIGURE 9-14. SRO. T e spectral retinal oximeter uses the spectrophotometric properties o oxygenated and
deoxygenated hemoglobin to determine the oxygen tension in the retina and optic nerve head. (Copyright
Glaucoma Research and Diagnostic Center.)
 Doppler Ocular Coherence Tomography 149

T ese images are used o derive he vessel’s

DO
O PPLER
PP
P LER R O CULAR
C U LA
AR cross-sec ional area, and a Doppler analysis
CO
O H ERENCE
E R EN
N CEE provides he veloci y o blood f ow in he
O MOO GRAPH
G R APHHY vessel. T e area and veloci y measuremen s
are combined o derive he blood f ow in ha
PURPOSE vessel. I he blood f ow in each o he major
vessels is analyzed, o al re inal blood f ow
o assess volume ric blood f ow in re inal can be de ermined. Similarly, his me hod can
vessels be u ilized o evalua e blood f ow in a single
quadran or hemisphere.
DESCRIPTION T e major advan ages o his me hod are
ha i measures re inal blood f ow in absolu e
T is me hodology couples a measuremen uni s and ha i is noninvasive. Limi a ions
o Doppler requency shi o ligh ref ec - include sensi ivi y o ana omic ar i ac s, such
ing rom moving red blood cells wi h ocular as eye movemen s, and ha i has no ye
coherence omography (OC ), an imaging been su cien ly valida ed.
modali y ha provides a high-resolu ion
cross-sec ional image o he re ina, o provide BIBLIOGRAPH Y
volume ric blood f ow measuremen in abso- Wang Y, Lu A, Gil-Flamer J, e al. Measuremen o o al
lu e uni s. blood f ow in he normal human re ina using Doppler
Fourier-domain op ical coherence omography. Br J
T e OC ob ains cross-sec ional images Ophthalmol. 2009;93:634–637.
o a given blood vessel in wo di eren areas.
 C H AP T ER

10
Introduction to Clinical Syndromes
Douglas J. Rhee

T he glaucoma syndromes are divided into

two main groups: primary and secondary.
T e primary glaucomas are those or which
the cause o increased resistance to outf ow
and elevated intraocular pressure is unknown.
T e secondary glaucomas are associated with
known ocular or systemic conditions respon-
sible or the elevated intraocular pressure and
resistance to outf ow.
Primary open-angle glaucoma is the most
common orm o glaucoma in the United
States. It constitutes about two-thirds o all
cases o glaucoma. T is particular disease
is probably the nal common pathway or a
variety o yet undistinguished separate patho-
physiologic processes. As our understanding
o the genetic and pathophysiologic compo-
nents continues to expand, I predict that we
will eventually distinguish several other condi-
tions with these characteristic optic nerve and
visual eld de ects.

150

T e chapters in this section include represen-
tative photographs and brief y describe the
major glaucoma syndromes:
Congenital glaucomas
Primary open-angle glaucoma
Secondary open-angle glaucoma
Inf ammatory glaucomas
Introduction to Clinical Syndromes 151
Lens-associated glaucomas
Uveitic glaucomas
Primary angle-closure glaucoma
Secondary angle-closure glaucoma
In addition, some o the delayed-onset compli-
cations rom glaucoma surgery are discussed.
 C H AP T ER

11
Developmen al Glaucomas
(Congeni al Glaucomas)
Oscar V. Beaujon-Balbi, Oscar Beaujon-Rubin, and Douglas J. Rhee

T he developmen al glaucomas are a group

o condi ions charac erized by a develop-
men al abnormali y o he aqueous ou f ow
sys em o he eye. T is group includes he
ollowing:
Congeni al glaucoma, in which he devel-
opmen al abnormali y o he an erior cham-
ber angle is no associa ed wi h o her ocular
or sys emic anomalies
Developmen al glaucomas wi h associ-
a ed anomalies, in which ocular or sys emic
anomalies are presen
Secondary glaucomas o childhood, in
which o her ocular pa hologies are he cause
o he impairmen o he aqueous ou f ow
Several di eren sys ems are used o classi y
developmen al glaucomas. T e mos com-
mon are he Sha er–Weiss and he Hoskin
ana omic classi ca ions. T e ormer divides
congeni al glaucoma in o hree major groups:
Primary congeni al glaucoma
Glaucoma associa ed wi h congeni al
anomalies
Secondary glaucomas o childhood
T e lat er de nes he ac ual developmen al
disorder clinically eviden a he ime o exam-
ina ion and also includes hree groups:
Isola ed rabeculodysgenesis wi h mal-
orma ion o he rabecular meshwork in he
absence o iris or corneal anomalies
Irido rabeculodysgenesis ha includes
angle and iris anomalies
Corneo rabeculodysgenesis, usually asso-
cia ed wi h iris anomalies
T e iden i ca ion o ana omic de ec s can be
use ul in de ermining he appropria e herapy
and prognos ic ac ors.

152

PRIMARY CONGENITAL
GLAUCOMA
P rimary congeni al glaucoma (PCG) is
he mos common orm o in an ile glau-
coma, represen ing 50%o all cases o congeni-
al glaucoma. I is charac erized by a rabecular
meshwork anomaly and is no associa ed wi h
o her ocular or sys emic diseases. Seven y- ve
percen o PCG cases occur bila erally.
Epidemiology
T e incidence is 1 in 5,000 o 10,000 live
bir hs. More han 80% o cases presen be ore
1 year o age: 40% a bir h, 70% be ween 1
and 6 mon hs, and 80% be ore 1 year.
T e disorder is more common in males
(70% males, 30% emales), and 90% o cases
are sporadic, wi hou a amily his ory.
Al hough an au osomal recessive model
wi h variable pene rance has been sugges ed,
i is hough ha mos o he cases are a resul
o mul i ac orial inheri ance wi h nongene ic
ac ors involved (e.g., environmen al ac ors).
History
Epiphora, pho ophobia, and blepharo-
spasm orm he classic riad.
Usually, children wi h congeni al glau-
coma pre er dimly li places and avoid
exposure o in ense ligh . T e caregiver may
describe an excessive amoun o earing.
In unila eral cases, he mo her may no e
an asymme ry be ween eyes, re erring o an
enlarged (a ec ed side) or decreased (normal
side) eye size (Fig. 11-1).
Clinical Examination
T e normal horizon al corneal diame er
in a ull- erm newborn is 10 o 10.5 mm. T is
increases o he adul diame er o approxi-
ma ely 11.5 o 12 mm by 2 years o age. A
diame er grea er han 12 mm in an in an is
highly sugges ive o congeni al glaucoma.
Primary Congenital Glaucoma 153
Measuremen s o he cornea are made in
he horizon al meridian wi h calipers. O her
ndings include corneal cloudiness, ears in
Desceme ’s membrane (Haab’s s riae), deep
an erior chamber, in raocular pressure grea er
han 21 mm Hg, iris s roma hypoplasia, iso-
la ed rabeculodysgenesis on gonioscopy, and
increased op ic nerve cupping. Haab’s s riae
may be single or mul iple and are charac eris-
ically orien ed horizon ally or concen rically
o he limbus (Fig. 11-2).
Op ic nerve evalua ion is a very impor-
an par o he glaucoma evalua ion.
Glaucoma ous disc changes occur more
rapidly in in an s and a lower pressures han
in older children or adul s. Cup- o-disc ra ios
grea er han 0.3 are rare in normal in an s and
mus be considered suspicious or glaucoma.
Asymme ry o op ic nerve cupping is also
sugges ive o glaucoma, par icularly di er-
ences o grea er han 0.2 be ween he wo
eyes. T e glaucoma ous cupping may be oval
in con gura ion bu is more o en round and
cen ral (Fig. 11-3). Reversal o op ic nerve
cupping has been observed a er normaliza-
ion o in raocular pressure.
Evalua ion o he an erior chamber angle
is essen ial or an accura e diagnosis and
rea men . T e developmen al anomalies
can presen in wo major orms: (1) f a iris
inser ion, in which he iris inser s direc ly
or an erior o he rabecular meshwork wi h
iris processes ha can overcome he scleral
spur (Fig. 11-4A) and (2) concave iris inser-
ion, in which he iris is observed behind
he rabecular meshwork bu is covered wi h
a dense abnormal issue (Fig. 11-4B). For
comparison, a gonioscopic pho o o a normal
an erior chamber angle rom an in an is also
presen ed (Fig. 11-5).
Eleva ion o he in raocular pressure
causes rapid enlargemen o he globe wi h
progressive enlargemen o he cornea in
children younger han 3 years o age. As he
cornea enlarges, s re ching leads o rup ures
 154 11 DEVELO PMENTAL GLAUCO MAS (CO NGENITAL GLAUCO MAS)
in Desceme ’s membrane, epi helial and s ro-
mal edema, and corneal clouding. T e iris is
s re ched so ha he s roma appears hinned.
T e scleral canal hrough which he op ic
nerve passes also enlarges wi h eleva ed in ra-
ocular pressure. T is resul s in rapid cupping
o he op ic nerve, which can quickly reverse
i he in raocular pressure is normalized.
T is drama ic reversal in cupping is no
seen in adul eyes and is probably rela ed
o he grea er elas ici y o he op ic nerve
head connec ive issue in he in an . I he
in raocular pressure is no con rolled, a
buph halmos can develop (Fig. 11-6; see
also Fig. 11-1).
Dif erential Diagnosis
O her causes o corneal changes include
megalocornea, me abolic diseases, corneal
dys rophies, obs e ric rauma, and kera i is.
Epiphora or pho ophobia may occur in
nasolacrimal duc obs ruc ion, dacryocys i is,
and iri is.
Op ic nerve anomalies simula ing a glau-
coma ous nerve include op ic pi s, colobo-
mas, and hypoplasia.
Management
T e rea men o congeni al glaucoma is sur-
gical. Medical rea men can be used or a lim-
i ed ime while he surgery is being scheduled.
Procedures ha involve rabecular inci-
sions are he choice in his condi ion.
Gonio omy requires a clear cornea or
visualiza ion o he angle.
rabeculec omy wi h an ex ernal
approach o Schlemm’s canal does no
require corneal ransparency.
T e gonio omy is per ormed using a
gonio ome and direc gonioscopy lens.
radi ionally, a Barkan’s goniolens is pre-
erred. An incision is made in he dense
abnormal issue a he rabecular meshwork
or an ex ension o 90 o 180 degrees using
he gonio ome hrough clear cornea (Figs.
11-7 and 11-8).
Al erna ively, a modi ed Swan–Jacob lens
can be used wi h a sharp blade, e.g., MVR
blade, Wheeler kni e, or even a 30-gauge nee-
dle. T e advan age o a f a blade is ha i will
crea e less dis or ion o he corneal wound
and allow he wound o be closed more easily
(Fig. 11-9).
For a pa ien wi h cloudy or opaci ed
corneas, rabeculec omy is indica ed. A scleral
f ap is made and Schlemm’s canal mus be
ound in order o per orm he procedure. T e
rabecular meshwork is broken using a ra-
beculo ome (Fig. 11-10) or by using a su ure
(usually propylene) hrough Schlemm’s canal
(Lynch procedure). An al erna ive o su ure
rabeculec omy is o use a ber op ic ca he er
(iCa h, iScience), which has he advan age o
an illumina ed ip ha iden i es he loca ion
o he ca he er (Fig. 11-11).
In cases, where Schlemm’s canal is no
ound, rabeculec omy is per ormed. Ano her
choice is he placemen o a valved or non-
valved glaucoma- l ering device.
 Primary Congenital Glaucoma 155

FIGURE 11-1. Unilateral primar y congential glaucoma. T is photo demonstrates a cloudy cornea and
buphthalmos.

FIGURE 11-2. Haab’s striae. Arrows indicate breaks in Descemet’s membrane.

 156 11 DEVELO PMENTAL GLAUCO MAS (CO NGENITAL GLAUCO MAS)

FIGURE 11-3. Round cupping. Round cupping in congenital glaucoma.

A B
FIGURE 11-4. Gonioscopy. A. Flat iris insertion. B. Concave iris insertion.
 Primary Congenital Glaucoma 157

FIGURE 11-5. Gonioscopy. Note the relative paucity o anterior chamber pigment o a normal anterior
chamber angle in an in ant.

FIGURE 11-6. Buphthalmos. An extreme example o buphthalmos with corneal and scleral thinning.
 158 11 DEVELO PMENTAL GLAUCO MAS (CO NGENITAL GLAUCO MAS)

FIGURE 11-7. Congenital glaucoma during goniotomy. Note the di erence in angle con guration between
the goniotomy-treated portion o trabecular meshwork ( white arrow) and the nontreated portion ( black arrow) .

FIGURE 11-8. Angle visualization. Visualization o the angle af er goniotomy.

 Primary Congenital Glaucoma 159

A B

C D
FIGURE 11-9. Goniotomy procedures. A and B. raditional goniotomy per ormed with a Barkan lens,
goniotome kni e, with xation o the superior and in erior rectus muscles using locking toothed orceps. T is
technique relies upon an assistant to xate the orceps and rotate the eye to allow the surgeon access to a wider
portion o the angle. C and D. Alternative method that does not require an assistant using a modi ed Swan lens
and goniotome kni e.
 160 11 DEVELO PMENTAL GLAUCO MAS (CO NGENITAL GLAUCO MAS)

A B

FIGURE 11-10. Mechanical trabeculectomy.

A. Partial thickness dissection over the limbus.
B. Sharp cut down to Schlemm’s canal. C. Harms
trabeculotome is cannulated through into Schlemm’s
canal, then gently passed into the anterior chamber
C to allow a communication between the anterior
chamber and collecting channels.
 Primary Congenital Glaucoma 161

FIGURE 11-11. Suture trabeculectomy with i rack. T e red illuminated tip helps identi y the location o the
catheter.
 162 11 DEVELO PMENTAL GLAUCO MAS (CO NGENITAL GLAUCO MAS)
GLAUCOMA ASSO CIATED
WITH CONGENITAL
ANOMALIES
ANIRIDIA
Aniridia is a bila eral congeni al anomaly
in which he iris is markedly underdeveloped,
bu here is generally a rudimen ary iris
s ump o variable ex en visible on examina-
ion o he angle (Fig. 11-12).
wo- hirds o cases are dominan ly rans-
mit ed wi h a high-degree pene rance. wen y
percen are associa ed wi h Wilms’ umor.
A dele ion o he shor arm o chromo-
some 11 has been associa ed wi h Wilms’
umor and sporadic aniridia.
Poor visual acui y is common because
o oveal and op ic nerve hypoplasia. O her
associa ed ocular condi ions include kera-
opa hy, ca arac (60% o 80%), and ec opia
len is (Fig. 11-13). Pho ophobia, nys agmus,
decreased vision, and s rabismus are common
mani es a ions in aniridia. Progressive corneal
FIGURE 11-12 Aniridia. Gonioscopic photograph showing an iris remnant with ciliary processes below.
opaci ca ion and pannus usually occur cir-
cum eren ially in he periphery.
Glaucoma associa ed wi h aniridia does
no usually develop un il la e childhood or
early adul hood. I may be a resul o rabecu-
lodysgenesis or o progressive closure
o he rabecular meshwork by he residual
iris s ump. I i develops during in ancy,
a gonio omy or rabeculec omy may be
indica ed.
I has been sugges ed ha early gonio omy
may preven he progressive adherence o
he residual peripheral iris o he rabecular
meshwork.
In older children, medical herapy o
con rol in raocular pressure should rs
be at emp ed. Any orm o surgery has he
risk o injuring he unpro ec ed lens and
zonules, and l ering procedures have an
increased risk o vi reous incarcera ion.
Cyclodes ruc ive procedures may be neces-
sary in cer ain pa ien s wi h uncon rolled
advanced glaucoma.
 Glaucoma Associated with Congenital Anomalies 163

FIGURE 11-13 Aniridia and cataract. Arrows indicate the remnant portion o the iris.
 164 11 DEVELO PMENTAL GLAUCO MAS (CO NGENITAL GLAUCO MAS)
AXENFELD’S ANOMALY
Axen eld’s anomaly is charac erized by
peripheral cornea, an erior chamber angle,
and iris anomalies. A prominen Schwalbe’s
line, re erred o as posterior embryotoxon, is a
peripheral cornea al era ion in hese pa ien s.
Iris s rands at aching o he pos erior embryo-
oxon and hypoplasia o he an erior iris
s roma may be presen .
T e disease is usually bila eral and has
an au osomal-dominan inheri ance (Fig.
11-14).
Axen eld’s syndrome includes glaucoma
and occurs in 50% o pa ien s wi h he anom-
aly. I glaucoma occurs in in ancy, gonio omy
or rabeculec omy is o en success ul. I glau-
coma occurs la er, medical herapy should be
ried ini ially, and l ering surgery should be
used i needed.
RIEGER’S ANOMALY
Rieger’s anomaly represen s a more
advanced degree o angle dysgenesis. Besides
he clinical aspec observed in Axen eld’s
anomaly, a marked iris hypoplasia is observed
wi h polycoria and corec opia.
I is usually bila eral and inheri ed in an
au osomal-dominan pat ern, al hough he
anomaly can be presen sporadically.
Glaucoma develops in more han hal o
cases, o en requiring surgery.
RIEGER’S SYNDROME
When he ndings o Rieger’s anomaly are
associa ed wi h sys emic mal orma ions, he
erm Rieger’s syndrome is pre erred.
T e mos commonly associa ed sys emic
anomalies are developmen al de ec s o he
ee h and acial bones. Den al abnormali ies
include a reduc ion o crown size (microdon-
ia), a decreased bu evenly spaced number o
ee h, and a ocal absence o ee h (commonly
he an erior maxillary primary and permanen
cen ral incisors; Figs. 11-15 and 11-16).
Because o he similari y o an erior cham-
ber angle abnormali ies in hese en i ies, i has
been proposed ha hey represen a spec rum
o developmen al disorders ha have been
named anterior chamber cleavage syndrome
and mesodermal dysgenesis of cornea and iris.
T ey are also re erred o as Axen eld–Rieger
syndrome.
PETER’S ANOMALY
Pe er’s anomaly represen s a major degree
o an erior chamber developmen al disorder.
A corneal opaci y associa ed wi h a pos erior
s romal de ec is presen , also known as Von
Hippel’s corneal ulcer. T e iris is adheren o
he cornea a he collaret e. T e lens can be
included on hese adhesions wi h an absen
corneal endo helium.
Pe er’s anomaly is bila eral and requen ly
associa ed wi h glaucoma and ca arac .
Corneal ransplan wi h ca arac removal
o improve visual acui y has a guarded
prognosis. In hese cases, a rabeculec omy
or glaucoma drainage implan devices are
required or glaucoma con rol (Fig. 11-17).
 Glaucoma Associated with Congenital Anomalies 165

FIGURE 11-14 Axen eld’s anomaly diagram.

A B

FIGURE 11-15 Rieger’s syndrome. A. Notice prominent anterior embryotoxon (white arrows) and iris
hypoplasia ( black arrow) . B. T e mother o the patient in A, showing prominent anterior embryotoxon,
corectopia, and polycoria.
 166 11 DEVELO PMENTAL GLAUCO MAS (CO NGENITAL GLAUCO MAS)

FIGURE 11-16 Rieger’s syndrome. Lef . Facial anomalies, maxillary hypoplasia. Right. Dental anomalies,
hypodontia, and anodontia. (Courtesy o Dr. Adael Soares, Escola Paulista de Medicina, UNIFESP, São Paulo,
Brazil.)

B
A
FIGURE 11-17 Peter’s anomaly. A. Diagram o anomaly. B. Note the corneal opacity ( leukoma) , along with
corneal pannus.

MARFAN SYNDROME
Mar an syndrome is charac erized by
musculoskele al abnormali ies, such as arach-
nodac yly, excessive heigh , long ex remi ies,
hyperex ensive join s, and scoliosis, cardio-
vascular disease, and ocular abnormali ies.
ransmission is au osomal dominan wi h
high pene rance, al hough approxima ely 15%
o cases are sporadic (Fig. 11-18).
Ocular ea ures include ec opia len is,
microphakia, megalocornea, myopia, kera-
oconus, iris hypoplasia, re inal de achmen ,
and glaucoma (Fig. 11-19). T e zonules
are o en at enua ed and broken, leading
o upward subluxa ion o he lens ( he lens
A excavatum. B. Arachnodactyly.
Mar an Syndrome 167
may also become disloca ed in o he pupil
or an erior chamber, leading o lens-induced
glaucoma).
Open-angle glaucoma may also develop,
requen ly in childhood or adolescence, and
is associa ed wi h congeni al abnormali ies
o he an erior chamber angle. Dense iris
processes bridge he angle recess, inser ing
an erior o he scleral spur. Iris issue sweep-
ing across he recess may have a concave
con gura ion.
Usually he glaucoma occurs in older
childhood, and he medical herapy should
rs be at emp ed.
FIGURE 11-18 Mar an syndrome. A. Note the tall
and thin body habitus; this patient also has pectus
 168 11 DEVELO PMENTAL GLAUCO MAS (CO NGENITAL GLAUCO MAS)

FIGURE 11-19 Mar an syndrome. Anterior ectopia lentis. In this eye, the crystalline lens has dislocated anteriorly.

MICROSPHEROPH AKIA
Microspherophakia may occur as an
isola ed disorder ha is inheri ed as an
au osomal-recessive or -dominan rai or i
may be associa ed wi h Weill–Marchesani
syndrome. T is syndrome is charac erized by
shor s a ure, brachydac yly, brachycephaly,
and microspherophakia.
FIGURE 11-20 Microspherophakia. T e small, round lens can be visualized within the aperture o the dilated
pupil.
Microspherophakia 169
T e lens is small and spherical and may
move an eriorly, resul ing in pupillary-block
glaucoma (Fig. 11-20).
Angle-closure glaucoma can be rea ed
using mydria ics, iridec omy, or lens
ex rac ion.
Glaucoma usually occurs in la e childhood
or early adul hood.
 170 11 DEVELO PMENTAL GLAUCO MAS (CO NGENITAL GLAUCO MAS)

STURGE–WEBER SYNDROME occurs in in ancy looks and behaves like glau-

( ENCEPH ALOTRIGEMINAL
ANGIOMATOSIS)
Pa ien s wi h S urge–Weber syndrome
have a acial hemangioma ollowing he dis-
ribu ion o he rigeminal nerve. T e acial
hemangioma is usually unila eral bu may be
bila eral. Conjunc ival, episcleral, and cho-
roidal hemangiomas are also common abnor-
mali ies. Di use uveal involvemen has been
ermed he “ oma o-ca sup” undus.
No clear heredi ary pat ern has been
es ablished.
Glaucoma more o en occurs when he
ipsila eral acial hemangioma involves he lids
and conjunc iva.
Glaucoma may occur in in ancy, la e child-
hood, or young adul hood. T e glaucoma ha
coma associa ed wi h isola ed rabeculodys-
genesis and responds well o gonio omy. T e
glaucoma ha appears la er in li e is probably
rela ed o eleva ed episcleral venous pressure
rom ar eriovenous s ulas.
In older children medical herapy should
be at emp ed rs . However, i his is no suc-
cess ul, rabeculec omy should be considered.
Fil ering surgery has an increased risk o
choroidal hemorrhage, resul ing in shallowing
or f at ening o he an erior chamber rela ed
o he diminu ion o he in raocular pres-
sure a he momen o surgery. T is probably
occurs when he in raocular pressure level
alls below ha o ar erial blood pressure and
resul s in e usion o choroidal f uid in o sur-
rounding issues (Figs. 11-21 and 11-22).

FIGURE 11-21 Sturge–Weber syndrome. Note unilateral rontal and maxillary distribution. (Courtesy o
Dr. Claudia Pabon Bejarano, Escola Paulista de Medicina, UNIFESP, São Paulo, Brazil.)
 Sturge–Weber Syndrome Encephalotrigeminal Angiomatosis 171

A B

FIGURE 11-22 Sturge–Weber syndrome. Choroid

hemangioma. (Courtesy o Dr. Dario Fuenmayor,
C
Caracas, Venezuela.)
 172 11 DEVELO PMENTAL GLAUCO MAS (CO NGENITAL GLAUCO MAS)
NEUROFIBROMATOSIS
( VON RECKLINGH AUSEN’S
DISEASE AND
BILATERAL ACOUSTIC
NEUROFIBROMATOSIS)
Neuro broma osis is an inheri ed disorder
o he neuroec odermal sys em ha resul s
in hamar omas o he skin, eyes, and nervous
sys em. T e syndrome primarily a ec s is-
sue derived rom he neural cres , par icularly
he sensi ive nerves, Schwann’s cells, and
melanocy es.
Neuro broma osis has wo orms: NF-1,
or classic Von Recklinghausen’s neuro -
broma osis, and NF-2, or bila eral acous ic
neuro broma osis.
NF-1 is he mos common orm and
includes involvemen o skin wi h ca é-au-
lai spo s and cu aneous neuro bromas,
iris hamar omas called Lisch nodules, and
op ic nerve gliomas (Fig. 11-23). NF-1
occurs in an es ima ed 0.05% o popula-
ion and has a prevalence o 1 in 30,000.
I is inheri ed in an au osomal-dominan
pat ern wi h comple e pene rance.
NF-2 is less common, wi h an es ima ed
prevalence o 1 in 50,000.
Cu aneous involvemen includes
ca é-au-lai spo s, which appear as hyper-
pigmen ed macules on any par o body
and end o increase in age, and mul iple
neuro ibromas, which are benign umors o
nerve connec ive issue and vary rom iny,
isola ed nodules o huge pedunculus so
issue masses.
Oph halmic involvemen includes he
ollowing:
Iris hamar omas, clinically observed as
bila eral, raised, smoo h-sur aced, dome-
shaped lesions
Plexi orm neuro bromas o he upper
lid, which appear clinically as an area o
hickening o he lid margin wi h p osis
and an S-shaped de ormi y
Re inal umors, mos commonly as ro-
cy ic hamar omas
Op ic nerve gliomas, which mani es
as unila eral decreased visual acui y or
s rabismus and have been observed in 25%
o cases
Ipsila eral glaucoma is also occasionally
seen and is usually associa ed wi h plexi orm
neuro broma o he upper lid.
 Neurof bromatosis Von Recklinghausen’s Disease And Bilateral Acoustic Neurof bromatosis 173

FIGURE 11-23 Neuro bromatosis. Note ca é-au-lait spots and plexi orm neuro broma o the upper lid.
(Courtesy o Dr. Claudia Pabon Bejarano, Escola Paulista de Medicina, UNIFESP, São Paulo, Brazil.)
 C H AP ER
12
Primary Open-angle Glaucoma
George L. Spaeth, Shelly R. Gupta, and Robert J. Goulet III
W orldwide, glaucoma, in i s many orms,
is he leading cause o irreversible
blindness. T e glaucomas are of en classi ed
in o he primary and he secondary glauco-
mas. Primary glaucoma mos of en a ec s
bo h eyes, and is no he resul o any recog-
nized rauma occurring during a person’s li e-
ime. T e secondary glaucomas, in con ras ,
charac eris ically (bu no always) a ec only
one eye, and are a consequence o a recog-
nizable cause, such as in ec ion, mechanical
injury, or neovasculariza ion. T ere is also a
“cause” or he primary glaucomas, which may
be accelera ed aging or an abnormal gene, bu
i is no known a he presen ime how such a
“cause” can be iden i ed.
T e glaucomas can also be divided on he
basis o he na ure o he an erior chamber
T he de ni ion o glaucoma has changed
angle. In angle-closure glaucoma, he pres-
sure becomes eleva ed as a consequence o
he resis ance o aqueous ou ow due o
con ac or adhesions be ween he iris and
he rabecular meshwork. In con ras , in he
open-angle glaucomas, aqueous humor has
clear access o he rabecular meshwork. T e
primary glaucomas are of en ur her charac-
erized according o he age o onse o he
174
condi ion. I is impor an o recall ha he “pri-
mary glaucomas” represen a he erogeneous
group, which may be as unique as a par icular
cons ella ion o genes, or as general as aging.
T ose occurring a or shor ly af er bir h are
ermed congenital, hose occurring af er in ancy
and prior o age 40 years are ermed juvenile
glaucomas, and hose rs becoming apparen
af er he age o 40 are ermed adul -onse open-
angle glaucoma. T e glaucomas occurring in
in an s represen a separa e group and are dis-
cussed elsewhere in his ex (see Chap er 11).
DEFINITION
drama ically since i was rs used in
ancien Greece; he word has come o mean
di eren hings o di eren people. T e de -
ni ion is s ill evolving and, as a consequence,
here can be un or una e and dis urbing con-
usions during communica ion. Figure 12-1
illus ra es a rough imeline o hese changes.
Un il he la e 19 h cen ury, he de ni ion
o glaucoma was based largely on he pres-
ence o symp oms, blindness, or pain. T e

developmen o s a is ical heory, he avail-
abili y o he onome er, and he concep o
disease as a devia ion rom normal, all led
o glaucoma being de ned solely in erms o
eleva ed in raocular pressure (IOP) above 21
mm Hg (i.e., grea er han wo s andard devia-
ions above he mean) or IOP above 24 mm
Hg (grea er han hree s andard devia ions
above he mean).
S udies conduc ed largely in he 1960s
showed ha only around 5% o individuals
wi h an IOP above 21 mm Hg ac ually devel-
oped op ic nerve damage and visual eld loss.
O her s udies showed ha around one- hird
o he pa ien s who had charac eris ic op ic
nerve and visual eld changes o glaucoma
had IOPs in he range o normal. T ese wo
observa ions orced a o al re hinking o he
de ni ion o glaucoma. Many au hors began
using he erms “low- ension glaucoma” or
“normal-pressure glaucoma” or “high- en-
sion glaucoma.” As more at en ion became
ocused on he idea o glaucoma as an “op ic
neuropa hy,” he idea ha glaucoma could be
rela ed solely o eleva ed pressure, as occurs
in he angle-closure glaucomas, became less
recognized. T is led o he s range si ua ion
o a person wi h an IOP o 80 mm Hg in
Defnition 175
exquisi e pain being considered o have “angle
closure,” bu no being considered o have
“angle-closure glaucoma.” Recen ly, some
au hors have subdivided glaucoma in o pres-
sure-dependen and pressure-independen
ypes. While i is cer ain ha here are ac ors
ha increase or decrease he suscep ibili y
o he ocular issues o damage by IOP, i is
no clear ha hese ac ors ever ac alone and
wi hou some con ribu ion rom he e ec s
o IOP, even when he IOP is in he normal or
subnormal range.
In Figure 12-1, glaucoma is de ned as he
process leading o charac eris ic progressive
ocular issue damage, a leas par ially caused
by IOP, regardless o he level o IOP. Findings
and symp oms o early or modera e glaucoma
are ound in almos all people, even hose who
do no have glaucoma. T ere is no hing diag-
nos ic, or example, abou a cup- o-disc ra io
o 0.5. While his could represen a signi can
glaucoma ous change, i may also occur in a
person wi hou glaucoma. Fur hermore, here
is no hing diagnos ic abou an IOP o 25 mm
Hg. T is is a common nding in people who
do no have glaucoma. T us, iden i ca ion o
charac eris ics ha occur only (or almos only)
in glaucoma is impor an (Fig. 12-2).
 176 12 PRIMARY O PEN-ANGLE GLAUCO MA

FIGURE 12-1 How he def ni ion o glaucoma has changed over he years.

FIGURE 12-2 Overlap o charac eris ics ha occur in pa ien s wi h and wi hou glaucoma.

EPIDEMIOLOGY
G laucoma a ec s individuals o all ages, all
races, and in all geographic areas. I is no
surprising ha es ima es o he prevalence o
glaucoma vary widely. T is varia ion is rela ed
o he di ering de ni ions o glaucoma, di -
ering me hods o examina ion, and di eren
expressions o he cons ella ion o loosely
rela ed condi ions called primary open-angle
glaucoma.
Congeni al glaucoma represen s a dis inc
en i y ha is ex remely rare. Mos juvenile
glaucoma is gene ically de ermined, and while
much more common han he congeni al
ypes o open-angle glaucoma, i is s ill rela-
ively uncommon.
Epidemiology 177
T e majori y o pa ien s wi h glaucoma are
older han 60 years o age and he prevalence
o glaucoma increases wi h age.
I is di cul o generalize he amoun o blind-
ness caused by glaucoma. Again, glaucoma
is a variable condi ion, wi h variable de ni-
ions. However, he incidence clearly increases
markedly wi h age and especially in A rican
Americans. T e prevalence o glaucoma in
A rican Americans over he age o 80 may be
more han 20%.
Worldwide, he incidence o glaucoma is es i-
ma ed a around 2.5 million per year. T e prev-
alence o blindness due o primary open-angle
glaucoma is probably around 3 million. In he
Uni ed S a es around 100,000 individuals are
blind in bo h eyes as a resul o glaucoma.
 178 12 PRIMARY O PEN-ANGLE GLAUCO MA

PATHOPHYSIOLOGY he IOP-rela ed damage. Abnormal au oregu-

la ion o blood ow o he op ic nerve has also

T he hallmark o glaucoma is ocular issue

damage, especially o he op ic nerve and
re inal nerve ber layer (RNFL).
Figure 12-3 illus ra es possible ways in which
he issue damage can develop. An ini ia ing
ac or is IOP wi hin he normal or he eleva ed
range, causing mechanical de orma ion and
leading o subsequen damage. Also illus ra ed
are addi ional ac ors rela ed o he s ruc ural
de orma ion caused by IOP.
oxic agen s or au oimmune mechanisms may
cause damage and even ual dea h o he re i-
nal ganglion cells, leading o loss o issue and
s ruc ural damage, which i sel may acili a e
been pos ula ed o play a role in glaucoma ous
op ic neuropa hy.
T e nal common pa hway in all he primary
open-angle glaucomas is he dea h, some-
imes by necrosis, bu usually by apop osis,
o he re inal ganglion cells. T is may lead o
ur her damage in he re ina, op ic nerve, and
brain. Feedback loops make his oversimpli-
ed schema ar more complex. For example,
“s ruc ural change” i sel predisposes o cell
damage. Some o he ac ors ha may be
involved in his cascade o even s are shown in
able 12 1.

Vasospasm Hypotension
Anemia

Elevated IOP
Ischemia

Autoimmunity,
Mechanical excitotoxicity,
Cell
deformation growth factor
damage
deficiency

Decreased Cell
intracranial death
pressure

Structural
change

Functional
loss

FIGURE 12-3 Pa hogenesis o ocular issue damage in glaucoma.

 Pathophysiology 179

ABLE 12-1. Some Fac ors Involved in he Developmen o issue Damage in Glaucoma
Mechanical injury
Stretching o lamina cribrosa, blood vessels, corneal endothelial cells, etc
Abnormal glial, neural, or connective tissue
Metabolic deprivation
Direct compression o neurons, connective tissue, and vasculature by intraocular pressure
Lack o neurotrophins
Secondary to mechanical blockade o axons
Genetically determined
Defcient nerve growth actors
Ischemia and hypoxia
Abnormal autoregulation o retinal and choroidal vessels
Decreased per usion
Acute/ chronic
Primary/ secondary
Abnormal oxygen trans er
Autoimmune mechanisms
De ective protective measures
Defcient or inhibited nitric oxide synthase
Abnormal heat shock protein
Toxicity to retinal ganglion cells and other tissues
Glutamate
Genetic predisposition
Abnormal optic nerve structure
Large laminar pores
Large scleral canal
Abnormal connective tissue
Abnormal vasculature
Abnormal trabecular meshwork
Decreased permeability o extracellular matrix
Abnormal endothelial cells
Abnormal molecular biology
 180 12 PRIMARY O PEN-ANGLE GLAUCO MA

HISTORY ABLE 12-2. Risk Fac ors or he

Developmen o Glaucoma

A horough his ory is he single mos

impor an par o he examina ion o a
pa ien wi h primary open-angle glaucoma,
Genetic make-up
Positive amily history o glaucomatous visual loss
Identifcation o glaucoma related gene
even hough he disease is rela ively ree o
symp oms in i s early s ages. T e his ory is Intraocular pressure considerations
he par o he examina ion ha develops he Likelihood o eventually
rela ionship be ween he pa ien and he phy- mm Hg developing glaucoma
sician ha is essen ial o success ul diagnosis >21 5%
and managemen o glaucoma. Addi ionally, >24 10%
a me iculous his ory will requen ly uncover
>27 50%
symp oms o he disease, even in rela ively
early or modera e s ages. For example, pa ien s >39 90%
may no e di cul y seeing in he dark, mild eye Age
ache, or a sense ha vision simply is no as Age in years* Prevalence o glaucoma
good as i was in he pas . Fur hermore, ak- Rare
<40
ing a his ory leads o elici ing he risk ac ors
or glaucoma and, even more impor an ly, he 40—60 1%
risk ac ors or becoming blind rom glaucoma 60—80 2%
( ables 12 2 and 12 3). >80 4%

Average-pressure glaucoma has been associ- Vascular actors

a ed wi h various vascular risk ac ors (i.e., Migraine
migraine, low blood pressure, sleep apnea). Vasospastic disease
Par icular at en ion should be placed on hese Raynaud’s disease
issues in pa ien s suspec ed o having his ype
Hypotension
o glaucoma.
Hypertension
T e essen ial ques ion in his ory- aking is Myopia
“How are you?” T e physician mus s ress ha
Obesity
he mos impor an par o he ques ion is he
word “you.” Glaucoma is a righ ening condi- *T ese fgures are or Europeans and Asians; the prevalence in
ion ha requen ly decreases he pa ien ’s A ricans is approximately our times higher.
quali y o li e simply as a resul o he pa ien
knowing he or she has glaucoma. T ere is a
long his ory o associa ing glaucoma wi h IOP.
T e physician a every appropria e oppor u-
ni y mus indica e o he pa ien ha bo h he
pa ien ’s and physician’s concern rela es o he
pa ien ’s heal h, no primarily o he pa ien ’s
IOP. An evalua ion o heal h requires aking a
compe en , compassiona e his ory.
 History 181

ABLE 12-3. Risk Fac ors or Becoming

Blind rom Glaucoma
Disease process capable o causing blindness*
Lack o access to care
Geographic
Economic
Care unavailable
Lack o sel care competence
Intellectual limitation
Emotional limitation
Socioeconomic deprivation
*T ere is a great variation o disease severity with primary
open angle glaucoma; some patients do not get worse even in
the absence o any treatment, whereas others go rapidly blind
even with treatment.
 182 12 PRIMARY O PEN-ANGLE GLAUCO MA
CLINICAL EXAMINATION
T he clinical examina ion o he pa ien
suspec ed o having primary open-angle
glaucoma di ers only rom he s andard
examina ion in emphasis. An essen ial par
o he examina ion is a me iculous search or
he presence or absence o a rela ive a eren
pupillary de ec (APD). An APD can be pres-
en prior o de ec able eld loss. Addi ionally,
he presence o APD indica es ha here is
de ni e op ic nerve damage, which riggers
a necessary search or he cause o ha dam-
age. Checking he pa ien or an APD is a par
o every comple e examina ion o he pa ien
wi h glaucoma.
External and Biomicroscopic Examination
Biomicroscopic examina ions o he pa ien
wi h glaucoma di er only rom he s andard
biomicroscopic examina ion in ha he physi-
cian searches or he opical side e ec s o he
medica ions ha he pa ien may be using (Fig.
12-4) and ndings ha may be associa ed wi h
glaucoma, such as a Krukenberg spindle.
Gonioscopy
Gonioscopy is an essen ial par o he evalua-
ion o a pa ien wi h glaucoma. T e examiner
needs o search or he signs o pigmen disper-
sion syndrome, ex olia ion syndrome, and angle
recession. Gonioscopy needs o be repea ed a
abou yearly in ervals, because an erior cham-
ber angles ha are ini ially deep may become
increasingly narrow wi h age, leading even ually
o chronic or, in rare cases, acu e angle closure.
Because mio ics can cause marked shallow-
ing o he an erior chamber angle, gonioscopy
should be per ormed af er any mio ic is s ar ed
or af er a concen ra ion o a mio ic is changed.
T e Spae h gonioscopic grading sys em pro-
vides a rapid, quan i a ive, and clinically use ul
me hod o describing and recording he an e-
rior chamber angle (see Chap er 3).
Posterior Pole
Primary open-angle glaucoma is primarily a dis-
ease o he op ic disc. Consequen ly, knowledge-
able, ex ensive evalua ion o he op ic nerve is a
manda ory par o he evalua ion o he pa ien
suspec ed o having glaucoma and o he con-
inuing evalua ion o he pa ien . In he diagno-
sis o primary open-angle glaucoma, evalua ion
o he op ic nerve is he single mos impor an
aspec o he assessmen . In he management o
glaucoma, he na ure o he op ic disc is he sec-
ond mos impor an aspec o he examina ion,
a me iculous his ory being he rs .
T e op ic disc is bes examined wi h he pupil
in a dila ed s a e. Af er dila ion he op ic nerve
is examined s ereoscopically a he biomicro-
scope using a s rong plus lens such as a 60- or
66-diop er lens. T is is bes done wi h he
beam narrowed o a hin sli and he magni ca-
ion on high (1.6 or 16×) using a Haag-S rei
900 series sli -lamp biomicroscope. T e exam-
iner gains an idea o he disc opography by his
me hod. Also, he size o he disc is measured.
o measure he ver ical heigh o he disc he
beam is widened so ha he horizon al ex en
o he beam is he same wid h as he wid h o
he op ic nerve (Fig. 12-5). T e beam is hen
narrowed ver ically un il he ver ical diame er
o he op ic disc exac ly ma ches he ver ical
ex en o he beam. T e ver ical diame er o
he disc is de ermined by reading he re icule
on he sli lamp and applying he appropria e
correc ion ac or. T ough hese ac ors vary
sligh ly wi h he Volk and he Nikon lenses, a
rough approxima ion is ha or he 60-diop er
lens one mul iplies he reading on he re icule
by 0.9; or he 66-diop er lens, no correc ion
ac or is needed, and or he 90-diop er lens,
he measuremen on he re icule is mul i-
plied by 1.3. T e normal disc has a diame er
be ween 1.5 and 1.9 mm ver iclly.
T e nex s ep employs he direc oph hal-
moscope. T e beam o he oph halmoscope
should be narrowed so ha i projec s a beam

on he re ina approxima ely 1.3 mm in diam-
e er. T is is he size o he middle-sized beam
on some Welch-Allyn oph halmoscopes and
o he smalles beam on some o her Welch-
Allyn oph halmoscopes. T e examiner should
learn he size o he beam or he oph halmo-
scope he or she is using. T is can be readily
done by projec ing he beam on he re ina
nex o he op ic nerve, no ing he heigh o
ha beam rela ive o he op ic nerve, and hen
using he s rong plus lens o ge an exac mea-
suremen o he heigh o he projec ed beam.
Once his has been de ermined, he size o he
op ic nerve can be de ermined rela ively accu-
ra ely wi h he direc oph halmoscope i sel .
In eyes wi h more han 5 diop ers o hyperopia
or 5 diop ers o myopia, he disc size will be
abnormally large or abnormally small, respec-
ively, when viewed wi h he s rong plus lens
due o magni ca ion or mini ca ion.
T e op ic nerve can be bes examined using a
direc oph halmoscope wi h bo h he pa ien
and he examiner in he sea ed posi ion. T e
examiner’s head mus be in a posi ion o
avoid obs ruc ing he pa ien ’s gaze wi h he
o her eye, because ha o her eye mus xa e
rmly o allow care ul evalua ion o he eye
being examined. T e examiner direc s pri-
mary at en ion o he 6 and 12 o’clock posi-
ions o he nerve: Wha is he rim wid h? Is
an acquired pi or a disc hemorrhage presen ?
Is here peripapillary a rophy? Are he vessels
displaced, ben , engorged, narrowed, or “bayo-
ne ed”? T e examiner also es ima es he wid h
o he neurore inal rim a he 1, 3, 5, 7, 9, and
11 o’clock posi ions. T is is done in erms o a
rim- o-disc ra io, ha is, he rela ive wid h o
he rim in comparison o he diame er o he
op ic nerve in ha axis. T us, he maximum
rim- o-disc ra io is 0.5.
In Figure 12-6, he rim- o-disc ra io a
1 o’clock is 0.2; a 3 o’clock, 0.15; a 5 o’clock,
0.0; a 7 o’clock, 0.25; a 9 o’clock, 0.20, and
a 11 o’clock, 0.25. Figure 12-7A illus ra es
Clinical Examination 183
a disc wi h a narrow rim ha respec s Jonas’s
ISN rule, which s a es ha he rim should
be wides in eriorly, nex wides superiorly,
nex wides nasally, and narrowes temporally.
Despi e he large cup, here is no visual eld
loss (Fig. 12-7B). Figure 12-8 shows a disc
wi h a rela ively small “cup- o-disc ra io,” bu a
rim- o-disc ra io o 0 a he in erior pole. Cup-
o-disc ra ios are misleading and should no be
used. Figure 12-9 shows wo discs o di eren
sizes. Figure 12-9A is a small disc wi h a disc
diame er o approxima ely 1.2 mm. Figure
12-9B is a large disc wi h a disc diame er o
approxima ely 2.2 mm. T e examiner may be
misled by he rela ive size o he cup in hese
wo discs and incorrec ly conclude ha he
disc in Figure 12-9B is less heal hy han ha
in Figure 12-9A. In ac uali y, i is he o her
way around.
T e rim area is rela ively cons an in all
heal hy discs. T us, in large discs, he rim area
is spread over a much grea er area (recall ha
area involves he square o he radius). T e
consequence o his is ha he normal rim o
he large, healthy disc is narrower han he nor-
mal rim o he small, healthy disc. T e rim area
in Figure 12-9B is ac ually grea er han he
rim area in Figure 12-9A.
T e rela ive heal h o he op ic nerve can be
es ima ed by s aging he disc according o he
sys em illus ra ed in Figure 12-10.
In younger pa ien s, or pa ien s whose op ic
nerves are in he rela ively early s ages o
glaucoma ous damage, speci cally s ages 0
o 3 (see glaucoma graph), evalua ion o he
nerve ber layer can be help ul. T e examiner
ocuses me iculously on he re inal sur ace,
pre erably wi h a red- ree ligh in he direc
oph halmoscope, and looks or lines ha
would ollow he course o he nerve ber lay-
ers. A rough can indica e he presence o such
a de ec illus ra ed in Figure 12-11. In mos
cases, however, he opography o he op ic
 184 12 PRIMARY O PEN-ANGLE GLAUCO MA
nerve provides more valuable clues han does
he na ure o he nerve ber layer.
T e op ic nerves o he wo eyes should be
symme ric. Where asymme ry is presen , one
o he nerves is almos always abnormal, unless
he op ic nerves are o di eren sizes, as indi-
ca ed in Figure 12-9. Figure 12-12 shows he
righ and lef eyes o a pa ien wi h unila eral
op ic nerve damage resul ing rom glaucoma.
T e examiner should search ou he pres-
ence o an acquired pi o he op ic nerve.
T ese localized de ec s immedia ely adjacen
o he ou er edge o he rim, jus emporal o
A B
C resul o la anopros . (Pho o cour esy o Chris opher
he in erior or superior pole o he disc, are
pa hognomonic or glaucoma ous damage.
T e observer also speci cally looks or he
presence o a disc hemorrhage on he re ina
crossing he rim. Such hemorrhages may be
signs ha he glaucoma ous process is ou o
con rol. However, hese hemorrhages may
have o her e iologies, such as an icoagula-
ion or pos erior vi reous de achmen . T ey
are no reliable signs o poor glaucoma con-
rol. Disc hemorrhages are seen more of en
in pa ien s wi h average-pressure glaucoma.
Fur her in orma ion abou he op ic disc is
ound in Chap er 5.
FIGURE 12-4 A. Ex ernal exam showing ery hema
and edema rom an allergic reac ion o imolol.
B. Con ac derma i is involving he periorbi al
area o he le eye. T ere is also some conjunc ival
in ec ion. C. Corneal epi helial pseudodendri es as a
Rapuano, MD, Wills Eye Hospi al, Philadelphia, PA.)
 Clinical Examination 185

1. Size

2. Read reticule
3. Use correction factor
FIGURE 12-5 Me hod o measuring ver ical diame er
o he disc.

FIGURE 12-6 Op ic nerve pho ograph o a le eye showing an acquired pi a approxima ely 5 o’clock.
 186 12 PRIMARY O PEN-ANGLE GLAUCO MA

C
FIGURE 12-7 A. A disc wi h a narrow rim ha respec s Jonas’s ISN rule. B. Corresponding Goldmann visual
f eld showing no abnormali y. C. Corresponding Humphrey visual f eld showing no abnormali y.
 Clinical Examination 187

A B

FIGURE 12-9 wo discs o dif erent sizes. A. Small disc wi h a disc diame er o approxima ely 1.2 mm.
B. Large disc wi h a disc diame er o approxima ely 2.2.

FIGURE 12-8 Small cup to disc ratio. A disc wi h a rela ively small cup- o-disc ra io, bu a rim- o-disc ra io o
0 a he in erior pole.
 188 12 PRIMARY O PEN-ANGLE GLAUCO MA

Na rrowe s t width of rim (rim/dis c ra tio) Exa mple s

DDLS For S ma ll Dis c For Ave ra ge For La rge Dis c DDLS 1.25 mm optic 1.75 mm optic 2.25 mm optic
S ta ge <1.50 mm S ize Dis c >2.00 mm S ta ge ne rve ne rve ne rve
1.50-2.00 mm

1 .5 or more .4 or more .3 or more 0a

2 .4 to .49 .3 to .39 .2 to .29 0b

3 .3 to .39 .2 to .29 .1 to .19 1

4 .2 to .29 .1 to .19 less than .1 2

0 for less than

5 .1 to .19 less than .1 3
45°

0 for less than 0 for 46° to

6 less than .1 4
45° 90°

0 for 46° to 0 for 91° to

7 0 for less than 45º 5
90° 180°

0 for 91° to 0 for 181° to

8 0 for 46º to 90º 6
180° 270°

0 for 181° to 0 for more

9 0 for 91º to 180º 7a
270° than 270°

0 for more than 0 for more

10 7b
180º than 270°

FIGURE 12-10 T e DDLS. T e Disc Damage Likelihood Scale (DDLS) is a way o describe quan i a ively and
simply he changes ha occur in he Op ic Nerve Head (ONH) ( he disc) . I is used o quan i y he heal h o he
op ic disc, specif cally as i rela es o glaucoma.
T e DDLS is based on wo charac eris ics o he disc: (1) he wid h o he neurore inal rim and (2) he size o
he op ic disc. T e DDLS scale goes rom 1 o 10, 1 being he mos normal and 10 he mos pa hologic. T e wid h
o he neurore inal rim is described in erms o he rim- o-disc ra io. T us, he wides possible neurore inal rim
would be a rim- o-disc ra io o .5. T e narrowes would be .0.
Firs , one measures he size o he op ic disc and classif es he disc as small, average, large, or very large. Small
is less han 1.5 mm in heigh , average be ween 1.5 and 2.0 mm in heigh , large be ween 2 and 3 mm in heigh ,
and very large grea er han 3 mm. T e size is easily measured wi h he sli lamp or, bet er, by using he beam o
an oph halmoscope. Nex , one looks or where he neurore inal rim is he narrowes . (Please no e: “ hin” is he
wrong word, as hin re ers o he hickness o he issue, no o i s wid h.) T e narrowes rim would be .0 and he
wides rim possible would be .5. When he wid h o he rim is be ween .4 and .5 rim- o-disc ra io, hen i is s age
1; be ween .3 and .4, i is s age 2; be ween .2 and .3, i is s age 3; be ween .1 and .2, i is s age 4; and less han .1
bu s ill presen , i is s age 5—all in average-sized discs. Five is he area o indecision. A value o 5 can occasionally
be normal, bu usually i is pa hological and associa ed wi h visual f eld loss. T e DDLS also depends on disc size,
so he wid h o he rim mus be correc ed or disc size. In a small disc, one uni should be added o he DDLS. In
a large disc, one uni should be sub rac ed. In a very large disc, wo uni s should be sub rac ed. T us, an average-
sized disc wi h a rim- o-disc ra io o .25 would be a DDLS o 3; a small disc wi h a rim- o-disc ra io o .25 would
be a DDLS o 4; a large-sized disc wi h he same rim- o-disc ra io o .25 would be a DDLS o 2; and in a very large
disc wi h he same rim- o-disc ra io would be a DDLS o 1.
Some pa ien s wi h glaucoma lose an area o he neurore inal rim comple ely. When his happens, one hen
uses he circum eren ial amoun o rim loss o de ermine he DDLS score. I he amoun o rim loss is less han
45 degrees, hen i is a DDLS o 6; be ween 90 and 180 degrees, a DDLS o 7; and be ween 90 and 180 degrees, a
DDLS o 8. I he amoun o rim loss is grea er han 180 degrees, bu less han 270 degrees, hen i is a DDLS o 9,
and i here is vir ually no rim le , hen i is a DDLS o 10. Again, all o hese numbers re er o he average-sized
disc. Consider a disc wi h a no ch in which here is no rim or 30 degrees. In an average-sized disc, i would be a
DDLS o 6, and in a small disc, a DDLS o 7. In a large disc, i would be a DDLS o 5, and in a very large disc, i
would be a DDLS o 4. Discs wi h DDLS o 6 or more are never normal.
 Clinical Examination 189

FIGURE 12-11 A disc wi h an in ero emporal no ch and a nerve f ber layer de ec in ero emporally.

A B
FIGURE 12-12 Unilateral optic nerve damage rom glaucoma. Righ and le eyes o a pa ien wi h unila eral
op ic nerve damage caused by glaucoma. T ere is a loss o in erior rim issue in he righ eye.
 190 12 PRIMARY O PEN-ANGLE GLAUCO MA
SPECIAL TESTS
O p ic nerve examina ion is supplemen ed
by visual eld evalua ion. Visual eld
de ci s, ideally, should correla e wi h ana-
omical changes. Monocular, au oma ed s a ic
perime ry has become he me hod o choice
used in diagnosis and moni oring o glauco-
ma ous change. T e di eren pla orms avail-
able include he Humphrey visual eld and he
Oc opus visual eld. T e pla orm, program,
and set ings should remain consis en wi h
subsequen elds when assessing or progres-
sion. Progression is di cul o es ablish. T is
is especially rue once here are advanced
visual eld changes. When his si ua ion exis s,
unc ion is mos accura ely evalua ed wi h he
pa ien ’s subjec ive symp oms. T e Es erman
eld is a binocular program ha is use ul in
evalua ing he level o overall unc ional loss
caused by glaucoma. De ailed discussion o
visual elds can be ound in Chap er 7.
T ere is deba e in he li era ure abou he
pat ern o visual eld de ec s observed in
average-pressure glaucoma. We, and o hers,
have ound visual eld de ec s o be more
dense and closer o cen er in average-pressure
glaucoma as compared o primary open-angle
glaucoma a higher pressures. O her groups
have observed no di erence be ween he wo
groups.
Imaging modali ies o he op ic nerve head
(ONH) and RNFL at emp o provide objec-
ive and accura e quan i a ive da a. I is hoped
ha in he u ure hese echnologies can be
used o de ec ganglion cell loss a he earli-
es s age o he glaucoma process. Al hough
sof ware con inues o evolve, none o he
available modali ies has been shown o be
unequivocally superior in de ec ing progres-
sion over ime. Each echnology comes wi h
s reng hs, limi a ions, and nuances ha need
o be ully unders ood in order o in erpre
he da a each provides. As wi h visual elds,
ONH/ RNFL imaging is used in conjunc ion
wi h op ic nerve examina ion. De ailed dis-
cussion o imaging echnology can be ound
in Chap er 19.
 reatment 191

TREATMENT requires hough ul balance o (1) he risks

o pain or unc ional loss in he ace o no

A s previously s a ed, glaucoma is a pro-

cess ha resul s in charac eris ic changes
o he op ic disc. In primary open-angle
glaucoma, his process is usually slow. T e
course o average-pressure glaucoma seems
o be more variable and, possibly, less linear.
I has been shown ha 40% o he ganglion
cell axons composing he op ic nerve can be
los be ore unc ional de ci s are de ec ed on
visual eld es s. Op imally, one would like o
in ervene in he process o glaucoma when
accelera ed ganglion cell loss is con rmed bu
be ore unc ional loss is no ed. Un or una ely,
none o he curren in erven ions available
come wi hou a leas some risk o accelera ed
vision loss or o her side e ec s. Appropria e
managemen o primary open-angle glaucoma
in erven ion, (2) he po en ial bene o an
in erven ion (in erms o re arda ion or s a-
biliza ion o de eriora ion o visual unc ion
or ac ual improvemen ), and (3) he po en-
ial risks in roduced by he in erven ion i sel
( ables 12 4 and 12 5).
T e “process” o glaucoma is no visible. T e
process becomes “visible” only hrough he
e ec s o ha process. T ere ore, i is neces-
sary o rely on he examina ion and ancillary
es ing o es ablish progression over ime. T e
goal o managing a pa ien wi h primary open-
angle glaucoma is o main ain or enhance he
pa ien ’s heal h. Bo h he physician and he
pa ien wish o ensure ha he pa ien does
no develop unc ional loss prior o his or her
dea h. Es ima ing he need o s ar rea men

ABLE 12-4. Risks and Benef s o rea men

Risks Attendant to No Risks Attendant
Intervention to Intervention Benefits of Intervention
Pain Local side e ects Improvement o ability to do visually
Visual loss Pain related activities
Minimal Redness Stabilization o the ability to do
Moderate Cataract visually related activities
otal In ection Retardation o the rate o
Visual loss Bleeding deterioration o ability to do visually
Minimal Allergy related activities
Moderate Abnormal ashes
otal Increased pigmentation
Other
Systemic side e ects
Fatigue
Malaise
Cardiovascular changes
Neurologic changes
Psychological changes
Pulmonary changes etc.
Expense
Inconvenience
Embarrassment
Decreased quality o li e
 192 12 PRIMARY O PEN-ANGLE GLAUCO MA
ABLE 12-5. Risk o Losing Func ion I
No In erven ion
Low with:
Healthy optic nerve
Negative amilyhistoryo visual loss due to glaucoma
Good sel care skills
Good access to good care
Estimated years remaining less than 10 years
Intraocular pressure below 15 mm Hg
No ex oliation or pigment dispersion syndrome
changes
Normal cardiovascular status
Moderate with a situation between “low”
and “high”
High with:
Optic nerve already damaged by glaucoma
Positive amily history o visual loss due to glaucoma
or presence o recognized “gene” or glaucoma
Poor sel care skills
Poor access to good care
Estimated years remaining over 15 years
Intraocular pressure over 30 mm Hg
Ex oliation syndrome
Poor cardiovascular status
or he need o change he vigor o rea men
requires ha he physician have a good idea
o he likelihood ha he pa ien ’s glaucoma
will ul ima ely cause unc ional problems.
o make his de ermina ion appropria ely,
he physician mus consider our issues: (1)
he s age o he glaucoma, (2) he ra e o
change o he glaucoma, (3) he dura ion ha
he glaucoma will con inue o exis , and
(4) socioeconomic mat ers. T e use o he
“Glaucoma Graph” can be o grea help in his
regard (Fig. 12-13). S age o he glaucoma
is de ermined by u ilizing he Disc Damage
Likelihood Scale (DDLS) (Fig. 12-10). Ra e
o change is de ermined by serial evalua ions
o he his ory and op ic nerve. T e dura ion
he glaucoma will con inue o cause damage
is, in mos cases, de ermined by a reasonable
es ima e o he pa ien ’s remaining years o li e.
IOP reduc ion decreases he ra e o disease
progression in glaucoma pa ien s. Lowering
IOP is he only rea men proven o be bene -
cial or pa ien wi h glaucoma. In he Uni ed
S a es, a usual prac ice pat ern o lower IOP is
o s ar wi h he use o medica ions. I medi-
ca ions ail, laser rabeculoplas y is usually
advised in appropria e pa ien s. Surgery is yp-
ically reserved or pa ien s who do no respond
o o her measures ( ables 12 6 and 12 7).
wo impor an poin s should be no ed abou
his algori hm. Firs , here is con roversy
amongs oph halmologis s abou whe her use
o medica ions is appropria e as rs -line her-
apy. Medica ions require he pa ien o adhere
o an in rusive daily schedule. As he number o
medica ions prescribed o a pa ien increases,
he likelihood o he pa ien main aining such
a schedule decreases. Addi ionally, medica-
ions are wrough wi h local and sys emic
side e ec s ha can be debili a ing or even li e
hrea ening o pa ien s. T ere are some physi-
cians who believe laser rabeculoplas y is more
appropria e as an ini ial in erven ion because
i avoids hese concerns. T e con roversy
ABLE 12-6. Expec ed Benef o rea -
men Rela ed o Amoun o Lowering o
In raocular Pressure*
Expected beneft great i intraocular pressure lowering
greater than 30%
Expected beneft possible to probable i intraocular
pressure lowering is 15%–30%
No beneft expected i intraocular pressure lowering
is less than 15%
*In some cases stabilization o intraocular pressure appears to
be benefcial in itsel .

ABLE 12-7. Rela ive A ec o Various
rea men s on In raocular Pressure and on
Developmen o Side E ec s
Usual decrease in intraocular pressure:
In response to • 15%(range 0%–50%)
medications
In response to argon • 20%(range 0%–50%)
laser trabeculoplasty
In response to f ltering • 40%(range 0%–80%)
surgery
Likelihood o side ef ects as a result o
treatment
From medications • 30%
From argon laser Almost no lasting side
trabeculoplasty e ects
From selective laser Rare, but some
trabeculoplasty permanent and disabling
From incisional • 60%*
surgery
*T e lower the fnal intraocular pressure the greater the
likelihood o side e ects rom the surgery; the rate varies
with the type o surgery, severity o condition, and skill and
judgment o the surgeon.
exis s because laser rabeculoplas y is no
wi hou i s own risks. Also, here is a percep-
ion in he general popula ion ha laser rea -
men is more invasive han use o medica ions.
Please see he separa e chap er on his subjec .
T e second impor an poin o be made
abou he rea men algori hm rela es o sur-
gery done o lower IOP. T ere are a varie y o
ways one may accomplish his goal, bu , he
gold s andard con inues o be rabeculec omy
(guarded l ra ion procedure). Even hough
he echnique has improved, his surgery s ill
has a signi can risk o complica ions ha
can lead o decrease in vision, loss o vision,
or loss o he eye. O her procedures include
ube shun s (glaucoma drainage devices) and
endocyclopho ocoagula ion. T e ormer o
reatment 193
hese is well es ablished wi h advan ages and
disadvan ages o i s own. T e lat er has he
disadvan age o lowering pressure by reduc-
ing aqueous in ow. Newer procedures have
been developed in an at emp o achieve he
IOP lowering observed wi h rabeculec omy
while avoiding he complica ions associa ed
wi h his surgery. None o he newer proce-
dures (inaccura ely re erred o as “minimally
invasive”) have been proven o sa is y hese
quali ca ions. Fur her s udies may provide
evidence o suppor adop ion o one or more
o hese echniques. T e con inued pursui o
new surgical op ions highligh s he need or
improvemen upon wha is curren ly available
or he care o medically re rac ive pa ien s.
T e amoun ha he IOP should be lowered
in order o preven de eriora ion, s abilize he
condi ion, or resul in improvemen varies
rom individual o individual, bu guidelines
have been developed. A arge pressure is an
IOP level believed likely o be low enough o
preven ur her damage. One me hod o arriv-
ing a a arge pressure is shown in Figure
12-14. I is impor an o remember, however,
ha he arge IOP is only a rela ive, en a ive
guide o rea men . T e only valid me hod o
es ablishing he s a e o con rol in a pa ien
wi h primary open-angle glaucoma is by
de ermining s abili y or ins abili y o he op ic
nerve or visual eld, or bo h. T us, i he op ic
nerve and visual eld are s able despi e an IOP
higher han he calcula ed arge pressure, i is
no wise o at emp o lower he pressure more
vigorously in order o achieve he arge IOP.
Conversely, i he arge pressure is achieved,
bu he op ic nerve or visual eld con inues
o de eriora e, hen he arge pressure is oo
high, here is ano her cause or he con inu-
ing de eriora ion o her han glaucoma, or he
neurons are so badly damaged ha de eriora-
ion will progress no mat er wha level o IOP
is achieved.
 194 12 PRIMARY O PEN-ANGLE GLAUCO MA

Stage
Disc 1
Damage
Likelihood Scale 2
3
Not definitely
damaged 4
5
Asymptomatic
glaucoma 6
damage
7
8
Glaucomatous
disease/ 9
disability
10
Birth Death
FIGURE 12-13 Glaucoma graph and explanation. T e glaucoma graph is a way o de ermining and
unders anding he clinical course o glaucoma in an individual pa ien .
Green Zone: When a person has a Disc Damage Likelihood Scale (DDLS) o 2, 3, or 4, one canno be sure ha
op ic nerve damage is no presen , even hough one knows ha visual f eld loss is no presen . I is possible ha
a an earlier da e he pa ien had a smaller DDLS, in which case, he presen larger DDLS would represen a
de eriora ion. I his were he case, he pa ien s ill would no have visual f eld loss and he need or rea men
would s ill be de ermined by he our ac ors ha always de ermine i rea men is necessary: he amoun o
damage ha is presen , he ra e o change, he dura ion ha he change will con inue, and socioeconomic
considera ions. Valid serial measuremen s allow es ablishing a rend, such as a ra e o de eriora ion o f eld or
disc. I he ra e o change is su cien ly rapid ha he person would ge in o he red zone prior o dea h, hen
rea men is clearly necessary. On he o her hand, i he ra e o change is so slow ha he person will probably no
ge in o he red zone prior o dea h, hen rea men is no likely jus if ed.
Yellow Zone: When a pa ien is in he yellow zone ( wi h a DDLS o 5 o 7) , he op ic nerve is def ni ely damaged,
bu he person is asymp oma ic. Even hough asymp oma ic, i is cer ain ha he eye is no normal. Nobody
s ar s wi h DDLS scores in hose ranges. T e person’s op ic nerve mus have become worse. In such a si ua ion i
is likely ha he pa ien will need rea men , hough his is no always he case. For example, a person could have
developed damage in he pas which hen became s abilized. Or, a person’s an icipa ed-number-o -years- o-live
could be so shor ha even wi hou rea men he/ she would no move rom he yellow o he red zone. Such
individuals would no need rea men .
Red Zone: When a person is already in he red zone, ha is, he person has a decreased quali y o li e or impaired
abili y o per orm he ac ivi ies o daily living (wi h a DDLS o 8, 9, or 10), hey already have a disabili y.
Consequen ly, he goal is preven ing any worsening o he disabili y, because any increase in damage makes he
pa ien symp oma ically worse. T ere ore, remaining years o li e is no longer a considera ion in pa ien s who
already have disabili y. In such a si ua ion, he only reason or no rea ing he pa ien is i he disabili y is o ally
s able wi hou rea men .
 reatment 195

IOP* - [IOP*/100 x IOP*] – D – E = Targ e t IOP

Whe re : IOP* = Intrao c ular pre s s ure kno wn to be as s o c iate d with pro g re s s ive dis c
o r e ld damag e o r maximum IOP
D = 0 if Dis c Damag e Like liho o d S c ale is le s s than 5
D = 1 if Dis c Damag e Like liho o d S c ale = 6-8
D = 2 if Dis c Damag e Like liho o d S c ale is g re ate r than 8
E = 0 if e s timate d ye ars re maining is le s s than 10 ye ars
E = 1 if e s timate d ye ars re maining = 11-20 ye ars
E = 2 if e s timate d ye ars re maining is g re ate r than 20 ye ars

FIGURE 12-14 A Me hod o Es ima ing arge In raocular Pressure.

 C H AP T ER

13
Secondary Open-angle Glaucoma
Jonathan S. Myers

PIG
PIGMEN
PI
IGGM
ME
MEEN
N DISPERSIO
DII SP E
D ERR SI
SIOON de ec s. T e libera ed pigmen may hen be
SYNN DRO
DR
ROO ME
ME deposi ed hroughou he an erior segmen .
Obs ruc ion o he rabecular meshwork

P igmen dispersion syndrome (PDS) is a

condi ion in which pigmen is dislodged
rom he pigmen ed epi helium on he pos e-
rior sur ace o he iris, and is hen deposi ed
on various s ruc ures hroughou he an erior
segmen . Obs ruc ion o he rabecular mesh-
work by pigmen , and subsequen damage o
he meshwork, can lead o eleva ed in raocu-
lar pressure (IOP) and secondary open-angle
glaucoma.
Epidemiology
PDS occurs mos requen ly in young
(aged 20 o 45 years), myopic, Caucasian
men.
Approxima ely one- hird o PDS pa ien s
go on o develop pigmen ary glaucoma.
Pathophysiology
Curren ly, i is believed ha con ac
be ween he iris pigmen epi helium and
he lens zonular f bers leads o release o he
pigmen in o he an erior chamber and he
charac eris ic peripheral iris ransillumina ion
by he pigmen and subsequen damage may
reduce aqueous ou ow, increase IOP, and
lead o op ic nerve damage i uncon rolled.
History
Pa ien s o en rela e a his ory o myopia
and a amily his ory o glaucoma.
Jarring exercise, s renuous physical ac iv-
i y, or rarely dila ion may lead o drama ically
increased pigmen dispersion, a so-called
pigmen s orm, leading o sudden eleva ions
o IOP. Pa ien s may hen experience blurred
vision and headaches.
Clinical Examination
Sli lamp (Figs. 13-1 to 13-7):
Charac eris ic f ndings include Krukenberg’s
spindle, a ver ical deposi ion o pigmen on
he corneal endo helium, pigmen deposi ion
on he an erior sur ace o he iris, peripheral
iris ransillumina ion de ec s (bes seen on re -
roillumina ion wi h a small beam hrough he
pupil), and pigmen deposi ion on he zonular
f ber at achmen s near he equa or o he lens.

196
 Pigment Dispersion Syndrome 197

Gonioscopy: Pa ien s ypically have back-
ward bowing o he peripheral iris, increasing
lens–iris con ac . T e angle is very widely
open, wi h modera e o heavy pigmen a ion,
which is rela ively homogeneously spread
over he en ire circum erence o he angle.
Pos erior pole: Charac eris ic glaucoma-
ous op ic a rophy is seen wi h prolonged
eleva ion o IOP or in ermit en pressure
spikes. Myopic pa ien s, and possibly espe-
cially hose wi h PDS, are prone o peripheral
re inal ears, necessi a ing close examina ion.
Treatment
T e goal o herapy is o con rol IOP in
pa ien s wi h signif can ly eleva ed pressure or
glaucoma ous nerve changes, usually hrough
aqueous suppressan s.
Mio ics reduce pigmen shedding and
reduce IOP bu are o en poorly olera ed in
his young popula ion and may increase he
risk o re inal de achmen while making mon-
i oring o he re ina periphery more di cul .
Laser peripheral irido omy also may
reduce pigmen shedding, because i allows
he pos eriorly bowed iris o move an eriorly
as any buil -up uid pressure in he an erior
chamber is hen normalized wi h he pos e-
rior chamber (relie o so-called reverse pupil-
lary block). T is may help preven glaucoma
in individuals a higher risk bu have no ye
developed uncon rolled pressure.
Argon laser rabeculoplas y and f l ering
surgery are also e ec ive in individuals who
are uncon rolled medically.
BIBLIOGRAPH Y
Campbell DG. Pigmen ary dispersion and glaucoma: A
new heory. Arch Ophthalmol. 1997;97:1667.
Gandolf SA, Vecchi M. E ec o a YAG laser irido omy on
in raocular pressure in pigmen dispersion syndrome.
Ophthalmology. 1996;103:1693–1695.

FIGURE 13-1. Krukenberg’s spindle. A ver ical endo helial deposi ion o pigmen charac eris ic o PDS. May
slowly resolve when pigmen shedding s ops, bu may persis or many years or orever. Pat ern o deposi ion is
hough o be rela ed o convec ion curren s o aqueous wi hin he eye.
 198 13 SECO NDARY O PEN-ANGLE GLAUCO MA

FIGURE 13-2. Transillumination defects in PDS. Marked peripheral and mid peripheral ransillumina ion
de ec s in PDS. Many pa ien s may presen wi h only several mild radial spoke like de ec s.

FIGURE 13-3. PDS, dense pigmentation, and Krukenberg’s spindle. Krukenberg’s spindle ( oreground), heavily
pigmen ed deep angle ( background). Charac eris ic homogeneous dense pigmen a ion o rabecular meshwork.

FIGURE 13-4. PDS, pigment deposition. Pa hology specimen showing pigmen deposi ion on rabecular
meshwork and an erior o meshwork.
 Pigment Dispersion Syndrome 199

FIGURE 13-5. PDS, pigment deposition. Pa hology specimen showing pigmen deposi ion wi hin beams o
rabecular meshwork.

FIGURE 13-6. PDS, Zentmeyer ’s line. Deposi ion o pigmen near equa or o lens, a inser ion o lens zonular
f bers. Variously re erred o as Zen meyer’s line or Scheie’s s ripe.

A B
FIGURE 13-7. PDS, bowing of peripheral iris. A. UBM o pa ien wi h PDS, showing backward bowing
peripheral iris in con ac wi h lens sur ace. B. UBM o he same pa ien , ollowing irido omy, showing an erior
relaxa ion o iris wi h reduced con ac wi h lens.
 200 13 SECO NDARY O PEN-ANGLE GLAUCO MA
EXFO
E XFO
O LIA
L IA
A IO
ION S
SYN
YN
N DRO
O ME
E he causes o XFS, bu links o he LOXL1
E x olia ion syndrome (XFS) is a sys emic
condi ion ha can lead o secondary
open-angle glaucoma. T e charac eris ic aky
whi e ma erial, seen hroughou he an erior
segmen , can obs ruc he rabecular mesh-
work and has also been isola ed in issues
hroughou he body.
Epidemiology
XFS ranges in prevalence rom near
0 in Eskimos o near 30% in people in
Scandinavian coun ries. I s incidence
increases wi h age and ime, as does binocular
versus monocular involvemen .
XFS-associa ed glaucoma may
accoun or varying propor ions o pa ien s
wi h glaucoma, rom very small o he
majori y, depending on he popula ion
s udied.
Al hough pa ien s wi h XFS are a an
increased risk or he developmen o glau-
coma (es ima ed in he Blue Moun ains Eye
S udy a f ve old grea er), he majori y o
hese pa ien s do no develop glaucoma.
Pathophysiology
T e exac na ure o he ex olia ion
ma erial is no well unders ood, bu he
ma erial has been isola ed rom he iris, lens,
ciliary body, rabecular meshwork, corneal
endo helium, and endo helial cells in blood
vessels hroughou he eye and orbi , as well
as he skin, myocardium, lung, liver, gall-
bladder, kidney, and cerebral meninges. T e
ma erial leads o blockage o he rabecular
meshwork and hereby a secondary open-
angle glaucoma. I also leads o iris peripapil-
lary ischemia and pos erior synechiae. T is
resul s in pigmen release, increasing he
burden on he rabecular meshwork, and
increased pupillary block, predisposing o
angle closure.
Recen research has no ully uncovered
gene and solar exposure may be ac ors.
History
Al hough pa ien s rarely have symp om-
a ic eleva ions o IOP, mos pa ien s have no
con ribu ory his ory.
Some pa ien s repor a amily his ory, bu
no clear heredi ary pat ern is known.
A his ory o complica ed ca arac surgery
is po en ially sugges ive.
Clinical Examination
Sli lamp (Figs. 13-8 to 13-12): T e hall-
mark o XFS is he aky whi e ma erial seen
mos o en a he edge o he pupil and in
concen ric rings on he an erior sur ace o he
lens capsule a er dila ion. T is ma erial may
also be seen deposi ed on he iris, angle s ruc-
ures, endo helium, lens implan , and vi reous
ace in aphakic pa ien s.
Peripapillary ransillumina ion de ec s
and a rophy o he pigmen ed ru are o en
seen. Peripapillary pigmen deposi ion is
also requen .
A ec ed eyes are o en more mio ic and
dila e poorly secondary o synechiae and
iris ischemia.
Pigmen libera ion wi h dila ion may
cause signif can pressure spikes. Ca arac
orma ion is more requen .
Gonioscopy: T e an erior chamber
angle is o en more narrow in XFS, especially
in eriorly rela ive o superiorly. Acu e
angle-closure glaucoma is a risk, and here-
ore con inued moni oring o he angle is
necessary.
T ere is irregular pigmen a ion o he
meshwork, wi h large, dark pigmen par i-
cles. T e deposi ion o pigmen an erior o
Schwalbe’s line resul s in he charac eris ic,
wavy Sampaolesi’s line.

Pos erior pole: Charac eris ic glaucoma-
ous op ic a rophy is seen wi h prolonged ele-
va ion o IOP or in ermit en pressure spikes.
Treatment
XFS-rela ed glaucoma o en leads
o higher pressures wi h grea er diurnal
uc ua ion.
opical medica ions are appropria e bu
have been repor ed o be less e ec ive.
Argon laser rabeculoplas y is e ec ive,
al hough here are repor s o increased pos -
opera ive eleva ions in IOP. Lower energies
are indica ed o reduce he risk o pressure
spikes, given he heavily pigmen ed rabecular
meshwork.
FIGURE 13-8. XFS. Ex olia ion ma erial on an erior lens capsule wi h clear zone in he region be ween
undila ed pupil zone and more peripheral lens. Presumably, he movemen o he iris clears ex olia ive ma erial
rom his area.
Ex oliation Syndrome 201
T e resul s o f l ra ion surgery are similar
o hose seen in primary open-angle glaucoma.
Ca arac surgery should be per ormed
wi h ex ra cau ion, given he known ragil-
i y o he capsule and zonular f bers in hese
pa ien s.
BIBLIOGRAPH Y
Mi chell P, Wang JJ, Hourihan F. T e rela ionship be ween
glaucoma and pseudoex olia ion: T e Blue Moun ains
Eye S udy. Arch Ophthalmol. 1999;117:1319–1324.
Ri ch R, Schlo zer-Schrehard U. Ex olia ion syndrome.
Surv Ophthalmol. 2001;45:265–315.
T orlei sson G, Magnusson KP, Sulem P, e al. Common
sequence varian s in he LOXL1 gene con er suscep i-
bili y o ex olia ion glaucoma. Science. 2007;317(5843):
1397–1400.
 202 13 SECO NDARY O PEN-ANGLE GLAUCO MA

FIGURE 13-9. XFS, exfoliation material. Ex olia ion ma erial on lens sur ace. No e ypical scrolled edges.

A B

FIGURE 13-10. Comparison of XFS and normal

eyes. Sli lamp pho os o a ec ed (A) and clinically
una ec ed (B) eyes. In A, here is a rophy o he
pigmen ed ru s, seen in XFS, whereas i is preserved
in B. C. Peripapillary ransillumina ion corresponding
C o iris pigmen epi helial a rophy in A.
 Ex oliation Syndrome 203

FIGURE 13-11. XFS, angle structures. Heavy, dark, irregular pigmen a ion o angle s ruc ures in XFS.

FIGURE 13-12. XFS, dislocated lens. Spon aneously disloca ed lens in a pa ien wi h XFS highligh s ragili y
o zonular suppor .
 204 13 SECO NDARY O PEN-ANGLE GLAUCO MA
S ERO
E R O IID-RESPO
D -R
R ES
SP O N SIVE
E work–inducible glucocor icoid response)
GLAUCO
GLA
AU C O MA A gene has been shown o be upregula ed in
S econdary open-angle glaucoma may resul
rom nearly any rou e o s eroid adminis-
ra ion. Eleva ions in IOP may be severe and
prolonged.
Epidemiology
T e incidence o s eroid-induced glau-
coma in he general popula ion is unknown.
Signif can eleva ions in IOP in response o
opical s eroids have been repor ed in 50% o
over 90% o glaucoma pa ien s and 5% o 10%
o pa ien s wi h normal pressure.
T e incidence o he s eroid response is
rela ed o he ype, dose, and rou e o s eroid
adminis ra ion.
Eleva ed IOP has been observed wi h
opical, in raocular, periocular, inhaled,
oral, in ravenous, and derma ologic admin-
is ra ions o s eroids, as well as wi h endog-
enous eleva ions o s eroids in Cushing’s
syndrome.
S eroid-induced pressure eleva ion is
no uncommon ollowing in ravi real injec-
ion o s eroids, or inser ion o depo s e-
roid devices in he pos erior segmen .
Following in ravi real injec ion, approx-
ima ely 50% experience an eleva ion o
IOP, bu a low percen age require surgical
in erven ion.
Pathophysiology
Increased glycosaminoglycans in he
rabecular meshwork in response o s eroids
impede aqueous ou ow and lead o eleva ed
IOPs. S eroids may reduce he membrane
permeabili y o he rabecular meshwork, as
well as reduce local phagocy ic ac ivi y by
cells and he breakdown o ex racellular and
in racellular s ruc ural pro eins, ur her con-
ribu ing o reduced meshwork permeabili y.
T e myocilin/ IGR ( rabecular mesh-
rabecular meshwork endo helial cells in
response o s eroid applica ion.
History
S eroid use o any ype is a crucial
aspec o he his ory. Prior use o s eroids
in he dis an pas wi h subsequen normal-
iza ion o IOP may presen as an apparen
normal- ension glaucoma (Figs. 13-13 to
13-15).
A his ory o as hma, skin disorders, aller-
gies, au oimmune disorders, or he like may
hus sugges possible pas or curren s eroid
use.
Occasionally, pa ien s no e changes in
vision rela ed o advanced visual f eld loss.
Table 13-1 gives a clinical example.
Clinical Examination
Sli lamp: Usually unremarkable. Even
in cases wi h ex reme eleva ions o IOP, he
chronici y usually preven s corneal edema.
Gonioscopy: Usually unremarkable.
Pos erior pole: ypical glaucoma ous
op ic nerve changes are no ed i eleva ion o
IOP is su cien ly high and prolonged.
Special es s: Discon inua ion o he
s eroids, i possible, may lead o a s eady
reduc ion o IOP. T e ime course is variable
and may be prolonged in cases o prolonged
s eroid use. In cases in which here is concern
regarding hal ing an ocular s eroid (e.g., a cor-
neal gra a high risk o rejec ion), con rala -
eral s eroid challenge may demons ra e IOP
eleva ion and conf rm he diagnosis.
Treatment
Discon inua ion o he s eroids, i possible,
or excision o depo s eroids may yield com-
ple e resolu ion.
 Steroid responsive Glaucoma 205

TABLE 13-1 Clinical Example: S eroid induced Glaucoma Following

Subconjunc ival Depo S eroid Injec ion
Postop Day IOP (mm Hg) Course and Medications
Surgery #1: Vitrectomy/ membranectomy with subconjunctival depot steroid
1 25 Prednisolone, hyoscine, erythromycin
6 45 Add timolol, iopidine, acetazolamide
16 20 Stop acetazolamide
30 29 Add dorzolamide, taper prednisolone
48 19 Take of prednisolone
72 27 Continue timolol, apraclonidine, dorzolamide
118 44 Add latanoprost; consult glaucoma
154 31 Arrange steroid depot excision
Surgery #2: Excise depot steroid
1 32 Add timolol, dorzolamide
4 28 Continue same
23 24 Continue same
38 14 Stop dorzolamide
Note: Patient later discontinued timolol; IOP has been 10 to 14 mm Hg since discontinuation o drug.
IOP = intraocular pressure.

A B

FIGURE 13-13. Steroid responsive glaucoma.

(A) Visual f eld de ec seen in a 28 year old
in ernal medicine residen sel medica ing a opic
blepharoconjunc ivi is wi h opical s eroids. B and
C. IOPs were grea er han 40 mm Hg, wi h advanced
op ic nerve cupping and associa ed visual f eld loss.
C T e pa ien la er underwen a rabeculec omy.
 206 13 SECO NDARY O PEN-ANGLE GLAUCO MA
FIGURE 13-14. Steroid-responsive glaucoma. Excised s eroid depo 5 mon hs ollowing vi rec omy or Eales’
disease on a Weck cell sponge.
I opical s eroids are used, weaker or less
pressure–inducing s eroids may help (e.g.,
lo eprednol, rimexolone, uorome holone).
Pa ien s wi h signif can uvei is presen a
special challenge because hey may require
s eroids o con rol he uvei is. Addi ionally,
he uvei is may i sel lead o various orms o
glaucoma, or may mask glaucoma wi h aque-
ous hyposecre ion.
opical an iglaucoma medica ions o all
ypes are o en help ul or s eroid-induced
eleva ions o IOP.
In general, laser rabeculoplas y is less
e ec ive or hese pa ien s han or hose
wi h o her ypes o glaucomas, bu has been
repor ed o be e ec ive in selec ed cases.
Fil ering surgery has similar resul s o
hose in primary open-angle glaucoma.
BIBLIOGRAPH Y
Johnson DH, Bradley JM, Acot S. T e e ec o dexa-
me hasone on glycosaminoglycans o human ra-
becular meshwork in per usion organ cul ure. Invest
Ophthalmol Vis Sci. 1990;31:2568–2571.
Johnson D, Got anka J, Flugel C, e al. Ul ras ruc ural
changes in he rabecular meshwork o human
eyes rea ed wi h cor icos eroids. Arch Ophthalmol.
1997;115:375–383.
Jones R, Rhee DJ. Cor icos eroid-induced ocular hyper-
ension and glaucoma: A brie review and upda e o he
li era ure. Curr Opin Ophthalmol. 2006;17:163–167.
Mi chell P, Cumming RG, Mackey DA. Inhaled cor-
icos eroids, amily his ory, and risk o glaucoma.
Ophthalmology. 1999;106:2301–2306.
Oh DJ, Mar in JL, Williams AJ, e al. Analysis o expres-
sion o ma rix me allopro einases and issue inhibi-
ors o me allopro einases in human ciliary body
ollowing la anopros . Invest Ophthalmol Vis Sci. 2006;
47:953–963.
 Steroid responsive Glaucoma 207

FIGURE 13-15. Steroid responsive glaucoma. Fundus pho o aken immedia ely ollowing an in ravi real
injec ion o riamcinolone. Whi e colored crys als seen beginning o disperse in he vi reous o a pa ien wi h
diabe ic macular edema.
 C H AP T ER

14
Uvei ic Glaucomas
Nicole Benitah, Ronald Buggage, and George N. Papaliodis

T he developmen o increased in raocular

pressure and glaucoma in pa ien s wi h
uvei is is a mul i ac orial process ha can be
increased in raocular pressure and glaucoma
mos commonly occur.
In common usage, he erm uveitis is used o
viewed as a complica ion o he in raocular
encompass all causes o in raocular inf am-
inf amma ion. Bo h direc ly and by he induc-
ma ion. Uvei is can cause acu e, ransien , or
ion o s ruc ural changes, inf amma ion in
chronic eleva ions in he in raocular pressure.
he eye can al er he aqueous humor dynam-
T e erms inf ammatory glaucoma and uveitic
ics resul ing in high, normal, or low in raocu-
glaucoma are commonly used o re er o any
lar pressures. T e glaucoma ous op ic nerve
pa ien wi h uvei is and increased in raocu-
damage and visual eld de ec s ha occur in
lar pressure. In pa ien s wi h uvei is and no
pa ien s wi h uvei is are primarily an e ec
demons rable “glaucoma ous” op ic nerve
o he uncon rolled in raocular pressure.
damage or “glaucoma ous” visual eld de ec s,
T e primary rea men objec ive or pa ien s
i is more correc , however, o use erms such
wi h uvei is-induced ocular hyper ension
as uveitis-induced ocular hypertension, ocular
and glaucoma is he con rol o he inf amma-
hypertension secondary to uveitis, or secondary
ory disease and he preven ion o permanen
ocular hypertension o re er o hose wi h uve-
s ruc ural al era ions o aqueous ou f ow by
i is and only eleva ed in raocular pressure.
he use o appropria e an i-inf amma ory her-
Wi h resolu ion or appropria e managemen
apy. Managemen o he in raocular pressure,
o he in raocular inf amma ion, he increased
ei her medically or surgically, is a secondary
in raocular pressure need no progress o sec-
objec ive.
ondary glaucoma.
T is chap er de nes and discusses he pa ho-
T e erms inf ammatory glaucoma, uveitic glau-
physiologic mechanisms, diagnosis, and
coma, or glaucoma secondary to uveitis should
rea men s ra egies or pa ien s wi h uvei is
be reserved or hose pa ien s wi h uvei is,
and eleva ed in raocular pressure or second-
increased in raocular pressure, and “glauco-
ary glaucoma. T e chap er concludes wi h a
ma ous” op ic nerve or “glaucoma ous” visual
descrip ion o speci c uvei ic en i ies in which

208

eld de ec s. In mos cases o uvei ic glau-
coma, he glaucoma ous op ic nerve injury is
primarily a sequela o he eleva ed in raocular
pressure; here ore, he diagnosis o uvei ic
glaucoma should be ques ioned in a pa ien
wi h no known his ory o increased in ra-
ocular pressure. Addi ionally, he diagnosis o
glaucoma secondary o uvei is should be ques-
ioned in any pa ien wi h visual eld de ec s
a ypical or glaucoma and a normal-appearing
op ic nerve head. T is is because many ypes
o uvei is, par icularly hose a ec ing he pos-
erior segmen , are charac erized by choriore -
inal and op ic nerve lesions ha can produce
visual eld de ec s ha do no represen glau-
coma. T is dis inc ion is impor an , because
he visual eld de ec s in pa ien s wi h ac ive
inf amma ory disease may resolve or improve
wi h appropria e herapy, whereas rue glau-
coma ous visual eld de ec s in pa ien s wi h
uvei is are irreversible.
EPIDEMIOLOGY
U vei is may accoun or 5% o 10% o
legal blindness in he Uni ed S a es and
Europe, and up o 25% o blindness in he
developing world.1 T e prevalence o uvei is
in he Uni ed S a es rom all causes has been
es ima ed be ween 114.5 and 204 cases per
100,000 persons, wi h an annual incidence
be ween 17 and 50 cases per 100,000 person-
years.2,3 Uvei is is ound in pa ien s o all ages;
hough earlier repor s indica ed a peak inci-
dence be ween ages 25 and 44, more recen
da a indica e increasing incidence ra es wi h
increasing age.2,3 Children cons i u e 5% o
10% o pa ien s wi h uvei is, bu children wi h
uvei is are a rela ively high risk o vision loss.4
Common causes o visual loss in pa ien s wi h
uvei is include secondary glaucoma, cys oid
macular edema, ca arac , hypo ony, re inal
de achmen , subre inal neovasculariza ion or
brosis, and op ic nerve a rophy.
Etiology 209
Abou 25% o all pa ien s wi h uvei is will
develop increased in raocular pressure a some
ime during he course o heir inf amma ory
disease.5 In general, uvei is-induced ocular
hyper ension and uvei ic glaucoma are more
commonly complica ions o an erior uvei is
and panuvei is because he inf amma ion in
he an erior segmen can in er ere direc ly
wi h he aqueous ou f ow rou e (Table 14-1).
Uvei ic glaucoma is also more common in
cases o granuloma ous han nongranuloma-
ous uvei is. When all causes o uvei is are con-
sidered, he prevalence o glaucoma secondary
o uvei is in adul s is es ima ed be ween 5.2%
and 19%.6 T e overall prevalence o glaucoma
in children wi h uvei is is similar o adul s,
ranging rom 5% o 13.5%; however, he
repor ed visual prognosis or children wi h
uvei ic glaucoma is worse.6,7
ETIOLO GY
T he in raocular pressure depends on he
balance o aqueous secre ion and aque-
ous ou f ow. In mos cases o uvei is, no single
mechanism can accoun or he developmen
o eleva ed in raocular pressure; ra her, i is
he resul o a combina ion o several pa ho-
logic ac ors. T e nal common pa hway o
all mechanisms con ribu ing o increased
in raocular pressure in uvei is, however, is he
impairmen o aqueous ou f ow hrough he
rabecular meshwork. In raocular inf amma-
ion can impair aqueous ou f ow by causing
derangemen s in aqueous secre ion, produc-
ing changes in aqueous con en , in l ra ing
ocular issues, and inducing irreversible al era-
ions in he an erior segmen ana omy such
as peripheral an erior synechiae and pos e-
rior synechiae ha can lead o angle closure.
T ese changes can produce a glaucoma ha is
no only severe bu also resis an o all medi-
cal herapies. Paradoxically, he rea men
o he uvei is wi h cor icos eroids can also
 210 14 UVEITIC GLAUCO MAS

TABLE 14-1. Uvei ic Condi ions Commonly Associa ed wi h Secondary Glaucoma

Anterior Uveitis
Juvenile rheumatoid arthritis
Fuchs’heterochromic uveitis
Glaucomatocyclitic crisis (Posner–Schlossman syndrome)
HLA-B27–associated uveitis (ankylosing spondylitis, Reiter’s syndrome, psoriatic arthritis)
Herpetic uveitis
Lens-induced uveitis (phacoantigenic uveitis, phacolytic glaucoma, lens particle, phacomorphic glaucoma)
Panuveitis
Sarcoidosis
Vogt–Koyanagi–Harada syndrome
Behçet’s syndrome
Sympathetic ophthalmia
Syphilitic uveitis
Intermediate Uveitis
Intermediate uveitis o the pars planitis subtype
Posterior Uveitis
Acute retinal necrosis
Toxoplasmosis

con ribu e o he developmen o eleva ed requen ly encoun ered in eyes wi h acu e

in raocular pressure.
T e pa hophysiologic mechanisms resul ing
in he developmen o eleva ed in raocular
pressure in pa ien s wi h uvei is can be simply
classi ed as ei her open angle or closed angle.
T is classi ca ion is clinically use ul because
he ini ial rea men approach di ers be ween
hese wo groups.
OPEN-ANGLE MECH ANISMS
Abnormal Aqueous Secretion
Inf amma ion o he ciliary body usually
resul s in decreased aqueous produc ion.
Decreased aqueous secre ion in eyes wi h nor-
mal ou f ow acili y resul s in he decreased
in raocular pressure or hypo ony ha is
uvei is. I , however, here is concomi an or
grea er impairmen o he aqueous ou f ow
in eyes wi h decreased aqueous produc ion
as may happen due o decreased aqueous
per usion o he rabecular meshwork, he
in raocular pressure may be normal or pos-
sibly increased.8 T ere is disagreemen as o
whe her or no aqueous hypersecre ion can
resul rom he breakdown o he blood–aque-
ous barrier in uvei ic eyes. I his were possible,
increased aqueous produc ion could con rib-
u e o he developmen o high in raocular
pressure in uvei ic eyes. ela ive o ciliary
body unc ion, he mos likely explana ion or
he eleva ed in raocular pressure in eyes wi h
in raocular inf amma ion, however, is ha he
aqueous produc ion remains normal while he
aqueous ou f ow is reduced.

Aqueous Humor Proteins
Al era ion in he aqueous humor con en was
one o he rs hypo heses o ered o explain
he onse o eleva ed in raocular pressure asso-
cia ed wi h uvei is. T e inf ux o pro eins in o
he eye resul ing rom he breakdown o he
blood–aqueous barrier is he earlies change
in uvei ic eyes ha can a ec he balance o
aqueous f ow and increase he in raocular
pressure.9 In normal eyes, he pro ein con-
en o he aqueous humor is approxima ely
100 imes less han ha in normal serum.10
However, when he blood–aqueous barrier
is disrup ed, he aqueous pro ein concen ra-
ion can resemble ha o undilu ed serum.
An increased aqueous pro ein concen ra ion
can impair aqueous ou f ow by decreasing he
f ow ra e o aqueous in o he an erior cham-
ber angle, mechanically obs ruc ing he ra-
becular meshwork, and causing dys unc ion
o he endo helial cells lining he rabecular
meshwork beams. Addi ionally, he pro eins
promo e he developmen o pos erior or
peripheral an erior synechiae. I he in egri y
o he blood–aqueous barrier is res ored, he
e ec o he aqueous pro ein concen ra ion
on he aqueous ou f ow and in raocular ocu-
lar pressure can be reversed. However, i he
permeabili y o he blood–aqueous barrier is
permanen ly damaged, leakage o serum pro-
eins in o he an erior chamber may persis
even a er he in raocular inf amma ion has
resolved.
Inf ammatory Cells
An inf ux o inf amma ory cells ha secre e
inf amma ory media ors such as pros aglan-
dins and cy okines occurs shor ly a er he pro-
ein inf ux in eyes wi h uvei is. Inf amma ory
cells in he an erior segmen are believed o
have a more direc e ec on he in raocular
pressure han aqueous pro eins. Inf amma ory
cells can increase he in raocular pressure
by in l ra ing he rabecular meshwork and
Open-angle Mechanisms 211
Schlemm’s canal, crea ing a mechanical
obs ruc ion o aqueous ou f ow. T e risk or
increased in raocular pressure is higher in
granuloma ous uvei is because o he grea er
in l ra ion o macrophages and lymphocy es
compared wi h nongranuloma ous uvei is
en i ies in which he cellular in l ra e may
con ain higher propor ions o polymorpho-
nuclear cells.5 Chronic, severe, or recurren
episodes o uvei is can cause permanen dam-
age o he rabecular meshwork rom injury o
he rabecular endo helial cells, scarring in he
rabecular meshwork and Schlemm’s canal, or
rom he orma ion o a hyaline membrane
overlying he rabeculum.6 Inf amma ory cells
and cellular debris in he an erior chamber
angle can also con ribu e o he orma ion o
peripheral an erior and pos erior synechiae.
Prostaglandins
Pros aglandins are known o produce many
o he signs o ocular inf amma ion, including
vasodila a ion, miosis, and increased vascular
permeabili y, and have complex in erac ions
on he in raocular pressure.11,12 Whe her or
no pros aglandins are direc ly responsible or
increased in raocular pressure in uvei ic eyes
is unclear. T rough heir ac ion on he blood–
aqueous barrier, hey may indirec ly con-
ribu e o increased in raocular pressure by
enhancing he inf ux o aqueous pro ein, cy o-
kines, and inf amma ory cells. Al erna ively,
hey can also decrease he in raocular pressure
by he enhancemen o uveoscleral ou f ow.
rabeculitis
rabeculi is is diagnosed when he in raocu-
lar inf amma ory response is localized o he
rabecular meshwork. Clinically, rabeculi is
is charac erized by he presence o inf am-
ma ory precipi a es on he rabecular mesh-
work in he absence o o her signs o ac ive
in raocular inf amma ion such as kera ic
precipi a es, aqueous cells, or f are. T e
 212 14 UVEITIC GLAUCO MAS
aqueous ou f ow in rabeculi is is decreased
by mechanical obs ruc ion o he rabecular
meshwork resul ing rom he accumula ion
o inf amma ory cells, swelling o he rabecu-
lar beams, and decreased phagocy osis o he
rabecular endo helial cells. Because aqueous
produc ion in he ciliary body unc ion is usu-
ally una ec ed, he in raocular pressure in eyes
wi h rabeculi is can be signi can ly eleva ed
rom he reduced aqueous ou f ow.6
Steroid-induced Ocular Hypertension
Cor icos eroids are considered rs -line drug
herapy or pa ien s wi h uvei is. Whe her
given opically, sys emically, or by periocular
or sub- enon’s injec ion, cor icos eroids are
known o accelera e he orma ion o ca arac s
and cause increased in raocular pressure via
increased ou f ow resis ance.13 T is may hap-
pen in hree ways: by inducing physical and
mechanical changes in rabecular meshwork
micros ruc ure, by increasing he deposi ion
o subs ances in he rabecular meshwork, and
by decreasing he breakdown o subs ances
in he rabecular meshwork.13 Inhibi ion o
pros aglandin syn hesis is ano her mechanism
by which cor icos eroids may impair ou f ow
acili y.
T e erms steroid-induced ocular hyperten-
sion and steroid responder are used o re er o
pa ien s who develop eleva ed in raocular
pressures rela ed o cor icos eroid herapy.
A er 4 o 6 weeks o opical s eroid rea -
men , 35% o he popula ion will have an
increase in in raocular pressure o a leas
5 mm Hg, and 5% will have grea er han a
16 mm Hg rise.14 T e risk o a s eroid response
is rela ed o he dura ion and he dose o cor-
icos eroid herapy. Pa ien s wi h glaucoma,
glaucoma suspec s, rs -degree rela ives o
people wi h glaucoma, he elderly, pa ien s
wi h connec ive issue disease, ype I diabe -
ics, high myopes, and children younger han
10 years o age are a grea es risk or a s eroid
response.6,13 Al hough s eroid-induced ocular
hyper ension may occur a any ime a er he
induc ion o cor icos eroid herapy, i is mos
requen ly de ec ed wi hin 2 o 8 weeks a er
he rea men is s ar ed. Compared wi h he
o her rou es o adminis ra ion, local s eroids
are mos requen ly associa ed wi h a s eroid
response. Periocular and in ravi real s eroid
injec ions can cause an acu e pressure rise in
suscep ible pa ien s ha may be di cul o
con rol. In mos cases, he in raocular pressure
re urns o normal a er discon inua ion o he
cor icos eroid; however, in some cases, par-
icularly ollowing a s eroid depo injec ion,
he in raocular pressure can remain eleva ed
or 18 mon hs or longer. In hese cases, surgi-
cal removal o he depo s eroid or l ra ion
surgery may be required i he in raocular
pressure canno be con rolled medically (Fig.
14-1). For his reason, depo s eroids should
be avoided when possible in known s eroid
responders. A more recen s eroid-delivery
me hod or he rea men o pos erior uvei is,
he f uocinolone ace onide implan ( e iser ),
is associa ed wi h a 71% risk o an increase in
in raocular pressure over 3 years; a combined
surgery implan ing he s eroid device and a
glaucoma drainage device may be bene cial in
selec pa ien s.15,16
When a pa ien wi h uvei is who is being
rea ed wi h cor icos eroids develops an
increased in raocular pressure, i is o en di -
cul o know i he pressure rise is a resul o
he res ored aqueous secre ion, o impaired
aqueous ou f ow caused by he in raocular
inf amma ion, a s eroid response, or a combi-
na ion o all hree. Al hough a all in he in ra-
ocular pressure as he s eroid is apered may
be evidence o s eroid-induced ocular hyper-
ension, he decline in pressure could also
be secondary o improved ou f ow hrough
he rabecular meshwork or a recurrence o
inf amma ion wi h aqueous hyposecre ion.
I a s eroid response ha canno be easily
 Open-angle Mechanisms 213

con rolled medically is suspec ed in a pa ien
who main ains ac ive in raocular inf amma-
ion requiring sys emic cor icos eroids, his
may be an indica ion or he ini ia ion o
a s eroid-sparing agen . I s eroid-induced
ocular hyper ension is suspec ed in a pa ien
wi h con rolled or quiescen uvei is, a reduc-
ion in he concen ra ion, dose, or re-
quency o he cor icos eroid used should be
at emp ed.

FIGU E 14-1. Periocular steroid injection in a steroid responder. Periocular s eroid injec ions, use ul in
rea ing bo h an erior and pos erior uvei is, can some imes induce a severe eleva ion in he in raocular pressure
in pa ien s wi h ocular hyper ension and known s eroid responders. T e an eriorly placed s eroid depo in his
16 year old pa ien wi h presumed sarcoidosis was removed when medical herapy ailed o con rol his eleva ed
in raocular pressure. Subsequen ly, his pressures normalized.
 214 14 UVEITIC GLAUCO MAS
CLOSED-ANGLE
MECH ANISMS
M orphologic changes in he an erior
chamber s ruc ures as a resul o uve-
i is are o en irreversible and lead o signi -
can eleva ions in he in raocular pressure by
al ering or preven ing he f ow o aqueous
rom he pos erior chamber o he rabecular
meshwork. T e s ruc ural changes ha ypi-
cally lead o secondary angle closure include
peripheral an erior synechiae, pos erior syn-
echiae, and pupillary membranes ha can
cause pupillary block and, less commonly, or-
ward ro a ion o he ciliary body.
Peripheral Anterior Synechiae
Peripheral an erior synechiae are adhesions
be ween he iris and he rabecular mesh-
work or cornea ha can comple ely block or
impair access o he aqueous o he rabecu-
lar meshwork. Bes de ec ed by gonioscopy,
peripheral an erior synechiae are a common
complica ion o an erior uvei is and occur
more commonly in granuloma ous han non-
granuloma ous causes o uvei is. Peripheral
an erior synechiae resul rom he organiza-
ion o inf amma ory ma erial ha pulls he iris
sur ace in o he angle. T ey develop more re-
quen ly in eyes wi h preexis ing narrow angles
or hose narrowed by iris bombé. T e iris
at achmen s are usually broad, covering large
segmen s o he angle, bu can also be pa chy
or peaked, a ec ing only small por ions o he
rabecular meshwork or cornea (Fig. 14-2). In
cases o peripheral an erior synechiae rela ed
o uvei is, even hough large por ions o he
angle may remain open, he pa ien may s ill
have increased in raocular pressure because
he remaining angle is unc ionally compro-
mised because o prior inf amma ory damage
ha may no be de ec able by gonioscopy.5
In cases o recurren or chronic uvei is, con in-
ued peripheral an erior synechiae orma ion
can resul in comple e angle closure. Neova-
sculariza ion o he iris and he angle should
be sough in all cases o uvei is presen ing wi h
angle closure or ex ensive peripheral an erior
synechiae. Con rac ion o he brovascu-
lar issue in he angle or an erior iris sur ace
may rapidly induce a comple e and severe
angle closure. Neovascular glaucoma second-
ary o uvei is is ypically resis an o medical
and surgical herapy and has a poor prognosis
(Fig. 14-3).
Posterior Synechiae
Inf amma ory cells, pro ein, and brin in he
aqueous humor can s imula e pos erior syn-
echiae orma ion. Pos erior synechiae are
adhesions be ween he pos erior iris sur ace
and he an erior lens capsule, he vi reous ace
in aphakic pa ien s, or he in raocular lens in
pseudophakic individuals. T e likelihood o
developing pos erior synechiae is rela ed o
he ype, dura ion, and severi y o he uvei is.
T e grea er he ex en o he pos erior syn-
echiae, he less he pupil is able o dila e and
he grea er he risk or ur her synechiae or-
ma ion in subsequen uvei ic recurrences.
T e erm pupillary block is used o deno e
impaired aqueous f ow be ween he pos erior
and an erior chamber hrough he pupillary
aper ure as a resul o pos erior synechiae.
Seclusio pupillae, pos erior synechiae ha
ex end or 360 degrees around he pupil, and
pupillary membranes can cause comple e
pupillary block. In his condi ion, here is
no f ow o aqueous rom he pos erior o he
an erior chamber. T e buildup o aqueous in
he pos erior chamber may produce a severe
eleva ion o he in raocular pressure ha
causes orward bowing o he iris in o he an e-
rior chamber, or iris bombé (Fig. 14-4). Iris
bombé in an eye wi h ongoing inf amma ion
may resul in he rapid developmen o angle
closure caused by he orma ion o periph-
eral an erior synechiae due o apposi ional
 Closed-angle Mechanisms 215

iridocorneal con ac , even in an eye ha may suprachoroidal e usions ha may resul in

have previously had an open angle.17 In some
cases o uvei is wi h pupillary block, i he iri-
dolen icular adhesions are su cien ly broad,
only he peripheral iris may bulge orward and
he iris bombé may be di cul o diagnose
wi hou he use o gonioscopy.
Forward Rotation o the Ciliary Body
Acu e in raocular inf amma ion can cause
ciliary body swelling and supraciliary or
he orward ro a ion o he ciliary body, caus-
ing angle closure no associa ed wi h pupillary
block. Eleva ed in raocular pressure due o
his ype o angle closure occurs mos o en in
pa ien s wi h iridocycli is, annular choroidal
de achmen s, and pos erior scleri is, and can
be seen in he acu e s age o Vog –Koyanagi–
Harada syndrome.5

FIGU E 14-2. HLA B27–associated anterior uveitis. Bo h pos erior synechiae and a broad area o peripheral
an erior synechiae obli era ing he an erior chamber angle and ex ending on o he cornea superior are
seen in his pa ien wi h HLA B27 associa ed an erior uvei is ollowing a severe exacerba ion o in raocular
inf amma ion.
 216 14 UVEITIC GLAUCO MAS

A B
FIGU E 14-3. Neovascular glaucoma. T is pa ien wi h granuloma ous panuvei is developed in rac able
neovascular glaucoma, one o he mos severe complica ions o uvei is. No e he di use, mut on a kera ic
precipi a es and iris bombé (A) and he neovasculariza ion in he broad peripheral an erior synechiae (B).

FIGU E 14-4. Posterior synechiae causing pupillar y block and iris bombé. T is pa ien wi h Vog
Koyanagi Harada syndrome presen ed wi h an erior segmen inf amma ion and increased in raocular pressure
as a resul o pos erior synechiae causing pupillary block wi h iris bombé. T e uvei is was managed wi h opical
and sys emic cor icos eroids, and he eleva ed in raocular pressure normalized ollowing a laser irido omy.

DIAGNOSIS
T he accura e diagnosis and managemen
o glaucoma in pa ien s wi h uvei is relies
on a horough oph halmic examina ion and
he appropria e use o ancillary es s. Sli -lamp
examina ion is required o es ablish he clas-
si ca ion o he uvei is, he degree o inf am-
ma ory ac ivi y, and he ype o inf amma ory
reac ion. Uvei is can be classi ed ana omically
as an erior, in ermedia e, pos erior, or panuve-
i is according o he primary si e o he inf am-
ma ion in he eye.
T e likelihood o uvei ic glaucoma is grea er
in cases o an erior uvei is and panuvei is in
which he s ruc ures involved in he aque-
ous ou f ow are more likely o be damaged by
in raocular inf amma ion. T e severi y o he
in raocular inf amma ion can be de ermined
by assessing he aqueous cells and f are in he
an erior chamber and he vi reous cells and
haze. Addi ionally, he s ruc ural changes in
he ocular archi ec ure induced by he inf am-
ma ory disease, such as peripheral an erior
and pos erior synechiae, should be no ed.
T e inf amma ory response in eyes wi h uvei is
can be ei her granuloma ous or nongranulo-
ma ous. Signs o granuloma ous uvei is in he
an erior segmen include mut on a kera ic
precipi a es and iris nodules (Figs. 14-3 and
14-5). Granuloma ous uvei is is associa ed
wi h a higher incidence o uvei ic glaucoma
han nongranuloma ous uvei is.
Gonioscopy is he mos cri ical par o oph-
halmic examina ion in pa ien s wi h uvei is
and increased in raocular pressure and should
be per ormed using a lens ha inden s he cen-
ral cornea and pushes he aqueous in o he
angle. Gonioscopic examina ion reveals he
Diagnosis 217
presence o inf amma ory ma erial, peripheral
an erior synechiae, and neovasculariza ion in
he angle, allowing di eren ia ion be ween
open-angle and closed-angle glaucoma.
On undus examina ion, par icular at en ion
should be direc ed o he op ic nerves, which
should be assessed or excava ion, hemor-
rhage, edema, or hyperemia. T e re inal nerve
ber layer should also be evalua ed. T e diag-
nosis o uvei ic glaucoma should no be made
wi hou documen ed glaucoma ous disk dam-
age and or visual eld loss. Al hough re inal or
choriore inal lesions in he pos erior pole do
no con ribu e o he developmen o uvei ic
glaucoma, he presence and loca ion o lesions
ha may mani es as a visual eld de ec and
resul in an incorrec diagnosis o uvei ic glau-
coma should be no ed (Fig. 14-6).
Applana ion onome ry is required during
every clinical assessmen , and reliable per-
sonnel should rou inely per orm visual eld
es ing. O her ancillary es s ha may be use-
ul or he diagnosis and ollow-up o pa ien s
wi h uvei is and increased in raocular pres-
sure include laser f are pho ome ry and ocu-
lar ul rasonography. Laser f are pho ome ry
is able o de ec sligh changes in he aque-
ous humor f are or pro ein con en ha can-
no be assessed by he sli -lamp examina ion.
T e changes de ec ed by he pho ome er have
been shown o be use ul in de ermining he
ac ivi y o he uvei is.5 B-scan ul rasonogra-
phy and ul rasound biomicroscopy are use-
ul in he assessmen o uvei ic glaucoma by
demons ra ing he morphology o he ciliary
body and iridocorneal angle, which is help-
ul in de ermining he cause o bo h eleva ed
and abnormally low in raocular pressures in
pa ien s wi h uvei is.5
 218 14 UVEITIC GLAUCO MAS

A B
FIGU E 14-5. Sarcoidosis and active granulomatous panuveitis. A. T is pa ien wi h sarcoidosis presen ed
wi h an ac ive granuloma ous panuvei is, including Busacca nodules seen here in he iris s roma and secondary
glaucoma resul ing rom pos erior synechiae wi h pupillary block. Despi e managemen wi h opical and
sys emic cor icos eroids and opical an iglaucoma medica ions, his in raocular pressures were uncon rolled.
Examina ion o he op ic nerve head and visual eld es ing were consis en wi h glaucoma. B. wo mon hs
ollowing ube shun placemen or uvei ic glaucoma, he in raocular pressures were con rolled and he iris
nodules were resolved.

B
A
FIGU E 14-6. Multifocal choroiditis. T is pa ien wi h mul i ocal choroidi is demons ra es he need or
care ul examina ion o he op ic nerve or evidence o glaucoma in pa ien s wi h uvei is. Because o he ex ensive
pos erior pole lesions, visual eld es ing did no reliably demons ra e he developmen o glaucoma in he le
eye, evidenced by he progressive cupping o he op ic disc. A. Righ eye. B. Le eye.

MANAGEMENT
T he rs goal in he rea men o pa ien s
wi h uvei is-induced ocular hyper en-
sion or uvei ic glaucoma is he con rol o he
in raocular inf amma ion and preven ion o
permanen s ruc ural changes in he eyes.
In some pa ien s, resolu ion o he in raocu-
lar inf amma ion wi h appropria e herapy
alone may normalize he in raocular pressure.
Addi ionally, irreversible consequences o
uvei is such as peripheral an erior and pos-
erior synechiae can be preven ed wi h early
an i-inf amma ory herapy combined wi h
mydria ics and cycloplegics.
T e rs -line rea men in mos cases o uvei is
requires he use o cor icos eroids opically,
locally via periocular or sub- enon’s injec-
ion, or sys emically. opical cor icos eroids
are use ul or an erior segmen inf amma-
ion bu alone are inadequa e herapy or a
phakic pa ien wi h ac ive pos erior segmen
inf amma ion. T e requency o adminis ra-
ion o he opical cor icos eroids depends
on he severi y o he inf amma ion in he
an erior segmen . Prednisolone ace a e 1%
(Pred For e) is superior o mos o her opical
cor icos eroid ormula ions or he con rol o
an erior segmen inf amma ion. Likewise, i
is also he opical s eroid ormula ion ha is
mos likely o cause s eroid-induced in raocu-
lar hyper ension and pos erior subcapsular
ca arac s. A new opical s eroid, dif upredna e
(Durezol), has been shown o have equal e -
cacy o prednisolone ace a e 1% wi h less re-
quen dosing; da a on i s propensi y o cause
eleva ed in raocular pressure and ca arac are
no ye available.18 Less-po en opical s eroid
ormula ions such as rimexolone, f uorome h-
olone, medrysone, and lo eprednol e abona e
(Lo emax) are less likely o cause a s eroid
response bu are also less e ec ive in con rol-
ling he in raocular inf amma ion. In our expe-
rience, opical nons eroidal an i-inf amma ory
Management 219
agen s play no signi can role in he rea men
o uvei is or preven ion o i s complica ions.
Periocular s eroid injec ions o riamcinolone
(Kenalog, 40 mg per mL) in o sub- enon’s
space or ranssep ally hrough he lower lid,
or in ravi real injec ions o preserva ive- ree
ormula ions o riamcinolone, can be e ec-
ive or he con rol o bo h an erior and pos-
erior segmen in raocular inf amma ion. T e
main drawback o periocular and in raocu-
lar s eroids is heir grea er po en ial o cause
eleva ed in raocular pressure and ca arac in
suscep ible pa ien s. I is here ore inadvisable
o adminis er periocular injec ions o depo
s eroid in pa ien s wi h uvei is and in raocu-
lar hyper ension because o heir long-las ing
e ec , which canno be readily discon inued.
Oral cor icos eroids are he mains ay o uvei is
herapy, wi h s ar ing doses as high as 1 mg/
kg/ day, depending on he severi y o he dis-
ease. Sys emic s eroids should be apered once
he in raocular inf amma ion is con rolled. I
sus ained con rol o he in raocular inf am-
ma ion using cor icos eroids alone is no pos-
sible because o heir side e ec s or because
o persis en disease ac ivi y, a second-line
immunosuppressan or a s eroid-sparing med-
ica ion may be needed. S eroid-sparing agen s
commonly used or he rea men o uvei is
include cyclosporine, me ho rexa e, aza-
hioprine, mycophenola e mo e il, and more
recen ly NF-α inhibi ors and o her biologic
agen s.19–21 Alkyla ing agen s such as cyclo-
phosphamide and chlorambucil are generally
reserved or severe cases o uvei is.19
Mydria ic and cycloplegic agen s are used in
he rea men o pa ien s wi h an erior seg-
men in raocular inf amma ion o relieve he
pain and discom or associa ed wi h ciliary
muscle and iris sphinc er spasm. Because
hese agen s also dila e he pupil, hey are also
use ul in preven ing and breaking synechiae,
which can al er aqueous f ow and con ribu e
 220 14 UVEITIC GLAUCO MAS
o eleva ed in raocular pressure. Commonly
prescribed agen s or his purpose are a ro-
pine, scopolamine, homa ropine, phenyleph-
rine, cyclopen ola e, and ropicamide. Some
clinicians pre er rela ively shor -ac ing agen s
o reduce he risk o pos erior synechiae orm-
ing in a dila ed posi ion.
Medical T erapy
Once he in raocular inf amma ion has been
adequa ely addressed, speci c herapy should
also be adminis ered o con rol he in raocular
pressure. In general, he medical herapy or
uvei is-induced ocular hyper ension and uve-
i ic glaucoma relies primarily on aqueous sup-
pressan s or pressure con rol. An iglaucoma
medica ions used in he rea men o uvei ic
glaucoma include be a-blockers, carbonic
anhydrase inhibi ors, adrenergic agen s, and
hyperosmo ic agen s or emergen con rol o
acu e pressure eleva ions. As a group, mio ic
agen s and pros aglandin-like agen s are gen-
erally avoided in pa ien s wi h uvei is because
hey may exacerba e he in raocular inf amma-
ion. opical adrenergic an agonis s are he
drugs o choice or he rea men o increased
in raocular pressure in pa ien s wi h uvei ic
glaucoma because hey decrease aqueous
humor produc ion wi hou a ec ing he pupil
size. Be a-blockers commonly used in pa ien s
wi h uvei is include imolol, be axolol, car-
eolol, and levobunolol. Be axolol, which has
ewer pulmonary side e ec s, may be sa er o
use in pa ien s wi h sarcoid uvei is and known
pulmonary disease. Me ipranolol has been
repor ed o cause a granuloma ous iridocycli-
is in some pa ien s and should probably be
avoided in pa ien s wi h uvei is.22
Carbonic anhydrase inhibi ors ha reduce
in raocular pressure by inhibi ing aqueous
humor produc ion can be given opically,
orally, or in ravenously. T e oral carbonic
anhydrase inhibi or ace azolamide (Diamox)
has been repor ed o reduce cys oid macular
edema, which is a common cause o visual
loss in pa ien s wi h uvei is.23 opical carbonic
anhydrase inhibi ors are unlikely o have a
similar e ec on macular edema because su -
cien concen ra ions probably do no reach
he re ina.
Adrenergic agen s ha are used in he rea -
men o uvei ic glaucoma include apracloni-
dine, par icularly o con rol acu e in raocular
pressure eleva ions ha can occur a er a neo-
dymium (Nd):YAG capsulo omy and brimo-
nidine; bo h are alpha-2 agonis s ha lower
in raocular pressure by decreasing aqueous
humor produc ion and increasing uveoscleral
ou f ow. Granuloma ous an erior uvei is has
also been repor ed as a la e adverse e ec o
rea men wi h brimonidine (11 o 15 mon hs
a er s ar ing rea men ).17 Al hough hey are
now used in requen ly, epinephrine and dip-
ive rin, which bo h lower in raocular pressure
primarily by increasing aqueous ou f ow, also
cause mydriasis, which could be help ul in he
preven ion o synechiae in uvei ic eyes.
Pros aglandin analogs are hough o reduce
in raocular pressure by increasing uveoscleral
ou f ow.5 Al hough e ec ive a lowering
in raocular pressure, he bene o his class
o agen s in he rea men o uvei is is ques-
ioned because la anopros (Xala an) has been
repor ed o induce in raocular inf amma ion
and cys oid macular edema.24 However, ran-
domized con rolled rials have no es ablished
a causal rela ionship.17
Hyperosmo ic agen s rapidly lower in ra-
ocular pressure, primarily by a reduc ion in
he vi reous volume, and are help ul in he
managemen o uvei ic pa ien s wi h acu e
angle closure. Glycerin and isosorbide can be
adminis ered orally, whereas manni ol is given
in ravenously.
Cholinergic agen s such as pilocarpine, echo-
hiopha e iodide, eserine, and carbachol are
generally avoided in pa ien s wi h uvei is. T is

is because he induced miosis caused by hese
agen s may po en ia e orma ion o pos e-
rior synechiae, aggrava e ciliary body muscle
spasm, and con ribu e o a prolonga ion o he
ocular inf amma ory response by enhancing
he breakdown o he blood–aqueous barrier.
MANAGEMENT OF ANGLE-
CLOSURE GLAUCOMA
I ris bombé and angle closure caused by
pupillary block are requen ly he cause
o severe in raocular pressure eleva ions and
secondary glaucoma in pa ien s wi h uvei is.
When pupillary block is responsible or he
obs ruc ion o aqueous ou f ow, a commu-
nica ion be ween he pos erior and an erior
chambers can be rees ablished using an argon
or Nd:YAG laser irido omy or a surgical iri-
dec omy. Laser irido omy may worsen or
reac iva e an erior chamber inf amma ion.
o lessen he likelihood o his complica ion,
he pa ien should be rea ed aggressively
wi h opical cor icos eroids prior o and a er
he procedure. Compared wi h he argon
laser, he Nd:YAG laser requires he delivery
o less energy and induces less pos opera ive
inf amma ion. Because laser irido omies are
prone o closure, par icularly in eyes wi h
ac ive inf amma ion, several irido omies
should be per ormed o ensure adequa e
aqueous f ow (Fig. 14-7). epea procedures
are needed in approxima ely 40% o uvei ic
eyes.6 o reduce he risk o endo helial dam-
age, laser irido omy should no be per ormed
in eyes wi h severe ac ive uvei is or corneal
edema, or in he areas o peripheral an erior
synechiae.
Surgical iridec omy is indica ed when laser
irido omies are unsuccess ul or he use o
laser is con raindica ed. Surgical iridec omy is
repor ed o be success ul in uvei ic eyes wi h
peripheral an erior synechiae ha involves less
han 75% o he angle.6 Al hough generally
Management o Angle-closure Glaucoma 221
more e ec ive han laser irido omy, he pro-
cedure can lead o severe surgically induced
pos opera ive inf amma ion ha may be
blun ed by he use o aggressive preopera ive
and pos opera ive an i-inf amma ory herapy;
in ravenous cor icos eroids a he ime o he
procedure may also be bene cial. Compared
wi h a laser irido omy, a large-sec or surgical
iridec omy may delay ca arac progression.
In uvei ic eyes in which he angle closure is
caused by orward ro a ion o he ciliary body
wi hou evidence o pupillary block, laser
irido omy and surgical iridec omy are o no
use. T e angle closure and eleva ed in raocu-
lar pressure in his rare group o pa ien s are
bes rea ed wi h immunosuppressive herapy
and aqueous suppressan s. A surgical l ra ion
procedure may be required in hese cases i
he in raocular pressure canno be con rolled
medically and he angle closure canno be
reversed because o he orma ion o periph-
eral an erior synechiae.
Goniosynechialysis has been repor ed o be
success ul in lowering he in raocular pressure
and es ablishing a normal an erior chamber
angle in cases o acu e angle closure resul ing
rom ex ensive and recen orma ion o periph-
eral an erior synechiae. rabeculodialysis, he
disinser ion o he rabeculum rom he scleral
spur using a gonio omy kni e, allows he aque-
ous direc access in o Schlemm’s canal and has
been used in children and young adul s wi h
uncon rolled uvei ic glaucoma.6
Argon laser rabeculoplas y is no recom-
mended or he rea men o uvei is-induced
ocular hyper ension or he rea men o uve-
i ic glaucoma because he hermal energy and
addi ional laser-induced inf amma ion may
ur her damage he previously injured rabec-
ular meshwork.
In secondary uvei ic glaucoma, he damaging
mechanism is nearly always in raocular hyper-
ension. Because here is usually no primary
 222 14 UVEITIC GLAUCO MAS
disc pa hology and because pa ien s wi h uve-
i is are rela ively young, here is a endency o
olera e hyper ension or longer periods and
o olera e higher levels o in raocular pressure
be ore using surgical in erven ion. However,
when he in raocular pressure remains uncon-
rolled in pa ien s receiving maximal medical
herapy or here is evidence o op ic nerve
injury or visual eld de ec s, surgical in er-
ven ion o con rol he in raocular pressure is
required.
Surgical procedures per ormed in pa ien s
wi h uvei ic glaucoma include rabeculec-
omy wi h and wi hou he use o an ime ab-
oli es and ube shun procedures such as he
Ahmed, Baerveld , and Mol eno implan s5,6,25
(Fig. 14-8). T e bes surgical procedure or
pa ien s wi h uvei ic glaucoma has no been
es ablished.
All surgical procedures per ormed on pa ien s
wi h uvei is carry he risk o a pos opera ive
f are, which ypically occurs in he rs pos -
opera ive week. Pos opera ive inf amma ion
or reac iva ion o uvei is has been repor ed
o occur in 5.2% o 31.1% cases o uvei ic
glaucoma rea ed surgically.26 T e risk o a
pos opera ive f are is decreased in eyes ha
are quiescen prior o he surgical procedure.
For elec ive surgeries, we require ha he eyes
remain quie or a leas 3 mon hs prior o he
opera ive procedure. o help o decrease he
risk o a pos opera ive f are, approxima ely
1 week prior o he planned surgery day, he
pa ien ’s opical or sys emic immunosuppres-
sive regimen, or bo h, is increased and apered
pos opera ively according o inf amma ory
response. In raopera ively, periocular, in raoc-
ular, and/ or in ravenous s eroids are rou inely
given. For emergen glaucoma procedures
in pa ien s wi h ac ive disease, an exacerba-
ion o he exis ing inf amma ion should be
expec ed; here ore, aggressive opical herapy
and he use o high-dose oral (0.5 o 1.5 mg/
kg/ day) or in ravenous cor icos eroids may be
required in he periopera ive period. For many
pa ien s, we pre er a single in raopera ive dose
o 250 o 1,000 mg o in ravenous me hylpred-
nisolone, as a single pulse dose no requiring a
gradual aper.
epor ed success ra es or rabeculec omy
in pa ien s wi h uvei is glaucoma range rom
62% o 81%.27,17 However, depending o some
ex en on he ollow-up in erval, he rue sig-
ni cance o such ndings is no en irely clear.
In rabeculec omy cases per ormed in pa ien s
wi h uvei is, he pos opera ive inf amma-
ory response is believed o accelera e he
wound-healing process and cause ailure o
he l ering procedure.28 T e ou come o ra-
beculec omies in pa ien s wi h uvei is may
be improved by he use o aggressive periop-
era ive an i-inf amma ory herapy and an i-
me aboli es such as mi omycin C, which is
avored over 5-f uorouracil.6 T e higher suc-
cess ra es o l ering surgery wi h he use o
wound-modula ing agen s, however, is associ-
a ed wi h an eleva ed risk or hypo ony, bleb
leaks, and endoph halmi is, which has been
repor ed in up o 9.4% o eyes ollowing rab-
eculec omy.29 Ca arac progression is also very
common a er l ra ion surgery or uvei ic
glaucoma.
Implan drainage procedures have also been
used or he rea men o uvei ic glaucoma,
mos commonly in pa ien s who have ailed
previous l ering procedures.6,25 T ey have
been repor ed o be more success ul han a
repea rabeculec omy in pa ien s wi h uve-
i is.27 Glaucoma drainage devices are also used
as a primary rea men or uvei ic glaucoma
wi h increasing requency, and ur her s udy is
needed o de ni ively compare his approach
wi h rabeculec omy.17 Pos opera ive compli-
ca ions such as choroidal e usion, choroidal
hemorrhage, and shallow an erior chambers
may be grea er in eyes wi h uvei ic glaucoma
as compared wi h eyes wi h primary open-
angle glaucoma (Fig. 14-9).

Nonpene ra ing glaucoma surgery may also
have a role in he surgical managemen o uve-
i ic glaucoma, hough i is no an op ion in eyes
wi h ex ensive an erior synechiae obs ruc ing
he rabecular meshwork. Viscocanalos omy
has been shown o be e ec ive in pa ien s wi h
open-angle glaucoma wi h a lower ra e o com-
plica ions han rabeculec omy. A small series
has repor ed success ul in raocular pressure
con rol using nonpene ra ing surgery in eyes
wi h uvei ic glaucoma. However, addi ional
s udy is needed o valida e he sa e y and
e cacy o nonpene ra ing surgery in uvei ic
glaucoma.17
Ciliary body des ruc ive procedures should be
considered as a las resor or he rea men o
uvei ic glaucoma in which in raocular pressure
is no amenable o any o her medical or surgi-
cal glaucoma rea men . Cyclocryo herapy and
con ac and noncon ac laser cycloabla ion
procedures are generally similar in heir abili y
o success ully lower he in raocular pressures.
T e primary disadvan age o cycloabla ive rea -
men s is he induc ion o a severe in raocular
inf amma ory response and he developmen
o ph hisis bulbi in abou 10% o rea ed eyes.30
EFE ENCES
1. London NJS, a hinam S , Cunningham E . T e
epidemiology o uvei is in developing coun ries. Int
Ophthalmol Clin. 2010;50(2):1–17.
2. Gri z DC, Wong IG. Incidence and prevalence o uve-
i is in nor hern Cali ornia: T e nor hern Cali ornia
epidemiology o uvei is s udy. Ophthalmology.
2004;111:491–500.
3. Darrell W, Wagener HP, Kurland L . Epidemiology
o uvei is: Incidence and prevalence in a small urban
communi y. Arch Ophthalmol. 1962;68:502–514.
4. Cunningham E Jr. Uvei is in children. Ocul Immunol
Inf amm. 2000;8:251–261.
5. ran V , Mermoud A, Herbor CP. Appraisal and
managemen o ocular hypo ony and glaucoma asso-
cia ed wi h uvei is. Int Ophthalmol Clin. 2000;
40:175–203.
6. Moor hy S, Mermoud A, Baerveld G, e al.
Glaucoma associa ed wi h uvei is. Surv Ophthalmol.
1997;41:361–394.
Management o Angle-closure Glaucoma 223
7. Kanski JJ, Shun-Shin GA. Sys emic uvei is syn-
dromes in childhood: An analysis o 340 cases.
Ophthalmology. 1984;91:1247–1252.
8. Johnson DH. Human rabecular meshwork cell
survival is dependen on per usion ra e. Invest
Ophthalmol Vis Sci. 1996;37(6):1204–1208.
9. Ellio . A reatise on Glaucoma. London: Ox ord
Medical Publica ions; 1918.
10. Pere z WL, omasi B. Aqueous humor pro eins in
uvei is. Immunoelec rophore ic and gel di usion
s udies on normal and pa hological human aqueous
humor. Arch Ophthalmol. 1961;65:20–23.
11. Bei ch B , Easkins KE. T e e ec s o pros aglan-
dins on he in raocular pressure o he rabbi . Br J
Pharmacol. 1969;37:158–167.
12. Bhat acherjee P. T e role o arachidona e me abo-
li es in ocular inf amma ion. Prog Clin Biol Res.
1989;312:211–227.
13. Jones 3rd, hee DJ. Cor icos eroid-induced ocu-
lar hyper ension and glaucoma: a brie review and
upda e o he li era ure. Curr Opin Ophthalmol. 2006;
17:163–167.
14. Weinreb N, Mi chell MD, Polansky J . Pros aglandin
produc ion by human rabecular cells: In vi ro inhibi-
ion by dexame hasone. Invest Ophthalmol Vis Sci.
1983;24:1541–1545.
15. Golds ein DA, God rey DG, Hall A, e al. In raocular
pressure in pa ien s wi h uvei is rea ed wi h f uo-
cinolone ace onide implan s. Arch Ophthalmol. 2007;
125:1478–1485.
16. Malone P, Herndon LW, Muir KW, e al. Combined
f uocinolone ace onide in ravi real inser ion and
glaucoma drainage device placemen or chronic
uvei is and glaucoma. Am J Ophthalmol. 2010;
149:800–806.
17. Kuch ey W, Lowder CY, Smi h SD. Glaucoma
in pa ien s wi h ocular inf amma ory disease.
Ophthalmol Clin North Am. 2005;18:421–430.
18. Fos er CS, Davanzo , Flynn E, e al. Durezol
(dif upredna e oph halmic emulsion 0.05%) com-
pared wi h Pred For e 1% oph halmic suspension in
he rea men o endogenous an erior uvei is. J Ocul
Pharmacol T er. 2010;26(5):475–483.
19. Jabs DA, osenbaum J , Fos er CS, e al. Guidelines or
he use o immunosuppressive drugs in pa ien s wi h
ocular inf amma ory disorders: ecommenda ions
o an exper panel. Am J Ophthalmol. 2000;
130:492–513.
20. Larkin G, Ligh man S. Mycophenola e mo e il. A use-
ul immunosuppressive in inf amma ory eye disease.
Ophthalmology. 1999;106:370–374.
21. Heiligenhaus A, T urau S, Hennig M, e al. An i-
inf amma ory rea men o uvei is wi h biological:
New rea men op ions ha ref ec pa hogene ic
 224 14 UVEITIC GLAUCO MAS
knowledge o he disease. Grae es Arch Clin Exp
Ophthalmol. 2010;248:1531–1551.
22. Akingbehin , Villada J . Me ipranolol-associa ed
granuloma ous an erior uvei is. Br J Ophthalmol.
1991;75:519–523.
23. Whi cup SM, Csaky KG, Podgor MJ, e al. A ran-
domized, masked, cross-over rial o ace azolamide
or cys oid macular edema in pa ien s wi h uvei is.
Ophthalmology. 1996;103:1054–1062.
24. Warwar E, Bullock JD, Ballal D. Cys oid macu-
lar edema and an erior uvei is associa ed wi h
la anopros use. Experience and incidence in a re -
rospec ive review o 94 pa ien s. Ophthalmology.
1998;105:263–268.
25. Da Ma a A, Burk SE, Ne land PA, e al. Managemen
o uvei ic glaucoma wi h Ahmed glaucoma valve
implan a ion. Ophthalmology. 1999;106:2168–2172.
26. Pra a JA Jr, Neves R , Minckler DS, e al.
rabeculec omy wi h mi omycin C in glaucoma associ-
a ed wi h uvei is. Ophthalmic Surg. 1994;25:616–620.
27. Hill R , Nguyen QH, Baerveld G, e al.
rabeculec omy and Mol eno implan a ion or glau-
comas associa ed wi h uvei is. Ophthalmology. 1993;
100:903–908.
28. Sku a GL, Parrish K 2nd. Wound healing in glaucoma
l ering surgery. Surv Ophthalmol. 1987;32:149–170.
29. Wolner B, Liebmann JM, Sassani JW, e al. La e
bleb-rela ed endoph halmi is a er rabeculec omy
wi h adjunc ive 5-f uorouracil. Ophthalmology.
1991;98:1053–1060.
30. Schuman JS, Bellows A , Shingle on BJ, e al.
Con ac ransscleral Nd:YAG laser cyclopho oco-
agula ion. Mid erm resul s. Ophthalmology. 1992;
99:1089–1094.
 Management o Angle-closure Glaucoma 225

FIGU E 14-7. ecurrent iris bombé. T is pa ien presen ed wi h acu e eye pain and increased in raocular
pressure rom recurren iris bombé when he previous laser irido omy si e closed during a uvei ic f are associa ed
wi h he apering o her sys emic immunosuppression.

A B
FIGU E 14-8. Bilateral Baerveldt implants in patient with JR . T is 16 year old emale pa ien developed
bila eral an erior uvei is a he age o 3 years ha has been well managed wi h a combina ion o opical and
sys emic an i inf amma ory herapy. Because o uncon rolled in raocular pressure, she underwen bila eral
Baerveld implan s as a primary glaucoma procedure wi h excellen resul s. A. Righ eye showing he implan
ube in he an erior chamber. B. Righ eye looking down and nasally, revealing he conjunc ival bleb over he
implan .
 226 14 UVEITIC GLAUCO MAS

A B
FIGU E 14-9. Complications of glaucoma surger y in uveitis patient. Hypo ony wi h choroidal e usion and
a shallow an erior chamber A, di use illumina ion; B, sli beam is a common complica ion o implan drainage
procedures in pa ien s wi h uvei is.

SPECIFIC
SP
P E CII FIC E
ENN I IES
I ES
S
FUCHS’ HETEROCHROMIC
IRIDOCYCLITIS
F uchs’ he erochromic iridocycli is is ypi-
cally a unila eral, chronic, low-grade,
nongranuloma ous an erior uvei is ha is
associa ed wi h secondary pos erior subcap-
sular ca arac and glaucoma in 13% o 59% o
cases.1 T e he erochromia ha charac erizes
he condi ion is a resul o in raocular inf am-
ma ion leading o iris a rophy in he a ec ed
eye.
EPIDEMIOLOGY
Fuchs’ he erochromic iridocycli is is
hough o be a rela ively uncommon cause o
an erior uvei is, accoun ing or 1.2% o 3.2%
o all uvei is cases, hough recen ly repor ed
o comprise 12% o pa ien s evalua ed a a
er iary re erral cen er.2,3
T e condi ion is unila eral in 90% o cases
and appears o a ec men and women equally.
T e disease is mos commonly diagnosed
in he hird o our h decades o li e.
Es ima es o he overall incidence o glau-
coma in Fuchs’ pa ien s range rom 13% o 60%
and may be higher in pa ien s wi h bila eral dis-
ease and in A rican-American pa ien s.4,6
ETIOLO GY
A growing body o evidence correla es
Fuchs’ uvei is wi h he presence o in raocular
an ibodies o ubella virus.5
T e increased in raocular pressure in
Fuchs’ he erochromic iridocycli is is hough
o be he resul o decreased aqueous ou -
f ow caused by inf amma ory cells or a hya-
line membrane obs ruc ing he rabecular
meshwork.
Specifc Entities 227
HISTORY
Pa ien s wi h his condi ion are ypically
asymp oma ic, al hough some pa ien s may
have mild ocular discom or and blurred
vision.
T ey are no known o have associa ed
sys emic disease. T ey requen ly come o
medical at en ion because o decreased vision
associa ed wi h ca arac progression.
DIFFERENTIAL DIAGNOSIS
T e di eren ial diagnosis or Fuchs’ he -
erochromic iridocycli is includes herpe ic
uvei is, he Posner–Schlossman syndrome,
sarcoidosis, syphilis, and, in hose cases wi h
pos erior pole lesions, oxoplasmosis.
DIAGNOSTIC EVALUATION
Ophthalmic Examination
Ex ernal examina ion ypically reveals a
whi e, quie eye. T e an erior segmen gener-
ally shows a unila eral, low-grade, nongranu-
loma ous an erior uvei is. T e cornea shows
s ella e kera ic precipi a es scat ered over he
en ire endo helium, which are an impor an
clue o he diagnosis (Fig. 14-10A).
In pa ien s wi h dark irides, he s romal
a rophy ha resul s rom he in raocular
inf amma ion may cause he iris in he a ec ed
eye o appear ligh er in color (Fig. 14-10B)
or may cause only a f at ening o iris de ails
and a mo h-ea en appearance.4 In pa ien s
wi h ligh irides, however, he s romal a rophy
will cause he a ec ed eye o appear darker in
color because o he exposure o he iris pig-
men epi helium.
Ano her impor an diagnos ic nding
in pa ien s wi h Fuchs’ he erochromic
iridocycli is is he iden i ca ion o iris
neovasculariza ion or neovasculariza ion o
 228 14 UVEITIC GLAUCO MAS
he angle by gonioscopy. Despi e he chro-
nici y o he in raocular inf amma ion in
hese pa ien s, peripheral an erior synechiae
and pos erior synechiae almos never orm.
However, pos erior subcapsular ca arac is
very common.
Laboratory Studies
T ere are no labora ory s udies ha allow
or he diagnosis o Fuchs’ he erochromic
iridocycli is. Lymphocy es and plasma cells
have been iden i ed in aqueous humor rom
a ec ed pa ien s; he an ibody index o
rubella virus may be eleva ed i checked, bu
his is generally unnecessary.5
T e diagnosis is made clinically based on
he dis ribu ion o he kera ic precipi a es,
he low-grade an erior uvei is ypically
unresponsive o s eroids, iris he erochromia,
absence o synechiae, and he lack o ocular
symp oms.
COURSE
T e an erior uvei is in Fuchs’ he ero-
chromic iridocycli is is insidious and slowly
progressive. T e neovasculariza ion o he iris
and angle may cause mild in raocular hemor-
rhage spon aneously or wi h minor rauma
bu does no cause peripheral an erior syn-
echiae or neovascular glaucoma.
Ca arac and glaucoma are he mos com-
mon complica ions.
Ca arac orma ion has been repor ed in
more han 80% o pa ien s wi h he condi-
ion.4 Ca arac ex rac ion, when required,
is generally uncomplica ed and is less likely
o be complica ed by he pos opera ive
inf amma ion ha is charac eris ic o o her
ypes o uvei is.6 Pos erior lens implan a-
ion is considered o be sa e.
T e glaucoma associa ed wi h Fuchs’
he erochromic iridocycli is closely
resembles he course o primary open-
angle glaucoma.
MANAGEMENT
Despi e he presence o a chronic an erior
uvei is, aggressive opical herapy wi h cor i-
cos eroids and he use o sys emic immuno-
suppressive herapy are no recommended
or he rea men o Fuchs’ he erochromic
iridocycli is because o he poor response o
rea men . In ac , he use o opical s eroids
may be con raindica ed because hey may
accelera e he developmen o ca arac and
glaucoma. Medical herapy is recommended
or he con rol o he glaucoma; however, as
many as 66% o pa ien s may require surgical
managemen .7
T e bes surgical procedure or pa ien s
wi h Fuchs’ he erochromic iridocycli is is
unknown. Argon laser rabeculoplas y is no
e ec ive because o he orma ion o a hyaline
membrane over he rabecular meshwork and
should no be used.
EFE ENCES
1. O’Connor G . Doyne lec ure. He erochromic iridocy-
cli is. rans Ophthalmol Soc U K. 1985;104:219–231.
2. Bloch-Michel E. Physiopa hology o Fuchs’s he ero-
chromic cycli is. rans Ophthalmol Soc U K. 1981;
101:384–386.
3. Birnbaum AD, Lit le DM, essler HH, e al. E iologies
o chronic an erior uvei is a a er iary re erral cen-
er over 35 years. Ocul Immunol Inf amm. 2011;
19(1):19–25.
4. Bon oli AA, Curi AL, Ore ce F. Fuchs’ he erochromic
cycli is. Semin Ophthalmol. 2005;20(3):143–146.
5. uokonen PC, Me zner S, Ücer A, e al. In raocular
an ibody syn hesis agains rubella virus and o her
microorganisms in Fuchs’ he erochromic cycli is.
Grae es Arch Clin Exp Ophthalmol. 2010;248:565–571.
6. ejwani S, Mur hy S, Sangwan V. Ca arac ex rac ion
ou comes in pa ien s wi h Fuchs’ he erochromic cycli-
is. J Cataract Re act Surg. 2006;32:1678–1682.
7. Liesegang J. Clinical ea ures and prognosis in
Fuchs’ uvei is syndrome. Arch Ophthalmol. 1982;
100:1622–1626.
 Specifc Entities 229

A B
FIGU E 14-10. Fuchs’ heterochromic iridocyclitis. A. Fuchs’ he erochromic iridocycli is is a unila eral,
nongranuloma ous an erior uvei is commonly charac erized by he riad o he erochromia, ca arac , and
glaucoma in he a ec ed eye. Pa ien s wi h his condi ion charac eris ically show s ella e kera ic precipi a es
dis ribu ed over he en ire corneal endo helium. B. T e iris he erochromia and ca arac in he le eye are a resul
o he chronic unila eral inf amma ion in he le eye.
 230 14 UVEITIC GLAUCO MAS

GLAUCO
G LAUU C O MAM A O C YCLI
YCC LII IC
IC HISTORY
CRISIS
C RISII S ( P
POSN
O SN E
ER–
R– Pa ien s have a his ory o recurring symp-
SCH
S CH L
LOSSMAN
O SS SM ANNS SYN
YN
N DRO
DRR O ME)
MEE) oms o mild ocular pain or discom or and
blurred vision wi hou ocular injec ion.

G laucoma ocycli ic crisis is a syndrome o

recurren episodes o mild, idiopa hic,
unila eral, nongranuloma ous an erior uve-
i is accompanied by marked eleva ion in he
in raocular pressure. Al hough his syndrome
was probably rs described in 1929, i carries
he eponym o Posner and Schlossman who
repor ed he syndrome in 1948.1
EPIDEMIOLOGY
Glaucoma ocycli ic crisis ypically occurs
in pa ien s be ween he ages o 20 and 50 years.
Al hough bila eral cases are repor ed, i is a
unila eral disease in he overwhelming major-
i y o cases.
ETIOLO GY
T e cause o glaucoma ocycli ic crisis is
unclear, bu s udies have implica ed cy omeg-
alovirus or herpes simplex virus (HSV) in a
leas some cases.2,3 T e increased in raocular
pressure is believed o resul rom an acu e
decrease in he aqueous ou f ow during he
at ack.
Pros aglandins have been demons ra ed o
play a role in he disease pa hogenesis, wi h
eleva ed levels in he aqueous humor correla -
ing wi h he increase in in raocular pressure
during an acu e at ack.4 Pros aglandins break
down he blood–aqueous barrier, resul ing in
an inf ux o pro eins and inf amma ory cells
ha can impair aqueous ou f ow and increase
he in raocular pressure.
Some pa ien s wi h glaucoma ocycli ic cri-
sis have abnormal aqueous humor dynamics
be ween episodes and may have an underly-
ing primary open-angle glaucoma.
Some pa ien s may also describe halos ha
may indica e he presence o corneal edema.
DIFFERENTIAL DIAGNOSIS
D iseases o be considered in he di eren-
ial diagnosis o glaucoma ocycli ic cri-
sis include Fuchs’ he erochromic iridocycli is,
herpes simplex or zos er uvei is, sarcoidosis,
HLA-B27–associa ed an erior uvei is, and
idiopa hic an erior uvei is.
DIAGNOSTIC EVALUATION
Ophthalmic Examination
Ex ernal ocular examina ion is requen ly
normal.
An erior segmen examina ion ypically
reveals ew kera ic precipi a es dis ribu ed
over he in erior corneal endo helium. In
some cases, par icularly i he in raocular
pressure is su cien ly eleva ed, he cornea
may show microcys ic edema.
Kera ic precipi a es may occasionally be
seen on gonioscopy, sugges ing he presence
o a rabeculi is.
T e an erior chamber charac eris ically
shows only mild aqueous cells and f are.
I pressure is signi can ly eleva ed, he
pupil may be sligh ly dila ed; however,
peripheral an erior synechiae and pos erior
synechiae do no occur.
In requen ly, he erochromia may be no ed
as a resul o s romal a rophy caused by he
recurren unila eral inf amma ory at acks.
T e in raocular pressure is generally much
grea er han would be expec ed or he degree
 Glaucomatocyclitic Crisis (Posner–Schlossman Syndrome)
o in raocular inf amma ion, ypically measur-
ing grea er han 30 mm Hg, o en in he 40 o
60 mm Hg range.
T e undus examina ion is ypically
normal.
Laboratory Studies
Glaucoma ocycli ic crisis is a clinical diag-
nosis, and here are no labora ory s udies ha
are speci c or he diagnosis.
COURSE
Posner–Schlossman syndrome is a sel -
limi ed ocular hyper ension ha resolves
spon aneously regardless o rea men .
T e recurren inf amma ory at acks end
o occur a in ervals o a ew mon hs o years
and may las rom several hours o a ew
weeks be ore spon aneously resolving.
T e developmen o op ic nerve damage
and visual eld de ec s in glaucoma ocycli ic
crisis may occur as a resul o he repea ed
bou s o ex remely eleva ed in raocular pres-
sure superimposed on an underlying primary
open-angle glaucoma.5
MANAGEMENT
Posner–Schlossman syndrome is rea ed
ini ially wi h opical cor icos eroids o con rol
he an erior uvei is.
I he in raocular pressure does no
respond o opical an i-inf amma ory herapy,
an iglaucoma medica ions may be required
231
o lower he in raocular pressure. Mydria ic
and cycloplegic agen s are no commonly
needed as ciliary muscle spasm is uncommon
and synechiae rarely orm. Oral indome ha-
cin, 75 o 150 mg daily—a pros aglandin
an agonis —has been repor ed o lower he
in raocular pressure in pa ien s wi h glau-
coma ocycli ic crises as er han s andard
an iglaucoma medica ions.4 opical nons e-
roidal an i-inf amma ory medica ions migh
likewise be an e ec ive rea men op ion or
pa ien s wi h ocular hyper ension, bu evi-
dence is lacking o suppor his.
Mio ics and argon laser rabeculoplas y
are generally no e ec ive. Be ween at acks,
prophylac ic an i-inf amma ory herapy is no
required.
Surgical l ra ion procedures are rarely
required and, i per ormed, do no preven
he recurren inf amma ory at acks.
EFE ENCES
1. Moor hy S, Mermoud A, Baerveld G, e al.
Glaucoma associa ed wi h uvei is. Surv Ophthalmol.
1997;41:361–394.
2. Chee SP, Bacsal K, Jap A, e al. Clinical ea ures o cy o-
megalovirus an erior uvei is in immunocompe en
pa ien s. Am J Ophthalmol. 2008;145(5):834–840.
3. Yamamo o S, Pavan-Langs on D, ada , e al.
Possible role o herpes simplex virus in he origin o
Posner-Schlossman syndrome. Am J Ophthalmol.
1995;119(6):796–798.
4. Masuda K, Izawa Y, Mishima SS. Pros aglandins
and glaucoma o-cycli is crisis. Jpn J Ophthalmol.
1975;19:368.
5. Kass MA, Becker B, Kolker AE. Glaucoma ocycli ic
crisis and primary open-angle glaucoma. Am J
Ophthalmol. 1973;75:668–673.
 232 14 UVEITIC GLAUCO MAS
HEERPE
R P E IIC
C ETIOLO GY
KERA
K ERA O U UVEI
VEI IS
S I remains unclear whe her he uvei is
I n he eye, in ec ion wi h he HSV can mani-
es as several dis inc , recurren , unila eral
ocular diseases such as blepharoconjunc ivi is,
epi helial kera i is, s romal kera i is, and uve-
i is. Al hough ocular involvemen may occur
wi h primary herpes zos er in ec ion (chicken-
pox), i more commonly accompanies herpes
zos er oph halmicus, a reac iva ion o herpes
zos er in older adul s a ec ing he dis ribu ion
o he oph halmic branch o cranial nerve V.
Uvei is associa ed wi h bo h HSV and herpes
zos er in ec ions ypically ollows previous
episodes o kera i is and accoun s or abou
5% o all uvei is cases seen in adul s.1 Eleva ed
in raocular pressure ha can progress o a sec-
ondary glaucoma is a prominen ea ure o
recurren herpe ic uvei is.
EPIDEMIOLOGY
Approxima ely 0.15% o he Uni ed S a es
popula ion has a his ory o ex ernal HSV
in ec ion.2
S romal kera i is and uvei is, which oge her
accoun or he grea es visual morbidi y rom
all orms o recurren herpes simplex ocular
disease, develop in ewer han 10% o pa ien s
wi h primary ocular herpes simplex in ec ion.1
T e incidence o herpes zos er has been
increasing, and ocular involvemen occurs
in wo- hirds o all cases o herpes zos er
oph halmicus.3 Uvei is and ocular hyper en-
sion in pa ien s wi h zos er may be associa ed
wi h ei her epi helial or s romal kera i is. T e
incidence o increased in raocular pressure in
pa ien s wi h herpe ic uvei is varies rom 28%
o 40%.4
T e incidence o secondary glaucoma
in pa ien s wi h herpes simplex uvei is and
herpes zos er uvei is is abou 10% and 16%,
respec ively.4,5
associa ed wi h herpes simplex kera i is is
a secondary inf amma ory response o he
corneal disease or whe her i is induced by
invasion o he virus in o he an erior uvea.
T e eleva ed in raocular pressure in herpes
simplex and herpes zos er uvei is is he resul
o normal aqueous secre ion in eyes wi h
impaired ou f ow resul ing rom rabecu-
li is, direc inf amma ion o he rabecular
meshwork. In herpes zos er uvei is, ischemia
resul ing rom an occlusive vasculi is may also
con ribu e o he increased in raocular pres-
sure.6 HSV has been cul ured rom he an e-
rior chamber o pa ien s wi h herpe ic uvei is,
and i s presence is posi ively correla ed wi h
ocular hyper ension.
Prolonged s eroid use may also con ribu e
o ocular pressure in pa ien s wi h herpe ic
uvei is.
HISTORY
Pa ien s wi h herpe ic uvei is ypically
presen wi h a complain o unila eral ocu-
lar redness, pain, pho ophobia, and, o en,
decreased vision.
A prior his ory o recurren kera i is is
commonly given.
Pa ien s wi h uvei is rela ed o herpes zos-
er are generally older and repor a his ory o
herpes zos er oph halmicus. Ocular disease
rela ed o HSV is rarely bila eral, whereas
herpes zos er oph halmicus only occurs
unila erally.
DIFFERENTIAL DIAGNOSIS
T e di eren ial diagnosis or herpe ic
uvei is includes Fuchs’ he erochromic iri-
docycli is, glaucoma ocycli ic crisis, and
sarcoidosis.

T e presence o corneal hypoes hesia
may be help ul in he diagnosis o herpe ic
uvei is.
DIAGNOSTIC EVALUATION
Ophthalmic Examination
Ex ernal ocular examina ion may reveal
evidence o previous cu aneous lesions
wi h herpes zos er and conjunc ival injec ion
and ciliary f ush in ei her ype o herpe ic
disease.
Corneal sensa ion is o en decreased in he
a ec ed eye.
T e cornea in pa ien s wi h herpes
kera ouvei is may show a range o ndings
rela ed o he prior epi helial or s romal dis-
ease, including epi helial dendri es, ghos
dendri es, ac ive disci orm or necro izing
s romal kera i is, neovasculariza ion, or
scarring.
Di use, nongranuloma ous, s ella e
kera ic precipi a es or granuloma ous precipi-
a es can be ound in bo h orms o herpe ic
uvei is. In severe cases o herpe ic uvei is,
pos erior synechiae and angle closure may be
ound.
Iris a rophy is a charac eris ic nding in
uvei is resul ing rom bo h herpes simplex
and zos er and may be pa chy or segmen al
(Fig. 14-11).
In pa ien s wi h herpes zos er, he iris a ro-
phy may be he resul o an occlusive vasculi-
is in he iris s roma.
LABORATORY STUDIES
T e diagnosis o herpe ic uvei is is clini-
cal and does no rou inely rely on labora ory
es ing.
Viral serologies or HSV and varicella zos-
er virus, i nega ive, exclude he diagnosis.
Herpetic Keratouveitis 233
T e de ec ion o viral DNA in he aqueous
by polymerase chain reac ion is suppor ive
bu no diagnos ic o herpe ic uvei is.
COURSE
Similar o he o her ocular mani es a ions
o herpes eye disease, he uvei is is recurren
and may or may no be associa ed wi h recur-
ren kera i is.
Eleva ed in raocular pressure is ypically
presen during he course o he in raocular
inf amma ion and may normalize or remain
eleva ed a er he uvei is has subsided.
Approxima ely 12% o pa ien s will have
persis en ly eleva ed in raocular pressure ha
will require an iglaucoma herapy or l ering
surgery.4
MANAGEMENT
Uvei is associa ed wi h HSV and her-
pes zos er should be rea ed wi h opical
cor icos eroids.
Cycloplegic agen s may also be necessary
i here is ocular pain resul ing rom ciliary
spasm. An an iviral agen should be used wi h
he opical s eroids o lessen he likelihood o
a reac iva ion o he epi helial kera i is.
Oral acyclovir or valacyclovir in pa ien s
wi h herpes zos er oph halmicus has been
ound o ameliora e he incidence and sever-
i y o dendri ic kera i is, s romal kera i is, and
uvei is.7
Pa ien s wi h increased in raocular
pressure should be rea ed as needed wi h
an iglaucoma herapy, hough con rol o
inf amma ion o en leads o resolu ion o ocu-
lar hypo ension.
Fil ra ion surgery may occasionally be
required. Argon laser rabeculoplas y is no
considered e ec ive in he managemen o
herpe ic uvei is.
 234 14 UVEITIC GLAUCO MAS

EFE ENCES 4. Falcon MG, Williams HP. Herpes simplex kera-

1. Barron BA, Gee L, Hauck WW, e al. Herpe ic Eye
Disease S udy. A con rolled rial o oral acyclovir
or herpes simplex s romal kera i is. Ophthalmology.
1994;101:1871–1882.
2. Parrish CM. Herpes simplex virus eye disease. In:
Focal Points: Clinical Modules or Ophthalmologists.
Vol. 15. San Francisco, CA: American Academy o
Oph halmology; 1997:2.
3. Leung J, Harpaz , Molinari NA, e al. Herpes zos er
incidence among insured persons in he Uni ed S a es,
1993–2006: Evalua ion o impac o varicella vaccina-
ion. Clin In ect Dis. 2011;52(3):332–340.
ouvei is and glaucoma. rans Ophthalmol Soc UK.
1978;98:101–104.
5. Panek WC, Holland GN, Lee DA, e al. Glaucoma
in pa ien s wi h uvei is. Br J Ophthalmol. 1990;
74:223–227.
6. Johns KJ, O’Day DM, Webb R , e al. An erior seg-
men ischemia in chronic herpes simplex kera ouvei is.
Curr Eye Res. 1991;10(suppl):117–124.
7. Sanjay S, Huang P, Lavanya . Herpes zos er oph-
halmicus. Curr reat Options Neurol. 2011;13(1):
79–91.

A B
FIGU E 14-11. Herpetic uveitis due to herpes simplex. In pa ien s presen ing wi h unila eral an erior uvei is
and eleva ed in raocular pressure, assessmen o corneal sensa ion and ransillumina ion o he pupil are help ul
in making a clinical diagnosis o herpe ic uvei is. Di use illumina ion o he iris (A) does no reveal he pa chy
a rophy o he iris s oma seen on ransillumina ion (B). A er he pa ien s ar ed oral acyclovir, he was able o
discon inue opical an iglaucoma herapy.

SYPH
S YPH
H ILI IC
C IN
N ERS
ER
R S I IAL
IA
AL
KERA
K ERA
A I IIS
S
O cular syphilis may occur congeni ally or
may be acquired by sexual ransmission.
Congeni al syphilis mainly a ec s he an erior
segmen o he eye, causing in ers i ial kera i is
and an erior uvei is, whereas acquired syphi-
lis more requen ly causes bo h an erior and
pos erior uvei is. Wi h he adven o e ec ive
diagnos ic es s and an ibio ic herapy, syphi-
li ic in ers i ial kera i is and secondary glau-
coma have become rare.
EPIDEMIOLOGY
Ocular involvemen in bo h congeni al
and acquired syphilis can be associa ed wi h
increased in raocular pressure and second-
ary glaucoma ha may occur during he
ac ive inf amma ory s age or many years a er
he in raocular inf amma ion has become
quiescen .
Secondary glaucoma has been repor ed in
15% o 20% o adul s wi h a his ory o in er-
s i ial kera i is caused by congeni al syphilis.1
Secondary glaucoma is less common in
pa ien s wi h acquired syphilis.
ETIOLO GY
T e pa hologic mechanism o he
increased in raocular pressure during he
ac ive s age o he disease is likely obs ruc ion
o he aqueous ou f ow by inf amma ory cells
and aqueous pro eins.
Synechiae orma ion, ocular maldevelop-
men , and lens subluxa ion may con ribu e
o he developmen o narrow-angle or angle-
closure glaucoma.
Endo helializa ion o he angle demon-
s ra ed by his opa hology is believed o be he
Syphilitic Interstitial Keratitis 235
underlying mechanism o he la e-onse glau-
coma in pa ien s wi h congeni al syphilis.
HISTORY
Pa ien s wi h ocular disease resul ing rom
congeni al syphilis ypically presen in he
rs or second decade o li e wi h acu e symp-
oms o ocular pain, pho ophobia, earing,
and decreased vision.
T e condi ion is bila eral in 90% o cases.
Nonocular signs o congeni al syphilis
ha may be presen include den al de ormi-
ies such as no ched incisors and mulberry
molars; skele al abnormali ies including a
saddle nose, pala al per ora ion, saber shins,
and ron al bossing; dea ness; rhagades; and
men al re arda ion.2
Pa ien s wi h acquired ocular syphilis
are more likely o presen wi h unila eral
symp oms.
DIFFERENTIAL DIAGNOSIS
T e di eren ial diagnosis or he ac ive
s age o ocular syphilis charac erized by in er-
s i ial kera i is and an erior uvei is includes
diseases such as herpes simplex and zos er
in ec ions, Mycobacterium tuberculosis and
leprae, Lyme disease, rubeola (measles),
Eps ein–Barr virus (in ec ious mononucleo-
sis), leishmaniasis and onchocerciasis, sar-
coidosis, and Cogan’s syndrome.
DIAGNOSTIC EVALUATION
Ophthalmic Examination
Ocular examina ion o pa ien s wi h con-
geni al syphilis may show a varie y o ndings,
including acu e and chronic an erior uvei is,
ca arac s, choriore ini is, re inal vasculi is, op ic
neuri is, and scleri is. O hese, in ers i ial kera-
i is is he mos charac eris ic mani es a ion.
 236 14 UVEITIC GLAUCO MAS
T e cornea in pa ien s wi h ac ive in ers i-
ial kera i is ypically shows sec oral edema,
opaci ca ion, and deep s romal vasculariza-
ion ha may be so pronounced as o give he
cornea a pink salmon pa ch appearance.3
An erior uvei is commonly accompanies
syphili ic in ers i ial kera i is and is o en
associa ed wi h eleva ed in raocular pressure.
Ocular ndings in pa ien s wi h acquired
syphilis requen ly include an erior uvei is,
choriore ini is, and op ic neuri is.
In ers i ial kera i is rarely develops in
pa ien s wi h acquired syphilis and when i
occurs is ypically unila eral.
Nodular iris lesions o en accompany he
an erior uvei is in pa ien s wi h acquired
syphilis.4
Laboratory Studies
Ocular syphilis is diagnosed on he basis o
a posi ive serology. T e non reponemal es s
such as he Venereal Disease esearch Lab
(VD L) or he rapid plasma reagin ( P )
alone are insu cien and a reponemal es ,
such as he f uorescen reponemal an ibody
absorp ion (F A-ABS) es or he micro-
hemagglu ina ion es , reponema pallidum
(MHA- P), mus be ob ained.
Any pa ien wi h syphili ic uvei is should
have a spinal f uid examina ion o rule ou
asymp oma ic neurosyphilis.
COURSE
T e in ers i ial kera i is and an erior
uvei is ypically persis or several weeks o
mon hs be ore spon aneously resolving, leav-
ing ghos vessels in he deep corneal s roma.
Glaucoma is a la e complica ion o
pa ien s wi h congeni al syphilis ha ypically
develops in eyes wi hou evidence o ongoing
in raocular inf amma ion decades a er he
in ers i ial kera i is has subsided.
Bo h open-angle and narrow-angle glau-
coma have been described in hese pa ien s
wi h equal requency.
MANAGEMENT
During he ac ive s age o he disease,
managemen o he increased in raocular
pressure relies on opical cor icos eroids,
cycloplegic agen s, and an iglaucoma medica-
ions as needed.
Sys emic syphilis should be rea ed wi h
an appropria e an ibio ic course; when a ec -
ing he eyes, i should be rea ed as would be
neurosyphilis.
Laser irido omy or surgical iridec omy
should be per ormed in pa ien s wi h narrow-
angle or closed-angle glaucoma.
Pa ien s wi h la e-onse open-angle glau-
coma are less responsive o an iglaucoma
medica ions and may require a l ering
procedure.
Argon laser rabeculoplas y is o lit le ben-
e because o he angle endo helializa ion.
EFE ENCES
1. Lich er P , Sha er N. In ers i ial kera i is and glau-
coma. Am J Ophthalmol. 1969;68:241–248.
2. Woods C . Congeni al syphilis – persis ing pes ilence.
Pediatr In ect Dis J. 2009;28(6):536–537.
3. Lee ME, Lindquis D. Syphili ic in ers i ial kera i is.
JAMA. 1989;262(20):2921.
4. Aldave AJ, King JA, Cunningham E Jr. Ocular syphi-
lis. Curr Opin Ophthalmol. 2001;12(6):433–441.

JJUVEN
UVVE N ILE
I L E IDIO
O PA
PA H IC
C ETIOLO GY
AR
A R H RI
R I IS
J uvenile idiopa hic ar hri is ( JIA), or-
merly known in he Uni ed S a es as juve-
nile rheuma oid ar hri is, is a common cause
o pedia ric uvei is o en complica ed by
increased in raocular pressure and glaucoma.
T ree sub ypes o JIA wi h di eren risks or
he developmen o uvei is can be diagnosed
based on he ex en o ar icular and sys emic
involvemen wi hin he rs 3 mon hs o pre-
sen a ion. Sys emic-onse JIA, or S ill’s dis-
ease, is commonly seen in boys younger han
4 years o age and is an acu e sys emic disease
consis ing o a cu aneous rash, ever, poly-
ar hri is, hepa osplenomegaly, leukocy osis,
and polyserosi is. Young girls more commonly
presen wi h he oligoar icular ( ewer han ve
join s, also known as pauciar icular) and poly-
ar icular ( ve or more join s) orms o JIA ha
lack he sys emic ea ures.
EPIDEMIOLOGY
T e incidence o uvei is in JIA varies rom
2% o 30%, depending on he sub ype.1,2
Uvei is is ypically no associa ed wi h
S ill’s disease or sys emic-onse JIA.
An erior uvei is is more common in
pa ien s wi h he oligoar icular orm (19%
o 29%) han in hose wi h he polyar icular
orm (2% o 5%) o JIA.3,4
Children wi h he oligoar icular or mono-
ar icular onse o join involvemen accoun
or more han 90% o he JIA pa ien s wi h
uvei is.
Secondary glaucoma is seen in
approxima ely 14% o 22% o pa ien s
wi h chronic an erior uvei is associa ed
wi h JIA.1
Juvenile Idiopathic Arthritis 237
T e developmen o increased in raocular
pressure and glaucoma in pa ien s wi h JIA is
mos o en caused by progressive angle clo-
sure as a resul o synechiae.
Open-angle glaucoma also occurs and may
be he resul o chronic inf amma ory dam-
age o he rabecular meshwork or s eroid-
induced glaucoma resul ing rom prolonged
opical s eroid rea men .
HISTORY
Al hough ar hri is develops rs in he
majori y o cases, JIA-associa ed uvei is may
be he presen ing sign.
Because he an erior uvei is associa ed wi h
JIA is mild, asymp oma ic, and rarely causes
ocular redness, he disease may go unno iced
or a long period o ime un il visual loss, ca a-
rac , or an irregular pupil is no ed.
T e uvei is in pa ien s wi h JIA is bila eral
in almos all cases.
DIFFERENTIAL DIAGNOSIS
T e di eren ial diagnosis or chronic an e-
rior uvei is in children includes sarcoidosis,
pars plani is, HLA-B27–associa ed diseases,
and idiopa hic an erior uvei is.
DIAGNOSTIC EVALUATION
Ophthalmic Examination
Band kera opa hy is a ound in up o 50%
o children wi h an erior uvei is, and i s pres-
ence is hough o be associa ed wi h he
chronici y o he disease.1
T e an erior uvei is in pa ien s wi h JIA is
nongranuloma ous in he vas majori y o cases.
 238 14 UVEITIC GLAUCO MAS
Kera ic precipi a es are generally dis rib-
u ed over he in erior hal o he cornea.
Mio ic pupils resul ing rom pos erior
synechiae or pupillary membranes, iris
bombé, and peripheral an erior synechiae are
requen ndings in a ec ed pa ien s ha can
con ribu e o he developmen o glaucoma.
An erior and pos erior subcapsular ca a-
rac s are presen in up o one- hird o a ec ed
pa ien s. Pos erior segmen examina ion in
pa ien s wi h JIA may show papilli is and cys-
oid macular edema, which can con ribu e o
visual loss.
Laboratory Studies
Up o 80% o pa ien s wi h an erior uvei is
and JIA are an inuclear an ibody posi ive and
rheuma oid ac or nega ive.
COURSE
T e uvei is associa ed wi h JIA is a chronic
disease ha is di cul o con rol despi e
rea men .
In pa ien s wi h JIA, here is no direc
correla ion be ween he ac ivi y o he ocular
disease and he join disease.
T e incidence o secondary complica-
ions, such as band kera opa hy, ca arac , and
glaucoma, increases wi h he dura ion o he
disease.
T e prognosis or children wi h uvei ic glau-
coma, previously considered poor, is improv-
ing wi h more e ec ive surgical managemen .
MANAGEMENT
T e ini ial rea men approach or he
managemen o in raocular inf amma ion in
pa ien s wi h JIA includes opical cor icos e-
roids and cycloplegic agen s o preven he
orma ion o synechiae.
O en, periocular s eroid injec ions and
even sys emic s eroids are necessary o con-
rol an erior uvei is. Oral nons eroidal agen s
are also used in JIA pa ien s. Me ho rexa e
alone or in combina ion wi h o her immu-
nosuppressive agen s such as prednisone or
cyclosporine has been used o rea he ocular
and join mani es a ions o JIA. Newer bio-
logic agen s such as inf iximab ( emicade),
adalimumab (Humira), and aba acep
(Orencia), shown o be o bene or he join
disease in JIA, are curren ly being evalua ed
or heir e cacy in he rea men o uvei is in
hese pa ien s.
Eleva ed in raocular pressure in JIA
is rea ed ini ially wi h an iglaucoma
medica ions. Medical managemen is
ini ially e ec ive in only abou 50% o JIA
pa ien s, wi h only 30% o pa ien s being
con rolled medically over he long erm.4
Laser irido omy or surgical iridec omy
may be necessary o relieve he pupillary
block in pa ien s wi h pos erior synechiae.
Surgical managemen is required
or pa ien s who are unresponsive o
medical herapy. o increase he likelihood
o a good ou come, i possible, surgical
in erven ion should be de erred un il he
in raocular inf amma ion has been ade
qua ely con rolled or a period o a leas
3 mon hs.
Opera ive procedures mos commonly
used in children wi h JIA include
rabeculec omy and ube shun s (see
Fig. 14-8).
Improved success has been repor ed wi h
he use o an ime aboli es in pa ien s under-
going rabeculec omy.1
rabeculodialysis in a small case series has
been shown o be sa e and e ec ive or con-
rolling he pressure in pa ien s wi h JIA or
up o 2 years.5

EFE ENCES
1. Moor hy S, Mermoud A, Baerveld G, e al.
Glaucoma associa ed wi h uvei is. Surv Ophthalmol.
1997;41:361–394.
2. avelli A, Felici E, Magni-Manzoni S, e al. Pa ien s
wi h an inuclear an ibody-posi ive juvenile idiopa hic
ar hri is cons i u e a homogeneous subgroup irrespec-
ive o he course o join disease. Arthritis Rheum.
2005;52:826–832.
Juvenile Idiopathic Arthritis 239
3. Calabro JJ, Parrino G , A choo PD, e al. Chronic
iridocycli is in juvenile rheuma oid ar hri is. Arthritis
Rheum. 1970;13:406–413.
4. O’Brien JM, Alber DM. T erapeu ic approaches or
oph halmic problems in juvenile rheuma oid ar hri is.
Rheum Dis Clin North Am. 1989;15:413–437.
5. Kanski JJ, McAllis er JA. rabeculodialysis or inf am-
ma ory glaucoma in children and young adul s.
Ophthalmology. 1985;92:927–930.
 240 14 UVEITIC GLAUCO MAS

and he developmen o an inf amma ory

LENS-IN
L EN
N S-IN
N DUCED
DUCC ED U
UVEI
VEII IS
IS
AN
A N D GL
GLAUCO
L AU CO MA
A Phacoly ic glaucoma ypically seen in older
T he libera ion o lens pro eins hrough an
in ac or disrup ed lens capsule in o he
an erior chamber or vi reous cavi y can rig-
ger a severe in raocular inf amma ory reac ion
ha may impair aqueous ou f ow and cause
an acu e eleva ion in he in raocular pressure
or glaucoma. Leakage o lens pro eins is ypi-
cally he resul o acciden al or surgical rauma
o he lens capsule or may be associa ed wi h
ca arac progression. Clinical en i ies charac-
erized by lens-induced uvei is and glaucoma
include phacoan igenic uvei is, phacoly ic
glaucoma, lens par icle glaucoma, and pha-
comorphic glaucoma. Uvei is and glaucoma
may also be a complica ion o in raocular lens
placemen .1
EPIDEMIOLOGY
Al hough he condi ion is well described,
he exac incidence o glaucoma due o
various orms o lens-induced uvei is is no
known.
In one s udy o pa ien s wi h phaco-
anaphylac ic uvei is (phacoan igenic uve-
i is), 17% o pa ien s were diagnosed wi h
glaucoma.2
HISTORY
Phacoan igenic uvei is, also re erred o as
phacoanaphylac ic uvei is or phacoanaphy-
lac ic endoph halmi is, is a granuloma ous
uvei is ini ia ed by he release o lens pro eins
hough a rup ured lens capsule.
T e onse o he inf amma ion is days o
weeks a er he rauma ic or surgical injury o
he lens. Pa ien s presen wi h a red, pain ul eye.
arely, phacoan igenic uvei is is
associa ed wi h sympa he ic oph halmia
reac ion in he ellow eye.3
pa ien s arises when lens pro eins leak rom
a ma ure or hyperma ure ca arac hrough an
in ac bu permeable lens capsule. Pa ien s wi h
phacoly ic glaucoma commonly presen wi h
he abrup onse o ocular pain and redness in a
poorly seeing eye wi h a known ca arac .
Lens par icle glaucoma, also known as
phaco oxic uvei is, occurs ollowing any ocu-
lar injury ha resul s in he libera ion o lens
cor ical ma erial in o he an erior chamber. In
mos cases, he onse o increased in raocular
pressure is de ec ed days or weeks a er he
inci ing injury.
In cases o phacomorphic glaucoma, he
lens capsule is no ypically viola ed and
he eye generally does no show signi can
in raocular inf amma ion. Pa ien s ypically
presen wi h a redness and pain rom angle
closure in an eye wi h decreased vision caused
by a ca arac .
T e uvei is–glaucoma–hyphema (UGH)
syndrome was a requen cause o pos opera-
ive in raocular inf amma ion and glaucoma
in pa ien s ollowing implan a ion wi h he
rs genera ion o rigid, an erior chamber
in raocular lenses. T e syndrome was at rib-
u ed o poor in raocular lens size selec ion
or manu ac uring de ec s in he lens ma e-
rial, causing mechanical irri a ion o an erior
chamber s ruc ures.
Chronic or severe pos opera ive inf amma-
ion can also resul in pseudophakic inf am-
ma ory glaucoma in pa ien s wi h pos erior
chamber in raocular lens implan s.
ETIOLO GY
Obs ruc ion o aqueous ou f ow a he level
o he rabecular meshwork is he common
mechanism in he lens-induced glaucomas.

In phacoan igenic uvei is, here is a
granuloma ous inf amma ory response
o he elabora ed lens pro eins ha can
induce he orma ion o synechiae and
blockage o he rabecular meshwork.
In phacoly ic glaucoma, he released
lens pro eins and macrophages engorged
wi h lens pro eins obs ruc he rabecular
meshwork, whereas in lens par icle glau-
coma, i is he ac ual ragmen s o lens cor-
ical ma erial ha are believed o injure he
rabecular meshwork.
Unlike he o her lens-induced glaucomas,
in which he an erior chamber angle is ypi-
cally open, in phacomorphic glaucoma he
in umescen lens can cause pupillary block
or disloca e he iris orward, resul ing in a
shallowed an erior chamber or acu e angle
closure. In pseudophakic eyes, in raocular
inf amma ion may be he resul o a preexis -
ing uvei is, a delayed-onse pos surgical endo-
ph halmi is, or irri a ion o he uveal issue
by he in raocular lens. Glaucoma may arise
because o damage o he rabecular mesh-
work or orma ion o synechiae on he lens
implan , causing pupillary block, and periph-
eral an erior synechiae orma ion, resul ing in
angle closure.
DIFFERENTIAL DIAGNOSIS
T e main di eren ial diagnoses or phaco-
an igenic and lens par icle glaucoma are pos -
rauma ic and pos surgical endoph halmi is.
O her causes o acu e angle closure should
be considered in pa ien s wi h phacomorphic
glaucoma.
DIAGNOSTIC EVALUATION
Ophthalmic Examination
Ex ernal examina ion o pa ien s wi h
acu e lens-induced uvei is and glaucoma
Lens-induced Uveitis and Glaucoma 241
commonly reveals conjunc ival injec ion and
ciliary f ush. T ere may also be ex ernal evi-
dence o a prior ocular injury.
I he pressure is signi can ly eleva ed, he
cornea is o en edema ous.
T e an erior chamber ypically con-
ains an erior chamber cells and f are, wi h
granuloma ous or nongranuloma ous kera ic
precipi a es. Whi e, f occulen ma erial and
ragmen s o lens cor ex may be seen circula -
ing wi hin he aqueous and in he an erior
chamber angle, which may be open, nar-
rowed, or closed. Peripheral an erior and pos-
erior synechiae are no uncommon.
In cases o phacoan igenic uvei is and
lens par icle glaucoma, evidence o injury o
he na ive lens or re ained lens ma erial can
usually be ound. In cases o phacoly ic and
phacomorphic glaucoma, examina ion reveals
a hyperma ure or in umescen ca arac ,
respec ively, and in cases o pseudophakic
inf amma ory glaucoma, an in raocular lens is
presen .
Pos erior segmen examina ion may show
vi reous cells and haze, lens ma erial in he
vi reous cavi y, and o her ndings rela ed o
he ocular injury.
Laboratory Studies
T e diagnosis o lens-induced uvei is and
glaucoma is clinical and does no rely on labo-
ra ory es ing.
His opa hologic examina ion o he lens in
pa ien s wi h phacoan igenic uvei is reveals a
zonal granuloma ous inf amma ion cen ered
a he si e o lens injury.
COURSE
T e clinical course o he lens-induced
glaucomas ends o be rela ively brie because
hey are e ec ively managed wi h surgical
in erven ion.
 242 14 UVEITIC GLAUCO MAS
MANAGEMENT
Ca arac ex rac ion or removal o he
re ained lens ma erial or lens implan is he
de ni ive herapy or lens-induced uvei is and
glaucoma.
Prior o surgical in erven ion, he in raocu-
lar inf amma ion is rea ed wi h opical cor ico-
s eroids and he increased in raocular pressure
is con rolled wi h an iglaucoma medica ions.
In some pa ien s wi h uvei ic glaucoma
rela ed o an in raocular lens, medical man-
agemen may be adequa e, and he inf am-
ma ion may resolve over ime, avoiding he
need or surgery.
In pa ien s wi h phacomorphic glaucoma,
a er he in raocular pressure is lowered medi-
cally, laser irido omy can be used o empo-
rize he condi ion i ca arac ex rac ion needs
o be delayed or canno be per ormed.
EFE ENCES
1. Ellan JP, Obs baum SA. Lens-induced glaucoma. Doc
Ophthalmol. 1992;81(3):317–338.
2. T ach AB, Marak GE Jr, McLean IW, e al.
Phacoanaphylac ic endoph halmi is: A clinicopa ho-
logic review. Int Ophthalmol. 1991;15:271–279.
3. Allen JC. Sympa he ic uvei is and phacoanaphylaxis.
Am J Ophthalmol. 1967;63:280–283.

SARCO
S AR
R C O IDOSIS
ID
D O SIS
S wi h iris bombé.
S arcoidosis is a sys emic disease charac-
erized by noncasea ing, granuloma ous,
inf amma ory in l ra es a ec ing he lungs,
skin, liver, spleen, cen ral nervous sys em,
and eyes. Ocular disease occurs in 10% o
38% o pa ien s wi h sys emic sarcoidosis.3
Sarcoidosis, which can presen as an erior,
in ermedia e, pos erior, or panuvei is, is he
pro o ype or a chronic, granuloma ous uvei is.
EPIDEMIOLOGY
Sarcoidosis is 8 o 10 imes more requen
among A rican Americans han whi es, having
an es ima ed prevalence o 82 per 100,000 in
his popula ion.1
Al hough sarcoidosis can develop a any
age, he disease is ypically diagnosed in adul s
be ween 20 and 50 years o age. Sarcoidosis
accoun s or 5% o all uvei is cases in adul s
bu abou 1% o uvei is cases in children.2
Sarcoidosis involves he an erior segmen
in up o 70% o cases wi h ocular involve-
men , whereas he pos erior segmen is
a ec ed in less han 33% o cases.3
Secondary glaucoma occurs in approxi-
ma ely 11% o 25% o all pa ien s wi h
sarcoidosis and is more commonly a compli-
ca ion o he an erior segmen disease.4
A rican-American pa ien s wi h sarcoid-
osis have a higher incidence o uvei ic glau-
coma and blindness.
ETIOLO GY
Ocular hyper ension and glaucoma
in pa ien s wi h sarcoidosis resul s rom
obs ruc ion o he rabecular meshwork by
he chronic granuloma ous inf amma ion
and angle closure caused by he orma ion o
Sarcoidosis 243
peripheral an erior and pos erior synechiae
An erior segmen neovasculariza ion and
he prolonged use o s eroids can also con-
ribu e o he impairmen o aqueous ou f ow.
HISTORY
Mos adul pa ien s wi h sarcoidosis
presen wi h pulmonary involvemen ha
may mani es as cough, shor ness o brea h,
wheezing, or dyspnea on exer ion.
Ano her common presen a ion o sarcoid-
osis is wi h generalized symp oms such as
ever, a igue, and weigh loss.
Many pa ien s are asymp oma ic a he
ime o diagnosis.
Pa ien s wi h ocular involvemen ypically
presen wi h complain s o ocular pain, red-
ness, pho ophobia, f oa ers, and blurred or
decreased vision.
DIFFERENTIAL DIAGNOSIS
T e di eren ial diagnosis o sarcoidosis
includes he o her causes o granuloma ous
panuvei is such as he Vog –Koyanagi–
Harada syndrome, sympa he ic oph halmia,
and uberculosis.
Syphilis, Lyme disease, primary in raocu-
lar lymphoma, and pars plani is should also
be considered.
DIAGNOSTIC EVALUATION
Ophthalmic Examination
T e ocular disease o sarcoidosis is ypi-
cally bila eral, al hough i may be unila eral or
very asymme ric.
Mos requen ly a cause o granuloma ous
uvei is, sarcoidosis may also cause a nongran-
uloma ous uvei is.
 244 14 UVEITIC GLAUCO MAS
Oph halmic ndings in he an erior seg-
men include orbi al and cu aneous granulo-
mas, enlarged lacrimal glands, and palpebral
and bulbar conjunc ival nodules.
T e cornea mos commonly shows
large, mut on a kera ic precipi a es, wi h
nummular corneal in l ra es and in erior
areas o endo helial opaci ca ion being
no ed less requen ly.
Pos erior and peripheral an erior syn-
echiae, when ex ensive, resul in eleva ed
in raocular pressure or secondary uvei ic
glaucoma caused by angle closure or iris
bombé.
Koeppe and Busacca- ype iris nodules
are o en seen in he more severe cases o
an erior segmen disease (see Fig. 14-5).
Pos erior segmen involvemen in sarcoid-
osis occurs less requen ly han an erior seg-
men disease.
T e vi reous requen ly shows a vi ri-
is wi h vi reous snowballs and in erior
inf amma ory debris.
Examina ion o he pos erior pole
may reveal a varie y o ndings, including
peripheral re inal vasculi is, peripheral exu-
da es similar o snowbanks, hemorrhages,
re inal exuda es, and perivascular nodular
granuloma ous lesions, Dalen–Fuchs’ nod-
ules, and re inal, subre inal, or disc neo-
vasculariza ion. Granulomas in he re ina,
choroid, and op ic nerve may also be seen.
Visual loss in sarcoidosis is mos o en a
resul o cys oid macular edema, op ic neuri-
is caused by granuloma ous in l ra ion o he
op ic nerve, and secondary glaucoma.
Laboratory Studies
T e diagnosis o sarcoidosis is con rmed
wi h a issue biopsy showing noncasea ing or
nonnecro izing granulomas or granuloma ous
inf amma ion in a pa ien in whom all o her
causes o granuloma ous disease, such as
uberculosis and ungal in ec ions, have been
excluded.
An ini ial diagnos ic evalua ion or sar-
coidosis should include a ches x-ray and
serum angio ensin-conver ing enzyme (ACE)
level. Serum lysozyme levels may also be
eleva ed in pa ien s wi h sarcoidosis; his es
may have a bet er combina ion o sensi ivi y,
speci ci y, posi ive predic ive value, and nega-
ive predic ive value han ACE level.5
Addi ional s udies ha may be use ul in
con rming he diagnosis include anergy es -
ing, pulmonary unc ion es ing, gallium scan,
compu ed omographic scan o he horax,
bronchoalveolar lavage, and ransbronchial
biopsy.
Because ACE levels may be high in normal
children, serum ACE level is a less use ul
diagnos ic es or sarcoidosis in children.
Eleva ed ACE levels have been repor ed in
he aqueous humor and cerebrospinal f uids
o pa ien s wi h ocular and cen ral nervous
sys em sarcoid uvei is and neurosarcoidosis,
respec ively.
COURSE
T e clinical course o ocular sarcoidosis
can be acu e and sel -limi ed or chronic,
recurren , and relen less.
T e chronic orm o sarcoid uvei is has
he worse prognosis because o he onse o
complica ions such as glaucoma, ca arac , and
macular edema.
MANAGEMENT
T e mains ay o herapy or bo h sys emic
and ocular sarcoidosis is cor icos eroids.
An erior segmen disease may be managed
wi h opical or periocular cor icos eroid injec-
ions. Sys emic herapy is ypically required
or bila eral pos erior segmen uvei is. O her

immunosuppressive agen s such as me ho-
rexa e and inf iximab have demons ra ed
herapeu ic bene in he managemen o
sarcoidosis and should be considered early
or pa ien s wi h chronic sarcoidosis requiring
prolonged s eroid herapy.6,7 Cyclosporine
and e anercep do no appear o have bene
in he rea men o sarcoidosis.8–10
T e glaucoma should be rea ed medi-
cally wi h aqueous suppressan s or as long as
possible.
Argon laser rabeculoplas y is requen ly
ine ec ive.
Laser irido omy and surgical iridec omy
are he rea men s o choice or pa ien s wi h
pupillary block.
I he in raocular pressure remains uncon-
rolled, surgical in erven ion wi h ei her a
l ering procedure or a ube shun is required.
Surgical success is improved i he in ra-
ocular inf amma ory disease can be con rolled
prior o he surgical procedure.
10. U z JP, Limper AH, Kalra S, e al. E anercep or he
Sarcoidosis 245
EFE ENCES
1. Nussenblat B, Whi cup SM, Pales ine AG.
Sarcoidosis. In: Nussenblat B, Whi cup SM,
Pales ine AG, eds. Uveitis: Fundamentals and Clinical
Practice. S Louis, MO: Mosby; 1996:289–298.
2. Hoover DL, Khan JA, Giangiacomo J. Pedia ric ocu-
lar sarcoidosis. Surv Ophthalmol. 1986;30:215–228.
3. Jabs DA, Johns CJ. Ocular involvemen in chronic sar-
coidosis. Am J Ophthalmol. 1986;102:297–301.
4. Obenau CD, Shaw HE, Sydnor CF, e al. Sarcoidosis
and i s oph halmic mani es a ions. Am J Ophthalmol.
1978;86:648–655.
5. Herbor CP, ao NA, Mochizuki M; members o
Scien i c Commit ee o Firs In erna ional Workshop
on Ocular Sarcoidosis. In erna ional cri eria or he
diagnosis o ocular Sarcoidosis: esul s o he rs
In erna ional Workshop On Ocular Sarcoidosis
(IWOS). Ocul Immunol Inf amm. 2009;17(3):
160–169.
6. Chan ES, Crons ein BN. Molecular ac ion o me h-
o rexa e in inf amma ory diseases. Arthritis Res.
2002;4(4):266–273.
7. Baughman P, Dren M, Kavuru M, e al. Inf iximab
herapy in pa ien s wi h chronic sarcoidosis and
pulmonary involvemen . Am J Respir Crit Care Med.
2006;174(7):795–802.
8. Wyser CP, van Schalkwyk EM, Alhei B, e al.
rea men o progressive pulmonary sarcoidosis wi h
cyclosporine A: A randomized con rolled rial. Am J
Respir Crit Care Med. 1997;156(5):1371–1376.
9. Mar ine Y, Pinks on P, Sal ini C, e al. Evalua ion
o he in vi ro and in vivo e ec s o cyclosporine on
he lung -lymphocy e alveoli is o ac ive pulmo-
nary sarcoidosis. Am Rev Respir Dis. 1988;138(5):
1242–1248.
rea men o s age II and III progressive pulmonary
sarcoidosis. Chest. 2003;124(1):177–185.
 C H AP T ER

Lens-associa ed Open-angle
Glaucomas
Michele C. Lim and Ashley G. Lesley

L ens-associa ed open-angle glaucomas are

composed o hree separa e diagnoses wi h
similar clinical presen a ions. Lens pro ein
glaucoma, lens par icle glaucoma, and lens-
associa ed uvei is (LAU) may each presen
wi h in raocular in amma ion, an abnormal
lens, and eleva ed in raocular pressure (IOP),
al hough hypo ony may commonly occur in
he lat er. Dis inguishing among he hree
en i ies requires care ul examina ion and an
unders anding o he mechanisms ha def ne
each diagnosis (Table 15-1).

TABLE 15-1. Clinical Presentation o Lens associated Open angle Glaucomas

Lens Particle Glaucoma Lens Protein Glaucoma LAU
Mechanism Lens material obstructs HMW lens proteins obstruct Loss of immune tolerance
TM TM
IOP Elevated Elevated Low or elevated
Gonioscopy Open angle Open angle Open angle
Lens status Disruption to lens capsule Mature or hypermature Disruption to lens capsule;
with release of lens particles cataract exposure of large lens fragments
Management Antiglaucoma medication, Antiglaucoma medication, Antiglaucoma topical medication,
steroids, surgical removal of topical steroids, cataract topical steroids, removal of lens
lens material removal fragments
LAU, lens associated uveitis; HMW, heavy molecular weight proteins; TM, trabecular meshwork; IOP, intraocular pressure.

246

LEN
L ENSS PROO EIN
EII N O R
PH
P H AC
ACO
C O LY
LY IC
CG GLAUCO
LA
AUCO
O MA
A Pa ien s repor gradually diminishing
L ens pro ein glaucoma occurs in he pres-
ence o a ma ure or a hyperma ure ca arac
(Fig. 15-1). Soluble lens pro eins seep in o he
an erior chamber and obs ruc he rabecular
meshwork, causing an eleva ion in IOP.
Pathophysiology
In lens pro ein glaucoma, heavy-molecular-
weigh (HMW) pro eins (grea er han 150
× 106 Da) obs ruc rabecular meshwork
ou ow, causing a rise in IOP. Previously, i
was hough ha he rise in pressure resul ed
exclusively rom macrophage ou ow
obs ruc ion, based on he ac ha hey were
iden if ed in he aqueous humor and in he
rabecular meshwork o pa ien s wi h lens
pro ein glaucoma1,2 (Fig. 15-2). However,
Eps ein e al.3,4 sugges ed ha HMW pro eins
obs ruc he rabecular meshwork based on
he ollowing experimen al evidence:
Eps ein sampled aqueous uid o
pa ien s wi h phacoly ic glaucoma and
showed an abundance o HMW pro eins,
which increase in concen ra ion as he
ca arac ma ures.
In vi ro per usion o cadaver eyes wi h
HMW soluble pro eins caused a 60%
decrease in ou ow acili y a er 1 hour.
T e HMW pro eins were presen in
high-enough concen ra ions in he aque-
ous humor o pa ien s wi h lens pro ein
glaucoma o cause obs ruc ion o ou ow.
Several o he eyes wi h phacoly ic glau-
coma had a pauci y o macrophages.
Lens pro eins can induce he migra ion
o peripheral blood monocy es5 and macro-
phages probably unc ion as scavengers o
remove soluble lens pro eins and ragmen s
rom he an erior chamber and rabecular
meshwork.
Lens Protein or Phacolytic Glaucoma 247
History
vision rom he ma ure or hyperma ure ca a-
rac and pain rom in amma ion and eleva ed
IOP.
Clinical Examination
Lens pro ein glaucoma occurs in he pres-
ence o a ma ure or hyperma ure ca arac .
T ese pa ien s have an acu ely eleva ed IOP,
ocular redness, and pain. T ere is in ense
are, which correla es wi h soluble pro eins
released rom he ma ure ca arac (Fig. 15-3).
A cellular response composed mos ly o
macrophages is presen , and he cells appear
larger and more ranslucen han lympho-
cy es. Hypopyon is uncommon.
Whi e pa ches may be observed on he
lens and are hough o correspond o aggre-
ga es o macrophages phagocy osing lens pro-
eins a leakage si es on he capsule.
Gonioscopy reveals open angles. A re inal
perivasculi is has been observed in some cases.6
Special Tests
Samples aken rom he aqueous humor
and concen ra ed via Millipore f l ra ion may
reveal macrophages and an amorphous sub-
s ance corresponding o lens pro ein.
T e diagnosis is usually made on clinical
observa ion alone.
Treatment
Managemen o lens pro ein glaucoma
should s ar wi h medical herapy o empo-
rize he eleva ed IOP. Be a-blockers, pros-
aglandin analogs, alpha-adrenergic drugs,
and carbonic anhydrase inhibi ors are he
mains ays o medical herapy. opical s eroids
o reduce he in amma ion and cycloplegics
o s abilize he blood–aqueous barrier and o
reduce pain may also be used.
Medical herapy may help o par ially lower
he pressure, bu def ni ive rea men can
 248 15 LENS-ASSO CIATED O PEN-ANGLE GLAUCO MAS

only be ob ained by removal o he ca arac .
In developing coun ries, small-incision ex ra-
capsular ca arac surgery has been shown in a
case series o be a sa e and e ec ive me hod o
surgical herapy wi h minimal morbidi y.7
REFERENCES
1. Hogan M, Zimmerman L. Ophthalmic Pathology: An
Atlas and Textbook. 2nd ed. Philadelphia, PA: WB
Saunders; 1962:797.
2. Irvine S, Irvine A. Lens-induced uvei is and glaucoma.
Am J Ophthalmol. 1952;35:489.
3. Eps ein D, Jedziniak J, Gran W. Iden if ca ion o heavy-
molecular-weigh soluble pro ein in aqueous humor in
human phacoly ic glaucoma. Invest Ophthalmol Vis Sci.
1978;17(5):398–402.
4. Eps ein D, Jedziniak J, Gran W. Obs ruc ion o aque-
ous ou ow by lens par icles and by heavy-molecular-
weigh soluble lens pro eins. Invest Ophthalmol Vis Sci.
1978;17(3):272–277.
5. Rosenbaum J. Chemo ac ic ac ivi y o lens pro eins
and he pa hogenesis o phacoly ic glaucoma. Arch
Ophthalmol. 1987;105:1582.
6. Uemura A, Sameshima M, Nakao K. Complica ions
o hyperma ure ca arac : Spon aneous absorp ion
o lens ma erial and phacoly ic glaucoma-associa ed
re inal perivasculi is. Jpn J Ophthalmol. 1988;32(1):
35–40.
7. Venka esh R, an CS, Kumar , e al. Sa e y and
e cacy o manual small incision ca arac surgery
or phacoly ic glaucoma. Br J Ophthalmol. 2007;91:
279–281.

FIGURE 15-1. Mature cataract. Mature cataract with olds in the anterior capsule. Courtesy o Donald L.
Budenz, MD, Bascom Palmer Eye Institute, Miami, FL.

FIGURE 15-2. Lens protein glaucoma. Macrophages in the trabecular meshwork in lens protein glaucoma.
Courtesy o Donald L. Budenz, MD, Bascom Palmer Eye Institute, Miami, FL.
 Lens Protein or Phacolytic Glaucoma 249

FIGURE 15-3. Lens protein glaucoma. Intense anterior chamber inf ammation with mature cataract in lens
protein glaucoma. Courtesy o Donald L. Budenz, MD, Bascom Palmer Eye Institute, Miami, FL.
 250 15 LENS-ASSO CIATED O PEN-ANGLE GLAUCO MAS
LEN
L E N S PA
PAR
AR ICL
ICLE
LE
GLAUCO
G L AU C O MA
MA
L ens par icle glaucoma occurs when he lens
capsule is disrup ed and lens cor ex and
pro eins are released in o he an erior chamber.
T is may occur a er ex racapsular ca arac sur-
gery, lens rauma wi h capsular disrup ion, and
neodymium (Nd):YAG pos erior capsulo -
omy in which libera ed lens par icles obs ruc
he rabecular meshwork, reducing aqueous
ou ow. Lens par icle glaucoma a er sublux-
a ion o a pos erior chamber in raocular lens
(PCIOL) in a pa ien wi h pseudoex olia ion
syndrome has also been repor ed1 (Fig. 15-4).
PATHOPHYSIOLOGY
T e eleva ed IOP in lens par icle glaucoma
can be caused by he ollowing:
Lens par icles obs ruc ing he rabecu-
lar meshwork
In amma ory cells
Peripheral an erior synechiae and angle
closure rela ed o he in amma ion
Pupillary block rom pos erior synechiae
Eps ein e al.2 per used whole enuclea ed
human eyes wi h par icula e lens ma erial
as well as wi h HMW soluble lens pro eins.
Aqueous ou ow acili y decreased in a
s epwise ashion as he concen ra ion o lens
par icles increased. No all pa ien s undergo-
ing ca arac surgery wi h lens par icles in he
an erior chamber develop eleva ed pressures.
T is sugges s ha a dynamic s a e exis s
be ween lens par icle obs ruc ion o he ra-
becular meshwork and lens par icle clearance
by phagocy ic cells. Phagocy ic cells in he
rabecular meshwork can inges lens par icles
and clear he ou ow pa hways. Macrophages
have been observed o con ain lens pro-
eins and lens par icles. Perhaps, in pa ien s
who develop lens par icle glaucoma, he
mechanism o meshwork clearance becomes
overloaded or ha phagocy ic cells or he
meshwork i sel are abnormal.
Eleva ed IOP can occur a er Nd:YAG cap-
sulo omy. Smi h3 has shown ha he aqueous
ou ow acili y a er Nd:YAG capsulo omy
decreases. One hour a er he laser procedure,
he aqueous ou ow acili y decreased by an
average o 43% and he IOP increased by an
average o 38%. A 24 hours and 1 week a er
he laser procedure, ou ow acili y dri ed
back o normal values. A er Nd:YAG capsu-
lo omy, lens debris consis ing o lens capsule
ragmen s and ragmen s o cor ex can be
seen on sli -lamp examina ion. I was sug-
ges ed ha his is one o he mechanisms or
he decrease in ou ow acili y.
HISTORY
Pa ien s have decreased vision resul ing
rom corneal edema and pain i he IOP is
ex remely high.
In some cases, a recen his ory o rauma,
ca arac surgery, or laser procedure o he eye
exis s, hough pressure rise can also occur
years a er ca arac surgery.4
CLINICAL EXAMINATION
T e rise in IOP seen wi h lens par icle
glaucoma may correla e wi h he amoun o
lens par icles circula ing in he an erior cham-
ber. T ere can be a delay o days o weeks
be ween he release o he ma erial and he
onse o eleva ed IOP. Small whi e ragmen s
o he lens cor ex can be seen circula ing in
he an erior chamber and can deposi on he
corneal endo helium.
Eleva ed IOP can cause corneal edema,
and in amma ion can be marked, as evi-
denced by are and cell.

A hypopyon may be presen , and one
case series has also described spon aneous
hyphema in he absence o rauma or neovas-
culariza ion in hese pa ien s.5
Early in he process he angle is open on
gonioscopy, al hough peripheral an erior syn-
echiae may develop.
SPECIAL TESTS
T e diagnosis is made by he observa ion
o ree lens par icles oa ing around in he
an erior chamber and an eleva ed IOP. I he
appearance is a ypical or i here is a pauci y
o lens par icles, an aqueous sample can be
aken o iden i y lens ma erial his ologically
(Fig. 15-5).
TREATMENT
Glaucoma medica ions men ioned previ-
ously or rea men o lens pro ein glaucoma
should be used in conjunc ion wi h he degree
o pressure eleva ion. A cycloplegic agen
may be used o preven pos erior synechiae.
opical cor icos eroids should be used, bu
he in amma ion should no be comple ely
suppressed or lens absorp ion will be delayed.
Lens Particle Glaucoma 251
I medical herapy is no e ec ive, he lens
par icles should be removed surgically by
aspira ion. I surgery is delayed, he persis en
in amma ion may cause peripheral an erior
synechiae, pupillary block, and in amma-
ory membranes ha may ex end pos eriorly,
placing ension on he re ina. A his s age,
membranes and debris mus be cu ou wi h
vi rec omy ins rumen s.
Usually, surgical aspira ion o he ma erial
is enough o bring abou con rol o IOP and
in amma ion.
REFERENCES
1. Lim MC, Doe EA, Vroman D , e al. La e onse lens
par icle glaucoma as a consequence o spon ane-
ous disloca ion o an in raocular lens in pseudoex o-
lia ion syndrome. Am J Ophthalmol. 2001;132(2):
261–263.
2. Eps ein D, Jedziniak J, Gran W. Obs ruc ion o aque-
ous ou ow by lens par icles and by heavy-molecular-
weigh soluble lens pro eins. Invest Ophthalmol Vis Sci.
1978;17(3):272–277.
3. Smi h C. E ec o neodynium: YAG pos erior capsu-
lo omy on ou ow acili y. Glaucoma. 1984;6:171.
4. Barnhors D, Meyers S, Myers . Lens-induced glau-
coma 65 years a er congeni al ca arac surgery. Am J
Ophthalmol. 1994;188:807–808.
5. Ra hinam SR, Cunningham E . Spon aneous hyphema
and acu e ocular hyper ension associa ed wi h severe
lens-induced uvei is. Eye. 2010;24(12):1822–1824.
 252 15 LENS-ASSO CIATED O PEN-ANGLE GLAUCO MAS

FIGURE 15-4. Pseudoex oliation. Subluxed PCIOL in a patient with pseudoex oliation. T is patient
developed lens particle glaucoma as a result o released lens cortex a er the PCIOL dislocation.

FIGURE 15-5. Lens f ber. Lens ber recovered rom aqueous aspirate o the eye shown in Figure 15-4.
 Lens associated Uveitis (Phacoanaphylaxis) 253

con ribu ions. Mas -cell degranula ion is

LENS-ASSO
L E NS-A
ASS
SO CIA
C IA
A ED
D UV
UVEI
VEI IIS
S regularly observed in experimen al dis-
(P
PHH ACOANAPH
AC
C OA
AN APHH YLAXIS)
YLA
AXII S) ease, and immune complexes seem o he
be he main media or o he in amma ory

L AU, also known as phacoanaphylac ic

uvei is or endoph halmi is, can be con-
used wi h he previous wo en i ies al hough
i is usually associa ed wi h hypo ony. T is is
a rare granuloma ous in amma ion ha devel-
ops in si ua ions in which he immune sys em
is exposed o lens pro eins:
A er ca arac ex rac ion
A er rauma ic rup ure o he lens capsule
A er ca arac ex rac ion in one eye ol-
lowed by ca arac ex rac ion or leaking ma ure
ca arac in he o her eye
PATHOPHYSIOLOGY
LAU was once hough o represen an
immune rejec ion o previously seques ered
lens pro eins. However, lens pro eins are
ound in he aqueous o normal eyes. I is
now hough ha an al era ion in immune
olerance o lens pro eins occurs, because
no all eyes wi h disrup ed lens capsules
develop LAU, while rejec ion o oreign is-
sue gra s approaches 100%. Cousins and
Kraus-Mackiw1 specula e ha LAU is a
spec rum o diseases ha may be explained
by au oimmune, in ec ious, and oxic
mechanisms.
T e au oimmune heory has never been
proven in human eyes, bu experimen al
lens-induced granuloma ous endoph hal-
mi is in ra s closely resembles LAU. T e
animals are sensi ized o lens homogena es
and, upon surgical injury o he lens, develop
uvei is wi h his ology similar o LAU. -cells
have been shown o be a requiremen or
he induc ion o experimen al LAU.2 Cell-
media ed immune response, macrophages,
mas cells, and o her elemen s may all make
response.2
T e in ec ious mechanism pos ula es ha
an in amma ory response is moun ed agains
indolen bac eria such as Propionibacterium
acnes ha are ound in lens ma erial or ha
bac eria ins iga e a loss o immune olerance
in he eye. Finally, he heory o lens oxici y
may be described as lens ma erial ha direc ly
riggers an in amma ory reac ion wi hou
previous immuni y. All hree en i ies are pos-
sibili ies in he explana ion o LAU, bu none
has been proven hus ar.
Un or una ely, LAU is o en diagnosed
a er enuclea ion when his ology can be exam-
ined. T e his ology o LAU may be described
as a zonal granuloma ous in amma ion wi h
hree popula ions o cell ypes ound in layers
around he lens ma erial (Fig. 15-6):
Zone 1—Neu rophils closely surround
and inf l ra e he lens.
Zone 2—A secondary zone o mono-
cy es, macrophages, epi helioid cells, and
gian cells surround he neu rophils.
Zone 3—A nonspecif c mononuclear
cell inf l ra e orms he ou er zone o
in amma ion.
HISTORY
Pa ien s have pain, decreased vision, and
red eye.
CLINICAL EXAMINATION
T e presen a ion can be variable and
may presen as a low-grade an erior segmen
in amma ion, especially a er ca arac surgery.
Once remaining lens ma erial is resorbed, he
in amma ion resolves.
 254 15 LENS-ASSO CIATED O PEN-ANGLE GLAUCO MAS
Panuvei is wi h a hypopyon represen s
a more severe presen a ion ha is di cul
o dis inguish rom endoph halmi is (Fig.
15-7). T ere is usually a his ory o re ained
lens ragmen s in he vi reous. T e granu-
loma ous in amma ory reac ion can occur
wi hin days or mon hs a er disrup ion o he
lens.
LAU is usually associa ed wi h hypo ony
ra her han wi h eleva ed IOP, al hough high
pressures may occur.3 Kera ic precipi a es are
presen , and synechiae can lead o pupillary
block or angle-closure glaucoma.
SPECIAL TESTS
Aspira es o aqueous or vi reous wi h neg-
a ive bac erial cul ures may help di eren ia e
LAU rom bac erial endoph halmi is, bu
cy ology is rarely help ul.
Ul rasound may help loca e large lens rag-
men s in he vi reous chamber in he set ing
o ca arac surgery or rauma.
TREATMENT
I le un rea ed, a relen less uvei is can
lead o ph hisis. opical, sub- enon’s, and
oral s eroids may be used as a emporizing
measure, and conserva ive herapy only may
be appropria e i he lens ma erial resorbs
wi hou des ruc ive in amma ion.
Def ni ive rea men involves removal
o lens ragmen s, op imally by pars plana
vi rec omy.
His orically, he prognosis o severe cases
o LAU has been very poor, bu curren ly,
wi h bet er surgical echniques and equip-
men , he possibili y o re aining good vision
is improving.4
REFERENCES
1. Cousins SW, Kraus-Mackiw E. Ocular In ection &
Immunity. 1s ed. S . Louis, MO: Mosby; 1996:1552.
2. Marak G. Phacoanaphylac ic endoph halmi is. Surv
Ophthalmol. 1992;36:325–339.
3. Ra hinam S, Cunningham E . Spon aneous hyphema
and acu e ocular hyper ension associa ed wi h severe
lens-induced uvei is. Eye. 2010:1–3.
4. Oruc S, Kaplan H. Ou come o vi rec omy or re ained
lens ragmen s a er phacoemulsif ca ion. Ocul Immunol
Inf amm. 2001;9(1):41–47.
 Lens associated Uveitis (Phacoanaphylaxis) 255

FIGURE 15-6. LAU. Zonal granulomatous ormation in a patient with LAU. Courtesy o Donald L. Budenz,
MD, Bascom Palmer Eye Institute, Miami, FL.

FIGURE 15-7. LAU. Severe anterior chamber inf ammation, hypopyon, and corneal edema in a patient with
LAU. Courtesy o Donald L. Budenz, MD, Bascom Palmer Eye Institute, Miami, FL.
 256 15 LENS-ASSO CIATED O PEN-ANGLE GLAUCO MAS
PH
HAACO
COMMOOR
RPH
P H IC
C CLINICAL EXAMINATION
GLAUCO
GL
L AU
U CO
O MA
MA
P hacomorphic glaucoma resul s rom angle
closure secondary o a ma ure or hyper-
ma ure lens. I may be dis inguished rom he
previous en i ies by he clinical appearance o
an in umescen lens, shallow an erior cham-
ber, and angle closure.
PATHOPHYSIOLOGY
Phacomorphic glaucoma is a direc
sequela o a ma ure or hyperma ure lens ha
has become in umescen , causing crowd-
ing o he an erior segmen s ruc ures.1 In
he early s age, pupillary block may cause
high IOP. La er, he growing size o he lens
presses orward on he iris in he periphery,
blocking o ou ow hrough he rabecular
meshwork.
Phacomorphic glaucoma is a common
condi ion in developing coun ries in which
ca arac surgery is delayed.
T e visual prognosis is poor, wi h
one s udy repor ing ha only 57% o 49
pa ien s wi h phacomorphic glaucoma
at ained visual acui y o 6/ 12 or bet er,
al hough ano her s udy repor ed ha over
80% o pa ien s had some long- erm visual
improvemen and IOP normaliza ion a er
ca arac ex rac ion. Bet er visual ou come
appears o be rela ed o a shor er dura ion
o eleva ed IOP.2,3
HISTORY
Pa ien s have chronic or acu e decrease
in vision, ocular pain, headache, and
pho ophobia.4
T e crux o he problem is he ma ure or
hyperma ure ca arac causing a shallow an e-
rior chamber (Fig. 15-8). T e pupil may be
mid-dila ed wi h or wi hou iris bombé, and
gonioscopy reveals angle closure.
T e IOP is high rom obs ruc ion o aque-
ous ou ow, and as a resul he cornea may be
edema ous (Fig. 15-9).
TREATMENT
Medical herapy o suppress aqueous or-
ma ion is he f rs line o rea men . Mio ics
may increase con ac be ween he lens and
iris and should no be used.1 Laser irido omy
should be per ormed o allevia e any com-
ponen o pupillary block. Irido omy may
open up he angle, lower he IOP, and allow
he eye o quie be ore ca arac removal. I
may also give he clinician an oppor uni y
o examine he angle or peripheral an erior
synechiae.4
T e degree o scarring in he angle
may signal he need or glaucoma surgery
ei her a he ime o ca arac removal or in
he u ure. One s udy ound a 20% ra e o
glaucoma progression over 2 years indica -
ing ha hese pa ien s need o be ollowed
or long erm.3 T e def ni ive rea men or
phacomorphic glaucoma is removal o he
in umescen lens. One case series repor ed
manual small-incision ca arac surgery as a
sa e and e ec ive rea men in developing
coun ries where his en i y is more common,
bu phacoemulsif ca ion has also proven an
e ec ive echnique.5,6
Capsulorhexis in he set ing o a dense lens
may be acili a ed by he use o indocyanine
green or rypan blue s aining o he an erior
capsule.
 Phacomorphic Glaucoma 257

REFERENCES 4. omey K. Neodynium: YAG laser irido omy in he

1. Liebmann JM, Ri ch R. Glaucoma Associated with Lens
Intumescence and Dislocation. Vol 2. 2nd ed. S . Louis,
MO: Mosby-Year Book, Inc.; 1996:1033–1053.
2. Prajna N, Ramakrishnan R, Krishnadas R, e al. Lens
induced glaucomas-visual resul s and risk ac ors or
f nal visual acui y. Indian J Ophthalmol. 1996;44(3):
149–155.
3. Lee J, Lai J, Yick D, e al. Re rospec ive case series
on he long- erm visual and in raocular pressure ou -
comes o phacomorphic glaucoma. Eye. 2010;24(11):
1675–1680.
ini ial managemen o phacomorphic glaucoma.
Ophthalmology. 1992;99:660–665.
5. Lee S, Lee C, Kim W. Long- erm herapeu ic e cacy
o phacoemulsif ca ion wi h in raocular lens implan-
a ion in pa ien s wi h phacomorphic glaucoma. J
Cataract Re act Surg. 2010;36(5):783–789.
6. Ramakrishanan R, Maheshwari D, Kader M, e al.
Visual prognosis, in raocular pressure con rol and com-
plica ions in phacomorphic glaucoma ollowing man-
ual small incision ca arac surgery. Indian J Ophthalmol.
2010;58(4):303–306.

FIGURE 15-8. Phacomorphic glaucoma. A hyperdense crystalline lens causing a shallow anterior chamber. T e
lens is dislocated in eriorly. Courtesy o Richard K. Lee, MD, PhD, Bascom Palmer Eye Institute, Miami, FL.

A B

FIGURE 15-9. Phacomorphic glaucoma. A. Slit lamp photograph o an eye with phacomorphic glaucoma.
Descemet’s olds rom an edematous cornea and darkly brunescent cataract are seen. B. Slit beam photograph o
the same eye shows the corneal swelling and narrow anterior chamber. Courtesy o Douglas J. Rhee, MD, Wills
Eye Hospital, Philadelphia, PA.
 C H AP T ER
F ollowing ocular rauma, pa ien s of en
develop di cul ies wi h in raocular pres-
sure con rol. In raocular pressure may be ele-
va ed or he eye may be hypo onous. Pa ien s
may have di cul y acu ely or many years ol-
lowing he injury. In ei her case, a horough
his ory and examina ion of en will de ermine
he cause and severi y o he in raocular dam-
age and he appropria e course o rea men
and ollow-up. Open and closed globe injuries
can resul in damage o any o he ocular s ruc-
ures. T is chap er ocuses on rauma ic hyphe-
mas, angle recession, and cyclodialysis clef s.
TRAUMATIC HYPHEMA
T he erm hyphema re ers o blood in he
an erior chamber. T e amoun o blood
may be microscopic, ermed microhyphema,
visible only a he sli lamp as nonlayering red
blood cells in he aqueous. Red blood cells may
also layer or orm clo s in he an erior chamber
(Figs. 16-1 o 16-3). A total hyphema re ers o
layered blood lling he en ire an erior cham-
ber (Fig. 16-4). A o al hyphema ha has clo -
ed and appears black in color is re erred o as
an eight-ball hyphema (Fig. 16-5). A rauma ic
258
rauma ic Glaucoma
Angela V. Turalba and Mary Jude Cox
hyphema can resul rom ei her blun or pen-
e ra ing injury o he globe. T e majori y o
hyphemas resolve gradually wi hou sequelae;
however, complica ions such as rebleeding,
increased in raocular pressure, and corneal
blood s aining (Fig. 16-6) can occur.
Epidemiology
rauma ic hyphemas are mos common in
young ac ive men, wi h a male- o- emale ra io
o approxima ely hree o one. In general,
he risk o complica ions such as rebleeding,
uncon rolled in raocular pressure, or corneal
blood s aining increases wi h he size o he
hyphema. Pa ien s wi h sickling hemoglobin-
opa hies, however, are he excep ion. T ese
pa ien s are a increased risk or complica-
ions regardless o he size o he hyphema.
Rebleeding occurs in up o 35% o pa ien s.
T e majori y o rebleeding episodes ake
place wi hin 2 o 5 days o he ini ial injury.
Rebleeding is of en larger han he original
hyphema and more prone o complica ions.
Pathophysiology
Blun rauma causes compressive orces
ha resul in he shearing o iris and ciliary

body vessels. ears in he ciliary body resul
in damage o he major ar erial circle o he
iris. Pene ra ing injuries can cause direc
damage o blood vessels. Clo s plug hese
damaged blood vessels and rebleeding
occurs as hese clo s re rac and lyse
(Fig. 16-2B).
In raocular pressure rises acu ely as red
blood cells, in amma ory cells, and debris
obs ruc he rabecular meshwork (Fig.
16-1C). Eleva ed in raocular pressure can
also be he resul o pupillary block caused by
he clo in he an erior chamber. Eigh -ball
hyphemas of en cause his orm o pupillary
block and can impair aqueous circula ion
(Fig. 16-5). T e impaired aqueous circula-
ion causes a decrease in oxygen concen ra-
ion in he an erior chamber resul ing in he
black appearance o he clo .
In pa ien s wi h sickle cell disease or rai ,
sickling causes he red blood cells o be rigid
and easily rapped in he rabecular mesh-
work, leading o eleva ed in raocular pressure
even in he presence o a small hyphema.
Sickle cell pa ien s are subjec o vascular
occlusion and op ic nerve damage a lower
in raocular pressures as he resul o microvas-
cular compromise.
History and Clinical Examination
For pa ien s presen ing wi h a rauma ic
hyphema, a horough evalua ion o he im-
ing and na ure o he rauma is impor an
o de ermine he likelihood o addi ional
injuries and he need or close observa ion
and rea men . Pa ien s may be asymp oma ic
or have decreased vision, pho ophobia, and
pain. Nausea and vomi ing may accompany
a rise in in raocular pressure. T ere may be
evidence o orbi al rauma or damage o o her
ocular issues.
Sli lamp: Sli -lamp examina ion may show
circula ing red blood cells alone or in combi-
na ion wi h a layered hyphema in he an erior
Traumatic Hyphema 259
chamber. When here is eleva ed in raocular
pressure associa ed wi h a large hyphema,
corneal blood s aining can occur (Fig. 16-6).
T ere may be evidence o rauma o o her
ocular s ruc ures such as ca arac (Fig. 16-7A,
B), phacodonesis, subconjunc ival hemor-
rhage, oreign bodies, lacera ions, or iris dam-
age such as sphinc er ears, iridodialysis, or
rauma ic aniridia (Figs. 16-7A and 16-8).
Gonioscopy: Gonioscopy should be
delayed un il he risk o rebleeding has passed.
When per ormed 3 o 4 weeks af er he ini ial
injury, he angle may appear undamaged or
may show residual blood (see Fig. 16-1C)
or angle recession (Fig. 16-9). Occasionally,
peripheral an erior synechiae or a cyclodialy-
sis clef (Fig. 16-10A) may be presen .
Pos erior pole: T e pos erior pole may
show evidence o blun or pene ra ing rauma.
Commo io re inae, choroidal rup ures, re inal
de achmen s, in raocular oreign bodies, or
vi reous hemorrhage may be presen . Scleral
depression should be delayed un il he risk o
rebleeding has passed. A persis en vi reous
hemorrhage can also cause eleva ed in raocu-
lar pressures in he orm o ghos cell glau-
coma. Unlike he ypical red blood cells seen
in hyphemas, an-colored ghos cells can be
observed in he an erior chamber. Ghos cells
are degenera ed ery hrocy es ha clog he
rabecular meshwork as hey make heir way
rom he pos erior segmen o he an erior
chamber presumably hrough a break in he
an erior hyaloid ace.
Special Tests
B-scan ul rasonography should be per-
ormed in any pa ien in whom here is
no view o he pos erior pole. Compu ed
omographic scan o he orbi s should be per-
ormed i here is clinical suspicion or orbi al
rac ures or in raocular oreign bodies.
Any black or Hispanic pa ien or any
pa ien wi h a posi ive amily his ory should
 260 16 TRAUMATIC GLAUCO MA
undergo sickle prep or hemoglobin elec ro-
phoresis o de ermine he presence o sickling
hemoglobinopa hies.
Treatment
T e a ec ed eye is shielded and he pa ien
is ypically placed on ac ivi y res ric ions. T e
pa ien is asked o keep he head o he bed
eleva ed o allow he blood o set le below
he visual axis (Fig. 16-3). T e pa ien is
ins ruc ed o avoid aspirin and nons eroidal
agen s. opical cycloplegics and s eroids
are given o rea in amma ion and preven
orma ion o synechiae. Aminocaproic acid,
an an i brinoly ic, can be given sys emically
o preven rebleeding. Aminocaproic acid
may cause pos ural hypo ension, nausea, and
vomi ing, and should be avoided in pregnan
pa ien s and hose wi h cardiac, hepa ic, or
renal disease.
Eleva ed in raocular pressure is rea ed
opically wi h be a-blockers, alpha-agonis s,
or carbonic anhydrase inhibi ors (CAIs).
Mio ics and pros aglandin analogs may
increase in amma ion and are avoided i
possible. Oral or in ravenous CAIs or hyper-
osmo ic agen s may also be given o lower
in raocular pressure. However, hese sys emic
agen s should be avoided in pa ien s wi h
sickling hemoglobinopa hies because CAIs
increase he pH o he aqueous and hyperos-
mo ic agen s can cause hemoconcen ra ion,
resul ing in increased sickling.
Surgical in erven ion is indica ed in
pa ien s a risk or corneal blood s aining
(Fig. 16-6), uncon rolled in raocular pres-
sure, or persis en pain. T e iming o surgical
in erven ion or in raocular pressure con rol
depends on he individual pa ien . In a pa ien
wi h a heal hy op ic nerve, an in raocular
pressure o 60 mm Hg or 2 days, 50 mm Hg
or 5 days, or 35 mm Hg or 7 days requires
surgical in erven ion. Pa ien s wi h compro-
mised op ic nerves or corneal endo helium
require earlier in erven ion, as do pa ien s
wi h sickle cell disease or rai . Surgical in er-
ven ion is indica ed in sickle cell pa ien s wi h
an in raocular pressure grea er han 24 mm
Hg or more han 24 hours.
Surgical op ions o remove he hyphema
include an erior chamber washou , clo
expression a he limbus, or removal wi h
an erior vi rec omy ins rumen a ion. I pos-
sible, clo removal should be per ormed 4 o
7 days pos - rauma in order o preven new
bleeding. In some cases, a guarded l ra ion
procedure is per ormed concurren ly o con-
rol in raocular pressure.
 Traumatic Hyphema 261

A B

C
FIGURE 16-1. Small hyphema. A. T is small hyphema is layering in eriorly in he an erior chamber. Mos
hyphemas resorb gradually. B. T e same eye 4 days la er has a much smaller clo in he an erior chamber. C. T is
is ano her eye wi h persis en ly eleva ed pressures 3 weeks af er an injury when he layered hyphema was no
longer eviden on sli -lamp examina ion. Gonioscopy reveals residual blood in he angle.

A B
FIGURE 16-2. Traumatic hyphema with rebleed. A. Blood layers in he an erior chamber o his eye wi h a
rauma ic hyphema. B.T e same eye rebleeds 24 hours la er, demons ra ing an increase in he amoun o blood
in he an erior chamber.
 262 16 TRAUMATIC GLAUCO MA

FIGURE 16-3. Layering hyphema. T e hemorrhage in his new hyphema obscures he visual axis, bu is
beginning o set le in o he in erior an erior chamber. Pa ien s are ins ruc ed o keep heir head eleva ed o assis
his process.

FIGURE 16-4. Total hyphema. A o al hyphema is presen ollowing a baseball injury. T e an erior chamber is
lled wi h brigh red blood.
 Traumatic Hyphema 263

FIGURE 16-5. Eight ball hyphema. An eigh -ball hyphema is a o al clo o he an erior chamber ha ge s i s
black appearance rom decreased oxygena ion as a resul o impaired aqueous circula ion.

A B
FIGURE 16-6. Corneal blood staining. A. Corneal blood s aining persis s in his eye ollowing surgical
evacua ion o he hyphema. B. A ew mon hs af er a pene ra ing eye injury and a o al hyphema, his eye has
persis en cen ral corneal blood s aining wi h peripheral clearing.
 264 16 TRAUMATIC GLAUCO MA

A B
FIGURE 16-7. Traumatic cataracts. A. rauma ic ca arac s can occur immedia ely or mon hs af er blun or
pene ra ing eye injuries. T is eye has a comple e rauma ic ca arac ha developed immedia ely af er a blun
injury. T e ca arac and mul iple iris sphinc er ears became more eviden as he o al hyphema cleared. B. T is
pa ien had a pene ra ing injury resul ing in a ull- hickness corneal lacera ion ( solid arrows) wi h a corresponding
de ec in he lens (dashed arrow) and a di use rauma ic ca arac . T e pa ien developed lens par icle glaucoma
rom he disrup ion o he lens capsule.
 Traumatic Hyphema 265

A B

FIGURE 16-8. Iris injur y. A. Blun rauma

can cause iridodialysis or ears a he iris roo .
B. Re roillumina ion highligh s he area o
iridodialysis in he same pa ien . C. T is young
pa ien has rauma ic aniridia and aphakia as a resul
C o a pene ra ing injury hrough he cornea. T e arrows
poin o he edge o he remaining iris.
 266 16 TRAUMATIC GLAUCO MA
ANGLE RECESSION
A ngle recession re ers o a ear in he ciliary
body be ween he longi udinal and circu-
lar muscle layers. Clinically, here is abnormal
widening o he ciliary body band on gonios-
copy (Fig. 16-9).
Epidemiology
Angle recession occurs as a resul o blun
or pene ra ing rauma o he an erior seg-
men . T e risk o developing angle recession
glaucoma is propor ional o he ex en o cili-
ary body damage, wi h an incidence as high
as 10% in eyes wi h grea er han 180 degrees
o damage. Glaucoma may develop mon hs o
years af er he original injury.
Pa ien s who develop angle recession
glaucoma may be predisposed o open-angle
glaucoma, as evidenced by he ac ha up o
50% o hese pa ien s will develop eleva ed
pressures in he con rala eral eye.
Pathophysiology
Angle recession is caused by a ear be ween
he circular and longi udinal muscle layers
o he ciliary body. Angle recession glau-
coma resul s rom decreased ou ow acili y.
Impaired ou ow may occur as he resul o
direc damage o he rabecular meshwork or
as he resul o a Desceme -like endo helial
proli era ion over he rabecular meshwork.
History and Clinical Examination
Pa ien s presen wi h ei her a recen or
remo e his ory o rauma in he a ec ed eye.
Pa ien s may be asymp oma ic or presen
wi h pain, pho ophobia, and decreased vision
as he resul o eleva ed in raocular pressure.
T ey may have evidence o visual eld loss or
an a eren pupillary de ec rom glaucoma-
ous op ic nerve damage. T ere may also be
evidence on examina ion o damage o o her
ocular or orbi al s ruc ures.
Sli lamp: Sli -lamp examina ion may show
evidence o previous rauma. Corneal scarring
or blood s aining (Figs. 16-6 B and 16-8C),
ca arac (Fig. 16-7A, B, phacodonesis, iris
sphinc er ears, or ears a he iris roo (irido-
dialysis) (Fig. 16-8) may be presen .
Gonioscopy: Gonioscopy demons ra es
an irregular widening o he ciliary body
band (Fig. 16-9). T ere may be evidence o
orn iris processes or increased prominence
o he scleral spur. T e normal ciliary body
should be roughly even in size around i s cir-
cum erence and no as wide as he rabecular
meshwork. Occasionally, peripheral an erior
synechiae orma ion may obscure he ull
ex en o angle recession resul ing rom he
ini ial rauma. Comparison wi h he una -
ec ed eye of en aids in diagnosis.
Pos erior pole: T e pos erior pole may
show evidence o previous blun or pen-
e ra ing rauma. Choroidal rup ures, re inal
de achmen s, or vi reous hemorrhage may
be presen . Asymme ric op ic nerve cupping
rom eleva ed in raocular pressure in he
a ec ed eye may also be presen .
Special Tests
Visual eld es ing may demons ra e glau-
coma ous eld loss.
Treatment
Pa ien s who demons ra e angle recession
on gonioscopy ollowing rauma need o be
ollowed inde ni ely or he developmen o
glaucoma. I eleva ed in raocular pressures
are ound, hey are of en di cul o con rol.
Ini ially hey can be rea ed medically wi h
aqueous suppressan s. Hyperosmo ics may be
added i necessary. Mio ics of en make angle
recession worse, because hey decrease uveo-
scleral ou ow in eyes ha rely on uveoscleral
ou ow or in raocular pressure con rol.
Laser rabeculoplas y has limi ed success in
eyes wi h angle recession. A guarded l ra-
ion procedure or implan a ion o a glaucoma
drainage device is of en required o con rol
in raocular pressure in hese pa ien s.
 Angle Recession 267

A B
FIGURE 16-9. Angle recession. A. T is eye wi h angle recession shows irregular widening o he ciliary body
band on gonioscopy. B. T is eye has angle recession adjacen o an area o iridodialysis as seen on gonioscopy.
T e ciliary body processes can be seen hrough he de ec in he iris.
 268 16 TRAUMATIC GLAUCO MA
CYCLODIALYSIS CLEFT
T he erm cyclodialysis clef re ers o a ocal
de achmen o he ciliary body rom i s
inser ion a he scleral spur (Fig. 16-10 A, C).
I may occur as he resul o blun or pene ra -
ing rauma or as a complica ion o in raocular
surgery, and leads o emporary or permanen
hypo ony.
Epidemiology
Cyclodialysis clef s ha resul rom blun
or pene ra ing rauma are less common han
angle recession. T e presence o a clef should
be considered in any hypo onous eye wi h a
his ory o rauma or prior in raocular surgery.
Pathophysiology
rauma causes a separa ion o he ciliary
body rom i s at achmen o he scleral spur.
T is allows a direc passage o aqueous rom he
an erior chamber o he suprachoroidal space,
leading o hypo ony. Spon aneous or induced
closure o he clef of en resul s in an acu e ele-
va ion o he in raocular pressure as he primary
ou ow pa hway o aqueous is disrup ed.
History and Clinical Examination
Pa ien s presen wi h a his ory o rauma
or in raocular surgery in he a ec ed eye.
T ey may be asymp oma ic or have decreased
vision. T e a ec ed eye may be hypo onous
or have eleva ed in raocular pressure, pain,
pho ophobia, and redness as a resul o spon-
aneous closure o a previous clef .
Sli lamp: Sli -lamp examina ion may show
evidence o previous blun or pene ra ing
rauma such as corneal scarring or blood s ain-
ing, ca arac , disrup ion o he zonules sup-
por ing he lens (phacodonesis), iris sphinc er
ears, or ears a he iris roo (iridodialysis)
(Fig. 16-8A). Evidence o previous in raocu-
lar surgery, such as pos erior or an erior in ra-
ocular lens placemen , may also be presen .
T e a ec ed eye can be hypo onous wi h
corneal olds and a shallow an erior chamber
when compared wi h he con rala eral eye.
Gonioscopy: Gonioscopy demons ra es
a deep angle recess wi h a gap be ween he
sclera and ciliary body (Fig. 16-10A). T is is
in con ras o angle recession, which appears
as an irregular, widened ciliary body band.
Angle recession may also be presen in he
a ec ed eye as a resul o rauma.
Pos erior pole: Hypo ony can resul in
choroidal e usions, de achmen s and olds.
Hypotony maculopathy re ers o choroidal olds
ha involve he macula and some imes have
accompanying disc edema (Fig. 16-10B).
Pa ien s can experience markedly decreased
vision rom his. Evidence o previous rauma
may also be presen , such as choroidal rup ure,
pos erior vi reous de achmen , or macular hole.
Special Tests
B-scan ul rasonography should be per-
ormed in any hypo onous pos - rauma ic eye
wi h a limi ed view o he pos erior pole in
order o rule ou occul scleral rup ure or re i-
nal de achmen . Ul rasound biomicroscopy
(UBM) is a help ul imaging ool in de ec ing
suspec ed clef s and delinea ing he ex en o
he clef o aid in planning or laser or surgical
rea men (Fig. 16-10C).
Treatment
Occasionally, medical rea men wi h a ro-
pine may resul in cyclodialysis clef closure.
Argon laser and cryo herapy can also be used
o rea smaller clef s by inducing scarring
be ween he ciliary body and sclera. However,
larger cyclodialysis clef s wi h persis en
hypo ony require surgical closure. Following
closure o he clef , he in raocular pressure
of en rises drama ically and should be moni-
ored closely. Medical rea men wi h aqueous
suppressan s and hyperosmo ics may be ini i-
a ed as necessary.
 Cyclodialysis Clef 269

A B

FIGURE 16-10. Cyclodialysis clef . A.

A cyclodialysis clef appears as a deep angle recess
wi h a gap be ween he sclera and ciliary body.
B. Disc edema and macular olds are no ed here
in a pa ien wi h hypo ony maculopa hy secondary
o a persis en cyclodialysis clef rom blun rauma.
C. On ul rasound biomicroscopy, a cyclodialysis clef
can be seen as an abnormal space be ween he ciliary
C
body and he sclera.
 C H AP T ER
Primary Acu e Angle-closure and
Chronic Angle-closure Glaucoma
BACKGROUND
Def nition
Angle closure is charac erized by iris apposi-
ion o he rabecular meshwork. T e mecha-
nisms o his ana omic abnormali y can be
ca egorized a our s ruc ural levels1:
T e pupillary margin (e.g., pupillary block)
T e ciliary body (e.g., pla eau iris and iri-
dociliary cys s)
T e lens (e.g., phacomorphic glaucoma)
Pos erior o he lens (e.g., malignan
glaucoma)
Primary angle closure generally re ers o he
condi ion o an increase in resis ance o he
ow o aqueous humor a he pupillary margin
(i.e., rela ive pupillary block).
T e clinical course can be classif ed in o hree
concep ual s ages2:
1. Primary angle–closure suspec s—individu-
als wi h narrow angles, usually def ned as an
angle in which ≥270 degrees o he pos erior
rabecular meshwork canno be seen
270
Christopher Kai-Shun Leung
2. Primary angle closure—def ned by he
presence o narrow angles combined wi h
peripheral an erior synechiae, eleva ed in ra-
ocular pressure, or a pas acu e angle–closure
at ack
3. Primary angle–closure glaucoma—
primary angle closure combined wi h evi-
dence o glaucoma ous op ic neuropa hy
More han hal o primary angle–closure glau-
coma pa ien s are asymp oma ic or many
years (chronic angle–closure glaucoma) un il
an acu e angle–closure at ack or severe loss
o vision occurs. Acu e angle closure deno es
a specif c orm o primary angle closure char-
ac erized by an acu e eleva ion o in raocu-
lar pressure (usually >40 mm Hg). Pa ien s
complain symp oms o ocular pain, nausea, or
vomi ing, and demons ra e signs o conjunc-
ival injec ion, corneal epi helial edema, mid-
dila ed pupil, and shallow an erior chamber.
Pathophysiology
In primary angle closure, he increase in resis-
ance a he pupillary margin raises he pres-
sure gradien be ween he an erior chamber

(an erior o he iris) and pos erior chamber
(pos erior o he iris) (Fig. 17-1). T e iris
has a charac eris ic orward-bowing con-
f gura ion leading o narrowing o he angle
(primary angle–closure suspec s). T e adhe-
sion o he peripheral iris o he rabecular
meshwork may obs ruc he angle and resul
in eleva ion o in raocular pressure and or-
ma ion o peripheral an erior synechiae (pri-
mary angle closure). I he degree o rela ive
pupillary block is ex ensive and he angle is
already very narrow, comple e obs ruc ion o
he angle occurs, and he rise in in raocular
pressure would be precipi ous, leading o an
acu e at ack (primary acu e angle closure). I
he degree o rela ive pupillary block is small
and he rabecular meshwork is blocked only
in small por ions, he in raocular pressure may
increase gradually over he years resul ing in
chronic progressive degenera ion o he op ic
nerve wi hou developmen o any symp om
(chronic angle–closure glaucoma).
Epidemiology
Al hough angle-closure glaucoma represen s
approxima ely 25% o glaucoma worldwide,
i accoun s or nearly hal o glaucoma blind-
ness.3 I is more prevalen in China, India,
and Sou h-eas Asia han in Europe and La in
America. A shallow an erior chamber, shor
axial leng h, and small corneal diame er are
biome ric risk ac ors or primary angle clo-
sure. T e incidence o acu e angle closure
has been es ima ed a approxima ely 4 o 16
per 100,000 per year in he popula ion aged
30 years and older.4 In addi ion o he biome -
ric risk ac ors or primary angle closure, age
≥60 years, emale gender, a posi ive amily his-
ory, and a hick and bulky lens impose addi-
ional risks or acu e at ack.
Clinical Examination
Gonioscopy
Gonioscopy is an indispensable echnique
o visualize he angle s ruc ures and de ec
Background 271
angle closure. I is impor an o per orm
gonioscopy in a comple ely darkened room
using he smalles square o ligh or a sli
beam ha se s o he pupil as sli -lamp illu-
mina ion can s imula e pupillary ligh re ex
and widen he angle (Fig. 17-2). A narrow
angle is usually def ned as an angle in which
≥270 degrees o he pos erior rabecular
meshwork canno be seen. Inden a ion goni-
oscopy is use ul o di eren ia e synechial
closure rom apposi ional closure. Synechial
closure is recognized when here are consid-
erable acquired adhesions be ween he iris
and he corneoscleral junc ion a he angle.
Peripheral an erior synechiae may ex end cir-
cum eren ially resul ing in synechial closure
and progressive increase in in raocular pres-
sure (Fig. 17-3). T e ex en o peripheral
an erior synechiae correla es wi h he risk o
developing glaucoma.
Ultrasound Biomicroscopy and Optical
Coherence Tomography
While assessmen o he angle wi h goni-
oscopy is largely quali a ive and subjec ive,
objec ive and reproducible measuremen
o he an erior chamber angle can only
be ob ained wi h cross-sec ional imaging
devices like he ul rasound biomicroscopy
(UBM) and he op ical coherence omogra-
phy (OC ) (Fig. 17-4). OC has a number
o advan ages over UBM or an erior cham-
ber angle imaging. I is a noncon ac ech-
nique, has a higher image resolu ion, and
is more precise in loca ing he posi ion o
in eres or evalua ion compared wi h UBM.
UBM, however, has a unique role in visualiz-
ing he ciliary body. Commercially available
ime-domain and spec ral-domain models
have been developed or an erior cham-
ber angle imaging5 (Table 17-1). Wi h he
developmen o high-resolu ion OC imag-
ing sys ems, angle s ruc ures including he
scleral spur, Schwalbe’s line, Schlemm’s canal,
and collec ing channels can be examined
 272 17 PRIMARYACUTE ANGLE– CLOSURE AND CHRONIC ANGLE– CLOSURE GLAUCOMA

ABLE 17-1. Comparison o Commercially Available OC Sys ems or An erior

Chamber Angle Imaging
Stratus Visante RTVue Cirrus CASIA
OCT OCT SL-OCT FD-OCT HD-OCT OCT
Manufacturer Carl Zeiss Carl Zeiss Heidelberg Optovue Carl Zeiss Tomey
Meditec Meditec Engineering Meditec
Year available 2002 2005 2006 2006 2007 2009
Light source Superlumine- Superlumine- Superlumine- Superlumine- Superlumine- Swept-source
scent diode scent diode scent diode scent diode scent diode laser
820 nm 1,310 nm 1,310 nm 840 nm 840 nm 1,310 nm
Axial 10 18 <25 5 5 <10
resolution
(µm)
Scan size 6 mm (width) 16 mm × 15 mm × 2 mm × 2 mm 3 mm × 16 mm ×
× 2 mm 6 mm 7 mm (CAM-S) 1 mm 6 mm
(depth) 6 mm × 2 mm
(CAM-L)
Scan speed 400 A-scans 2,000 A-scans 200 A-scans 26,000 27,000 30,000
per second per second per second A-scans per A-scans per A-scans per
second second second
Fixation Internal and Internal and External Internal and Internal and Internal and
target external external external external external
CAM-S, cornea-anterior module short; CAM-L, cornea-anterior module long. (Reprinted with permission from Leung CK, Weinreb
RN. Anterior chamber angle imaging with optical coherence tomography. Eye (Lond). 2011;25:261 267.)

in grea er de ails (Fig. 17-5). High-speed REFERENCES

imaging allows evalua ion o he angle in
360 degrees. Visualiza ion o he iris prof les
and he angle conf gura ions is enhanced
wi h hree-dimensional recons ruc ion (Fig.
17-6). Examina ion o he an erior chamber
angle wi h OC and UBM no only augmen s
he diagnos ic per ormance o de ec angle
closure, bu also improves our unders and-
ing in he pa hophysiology o primary angle
closure.
1. Ri ch R, Liebmann JM, ello C. A cons ruc or
unders anding angle closure glaucoma: T e role o
ul rasound biomicroscopy. Ophthalmol Clin North Am.
1995;8:281–293.
2. Fos er PJ, Buhrmann R, Quigley HA, e al. T e def ni-
ion and classif ca ion o glaucoma in prevalence sur-
veys. Br J Ophthalmol. 2002;86:238–242.
3. Quigley HA, Broman A . T e number o people
wi h glaucoma worldwide in 2010 and 2020. Br J
Ophthalmol. 2006;90:262–267.
4. He M, Fos er PJ, Johnson GJ, e al. Angle closure glau-
coma in Eas Asian and European people. Di eren
diseases? Eye (Lond). 2006;20:3–12.
5. Leung CK, Weinreb RN. An erior chamber angle
imaging wi h op ical coherence omography. Eye
(Lond). 2011;25:261–267.
 Background 273

C
FIGURE 17-1. Pathophysiology o primar y angle closure. Drawing o (A) apposi ion o pupillary margin o
he an erior sur ace o he lens, (B) aqueous pressure developing behind he iris ( arrows) and pushing he iris
orward, and (C) iris bombé (arrows) causing obs ruc ion o he rabecular meshwork.

A B
FIGURE 17-2. Ef ect o illumination on the anterior chamber angle. An erior segmen OC images ob ained
wi h he Visan e OC (Carl Zeiss Medi ec, Dublin, CA) demons ra ing narrowing o he angle rom ligh (A) o
dark (B). Apposi ional closure is de ec ed only in he dark.
 274 17 PRIMARYACUTE ANGLE– CLOSURE AND CHRONIC ANGLE– CLOSURE GLAUCOMA

A B
FIGURE 17-3. Peripheral anterior synechiae. A. Gonioscopic view o an eye wi h ex ensive peripheral
an erior synechiae. B. T ree-dimensional recons ruc ion o mul iple OC images collec ed rom he same eye
wi h a swep -source OC (Casia OC , omey, Nagoya, Japan) .

A B
FIGURE 17-4. Anterior chamber angle imaging with UBM and the OC . While he ciliary body is bet er
visualized wi h UBM (A), OC (Casia OC , omey, Nagoya, Japan) (B) provides a higher image resolu ion.
T e UBM and OC images were ob ained wi h model 840 (Paradigm Medical Indus ries, Sal Lake Ci y, U )
and a swep -source OC (Casia OC , omey, Nagoya, Japan) , respec ively.
 Background 275

FIGURE 17-5. Anterior chamber angle Imaging with spectral domain OC . De ailed angle s ruc ures
including he scleral spur, Schwalbe’s line, and Schlemm’s canal ( *) can be visualized wi h a spec ral-domain
OC (Cirrus HD OC , Carl Zeiss Medi ec, Dublin, CA) .

A B
FIGURE 17-6. T ree dimensional visualization o the anterior chamber angle. T e Casia OC ( omey,
Nagoya, Japan) is a swep -source OC wi h a scan speed o 30,000 A-scans per second. T e whole an erior
segmen can be radially imaged in 64 cross sec ions in 1.2 seconds. An open (A) and a closed angle (B) in
hree-dimensional display are illus ra ed.
 276 17 PRIMARYACUTE ANGLE– CLOSURE AND CHRONIC ANGLE– CLOSURE GLAUCOMA
PR
PRIMARY
R IM
M ARY ACU
ACUU E
AN
A NG
GLE
LE C
CLOSURE
LO
O SURE
E closure glaucoma.
DIAGNOSTIC EVALUATION
Symp oms o primary acu e angle closure
range rom unila eral blurring and ocular
pain o ex reme ocular or periocular pain,
headache, nausea, vomi ing, and diaphoresis.
Pa ien s may have a his ory o subacu e angle
closure including in ermit en at acks o pain
and possibly mildly blurry vision, which may
be con used wi h migraine headache. Acu e
at acks may be precipi a ed by pharmacologi-
cal mydriasis, dim illumina ion, s ress, or pro-
longed near work.
Clinical examina ion shows conjunc ival
injec ion, corneal epi helial edema, mid-
dila ed pupil, a shallow an erior chamber, and
o en imes, he iris in a classic bombé pat ern
(Figs. 17-7 to 17-9). T e in raocular pres-
sure may be as high as 80 mm Hg. Mild cell
and are are o en presen .
Gonioscopy can be di cul in he pres-
ence o he corneal edema. Early in he at ack,
he op ic nerve head can show edema and
hyperemia.
T e ellow eye should be examined closely
because i will almos always have a shallow
an erior chamber wi h a narrow angle.
PROGNOSIS
Depending on he level o in raocular
pressure and he dura ion o at ack, variable
degree o irreversible ischemic damages can
be incurred on he iris, he lens, and he op ic
nerve resul ing in iris a rophy, glaukom ecken
( ecks o an erior subcapsular opaci ies rep-
resen ing in arc ion o an erior lens epi helial
cells) (Fig. 17-10), and a pale op ic nerve
head wi h visual f eld loss dispropor iona e o
he disc cupping.
Pa ien s may develop chronic eleva ion o
in raocular pressure and progress o angle-
TREATMENT
T e goals o rea men are o reduce he
in raocular pressure and preven recurren
at ack.
opical be a-blocker, cholinergic agen ,
and carbonic anhydrase inhibi or, and sys-
emic ace azolamide (i.v. 250 o 500 mg) are
usually e ec ive in lowering he in raocular
pressure and abor ing he at ack. An osmo ic
agen (e.g., i.v. manni ol 1 o 2 g per kg over
45 minu es) may be considered i in raocular
pressure con rol is subop imal.
In pa ien s no responsive o medical
rea men , argon laser peripheral iridoplas y
(ALPI) can be applied rom 180 degrees o
360degrees a he ar peripheral iris o pull
open he closed angle mechanically. T e spo
size is usually se a 500 µm wi h dura ion o
0.5 seconds and energy o 200 o 400 mJ or
each applica ion. T e op imal energy should
be i ra ed wi h an end poin o visualizing he
con rac ion o he peripheral iris. Charring
o he iris wi h excessive laser energy should
be avoided. ALPI alone is a sa e and e ec ive
al erna ive in abor ing acu e angle closure.
Once he corneal edema has cleared, a laser
peripheral irido omy (LPI) can be per ormed
o preven recurren at ack. By providing a
communica ing channel, LPI reduces he
pressure gradien be ween he an erior and he
pos erior chambers and at ens he iris (Fig.
17-11). LPI should be considered in he el-
low eye because o an associa ed increased risk
o developing acu e angle closure.
Pa ien s should be moni ored or develop-
men o peripheral an erior synechiae, chronic
eleva ion o in raocular pressure, and angle-
closure glaucoma in he ollow-up visi s.

CHRONIC ANGLE–CLOSURE
GLAUCOMA
Diagnostic Evaluation
T e diagnosis o angle-closure glaucoma
requires he de ec ion o primary angle clo-
sure oge her wi h evidence o glaucoma ous
op ic neuropa hy. T e eye is usually quie
wi h a shallow an erior chamber.
Gonioscopy demons ra es a narrow angle
and areas o peripheral an erior synechiae.
Advanced cases may have lit le rabecular
meshwork visible. T e op ic nerve head may
show ypical glaucoma ous cupping and pro-
gressive narrowing o he neurore inal rim
and hinning o he re inal nerve f ber layer
similar o open-angle glaucoma.
Treatment
Preven ing acu e angle closure and reduc-
ing he ra e o glaucoma progression are he
key objec ives in he managemen o angle-
closure glaucoma.
An LPI can widen he angle and preven
ur her angle closure. However, he rabecular
meshwork may have sus ained enough dam-
age ha he in raocular pressure will s ill be
eleva ed despi e a pa en irido omy, necessi-
a ing con inued use o opical medica ions o
lower he in raocular pressure.
In pa ien s wi h coexis ing ca arac , lens
removal can widen he angle and lower he
in raocular pressure (Fig. 17-12).
PLATEAU IRIS
I n pla eau iris conf guration, he iris is dis-
placed an eriorly a i s roo by large or
abnormally posi ioned ciliary processes. A
componen o rela ive pupillary block may
also be presen , par icularly in older individu-
als. T e rabecular meshwork may be occluded
i he displacemen is an erior enough. Pla eau
Primary Acute Angle Closure 277
iris syndrome is def ned by a persis en narrow
angle despi e a pa en LPI. In he comple e
orm, here is occlusion o rabecular mesh-
work and he in raocular pressure increases. In
he incomple e orm, he upper por ion o he
f l ering rabecular meshwork is open and he
in raocular pressure remains normal.
Diagnostic Evaluation
Pla eau iris ypically occurs in he our h
hrough six h decades in women.
Symp oms, as wi h angle closure second-
ary o rela ive pupillary block, are dependen
on he rapidi y o he angle closure. An acu e
at ack may occur i a componen o rela ive
pupillary block exis s; he symp oms will mir-
ror hose o acu e angle closure. In mos cases,
he angle closes slowly and here are no symp-
oms un il he in raocular pressure is eleva ed
or he visual f eld loss becomes severe.
Under sli -lamp examina ion, he iris is
a and he cen ral an erior chamber is deep.
Compression gonioscopy demons ra es he
“double-hump” sign wi h he peripheral hump
represen ing a prominen roll o iris pushed
orward by he ciliary processes, and he cen-
ral hump represen ing he por ion o iris over
he an erior lens sur ace (Fig. 17-13).
UBM is use ul o conf rm he diagnosis o
pla eau iris (Fig. 17-14).
Treatment
No in erven ion is needed or pla eau iris
conf gura ion i no obs ruc ion o he ra-
becular meshwork is occurring. However, an
LPI may be indica ed i here is an elemen o
rela ive pupillary block.
In pla eau iris syndrome, ALPI is use ul
o open apposi ionally closed angle. ypical
rea men includes approxima ely 20 o 30
spo s o argon laser placed in he ar periphery
over 360 degrees. Fil ra ion surgery may ul i-
ma ely become necessary in some pa ien s.
 278 17 PRIMARYACUTE ANGLE– CLOSURE AND CHRONIC ANGLE– CLOSURE GLAUCOMA

FIGURE 17-7. Iris bombé. Sli -beam pho ograph showing he appearance o iris bombé. T is eye has iris bombé
rom uvei is causing 360 degrees o pos erior synechiae (scarring be ween he pupillary margin and he an erior
sur ace o he in raocular lens) , causing secondary pupillary block by mechanically blocking aqueous f ow
hrough he pupil.

A B
FIGURE 17-8. Primar y acute angle closure. A pa ien wi h primary acu e angle closure and a rela ively clear
cornea. A. T e sli -beam pho ograph o he gonioscopic view shows he s eep approach o he iris in his case o
acu e angle closure (iris bombé). B. T e di use illumina ion o he gonioscopic view o he same eye shows no
angle s ruc ures (i.e., an occluded angle) .
 Primary Acute Angle Closure 279

A B
FIGURE 17-9. Narrow anterior chamber angle. A. Sli -beam pho ograph showing a narrow an erior
chamber angle. B.T e gonioscopic view o he same eye showing he absence o angle s ruc ures in an eye wi h
normal in raocular pressure. T is angle is occludable. (Cour esy o Douglas J. Rhee, MD, Wills Eye Hospi al,
Philadelphia, PA.)

FIGURE 17-10. Glaukom ecken. Sli -lamp pho ograph o an eye 2 mon hs a er acu e angle–closure at ack
shows f ecks o an erior subcapsular opaci ies.
 280 17 PRIMARYACUTE ANGLE– CLOSURE AND CHRONIC ANGLE– CLOSURE GLAUCOMA

A B

FIGURE 17-11. Ef ect o LPI on the anterior chamber angle. OC imaging ( Visan e OC , Carl Zeiss
Medi ec, Dublin, CA) o an eye wi h narrow angle be ore (A) and a er (B) LPI. A er LPI, he angle is widened
and he iris is f at ened.

A B

FIGURE 17-12. Ef ect o lens extraction on the anterior chamber angle. OC imaging (Casia OC , omey,
Nagoya, Japan) o an eye wi h chronic angle–closure glaucoma be ore (A) and a er (B) ca arac ex rac ion. T e
angle is widened and he iris is f at ened a er ca arac ex rac ion.
 Primary Acute Angle Closure 281

A B

C
FIGURE 17-13. Plateau iris con guration. A. Sli -beam pho ograph showing a rela ively deep an erior chamber.
T ere is a pa en peripheral irido omy superiorly. B. Gonioscopy wi h no pressure on he cornea; no angle
s ruc ures are visible. T e black arrow shows a prominen las iris old. C. Inden a ion gonioscopy. T e arrows pointing
up shows he same iris old seen in B; he arrowheads showing he rabecular meshwork now revealed behind he
prominen iris old. T e image is dis or ed because o he corneal s riae induced by he inden a ion. (Cour esy o
Douglas J. Rhee, MD, Wills Eye Hospi al, Philadelphia, PA.)

FIGURE 17-14. UBM o the same eye shown in Figure 17-13. T e arrow shows he an eriorly displaced ciliary
body causing a prominen iris roll direc ly above i in his pic ure. (Cour esy o Douglas J. Rhee, MD, Wills Eye
Hospi al, Philadelphia, PA.)
 C H AP T ER
18
Secondary Angle-closure Glaucoma
NEOVASCULAR GLAUCOMA
N eovascular glaucoma (NVG) is a sec-
ondary closed-angle orm o glaucoma.
Ini ially, a f brovascular membrane grows over
he rabecular meshwork. T is is an occluded
bu open angle. Wi hin a shor period o ime,
he f brovascular membrane con rac s, closing
he an erior chamber angle. T is o en leads o
a drama ic eleva ion o in raocular pressure,
usually grea er han 40 mm Hg.
Epidemiology and Pathophysiology
T e exac incidence o all NVGs is no
known. NVG can occur as he sequela o sev-
eral di eren possible condi ions, mos com-
monly, ischemic cen ral re inal vein occlusions
and proli era ive diabe ic re inopa hy.
O her predisposing ac ors include isch-
emic cen ral re inal ar erial occlusions, ocular
ischemic syndrome, branch re inal ar erial or
vein occlusions, chronic uvei is, chronic re i-
nal de achmen s, and radia ion herapy.
Some o he bes es ima es o he inci-
dence o NVG come rom s udies on cen-
ral re inal vascular occlusions (CRVOs).
282
Douglas J. Rhee and Jamie E. Nicholl
Approxima ely one- hird o all CRVOs are
ischemic. Be ween 16% and 60%, depending
on he ex en o capillary nonper usion, o
ischemic CRVOs will develop neovascu-
lariza ion o he iris. Approxima ely 20% o
eyes wi h proli era ive diabe ic re inopa hy
will develop NVG. Approxima ely 18% o
eyes wi h cen ral re inal ar erial occlusions
will develop neovasculariza ion o he iris
(Fig. 18-1). Eyes wi h neovasculariza ion
o he iris are a high risk or developing
NVG.
History
Pa ien s may be asymp oma ic or may
complain o pain, red eye, and decreased
vision.
Clinical Examination
Sli lamp: Corneal edema may be presen
in he an erior chamber rom eleva ed in ra-
ocular pressure. T e an erior chamber is usu-
ally deep wi h some are. Hyphema and rare
whi e cells may be presen . Fine, nonradial
vessels are presen on he iris (Fig. 18-1).
Gonioscopy: I he cornea is clear, gonios-
copy may show a vascular ne over he angle

in he early s ages (Fig. 18-2). La er, broad
peripheral an erior synechiae occluding some
or all o he angles may be seen.
Pos erior pole: Re inal f ndings are consis-
en wi h he underlying pa hology.
Management
ypically, medical managemen is no ade-
qua e in con rolling he in raocular pressure.
Surgical in erven ion is usually required.
Op ions include rabeculec omy wi h an
an if bro ic agen , a glaucoma drainage implan
device, and cyclodes ruc ive procedures.
BIBLIOGRAPH Y
Anchala AR, Pasquale LR. Neovascular glaucoma: A his-
orical perspec ive on modula ing angiogenesis. Semin
Ophthalmol. 2009;24:106–112.
Chappelow AV, an K, Waheed NK, e al. Panre inal pho-
ocoagula ion or proli era ive diabe ic re inopa hy:
Pat ern scan laser versus argon laser. Am J Ophthalmol.
2012;153:137–142.
Chan CK, Ip MS, Vanveldhuisen PC, e al.; SCORE
S udy Inves iga or Group. SCORE S udy repor #11:
Incidences o neovascular even s in eyes wi h re inal
vein occlusion. Ophthalmology. 2011;118:1364–1372.
Ci ci S, Sakalar YB, Unlu K, e al. In ravi real bevacizu-
mab combined wi h panre inal pho ocoagula ion in
he rea men o open angle neovascular glaucoma. Eur
J Ophthalmol. 2009;19:1028–1033.
Diabe ic Re inopa hy S udy Research Group. Preliminary
repor on e ec s o pho ocoagula ion herapy. Am J
Ophthalmol. 1976;81:383.
Eid M, Radwan A, el-Manawy W, e al. In ravi real beva-
cizumab and aqueous shun ing surgery or neovascu-
lar glaucoma: Sa e y and e cacy. Can J Ophthalmol.
2009;44:451–456.
Ghosh S, Singh D, Ruddle JB, e al. Combined diode laser
cyclopho ocoagula ion and in ravi real bevacizumab
(Avas in) in neovascular glaucoma. Clin Experiment
Ophthalmol. 2010;38:353–357.
Neovascular Glaucoma 283
Hayreh SS, Podhajsky P. Ocular neovasculariza ion wi h
re inal vascular occlusion II. Occurrence in cen ral
and branch re inal ar ery occlusion. Arch Ophthalmol.
1982;100:1585.
Laa ikainen L, Kohner EM, Khoury D, e al. Panre inal
pho ocoagula ion in cen ral re inal vein occlusion: A
randomized con rolled clinical s udy. Br J Ophthalmol.
1977;61:741.
Magargal LE, Brown GC, Augsburger JJ, e al. E cacy o
panre inal pho ocoagula ion in preven ing neovascu-
lar glaucoma ollowing ischemic cen ral re inal vein
obs ruc ion. Ophthalmology. 1982;89:780.
Miki A, Oshima Y, O ori Y, e al. One-year resul s o in ra-
vi real bevacizumab as an adjunc o rabeculec omy
or neovascular glaucoma in eyes wi h previous vi rec-
omy. Eye (Lond). 2011;25:658–659.
Ne land PA, Ishida K, Boyle JW. T e Ahmed Glaucoma
Valve in pa ien s wi h and wi hou neovascular glau-
coma. J Glaucoma. 2010;19:581–586.
Park UC, Park KH, Kim DM, e al. Ahmed glaucoma valve
implan a ion or neovascular glaucoma a er vi rec-
omy or proli era ive diabe ic re inopa hy. JGlaucoma.
2011;20:433–438.
akihara Y, Ina ani M, Kawaji , e al. Combined in ra-
vi real bevacizumab and rabeculec omy wi h mi o-
mycin C versus rabeculec omy wi h mi omycin
C alone or neovascular glaucoma. J Glaucoma.
2011;20:196–201.
olen ino M. Sys emic and ocular sa e y o in ravi real
an i-VEGF herapies or ocular neovascular disease.
Surv Ophthalmol. 2011;56:95–113.
Shazly A, La ina MA. Neovascular glaucoma: E iology,
diagnosis and prognosis. Semin Ophthalmol.
2009;24:113–121.
Sido i PA, Dunphy R, Baerveld G, e al. Experience wi h
he Baerveld glaucoma implan in rea ing neovascu-
lar glaucoma. Ophthalmology. 1995;102:1107–1118.
Yildirim N, Yalvac IS, Sahin A, e al. A compara ive
s udy be ween diode laser cyclopho ocoagula ion
and he Ahmed glaucoma valve implan in neovas-
cular glaucoma: A long- erm ollow-up. J Glaucoma.
2009;18:192–196.
Yalvac IS, Eksioglu U, Sa ana B, e al. Long- erm resul s o
Ahmed glaucoma valve and Mol eno implan in neo-
vascular glaucoma. Eye (Lond). 2007;21:65–70.
 284 18 SECO NDARY ANGLE– CLO SURE GLAUCO MA

FIGURE 18-1. Neovascularization of the iris. Neovasculariza ion o he iris f ne, noncircular vessels is seen
near he papillary border ex ending on o he iris.

FIGURE 18-2. Neovascularization of the iris. Gonioscopic pho o showing neovasculariza ion f ne, nonradial
vessels over he rabecular meshwork be ore he f brovascular membrane has con rac ed causing peripheral
an erior synechiae.

IR
IRIDO
R ID
DO C
COOR
RNN EAL
EA
AL
SYN
SY
YN DRO
D RO
O MES
MES
T he iridocorneal (ICE) syndrome is a
group o secondary angle–closure glau-
comas wi h overlapping ea ures. T ere are
hree en i ies wi hin his syndrome:
Essen ial iris a rophy (Figs. 18-3 to 18-5)
Chandler’s syndrome (Fig. 18-6)
Cogan–Reese syndrome (iris nevus;
Fig. 18-7)
EPIDEMIOLOGY
ICE syndrome is rare; he exac incidence
is no known. ypically, i a ec s middle-aged
women in one eye.
PATHOPHYSIOLOGY
All hree o he ICE syndrome en i ies
share a common pa hophysiology. T e cor-
neal endo helium grows abnormally over
he an erior chamber angle covering he iris,
which gives he iris he charac eris ic f ndings.
Ini ially, he an erior chamber angle is open
bu occluded. Over ime, he endo helial
membrane con rac s, secondarily closing he
angle and dis or ing he pupil and iris.
HISTORY
Pa ien s are usually asymp oma ic in he
early s ages. La er, he pa ien may no ice
decreased vision in one eye and irregular
appearance o he iris. As he in raocular pres-
sure rises, he pa ien may have pain or a red
eye, or bo h.
CLINICAL EXAMINATION
Sli lamp: T e cornea has a f ne, bea en-
me al appearance in he endo helial layer
Iridocorneal Syndromes 285
unila erally. T ere are iris abnormali ies ha
are more specif c o he separa e en i ies:
Essen ial iris a rophy— here are areas
o hinning and a displaced and dis or ed
pupil as he endo helial membrane con-
rac s, pulling on he iris.
Chandler’s syndrome— he iris changes
are nearly iden ical o hose o essen ial
iris a rophy, bu here is a grea er degree o
corneal edema, and he corneal f ndings are
more apparen .
Cogan–Reese syndrome— he iris has a
at ened appearance wi h small nodules o
normal iris issue poking hrough holes in
he endo helial layer, giving he appearance
o a mushroom pa ch.
Gonioscopy: Early in he disease process,
gonioscopy may show a normal-appearing
an erior chamber angle. La er, broad and
irregular peripheral an erior synechiae
occluding some or all o he angles may be
seen.
Pos erior pole: T e appearance o he pos-
erior pole is normal, aside rom some degree
o glaucoma ous op ic nerve cupping as he
in raocular pressure rises.
MANAGEMENT
ypically, medical managemen is no ade-
qua e in con rolling he in raocular pressure.
Surgical in erven ion is usually required.
Op ions include rabeculec omy wi h an
an if bro ic agen , a glaucoma drainage implan
device, and cyclodes ruc ive procedures.
Corneal ransplan a ion is help ul once he
corneal edema has signif can ly a ec ed he
pa ien ’s vision.
BIBLIOGRAPH Y
Alvarado JA, Underwood JL, Green WR, e al.
De ec ion o herpes simplex viral DNA in he iri-
docorneal endo helial syndrome. Arch Ophthalmol.
1994;112:1601–1609.
 286 18 SECO NDARY ANGLE– CLO SURE GLAUCO MA
Alvim P , Cohen EJ, Rapuano CJ, e al. Pene ra ing kera -
oplas y in iridocorneal endo helial syndrome. Cornea.
2001;20:134–140.
Anderson NJ, Badawi DY, Grossniklaus HE, e al.
Pos erior polymorphous membranous dys rophy wi h
overlapping ea ures o iridocorneal endo helial syn-
drome. Arch Ophthalmol. 2001 119:624–625.
Doe EA, Budenz DL, Gedde SJ, e al. Long- erm surgical
ou comes o pa ien s wi h glaucoma secondary o he
iridocorneal endo helial syndrome. Ophthalmology.
2001;108:1789–1795.
Groh MJ, Sei z B, Schumacher S, e al. De ec ion o
herpes simplex virus in aqueous humor in irido-
corneal endo helial (ICE) syndrome. Cornea. 1999;
18:359–360.
Grupcheva CN, McGhee CN, Dean S, e al. In vivo
con ocal microscopic charac eris ics o iridocorneal
endo helial syndrome. Clin Experiment Ophthalmol.
2004;32:275–283.
Hirs LW. Bila eral iridocorneal endo helial syndrome.
Cornea. 1995;14:331.
Hooks JJ, Kup er C. Herpes simplex virus in irido-
corneal endo helial syndrome. Arch Ophthalmol.
1995;113:1226–1228.
A B
FIGURE 18-3. Essential iris atrophy. A. Essen ial iris a rophy showing pulling and dis or ion o he pupil.
B. Sli -beam pho ograph o he same eye.
Huang , Wang Y, Ji J, e al. Deep lamellar endo helial
kera oplas y or iridocorneal endo helial syndrome in
phakic eyes. Arch Ophthalmol. 2009;127:33–36.
Kup er C, Kaiser-Kup er MI, Da iles M, e al. T e con ral-
a eral eye in he iridocorneal endo helial (ICE) syn-
drome. Ophthalmology. 1983;90:1343–1350.
Lanzl IM, Wilson RP, Dudley D, e al. Ou come o
rabeculec omy wi h mi omycin-C in he irido-
corneal endo helial syndrome. Ophthalmology. 2000;
107:295–297.
Lobo AM, Rhee DJ. Delayed in erval o involvemen
o he second eye in a male pa ien wi h bila eral
Chandler’s syndrome. Br J Ophthalmol. 2012;96:134–
135, 146–147.
Olawoye O, eng CC, Liebmann JM, e al. Iridocorneal
endo helial syndrome in a 16-year-old. J Glaucoma.
2011;20:294–297.
Price MO, Price FW Jr. Desceme s ripping wi h endo he-
lial kera oplas y or rea men o iridocorneal endo he-
lial syndrome. Cornea. 2007;26:493–497.
Shields MB. Progressive essen ial iris a rophy, Chandler’s
syndrome, and he iris nevus (Cogan-Reese) syn-
drome: A spec rum o disease. Surv Ophthalmol. 1979;
24:3–20.
 Iridocorneal Syndromes 287

A B
FIGURE 18-4. Essential iris atrophy. A. Ano her example o essen ial iris a rophy. B. Gonioscopic view o he
same eye, showing peripheral an erior synechiae.

A B
FIGURE 18-5. Essential iris atrophy. A. Ex reme example o essen ial iris a rophy. B. Gonioscopic view o he
same eye, showing peripheral an erior synechiae.
 288 18 SECO NDARY ANGLE– CLO SURE GLAUCO MA

A B

FIGURE 18-6. Chandler’s syndrome. A. T e iris f ndings are similar o essen ial iris a rophy excep ha he
corneal f ndings are more prominen . B. Sli -beam pho ograph o he same eye.

FIGURE 18-7. Advanced iris nevus syndrome. Sli -lamp pho ograph o an individual wi h an advanced case
o iris nevus syndrome. T e emporal aspec o he iris clock hours 8 o 9 shows loss o he normal cryp s o
he iris. T e small brown do s are u s o normal iris issue poking hrough he abnormal corneal endo helial
membrane. From clock hours 9 o 11, he membrane has re rac ed, causing s re ch ears in he iris. T e
subconjunc ival hemorrhage is a resul o his pa ien ’s recen guarded f l ra ion surgery.
 Aqueous Misdirection Syndrome (Malignant Glaucoma)
AQ
A Q UE
UEO
EO UUSS MISD
MISDIREC
D IR
R ECC IO N
SYN
S YN DRO
D R O ME
ME ( M
MALIGNAN
ALL IGNAAN
GLAUCO
G LAUU COO MA)
M A)
T his syndrome usually occurs ollowing
pene ra ing surgery o he eye, al hough
i has cer ainly been repor ed ollowing laser
procedures.
EPIDEMIOLOGY
In 1951, Chandler repor ed he incidence
o malignan glaucoma o be 4% o eyes
undergoing glaucoma surgery.
Since hen, f l ering surgery has under-
gone some changes, and i is he impression
o many clinicians ha malignan glaucoma
occurs less requen ly in modern imes.
PATHOPHYSIOLOGY
I is believed ha he in erven ion in he
eye changes he direc ion o aqueous humor
ow. Ins ead o moving orward around he
pupil, he aqueous goes in o he vi reous. T is
causes a at ening o he an erior chamber
angle and a rela ively high or rankly high
in raocular pressure (Fig. 18-8). Rela ively
high can be considered grea er han 8 mm Hg.
ypically, a a an erior chamber is he
resul o overf l ra ion causing hypo ony
and choroidal de achmen s. One would
no expec an in raocular pressure grea er
han 10 mm Hg wi h a a an erior cham-
ber. Some imes he pressure can be over ly
eleva ed (more han 30 mm Hg).
HISTORY
ypically, here is a recen his ory
o eye surgery. T e pa ien has blurry
vision rom an erior movemen o he iris
or lens complex, bu his may be di cul
289
o dis inguish rom normal pos opera ive
blurring o vision.
Unless he in raocular pressure is rankly
eleva ed, here is usually no pain.
CLINICAL EXAMINATION
Sli lamp: T e an erior chamber is evenly
narrow. T ere is no iris bombé. I a glaucoma-
f l ering procedure has been per ormed, he
bleb is usually low wi h no evidence o wound
leak. T e in raocular pressure is as discussed
earlier. Corneal edema may be presen i he
in raocular pressure is markedly eleva ed, or i
here is lens–corneal con ac .
Gonioscopy: Usually, gonioscopy is no
possible secondary o obvious ICE con ac .
Pos erior pole: T e hallmark o he disease
is ha here are no choroidals.
SPECIAL STUDIES
Ul rasound biomicroscopy can be qui e
help ul. I will ypically show at ening o
he ciliary body processes and no an erior
choroidals.
MANAGEMENT
O en, he episode can be rea ed medi-
cally wi h opical cycloplegics and aqueous
suppressan s. Surgical in erven ion may be
required i medical managemen ails.
T e key componen o resolving he
at ack is disrup ion o he an erior hyaloid
ace. Some imes his can be done using lasers
i he an erior hyaloid ace can be visualized
peripheral o he lens or in raocular lens
implan .
I his is no possible, a pars plana vi rec-
omy may be required. During he pars plana
vi rec omy, he re inal surgeon mus be aware
o he need o break he an erior hyaloid ace.
 290 18 SECO NDARY ANGLE– CLO SURE GLAUCO MA

BIBLIOGRAPH Y epidemiological s udy o diabe es melli us on he

Chandler PA. Malignan glaucoma. Am J Ophthalmol. island o Fals er, Denmark. Acta Ophthalmol Scand.
1951;34:993. 1983;27:662.
Nielsen NV. T e prevalence o glaucoma and ocular
hyper ension in ype 1 and 2 diabe es melli us: An

A B

C D
FIGURE 18-8. Aqueous misdirection following glaucoma drainage device implantation. A–C. Ul rasound
biomicroscopy o a pa ien wi h aqueous misdirec ion syndrome ollowing a glaucoma drainage device
implan a ion procedure. In panels A and B, he s ar indica es he cornea. A. T e arrow shows he an erior lens
capsule; his cen ral view shows a shallow an erior chamber wi h ICE ouch o he papillary margin. B. T e
arrow shows he at ened ciliary body processes diagnos ic o aqueous misdirec ion syndrome; his view o he
angle also shows he ICE ouch. C. A magnif ed view o he at ened ciliary processes. Appearance a er limi ed
vi rec omy. D and E. T e same pa ien ollowing limi ed vi rec omy wi h disrup ion o he an erior hyaloid
ace. T e star indica es he cornea while he arrow indica es he an erior capsule o he lens. D. Deepening o
he an erior chamber angle; he ube can be seen lying a on he iris.
(continued)
 Aqueous Misdirection Syndrome (Malignant Glaucoma) 291

FIGURE 18-8. Continued Aqueous misdirection

E following glaucoma drainage device implantation.
E. A cen ral view showing he deep an erior chamber.
 C H AP T ER
19
Glaucoma Secondary o Eleva ed
Venous Pressure
Douglas J. Rhee, Ribhi Hazin, and Louis R. Pasquale
T his group o disorders shares a common
mechanism o disease— he glaucoma is
he resul o an eleva ion o episcleral venous
pressure causing he eleva ed in raocular pres-
sure (IOP). Mos cases can be at ribu ed o
caro id-cavernous sinus f s ulas, cavernous
sinus (CS) hrombosis, dural ar eriovenous
(AV) shun s, superior vena cava syndrome,
S urge–Weber syndrome (SWS), hyroid
oph halmopa hy, orbi al obs ruc ive lesions,
or orbi al varices.
CAROTID-CAVERNOUS
FISTULA
C aro id-cavernous f s ula (CCF) describes
he po en ially blinding AV communica-
ion be ween he caro id ar ery or i s branches
and he CS.
Classif cation
CCF is classif ed as ei her a direct CCF
in which highly pressurized blood rom he
caro id ar ery is direc ly shun ed in o he
292
venous CS or an indirect CCF, which devel-
ops as a resul o communica ion be ween
smaller, low-pressure ar erial branches and
veins o he CS.1
Direc CCFs end o be “high ow” and
ypically arise in he set ing o rauma, while
indirec CCFs end o be “low ow” and ypi-
cally arise congeni ally, during pregnancy, or
spon aneously in pos -menopausal women.1
Pathophysiology
Individuals wi h diseases like Ehlers–
Danlos syndrome, collagen def ciency syn-
dromes, or o her condi ions ha weaken he
in egri y o vessel walls may be a increased
risk or spon aneous developmen o CCFs.
CCFs al er ocular hemodynamics in a
way whereby he high- ow, high-pressure
prof le o he caro id ar ery is ransmit ed o
orbi al and ocular s ruc ures o produce a
cons ella ion o ocular signs and symp oms.
T ese signs and symp oms are direc ly pro-
por ional o he degree o abnormal commu-
nica ion be ween ar erial and venous blood
in he CS.

T is orm o venous hyper ension yields
he classic clinical riad or CCF:
Unila eral exoph halmos which may be
pulsa ile in high- ow direc CCFs
Ocular or cephalic brui which is more
audible in high- ow CCFs
Injec ion and chemosis o he conjunc-
iva which is more pronounced in high-
ow CCFs.1
Clinical Presentation
Pa ien s wi h direc CCF have more pro-
nounced complain s o headaches, diplopia,
epis axis, and visual loss han pa ien s wi h
indirec CCFs.1,2
Indirec f s ulas are associa ed wi h a myriad
o clinical f ndings ha are more subacu e or
chronic in na ure. T e presen a ion o he indi-
rec CCF may include ocular redness and swell-
ing ha can be ini ially cons rued as an ocular
in ec ion. Visual acui y and color vision can
be compromised in a gradual manner. T ere
can be ex raocular muscle imbalance rela ed o
compression o he hird and/ or six h cranial
nerve in he CS. T e prop osis in indirec f s u-
las is ypically no pulsa ile in na ure.
Diagnostic Evaluation
Sli -lamp exam will o en reveal ar erial-
ized and or uous, corkscrew conjunc ival
vessels (Fig. 19-1), and shallow an erior
chamber. T e corkscrew appearance dis in-
guishes his condi ion rom episcleri is where
he vessels have a crossing pat ern or scleri is
where here is a deep violaceous hue o ocular
sur ace issues.
Carotid-Cavernous Fistula 293
T e IOP is o en eleva ed and wide
mires can be apprecia ed while he IOP is
measured.
On gonioscopy, he f l ra ion appara us
may be apposi ionally closed and blood re ux
may be no ed where he angle is open. T e
undus may exhibi an impending re inal
venous occlusive pic ure wi h or uous ves-
sels and in rare inal hemorrhage. T e eleva ed
IOP in CCF cases is o en re rac ory o medi-
cal herapy.3
Orbi al imaging wi h C or MRI will
reveal enlargemen o he ex raocular muscles
and engorgemen o superior oph halmic vein
(Fig. 19-2). Wi h con ras , enhancemen o
CS will be eviden . Caro id angiography is
o en diagnos ic in revealing he exac f s ula
si e.
Management
T e rea men or bo h direc and indirec
CCFs is he same: endovascular emboliza-
ion using balloons or de achable coils or
s en ing or bo h ypes o f s ulas is ypically
def ni ive in resolving mos signs and symp-
oms (Fig. 19-3).
A more conserva ive approach is adop ed
or indirec CCFs because spon aneous
regression can occur. However, in cases o
indirec CCF where here is evolu ion o pro-
gressive ocular and orbi al conges ion wi h
compromise o vision and in rac ably eleva ed
IOP, def ni ive closure is indica ed.1,4
Fil ra ion surgery is o be avoided in eyes
wi h CCFs i a all possible.
 294 19 GLAUCO MA SECO NDARY TO ELEVATED VENO US PRESSURE

FIGURE 19-1. Arterialization o conjunctival vessels in a 78-year-old patient with a spontaneous indirect CCF
prior to endovascular embolization with detachable coil. (Courtesy o Dr. Peter Veldman.)

FIGURE 19-2. Magnetic resonance angiography o the patient in Figure 19-1 revealing an enlarged right
superior ophthalmic vein. (Courtesy o Dr. Peter Veldman.)
 Carotid-Cavernous Fistula 295

FIGURE 19-3. Improved clinical appearance o the patient in Figure 19-1 with marked reduction o conjunctival
arterialization ollowing endovascular embolization with detachable coil. (Courtesy o Dr. Peter Veldman.)
 296 19 GLAUCO MA SECO NDARY TO ELEVATED VENO US PRESSURE
STURGE–WEBER SYNDROME
S WS describes he congeni al neurocu a-
neous condi ion charac erized by a riad
o neuropsychia ric, derma ological, and
oph halmological mani es a ions. I is o en
re erred o as he “ our h phacoma osis” bu
unlike he o her phacoma oses, i has no
known inheri ance pat ern.
Etiology
During developmen here is an abnormal
vascular plexus adjacen o he neural ube
during developmen . T is vascular nexus ails
o regress and is dragged o various sur ace
ec odermal and neuroec odermal loca ions
during developmen . T ese loci o aberran
vascular issue con ribu e o he clinical mani-
es a ions in SWS.
Visual Loss
T e mos impor an cause o vision loss
in SWS is he developmen o glaucoma.
As many as 30% o 70% o individuals wi h
SWS develop glaucoma, wi h 60% o cases
repor ed a bir h or in in ancy and 40%
repor ed in adolescence or young adul hood.1
When glaucoma is presen in he f rs year
o li e, he signs (including buph halmos) and
symp oms o congeni al glaucoma are o en
presen . I glaucoma does no develop by
adul hood, i is generally unlikely o develop
la er on, unless here is some o her predispo-
si ion o ano her ype o glaucoma.
Cases wi h glaucoma requen ly have an
episcleral vascular mal orma ion ha can be
qui e sub le (Fig. 19-4). As poin ed ou by
Aggarwal and associa es,2 on gonioscopy he
eye wi h glaucoma will o en have decreased
visibili y o he scleral spur and ciliary body
band (Fig. 19-5).
Eleva ed episcleral venous pressure sec-
ondary o he episcleral vascular mal orma-
ion appears o be he mos common cause
o glaucoma in SWS pa ien s.3 T e eye wi h
glaucoma will also o en have iris he erochro-
mia (wi h he darker iridis on he side wi h
he acial vascular mal orma ion).
Eyes wi h or wi hou glaucoma may har-
bor a choroidal vascular mal orma ion, giving
he re ina a “ oma o ke chup” appearance.
O her causes o visual loss in SWS include
serous re inal de achmen rom large choroi-
dal vascular mal orma ions and homonymous
hemianopsia.
Clinical Presentation
Clinically, pa ien s wi h SWS o en exhibi
a acial vascular mal orma ion or “por wine
s ain” ha arises secondary o AV mal orma-
ions in he skin. T ese skin lesions do no
necessarily respec a s ric derma omal dis ri-
bu ion (Fig. 19-6).
In some pa ien s, he por wine s ain
can hyper rophy wi h age. Some imes such
hyper rophy can make measuremen o IOP
challenging. Al hough he charac eris ic “por
wine s ain” in SWS is ypically unila eral i
can be bila eral as well.4
Management
Pulsed dye laser pho ocoagula ion is suc-
cess ul in mi iga ing he cosme ic e ec s o
cu aneous por wine lesions, and does no
con ribu e o clinically signif can changes in
IOP in SWS pa ien s.1
Medical herapy can be e ec ive in man-
aging SWS-induced glaucoma bu surgical
in erven ion is o en warran ed. In ins ances
o glaucoma occurring in in ancy, gonio omy
should be considered.
rabeculec omy is recommended in chil-
dren over he age o 3 years and in adul s.3
While prophylac ic pos erior sclero omy
is o en recommended o preven periopera-
ive choroidal hemorrhage in SWS pa ien s
requiring rabeculec omy, Eibschi z-
simhoni and colleagues repor ed ha in
 Sturge–Weber Syndrome 297

17 consecu ive pa ien s wi h ei her SWS or a REFERENCES

rela ed condi ion (Klippel– renaunay–Weber
syndrome), per ormance o rabeculec omy
wi hou prophylac ic sclero omy did no
resul in choroidal hemorrhage or e usion
requiring ur her surgical in erven ion.5
Cyclodesc ruc ion and glaucoma drainage
device implan a ion can also be considered
in uncon rolled glaucoma, depending on he
clinical scenario.
1. Sharan S, Swamy B, arana h DA, e al. Por -wine
vascular mal orma ions and glaucoma risk in S urge-
Weber syndrome. J AAPOS. 2009;13(4):374–378.
2. Aggarwal NK, Gandham SB, Weins ein R, e al.
He erochromia iridis and per inen clinical f ndings
in pa ien s wi h glaucoma associa ed wi h S urge-
Weber Syndrome. J Pediatr Ophthalmol Strabismus.
2010;47:361–365.
3. Pa rianakos D, Nagao K, Wal on DS. Surgical man-
agemen o glaucoma wi h he s urge weber syndrome.
Int Ophthalmol Clin. 2008;48(2):63–78.
4. Quan SY, Comi AM, Parsa CF, e al. E ec o a single
applica ion o pulsed dye laser rea men o por -wine
bir hmarks on in raocular pressure. Arch Dermatol.
2010;146(9):1015–1018.
5. Eibschi z- simhoni M, Lich er PR, Del Mon e MA,
e al. Assessing he need or pos erior sclero omy a he
ime o f l ering surgery in pa ien s wi h S urge-Weber
syndrome. Ophthalmology. 2003;110(7):1361–1363.

A B
FIGURE 19-4. Slit-lamp view o the episcleral vascular mal ormation o a 49-year-old man with SWS and
late-onset glaucoma. Note that the episcleral mal ormation can be subtle and construed as episcleritis i it were
unaccompanied by a port-wine stain. A. Lower magnif cation slit lamp view. B. Higher magnif cation view o the
in eronasal limbal area showing terminal end bulbs at the limbus di erentiating these as abnormal vessels.
 298 19 GLAUCO MA SECO NDARY TO ELEVATED VENO US PRESSURE

FIGURE 19-5. A. ypical appearance o the drainage angle in SWS with decreased visibility o scleral spur
and ciliary body band. Note the decreased visibility o the ciliary body band, scleral spur, and irregular border
o the peripheral iris. B. Gonioscopy o the anterior chamber angle o the contralateral eye o the same patient
showing the normal landmarks. (From Aggarwal NK, Gandham SB, Weinstein R, et al. Heterochromia iridis and
pertinent clinical f ndings in patients with glaucoma associated with Sturge-Weber syndrome. J Pediatr Ophthalmol
Strabismus. 2010;47:361–365, permission pending.)

FIGURE 19-6. Angiomatosis involving periocular skin and episclera in the same patient as in Figure 19 4.
Note that the periocular involvement does not ollow a strict dermatomal distribution.
 Idiopathic Elevated Episcleral Venous Pressure 299

IIDIO
DIOO PA
A H ICC ELEVA
E L E VA EDD o li e. T e dila ed vessels can be unila eral
EPISCLERAL
E PIS
SC LE
E R AL
L VEN
VE N O UUS
S or bila eral, usually wi h asymme ry even in
bila eral cases (Fig. 19-7).1
PRESSURE
P RE
E SSU
U RE
Pa ien s may have blood in Schlemm’s
Idiopa hic eleva ed episcleral venous pres- canal which is a generalized sign o eleva ed
sure (IEEVP) is a diagnosis o exclusion. An episcleral venous pressure (Fig. 19-8), bu
ex ensive his ory, clinical exam, and comple e his f nding is no necessary o es ablish he
diagnos ic evalua ion including radiologic diagnosis.
es ing should be under aken o de ec any
primary causes such as hose lis ed in he REFERENCE
in roduc ion o his chap er. 1. Rhee DJ, Gup a M, Moncavage M, e al. Idiopa hic
eleva ed episcleral venous pressure and open angle
Pa ien s wi h IEEVP have dila ed, or uous
glaucoma. Br J Ophthalmol. 2009;93:231–234; Epub
episcleral vessels wi h onse occurring sub- 2008 Jun 20.
acu ely ypically in he hird or our h decade

FIGURE 19-7. Bilateral case o IEEVP with greater involvement o the le eye. Images o the individual eyes.
 300 19 GLAUCO MA SECO NDARY TO ELEVATED VENO US PRESSURE

FIGURE 19-8. Blood in Schlemm’s canal.

OTHER CAUSES OF in roduc ion, an AV mal orma ion dis al o

ELEVATED EPISCLERAL he venous drainage o he eye can cause an
eleva ed IOP (Fig. 19-9).
VENOUS PRESSURE

Al hough mos pa ien s can be explained

by he di eren ial diagnosis lis ed in he

C
FIGURE 19-9. AV malformation. T is patient had bilateral AV mal ormations involving the trochlear arteries
that were discovered using C angiography. T e le side had greater involvement and received a trabeculectomy.
A. external photograph showing involvement o both eyes but with greater involvement o the le eye. B. view o
the right eye with C. the lid raised showing engorgement o episcleral veins. D–G. Images o the le eye ollowing
trabeculectomy surgery with ExPress shunt (Alcon, Ft. Worth, X) and Ologen (Aeon Astron, Netherlands)
implants.
( continued)
 Idiopathic Elevated Episcleral Venous Pressure 301

D E

F G

FIGURE 19-9. (Continued)

 C H AP T ER
20
In roduc ion o Glaucoma
Managemen
WH AT IS THE GOAL OF
TREATMENT ?
W e curren ly unders and he pa hophysi-
ology o glaucoma o be a progressive
loss o ganglion cells resul ing in visual f eld
damage ha is rela ed o in raocular pres-
sure. T e goal o rea men is o re ard or hal
he ganglion cell loss and preven symp om-
a ic visual loss while at emp ing no o cause
un oward side e ec s.
Al hough many clinicians now eel ha here
are several ac ors involved in he pa hogen-
esis o glaucoma, he only rigorously proven
me hod o rea men is he lowering o in raoc-
ular pressure. T ere con inues o be a moun -
ing body o evidence ha suppor s his ac .
HOW DO WE TREAT
GLAUCOMA?
G laucoma was f rs hough o as a surgi-
cal disease. T e f rs f l ra ion procedure
(no iridec omy) was sugges ed by Louis de
302
Douglas J. Rhee
Wecker (1832 o 1906) in 1869. Al hough he
mio ic e ec s o eserine and pilocarpine had
been repor ed in he early 1860s, hey were
no used or rea men un il la er. Adol Weber
(1829 o 1915) f rs in roduced hem as medi-
cal rea men s o glaucoma in 1876. T e f rs
s udy comparing he wo available orms o
glaucoma rea men , eserine and iridec omy,
was per ormed a Wills Eye Hospi al in 1895
by Zen mayer e al. T is s udy showed ha
bo h rea men s are equivalen and ha a
pa ien ’s visual s a us could be main ained or
periods ranging rom 5 o 15 years on chronic
medical rea men .
T e deba e over he bes ini ial herapy con in-
ues oday. In Europe, many clinicians per orm
surgery as he ini ial rea men or glaucoma.
Mos clinicians in he Uni ed S a es con-
inue o use medica ions as he ini ial rea -
men or glaucoma. In he Uni ed S a es, wo
large s udies were per ormed o compare
medical rea men wi h laser rabeculoplas y
[Glaucoma Laser rial (GL )] and medical
rea men wi h rabeculec omy [Collabora ive
Ini ial Glaucoma rea men S udy (CIG S)].

A 2 years o ollow-up in he GL , eyes ha
received argon laser rabeculoplas y (AL )
showed a lower mean in raocular pressure
(be ween 1 and 2 mm Hg) compared o eyes
s ar ed on imolol, bu showed no di er-
ence in visual f eld or acui y. A 7 years, eyes
ha received AL had a grea er reduc ion in
in raocular pressure (1.2 mm Hg) and grea er
sensi ivi y in he visual f eld (0.6 dB). T ese
resul s seem o indica e ha AL is a leas as
good as medical rea men or glaucoma.
Resul s rom he CIG S s udy show no di -
erence in visual f eld ou comes despi e a
lower in raocular pressure in he surgical
group. One excep ion no ed in CIG S was
ha ini ial surgery led o less visual f eld pro-
gression han ini ial medicine rea men in
pa ien s wi h advanced f eld loss a enroll-
men . Pa ien s wi h diabe es had more visual
f eld loss over ime i rea ed ini ially wi h
surgery. Smoking was also ound o in uence
f nal pressure–lowering responses. We do no
unders and why hese di erences exis in di -
eren subgroups. Overall, visual acui y and
local eye symp oms seem o be worse in he
surgical group. However, he CIG S resul s
do no unequivocally suppor changing he
curren paradigm o medical rea men as
ini ial rea men . However, longer ollow-up
da a are needed o provide more conclusive
recommenda ions or a chronic disease such
as glaucoma.
T e subsequen chap ers in his sec ion
describe he di eren herapies commonly
u ilized or glaucoma.
HowDo We Treat Glaucoma? 303
BIBLIOGRAPH Y
AGIS Inves iga ors. T e Advanced Glaucoma In erven ion
S udy (AGIS): 7. T e rela ionship be ween con rol o
in raocular pressure and visual f eld de eriora ion. Am
J Ophthalmol. 2000;130:429–440.
Bergea B, Bodin L, Svedbergh B. Impac o in raocular
pressure regula ion on visual f elds in open-angle glau-
coma. Ophthalmology. 1999;106:997–1005.
Bhorade AM, Wilson BS, Gordon MO, e al.; Ocular
Hyper ension rea men S udy Group. T e u il-
i y o he monocular rial: Da a rom he ocular
hyper ension rea men s udy. Ophthalmology. 2010;
117:2047–2054.
Collabora ive Normal- ension Glaucoma S udy Group.
Comparison o glaucoma ous progression be ween
un rea ed pa ien s wi h normal- ension glaucoma and
pa ien s wi h herapeu ically reduced in raocular pres-
sures. Am J Ophthalmol. 1998;126:487–497.
Glaucoma Laser rial Research Group. T e Glaucoma
Laser rial (GL ). 2. Resul s o argon laser rabecu-
loplas y versus opical medicines. Ophthalmology.
1990;97:1403–1413.
Glaucoma Laser rial Research Group. T e Glaucoma
Laser rial (GL ) and glaucoma laser rial ollow-up
s udy. 7. Resul s. Am JOphthalmol. 1995;120:718–731.
Janz NK, Wren PA, Lich er PR, e al.; CIG S S udy
Group. T e Collabora ive Ini ial Glaucoma rea men
S udy: In erim quali y o li e f ndings a er ini ial medi-
cal or surgical rea men o glaucoma. Ophthalmology.
2001;108:1954–1965.
Lich er PR, Musch DC, Gillespie BW, e al.; CIG S S udy
Group. In erim clinical ou comes in he Collabora ive
Ini ial Glaucoma rea men S udy comparing ini ial
rea men randomized o medica ions or surgery.
Ophthalmology. 2001;108:1943–1953.
Musch DC, Gillespie BW, Lich er PR, e al.; CIG S
S udy Inves iga ors. Visual f eld progression in he
Collabora ive Ini ial Glaucoma rea men S udy:
T e impac o rea men and o her baseline ac ors.
Ophthalmology. 2009;116:200–207.
Mao LK, S eward WC, Shield MB. Correla ion be ween
in raocular pressure con rol and progressive glauco-
ma ous damage in primary open-angle glaucoma. Am J
Ophthalmol. 1991;111:51–55.
Realini D. A prospec ive, randomized, inves iga or-
masked evalua ion o he monocular rial in ocular
hyper ension or open-angle glaucoma. Ophthalmology.
2009;116;1237–1242.
Zen mayer W, Posey WC. A clinical s udy o 167 cases o
glaucoma simplex. Arch Ophthalmol. 1895;24:378–394.
 C H AP T ER
21
Medical Managemen
M edical rea men o glaucoma began in
he la e 1800s wi h use o eserine and
pilocarpine. In he Uni ed S a es, glaucoma
rea men is ypically begun wi h opical med-
ica ions. T e shor - erm goal o medica ions is
o lower in raocular pressure (IOP). T e long-
erm goals are o preven symp oma ic visual
loss while minimizing he side ef ec s rom he
rea men s.
DESCRIPTION AND
PHYSIOLO GY
U nless here are ex reme circums ances,
such as an IOP higher han 40 mm Hg or
an impending risk o cen ral xa ion, rea men
is s ar ed using a so-called one-eyed herapeu-
ic rial. ypically, one ype o drop is s ar ed
in only one eye wi h reexamina ion in 3 o 6
weeks o check or ef ec iveness. Ef ec iveness
is de ermined by comparing he dif erence in
IOP in he wo eyes prior o herapy wi h he
dif erence in IOP a er ini ia ing herapy. For
example, i IOP is 30 mm Hg OD (oculus dex-
ter; in he righ eye) and 33 mm Hg OS (oculus
sinister; in he le eye) prior o rea men , and,
ollowing rea men o he righ eye, IOP is
304
Malik Y. Kahook and Douglas J. Rhee
20 mm Hg OD and 23 mm Hg OS, he drug
is no having any ef ec . I he IOP a er s ar -
ing rea men is 25 mm Hg OD and 34 mm Hg
OS, hen he drug is having an ef ec .
I should be no ed ha he monocular rial
when ins i u ing medical herapy has been he
opic o in ensive s udies over he pas ew years.
Realini has repor ed ha he monocular drug
rial is o lit le clinical value in pa ien s being
rea ed wi h la anopros . Bhorade and colleagues
came o a similar conclusion in heir s udy o
pros aglandin analogs. Regardless, i is common
prac ice o ini ia e medical herapy once glauco-
ma ous op ic neuropa hy is diagnosed and a rm
unders anding o he limi ed number o glau-
coma herapeu ics is necessary o choose he
bes herapy or each individual pa ien .
T ere are several dif eren classes o medi-
ca ions. All medica ions work o lower IOP
hrough varying pharmacologic mechanisms.
IOP is de ermined by he balance be ween
secre ion and drainage o aqueous humor.
All medica ions ei her decrease secre ion or
increase ou ow. In he subsequen sec ions,
he mechanism o ac ion, common side ef ec s,
and con raindica ions or he dif eren classes
o medica ions are presen ed. Table 21-1
 Description and Physiology 305

TABLE 21-1. Pharmacologic Agen s Organized by Pharmacologic Class

Medication* Available Strengths
Alpha Agonists
Apraclonidine (Iopidine) 0.5%, 1%
Brimonidine (Alphagan) 0.1%(with purite), 0.15%, 0.2%
Beta-blockers
Betaxolol (Betoptic) 0.5%
Carteolol (Ocupress) 1%
Levobunolol (Betagan) 0.25%, 0.5%
Metipranolol (OptiPranolol) 0.3%
Timolol hemihydrate (Betimol) 0.25%, 0.5%
Timolol maleate (Timoptic) 0.25%, 0.5%
Carbonic Anhydrase Inhibitors—Oral
Acetazolamide (Diamox) 125–500 mg
Methazolamide (Neptazane, Glauctabs) 25–50 mg
Carbonic Anhydrase Inhibitors—Topical
Brinzolamide (Azopt) 1%
Dorzolamide (Trusopt) 2%
Hyperosmolar Agents
Glycerin (Osmoglyn) 50%solution
Isosorbide (Ismotic) 4%solution
Mannitol (Osmitrol) 5%–20%solution
Miotics
Physostigmine (Eserine) 0.25%
Pilocarpine hydrochloride (Pilocarpine, Pilocar) 0.25%, 0.5%, 1%, 2%, 4%, 6%
Pilocarpine nitrate (Pilagan) 1%, 2%, 4%
Prostaglandins
Bimatoprost (Lumigan) 0.01%, 0.03%
Latanoprost (Xalatan) 0.005%
Travoprost (Travatan) 0.004%
Unoprostone isopropyl (Rescula) 0.15%
Sympathomimetic Agents
Dipivefrin (Propine) 0.1%
Epinephrine (Epif in) 0.5%, 1%, 2%
Combination Medications Available formulations
Dorzolamide/ Timolol (Cosopt) 2%/ 0.5%
Brimonidine/ Timolol (Combigan) 0.2%/ 0.5%
*Trade names available in the United States are indicated in italics.
 306 21 MEDICAL MANAGEMENT

lis s he medica ions wi hin each class ha are Side Ef ects

available in he Uni ed S a es as o 2010 o 2011.
T e side ef ec s and con raindica ions des-
cribed in his chap er are no a comple e lis -
ing. I recommend ha all clinicians read he
package inser be ore prescribing any medica-
ion. T e gures show sample bot les o medi-
ca ions available in he Uni ed S a es.
ALPH A AGONISTS
Mechanism o Action
Ac iva ion o alpha-2 recep ors in ciliary
body inhibi s aqueous secre ion
(Fig. 21-1).
Local irri a ion, allergy (Fig. 21-2),
mydriasis, dry mou h, dry eye, hypo ension,
and le hargy
Contraindications
Monoamine oxidase (MAO) inhibi or
use. Brimonidine is no o be used in children
younger han 2 years o age; i has been asso-
cia ed wi h apnea in children.
Comments
Apraclonidine is or shor - erm use and
prophylaxis o pos laser IOP spikes.

FIGURE 21-1. Alpha agonists. All rade-name alpha agonis s available in he Uni ed S a es a he ime o
publica ion. From lef o righ : Alphagan (Allergan; Irvine, CA), Alphagan-P (Allergan; Irvine, CA) , and
Iopidine 0.5% (Alcon; For Wor h, X) . No e: Iopidine 1% is no shown.
 Alpha Agonists 307

FIGURE 21-2. Allergic reaction from chronic brimonidine. ypical allergic reac ion rom brimonidine is
a ollicular conjunc ivi is ha occurs mon hs o years af er chronic use. T e prevalence is dose rela ed o he
concen ra ion o brimonidine wi h lower concen ra ions having lower prevalence o allergic reac ions. T is is an
ex reme case in which an ec ropion occurred as a resul o periorbi al skin excoria ion and brosis. T ese ndings
resolved spon aneously over a ew weeks o discon inuing he medica ion.
 308 21 MEDICAL MANAGEMENT

BETA-BLO CKERS TABLE 21-2. Rela ive Recep or

Selec ivi y o he Various Be a-blocker
Mechanism o Action Medica ions
Blockade o he be a recep ors in he cili- Relative Specificity of
ary body reduces IOP by decreasing aqueous Drug the Receptor Effect
humor produc ion (Fig. 21-3). Betaxolol Relatively cardioselective
Carteolol Nonselective; has intrinsic
Side Ef ects
sympathomimetic activity
Local: Blurred vision, corneal anes hesia, Levobunolol Nonselective (long half-life)
and super cial punc a e kera i is
Metipranolol Nonselective (whitetop)
Sys emic: Bradycardia or hear block,
Timolol hemihydrate Nonselective
bronchospasm, a igue, mood change, impo-
ence, decreased sensi ivi y o hypoglycemic Timolol maleate Nonselective
symp oms in insulin-dependen diabe ics,
worsening o myas henia gravis Comments
Some medica ions are considered non-
Contraindications
selec ive versus rela ively cardioselec ive
As hma, severe chronic obs ruc ive pul- (Table 21-2). T e rela ively cardioselec ive
monary disease (COPD), bradycardia, hear medica ions may have ewer pulmonary side
block, conges ive hear ailure (CHF), myas- ef ec s.
henia gravis

FIGURE 21-3. Beta blockers. Nearly all single-agen , rade-name be a-blockers available in he Uni ed S a es
a he ime o publica ion; Be agan 0.25% and Be op ic are missing. From lef o righ : Be agan 0.5% (Allergan;
Irvine, CA), Be imol 0.25% and 0.5%, respec ively (San en; ampere, Finland) , Be op ic-S (Alcon; For Wor h,
X) , Op iPranolol (Bausch & Lomb; Claremon , CA) , Ocupress (Novar is; A lan a, GA) , and imop ic XE
0.25% and 0.5%, respec ively ( Merck; Whi ehouse S a ion, NJ) .

CARBONIC ANHYDRASE
INHIBITORS
Mechanism o Action
Inhibi ion o he enzyme carbonic anhy-
drase decreases aqueous produc ion in he
ciliary body. When given paren ally, carbonic
anhydrase inhibi ors (CAIs) will also cause
dehydra ion o he vi reous (Figs. 21-4 and
21-5).
Side Ef ects
Local (wi h opical herapy): Bit er as e.
Sys emic
Carbonic Anhydrase Inhibitors 309
Wi h opical herapy: Diuresis, a igue,
gas roin es inal upse , S evens–Johnson
syndrome, heore ical risk o aplas ic anemia
Wi h sys emic herapy: Hypokalemia
and acidosis, renal s ones, pares hesias,
nausea, cramps, diarrhea, malaise, le h-
argy, depression, impo ence, unpleasan
as e, aplas ic anemia, S evens–Johnson
syndrome
Contraindications
Sul a allergy, hypona remia or hypokale-
mia, recen renal s ones, hiazide diure ics,
digi alis use
 310 21 MEDICAL MANAGEMENT

FIGURE 21-4. Oral CAIs. T e oral CAIs available in he Uni ed S a es a he ime o publica ion. From lef o
righ : Diamox (Lederle; PA) and me hazolamide ( generic made by Copley Pharmaceu ical; Can on, MA) . O her
companies have manu ac ured hese medica ions in heir generic orms in recen years.

FIGURE 21-5. Topical CAIs. All single-agen , rade-name opical CAIs available in he Uni ed S a es a he
ime o publica ion. From lef o righ : rusop , old and new bot le, respec ively ( Merck; Whi ehouse S a ion,
NJ) , and Azop (Alcon; For Wor h, X).
 Miotics 311

HYPEROSMOLAR AGENTS diaphragm o move an eriorly), decreased

Mechanism o Action
Dehydra es he vi reous and decreases
in raocular uid volume by osmo ically draw-
ing uid in o he in ravascular space. T e
agen s are given orally or in ravenously.
Side Ef ects
Manni ol: CHF, urinary re en ion in men,
backache, myocardial in arc ion, headache,
and men al con usion
Glycerin: Vomi ing; less likely o produce
CHF han manni ol, o herwise similar o
manni ol
Isosorbide: Same as glycerin excep ha i
is perhaps sa er in diabe ic pa ien s
Contraindications
CHF, diabe ic ke oacidosis (glycerin), sub-
dural or subarachnoid hemorrhage, preexis -
ing severe dehydra ion
MIOTICS
nigh vision, variable myopia, re inal ear
or de achmen , and possibly an erior sub-
capsular ca arac s
Sys emic: Rare
Indirec -ac ing cholinergic
Local: Re inal de achmen , ca arac ,
myopia, in ense miosis, angle closure,
increased bleeding pos surgery, punc al
s enosis, increased orma ion o pos erior
synechiae in chronic uvei is
Sys emic: Diarrhea, abdominal
cramps, enuresis, and increased ef ec o
succinylcholine
Contraindications
Direc cholinergic: Peripheral re inal
pa hology, cen ral media opaci y, young
pa ien (increases myopic ef ec ), uvei is
Indirec cholinergic: Succinylcholine
adminis ra ion, predisposi ion o re inal ear,
an erior subcapsular ca arac , ocular surgery,
uvei is

Mechanism o Action TABLE 21-3. Mechanism o Ac ion o

Various Mio ic Agen s
Direc -ac ing cholinergics s imula e
muscarinic recep ors, and indirec -ac ing Drug Notes
cholinergics block ace ylcholines erase Echothiophate iodide Indirect; avoid in phakic
(Table 21-3). Mio ics cause pupillary muscle patients
cons ric ion, which is believed o pull open Physostigmine Indirect; avoid in phakic
he rabecular meshwork o increase rabecu- patients
lar ou ow (Fig. 21-6). Demecarium bromide Indirect
Acetylcholine Direct; used during
Side Ef ects
surgery
Direc -ac ing cholinergic
Carbachol Direct/ indirect
Local: Brow ache, breakdown o blood/ Pilocarpine hydrochloride Direct
aqueous barrier, angle closure (increases
Pilocarpine nitrate Direct
pupillary block and causes he lens/ iris
 312 21 MEDICAL MANAGEMENT

FIGURE 21-6. Pilocarpine strengths. T e various s reng hs o pilocarpine, rom 0.5% o 6%.
 Prostaglandins 313

PROSTAGLANDINS redness; cys oid macular edema and an erior

Mechanism o Action
Pros aglandin F2α analogs increase
uveoscleral ou ow by increasing ex ra-
cellular ma rix urnover in he ciliary body
ace (Fig. 21-7).
Side Ef ects
Local: Increase in melanin pigmen a ion
in iris (Fig. 21-8), blurred vision, and eyelid
uvei is have been repor ed.
Sys emic: Sys emic upper respira ory
in ec ion symp oms, backache, ches pain,
and myalgia.
Contraindications
Pregnancy; consider no using in in am-
ma ory condi ions.
 314 21 MEDICAL MANAGEMENT

A B
FIGURE 21-7. Prostaglandin agonists. A. All pros aglandin agonis s available in he Uni ed S a es a he ime
o publica ion. From lef o righ : Xala an 0.005% (P zer; New York, NY) , Rescula (Sucampo Pharmaceu icals,
Inc., Be hesda, MD) is no longer in he US marke , and rava an 0.004% (Alcon; For Wor h, X) . Separa ed
rom he res o he group is he medica ion Lumigan 0.03% (Allergan; Irvine, CA) , which is chemically similar
o he o her drugs bu is considered a pros amide. B. A more recen version o Lumigan (Allergan; Irvine, CA)
con ains a lower dose o bima opros ( 0.01%) han he original ormula ion.

FIGURE 21-8. Prostaglandin analog induced heterochromia. T is pa ien had been rea ed monocularly in
he lef eye wi h a pros aglandin analog which resul ed in a darker iris in he rea ed eye.
 Sympathomimetic Agents 315

SYMPATHOMIMETIC dipive rin), mydriasis, rebound hyperemia,

AGENTS
Mechanism o Action
In he ciliary body, he response is variable
(be a s imula ion increases aqueous produc-
ion, bu alpha s imula ion decreases aqueous
produc ion); in rabecular meshwork, be a
s imula ion causes increased rabecular ou -
ow and increased uveoscleral ou ow; over-
all ef ec lowers IOP (Fig. 21-9).
Side Ef ects
Local: Cys oid macular edema in apha-
kia (more likely wi h epinephrine han
blurred vision, adrenochrome deposi s, and
allergic blepharoconjunc ivi is
Sys emic: achycardia/ ec opy, hyper en-
sion, headache
Contraindications
Narrow angles, aphakia, pseudophakia,
so lenses, hyper ension, and cardiac
disease
Comments
Dipive rin requires 2 o 3 mon hs o
ob ain he ull ef ec . Epinephrine has mixed
alpha- and be a-agonis ac ivi y.

FIGURE 21-9. Sympathomimetic agents. His orically u ilized sympa homime ic agen s rom lef o righ :
Epi rin (Allergan; Irvine, CA) and Propine (Allergan; Irvine, CA) .
 316 21 MEDICAL MANAGEMENT

COMBINATION AGENTS combines he be a-blocker imolol (0.5%)

wi h brimonidine (0.2%). T e mechanisms

O nly wo combina ion agen s are curren ly

available: Cosop , which combines he
be a-blocker imolol (0.5%) wi h he opi-
cal CAI dorzolamide, and Combigan, which
o ac ion, side ef ec s, and con raindica ions
or each componen o hese combina ion
medica ions apply as explained in de ail above
(Fig. 21-10).

A B
FIGURE 21-10. Combination agents. A. T e combina ion agen Cosop ( Merck; Whi ehouse S a ion, NJ) .
B. T e combina ion agen Combigan (Allergan, Irvine, CA) .
 Technique of Drop Instillation 317

TECHNIQUE OF DROP bot le will adminis er he drop. T is echnique

INSTILLATION
Sel -administration
In he uprigh posi ion, drops can be admin-
is ered in many ways. A wo-handed me hod
is brie y described here. Firs , he pa ien
should il he head back so ha he or she is
looking upward. Wi h he nondominan hand,
he pa ien uses he humb and ore nger o
hold open bo h upper and lower lids. Wi h he
dominan hand, he drop bot le is held over he
eye, and a drop is adminis ered (Fig. 21-11).
I remor or generalized weakness makes his
echnique di cul , an al erna e echnique
using one hand can be u ilized. Firs , he
pa ien should il he head back so ha he or
she is looking upward. T e dominan hand
holds he drop bot le so ha i res s gen ly
on he bridge o he nose. T e ip o he drop
bot le should be over he eye. Squeezing he
allows he pa ien ’s nose o help brace he bo -
le and assis he aim o he drop (Fig. 21-12).
Punctal Occlusion
O en, excess drops will drain in o he ear drain-
age sys em and hen in o he nose. Absorp ion
o he drug hrough he nasal mucosa can
grea ly increase he sys emic ef ec o he medi-
ca ion. T is increased sys emic absorp ion does
no ypically enhance he drug’s ocular ef ec s
because mos drugs pene ra e he cornea well
and in su cien quan i y o supersa ura e he
in raocular recep ors. However, he increased
sys emic absorp ion usually increases he likeli-
hood o undesired sys emic side ef ec s.
Manual punc al occlusion minimizes he
drug’s access o he nasal mucosa. o per orm
his maneuver, he pa ien simply holds a n-
ger over he common canaliculi (angle o he
nose) (Fig. 21-13).

A B

FIGURE 21-11. Self administration of drops: two handed method. wo-handed me hod or sel -
adminis ra ion o opical drop. A. Fron al view. B. La eral view.
 318 21 MEDICAL MANAGEMENT

A B
FIGURE 21-12. Self administration of drops: one handed method. Using he bridge o he nose o aid wi h
s eadiness o he hand or sel -adminis ra ion o opical drop. A. Fron al view. B. La eral view.
 Technique of Drop Instillation 319

A B

FIGURE 21-13. Punctal occlusion. Punc al occlusion o minimize sys emic absorp ion o opically
adminis ered medica ions hrough he nasolacrimal sys em. A. Fron al view. B. La eral view.
 C H AP T ER
Laser rabeculoplas y
INDICATIONS
For uncon rolled open-angle glaucoma,
ei her primary or secondary, laser rabecu-
loplas y has proved o be help ul in lowering
he in raocular pressure. Primary open-angle
glaucoma, normal- ension glaucoma, pig-
men ary glaucoma, and pseudoex olia ive
glaucoma are he mos amenable or a good
response.
In juvenile glaucoma and secondary glau-
comas, such as neovascular and in amma ory
glaucomas, resul s wi h laser rabeculoplas y
are ypically poor.
320
L. Jay Katz and Kathryn B. Freidl
Clear media and a good view o he ra-
becular meshwork are required. Eyes wi h
hazy corneas or ex ensive peripheral an erior
synechiae may preven proper rea men
applica ion wi h he laser.
Mas ery o gonioscopy and accura e iden-
if ca ion o he angle s ruc ures are essen ial
or proper laser rabeculoplas y.
BIBLIOGRAPH Y
Van Buskirk EM. Pa hophysiology o laser rabeculo-
plas y. Surv Ophthalmol. 1989;33:264–272.

AR
ARGO
R G O N LASER
LAASE R
RABECULO
R ABEE C ULO
O PLAS
P LA
AS Y
TECHNIQUE
Since he in roduc ion o argon laser
rabeculoplas y (AL ) in 1979 by Wit er
and Wise, here has been remarkably lit le
al era ion o he echnique. A 50-µm spo size
is applied o he rabecular meshwork wi h
up o 1000 mW o energy, enough o cause
minimal blanching o he pigmen . T e leas
amoun o energy is employed o at ain he
issue endpoin (Fig. 22-1).
T e laser spo is aimed a he junc ion o
he pigmen ed and nonpigmen ed rabecular
meshwork. Ei her a single rea men session o
he en ire 360 degrees wi h up o 100 applica-
ions, or wo sessions o 180 degrees each wi h
50 sho s, may be per ormed. A single- or hree-
mirrored Goldmann lens or Ri ch goniolens is
used o apply he laser sho s o he arge issue.
A opical alpha-agonis (apraclonidine or
brimonidine) is given pre- and pos laser rea -
men o minimize he possibili y o a ransien
in raocular pressure spike (Fig. 22-2). A opi-
cal cor icos eroid is prescribed our imes daily
or a week o preven pos laser in amma ion.
A er he rea men , he pa ien is exam-
ined 1 hour la er o measure he eye pressure.
I a pressure spike occurs, i is rea ed wi h
glaucoma medica ions such as oral carbonic
anhydrase inhibi ors or oral hyperosmo ic
agen s. T e pa ien is reexamined a 1 week
and again 1 mon h a er he rea men . A
he las visi , a de ermina ion is made as o
whe her he laser herapy was benef cial.
MECH ANISM OF ACTION
T eories have been o ered, bu none veri-
f ed, as o how laser herapy lowers he eye
pressure. T e ex en o pigmen a ion o he
rabecular meshwork seems o be cri ical or
Argon Laser Trabeculoplasty 321
he success o laser rabeculoplas y. Heavier
pigmen a ion is a posi ive predic or o suc-
cess. T e hermal burn wi h he argon laser
has been shown his ologically o cause mel -
ing and dis or ion o he rabecular beams.
T e f rs heory sugges ed ha hese con-
rac ion burns over he angle mechanically
helped adjacen rabecular beams open wider,
hus allowing easier aqueous ou ow. T e
second heory sugges ed ha he laser irradia-
ion s imula ed rabecular endo helial cells
o replica e (Fig. 22-3). Because hese cells
serve a phagocy ic role in he angle, i was
hough ha he endo helial cells keep he
in ra rabecular spaces ree o debris ha may
be implica ed in he increased resis ance o
ou ow seen in glaucoma ous eyes.
EFFICACY
In raocular pressure is ypically reduced
20% o 30% below baseline levels wi h AL .
No all eyes are responsive o laser rabecu-
loplas y. Posi ive predic ors o a avorable
response include heavy pigmen a ion o he
rabecular meshwork, age (older pa ien s),
and diagnosis (pigmen ary glaucoma, pri-
mary open-angle glaucoma, and ex olia ion
syndrome).
T ere is an apparen waning o he e ec
o AL over ime. In long- erm s udies o 5
o 10 years, AL ailure ranged rom 65% o
90%. Re rea men a er a previous comple e
360-degree applica ion o AL is a bes a
shor - erm benef wi h ailure a 1 year up
o 80%. Because here is s ruc ural al era ion
o he ou ow sys em wi h AL , repea rea -
men may lead o a paradoxical persis en
eleva ion o in raocular pressure. Repea
argon laser applica ion o he angle s ruc ures
in animals was used by Gaas erland o cre-
a e an experimen al open-angle glaucoma
model. I a promp reduc ion in in raocular
pressure is needed, or a rela ively large reduc-
ion in pressure is desired (e.g., more han a
 322 22 LASER TRABECULO PLAST Y
30% lowering below baseline pre rea men
in raocular pressure), hen AL may no be a
good choice. Medica ion or f l ering surgery is
more likely o achieve hose objec ives.
T e curren rea men paradigm or glau-
coma in he Uni ed S a es is medica ion f rs ,
hen AL , and, f nally, f l ering surgery. T is
s epping regimen is only a guideline, and
rea men needs o be individualized or each
pa ien o provide op imum care.
T ere have been s udies ha have reex-
amined he sequencing o rea men s or
open-angle glaucoma. In he Glaucoma
Laser rial, AL was compared wi h medi-
ca ion as he f rs s ep in he rea men o
newly diagnosed primary open-angle glau-
coma. A er 2 years, 44% o eyes wi h AL
alone were con rolled as opposed o only
20% wi h imolol alone being adequa ely
rea ed. In a subsequen paper, wi h a
mean ollow-up o 7 years, AL alone was
adequa e con rol or 20% o eyes and imo-
lol alone or 15%. Al hough here were
Corne a
S chle mm’s
ca na l
FIGURE 22-1. Tissue response to laser treatment. T e “ideal” tissue response is minimal bubble ormation
and mild blanching o the trabecular meshwork. T e laser is aimed at the junction o the pigmented and
nonpigmented trabecular meshwork. (Reprinted with permission rom Katz LJ. Argon laser trabeculoplasty.
Annu Ophthalmic Laser Surg. 1992;1:103–110.)
me hodological aws in he s udy design
or his s udy, here was in riguing suppor
o a leas consider AL as ini ial herapy
or cer ain pa ien s.
BIBLIOGRAPH Y
Damji F, Shah C, Rock WJ, e al. Selec ive laser ra-
beculoplas y argon laser rabeculoplas y: A pro-
spec ive randomised clinical rial. Br J Ophthalmol.
1999;83:718–722.
Feldman RM, a z LJ, Spae h GL, e al. Long- erm e cacy
o repea argon laser rabeculoplas y. Ophthalmology.
1991;98:1061–1065.
Glaucoma rial Research Group. T e Glaucoma Laser rial
2. Resul s o argon laser rabeculoplas y versus opical
medicines. Ophthalmology. 1990;97:1403–1413.
Glaucoma rial Research Group. T e Glaucoma Laser
rial (GL ) and glaucoma laser rial ollow-up s udy:
7. Resul s. Am J Ophthalmol. 1995;120:718–731.
a z LJ. Argon laser rabeculoplas y. Annu Ophthalmic
Laser Surg. 1992;1:103–110.
ramer R, Noecker RJ. Comparison o he morpho-
logic changes a er selec ive laser rabeculoplas y and
argon laser rabeculoplas y in human eye bank eyes.
Ophthalmology. 2001;108:773–779.
Wise JB. Long- erm con rol o adul open angle glaucoma by
argon laser rea men . Ophthalmology. 1981;88: 197–202.
S chwa lbe ’s
line
Tra be cula r
me s hwork
S cle ra l s pur
Cilia ry body
ba nd
Iris
Force
ve ctor
 Argon Laser Trabeculoplasty 323

FIGURE 22-2. Ef ects o postlaser medication administration. Blunting o the postlaser intraocular pressure
spike af er AL with apraclonidine is compared with other glaucoma medications.

2 Day 14 Day
FIGURE 22-3. ALT: One proposed mechanism o action. Cellular theory that AL stimulates the replication
o trabecular endothelial cells that promote aqueous out ow. (Reprinted with permission rom Van Buskirk EM.
Pathophysiology o laser trabeculoplasty. Surv Ophthalmol. 1989;33:264–272.)
 324 22 LASER TRABECULO PLAST Y

SEL
SELEC
L EC
C IVE
IV
VE LASER
LA
ASER R o rabecular meshwork cells were irradi-
RABECULO
R ABE
E C UL
L O PLAS
P LA
AS Y a ed by La ina wi h ei her argon or selec-
ive laser. Argon laser applica ion damaged
bo h pigmen ed and nonpigmen ed cells.
TECHNIQUE
In con ras , he selec ive laser arge ed only
he pigmen ed cells. Recrui men o mac-
A pulsed- requency doubled neodymium
rophages in o he ou ow sys em has been
(Nd):YAG laser was in roduced in 1998 by
demons ra ed in animal models and in human
La ina or rabeculoplas y. I was developed o
eyes. T ese macrophages may release chemi-
selec ively arge pigmen ed issue and mini-
cal media ors ha regula e he ou ow ra e.
mize any colla eral e ec . In con ras wi h he
Eleva ed in erleukin levels de ec ed ollow-
con inuous-wave argon laser, he selec ive
ing laser applica ion have been pos ula ed o
laser does no cause any hermal injury o
improve aqueous ou ow.
he rabecular region. T e f xed spo size o
400 µm dwar s he ypical 50-µm spo size Recen ly, Alvarado described a junc-
used wi h AL (Fig. 22-4). T ere ore, he ion disassembly in Schlemm’s canal cells
spacing be ween laser spo s wi h he selec ive when hey were exposed o laser-irradia ed
laser rabeculoplas y (SL ) is much more Schlemm’s canal cells and rabecular mesh-
compac and almos con uen (Fig. 22-5). work cells. T is same junc ion disassembly
T e spo size wi h SL is so large ha he was demons rable wi h pros aglandin analog
en ire angle is covered wi h he aiming beam. rea men as well. In bo h cases, endo helial
T e only variables in applying he laser are cell junc ion disassembly was associa ed
he number o sho s (50 o 60), ex en o he wi h a congruous increase in he conduc iv-
angle rea ed (180 o 360 degrees), and he i y. Alvarado concluded ha he in raocular
power (up o 0.8 J). pressure–lowering e ec s o SL and pros a-
glandin analogs share a common mechanism
T e power endpoin is de ermined by he
o ac ion a ec ing he barrier proper ies o
issue reac ion wi h he ini ial laser applica-
Schlemm’s canal cells.
ion. Blanching o he pigmen ed rabecular
meshwork wi h sligh bubble vaporiza ion is
ideal. I here is a grea deal o bubble orma- EFFICACY
ion, hen he power is adjus ed downward.
T e use o low power is s rongly recom- Compara ive rials have conf rmed ha
mended in heavily pigmen ed angles as seen AL and SL have equivalen e cacy in
in pigmen ary glaucoma. lowering he in raocular pressure in eyes ha
have ailed medical herapy. S udies sugges
ha ini ial herapy wi h SL prior o any glau-
MECH ANISM OF ACTION
coma medica ion use lowers he in raocular
pressure by 24% o 35% below baseline levels.
Scanning elec ron microscopy highligh s
Posi ive predic ors o success include higher
he di erence be ween argon laser applica-
baseline in raocular pressure and he 2-week
ion, wi h he “mel ing” o rabecular beams,
pos laser pressure response. Like AL , SL ’s
and he selec ive laser, wi h lit le, i any,
e cacy wanes over ime, on average ailing
observable s ruc ural al era ion (Fig. 22-6).
somewhere be ween 6 mon hs and 3 years.
T ere ore, he mechanical s re ching heory
is no applicable or he selec ive laser e ec Because here is no apparen s ruc ural
on he in raocular pressure. In vi ro cul ures damage wi h SL , repea SL is generally

hough o be sa e. Early s udies show ha
re rea men yields success ul (≥20%) in ra-
ocular pressure reduc ion. Also, in eyes ha
have ailed previous AL , success has been
repor ed in using SL o lower he in raocular
pressure. SL has also been shown o work
well wi h PXF glaucoma, pigmen ary glau-
coma, juvenile open-angle glaucoma, and
secondary pseudophakic glaucoma.
BIBLIOGRAPH Y
Alvarado JA, Iguchi R, Jus er R, e al. From he bedside
o he bench and back again: Predic ing and improv-
ing he ou comes o SL glaucoma herapy. Trans Am
Ophthalmol Soc. 2009;107:167–181.
Alvarado JA, Iguchi R, Mar inez J, e al. Similar e ec s o
selec ive laser rabeculoplas y and pros aglandin ana-
logs on he permeabili y o cul ured Schlemm canal
cells. Am J Ophthalmology. 2010;150(2):254–264.
Damji F, Shah C, Rock WJ, e al. Selec ive laser ra-
beculoplas y argon laser rabeculoplas y: A pro-
spec ive randomised clinical rial. Br J Ophthalmol.
1999;83:718–722.
Hodge WG, Damji F, Rock W, e al. Baseline IOP pre-
dic s selec ive laser rabeculoplas y success a 1 year
pos - rea men : Resul s rom a randomized clinical
rial. Br J Ophthalmol. 2005;89:1157–1160.
FIGURE 22-4. Comparison o argon and selective laser spots. Comparative size o the argon laser spot
(50 µm) versus the Nd:YAG selective laser spot ( 400 µm) . (Courtesy o Michael S. Berlin, MD, Associate
Clinical Pro essor, University o Cali ornia–Los Angeles; Jules Stein.)
Selective Laser Trabeculoplasty 325
HongB , Winer JC, Mar one JF, e al. Repea selec ive laser
rabeculoplas y. J Glaucoma. 2009;18(3):180–183.
Jindra LF. SL as primary rea men . Ophthalmol
Management. 2004;8(11):77–78.
Johnson PB, a z LJ, Rhee DJ. Selec ive laser rabecu-
loplas y: Predic ive value o early in raocular pres-
sure measuremen s or success a 3 mon hs. Br J
Ophthalmol. 2006;90:741–743.
ramer R, Noecker RJ. Comparison o he morpho-
logic changes a er selec ive laser rabeculoplas y and
argon laser rabeculoplas y in human eye bank eyes.
Ophthalmology. 2001;108:773–779.
Lai JSM, Chua J , T am CCY, e al. Five-year ollow up
o selec ive laser rabeculoplas y in Chinese eyes. Clin
Exp Ophthalmol. 2004;32(4):369–372.
La ina MA, Sibayan SA, Shin DH, e al. Q-Swi ched 532-
nm Nd:YAG laser rabeculoplas y (selec ive laser ra-
beculoplas y). Ophthalmology. 1998;105:2082–2090.
Lee R, Hu nik CM. Projec ed cos comparison o selec ive
laser rabeculoplas y versus glaucoma medica ion in
he On ario Heal h Insurance Plan. Can J Ophthalmol.
2006;1(4):449–456.
Nagar M, Shah N, apoor B. Selec ive laser rabeculo-
plas y in pseudophakic glaucoma. Ophthalmic Surg
Lasers Imaging. 2010;9:1–2.
Spae h GL, Baez K . Argon laser rabeculoplas y con-
rols one hird o cases o progressive, uncon rolled,
open angle glaucoma or 5 years. Arch Ophthalmol.
1992;110:491–494.
 326 22 LASER TRABECULO PLAST Y

FIGURE 22-5. Comparison o spacing o laser spots. Spacing o the argon laser versus the close application o
the selective laser. riangle indicates approximate 50-µm spot size with AL versus the 400-µm spot size o SL
(right arrow) . (Courtesy o Michael S. Berlin, MD, Associate Clinical Pro essor, University o Cali ornia–Los
Angeles, Jules Stein.)

A B

FIGURE 22-6. Scanning electron microscopy o cadaver eyes treated with argon or selective laser.
A. Argon burn resulted in coagulative melting o the trabecular beam. Le panel: a lower-magni cation view
showing the crater; right panel: a higher-magni cation view showing the curling o the collagen caused by the
thermal damage. B. T e selective laser did not cause any signi cant structural alteration. Le panel: a lower-
magni cation view showing the absence o a crater; right panel: a higher-magni cation view showing a racture
o one o the sheets o collagen. (Reprinted with permission rom Kramer R, Noecker RJ. Comparison o the
morphologic changes af er selective laser trabeculoplasty and argon laser trabeculoplasty in human eye bank eyes.
Ophthalmology. 2001;108:773–779.)

ECO
E CONOM
MICS
IC
CS
Mos prac i ioners oday s ill adhere o he
rea men paradigm o medica ions as f rs -
line herapy, ollowed by laser rabeculoplas y,
and hen possibly surgery. Ye , comparisons in
he li era ure be ween medica ions and laser
rabeculoplas y demons ra e equivalen e -
cacy in reducing in raocular pressure. On he
o her hand, economic s udies indica e ha
laser rabeculoplas y is more cos -e ec ive
han rea men wi h medica ion.
In Aus ralia, aylor e al. ound ha a
change in rea men paradigm o f rs -line
laser rabeculoplas y ollowed by opical
medica ion and hen rabeculec omy saved
$2.50 or every $1.00 spen . In pa ien s who
received mono-, bi- or ri-medica ion herapy,
a Canadian s udy o 6-year cumula ive cos s
ound ha primary SL repea ed every
2 years produced cos savings o $206,
$1,669, and $2,993 per pa ien . Similarly,
in he Uni ed S a es, he baseline 5-year
cumula ive cos or pa ien s rea ed wi h
laser rabeculoplas y was es ima ed o be
Economics 327
$4,838 compared wi h $6,571 or pa ien s
rea ed wi h medica ion alone. Fur her, laser
rabeculoplas y o ered a cos savings o
approxima ely $1,700 per pa ien rea ed by
up i ra ion in medica ion rom wo o our
over 5 years.
T e au hor does no recommend ha
prac i ioners base rea men decisions solely
on economic considera ions. Curren ly, ac-
ors such as age, access o medica ion, abili y
o adminis er and/ or olera e medica ion,
disease s age, and ra e o progression are s ill
he primary considera ions ha will guide
care. Ye , i is inevi able ha in he ace o
oday’s rising heal h care expendi ures and
shrinking heal hcare budge s, cos is a ac-
or ha will be increasingly brough o he
ore ron .
BIBLIOGRAPH Y
Can or LB, a z LJ, Cheng JW, e al. Economic evalua-
ion o medica ion, laser rabeculoplas y and f l ering
surgeries in rea ing pa ien s wi h glaucoma in he US.
Curr Med Res Opin. 2008;24(10):2905–2918.
aylor HR. Glaucoma: Where o now? Ophthalmology.
2009;116(5):821–822.
 C H AP T ER
Deep Sclerec omy Surgery
or Glaucoma
Konrad Schargel, José I. Belda, and Konrad W. Schargel
N onpene ra ing glaucoma surgery, such
as deep sclerec omy, has become more
popular among surgeons in Europe,1 primar-
ily because o i s sa e y. Deep sclerec omy was
described by Eps ein and Krasnov in he la e
1950s and subsequen ly revised by Fyodorov
and o hers. Deep sclerec omy involves cre-
a ing wo par ial- hickness scleral f aps wi h
he second, deeper f a a 99% dep h. During
he procedure, he inner f ap is removed, cre-
a ing an in rascleral lake. T e procedure is
ermed “nonpene ra ing” because he inner
wall o Schlemm’s canal, rabecular meshwork,
and Desceme ’s membrane remain in ac .
Fil ra ion across he ou er f ap is allowed o
crea e a conjunc ival bleb. A dual mechanism
o ac ion is proposed wi h bo h enhanced
uveoscleral ou f ow hrough he area o uvea
exposed during he inner dissec ion as well
as he orma ion o he conjunc ival bleb.
O her names or deep sclerec omy include
nonpene ra ing rabeculec omy and ex ernal
rabeculec omy.
328
STUDIES
In 1984, Zimmerman repor ed a re ro-
spec ive s udy showing comparable resul s
be ween he rabeculec omy (wi hou an i-
me aboli es) and nonpene ra ing rabeculec-
omy in erms o in raocular pressure (IOP)
con rol, bu wi h lower pos opera ive com-
plica ions such as shallow an erior chamber,
uvei is, hyphema and loss o vi reous; in heir
s udy, rabeculec omy (n = 86) con rolled
he IOP o 70% o pa ien s (wi h or wi hou
medica ions), while nonpene ra ing rabecu-
lec omy (n = 71) similarly con rolled he IOP
in 84% o pa ien s wi h a mean ollow-up o
1.7 years.2
In a prospec ive s udy in which pa ien s
(n = 39) were randomized o receive deep
sclerec omy in one eye and rabeculec omy
in he o her, El Sayyad e al. repor ed success
ra es (i.e. IOP < 21 mm Hg) o 92.3% and
94.9% a 1 year (p = 0.9) or deep sclerec omy
and rabeculec omy, respec ively; serious

complica ions were more prevalen wi h rab-
eculec omy.3 In El Sayyad’s s udy, bo h groups
were rea ed wi h pos opera ive 5-f uorouracil
(5-FU) subconjunc ival injec ions a he dis-
cre ion o he inves iga ors.
T e resul s o deep sclerec omy
are improved when combined wi h an
implan . T e implan is designed o main ain
he suprachoroidal space (i.e., in rascleral
bleb) avoiding he closure o he sclerec-
omy. T e implan s can be absorbable or
nonabsorbable.
In a case series o 105 eyes wi h an
average ollow-up o 64 mon hs, Shaarawy
e al., using absorbable implan s o col-
lagen, repor ed a success ra e o 91% (IOP
< 21 mm Hg wi h or wi hou medica ions)
a 96 mon hs; hal o he eyes underwen
laser goniopunc ure wi h a mean ime
o goniopunc ure a 21 mon hs and 23%
received pos opera ive 5-FU injec ions.4
T ey repor ed no incidence o f a an erior
chamber or endoph halmi is.4
Using he nonabsorbable -Flux
implan (IOL ECH Labora ories,
France), Jungkim e al.5 repor ed a case
series o 35 eyes wi h 12 mon hs o ollow-
up demons ra ing a lowering o IOP rom
33 mm Hg o an IOP o approxima ely
15 mm Hg wi h an average o 0.1 an iglau-
coma medica ions. A es e al.6 repor ed
similar resul s in a small case series o 25
eyes.
T e advan ages o o her me hods using
cheaper ma erials, like viscoelas ic, show
some long- erm validi y.7 I has been over
50 years since rabeculec omy was popular-
ized; we are amiliar wi h i s advan ages,
disadvan ages, and success ra e bu rab-
eculec omy has a price, he complica ions.
Mos o he complica ions are rela ed o
excessive l ra ion, especially in he early
pos opera ive period, such as f a an erior
chamber, hypo ony, choroidal de achmen ,
Studies 329
and ca arac orma ion. Wi h rabeculec-
omy, complica ions occur in 10% o 18%
o pa ien s. T e well-guarded dissec ion in
deep sclerec omy reduces he complica-
ions rela ed o over l ra ions.8
An impor an cavea exis s when
analyzing he resul s o nonpene ra ing
surgery. Deep sclerec omy is highly depen-
den upon he echnical skills o he surgeon
which can bring large di erences when
resul s rom di eren au hors are compared.
Deep sclerec omy has a long learning curve
during which he surgical ime is longer
and he ini ial ou comes migh no be very
sa is ac ory.
Dahan and Drusedau repor ed he
resul s including pa ien s rom he learn-
ing curve, during which ime per ora ions
in o he an erior chamber were 1 in 3,
necessi a ing conver ing o a s andard rab-
eculec omy. T ey also men ion ha as he
manual echnique improves, he per ora-
ion ra e drops o 1 in 20.9
In our personal experience, he rs 20
cases were conver ed o pene ra ing rab-
eculec omies or di eren reasons. Deep
sclerec omy is no a simple surgery; on he
con rary, ime is needed o learn i properly
bu once mas ered, i is elegan , secure, and
com or able or he pa ien s in mos o he
cases.
REFERENCES
1. Baudouin C, Rouland JF, Le Pen C. Change in medical
and surgical rea men s o glaucoma be ween 1997 and
2000 in France. Eur J Ophthalmol. 2003;13(suppl 4):
S53–S60.
2. Zimmerman J, Kooner KS, Ford VJ, e al.
rabeculec omy vs nonpene ra ing rabeculec omy:
A re rospec ive s udy o wo procedures in pha-
kic pa ien s wi h glaucoma. Ophthalmic Surg. 1984;
15:734–740.
3. El Sayyad F, Helal M, El-Kholi y H, e al. Non-
pene ra ing deep sclerec omy versus rabeculec-
omy in bila eral primary open angle glaucoma.
Ophthalmology. 2000;107:1671–1674.
 330 23 DEEP SCLERECTO MY SURGERY FO R GLAUCO MA
4. Shaarawy , Mansouri K, Schnyder C, e al. Long- erm
resul s o deep sclerec omy wi h collagen implan . J
Cataract Ref act Surg. 2004;30:1225–1231.
5. Jungkim S, Gibran SK, Khan K, e al. Ex ernal rab-
eculec omy wi h -Flux implan . Eur J Ophthalmol.
2006;16:416–421.
6. A es H, Ure men O, Andaç K, e al. Deep sclerec omy
wi h nonabsorbable implan ( -Flux): Preliminary
resul s. Can J Ophthalmol. 2003;38:482–488.
7. Ravine E, Bovey E, Mermoud A. -f ux implan
versus Healon GV in deep sclerec omy. J Glaucoma.
2004;13:46–50.
8. Drolsum L. Conversion rom rabeculec omy o deep
sclerec omy. Prospec ive S udy o he rs 44 cases. J
Cataract Ref act Surg. 2003;29:1378–1384.
9. Dahan E, Drusedau M. Non pene ra ing l ra ion sur-
gery or glaucoma: Con rol by surgery only. J Cataract
Ref act Surg. 2000;26:696–701.

SURGICAL
S UR
R G IC
C AL E
ECH
CH
H N IQ
Q UE
E erized (Fig. 23-4A), or ano her op ion is
T e surgery can be done wi h any ype o
local anes hesia, we pre er sub- enon’s and
peribulbar anes hesia. I he procedure is o
be combined wi h phacoemulsi ca ion i will
also bene rom he in racameral anes hesia.
For sub- enon´s anes hesia we use a
mix ure o Lidocaine 5% plus Bupivacaine
0.75%, 1.5 mL o each or a o al volume o
3 mL, we used around 1.5 mL o his and
keep he res in case we need complimen-
ary anes hesia.
We also use drops o opical anes he-
sia and begin wi h a but onhole in he
conjunc iva/ enon (Fig. 23-1A) in he
in ero emporal quadran in roducing an
18-gauge angioca h (Fig. 23-1B); usually
a er he in usion o he anes he ic we have
a chemosis (Fig. 23-1C) ha helps wi h
he ini ial conjunc ival dissec ion.
Nonpene ra ing surgery should be per-
ormed in he superior quadran o he globe;
he reason is ha l ra ion surgery has been
associa ed wi h in ec ions when i is done a
he in erior quadran .
o ro a e he globe in eriorly, a rac-
ion su ure can be a he superior rec us o
5–0 black silk or a corneal rac ion su ure
o 7–0 or 8–0 (Fig. 23-2A) black silk or
Vicryl can be used. We pre er using a cor-
neal rac ion su ure (Fig. 23-2B).
T e conjunc ival f ap can be ini ia ed
a he limbus (Fig. 23-3A) or ornix
(Fig. 23-3B, C); I pre er a limbus base wi h
an L-shape incision which is a modi ca ion o
he Dahan incision (inverse L) (Fig. 23-3D).
T is is made using a Wes cot scissors and a
non oo hed u ili y orceps. I is impor an o
ex end he dissec ion in he subconjunc ival
space a ei her nasal or emporal side o he
main incision. T e sclera can be cleaned o
any adhesions wi h he use o a scari er.
Surgical Technique 331
Bleeding vessels should be ligh ly cau-
o use he scari er (Fig. 23-4B); he ech-
nique is he same as wi h rabeculec omy.
T e super cial scleral f ap measures 5 ×
5 mm, and he measuremen s can be done
wi h a caliper (Fig. 23-5A, B) or a marker,
or example he HUCO VISION SA has
wo di eren makers. One is he (Dahan)
double-ended -Flux rapezoidal marker ha
measures 5 × 5 × 2 mm in one end and on he
o her a smaller 3.3 × 3 × 1 mm (Fig. 23-5C, D).
Ano her is he (Mermoud) non pene ra ing
glaucoma surgery (NPGS) double-ended
marker which is 4 × 5 mm and in he o her
end 4 × 4 mm. I pre er he rapezoidal maker
bu here are no di erences i he f ap is square
(Fig. 23-5E, F) or rapezoidal (Fig. 23-5G, H).
I is impor an o dissec he f ap ar
an eriorly in o he cornea, enough o
ensure ha we will be able o crea e a wide
rabeculodesceme ic window. T e f ap
hickness should be be ween one-hal o
one- hird o he sclera.
T e second or deep scleral f ap
(Fig. 23-6A) is he cri ical par and i
should have a o al dep h o 90% o 99% o
scleral hickness; one ip is o s ar he dis-
sec ion ar back away rom he limbus and
progressively deepen in order o ge he
desired dep h (Fig. 23-6B). As we ge closer
o he clear cornea more care has o be aken.
T e reason is ha he rabeculodesceme ic
membrane is very hin and can break easily
jus by a lit le excess o pressure, he dissec-
ion can be made more sa ely wi h a spa ula
(Fig. 23-6C). A diamond kni e is oo sharp
and makes i very easy o per ora e he ra-
beculodesceme ic membrane. T ere is a
diamond kni e designed or dissec ion o he
rabeculodesceme ic membrane which is he
Dahan Diamond Schlemm’s canal opener
kni e, I personally pre er a 45-degree-angled
s eel kni e.
 332 23 DEEP SCLERECTO MY SURGERY FO R GLAUCO MA
During he dissec ion when we s ar
observing he change in color be ween he
sclera and he clear cornea, i is bet er o
do small cu s a he sides o he f ap (Fig.
23-7A). We need o ge ar an erior in o he
clear cornea be ore we ampu a e he deep
scleral f ap (Fig. 23-7B). I he surgeon is
working alone, a ip is o use an addi ional
s i ch o x he superior scleral f ap and o
ge a bet er view o he surgical eld (Fig.
23-7C).
T e rabeculodesceme ic membrane
(Fig. 23-8A–B) is he key poin o he sur-
gery and special care mus be aken o pre-
pare i . T e rs and mos impor an s ep
is o ge he righ dep h. T e second s ep is
o peel Schlemm’s canal (Fig. 23-8C), and
some imes when we have done a very deep
dissec ion i will be already removed, a er
his we can dila e he emporal and nasal
sides o Schlemm’s canal, his maneuver
can be done wi h a spa ula (Fig. 23-8D). I
pre er he Mermoud predesceme ic spa ula
(Huco Vision SA) or a scraper; he one
shown in he gure is he Dahan rabecular
meshwork scraper (Fig. 23-8E). When
he canal is properly dila ed, he aqueous
humor will f ow abundan ly.
T e las aspec are he implan s (no avail-
able in he Uni ed S a es). T ey are used o
keep he scleral lake open, he posi ion where
hey all are going o be is in he space crea ed
a er making he deep f ap and will be covered
by he remaining superior f ap. T ere are wo
ypes, he absorbable like he Aquaf ow colla-
gen glaucoma drainage device (S aar Surgical,
Monrovia, CA) (Fig. 23-9A) which has a
comple e resorp ion wi hin 6 o 9 mon hs
and he SKGEL (Corneal, Paris, France) (Fig.
23-9B) made o re icula ed sodium hyaluro-
na e. T e nonabsorbable implan s, like -Flux
(Carl Zeiss Medi ec Company, La Rochelle,
France) (Fig. 23-9C), have a shape and he
superior hap ics are inser ed in o Schlemm’s
canal a er hey have been dila ed. T e body
will res in he scleral bed and can be xa ed
wi h 10–0 nylon su ure.
We recen ly worked on a modi ca ion o
he Esnoper V-2000 (AJL Oph halmic S.A.,
Álava, Spain) (Fig. 23-10A). T is implan has
a rapezoidal shape wi h longi udinal s ria-
ions (Fig. 23-10B) ha we believe enhance
he f ow o he aqueous humor in o he supra-
choroidal space; i can also be xa ed wi h
su ure or by in roducing he pos erior par
in o he suprachoroidal pocke as described
by Muñoz.1 He repor ed a ur her decrease in
IOP o 2 mm Hg a more han 1 year a er he
surgery.
T is echnique involves making an inci-
sion in he scleral bed abou 2 or 3 mm
pos erior o he rabeculodesceme ic mem-
brane wi h very small cu s o expose he
suprachoroidal space (Fig. 23-11A, B); his
space is dila ed wi h a spa ula (Fig. 23-11C)
and we place par o he implan inside ha
pocke (Fig. 23-11D). A ip wi h he use o
hese implan s, as hey are colorless (Fig.
23-11E), is o in hem wi h f uorescein
(personal echnique) (Fig. 23-11F); his is
especially help ul during he learning process.
I every hing is success ul – good dissec ion
wi h he correc hickness and dep h, implan
in place, and appropria e aqueous humor
l ra ion (Fig. 23-11G) – we should be able
o observe a ormed an erior chamber i no
rup ures o he membrane has occurred. We
can now close he super cial f ap wi hou
su ure (Muñoz echnique) 1 (Fig. 23-11H)
or using a 10–0 nylon su ure (Fig. 23-11I).
T e conjunc iva can be closed wi h a running
su ure o 8–0 Vicryl (E hicon) (Fig. 23-11J)
par icularly i i is a limbus-based conjunc ival
f ap or wi h 10–0 nylon i i is ornix based.
We also like o use an ime aboli es like
Mi omycin-C 0.05 mg per mL or 1.0 o 1.5
minu es or 5-FU a 50 mg per mL or 3 min-
u es. We apply i by cut ing wo small pieces
 Surgical Technique 333

o sponge which are in roduced in he dis- REFERENCE

sec ed conjunc ival pocke s (Fig. 23-12A) 1. Muñoz G. Nons i ch suprachoroidal echnique or
and under he super cial scleral f ap (Fig. -f ux implan a ion in deep sclerec omy. J Glaucoma.
2009;18(3):262–264.
23-12B); a er he elapsed ime he area
should be irriga ed wi h su cien saline solu-
ion or balanced sal solu ion (Fig. 23-12C).

A B

FIGURE 23-1. A. Incision in he conjunc iva or

sub- enon’s anes hesia, bet er in he in erior emporal
or nasal. B. ef on cannula, 18 gauge. A mix ure ( 1
o 1.5 mL) o Lidocaine 5% + Bupivacaine 0.50%. C.
C Chemosis a er applying he sub- enon’s anes hesia,
i is use ul or he dissec ion.
 334 23 DEEP SCLERECTO MY SURGERY FO R GLAUCO MA

A B

FIGURE 23-2. A. Silk su ure 7–0 placed in clear cornea a 12 hours. B. Ro a ion o he globe or bet er
presen a ion o he eld.

A B

C D

FIGURE 23-3. A. Limbus-based incision. B. Fornix-based incision, rs s ep. C. Fornix-based incision, second
s ep. D. L shape inverses. Dahan describes he L incision, ornix based.
 Surgical Technique 335

A B
FIGURE 23-4. A. Cau eriza ion o he eld, dia hermy very ligh . B. Scari ca or, ins rumen used o break he
unions a he sclera and conjunc iva.

A B

C D
FIGURE 23-5. A. Dimension o he f ap can be measured wi h a compass, horizon al. B. Dimension o he f ap
can be measured wi h a compass, ver ical. C. Fix rapezoidal-shaped marker. D. Area marked.
( continued)
 336 23 DEEP SCLERECTO MY SURGERY FO R GLAUCO MA

E F

G H
FIGURE 23-5. (Continued) E. Super cial f ap, rec angular shape, rs s ep. F. Super cial f ap, rec angular
shape. G. Super cial f ap, rapezoidal shape, rs s ep. H. Super cial f ap, rapezoidal shape, 5 × 5 × 2 mm.

A B

FIGURE 23-6. A. Deep f ap 3.3 × 3 × 1 mm. B. I

is bet er o s ar ar back un il ge he righ plane.
C. When we ge close o he rabeculodesceme ic
C membrane he dissec ion can be done wi h a blun
spa ula.
 Surgical Technique 337

A B

FIGURE 23-7. A. Checking he window, which is

he rabeculodesceme ic membrane. B. Cut ing he
deep scleral f ap. C. Fixing he super cial f ap wi h
C a su ure nylon 10–0 or silk 8–0 helps o nish he
rabeculodesceme ic membrane.
 338 23 DEEP SCLERECTO MY SURGERY FO R GLAUCO MA

A B

C D

FIGURE 23-8. rabeculodesceme ic membrane.

T e mos impor an s ep. I is where he di erence
be ween per ora ing and no is made.
A. Square f ap.
B. rapezoidal f ap.
C. Peeling Schlemm’s canal o enhance l ra ion.
D. Dila ing Schlemm’s canal also enhances l ra ion.
E. Using he scraping over he rabeculodesceme ic
membrane will enhance he l ra ion and acili a ed
E
Peeling o he Schlemm´s canal.
 Surgical Technique 339

A B

FIGURE 23-9. A. Aquaf ow collagen absorbable

implan (S aar Surgical Monrovia, CA) . B. SKGEL
(Corneal, Paris, France) made o re icula ed
C sodium hyalurona e. C. -Flux (Carl Zeiss Medi ec
Company, La Rochelle. France) .
 340 23 DEEP SCLERECTO MY SURGERY FO R GLAUCO MA

A B
FIGURE 23-10. A. Esnoper V-2000 (AJL Oph halmic S.A., Álava. Spain) schema ic design. B. Esnoper V-2000,
rapezoidal shape wi h longi udinal s ria ions.

A B

C D
FIGURE 23-11. A. Crea ing he scleral pocke in a square f ap. B. Crea ing he scleral pocke in a rapezoidal
f ap. C. Widen he pocke wi h a spa ula. D. Esnoper V-2000 nonabsorbable implan in he scleral bed wi h he
pos erior par in he suprachoroidal pocke .
( continued)
 Surgical Technique 341

E F

G H

I J
FIGURE 23-11. (Continued) E. -Flux nonabsorbable colorless implan . F. -Flux a er using f uorescein.
G. Aqueous humor ou f ow. H. Closing he scleral f ap, no su ure. I. Closing he scleral f ap wi h nylon 10–0.
J. Conjunc ival closing wi h Vicryl 8–0 running su ure.
 342 23 DEEP SCLERECTO MY SURGERY FO R GLAUCO MA

A B

FIGURE 23-12. A. Sponge soaked in

an ime aboli es, hree ragmen s, one or each side,
nasal and emporal, and he hird o leave under he
scleral f ap. B. Sponge soaks in an ime aboli e under
C he scleral f ap. C. Wash ou he an ime aboli e wi h a
saline solu ion o abou 250 mL.
 Postoperative Care 343

membrane can be blocked by he iris

POS
PO S O PERA
P E RA
A IVE
IV
VE CA
CARE
AR E (Fig. 23-16C), and an applica ion o
argon laser a he base o he iris wi h mild
T e pos opera ive care o he deep scle-
power and spo s o 500 Mc (gonioplas y)
rec omy is cri ical. Some advan ages o deep
will f at en he iris and pull i away rom he
sclerec omy include minimal in raocular
rabeculodesceme ic membrane; in some
inf amma ion (Fig. 23-13A) and ha he risk
cases, opical pilocarpine is enough o pull
o f a an erior chamber is very low i here
he iris away rom he rabeculodesceme ic
was no per ora ion in o he an erior cham-
membrane. I he rise o IOP is no due o a
ber. A he presen ime, we are leaving he
mechanical blockage, goniopunc ure can be
superior f ap unsu ured bu in case o doub
per ormed. T is consis s o using he
or excessive l ra ion we place an 8–0 Vicryl
YAG laser o crea e small holes hrough he
su ure as a bel over he scleral f ap (Fig.
rabeculodesceme ic membrane. I believe
23-13B). T is will keep a pressure over he
ha i should be done only in case o
f ap which will be slowly reduced due o he
need where a rise o IOP is no rela ed o
absorp ion o he su ure.
mechanical blockage bu is due o rabecular
For in ec ion prophylaxis and rea men pa hology.
o inf amma ion, we use moxif oxacin or cip-
T e bleb can become encapsula ed and
rof oxacin our imes a day or 7 days, dexa-
can bene rom needling. Bleb needling
me hasone also our imes a day or 2 weeks,
can be done wi h opical anes hesia a he
and nons eroidal an i-inf amma ory like opi-
sli lamp. T e surgeon in roduces 25-gauge
cal diclo enac hree imes a day or 6 weeks.
needle, xed on a syringe or s abili y, in o
Generally, we see our pos opera ive pa ien s
he subconjunc ival space a 2 mm rom
a 24 hours (Fig. 23-14A) and every week or
he cys (Fig. 23-17A). In he rs s ep
1 mon h (Fig. 23-14B) and every 2 weeks
we ry o break he adhesions be ween he
or ano her mon h (Fig. 23-14C). T e visual
conjunc iva and he wall o he bleb (Fig.
acui y is usually very s able bu we pre er no
23-17B). Nex , we ry o ge under he f ap;
o change he re rac ion (Fig. 23-14D) un il
mos o our pa ien s have no su ure which
he hird mon h.
helps o li he f ap. T en we very gen ly
T e bleb ends o be di use, and as we use break any subconjunc ival synechia. 5-FU
an ime aboli es in mos o our pa ien s, we can be used o reduce healing and brosis
usually see an avascular bleb in he rs injec ed, a er comple ing all maneuvers, in o
12 weeks (Fig. 23-15A, B). In some cases he subconjunc ival space (Fig. 23-17C).
he bleb can be very exuberan in he rs T e bleb brosis can be observed using he
week bu his is no common; in mos o an erior-segmen op ical coherence omogra-
hese pa ien s when his happens he bleb will phy (OC ) (Fig. 23-17D, E) o help guide
become di use in ime (Fig. 23-15C). herapy.
During he nex 3 mon hs i is very impor- T e an erior-segmen image, such as can
an o observe he IOP, i i s ar s o rise, a be ob ained using spec ral domain OC , is
gonioscopy is very help ul (Fig. 23-16A); very use ul or he pos opera ive managemen
he rabeculodesceme ic membrane is so hin ollowing deep sclerec omy. T e hickness o
ha i can break and he implan can en er he he rabeculodesceme ic membrane and i s
an erior chamber (Fig. 23-16B). T is is very in egri y can be moni ored (Fig. 23-18A);
uncommon and is rela ed o ocular rauma or also, he implan posi ion (Fig. 23-18B),
echnical problem. T e rabeculodesceme ic
 344 23 DEEP SCLERECTO MY SURGERY FO R GLAUCO MA
brosis o he bleb (Fig. 23-18C), and he
posi ion o he implan can be s udied (Fig.
23-18D). When he bleb has become f a or
collapses, one can consider needling o imely
resolve his complica ion (Fig. 23-18E).
Microper ora ions and iris incarcera ion
be ween o her complica ions can also be seen
(Fig. 23-18F) wi h he an erior-segmen
OC or ul rasonic biomicroscopy.
CONCLUSION
T is chap er is no in ended as a compre-
hensive review o he li era ure. Deep sclerec-
omy is a complex surgery wi h a signi can
learning curve. Fur hermore, here are di er-
ences in each surgeon’s echnique, making i
di cul o compare resul s be ween surgeons,
bu he IOP is ypically in he high eens in
mos o he me a-analyses.1 Wha seems o be
clear is ha rabeculec omies lower he IOP
more han deep sclerec omies bu wi h more
complica ions. 2–4
T ere are many ques ions abou he
implan s, absorbable or no , regarding
heir abili y o enhance he l ra ion and
improve he resul s. For many surgeons, hey
do. T e concep o keeping he vir ual space
or a lake improves he resul s.5–9 For some
o hers, hey do no change he long- erm
resul s.10,11 Deep sclerec omy seems o work
in primary open-angle glaucoma and second-
ary as pseudoex olia ion,12,13 uvei ic,14 and in
some rauma ic cases wi h hyphema (per-
sonal experience).
In mos o he published ar icles, wi h
deep sclerec omy, he ra e o complica ion is
compara ively very low, here is no f a an e-
rior chamber, less incidence o ca arac , and
less choroidal hemorrhage. Due o he low
ra e o complica ions in early and la e pos op-
era ive period i can be done early in mild o
modera e glaucomas o di eren ypes.
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pene ra ing glaucoma surgery me a-analysis o recen
resul s. J. Glaucoma. 2008;17:139–146.
2. Chiseli a D. Non-pene ra ing deep sclerec omy ver-
sus rabeculec omy in primary open angle glaucoma
surgery. Eye. 2001;15:197–201.
3. Lachkar Y, Neverauskiene J, Jean eur-Lunel MN, e al.
Nonpene ra ing deep sclerec omy: 6 year re rospec-
ive s udy. Eur J Ophthalmol. 2004;14:26–36.
4. Khary HA, Green FD, Nassar MK, e al. Con rol
o in raocular pressure a er deep sclerec omy. Eye.
2006;20:336–340.
5. Sanchez E, Schnyder CC, Sickenberg M, e al. Deep
sclerec omy: Resul s wi h and wi hou collagen
implan . Int Ophthalmol. 1997;20:157–162.
6. Karlen ME, Sanchez E, Schnyder CC, e al. Deep scle-
rec omy wi h collagen implan : Medium erm resul s.
Br J Ophthalmol. 1999;83:6–11.
7. Dahan E, Ravine E, Ben-Simon GJ, e al.
Comparison o he e cacy and longevi y o nonpen-
e ra ing glaucoma surgery wi h and wi hou a new
nonabsorbable hydrophilic implan . Ophthalmic
Surg Laser Imaging. 2003;34:1–7.
8. Shaarawy , Nguyen C, Schnyder C, e al.
Compara ive s udy be ween deep sclerec omy wi h
and wi hou collagen implan : Long erm ollow up.
Br J Ophthalmol. 2004;88:95–98.
9. Bissig A, Rivier D, Zaninet i M, e al. en years ol-
low-up a er deep sclerec omy wi h collagen implan .
J Glaucoma. 2008;17:680–686.
10. Demailly P, Lava P, Kre z G, e al. Non pene ra ing
deep sclerec omy (NPDS) wi h or wi hou colla-
gen device (CD) in primary open-angle glaucoma:
Middle erm re rospec ive s udy. Int Ophthalmol.
1997;20:131–140.
11. Cheng JW, Wei RL, Cai JP, e al. E cacy and oler-
abili y o nonpene ra ing l ering surgery wi h and
wi hou implan in rea men o open angle glau-
coma. A quan i a ive evalua ion o he evidence. J
Glaucoma. 2009;18:233–237.
12. Mendrinos E, Mansouri K, Mermoud A, e al. Long-
erm resul s o deep sclerec omy wi h collagen
implan in ex olia ive glaucoma. J Glaucoma. 2009;
18:361–367.
13. Drolsum L. Deep sclerec omy in pa ien s wi h
capsular glaucoma. Acta Ophthalmol Scand. 2003;
81:567–572.
14. Auer C, Mermoud A, Herbor CP. Deep sclerec omy
or he managemen o uncon rolled uvei ic glau-
coma: Preliminary da a. Klin Monatsbl Auehenheilkd.
2004;221:339–342.
 Postoperative Care 345

A B
FIGURE 23-13. A. Eye 24 hours a er a deep sclerec omy and phacoemulsi ca ion. B. Bel su ure over he
scleral f ap made wi h Vicryl 8–0.

A B

C D
FIGURE 23-14. A. Eye 24 hours a er a deep sclerec omy. B. Eye 1 mon h a er deep sclerec omy. C. Eye
6 weeks a er deep sclerec omy. D. Eye 3 mon hs a er deep sclerec omy.
 346 23 DEEP SCLERECTO MY SURGERY FO R GLAUCO MA

A B

C FIGURE 23-15. A. Di use bleb. B. Avascular bleb.

C. Enlarged bleb.
 Postoperative Care 347

A B

FIGURE 23-16. A. rabeculodesceme ic membrane,

gonioscopic view. B. -Flux hap ics and par o he
body in an erior chamber in an eye wi h a previous
C rabeculec omy. C. rabeculodesceme ic membrane
block by he iris, gonioscopic view.
 348 23 DEEP SCLERECTO MY SURGERY FO R GLAUCO MA

A B

C D

FIGURE 23-17. A. Needling breaking he adhesion

be ween conjunc iva and sclera. B. Needling rying
o ge under he super cial f ap. C. A er needling in
injec ion o 5-FU. D. OC rom an erior segmen
E be ore needling. E. An erior-segmen OC a er
needling, bleb crea ed a er he injec ion o 5-FU.
 Postoperative Care 349

A B

C D

E F
FIGURE 23-18. A. An erior-segmen OC de ails o he rabeculodesceme ic membrane, implan , and
posi ion o he iris can be observed. B. Coronal view rom an erior-segmen OC , -Flux in posi ion, end o
he hap ics can be seen and measured. C. Visan e OC o a deep-sclerec omy big bleb over he implan can be
seen. D. An erior-segmen OC wi h a suprachoroidal view rom he implan . E. An erior-segmen OC in a
non unc ioning deep sclerec omy, showing he collapse o he issue over he surgery. No l ra ion. F. Esnoper
V-2000 nonabsorbable implan coronal view wi h an iris incarcera ion a er a per ora ion.
 C H AP T ER
24
rabeculec omy and Ex-PRESS
Mini Glaucoma Shun
RABECULEC
R AB E C
CUU L EC
ULEC
UL E O MY
MY
G uarded f l ra ion surgery, or rabeculec-
omy, lowers he in raocular pressure
(IOP) by crea ing a f s ula be ween he inner
compar men s o he eye and he subcon-
junc ival space (i.e., f l ering bleb; Fig. 24-1).
Cairns1 repor ed he f rs series in 1968. A
number o echniques are available o assis
in es ablishing and main aining he unc ion
o f l ra ion blebs and avoiding complica ions
(see below).
rabeculec omy is he mos commonly used
surgical procedure in pa ien s wi h glau-
coma, bu i s role is cons an ly evolving.
rabeculec omy has been compared wi h
ini ial opical medical rea men as an ini ial
rea men or glaucoma in a large random-
ized con rolled rial.2,3 I seems ha pa ien s
350
Marlene R. Moster and Augusto Azuara-Blanco
presen ing wi h more advanced disease had
slower visual f eld progression i heir primary
in erven ion was surgical ra her han medical.
rabeculec omy as he primary surgical in er-
ven ion in glaucoma has been recen ly chal-
lenged, and randomized compara ive s udies
be ween rabeculec omy and glaucoma drain-
age device (GDD) are ongoing.
REFERENCES
1. Cairns JE. rabeculec omy: Preliminary repor o
a new me hod. Am J Ophthalmol. 1968;(66):673–
679.
2. Burr J, Azuara-Blanco A, Avenell A. Medical versus sur-
gical in erven ions or open angle glaucoma. Cochrane
Database Syst Rev. 2005;18(2):CD004399.
3. Musch DC, Gillespie BW, Lich er PR, e al. Visual
f eld progression in he Collabora ive Ini ial
Glaucoma rea men S udy: T e impac o rea men
and o her baseline ac ors. Ophthalmology. 2009;
116(2):200–207.
 Trabeculec omy 351

A B
FIGURE 24-1. Sli lamp view o an eye ha underwen rabeculec omy 3 mon hs earlier (A and B) . No e he
non localized eleva ion o he conjunc iva.
 352 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT
SURGICAL TECHNIQUE
Any ype o regional anes hesia (re ro-
bulbar, peribulbar, and sub- enon’s) can be
used. opical anes hesia is also possible, wi h
opical 2% lidocaine gel, 0.1 mL o in racam-
eral 1% nonpreserved lidocaine (Fig. 24-2),
and 0.5 mL o subconjunc ival 1% lidocaine
injec ed rom he superior- emporal quadran
o balloon he conjunc iva over he superior
rec us muscle (Fig. 24-3).
rabeculec omy should be done a he
superior limbus, because in eriorly loca ed
blebs are associa ed wi h a much higher risk
o bleb-associa ed in ec ions.
A f xa ion or rac ion su ure is used o keep
he eye in downward posi ion giving a good
area o exposure.
A corneal rac ion su ure in he quadran
o he planned surgery (7–0 or 8–0 black silk
or nylon, or 8–0 Vicryl on a spa ula ed nee-
dle) is he pre erred op ion o he au hors.
T e needle is passed hrough clear, mids ro-
mal cornea approxima ely 2 mm rom he
limbus or approxima ely 3 o 4 mm.
Al erna ively, a superior rec us rac-
ion su ure (4–0 or 5–0 black silk on a
apered needle) can be used o ro a e he
globe in eriorly and bring he superior
bulbar conjunc ival in o view. Using a
muscle hook o ro a e he globe down-
ward, he conjunc iva and superior rec us
are grasped wi h oo hed orceps and he
hreaded needle is passed hrough he is-
sue bundle (Fig. 24-4).
A limbus- (Fig. 24-5) or ornix-based
(Fig. 24-6) conjunc ival ap is made wi h
Wes cot scissors and non oo hed u ili y or-
ceps. A ornix-based ap is more likely o be
associa ed wi h di use blebs.
When orming limbus-based aps, he
conjunc ival incision is placed 8 o 10 mm
pos erior o he limbus. T e conjunc ival
and enon’s wound should be leng hened
o approxima ely 8 o 12 mm cord leng h.
T e ap is hen ex ended an eriorly o
expose he corneoscleral sulcus.
When making ornix-based aps, he
conjunc iva and enon’s are disinser ed.
Approxima ely a 2-clock-hour limbal
peri omy (6 o 8 mm) is su cien . Blun
dissec ion is carried pos eriorly.
A scleral ap is hen dissec ed. T e scleral
ap should comple ely cover he f s ula o
provide resis ance o he aqueous ou ow.
T e uid will ow around he scleral ap.
Di erences in he shape or size o
he scleral ap probably have lit le e ec
on surgical ou come. T e ap hickness
should be be ween one-hal and wo- hirds
(Fig. 24-7).
I is impor an o dissec he ap an e-
riorly (approxima ely 1 mm in o clear
cornea) o ensure ha he f s ula is crea ed
an erior o he scleral spur and ciliary body.
A corneal paracen esis is made be ore
opening he globe (Fig. 24-8) wi h ei her
a 30- or a 27-gauge needle or a sharp poin
blade. A block o issue a he corneoscleral
junc ion is hen excised.
wo radial incisions are made f rs wi h
a sharp blade or kni e s ar ing in clear
cornea, and ex ending pos eriorly approxi-
ma ely 1 o 1.5 mm. T e radial incisions are
made approxima ely 2 mm apar . T e blade
or Vannas scissors are used o connec he
incisions; hereby a rec angular piece o is-
sue is removed (Fig. 24-9).
Al erna ively, an an erior corneal inci-
sion, parallel o he limbus and perpen-
dicular o he eye, is made o en er in o
he an erior chamber and a Kelly or Gass
punch is used o excise he issue.
A peripheral iridec omy may hen be per-
ormed. Iridec omy is no necessary in many
cases (e.g., pseudophakic eyes wi h open

an erior chamber angle), bu recommended
in pa ien s wi h shallow an erior chamber and
angle-closure glaucoma.
T e iris is grasped near i s roo wi h
oo hed orceps. T e iris is re rac ed
hrough he scleros omy, and an iri-
dec omy is per ormed wi h Vannas or
DeWecker scissors (Fig. 24-10).
T e iridec omy should avoid damage o
he iris roo and ciliary body or bleeding.
T e scleral ap is su ured ini ially wi h wo
in errup ed 10–0 nylon su ures (in he case
o rec angular ap; Fig. 24-11) or wi h one
su ure (in a riangular ap). Slipkno s are use-
ul o adjus he igh ness o he scleral ap and
he ra e o aqueous ou ow. Addi ional su ures
can be used o bet er con rol he ou ow.
During he su uring o he scleral ap,
he an erior chamber is f lled hrough he
paracen esis, and he ow around he scleral
ap is observed. I ow seems excessive,
or he an erior chamber shallows, he slip-
kno s are igh ened or addi ional su ures are
placed. I aqueous does no ow hrough he
ap, he surgeon may loosen he slipkno s or
replace igh su ures wi h looser ones.
In some si ua ions, he scleral ap is
igh ly closed o avoid hypo ony, e.g.,
FIGURE 24-2. Topical anes he ic agen s. Xylocaine 2% gel or opical applica ion and lidocaine 1%
nonpreserved or sub enon’s or subconjunc ival injec ion.
Trabeculec omy 353
angle-closure glaucoma, high preopera-
ive IOP. Releasable su ures can be used
(Fig. 24-12) ins ead o in errup ed ones.
Ex ernalized releasable su ures are eas-
ily removed and are e ec ive in cases o
in amed or hemorrhagic conjunc iva or
hickened enon’s capsule.
Conjunc ival closure in limbus-based aps
is done wi h a double or single running su ure
(Fig. 24-13), wi h an 8–0 or 9–0 absorbable
su ure, or wi h 10–0 nylon. Many surgeons
avor a round-body needle.
In ornix-based aps, a igh conjunc ival–
corneal apposi ion is needed. Su ures (e.g.,
mat ress 10–0 nylon su ure; Fig. 24-14)
a he edges o he incision can be used o
anchor he conjunc iva o he cornea.
A er he wound is closed, a 30-gauge
cannula is used o f ll he an erior chamber
wi h balanced sal solu ion (BSS) hrough
he paracen esis rack o eleva e he conjunc-
ival bleb and es or leaks (Fig. 24-13).
An ibio ics and cor icos eroids can be
injec ed in he in erior ornix.
Pa ching he eye is individualized, depend-
ing on he pa ien ’s vision and he anes hesia
used.
 354 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT

FIGURE 24-3. Ballooning o conjunc iva. Ballooning o he conjunc iva wi h nonpreserved lidocaine 1%
( 0.5 mL) using a 30 gauge sharp needle in he direc ion o he superior rec us muscle.

FIGURE 24-4. Trac ion su ure placemen . Placemen o a superior rec us rac ion su ure prior o a limbus
based rabeculec omy.
 Trabeculec omy 355

A B
FIGURE 24-5. Limbus based f ap. Developing a limbus based conjunc ival– enon’s f ap. A. T e conjunc iva
is grasped wi h orceps and eleva ed be ore he ini ial cu , placed approxima ely 8 o 10 mm pos erior o he
limbus. B. T e incision is ex ended la erally exposing he episclera.

A B
FIGURE 24-6. Fornix based f ap. Developing a ornix based conjunc ival– enon’s f ap. A. T e conjunc iva
is grasped wi h orceps and eleva ed be ore he ini ial cu . B. T e conjunc ival incision is done as an eriorly as
possible a he limbus, ex ending 2 o 3 clock hours.
 356 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT

A B

FIGURE 24-7. Par ial hickness f ap. Developing

a one hal o wo hirds par ial hickness scleral
rabeculec omy f ap wi h a ornix based conjunc ival
C
f ap (A and B) or wi h a limbal based f ap (C).

FIGURE 24-8. Corneal paracen esis. A corneal paracen esis is done emporally or nasally be ore crea ing he
s ula. A sharp kni e is used, and a long rack is crea ed.
 Trabeculec omy 357

A B
FIGURE 24-9. Removing he in ernal issue block. A er an ini ial incision wi h a sharp kni e o en er in o
he an erior chamber, he s ula can be crea ed wi h a punch or wi h Vannas scissors (A). T e block excised can
be corneal and/ or limbal (B). No e he preplaced scleral f ap su ures, used o expedi e closure and minimize he
ime when he eye is hypo onous ( A and B) .

A B
FIGURE 24-10. Surgical iridec omy. Surgical iridec omy wi h Vannas scissors ( A and B) a er rabeculec omy
block removal. I is impor an o avoid he iris roo when grasping he peripheral iris.

A B
FIGURE 24-11. Su uring he scleral f ap. T ere are many di eren echniques or su uring he scleral f ap. In
his echnique he surgeon is ying he preplaced in errup ed su ures a each corner o he scleral f ap ( A). A
slipkno is help ul o adjus he igh ness and he amoun o drainage hrough he f ap (B) .
 358 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT

A B

C D

E F
FIGURE 24-12. Releasable su ures. Releasable su ure echnique in a limbus based conjunc ival incision
described by Richard P. Wilson, MD. A 10–0 nylon su ure is used. A and B. S ep 1: T e su ure en ers in o
he cornea 1 mm an erior o he limbus (dep h: mids romal) and exi s hrough he sclera adjacen o he f ap
(going undernea h he corneoscleral limbus and he inser ion o he conjunc iva) . C. S ep 2: T e needle is
passed hrough he scleral f ap and sclera adjacen o he f ap. D. S ep 3: T e needle en ers he sclera and exi s
hrough he cornea (direc ion parallel o s ep 1) . E. S ep 4: T e su ure is hen ied up securely. F. Illus ra ion
o he dep h o he su ure. (Illus ra ions by Chris ine Gralapp; adap ed wi h permission o American Academy
o Oph halmology rom Mos er MR, Azuara Blanco A. Focal Points Volume XVIII, number 6. San Francisco, CA:
American Academy o Oph halmology, 2000.)
 Trabeculec omy 359

A B

FIGURE 24-13. Conjunc ival closure: limbus

based f ap. A. A running 8–0 Vicryl su ure echnique
is used, closing separa ely enon’s layer ( rs )
ollowed by he conjunc iva. B. T e wa er igh ness
o he wound is con rmed by injec ing BSS in o
he an erior chamber and eleva ing he bleb. C.
C Immedia e pos opera ive appearance o a limbus
based conjunc ival– enon’s f ap.
 360 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT

A B

C D
FIGURE 24-14. Conjunc ival closure: ornix based f ap. Closure o a ornix based conjunc ival– enon’s f ap
wi h 10–0 nylon mat ress su ures a each corner ( A–D) .
 Trabeculec omy 361

Intraoperative Application of or 1 o 5 minu es using soaked cellulose

Antimetabolites
o reduce pos opera ive subconjunc ival
f brosis, especially impor an in cases a high
risk or ailure, mi omycin C (MMC) (Fig.
24-15) and 5- uorouracil (5-FU) are used.
T e use o an if bro ic agen s is associa ed
wi h a higher success ra e al hough he risk o
complica ions may also increase. An individu-
alized considera ion o he risk/ benef ra io
is recommended.
MMC (0.2 o 0.5 mg per mL solu ion)
or 5-FU (50 mg per mL solu ion) is applied
sponges (Fig. 24-16) placed over he epi-
sclera. Applica ion under he scleral ap is
also possible. T e conjunc ival– enon layer
is draped over he sponge, avoiding con ac
o he MMC wi h he wound edge.
A er he applica ion, he sponge is
removed and he en ire area is irriga ed hor-
oughly wi h BSS. T e plas ic devices ha
collec he liquid runo (Fig. 24-17A) are
changed and disposed o according o oxic
was e regula ions (Fig. 24-17B).

FIGURE 24-15. MMC solu ion. MMC ( 0.4 mg per mL) as delivered o he opera ing room.

A B
FIGURE 24-16. Applica ion o MMC. Delivery o MMC on a Weck cell sponge under he conjunc iva and
enon’s capsule ( A and B) . In his case large sponges are used. O her surgeons pre er o use mul iple small
sponges.
 362 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT

A B
FIGURE 24-17. Handling o MMC sponges. A. Collec ion o sponges o MMC and con amina ed f uid
a er irriga ion. T e number o sponges used and collec ed should be coun ed. B. Proper disposal o MMC
con amina ed ma erials is manda ory.

POSTOPERATIVE CARE
Topical steroids (e.g., prednisolone ace a e
1%, our imes daily) are apered a er 6 o
8 weeks. Some clinicians use opical non-
s eroidal an i-in amma ory agen s (e.g., 2
o 4 imes a day or 1 mon h). An ibio ics
are required or 1 o 2 weeks a er surgery.
Pos opera ive cycloplegics are u ilized on an
individual basis in pa ien s wi h shallow an e-
rior chambers or in ense in amma ion.
Digi al ocular compression applied o he
in erior sclera or cornea hrough he in erior
eyelid, and ocal compression wi h a mois -
ened cot on ip a he pos erior edge o he
scleral ap, can be use ul o eleva e he bleb
and reduce he IOP in he early pos opera ive
period, especially a er laser su ure lysis (Fig.
24-18).
Su ure lysis and cut ing and pulling releas-
able su ures are necessary when here is a high
IOP, a a f l ra ion bleb, and a deep an erior
chamber. Gonioscopy mus be per ormed
prior o he laser rea men o conf rm an open
scleros omy wi h no issue or clo occluding
i s en rance. Su ure lysis and cut ing and pull-
ing releasable su ures should be done wi hin
he f rs ew weeks a er surgery, al hough i
may be success ul even mon hs a er surgery
in pa ien s in whom MMC had been used.
In cases prone o early ailure (e.g., vascu-
larized and hickened blebs), repea ed subcon-
junc ival applica ions o 5-FU (5 mg in 0.1 mL
solu ion) and an i-vascular endo helial grow h
ac or (an i-VEGF) herapy (see below) over
he f rs 2 o 3 weeks are recommended.
Anti-VEGF T erapy for Bleb Vascularization
T e use o an i-VEGF agen s (bevaci-
zumab and ranibizumab) as an adjunc o
rabeculec omy has recen ly been proposed.
T e wound-healing process is po en ia ed
hrough bo h f broblas ac ivi y and angiogen-
esis. T ere ore, an an i-VEGF agen should
Trabeculec omy 363
decrease new vascular grow h and po en ially
lead o a heal hier bleb wi h less scarring and
bet er long- erm IOP con rol. An i-VEGF
agen s may have a synergis ic e ec wi h
MMC and 5-FU in hose pa ien s whose ra-
beculec omies may ail wi h he use o MMC
or 5-FU alone.
T e use o subconjunc ival an i-VEGF
(bevacizumab 1 mg) a er glaucoma surgery
was f rs repor ed associa ed wi h a bleb nee-
dling procedure a er ailed rabeculec omy.
Many subsequen repor s have illus ra ed he
use o bo h bevacizumab and ranibizumab
as sub-conjunc ival or sub- enon’s injec ions
a er f l ra ion surgery or a he ime o bleb
needle revision.
I has been proposed o use an i-VEGF
injec ions adminis ered proximal o blebs
a er rabeculec omy a he earlies sign o vas-
culariza ion, and his is our curren prac ice.
Fur her s udies are needed o bet er under-
s and he dose and rou e o injec ion as well
as he side e ec prof le o bevacizumab.
Bleb Needling
In cases o subconjunc ival–episcleral
f brosis an ex ernal revision or bleb needling
can be ried. A 27- or 25-gauge needle is used
o cu he edge o he scleral ap and res ore
aqueous ou ow. En ry o he needle ip
in o he an erior chamber benea h he ap
is impor an bu should be under aken wi h
ex reme cau ion in phakic eyes.
T e ou come may be more avorable i here
was a previously well-es ablished f l ra ion bleb
be ore he f s ula became occluded. Repea ed
subconjunc ival injec ions o 5-FU a er revi-
sion increases he probabili y o success.
MMC be ore or a er needling has been
used also in conjunc ion wi h needling o
blebs. We are curren ly using 0.1 cc o non-
preserved 1% lidocaine mixed wi h 0.1 mL
o 0.4 mg per mL MMC a he ime o he
needling.
 364 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT

FIGURE 24-18. Su ure lysis. A Hoskins lens is used o compress he conjunc iva and acili a e he view o he
nylon su ure. (Cour esy o Richard P. Wilson, MD, Wills Eye Hospi al, Philadelphia, PA.)

COMPLICATIONS
Complica ions and heir managemen are
brie y described in Table 24-1 (Figs. 24-19
to 24-29).
TABLE 24-1. Complica ions o
Complication
Conjunc ival but onholes
Early overf l ra ion (Fig. 24-25)
Choroidal e usions (Fig. 24-21)
Suprachoroidal hemorrhage
In raopera ive
Pos opera ive
Aqueous misdirec ion
Bleb encapsula ion (Fig. 24-30) Bleb
dyses hesia (Fig. 24-26)
La e bleb leak (Fig. 24-29)
Chronic hypo ony (Fig. 24-24)
Blebi is (Fig. 24-27), endoph halmi is
AC, an erior chamber; IOP, in raocular pressure.
Trabeculec omy 365
ransien hypo ony is very common a er
glaucoma surgery, and o en well olera ed, bu
occasionally i may lead o o her possible com-
plica ions including a an erior chamber (Fig.
24-19), Desceme ’s membrane olds, cho-
roidal e usions (Fig. 24-20), suprachoroidal
rabeculec omy
Treatment
Purse s ring su ure wi h 10–0 or 11–0 needle on a rounded
(“vascular”) needle.
I AC is shallow or a wi h no lens–cornea ouch, use cycloplegics,
res ric ion in ac ivi y, and avoidance o Valsalva maneuvers. I
here is lens–corneal ouch, per orm urgen re orma ion o AC. I
complica ion persis s, resu ure scleral ap.
Observa ion, cycloplegics, s eroids. Drainage is considered i
e usions are apposi ional and associa ed wi h a an erior chamber.
Promp closure o eye and gen le reposi ion o prolapsed uvea.
In ravenous manni ol and ace azolamide.
Observa ion; con rol IOP and pain. Drain (a er 7–10 days) cases
wi h persis en a AC and in olerable pain.
Ini ial medical rea men : in ensive opical cycloplegic–mydria ic
regimen, opical and oral aqueous suppressan s, and osmo ics.
In pseudophakics: Nd:YAG laser hyaloido omy or peripheral an erior
vi rec omy via AC.
In phakics: phacoemulsifca ion and an erior vi rec omy. Pars plana
vi rec omy.
Ini ial observa ion. Aqueous suppressan s i IOP is eleva ed. Consider
needling wi h 5 uorouracil or surgical revision.
Topical lubrica ion. Compression su ures (Palmberg’s).
I leak is no brisk, ini ial observa ion and opical an ibio ics. I i
persis s, surgical revision (conjunc ival advancemen or au ogra ).
I here is decreased vision or maculopa hy: ransconjunc ival ap
su ures or revision o he scleral ap.
Bleb in ec ion wi hou in raocular involvemen : in ensive opical
rea men wi h wide spec rum or ifed an ibio ics.
Bleb in ec ion wi h mild an erior segmen cellular reac ion: in ensive
opical rea men wi h or ifed an ibio ics.
Bleb in ec ion wi h severe an erior segmen cellular reac ion or
vi reous involved: vi reous sample and in ravi real an ibio ics.
 366 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT
hemorrhage (Fig. 24-21), ca arac , macular
and op ic disc edema, and chorio-re inal olds
(predominan ly in young myopic pa ien s).
Hyphema is no rare (Fig. 24-22), bu o en
resolves wi h conserva ive managemen .
T e ini ial managemen o early pos -
opera ive hypo ony wi h a ormed an e-
rior chamber is conserva ive wi h opical
s eroids and cycloplegics. In erven ion is
indica ed in cases when hypo ony is associ-
a ed wi h o her complica ions such as per-
sis en low IOP wi h loss o visual acui y
and hypo ony maculopa hy (Fig. 24-23,
see below). rea men should be aimed a
correc ing he specif c cause o hypo ony.
Mos commonly, hypo ony is due o over-
f l ra ion o a f l ering bleb. When here is
a a an erior chamber wi h lens–corneal
ouch, immedia e surgical in erven ion is
necessary o preven endo helial damage
and ca arac orma ion. Re orma ion o he
an erior chamber wi h viscoelas ic can be
done a he sli lamp or under he opera ing
microscope hrough he paracen esis made
in raopera ively. When here are large
apposi ional choroidal e usions, drainage
o he uid is recommended.
When overf l ra ion persis s (Fig. 24-24),
several rea men s can be used o induce an
in amma ory or healing reac ion in he f l er-
ing bleb which modif es he morphology o he
f l ering blebs and increase he IOP. Surgical
revision is he mos e cacious op ion.
Palmberg ransconjunc ival su ures are
o en help ul o reduce overf l ra ion (Fig.
24-25). Resu uring he scleral ap hrough
he conjunc iva is curren ly our avored
op ion. Su uring he scleral ap can be
done hrough he conjunc iva, as repor ed
by Shira o e al.1 A er anes he ic eye drops
are ins illed, he f l ering bleb on he scleral
ap is compressed by a cot on ip and
released o conf rm he si e o excess f l ra-
ion a he scleral ap. T en, using a round,
apered needle wi h 10–0 nylon su ure,
he scleral ap is su ured igh ly direc ly
hrough he conjunc iva. I necessary, an
addi ional su ure can be placed and igh -
ened o achieve a wa er igh closure.
When i is di cul o see he margin
o he scleral ap, compression and su ur-
ing while observing wi h a Hoskins lens
are help ul. Usually, small leakage a he
su ured poin s occurs, especially when he
bleb wall is hin. However, i s ops spon a-
neously a ew hours or days la er because
o he decrease in f l ra ion and/ or down-
sizing o he f l ering bleb. A er he proce-
dure, a opical an ibio ic is prescribed. T e
su ure is buried in he conjunc iva spon a-
neously in 1 week in all cases.
Occasionally, bleb revision or overf l-
ra ion mus be associa ed wi h pa ch gra -
ing (i.e., in cases o incompe en scleral
ap, when resu uring is no possible).
La e bleb-rela ed in ec ion can be a very
severe complica ion, po en ially leading o
endoph halmi is (Fig. 24-26). I is more
common in surgeries supplemen ed wi h
MMC and in leaking (Figs. 24-27 to 24-29),
avascular, hin, localized f l ering blebs.
REFERENCE
1. Shira o S, Maruyama K, Haneda M. Resu uring he
scleral ap hrough conjunc iva or rea men o excess
f l ra ion. Am J Ophthalmol. 2004;137:173–174.
 Trabeculec omy 367

FIGURE 24-19. Fla an erior chamber. T e an erior chamber is f a (Spae h’s Grade II), wi h iris–cornea
ouch, bu no lens–cornea ouch.

FIGURE 24-20. Choroidal de achmen . Fundus pho ograph showing serous choroidal de achmen obscuring
he op ic nerve.
 368 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT

FIGURE 24-21. Suprachoroidal hemorrhage. Sli lamp pho ograph o an eye wi h suprachoroidal hemorrhage;
he re ina can be seen hrough he pupil.

FIGURE 24-22. Hyphema. Hyphema ollowing rabeculec omy.

 Trabeculec omy 369

FIGURE 24-23. Chronic hypo ony. Fundus pho ograph showing re inal olds and or uosi y o he vessels—
so called hypo ony maculopa hy.

FIGURE 24-24. Over l ra ion. Over l ra ion caused by an exuberan bleb. No e he avasculari y o he bleb.
 370 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT

FIGURE 24-25. Palmberg su ures. ransconjunc ival su ures can be used o reduce he size o he bleb and
rea over l ra ion.

A B
FIGURE 24-26. Bleb rela ed ocular in ec ion. A. Sli lamp pho ograph o a case o blebi is; purulen discharge
can be seen on he bleb. B. Hypopyon in a pa ien wi h blebi is.
 Trabeculec omy 371

FIGURE 24-27. Conjunc ival but onhole. Conjunc ival but onhole iden i ed several weeks ollowing
rabeculec omy wi h MMC. T e arrowheads deno e he edges o he hole.

FIGURE 24-28. La e bleb leak. La e bleb leak 3 years ollowing rabeculec omy surgery. Under cobal blue
ligh , he Seidel es shows aqueous f ow rom he leak. T e bleb is small, localized, and avascular.
 372 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT

FIGURE 24-29. Encapsula ed bleb. ypically encapsula ed blebs are localized and vascularized.
 Ex Press Mini Glaucoma Shun 373

reliable pos opera ive course han s andard

EX
EX-PRESS
X-PR
R E SS MIN
M IN
NI rabeculec omy. Noncompara ive s udies have
GLAUCO
GL
L AU C O MA
M A SH
SH UN sugges ed ha he incidence o early hypo ony
wi h Ex-PRESS shun is lower han a er rab-

T he Ex-PRESS is a small s ainless-s eel

implan ha is inser ed hrough he
limbus in o he an erior chamber under a 4
eculec omy, wi h similar clinical e cacy.1
REFERENCE
1. Lankaranian D, Razeghinejad MR, Prasad A, e al.
o 5-mm–wide par ial- hickness scleral ap, In ermedia e- erm resul s o he Ex-PRESS( M)
similar o a s andard rabeculec omy ap. minia ure glaucoma implan under a scleral ap
T ere are several models wi h di eren shapes in previously opera ed eyes. Clin Experiment Ophth-
and sizes (leng h range rom 2.4 o 2.9 mm). almol. 2011;39(5):421–428. doi: 10.1111/ j.1442–
9071.2010.02481.x. [Epub ahead o prin ]
T e in ernal lumen diame er varies be ween
50 µm (mos commonly used) and 200 µm.
T e appeal o he Ex-PRESS shun is ha i
may provide a more uni orm, consis en , and
 374 24 TRABECULECTO MY AND EX-PRESS MINI GLAUCO MA SHUNT

SURGICAL TECHNIQUE in roduced wi h a disposable inser er, paral-

T e surgical echnique or Ex-PRESS
implan a ion is similar o s andard rabeculec-
omy. A er conjunc ival dissec ion subcon-
junc ival MMC is usually applied o minimize
scarring and bleb ailure. T en he scleral ap is
li ed, ex ending he dissec ion 1 o 2 mm in o
he cornea. A 27- o 25-gauge needle (depend-
ing on he size o he device, and avoiding
la eral movemen o he needle) is needed
o crea e he en rance, and he shun is hen
lel o he iris plane (Fig. 24-30). T e shun is
inser ed all he way in o he wound making he
pla e ush wi h he scleral bed. Immedia e ow
is hen observed hrough he lumen.
Scleral ap closure wi h adjus able or
releasable su ures is similar o rabeculec omy.
Wa er igh conjunc ival closure is impor an
(see above). Pos opera ive managemen
( opical s eroids, use o pos opera ive 5-FU or
an i-VEGF) is similar o he s andard rabecu-
lec omy (Fig. 24-31).

A B

FIGURE 24-30. Ex PRESS mini shun

implan a ion. Surgical echnique. A. A er he
scleral f ap is dissec ed, a needle is used o crea e
he en rance. B. T e shun is in roduced using a
C disposable inser er. C. T e implan is secure and he
scleral f ap will be su ured over he implan .
 Ex Press Mini Glaucoma Shun 375

FIGURE 24-31. Ex PRESS mini shun . Pos opera ive appearance.

 C H AP T ER
25
Glaucoma Drainage Devices
DESCRIPTION
Glaucoma drainage devices (GDDs) are
designed o lower in raocular pressure (IOP).
T ey are also known as aqueous shun s, aque-
ous shun ing devices, or ube shun s.
radi ionally, hey have been used o
lower IOP in cases o ailed f l ra ion surgery,
recalci ran and complex glaucomas, such
as in amma ory, neovascular, and rauma ic
glaucoma. Increasingly since he publica ion
o he ube versus rabeculec omy S udy in
2007,1 some cen ers are using GDDs as a pri-
mary glaucoma surgery.
All GDDs consis o a silicone ube con-
nec ed o an episcleral pla e (or explan ),
which is made o various ma erials in di eren
shapes and sur ace area sizes depending on
he specif c model (Figs. 25-1 and 25-2).
T e pla e is placed agains sclera a he globe’s
equa or, while he ube is inser ed in o he
an erior chamber (or more rarely hrough he
pars plana). Despi e nonreac ive ma erials,
a collagenous and f brovascular capsule (or
bleb) develops around he episcleral pla e.
Once wi hin he bleb, aqueous ows passively
376
JoAnn A. Giaconi and Marlene R. Moster
hrough he wall o he capsule and is reab-
sorbed by venous capillaries and lympha ics.
GDDs can be divided in o res ric ive and
nonres ric ive devices.
Nonrestrictive, or nonvalved, devices per-
mi he ree ow o uid rom inside he
eye o he episcleral pla e. Nonres ric ive
shun s on oday’s marke include he vari-
ous models o Mol eno and he Baerveld
GDDs (Fig. 25-1). Resis ance o ou ow
depends on he densi y o he capsule ha
orms around he pla e.
Restrictive, or valved, devices incorpora e
a ow-con rolling elemen wi hin he pos-
erior par o he ube (i.e., valve or mem-
brane) designed o limi uid ow in an
at emp o preven pos opera ive hypo ony.
Res ric ive models available are he Ahmed
(Fig. 25-2) and Krupin GDDs.
T us ar, no one GDD model is supe-
rior o he o hers. T e Ahmed Baerveld
Comparison (ABC) rial is a mul icen er,
randomized clinical rial comparing he sa e y
and e cacy o he Ahmed FP-7 and Baerveld
350-mm2 implan s. As o his prin ing, here
is no ye a published paper rom his s udy;
 Description 377

however, preliminary resul s have been pre- REFERENCES

sen ed a mee ings. T e mos recen resul s
presen ed in 2010 showed ha a 1 year o
ollow-up, mean IOP was 2.2 mm Hg lower
in he Baerveld group on a similar number
o glaucoma medica ions, bu ha here were
also a grea er number o early and serious
complica ions in he Baerveld group com-
pared o he Ahmed group.2
1. Gedde SJ, Schi man JC, Feuer WJ, e al. rea men
ou comes in he ube versus rabeculec omy s udy
a er one year o ollow-up. Am J Ophthalmol.
2007;143(1):9–22. Epub 2006 Sep 1.
2. Budenz DL. Ahmed or Baerveld —which is be -
er? Resul s o he ABC rial. American Academy
o Oph halmology 2010 Subspecial y Day Program
Book, 51–52.

FIGURE 25-1. Nonrestrictive GDD. Models commercially available. A–D. Mol eno devices. T e newer
Mol eno3 shun s (A and B) are charac erized by a larger, more f exible episcleral pla e han he older models
(C and D). Numbers adjacen o models indica e sur ace area o episcleral pla e. E and F. Baerveld devices, he
pla es o which are made o barium-impregna ed silicone.
 378 25 GLAUCO MA DRAINAGE DEVICES

FIGURE 25-2. Restrictive devices. Ahmed valves come wi h a f exible pla e (FP models) or rigid
polypropylene pla e (S models) . T e smaller sizes are in ended or pedia ric eyes. T e double-pla e model
consis s o a valved pla e connec ed o a nonvalved pla e by an in ervening silicone ube ha crosses over a rec us
muscle (in his gure, he in ervening ube is no connec ing he wo pla es on he bot om righ ) .

SURGICAL
S UR
R G IC
C AL E
ECH
CH
H N IQ
Q UE
E ube may or may no be f xed o he sclera
Local anes hesia is recommended, as some
o he surgical s eps can be pain ul wi hou
adequa e anes hesia. A re robulbar, peribul-
bar, or sub- enon’s injec ion can be u ilized.
T e supero emporal quadran (Fig. 25-3)
is he pre erred loca ion or implan a ion
o a f rs GDD (addi ional shun s can be
implan ed in o her quadran s). For bet er
exposure o he surgical si e, a corneal rac-
ion (Fig. 25-4) or superior rec us bridle
su ure is help ul. T e ube should be ushed
wi h balanced sal solu ion o ensure pa ency
using a 30-gauge cannula.
Generally, a ornix-based peri omy is used.
A 90- o 110-degree conjunc ival incision is
adequa e or single-pla e implan s. S evens
eno omy scissors are used o blun ly dissec
pos eriorly in he quadran o make room
or he pla e. T e episcleral pla e is placed
be ween adjacen rec us muscles wi h i s
an erior edge a leas 8 mm pos erior o he
limbus (Fig. 25-5). Nonabsorbable su ures
(6–0 o 8–0 nylon, prolene, mersilene) are
passed hrough he f xa ion holes o he epi-
scleral pla e and su ured o he sclera.
T e op imal leng h o ubing is es ima ed
by laying he ube across he cornea. T e ube
is hen rimmed, bevel up, o ex end 2 o
3 mm in o he an erior chamber (Fig. 25-6).
A corneal paracen esis is made o provide
access o he an erior chamber in case o col-
lapse (Fig. 25-7). A 22- or 23-gauge needle
is used o crea e a rack in o he an erior
chamber, parallel o he plane o he iris, s ar -
ing approxima ely 1 o 2 mm pos erior o he
corneoscleral limbus (Fig. 25-8). T e ube is
hen inser ed hrough his rack in o he
an erior chamber wi h smoo h orceps
(Fig. 25-9).
Proper posi ioning o he ube in he
an erior chamber is essen ial, ensuring ha i
does no ouch he iris, lens, or cornea. T e
Surgical Technique 379
wi h su ures o 10–0 nylon or prolene (Fig.
25-10). T is an erior su ure is wrapped
igh ly around he ube o preven movemen
in o or ou o he an erior chamber, bu no
so igh ly ha i occludes he ube lumen.
o avoid conjunc ival erosion by he ube,
processed pericardium, donor sclera or cor-
nea, and less commonly ascia la a or dura,
are used o cover he an erior scleral por ion
o he ube (Fig. 25-11). T e pa ch gra is
su ured in place using in errup ed su ures.
Al erna ively, a par ial- hickness limbal-based
scleral ap can be ashioned, and he ube
en ry is made undernea h his ap so ha he
su ured ap covers he ube.
T e ube can also be placed hrough he
pars plana (Figs. 25-8 to 25-26) in cases
where placemen in o he an erior chamber
is di cul or undesirable. T is approach
requires a pars plana vi rec omy by a re ina
surgeon wi h care ul at en ion o remove he
vi reous skir in he quadran where he ube
will be inser ed.
During he inser ion o nonrestrictive
devices, an addi ional s ep is needed o pre-
ven immedia e and prolonged pos opera-
ive hypo ony—occlusion o he ube. T is
s ep can be per ormed be ore su uring down
he episcleral pla e in a number o ways.
Absorbable 6–0 o 8–0 Vicryl su ure can be
ied around he ube so ha here is no ow
o he pla e. Vicryl o his size will dissolve in
4 o 8 weeks, by which ime a ow-res ric ive
capsule will have ormed around he pla e.
Because he ube is comple ely liga ed, several
ven ing sli s in he an erior ex rascleral por-
ion o he ube can be made wi h a needle
or 15-degree blade o allow some aqueous
ou ow in he early pos opera ive period. T e
amoun o aqueous egress can be checked
wi h a 27-gauge cannula on a syringe wi h
saline inser ed in o he end o he ube. I he
pressure canno be con rolled wi h
 380 25 GLAUCO MA DRAINAGE DEVICES
medica ion during he period be ore he
liga ure dissolves, abla ing he Vicryl su ure
wi h an argon laser can open he ube
( he su ure needs o be pos erior enough o
he pa ch gra in order o be seen). Ano her
op ion is a ripcord su ure o 5–0 nylon
su ure, 3–0 prolene, or 3–0 mersilene placed
in o he ube, en ering rom he pla e end
(Fig. 25-13). An absorbable su ure is hen
ied around he ube and he ripcord su ure
(Fig. 25-14). T e dis al end o he ripcord
is placed subconjunc ivally in he in erior
quadran , where i can be accessed by cut ing
FIGURE 25-3. Superotemporal quadrant. Placemen o an Ahmed ube shun in he supero emporal quadran
o he eye receiving he rs GDD.
hrough he conjunc iva and pulled weeks
a er surgery, i IOP needs o be lowered.
T e ripcord su ure has he advan age o no
requiring rea men wi h an argon laser i
early opening o he ube is needed. T e hird
op ion is o ie o he in racameral por ion
o he ube be ore inser ing i in o he an e-
rior chamber (Fig. 25-15). T is is generally
done wi h nylon or prolene su ure, which
can be lysed wi h he laser i IOP needs o be
brough down in he pos opera ive period. A
wa er igh conjunc ival closure comple es he
procedures.
 Surgical Technique 381

FIGURE 25-4. Traction suture. A superior rac ion su ure o 6–0 silk placed hrough corneal s roma allows
in raduc ion o he eye or bet er exposure o he supero emporal quadran .

FIGURE 25-5. Scleral f xation. Ahmed ube shun sewn down o sclera 8–10 mm pos erior o he limbus using
nonabsorbable su ure.
 382 25 GLAUCO MA DRAINAGE DEVICES

FIGURE 25-6. Tube trimming. Prior o inser ion in he eye, he ube is rimmed o an appropria e leng h wi h
Wes cot scissors so ha i will res 2 o 3 mm long wi hin he an erior chamber. When cu , he bevel should ace
upwards o decrease he risk o iris incarcera ion in o he ube.

FIGURE 25-7. Corneal paracentesis. T is is no considered o be a manda ory s ep, bu in he case o an erior
chamber collapse in raopera ively or f a chamber pos opera ively, i allows access o re orm he an erior
chamber.
 Surgical Technique 383

FIGURE 25-8. Track creation. A 23-gauge needle en ering he an erior chamber prior o placemen o he ube.
T is rack should be crea ed a he pos erior surgical limbus or ideal ube posi ion.

FIGURE 25-9. Tube insertion. Placemen o he ube shun in o he an erior chamber.

 384 25 GLAUCO MA DRAINAGE DEVICES

FIGURE 25-10. Tube f xation. Su uring he ube shun o he sclera wi h 10–0 nylon. T is ype o su ure can
help direc he ube oward a rack ha is no direc ly in ron o i (i.e., i can change he course o he rack so
ha i will hold a bend where he su ure is placed) , or i can be used o help preven movemen o he ube. T is
is an op ional s ep.

FIGURE 25-11. Patch gra . A pericardial pa ch gra is placed over he ube be ore su uring i o episclera a
wo o our corners.

FIGURE 25-12. Pars plana tube shunt. T e ube en ers hrough he scleros omy used or pars plana vi rec omy.
 Surgical Technique 385

FIGURE 25-13. Latina or ripcord suture. T e Baerveld 350-mm2 ube shun wi h prepared ripcord placed in
he an erior chamber.

A B
FIGURE 25-14. Ripcord suture. A. Ripcord su ure easily seen wi hin lumen o ube. T e absorbable Vicryl
su ure is ied igh ly around he ube wi h ripcord o preven ou f ow. B. Subconjunc ival end o a ripcord is
visible in he pos opera ive eye.

FIGURE 25-15. Ligature suture. Nonabsorbable su ure liga ing in racameral ube.
 386 25 GLAUCO MA DRAINAGE DEVICES
POSTOPERATIVE CARE
T e pos opera ive regimen includes a opical
an ibio ic or 1 o 2 weeks and opical s eroids
or 2 o 3 mon hs pos opera ively. Occasionally,
a cycloplegic is used i here is an erior chamber
shallowing. Nons eroidal an i-in amma ory
drops can also be used concomi an ly.
Wi h res ric ive devices, a hyper ensive
phase may develop 4 o 16 weeks pos op-
era ively as he capsule orms. Pos opera ive
checks should be scheduled o ca ch his
phase as i develops, as i may require he rein-
roduc ion o hypo ensive medica ions.
Wi h nonres ric ive, liga ed devices, pre-
opera ive hypo ensive medica ions mus be
con inued un il he ube opens.
Weekly visi s should s ar around 5
o 6 weeks an icipa ing liga ure opening.
Hypo ensive medica ions and in amma ory
medica ions will be adjus ed as necessary.
COMPLICATIONS
Implan a ion o aqueous ube shun s is
associa ed wi h a risk o signif can pos opera-
ive complica ions.
Early pos opera ive complica ions
Hypo ony: T is is usually he resul o
excessive aqueous ou ow and may lead o
complica ions lis ed below.
Hypo ony maculopa hy (Fig. 25-16)
Fla an erior chamber (Fig. 25-17)
Choroidal e usions (Figs. 25-18 and
25-19)
Suprachoroidal hemorrhage (Fig.
25-20)
Aqueous misdirec ion (Fig. 25-21)
Hyphema
Ocular hyper ension: High IOP can be
rela ed o occlusion o he ube by f brin
(Fig. 25-22), a blood clo , iris, or vi reous.
Fibrin or blood may resolve spon ane-
ously. An in racameral injec ion o issue
plasminogen ac iva or may help o dis-
solve he clo wi hin a ew hours bu can
be associa ed wi h severe bleeding. When
iris issue occludes he lumen o he ube,
neodymium (Nd):YAG laser irido omy or
argon laser iridoplas y may rees ablish he
pa ency o he ube. Vi reous incarcera ion
can be success ully rea ed wi h Nd:YAG
laser, bu an an erior vi rec omy may be
necessary o preven recurrence.
La e pos opera ive complica ions
Ocular hyper ension: La e ailure wi h
increased IOP is usually caused by exces-
sive f brosis around he pla e.
Hypo ony
Conjunc ival erosion over he ube
(Fig. 25-23) or episcleral pla e
Corneal edema or decompensa ion
(Fig. 25-24). Corneal decompensa ion
may resul rom direc con ac be ween
he ube and he cornea. When he ube is
ouching he cornea, reposi ioning o he
ube should be considered, especially in
cases where here is a risk o endo helial
ailure (i.e., cases wi h ocal corneal edema,
or a er pene ra ing kera oplas y).
ube re rac ion, migra ion, or malposi-
ion: Figure 25-25 shows a ube ha has
eroded hrough he cornea and is res ing
on he cornea’s sur ace. ubes placed in
young pedia ric eyes are especially prone
o changing posi ion wi hin he an erior
chamber over ime because he cornea and
sclera are pliable and do no always hold
he ube in place (Fig. 25-26).
Ca arac
Diplopia: Mechanical res ric ion o he
ex raocular muscles by he bleb or epi-
scleral pla e can cause diplopia. I diplopia
is persis en and no responsive o prisms,
 Surgical Technique 387

he shun may need o be removed or pla e go ull hickness hrough sclera and
reloca ed. re ina.
Re inal de achmen : T is can occur i Endoph halmi is
he needle passes o secure he episcleral

FIGURE 25-16. Chronic hypotony. Chronic hypo ony wi h maculopa hy and choroidal olds.

FIGURE 25-17. Shallow anterior chamber. Shallow an erior chamber ollowing ube shun wi h chamber-
main aining su ure holding back he lens implan .
 388 25 GLAUCO MA DRAINAGE DEVICES

FIGURE 25-18. Choroidal e usion. Fundus pho ograph showing serous choroidal de achmen impinging on
he op ic nerve.

FIGURE 25-19. Choroidal detachment. B-scan ul rasound o serous choroidal de achmen s.

FIGURE 25-20. Suprachoroidal hemorrhage. Sli -lamp pho ograph o an eye wi h suprachoroidal
hemorrhage; he re ina can be seen hrough he pupil.
 Surgical Technique 389

FIGURE 25-21. Aqueous misdirection. Sli -lamp pho ograph o an eye wi h aqueous misdirec ion. T ere is a
f a an erior chamber and an eleva ed IOP.

FIGURE 25-22. Implant occlusion. Fibrous membrane occluding he lumen o a GDD.

 390 25 GLAUCO MA DRAINAGE DEVICES

FIGURE 25-23. Conjunctival erosion. Erosion o he conjunc iva over he an erior scleral por ion o a GDD
near he limbus. T ere is an increased risk o endoph halmi is wi h his complica ion. (Cour esy o Simon Law,
MD.)

FIGURE 25-24. Corneal decompensation. Pericardial pa ch gra overlying ube visible a limbus in
decompensa ed cornea.
 Surgical Technique 391

FIGURE 25-25. Tube malposition. ube erosion hrough cornea and res ing on he sur ace o cornea.
(Cour esy o Simon Law, MD.)

FIGURE 25-26. Implant migration. Migra ion o he ube shun an eriorly in o he an erior chamber in a
14-year-old pa ien wi h congeni al ca arac and glaucoma.
 C H AP T ER
26
Schlemm’s Canal-based Surgery
T he surgical rea men o eleva ed in raoc-
ular pressure (IOP) a he si e o ou f ow
obs ruc ion has long been a goal o glaucoma
managemen . Canal-based surgery is no new.
In ac , rabeculec omy was rs described as
a way o enhance ou f ow hrough he excised
rabecular meshwork ( M) in o he canal.
T ough ur her s udies revealed ha rabecu-
lec omy unc ioned by direc ed f ow o he
subconjunc ival space, he search or a sa e and
e ec ive canal-based surgery did no s op. T e
curren in eres in procedures in and around he
canal is a resul o a number o ac ors includ-
ing he complica ions ha arise in he early and
la e pos opera ive period a er rabeculec omy
bu also as a resul o he developmen o more
sophis ica ed surgical ins rumen s and devices
allowing easier access o he canal; such is he
case or canaloplas y, rabec ome, and iS en .
INDICATIONS
Canal-based procedures have been used
success ully in he ull spec rum o open-
angle glaucomas rom congeni al o adul
primary open angle including pigmen ary and
pseudoex olia ion.
392
Richard A. Lewis
Open angles are he only prerequisi e.
Clear media is necessary or he ab
in erno-based rabec ome and iS en .
Canaloplas y is per ormed ab ex erno and
can be per ormed in he presence o hazy
media or scarred cornea.
CANALOPLAST Y
T
he concep o nonpene ra ing glaucoma
surgery has evolved as an al erna ive o
ull- or par ial- hickness procedures ha rely
on subconjunc ival f ow and a bleb. T e com-
plex and uniquely individual variabili y o
hese procedures due o wound healing lead o
a search or a more direc surgical rea men o
glaucoma. T e rs nonpene ra ing procedure
o u ilize a microca he er (i rack, iScience
In erven ional Inc., Menlo Park, CA) o ake
advan age o he ull ex en o he canal was
canaloplas y, rs described in 2007.
Mechanism of Action
Ul rasound s udies o pa ien s wi h primary
open-angle glaucoma (POAG) demons ra e
collapse or narrowing o he canal o Schlemm.
During he procedure, he canal is dila ed, he

M is ensioned, and a er removal o he deep
scleral f ap, a Desceme ’s window is crea ed.
Canaloplas y is hough o lower IOP
primarily by enhancing conven ional cir-
cum eren ial ou f ow hrough he canal and
he collec or sys em. S udies using dyes and
viscoelas ic have con rmed enhanced in ra-
opera ive ou f ow in his ashion. T e grea er
he canal su ure ension, he grea er he IOP-
lowering e ec . Whe her his con inues o
unc ion mon hs o years pos opera ively
has no been valida ed. S ruc ural dila ion o
he canal has been demons ra ed or a leas
2 years (Fig. 26-1).
O her si es o drainage have been pos-
ula ed. T ese include percola ion or f ow
hrough Desceme ’s window in o a “scleral
lake.” Some pa ien s are no ed o have orma-
ion o a bleb sugges ing ransscleral f ow or a
“mini” per ora ion hrough he window.
Technique
A er crea ing a super cial scleral f ap wi h
4-mm base, a deeper scleral f ap is used o
access he canal (Fig. 26-2).
T e i rack microca he er (Fig. 26-3) is
placed in he canal and hreaded or he ull
360 degrees un il i comes ou he o her end.
A 10–0 prolene su ure is at ached o he
dis al end and he ca he er is wi hdrawn
while injec ing viscoelas ic. T e remaining
prolene su ure is ied igh ly, crea ing ension
in he meshwork o enhance ou f ow in o he
canal.
Be ore su uring he super cial f ap and
conjunc iva, he deeper scleral f ap is excised
Canaloplasty 393
leaving a Desceme ’s window o ur her
enhance ou f ow (Fig. 26-4).
Blebs are avoided.
Outcomes
On he basis o he published da a, canalo-
plas y e cacy resul s are comparable o he
published repor s o rabeculec omy. Mean
IOP a 2 years decreased o 16.2 mm Hg in
eyes having canaloplas y alone. In eyes under-
going combined canaloplas y and ca arac
surgery, IOP decreased o 13.7 mm Hg and
medica ion use decreased o 0.6 and 0.2,
respec ively
S udies demons ra e ha canaloplas y is
sa er han rabeculec omy. Hypo ony, cho-
roidal de achmen , and bleb in ec ions were
repor ed in less han 1% o all cases. T e mos
common side e ec is ransien hyphema.
BIBLIOG APH Y
Lewis R , von Wol K, e z M, e al. Canaloplas y:
Circum eren ial viscodila ion and ensioning o
Schlemm’s canal using a f exible microca he er or he
rea men o open-angle glaucoma in adul s: In erim
clinical s udy analysis. J Cataract Ref act Surg. 2007;
33:1217–1226.
Lewis R , von Wol K, e z M, e al. Canaloplas y:
Circum eren ial viscodila ion and ensioning o
Schlemm’s canal using a f exible microca he er or he
rea men o open-angle glaucoma in adul s: wo-year
in erim clinical s udy resul s. J Cataract Ref act Surg.
2009;35:814–824.
Shingle on B, e z M, Korber N. Circum eren ial viscodi-
la ion and ensioning o Schlemm canal (canaloplas y)
wi h emporal clear corneal phacoemulsi ca ion ca a-
rac surgery or open-angle glaucoma and visually sig-
ni can ca arac ; one-year resul s. J Cataract Ref act
Surg. 2008;34:433–440.
 394 26 SCHLEMM’S CANAL-BASED SURGERY

A B
FIGURE 26-1. Canaloplasty ef ect. Ul rasound image o canal pre-op (A) and 2 years pos op (B). A. T e
collapsed canal in open-angle glaucoma sugges s limi ed f ow. B. Canaloplas y viscodila es he canal during
surgery and acili a es drainage in o he collec ors.

A B

FIGURE 26-2. Scleral dissection. A guarded blade is used o crea e a 300-µm par ial- hickness groove (A and
B), ollowed by sharp dissec ion o crea e a sclerocorneal f ap (C). D. A deeper f ap is dissec ed a approxima ely
99% dep h. E. Once Schlemm’s canal is unroo ed, a modi ed Drysdale spa ula is used o blun ly dissec he
inner corneoscleral f ap rom Desceme ’s membrane wi h care ul at en ion no o rup ure and en er he an erior
chamber. F. T e appearance o bo h dissec ed f aps; here is some blood ref ux rom each end o he exposed
Schlemm’s canal.
( continued)
 Canaloplasty 395

C D

E F

FIGURE 26-2. ( Continued)

 396 26 SCHLEMM’S CANAL-BASED SURGERY

FIGURE 26-3. iTrack 250A canaloplasty microcatheter. T e 200-µm–diame er ca he er wi h a 250-µm ip is

at ached o a bat ery-powered ligh source wi h a second at achmen o acili a e injec ing viscoelas ic o dila e
he canal upon removal. (Cour esy o iScience In erven ional.)

FIGURE 26-4. Prolene suture in canal adjacent to Descemet’s window. Wi h removal o he microcannula,
he wo ends o he at ached prolene su ure are ied igh . No e he large clear Desceme ’s window a er excising
he deep scleral f ap.

RABEC
R ABE
E C O ME
ME
T rabec ome (NeoMedix Inc., us in, CA)
became commercially available in 2006
(Fig. 26-5). I is a minimally invasive ab in erno
procedure designed o reduce IOP by enhanc-
ing drainage in o he canal o pa ien s wi h
open-angle glaucoma. I can be combined wi h
ca arac surgery or as a s andalone procedure.
MECH ANISM OF ACTION
I is generally agreed ha he primary si e
o resis ance in open-angle glaucoma resides
in he jux acanalicular area. rabec ome
procedures abla e he jux acanalicular area o
elimina e he area o resis ance, crea ing direc
f ow in o he canal and collec ors.
T e absence o resis ance has o her impli-
ca ions. In set ings o eleva ed episcleral pres-
sure (as migh occur when a pa ien ’s head is
posi ioned lower han he hear ), here is con-
cern o blood ref ux rom he collec or chan-
nels back in o he canal and an erior chamber
and po en ially inducing a hyphema.
TECHNIQUE
T e surgery uses a Swann–Jacobs gonio-
prism or direc visualiza ion. In order o
maximize he angle s ruc ures, he pa ien ’s
head posi ion is ro a ed opposi e o he eye
receiving rea men .
A er crea ing a clear corneal paracen esis
si e, a viscoelas ic is used o main ain an erior
chamber dep h.
T e elec rosurgical pulse is applied
hrough he handpiece o remove 3 o 6 clock
hours o M.
T e rabec ome hand piece simul ane-
ously irriga es, abla es, and aspira es he
rea ed area (Fig. 26-6).
Trabectome 397
OUTCOMES
A case series sugges ed an IOP reduc-
ion o 40% or up o 2 years. Success ra es
(de ned as a decrease in IOP by 20% and
decrease in medica ions o achieve arge
IOP) were 78% and 64% a 6 and 12 mon hs,
respec ively. However, sample size at ri ion
was a signi can issue.
A subsequen s udy showed a much lower
success ra e o be ween 30% a 1 year wi h
success being de ned as a decrease o IOP
<21 mm Hg and >20% rom baseline as well
as he lack o hypo ony or need or addi ional
glaucoma surgery.
T e presence o blood ref ux a he con-
clusion o he procedure is con rma ion o
reduced ou f ow resis ance. T is is a ransien
complica ion ha usually resolves wi hin 1 o
2 weeks.
O her possible complica ions rom
rabec ome such as in ec ion, wound leak,
and choroidal e usion are seldom seen.
T e value o his procedure is i s rela ive
echnical ease per orming he surgery. Also,
his ab in erno procedure allows sparing o
he conjunc iva; hus, u ure l ering or drain-
age device surgery will no be compromised.
BIBLIOG APH Y
Francis BA, Minckler DS, Dus in L, e al. Combined
ca arac ex rac ion and rabeculec omy by in ernal
approach or coexis ing ca arac and open-angle glau-
coma. J Cataract Ref act Surg. 2008;34:1096–1103.
Francis BA, See F, ao NA, e al. Ab in erno rabeculec-
omy: Developmen o a novel device ( rabec ome)
and surgery or open-angle glaucoma. J Glaucoma.
2006;15:68–73.
Jea SY, Mosaed S, Vold SD, e al. E ec o ailed rabec-
ome on subsequen rabeculec omy. JGlaucoma. 2011
Feb 17 [Epub ahead o prin ]
Jea SY, Francis BA, Vakili G, e al. Ab in erno rabeculec-
omy ( rabec ome) versus rabeculec omy or open
angle glaucoma. Ophthalmology. 2012;119:36–42.
 398 26 SCHLEMM’S CANAL-BASED SURGERY

Minckler DS, Baerveld G, Al aro M , e al. Clinical Minckler DS, Baerveld G, amirez MA, e al. Clinical
resul s wi h he rabec ome or rea men o open- resul s wi h he rabec ome, a novel surgical device
angle glaucoma. Ophthalmology. 2005;112:962–967. or he rea men o open-angle glaucoma. Trans Am
Ophthalmol Soc. 2006;104:40–50.

Irrigation Port

Protective
Footplate

Aspiration Port
Return Electrode
Active Electrode

a. Handpiece
b. Power, IA Line
c. Irrigation/Aspiration Unit
d. High Frequency Generator
e. Clean Tray
f. Main Stand
g. Foot Control

FIGURE 26-5. Trabectome handpiece and setup. T e irriga ion sleeve ends 5 mm above he ip. T e oo pla e
is 800 µm heel o ip, wi h a maximum wid h o 230 µm and maximum hickness o 110 µm. T e gap be ween
he elec rocau ery pole and he oo pla e is 150 µm. (Cour esy o NeoMedix Corpora ion, us in, Cali ornia)

A B
FIGURE 26-6 Trabectome. A. Canal view ollowing rabec ome procedure exposing he rabecular meshwork
and collec ors. (Cour esy o NeoMedix Corpora ion, us in, Cali ornia.) B. In raopera ive view o rabec ome
procedure. T e ip is wi hin Schlemm’s canal and moving o he le . Behind he probe, he opalescen colored
ou er wall o Schlemm’s canal can be seen ex ending rom he righ o he probe o he edge o he mirror.

iS E
ENN
T he iS en rabecular micro-bypass sys-
em (Glaukos, Laguna Hills, CA) is also
a minimally invasive ab in erno device placed
hrough he canal. I is a heparin-coa ed, non-
erromagne ic, surgical-grade i anium s en
approxima ely 0.3 mm in heigh (Fig. 26-7)
ha is considered he smalles implan placed
in humans.
MECH ANISM OF ACTION
While small, he s en is o su cien size
o main ain a s able posi ion in he M, ye
also allow or mul iple implan s (Fig. 26-8).
When success ully placed hrough he M,
he iS en re-es ablishes ou f ow and reduces
IOP.
S udies have sugges ed ha placing more
han one s en provides grea er IOP lowering.
TECHNIQUE
T e iS en is inser ed hrough a small em-
poral clear corneal incision, generally, in he
nasal quadran .
Wi h one lumen ex ending in o he an e-
rior chamber and he o her in o he canal,
he resis ance imposed by he M is reduced
(Fig. 26-9).
iStent 399
OUTCOMES
T e iS en appears o be sa e and e ec ive.
I is o en placed a he ime o ca arac
surgery. Implan ing he iS en in conjunc-
ion wi h ca arac surgery resul ed in a mean
IOP reduc ion o 5.1 mm Hg. T e s en also
reduced he mean number o medica ions
(1.1 ewer medica ions).
A 1 year, 66% o eyes rea ed wi h com-
bined ca arac surgery and he iS en achieved
20% IOP reduc ion wi hou medica ion.
Adverse even s repor ed include s en
lumen obs ruc ion, iris ouch by device, and
malposi ioning o he s en . Hyphema was
repor ed o be ransien .
No changes were no ed in he endo he-
lium, no hypo ony, f a chambers, or choroi-
dal e usion.
BIBLIOG APH Y
Bahler CK, Smedley G , Zhou J, e al. rabecular
bypass s en s decrease in raocular pressure in cul-
ured human an erior segmen s. Am J Ophthalmol.
2004;138:988–994.
Samuelson W, Ka z LJ, Wells JM, e al. andomized
evalua ion o he rabecular micro-bypass s en wi h
phacoemulsi ca ion in pa ien s wi h glaucoma and
ca arac . Ophthalmology. 2011;118:459–467.
Spiegel D, We zel W, Neuhann , e al. Coexis en primary
open-angle glaucoma and ca arac : In erim analysis o
a rabecular micro-bypass s en and concurren ca a-
rac surgery. Eur J Ophthalmol. 2009;19:393–399.
 400 26 SCHLEMM’S CANAL-BASED SURGERY

FIGURE 26-7. iStent. Surgical-grade i anium wi h wo openings and ridged ube o provide grea er re en ion
in he canal. (Cour esy o Glaukos, Laguna Hills, Cali ornia.)

FIGURE 26-8. Postoperative iStent appearance. (Cour esy o om Samuelson.)

 iStent 401

A B
FIGURE 26-9. iStent positioning. A. Op imal posi ion o he s en . B. iS en placed hrough he M posi ioned
in he canal. No e he proximi y o he iS en in rela ion o he landmarks o he angle. (Cour esy o Glaukos,
Laguna Hills, Cali ornia.)
 C H AP ER
27
Cyclodes ruc ive Procedures
or Glaucoma
Shan Lin, Geof rey P. Schwartz, and Louis W. Schwartz
I n raocular pressure is he major risk ac-
or or glaucoma ha oph halmologis s are
able o con rol. Medically, ei her eye drops or
pills are used o decrease aqueous produc ion
or increase aqueous ou f ow o e ec ively
lower in raocular pressure. Mos surgical
and laser procedures, including rabeculec-
omy, ube shun s, gonio omy, iridec omies,
laser rabeculoplas y, and laser irido omy,
decrease he in raocular pressure by increas-
ing ou f ow. Cyclodes ruc ive procedures
are designed o des roy he ciliary processes,
hereby decreasing aqueous produc ion.
Because o he unpredic abili y o hese pro-
cedures in lowering in raocular pressure and
he complica ions associa ed wi h heir use,
such cyclodes ruc ive procedures are o en
considered a surgery o las resor . However,
recen s udies and prac ices have employed
hese procedures as a rs -line surgery and
even prior o medica ion rea men .
402
INDICATIONS
Cyclodes ruc ion o he ciliary body has
radi ionally been reserved or use in pa ien s
who have ailed previous rea men wi h
medicines or surgeries.
Excep ions o his rule include pa ien s
who are no willing o undergo l ra ion
surgery, hose who canno undergo surgery
owing o medical condi ions, and pa ien s in
underdeveloped coun ries. In underdevel-
oped coun ries, where medical care is expen-
sive and no always available, diode con ac
ransscleral cyclopho ocoagula ion (CPC),
which is por able and rela ively easy o use,
may be e ec ive and sa e as he rs line o
rea men or glaucoma, al hough he energy
delivered is ypically lower han ha used in
nonseeing eyes.
Nonpene ra ing orms o CPC may have
bene in con rolling pain in blind, pain-
ul eyes, and may allow he pa ien o avoid

removal o he eye as long as visualiza ion or
ul rasound reveals no in raocular umor.
ypes o glaucoma ha have been rea ed
wi h varying degrees o success include end-
s age open-angle glaucoma; neovascular
glaucoma; glaucoma pos pene ra ing kera-
oplas y; advanced angle closure, bo h pri-
mary and secondary; rauma ic glaucoma;
malignan glaucoma; silicone oil glaucoma;
congeni al glaucoma; pseudophakic and
aphakic open-angle glaucoma; and second-
ary open-angle glaucoma.
Al erna ive rea men s ha are usually
considered in his group o pa ien s include
l ering surgery wi h an ime aboli e or
ube shun s.
Bo h ransscleral CPC and endoscopic
cyclopho ocoagula ion (ECP) have been used
in cases o rela ively good po en ial vision.1,2
In he case o ECP, his is o en in he set ing
o combined cases wi h ca arac surgery.2
Inf amma ion and cys oid macular edema
(CME) are no in requen complica ions and
should be an icipa ed and preven ed wi h
appropria e s eroid herapy.
CONTRAINDICATIONS
T ere are rela ively ew s ric con raindica-
ions o he various orms o CPC.
Contraindications 403
A phakic pa ien wi h good vision has his-
orically been he primary con raindica ion;
however, recen s udies have used ransscleral
CPC and ECP in such cases.1,2
Marked uvei is is a rela ive con raindica-
ion because pa ien s have increased inf am-
ma ion and risk or CME ollowing he
rea men ; care should be aken o ry o quie
he eye as much as possible be ore he proce-
dure. However, uvei ic glaucoma is one o he
secondary glaucomas ha have been rea ed
success ully wi h ECP and ransscleral CPC.
For all o he nonpene ra ing orms o
CPC, he procedure is usually per ormed
in he o ce, and pa ien coopera ion is
required; inabili y o coopera e may be a con-
raindica ion in such cases.
For ECP, very poor visual po en ial (no
ligh percep ion or hand mo ions) are con ra-
indica ions since here are po en ial risks or
endoph halmi is and choroidal hemorrhage
wi h his in raocular surgery.
EFE ENCES
1. Egber P , Fiadoyor S, Budenz DL, e al. Diode laser
ransscleral cyclopho ocoagula ion as a primary surgi-
cal rea men or primary open-angle glaucoma. Arch
Ophthalmol. 2001;119(3):345–350.
2. Chen J, Cohn R , Lin SC, e al. Endoscopic pho oco-
agula ion o he ciliary body or rea men o re rac ory
glaucomas. Am J Ophthalmol. 1997;124(6):787–796.
 404 27 CYCLO DES RUC IVE PRO CEDURES FO R GLAUCO MA
ECH
E C H N IQ
Q UES
U ES
UE
S everal echniques are used or cyclode-
s ruc ion. T ey include noncon ac
ransscleral CPC, con ac ransscleral CPC,
cyclocryo herapy (CC ), ranspupillary
CPC, and ECP. All o he procedures may be
repea ed and he nonpene ra ing orms may
o en require mul iple rea men s.
NONCCOON A ACC
RANSSCLERAL
R AN SSCL
L E R AL CPC
C PC
C grea er he energy levels used, he grea er is
A neodymium (Nd):YAG laser is used
o per orm noncon ac CPC. In he pas , a
semiconduc or diode laser, Microlase (Keeler,
Inc., Broomall, PA), was also u ilized.1
e robulbar anes hesia is given.
A lid speculum is placed i a con ac lens is
no used.
A con ac lens developed by Bruce Shields
may or may no be used. T e con ac lens has
he advan ages o having markers a 1-mm
in ervals o bet er judge he dis ance rom he
limbus, blocking some o he laser ligh rom
en ering he pupil, and blanching an inf amed
conjunc iva o decrease super cial charring o
he conjunc iva2 (Fig. 27-1).
Eigh o en burns are placed 1 o 3 mm
(op imal: 1.5 mm) rom he limbus or 180
o 360 degrees, aking care o avoid he 3 and
9 o’clock meridians in order o avoid coagu-
la ing he long pos erior ciliary ar eries and
causing an erior segmen necrosis. Energy
levels o 4 o 8 J are used. T e laser beam is
ocused on he conjunc iva; however, he
laser is de ocused such ha i s e ec is ac u-
ally 3.6 mm beyond he conjunc ival sur ace,
wi h mos o he energy being absorbed by
he ciliary body (Fig. 27-2). In general, he
he inf amma ion.3–5
EFE ENCES
1. Hennis HL, S ewar WC. Semi-conduc or diode laser
ransscleral cyclopho ocoagula ion in pa ien s wi h
glaucoma. Am J Ophthalmol. 1992;113:81–85.
2. Simmons B, Blasini M, Shields MD, e al. Comparison
o ransscleral neodymium:YAG cyclopho ocoagula-
ion wi h and wi hou a con ac lens in human au opsy
eyes. Am J Ophthalmol. 1990;109:174–179.
3. Frankhauser F, Van der Zypen E, Kwasniewska S,
e al. ransscleral cyclopho ocoagula ion using a neo-
dymium YAG laser. Ophthalmic Surg Lasers. 1986;
1:125–141.
4. Schwar z LW, Mos er M . Neodymium:YAG laser
ransscleral cyclodia hermy. Ophthalmic Laser T er.
1986;1:135–141.
5. Crymes BM, Gross L. Laser placemen in noncon ac
Nd:YAG cyclopho ocoagula ion. Am J Ophthalmol.
1990;110:670–673.
 Noncontact ransscleral CPC 405

A B
FIGURE 27-1. A. Shields lens. Shields lens or noncontact transscleral CPC. B. Placement o laser burns 1.5 mm
rom the limbus. Note blanching o inf amed conjunctiva.

FIGURE 27-2. Noncontact transscleral CPC. Diagram o noncontact transscleral CPC showing that the laser
energy is actually ocused within the ciliary body.
 406 27 CYCLO DES RUC IVE PRO CEDURES FO R GLAUCO MA

2 seconds over 360 degrees, avoiding he 3

CO
O N AC
AC and 9 o’clock posi ions (Fig. 27-3).
RANSSCLERAL
R AN SSCL
L E R AL CPC
C PC
C T e energy level is i ra ed o be sligh ly
below (250 mW lower) he audible pop,
T is echnique is curren ly he mos
because audible pops are associa ed wi h
popular nonpene ra ing cyclodes ruc ive
grea er inf amma ion and hyphema.4
procedure. I uses a con ac diode laser probe
ha is rela ively small and por able (G-Probe;
I IDEX Corpora ion, Moun ain View, CA). EFE ENCES
Kryp on and Nd:YAG lasers have also been 1. Schuman JS, Pulia o CA, Allingham , e al.
Con ac ransscleral con inuous wave neodymium:
used o per orm con ac ransscleral CPC.1–3 YAG laser cyclopho ocoagula ion. Ophthalmology. 1990;
In his echnique, re robulbar anes hesia is 97:571–580.
given and a lid speculum is placed. 2. Schuman JS, Bellows A , Shingle on BJ, e al. Con ac
ransscleral Nd:YAG laser cyclopho ocoagula ion:
T e pa ien is placed in he supine Mid erm resul s. Ophthalmology. 1992;99:1089–1095.
posi ion. 3. Immonen IJ, Puska P, ait a C. ransscleral con ac
kryp on laser cyclopho ocoagula ion or rea men o
T e “heel edge” (as opposed o he ip
glaucoma. Ophthalmology. 1994;101(5):876–882.
por ion) o he probe is placed a he limbus. 4. Allingham , DeKa er AW, Bellows A , e al.
Because o he design o he G-probe, he Probe placemen and power levels in con ac rans-
energy is ac ually delivered 1.2 mm rom he scleral neodymium:YAG cyclopho ocoagula ion. Arch
limbus; approxima ely 30 applica ions o 1.5 Ophthalmol. 1990;109:738–742.
o 2.0 W o energy are applied or 1.5 o

A B

FIGURE 27-3. Contact transscleral CPC. A. G probe or diode laser treatment. B. Diagram showing the
placement o the G probe relative to the limbus so that laser application is at the ciliary body.
 Cyclocryotherapy 407

EFE ENCE
C YC
YCLO
C LO
OCCRYO
RY
YO H ERAP
ERA
AP Y 1. Biet i G. Surgical in erven ion on he ciliary body:
New rends or he relie o glaucoma. JAMA. 1950;
A ni rous oxide cryosurgical uni is used 142:889–897.
o cool he 2.5-mm probe o −80 °C, which is
placed approxima ely 1 mm pos erior o he
limbus or 60 seconds.
wo o hree quadran s are rea ed wi h
our spo s per quadran , avoiding he 3 and
9 o’clock posi ions (Fig. 27-4).1

FIGURE 27-4. CCT. Photograph demonstrating CC . T e probe is placed approximately 1 mm posterior to the
limbus.
 408 27 CYCLO DES RUC IVE PRO CEDURES FO R GLAUCO MA

Laser set ings o 50- o 100-µm spo size,

RAN
RA
AN SP
SPUPILLARY
P U PII L LA
ARY
Y CPC
C PC
C 700 o 1000 mW, or 0.1 second, wi h he
energy level being adjus ed o crea e a whi en-
A con inuous-wave argon laser is delivered
ing o he issue, are used o rea all visible
via a biomicroscope. T e concep behind his
ciliary processes (Fig. 27-5).
echnique is o apply he laser energy direc ly
o he ciliary processes ins ead o having o T e major disadvan age o his echnique
go hrough o her s ruc ures, such as he is visualiza ion problems.1 I is rarely used or
conjunc iva and sclera. his reason.
o visualize he ciliary processes, a
Goldmann- ype gonioprism and scleral EFE ENCE
depression are necessary. Wide dila ion o he 1. Shields S, S ewar WC, Shields MD. ranspupil-
lary argon laser cyclopho ocoagula ion in he rea -
pupil, iridec omy, or bo h are usually required men o glaucoma. Ophthalmic Surg Lasers. 1988;19:
or visualiza ion. 171–175.

FIGURE 27-5. Transpupillar y CPC. Diagram showing transpupillary CPC. T e laser energy is being ocused
by a mirrored lens onto the ciliary body, which has been moved into view by scleral depression.
 Endoscopic Cyclophotocoagulation 409

limbus, an an erior vi rec omy is per ormed,

EN
E ND
DOOS
SCO
C O PIC
C and he laser endoscope is inser ed. wo
C YC
YCLO
C LO P
PHHO O- incisions can be crea ed i more han 180 o
COAGULA
C OA
AG U L A IOON 270 degrees o processes are o be rea ed.
T e sclero omies are closed wi h 7–0 Vicryl
T is echnique is per ormed in he opera - su ure. T e laser endoscope has camera
ing room under local re robulbar, sub- enon’s, imaging, He–Ne aiming beam, ligh source,
or opical anes hesia. and laser delivery ransmit ed hrough an
T ere are wo di eren approaches: limbal 18-, 19-, or 20-gauge endoprobe. T e probe
and pars plana. is connec ed o a video camera, ligh source,
and video moni or. T ere is a 110-degree
In he limbal approach, he pupil is maxi-
view in s andard probes; however, a new
mally dila ed, an incision approxima ely
line o high-resolu ion endoprobes have
2.5 mm in wid h is made wi h a kera ome,
a 170-degree eld. An advan age o he
and viscoelas ic is in roduced be ween he iris
18-gauge probe is i s s urdiness and grea er
and he crys alline lens or he pseudophakic
po en ial or mul iple uses. A semiconduc-
pos erior chamber lens o access he ciliary
or diode laser a 810 nm waveleng h can
processes. A maximum o 180 degrees can
deliver energy con inuously or as imed
be rea ed hrough an incision wi h a s raigh
pulses. Applica ions are ypically rom 0.5
probe or up o 270 degrees wi h a curved
o 5 seconds, 300 o 900 mW, o achieve an
probe. A second incision can be made direc ly
end poin o whi ening and shrinkage o each
opposi e he original one o rea he remain-
ciliary process (Fig. 27-6). o avoid a visible
ing un rea ed processes. Viscoelas ic is irri-
explosion (‘pop’) o he ciliary process, laser
ga ed ou a er he procedure and he wound
power, dura ion, or bo h can be decreased.
closed wi h 10–0 nylon su ure. A ca arac
T e surgeon per orms he procedure by
ex rac ion may be combined wi h his proce-
viewing he video moni or.1
dure a he same ime, usually wi h he ECP
ollowing he ex rac ion.
EFE ENCE
When per orming he ECP hrough he
1. Chen J, Cohn R , Lin SC, e al. Endoscopic pho oco-
pars plana incision, he pa ien mus be apha- agula ion o he ciliary body or rea men o re rac ory
kic or pseudophakic. A ypical pars plana glaucomas. Am J Ophthalmol. 1997;124(6):787–796.
incision is made 3.5 o 4.0 mm rom he
 410 27 CYCLO DES RUC IVE PRO CEDURES FO R GLAUCO MA

A B
FIGURE 27-6. ECP. A. Diagram showing an endolaser probe delivering energy to the ciliary body. (Courtesy o
Martin Uram, MD.) B. View through the endoscopic camera o the whitened, shrunken ciliary processes on the
le ollowing delivery o the laser energy. T e processes on the right have not received treatment but one process
has the red He–Ne beam ocused on its tip.
 Postprocedure Care 411

TABLE 27-1. Potential Complications o

POS
P O S PR
PRO
R O CEDURE
CE
E DUR
R E CARE
CA
ARE
E Cyclodestructive Procedures*

In all o hese rea men s, opical s eroids Uveitis–iritis (hypopyon)

are used pos opera ively a varying requency Cystoid macular edema (with ECP)
depending on he inf amma ion level and risk Vitritis
or CME or uvei is. Chronic fare
Sub- enon’s s eroids are o en given o Pain
blun he inf amma ion and preven CME. Hyphema
Cycloplegic and nons eroidal an i- Vitreous hemorrhage
inf amma ory drops may also be recom-
Corneal edema
mended. Analgesics and ice packs are
some imes prescribed or pain. T inning o the sclera (with transscleral CPC)
Retinal detachment
Sympathetic ophthalmia (controversial)
COMPLICATIONS
Malignant glaucoma
Table 27-1 lis s he complica ions o Anterior segment necrosis or ischemia
cyclodes ruc ive procedures. Decreased vision
T e mos eared o hese complica ions are Blindness
chronic hypo ony leading o ph hisis, which Chronic hypotony
occurs in 5% o 10% o pa ien s rea ed by
Phthisis
ransscleral CPC, and sympa he ic oph hal-
raumatic cataract (with ECP)
mia, which is ex remely rare and may be sec-
ondary o o her prior in raocular surgeries. Endophthalmitis (with ECP)
Signi can pain occurs in abou 50% o Choroidal hemorrhage (with ECP)
pa ien s rea ed by he ransscleral rou e and *T ose that are speci c to the transscleral or endoscopic
may las or several hours o several weeks, he approach are noted.
usual dura ion being 2 o 3 days, ollowing ECP, endoscopic cyclophotocoagulation.
he procedure. One canno predic rom he
ype o glaucoma which pa ien s will have his
signi can pain. I can be rea ed wi h analge-
sics and ice. EFE ENCE
Wi h ECP, he mos common vision- 1. Chen J, Cohn R , Lin SC, e al. Endoscopic pho oco-
agula ion o he ciliary body or rea men o re rac ory
hrea ening complica ion is CME, occurring glaucomas. Am J Ophthalmol. 1997;124(6):787–796.
in 10% o cases rom a large series.1
 C H AP ER

La e Complica ions o
Glaucoma Surgery
Gabriel Chong, Francisco Fantes, and Paul F. Palmberg

I n mos cases, glaucoma-f l ering surgery is

sa e and e ec ive a lowering in raocular
pressure. However, his rea men is no always
per ec . Many o he undesired ou comes o f l-
ering surgery are caused by echnical ailures
or by an undesirable wound healing response
(Table 28 1). eproducible, me hodical,
and sa e surgical echniques combined wi h
at emp ed modula ion o he biologic response
may minimize some o hese undesired ou -
comes. Despi e our bes e or s, however,
delayed problems can occur (Table 28 2).
T e goal o his chap er is o review some o
he more common delayed complica ions
o glaucoma-f l ering surgery and discuss
possible rea men s ra egies. Some o he
rea men s ra egies are more s rongly es ab-
lished and have passed he es o ime. O her
procedures and al erna ives are newer and
may have helped one or more o he au hors o
solve some individual problems. T e newer or
somewha in requen procedures may no ye
have passed he es s o rigorous research and
ime because o he rari y o he si ua ions in
which hey have been applied.
TABLE 28-2. Undesirable Ou comes
as a Resul o a Vigorous or Inadequa e
Healing Response
Vigorous Healing Response
Loss o lter due to scarring
Inadequate ltration
Bleb encapsulation

TABLE 28-1. Fac ors ha can Inf uence Inadequate Healing Response
Wound Healing Hypotony
Choroidal e usion
Impeccable and precise surgical techniques Macular olds
Use o antimetabolites Flat chambers
Etiologyo glaucoma,such as uveitic or neovascular cause Bleb leaks
Use o postoperative anti-infammatory medications Bleb-related in ections
Other biologic actors, such as genetics, age, and race Giant blebs

412

HYPOTONY
Hypo ony can resul in maculopa hy, cho-
roidal e usion, and delayed suprachoroidal
hemorrhages.
I is o en he resul o insu cien scleral ap
resis ance ha many imes will require resu ur-
ing he ap in rabeculec omies per ormed
wi h an ime aboli es. Al erna ive herapies have
also been described;1–4 however, hese hera-
pies are probably less likely o be success ul in
cases in which an ime aboli es have been used,
or a rapid resul is needed, such as pa ien s wi h
a a chamber, maculopa hy, or he so-called
kissing choroidals. When here is overf l ra ion
wi h a necro ic-looking scleral ap, su ures may
no provide enough resis ance o ow. In hese
cases, donor issue may be needed as a roo o
he ap o achieve he desired resis ance. I is
advisable o have donor issue available when-
ever one is at emp ing o revise a scleral ap or
o repair a leaking bleb.
Chronic hypo ony, which persis s or a
leas 3 mon hs, can lead o hypo ony macu-
lopa hy and loss o vision.5 Dr. Palmberg
employs a me hod using ranscorneal su ures
o rea hypo ony a he sli lamp. T e ech-
nique involves passing 10-0 nylon su ures
hrough he cornea, hrough he scleral ap,
and hen back hrough conjunc iva a he sli
lamp wi h he objec ive o increasing resis-
ance a he scleral ap o raise in raocular
pressure (Fig. 28-1).
Hypotony Maculopathy
Hypo ony maculopa hy is a condi ion in
which olds in he choroid or re ina, or bo h,
involving he oveal region cause blurred
vision in he set ing o hypo ony. T e mecha-
nism is probably scleral con rac ion.
T e maculopa hy does no occur in all
cases o hypo ony, bu is more likely o occur
in eyes o pa ien s who are young and myopic,
Hypotony 413
and hose wi h marked reduc ions in in ra-
ocular pressure.
I is bes o rea his condi ion quickly,
because i can become permanen , al hough
here are repor s o success a er years o
involvemen 6 (Fig. 28-2A, B).
T e bes herapy is preven ion, such as
he cornea sa e y valve incision as devised by
Palmberg.7
Palmberg also described a echnique or
bleb revisions o f x maculopa hies whereby
wo se s o su ures are added. T e f rs se o
wo su ures adjus s he ou ow rom he ap
o an in raocular pressure o 8 o 12 mm Hg.
T e second se is adjus ed o an in raocular
pressure o 20 o 25 mm Hg.7 I is impor an
o keep in mind he possibili y ha donor is-
sue may be needed when revising a ap (see
Fig. 28-2C, D).
Shallow and Flat Anterior Chamber
Depending on he e iology, a chambers
can be associa ed wi h high or low in raocular
pressures (Table 28-3; Fig. 28-3). Wi h a
pos opera ive a or shallow chamber, he
clinical his ory, examina ion, and in raocular
TABLE 28-3. Causes o Al ered
In raocular Pressure and a Fla An erior
Chamber
High Intraocular Pressure and Flat Chamber
Aqueous misdirection syndrome (i.e., malignant
glaucoma)
Suprachoroidal hemorrhage
Pupillary block
Low Intraocular Pressure and Flat Chamber
Over ltration caused by insu cient fap resistance
Uveal–scleral outfow tract due to choroidal
detachment
Cyclodialysis cle
Atrue fat chamber with lens–cornea or
intraocular lens–cornea touch (should be xed
immediately)
 414 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY
pressure guide he examiner in making he
diagnosis.
T e indica ions or draining a choroidal
e usion include:
Fla chamber resul ing in lens–corneal
con ac
So-called kissing choroidals (re ina–
re ina con ac be ween he choroidal swell-
ings) o avoid f brin adherence be ween
he overlying re ina
Persis ence (a er rea ing wi h cyclople-
gics or opical s eroids)
I is appropria e o observe hese eyes or
several weeks so long as nei her o he f rs
wo condi ions is presen .
Strategies or Re ormation
o the Anterior Chamber
amponade via pressure or Simmons’
shell: T e s ra egy is likely o be more
success ul in surgeries wi hou an ime-
aboli es, and is o be used in si ua ions o
overf l ra ion.
Viscoelas ic injec ion in o he an erior
chamber: T is s ra egy is also likely o have
success in f l ers wi hou an ime aboli es.
esu uring he ap: T is may end up
being he solu ion when an ime aboli es were
used.
Draining Choroidal Ef usions
A paracen esis is placed emporally (Figs.
28-4 and 28-5A).
Conjunc ival incisions are made a 4:30
and 7:30 meridians rom 2 o 7 mm rom he
limbus, or a limbal peri omy rom he 4 o
8 oclock posi ions.
A hal - hickness radial incision o 2 mm
is made, beginning 3 mm rom he limbus as
measured by calipers.
T e edge o he ap is grasped by a
oo hed orceps or coun er rac ion.
Wi h a sharp blade, he incision is slowly
and care ully deepened un il he suprachoroi-
dal space is opened (Fig. 28-5B).
T e incision is enlarged wi h a Kelly punch
(Fig. 28-5C).
I he incision is over a pocke o uid, i
will begin he ou ow, which can be helped
by in using balanced sal solu ion (BSS)
hrough he paracen esis, li ing he edges
o he ap, and rolling a cot on- ipped swap
along he scleral sur ace.
I he incision is no over a pocke o uid
and uid is no mobilized o he incision, a
cyclodialysis spa ula can be used o separa e
he choroids gen ly rom he scleral wall o
ob ain communica ion o an adjacen pocke
o uid. T is dissec ion should be done
ex remely care ully, and no more han a ew
millime ers rom he incision.
Indirec oph halmoscopy could be used a
his ime o look a he at ened re ina. T e
an erior chamber should be deep, as well.
T e conjunc ival incisions should be
closed, leaving he punched incisions open
(Fig. 28-5D).
Delayed Suprachoroidal Hemorrhages
A suprachoroidal hemorrhage is a condi-
ion in which bleeding occurs in he supra-
choroidal space separa ing he uvea rom he
sclera. T ese hemorrhages can occur in raop-
era ively as well as in he pos opera ive period.
I bleeding occurs in raopera ively, he
pos erior pressure can cause ex rusion
o he con en s o he eye (e.g., expulsive
hemorrhage). T e delayed suprachoroidal
hemorrhages can be he resul o de novo
bleeding in o he suprachoroidal space or
o bleeding in o a preexis ing choroidal
e usion.
T e risk ac ors include marked decom-
pressions o he in raocular pressure, mul iple

previous eye surgeries, myopia, previous vi -
rec omy, and sys emic hyper ension.
A suprachoroidal hemorrhage o en pres-
en s as sudden, severe pain associa ed wi h
a brown-colored choroidal eleva ion in one
side o he vi reous cavi y. I is advisable o
manage he pa ien wi h vi reore inal surgery
i possible.
Serial B-scan ul rasounds are help ul o
show he loca ion o he hemorrhage and
moni or he clo or lysis; his usually occurs 5
o 10 days a er he onse o he hemorrhage.
Many surgeons pre er wai ing un il he clo
liquef es (lyses) be ore draining he hemor-
rhage. T e echnique is he same as described
earlier or drainage o a choroidal e usion.
Smaller suprachoroidal hemorrhages may
reabsorb spon aneously in abou 1 mon h
wi h good visual resul s. While he clo is
lique ying, he in raocular pressure should
be con rolled medically o he bes degree
possible.
An ex remely eleva ed in raocular pressure
could orce an earlier in erven ion.
A
FIGURE 28-1. Transcorneal sutures. A. Diagram o he echnique. (From Eha J, Ho man E, P ei er N. Graefes
Arch Clin Exp Ophthalmol. 2008;246:869–874.) B. Pho o mon age o he e ec o ranscorneal su ures over ime.
Hypotony 415
EFE ENCES
1. Akova YA, Dursun D, Aydin P, e al. Managemen o
hypo ony maculopa hy and a large f l ering bleb a er
rabeculec omy wi h mi omycin C: Success wi h
argon laser herapy. Ophthalmic Surg Lasers. 2000;
31(6):491–494.
2. Marze a M, oczolowski J. Adminis ra ion o au olo-
gous blood o a pa ien via in rableb injec ion as a
me hod or rea ing hypo ony a er rabeculec omy.
Klin Oczna. 2000;102(3):199–200.
3. Okada K, sukamo o H, Masumo o M, e al.
Au ologous blood injec ion or marked overf l ra ion
early a er rabeculec omy wi h mi omycin C. Acta
Ophthalmol Scand. 2001;79(3):305–308.
4. Yieh FS, Lu DW, Wang HL, e al. T e use o au ologous
f brinogen concen ra e in rea ing ocular hypo ony
a er glaucoma f l ra ion surgery. J Ocul Pharmacol T er.
2001;17(5):443–448.
5. Azuara-Blanco A, Ka z LJ. Dys unc ional f l ering
blebs. Surv Ophthalmol. 1998;43(2):93–126.
6. Delgado MF, Daniels S, Pascal S, e al. Hypo ony mac-
ulopa hy: Improvemen o visual acui y a er 7 years.
Am J Ophthalmol. 2001;132(6):931–933.
7. Palmberg P. Surgery or complica ions. In: Alber D,
ed. Ophthalmic Surgery: Principles and echniques. v. 1.
London: Blackwell Science; 1999.
B
 416 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY

A B

C D
FIGURE 28-2. Hypotony. A. Drama ic example o hypo ony causing olds in he choroid and re ina involving
he oveal region. B. Example o race cys ic changes and sub le olds in he in ernal limi ing membrane in a
pa ien wi h hypo ony maculopa hy as shown in op ical coherence omography (OC ) . C. In raopera ive video
s ill- rame showing a cu piece o donor cornea being used o cover a f ap. D. In raopera ive video s ill- rame
showing a compression su ure o 10-0 nylon being used over he piece o donor cornea o increase he scleral
resis ance o aqueous ou f ow.
 Hypotony 417

FIGURE 28-3. Shallow an erior chamber sli -beam pho ograph showing a shallow an erior chamber. T ere is
signi can iridocorneal ouch; however, nei her he pupillary border nor in raocular lens is in con ac wi h he
cornea.

FIGURE 28-4. Peripheral choroidal ef usions. Fundus pho ograph showing peripheral choroidal e usions
( le ) .
 418 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY

A B

C D
FIGURE 28-5. Repair o choroidal ef usion. In raopera ive video s ill pho ographs o a repair o choroidal e usion.
A. Paracen esis side-por using a sharp poin number 75 blade is made a he corneoscleral limbus. B. A sharp
blade is used o gen ly en er he suprachoroidal space a he base o he par ial- hickness radial scleral incision.
C. Once he suprachoroidal space is en ered, he incision is widened using a Kelly punch; a he base o he
incision, a hole crea ed by he punch can be seen. D. T e conjunc ival incision is closed using 7-0 Vicryl su ures;
he sclero omy is le open.

BLEB LEAKS
A bleb leak is a iny hole in he wall o he
bleb causing leakage o aqueous. T is is a
direc communica ion be ween he ex erior
world and he in erior o he bleb. Use o
in raopera ive an ime aboli es is a risk ac or
or he developmen o a bleb leak.
T e mechanism o a bleb leak is hough
o be as ollows. Ischemic blebs are s re ched
and surrounded by heavily scarred issue,
which limi s he abili y o he aqueous o ow
beyond he scarred issue. T e bleb expands
locally, producing a rac ional hole when he
issue overreaches i s maximal s re ch.
T e bleb leaks are bes de ec ed by apply-
ing uorescein o he sur ace o he bleb and
viewing i under a sli lamp wi h a cobal blue
f l er in place. A posi ive Seidel es consis s o
change in color o he dye o green-yellow, in
response o he ou ow o aqueous rom he
leak. Some imes a leak can only be de ec ed
a er applying gen le pressure o he globe.
Leaks increase he risk o in ec ion and
endoph halmi is; here ore, early de ec ion and
managemen could be cri ical.1–4 T e repor ed
incidence o la e pos opera ive bleb-rela ed
in ec ions a er rabeculec omy ranges rom
0.4% o 9.6% a 2 o 6 years.5,6 I is also es i-
ma ed ha blebi is occurs wi h an incidence
o 5.7% per year while he incidence o endo-
ph halmi is ranges rom 0.8% o 1.3% per year.5
Care ul surgical echniques during surgery
are cri ical in decreasing he risk o bleb leaks.
Special at en ion has o be paid o echnique
in he rabeculec omy, in su uring he con-
junc iva, in he ime, area, and washou o
he an ime aboli es, and o being me hodical
when applying laser su ure lysis.7
Treatment
Conservative Management
Following are some o he echniques
described o manage wound healing. T ese
Bleb Leaks 419
echniques have he advan age o sparing
he pa ien rom surgery. T e disadvan age is
ha hey are no always success ul and leaks
can recur. Al hough hese rea men s are no
opera ive procedures, each has i s own se o
risks.
Use o 28-mm so con ac lenses or
2 weeks7
Use o bu yryl me hacryla e glue and a
silicon disk7
In usion o au ologous blood in o he
bleb8
Applica ion o compression su ures7
Pressure eye pa ching, oral carbonic
anhydrase inhibi or, and opical au ologous
serum5
Chemical irri an s ( richloroace ic acid,
sodium ni ra e) 9
Cryopexy and dia hermy10
Argon laser, hermal Nd:YAG laser11,12
Surgical Treatment
Op ions
Closing he rabeculec omy wi h a
scleral or corneal pa ch gra and replacing
wi h a glaucoma drainage device (GDD)—
in severe cases, corneal lamellar gra ing
has been described as a “f nal” solu ion o
a dys unc ional bleb whereby he scarred
conjunc iva and weakened spongy sclera
are dissec ed away and replaced wi h round
lamellar preserved donor corneal issue.13
Conjunc ival advancemen — his has
been demons ra ed o be highly success ul.
Pa ien s wi h la e bleb leaks managed wi h
conjunc ival advancemen were more likely
o have success ul ou comes and less likely
o have serious in raocular in ec ions han
hose managed more conserva ively.14–18
Free conjunc ival gra 19— ree con-
junc ival au ologous gra is a sa e and suc-
cess ul procedure or bleb repair and bleb
 420 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY
reduc ion. However, pa ien s should be
aware o he possibili y o requiring pos -
opera ive medical or surgical in erven ion
or in raocular pressure con rol a er he
revision.
Amnio ic membrane (AM) 14—in cases
in which he conjunc ival issue available
is considered by he surgeon o be very
limi ed (e.g., as a resul o hinning or scar-
ring), or here is already some degree o
p osis presen , an AM gra could be an
al erna ive. T e echnique described nex is
sligh ly di eren rom he one described by
Budenz e al.14 In his echnique, he gra
is olded upon i sel , leaving he basemen
layer ou ward, and he s romal layer on he
inside (Fig. 28-6).
T e echnique o su uring AM is as
ollows:
T e conjunc iva surrounding he isch-
emic bleb is reed (Fig. 28-7A, B).
T e old ischemic bleb is excised
(Fig. 28-7C).
T e donor AM is removed and olded
upon i sel (see Fig. 28-6).
T e an erior edges o he gra are
su ured a he corners o corneal limbus
using 9-0 nylon.
T e pos erior edge o he AM under-
nea h he ree undermined an erior con-
junc iva (Fig. 28-7D).
T e gra is igh ly su ured o he an e-
rior edge o he pa ien ’s ree conjunc iva
using a running 8-0 Vicryl su ure
(Fig. 28-7E).
A 9-0 nylon compression su ure is
placed a he an erior edge o he gra , a
he level o he limbus (Fig. 28-7F).
T e si e is checked or wound leaks wi h
uorescein s rips.
T e an erior compression su ure can be
removed a er 1 mon h (Fig. 28-8).
A varia ion o his echnique could be
applied o ree conjunc ival gra s as well,
adding he s eps o cut ing he issue rom he
selec ed si e, and wi hou olding he ree gra .
Results and Prognosis
T e s udy o AM ransplan a ion by
Budenz e al.14 does no o er an e ec ive
al erna ive o conjunc ival advancemen or
repair o leaking glaucoma-f l ering blebs. T e
cumula ive survival ra e or AM ransplan
was 81% a 6 mon hs, 74% a 1 year, and 46%
a 2 years. T e cumula ive survival ra e was
100% or conjunc ival advancemen hrough-
ou ollow-up.
Al hough Budenz e al.’s s udy showed ha
AM gra s were less success ul han resul s
o he s andard conjunc ival advancemen ,
heir s udy showed ha hey could be suc-
cess ul in cer ain si ua ions, providing an
al erna ive rea men or bleb leaks in special
circums ances.
T e long- erm resul s o he s udy o
Budenz e al. were published in 2007 wi h a
median ollow-up o 80 mon hs.20 Almos hal
o 15 pa ien s who received AM ransplan a-
ion developed ailures, our required reop-
era ion or bleb leakage, and hree required
reopera ion or uncon rolled in raocular
pressure.20 Four o 15 pa ien s wi h conjunc-
ival advancemen experienced ailure wi h
one requiring ano her opera ion or a bleb
leak and hree pa ien s required glaucoma
in erven ion.20 Al hough no s a is ically sig-
nif can , he Kaplan–Meier long- erm survival
curves rended oward earlier ailure wi h he
AM ransplan group.20
O her groups have had more success
wi h AMs including a group in Japan (Nagai-
Kusuhara e al.) which repor ed six pa ien s
who underwen AM ransplan a ion-assis ed
bleb revision or leaking blebs and all six
pa ien s had heir leaks resolved wi hou
complica ions.21

However, ano her group also in Japan
concluded ha he use o AM ransplan
did no improve he overall surgical ou -
come or heir pa ien s.22 T eir Kaplan–
Meier survival curves showed a success
ra e o 58.3% a 6 mon hs and 21.9% a er
1 year or he AM ransplan group com-
pared o 74.8% success in heir con rol
group rom 6 o 12 mon hs.22
In addi ion, i an AM gra ails, conjunc-
ival advancemen is s ill a possibili y. I may
even be possible o make modif ca ions in he
surgical echnique ha could al er he ou -
comes. T is las poin is only specula ive; i
will need o be proven by a randomized clini-
cal rial comparable o he Budenz e al. rial
and, o course, by he f nal es o ime.
EFE ENCES
1. Jampel HD, Quigley HA, Kerrigan-Baumrind LA,
e al. isk ac ors or la e-onse in ec ion ollow-
ing glaucoma f l ra ion surgery. Arch Ophthalmol.
2001;119(7):1001–1008.
2. Lehmann OJ, Bunce C, Ma heson MM, e al.
isk ac ors or developmen o pos - rabeculec-
omy endoph halmi is. Br J Ophthalmol. 2000;
84(12):1349–1353.
3. Liebmann JM, i ch . Bleb rela ed ocular in ec-
ion: A ea ure o he HELP syndrome. Hypo ony,
endoph halmi is, leak, pain. Br J Ophthalmol. 2000;
84(12):1338–1339.
4. Sol au JB, o hman F, Budenz DL, e al. isk
ac ors or glaucoma f l ering bleb in ec ions. Arch
Ophthalmol. 2000;118(3):338–342.
5. Sharan S, rope GE, Chipman M, e al. La e-onse
bleb in ec ions: Prevalence and risk ac ors. Can J
Ophthalmol. 2009;44(3):279–283.
6. Song A, Scot IU, Flynn HW Jr, e al. Delayed-onse
bleb-associa ed endoph halmi is: Clinical ea ures
and visual acui y ou comes. Ophthalmology. 2002;
109(5):985–991.
7. Palmberg P. Surgery or complica ions. In: Alber
D, ed. Ophthalmic Surgery: Principles and echniques.
London: Blackwell Science; 1999:v. 1.
8. Okada K, sukamo o H, Masumo o M, e al.
Au ologous blood injec ion or marked overf l ra-
ion early a er rabeculec omy wi h mi omycin C.
Acta Ophthalmol Scand. 2001;79(3):305–308.
Bleb Leaks 421
9. Gehring J , Ciccarelli EC. richlorace ic acid rea -
men o f l ering blebs ollowing ca arac ex rac ion.
Am J Ophthalmol. 1972;74(4):622–624.
10. Douvas NG. Cys oid bleb cryo herapy. Am J
Ophthalmol. 1972;74(1):69–71.
11. Bet in P, Carassa G, Fiori M, e al. rea men o
hyperf l ering blebs wi h Nd:YAG laser-induced
subconjunc ival bleeding. J Glaucoma 1999;8(6):
380–383.
12. Fink AJ, Boys-Smi h JW, Brear . Managemen
o large f l ering blebs wi h he argon laser. Am J
Ophthalmol. 1986;101(6):695–699.
13. Fukuchi , Ma suda H, Ueda J, e al. Corneal lamel-
lar gra ing o repair la e complica ions o mi omy-
cin C rabeculec omy. Clin Ophthalmol. 2010;4:
197–202.
14. Budenz DL, Bar on K, seng SC. Amnio ic mem-
brane ransplan a ion or repair o leaking glaucoma
f l ering blebs. Am J Ophthalmol. 2000;130(5):
580–588.
15. Budenz DL, Chen PP, Weaver YK. Conjunc ival
advancemen or la e-onse f l ering bleb leaks:
indica ions and ou comes. Arch Ophthalmol. 1999;
117(8):1014–1019.
16. Burns ein AL, WuDunn D, Knot s SL, e al.
Conjunc ival advancemen versus nonincisional
rea men or la e-onse glaucoma f l ering bleb
leaks. Ophthalmology. 2002;109(1):71–75.
17. O’Connor DJ, ressler CS, Caprioli J. A surgical
me hod o repair leaking f l ering blebs. Ophthalmic
Surg. 1992;23(5):336–338.
18. Wadhwani R , Bellows A , Hu chinson B . Sur-
gical repair o leaking f l ering blebs. Ophthalmology.
2000;107(9):1681–1687.
19. Schnyder CC, Shaarawy , avine E, e al. Free
conjunc ival au ologous gra or bleb repair and
bleb reduc ion a er rabeculec omy and nonpen-
e ra ing f l ering surgery. J Glaucoma. 2002;11(1):
10–16.
20. auscher FM, Bar on K, Budenz DL, e al. Long-
erm ou comes o amnio ic membrane ransplan a-
ion or repair o leaking glaucoma f l ering blebs.
Am J Ophthalmol. 2007;143(6):1052–1054.
21. Nagai-Kusuhara A, Nakamura M, Fujioka M, e al.
Long- erm resul s o amnio ic membrane rans-
plan a ion-assis ed bleb revision or leaking blebs.
Grae es Arch Clin Exp Ophthalmol. 2008;246(4):
567–571.
22. Kiuchi Y, Yanagi M, Nakamura . E cacy o amni-
o ic membrane-assis ed bleb revision or eleva ed
in raocular pressure a er f l ering surgery. Clin
Ophthalmol. 2010;4:839–843.
 422 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY

FIGURE 28-6. A single layer o amnio ic membrane being peeled rom he suppor ing membrane. T e s romal
layer is agains he paper and s icky. T e basemen membrane layer is shiny and non-s icky.

A B

C D
FIGURE 28-7. Amniotic membrane gra technique. A. T e conjunc ival issue surrounding he ischemic bleb
has been cu along he margins o he bleb; a superiorly placed 7-0 Vicryl corneal rac ion su ure is also seen.
B. T e conjunc ival- enon’s f ap has been blun ly undermined o mobilize he issue. C. T e ischemic bleb is
excised using a number 67 blade. D. T e pos erior layer o he amnio ic membrane sandwich is pushed and now
lying undernea h he conjunc ival- enon’s f ap.
(continued)
 Bleb Leaks 423

E F
FIGURE 28-7. ( Continued) Amniotic membrane gra technique. E. T e conjunc ival- enon’s f ap and
amnio ic membrane sandwich are su ured oge her using a running 8-0 Vicryl su ure. F. A he corneal edge o
he gra , a 9-0 nylon compression su ure is used o obs ruc f ow rom undernea h he amnio ic membrane gra
a he limbus.

FIGURE 28-8. Appearance a er bleb revision. Pos opera ive appearance o he same eye shown in
Figure 28-7 ollowing bleb revision using a double-layer amnio ic membrane gra .
 424 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY
GIANT BLEBS
G ian blebs can grow over he cornea,
crea ing dellen and producing irregular
as igma ism and loss o bes -correc ed visual
acui y. T e managemen o a gian bleb should
be in a s epwise ashion, moving rom he sim-
ples o more complex solu ions.
Treatment
Op ions
Cleavage and pushing echnique—a
cleavage plane o he hanging bleb is ound
using a dull spa ula; his is hen pushed
back pos erior o he limbus.
Same echnique wi h compression
s i ch— he same echnique is ollowed,
placing a compressive s i ch as he limbus
ha will encourage permanen con rac ion.
Ampu a ion o he corneal por ion in
spongy-looking blebs— his approach is
use ul or spongy-looking blebs over he
cornea. T e exuberan por ion is cu wi h
Vannas scissors.
Ampu a ion o he whole bleb— his is
generally unnecessary.
T ere are always excep ions. T e ollow-
ing clinical s udy describes an excep ional
case. T e pa ien was a 55-year-old A rican-
American man, who had only one unc ion-
ing eye in which mul iple surgeries had been
per ormed, including he la es , a success ul
mi omycin C rabeculec omy or advanced
glaucoma. T e o her eye had been los o
glaucoma.
T e pa ien developed corneal edema and
underwen cornea ransplan when his visual
acui y decreased rom 20/ 30 o 20/ 200 in
he unc ional eye. We per ormed a corneal
ransplan , and his visual acui y improved o
a baseline o 20/ 30 a er 6 mon hs.
T e rabeculec omy also remained unc-
ional and kep he in raocular pressure
con rolled hroughou he pos opera ive
course. A er 1 year, he pa ien began o
develop a larger bleb ha invaded he cor-
nea, signif can ly reducing his visual acui y
(Fig. 28-9A).
T e pa ien was managed as previously
described, bu he bleb always re urned,
growing larger. Even ually his visual acui y
worsened o 20/ 400, resul ing in an eye
ha was barely unc ional. In response o
he pa ien ’s rus ra ion, and a er a long
discussion wi h him abou he risks o sur-
gery, we decided o ake he unusual s ep o
revising he whole bleb.
In his case, he pa ien had ano her
problem—a lack o ree, unscarred con-
junc iva surrounding he bleb, or in ha
eye, or ha mat er. As a resul , we decided
o excise he bleb and o rebuild i wi h a
double layer o AM donor gra . A small
bleb wi h minimal vasculariza ion ormed,
and his has main ained he in raocular
pressure under good con rol or over
4 years (Fig. 28-9B).
Bleb-related Infections and
Use of Corneal Patches
Bleb leaks and in erior blebs are risk
ac ors or in ec ions. T e in ec ions can
be localized o he bleb (Fig. 28-10), produce
necrosis o he surrounding issue
(Fig. 28-11), or progress o ull-blown
endoph halmi is. In erior loca ion o he
bleb should be considered a high risk ac or
or in ec ions (around 1% in superior blebs
versus around 8% in in erior blebs in several
s udies1–3), so considera ion should be made
o heir closure, especially when here has
been a his ory o in ec ion or leaks.
In he case o bleb-rela ed endoph ha-
lmi is, Streptococcus species and Staphy-
lococcus species are he mos common
causa ive organisms.4 Al hough resul s rom
he Endoph halmi is Vi rec omy S udy

(pos ca arac endoph halmi is was s udied)
have been applied by clinicians as a paradigm
o rea bleb-rela ed endoph halmi is, here
is growing evidence ha hose resul s can-
no be direc ly applied due o di erences
in he virulence o he pa hogens involved
be ween pos ca arac endoph halmi is and
bleb-rela ed endoph halmi is, in par icular
he Streptococcus species.
egardless o promp rea men ( ap and
injec versus vi rec omy), visual prognosis
is poor wi h f nal visual acui ies >20/ 400 in
only 22% o 53% o pa ien s.4 Several s ud-
ies also repor con ic ing resul s regarding
rea men modali y wi h Song e al. repor ing
worse visual ou comes wi h vi rec omy rea -
men and Busbee e al. repor ing he oppo-
si e.4,5 Smiddy e al. repor ed 34 cases rom
Bascom Palmer wi h nei her ap-injec ion
nor ap-injec ion wi h vi rec omy proving
superior in he managemen o bleb-rela ed
endoph halmi is.6
Corneal Patching
Corneal issue ha is no o ransplan
quali y can be preserved in glycerin and used
or pa ching, as ollows:
T e donor cornea is cu o he needed
size.
Desceme ’s membrane is peeled o
wi h wo large oo hed orceps.
T e bed is cleaned o necro ic issue.
T e cornea pa ch is su ured wi h 9-0
nylon su ures and compressive su ures, as
needed.
T e pa ch is covered by conjunc iva. I
lit le conjunc iva is available, he surgeon
may consider covering i wi h AM, wi h
he s romal layer inside, in direc con ac
wi h pa ch.
o close he f l ra ion permanen ly, he
surgeon can consider placing more han
one igh compression s i ch, and leaving
Giant Blebs 425
he s i ches in place un il hey become
loose, or or 6 o 8 weeks i hey are reason-
ably well olera ed.
In one case, a pa ien developed necro iz-
ing blebi is (see Fig. 28-10), which required
use o a corneal pa ch gra , covered by an AM
gra . A he same ime, a GDD was placed
superiorly (Fig. 28-12).
Bleb Dysesthesia
On occasion, blebs can be associa ed wi h
a cer ain degree o discom or . T e e iology
o he pain is at ribu ed o he heigh and
shape o he bleb, which dis urbs he spread
o he ear f lm, producing dellen.7,8 T is con-
di ion has been associa ed wi h he presence
o bubbles a he sli -lamp examina ion, by
he cap ure o air bubbles wi hin he ears as
he upper eyelid moves over he irregular bleb
(Fig. 28-13).
Eyes wi h glaucoma-f l ering blebs expe-
rience more dyses hesia han eyes wi hou
f l ering blebs. Budenz e al. iden if ed young
age, supranasal bleb loca ion, poor lid cover-
age, and bubble orma ion as being associa ed
wi h glaucoma-f l ering–bleb discom or .7
Some blebs ha produce dyses hesia have
been described as ischemic, hin-walled,
and associa ed wi h low-normal pressures.
Palmberg described a echnique in which, by
using compressing s i ches or 3 weeks over
he bleb, here is a change in he o ending
prof le o he bleb, hereby reducing discom-
or or up o 83% o pa ien s es ed wi h his
echnique.9 T e echnique is as ollows:
I he bleb is very hin, and he su ures
could rauma ize he sur ace, he surgeon
may consider aspira ing a small amoun o
aqueous wi h a 30-gauge needle rom he
an erior chamber o decompress he bleb
(Fig. 28-14A).
One or more 9-0 nylon mat ress su ures
are anchored in he cornea.
 426 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY

Su ures are passed pos eriorly over he
por ion o he bleb o be compressed, and
passed again over he bleb o ie he kno
(Fig. 28-14B).
T e kno is ied igh ly and ro a ed in o
he cornea, making sure ha he area ar-
ge ed is well compressed.
Su ures are le in place rom 1 o
4 weeks, and hen removed (Fig. 28-14C).
EFE ENCES
1. Greenf eld DS, Suner IJ, Miller MP, e al.
Endoph halmi is a er f l ering surgery wi h mi omy-
cin. Arch Ophthalmol. 1996;114(8):943–949.
2. Higginbo ham EJ, S evens K, Musch DC, e al. Bleb-
rela ed endoph halmi is a er rabeculec omy wi h
mi omycin C. Ophthalmology. 1996;103(4):650–656.
3. Wolner B, Liebmann JM, Sassani JW, e al. La e
bleb-rela ed endoph halmi is a er rabeculec omy
wi h adjunc ive 5- uorouracil. Ophthalmology. 1991;
98(7):1053–1060.
4. Song A, Scot IU, Flynn HW Jr, e al. Delayed-onse
bleb-associa ed endoph halmi is: Clinical ea ures
and visual acui y ou comes. Ophthalmology. 2002;
109(5):985–991.
5. Busbee BG, ecchia FM, Kaiser , e al. Bleb-associa ed
endoph halmi is: Clinical charac eris ics and visual
ou comes. Ophthalmology. 2004;111(8):1495–1503;
discussion 503.
6. Ba’arah B , Smiddy WE. Bleb-rela ed endoph halmi-
is: Clinical presen a ion, isola es, rea men and visual
ou come o cul ure-proven cases. Middle East Af J
Ophthalmol. 2009;16(1):20–24.
7. Suner IV, Greenf eld DS, Miller MP, Nicolela M ,
Palmberg PF. Hypo ony maculopa hy ollowing f l er-
ing surgery wi h mi omycin C: Incidence and rea -
men . Ophthalmology. 1977;104:207–214.
8. Soong HK, Quigley HA. Dellen associa ed wi h f l er-
ing blebs. Arch Ophthalmol. 1983;101(3):385–387.
9. Palmberg P. Surgery or complica ions. In: Alber
D, ed. Ophthalmic Surgery: Principles and echniques.
London: Blackwell Science; 1999:v. 1.

A B
FIGURE 28-9. Giant bleb. A. An ischemic, gian cys ic bleb can be seen overhanging on o he cornea. A dell
can be seen a he an erior edge o he overhanging bleb. B. Pos opera ive appearance o he eye ollowing bleb
revision.
 Giant Blebs 427

FIGURE 28-10. Bleb related in ection. An in eriorly loca ed bleb wi h blebi is. T e overlying conjunc ival
issue is clear, showing hazy bleb f uid benea h.

FIGURE 28-11. Bleb related in ection. An in eriorly loca ed bleb, which is ischemic, and a necro ic bleb wi h
opaque conjunc ival issue overlying he bleb.
 428 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY

A B

FIGURE 28-12. Corneal patch. A. Pos opera ive

appearance a 6 weeks; he clear corneal issue
gives he illusion ha a bleb is presen . B. Sli -beam
C pho ograph shows ha here is no f uid benea h he
conjunc iva. C. Pos opera ive appearance a 1 year.

FIGURE 28-13. Bleb dysesthesia. An air bubble can be seen ex ending rom he bleb and he upper lid.
 Giant Blebs 429

A B

FIGURE 28-14. Dysesthetic bleb. A. Preopera ive

appearance o a large dyses he ic bleb. B. wo 9-0
nylon mat ress su ures (or compression su ures) can
be seen delimi ing he size and heigh o he bleb.
C. Pos opera ive appearance o he eye ollowing
C removal o he compression su ures; he bleb is
smaller.
 430 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY
THE FAILING BLEB:
ENCAPSULATION
T here are many reasons why rabeculec-
omies ail. T e f l er may s op unc ion-
ing or ex ernal causes, such as encapsula ion
(Fig. 28-15) or scarring. I may ail because o
in ernal causes, as when he os ium becomes
occluded as a resul o di eren e iologies,
such as membrane orma ion, iris clo , or iris
vi reous.
Treatment and Results
Medical managemen consis s simply o
an iglaucoma medica ions, opical s eroids,
and digi al compression. Mandal, in a re ro-
spec ive s udy o 503 pa ien s, no ed ha
18 pa ien s developed encapsula ion and
15 o hose pa ien s responded well o
conserva ive rea men alone. T ree who
did no respond underwen excisional bleb
revision wi h mi omycin.1 Ophir’s f ndings
emphasize in amma ion as he e iology o
encapsula ion.2
Meyer e al. showed ha bleb needling was
e ec ive in reducing in raocular pressure in
one- hird o he cases or more han
6 mon hs. T ey also showed ha reneedlings
are as success ul as he f rs one.3
Dr. Palmberg has devised a ranscorneal ap
revision echnique. A 30-gauge needle is ben
in a Z conf gura ion (Fig. 28-16A) and used o
en er he an erior chamber hrough clear cor-
nea (Fig. 28-16B). T e needle is hen direc ed
o he underside o he scleral ap rom he
an erior chamber aspec and swep rom side
o side o break any adhesions and improve
ow (Fig. 28-16C, D). T e advan age o he
corneal approach is avoiding any rauma o he
conjunc iva and hus avoiding leaks.
Excisional bleb revision may possibly be
augmen ed wi h an ime aboli es. T is could
be a las al erna ive in cases where medical
managemen and needling prove unsuccess ul.
EFE ENCES
1. Mandal AK. esul s o medical managemen and
mi omycin C-augmen ed excisional bleb revision or
encapsula ed f l ering blebs. Ophthalmic Surg Lasers.
1999;30(4):276–284.
2. Ophir A. Encapsula ed f l ering bleb. A selec ive
review–new deduc ions. Eye (Lond) 1992;6(P 4):
348–352.
3. Meyer JH, Guhlmann M, Funk J. How success ul is
he f l ering bleb “needling”? Klin Monbl Augenheilkd.
1997;210(4):192–196.
 T e Failing Bleb: Encapsulation 431

FIGURE 28-15. Encapsulated bleb. An encapsula ed bleb; he ense appearance and hickened appearance o
he wall o he “cys ” can be seen. Also no e he increased vasculariza ion o he overlying conjunc iva.

A B

C D
FIGURE 28-16. Transcorneal ap revision. A. A 30-gauge needle is ben in o a “Z” con gura ion. B. T e
needle is hen guided hrough clear cornea in o he an erior chamber. C. T e needle ip is seen pro ruding ou
undernea h he sclera f ap, visible under clear conjunc iva. D. T ere is a nice eleva ion o he bleb a er he needle
has been used o sever any brous adhesions, reeing he f ap.
 432 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY
CIRCUMFERENTIAL BLEB
T he use o an ime aboli es leads o he
po en ial developmen o hin-walled,
avascular blebs. Aqueous can spread more
readily in hin-walled blebs, leading o large,
exuberan circum eren ial blebs which can be
irri a ing o pa ien s (Fig. 28-17A). Pa ien s
wi h circum eren ial blebs may complain o
oreign body sensa ion, pho ophobia, and
earing.1
Treatment
ahman and T aller described a echnique
ermed bleb-limi ing conjunc ivoplas y or
he rea men o circum eren ial blebs.2
Brie y, a 75 blade is used o incise con-
junc iva and enon’s capsule down o sclera
in a circum eren ial bleb, bisec ing he bleb.
T e wo cu edges o enon’s capsule
are hen su ured back down on o bare
sclera.
Once he circum eren ial bleb has been
spli in o wo blebs, he lower bleb ha is no
longer connec ed o he aqueous f l ering
rom he ap is reabsorbed, leaving behind
a much smaller f l ering bleb.
Bovie handheld cau ery has also been
described as a echnique o rea circum er-
en ial blebs in he o ce set ing.3
Cau ery is applied o he bleb a he sli
lamp a er local anes hesia (Fig. 28-17B).
Excess uid rom he bleb is drained
hrough he cau ery punc ure wounds,
which hen sel -seal rom he hea
(Fig. 28-17C).3 T e hea seals he edge
o he bleb a he loca ion specif ed by he
clinician. T e res o he bleb ha is now
sealed o rom he aqueous is reabsorbed
and a smaller f l ering bleb is he f nal resul .
EFE ENCES
1. Anis S, i ch , Shihadeh W, e al. Surgical reduc ion
o symp oma ic, circum eren ial, f l ering blebs. Arch
Ophthalmol. 2006;124(6):890–894.
2. ahman , T aller V . Bleb-limi ing conjunc ivo-
plas y or symp oma ic circum eren ial rabeculec-
omy blebs. J Glaucoma. 2003;12(3):272–274.
3. Schwar z AL, Albano M. Applica ion o handheld cau-
ery or reduc ion o symp oma ic circum eren ial ra-
beculec omy blebs. J Glaucoma. 2010;19(7):497–498.
 Circum erential Bleb 433

A B

FIGURE 28-17. Circum erential bleb. A. Sli -lamp

image o a circum eren ial bleb. B. Cau ery is applied
o he bleb o allow drainage o f uid. C. A cot on ip
C applica or is used o express f uid rom he bleb. T e
hea rom he cau ery resul s in a sel -sealing wound.
 434 28 LA E CO MPLICA IONS O F GLAUCO MA SURGERY
TUBE SHUNT EROSIONS
G DDs have proved o be use ul or low-
ering in raocular pressure and rea ing
glaucoma. Some o hese devices are valveless
and need a liga ure o avoid hypo ony un il a
capsule has ormed around he pla e o provide
resis ance (Fig. 28-18). T ese devices, how-
ever, are no wi hou heir own unique com-
plica ions. One such complica ion, al hough
uncommon (30% in he pas bu now <5%), is
erosion o he ube (Fig. 28-19) and/ or drain-
age pla e hrough any rein orcing gra issue
and he conjunc iva.1 An exposed ube or pla e
can be a nidus or in ec ion and ul ima ely lead
o endoph halmi is.2–4 During he ini ial sur-
gery, sclera, dura, ascia, pericardium, or spli
hickness cornea (Fig. 28-20B) is used as a
pa ch gra o preven ube erosion.5
Treatment
Ini ial managemen o any ube erosion
should include rapid surgical in erven ion
consis ing o replacing he pa ch gra usually
wi h he same ma erial ini ially used o pre-
ven possible in ec ion.
Any signs o in ec ion should include
promp cul ure and rea ing he in ec ion
wi h opical an ibio ics.1
Causes o ube erosion are varied bu
no well def ned and likely consis o several
ac ors, including poor issue urgor and
mechanical ac ors such as mechanical rub-
bing rom he lid.1
Dr. Budenz recommends rou ing he
ube direc ly a 12 o’clock when en ering he
an erior chamber o place he en ire ube
and pa ch gra comple ely undernea h he
upper lid o minimize mechanical rauma.1
In cases o recurren erosion, several groups
have s udied uses o ma erials such as double-
layered AM,6 double- hickness–processed
pericardium pa ch gra ,7 and buccal mucous
membrane gra s.8 Five ou o six cases using
buccal mucous membrane did no re-erode,
all he double-layered AM gra pa ien s were
rea ed success ully, and none o he pa ien s
wi h double- hickness pericardium had ube
exposure.
T e basic premise behind all hese ideas
is o minimize mechanical ac ors and use
a pa ch gra ma erial o cover he ube and
preven he ube rom eroding hrough he
conjunc iva and exposing he pa ien o
possible in ec ion.
EFE ENCES
1. Heuer DK, Budenz D, Coleman A. Aqueous shun
ube erosion. J Glaucoma. 2001;10(6):493–496.
2. Al- orbak AA, Al-Shahwan S, Al-Jadaan I, e al.
Endoph halmi is associa ed wi h he Ahmed glaucoma
valve implan . Br J Ophthalmol. 2005;89(4):454–458.
3. Bayrak ar Z, Kapran Z, Bayrak ar S, e al. Delayed-
onse s rep ococcus pyogenes endoph halmi is ol-
lowing Ahmed glaucoma valve implan a ion. Jpn J
Ophthalmol. 2005;49(4):315–317.
4. Gedde SJ, Scot IU, abandeh H, e al. La e endo-
ph halmi is associa ed wi h glaucoma drainage
implan s. Ophthalmology. 2001;108(7):1323–1327.
5. Sarkisian S Jr. ube shun complica ions and heir
preven ion. Curr Opin Ophthalmol. 2009;20(2):
126–130.
6. Ainswor h G, o ch ord A, Dua HS, e al. A novel use
o amnio ic membrane in he managemen o ube
exposure ollowing glaucoma ube shun surgery. Br J
Ophthalmol. 2006;90(4):417–419.
7. Lankaranian D, eis , Henderer JD, e al. Comparison
o single hickness and double hickness processed
pericardium pa ch gra in glaucoma drainage device
surgery: A single surgeon comparison o ou come. J
Glaucoma. 2008;17(1):48–51.
8. oo man DB, rope GE, oo man DS. Glaucoma
aqueous drainage device erosion repair wi h buccal
mucous membrane gra s. J Glaucoma. 2009;18(8):
618–622.
 ube Shunt Erosions 435

A B
FIGURE 28-18. Ultrasound imaging o glaucoma drainage device. A. A Baerveld glaucoma drainage device
prior o capsule orma ion. T e pla e is indica ed by he yellow arrow. B. A Baerveld glaucoma drainage device
a er ube opening wi h f uid (red arrow) overlying he pla e ( yellow arrow) .

FIGURE 28-19. Tube exposure. An exposed glaucoma drainage device ube as indica ed by he yellow arrow.

A B
FIGURE 28-20. Patch gra . A. Scleral pa ch gra . B. Clear corneal pa ch gra .
 Index
Note: Locators ollowed by ‘ ’ and ‘t’ re er to f gures and tables respectively.

A Aniridia, 162, 162 technique, 321

Abatacept (Orencia), 238 and cataract, 163 tissue response to, 322
Abnormal aqueous secretion, 210 Anterior chamber cleavage syndrome, Arteriovenous (AV)
Absolute pupillary block and iris 164 mal ormation, 301, 301
conf guration, 53 Anterior chamber depth, estimation o , shunts, 292
Acetazolamide, 220 28–29, 29 Arteriovenous passage time (AVP),
or primary acute angle closure, 276 Anterior segment optical coherence 138, 139
Acute angle closure, def ned, 270. See tomography (AS-OCT), Astrocytic hamartomas, 172
also Primary angle-closure 48, 51 Automated achromatic static visual f eld
glaucoma characteristics o , 49t (AASVF), 121
Acyclovir, 233 in cornea measurement, 51 Automated perimeters test, 84
Adalimumab (Humira), 238 Antimetabolites, intraoperative Avascular bleb, 346
A erent pupillary de ect (APD), 182 application o , 361, Axen eld-Rieger syndrome, 164
Ahmed Baerveldt Comparison (ABC), 361 –362 Axen eld’s anomaly, 164, 165
376 Anti-VEGF therapy, or bleb characteristics o , 164
Ahmed FP-7, 376 vascularization, 363
Alpha-agonist Applanation tonometry, 217. See also B
or glaucoma, 306, 306 –307 Uveitic glaucoma Baerveldt implant, 376
or intraocular pressure spike, 321, Apraclonidine, 306 Balanced salt solution (BSS), 353
323 Aqua ow collagen Band keratopathy, 237
Aminocaproic acid, 260 absorbable implant, 339 Barkan’s device, 24
Amniotic membrane (AM), 420 glaucoma drainage device, 332 Barkan’s goniolens, 154
gra technique, 422 –423 Aqueous ow, 4–6, 9 Beta-blockers
Angiotensin-converting enzyme conventional pathway, 4 or glaucoma, 308, 308 , 308t
(ACE), 244 routes o , 8 in uveitis treatment, 220
Angle-closure glaucoma, 40, 42 , 49–51, uveoscleral or alternative pathway, 4 Betaxolol, 220
51 –55 Aqueous humor Bevacizumab, 363
malignant glaucoma, 50, 54 –55 dynamics, measurement o , 5–6 Bilateral aoustic neurof bromatosis, 172
management o , 221–223 production rate measurement o , Bilateral Baerveldt implants and J , 225
phacomorphic glaucoma, 50, 54 5, 12 Bilateral carotid cavernous sinus f stula,
plateau iris, 50, 53 –54 proteins, 211 (See also Uveitic 10
pseudophakic malignant glaucoma, glaucoma) Bimatoprost, 305t
51, 55 Aqueous misdirection syndrome, 287, Bjerrum’s region, 82
pseudophakic pupillary block, 51, 288 –289 Bleb
55 clinical examination o , 287 avascular, 346
relative pupillary block, 49–50, epidemiology o , 287 di use, 346
52 –53 history o , 287 dysesthesia, 425–426, 428
Angle conf guration, light e ect in, 51 management o , 287 encapsulated, 372
Angle recession, 182, 266, 267 pathophysiology o , 287 enlarged, 346
causes o , 266 special studies o , 287 ailing, 430
and corneal scarring, 266 Aqueous shunting devices, 376 limiting conjunctivoplasty, 432
epidemiology o , 266 Arcuate de ect, 125, 130 needling, 363
gonioscopy and, 46, 47 , 266 Area dilution analysis, 140, 141 related ocular in ection, 370
history and clinical examination Argon and selective laser spots, vascularization, anti-VEGF therapy
o , 266 comparison o , 325 or, 363
pathophysiology o , 266 Argon laser, 221, 380 Blebitis, 419, 425, 427
posterior pole and, 266 iridoplasty, 386 Bleb leak, 419–421, 422 –423
slit-lamp examination, 266 usage o , 268 conservative management o , 419
special tests or, 266 Argon laser peripheral iridoplasty disadvantages o , 419
treatment o , 266 (ALPI), 50, 276 mechanism o , 419
Angle structure Argon laser trabeculoplasty (ALT), 197, outcomes and prognosis o , 420–421
elements, 32 201, 221, 236, 303, 323 Seidel test in, 419
groups o , 32 e cacy, 321–322 surgical treatment o , 419–420
identif cation o , 34, 35 –37 mechanism o action, 321 treatment o , 419–421

437
 4 3 8   INDEX
Blood ow, in glaucoma, 136–149
Brimonidine, 220, 306, 316
allergic reaction rom chronic, 307
Brinzolamide, 305t
B-scan ultrasonography, 217, 415
or cyclodialysis cle , 268
or traumatic hyphema, 259
Buphthalmos, 154, 157
Bupivacaine, in deep sclerectomy
surgery, 331
C posterior synechiae, 214–215, 216
Cadaver eye, dissection o , 7
Canaloplasty, 392–393
e ect, 394
mechanism o action, 392–393
outcomes o , 393
technique, 393
Capsulorhexis, 256
Carbonic anhydrase inhibitors (CAI),
220, 260, 276, 321
or glaucoma, 309–310, 310
Carotid angiography, 293
Carotid-cavernous f stula (CCF), 32,
292–293
classif cation o , 292
clinical presentation o , 293
diagnostic evaluation o , 293, 294
management o , 293, 295
pathophysiology o , 292–293
Carteolol, 220, 305t, 308t
Cataract
and Fuchs’ heterochromic
iridocyclitis, 228
mature, 248
subcapsular, 228
traumatic, 264
Cavernous sinus (CS), 292
Central retinal vascular occlusions
(CRVO), 282
Chandler’s syndrome, 284, 286
Choroidal detachment, 367
Choroidal e usion
draining, 414
repair o , 418
Choroidal per usion and ocular pulse
amplitude, 21
Chronic angle-closure glaucoma,
270–281, 277
diagnostic evaluation o , 277
gonioscopy and, 277
treatment o , 277
Chronic hypotony, 369 , 387 , 413
Chronic obstructive pulmonary disease
(COPD), 308
Ciliary body
rotation o , 215 (See also Uveitic
glaucoma)
tumors, 58, 59
Cipro oxacin, 343
Circum erential bleb, 432, 433
treatment o , 432
Cirrus SD-OCT progression analysis
so ware, 81
Cirrus SD-OCT RNFL imaging scan, 80
Closed-angle mechanisms, in uveitic
glaucoma, 214–215. See also
Uveitic glaucoma
ciliary body, 215
peripheral anterior synechiae, 214,
215
Cogan–Reese syndrome, 284
Cogan’s syndrome, 235
Collaborative Initial Glaucoma
Treatment Study (CIGTS),
302
Collagen def ciency syndromes, 292
Color Doppler imaging (CDI), 142, 143
description o , 142
limitations o , 142
purpose o , 142
Color stereophotographs, 64
Combigan, 316
Con ocal scanning laser doppler
owmetry, 146
description o , 146
limitations o , 146
purpose o , 146
Con ocal scanning laser
ophthalmoscopy (CSLO),
67–68, 105, 106 –107
limitations o , 105
role o , 105
usage o , 105
Congenital anomalies, glaucoma
associated, 162–166
aniridia, 162, 162 , 163
Axen eld’s, 164, 165
Peter’s, 164, 166
Rieger’s, 164
Rieger’s syndrome, 164, 165 , 166
Congenital glaucoma, 152–173
in gonioscopy, 153, 156 –157
during goniotomy, 158
primary, 153–154, 155 –161
round cupping in, 153, 156
Congenital syphilis, 235
Congestive heart ailure (CHF), 308
Conjunctiva, ballooning o , 354
Conjunctival advancement, 419
Conjunctival bu onhole, 365t, 371
Conjunctival erosion, 386, 390
Conjunctival gra , 419–420
Contact transscleral
cyclophotocoagulation
(CPC), 402
Continuous-wave argon laser, 408
Contusion trauma, gonioscopy in,
46, 46
Cornea
measurement o , 51
mesodermal dysgenesis o , 164
Corneal blood staining, 263
Corneal decompensation, 390
Corneal edema, 386
Corneal paracentesis, 356 , 382
Corneal patches, usage o , 424–425. See
also Giant blebs
Corneal traction suture, 331
Corneotrabeculodysgenesis, 152
Corticosteroids
oral, 219
and uveitis treatment, 209–210, 212
Cosopt, 316
Cushing’s syndrome, 204
Cyclocryotherapy (CCT), 223, 404,
407, 407
Cyclodestructive procedures, 402–411
complications o , 411, 411t
contact transscleral, 406, 406
contraindications to, 403
cyclocryotherapy, 407, 407
endoscopic, 409, 410
indications or, 402–403
noncontact transscleral, 404, 405
postprocedure care or, 411
techniques, 404
transpupillary, 408, 408
Cyclodialysis cle , 58, 58 , 268, 269
B-scan ultrasonography or, 268
clinical examination o , 268
def ned, 268
epidemiology o , 268
gonioscopy and, 268
history o , 268
occurrence o , 268
pathophysiology o , 268
slit-lamp examination o , 268
special tests or, 268
treatment o , 268
Cyclodialysis, gonioscopy in, 46, 47
Cyclophotocoagulation (CPC), 402
Cyclosporine, 238, 245
Cystoid macular edema (CME), 220, 403
D
Dahan Diamond Schlemm’s canal
opener kni e, 331
Dahan incision, 331
Dahan trabecular meshwork scraper, 332
Dalen–Fuchs’ nodules, 244
Deep sclerectomy surgery
or glaucoma, 328–349
advantages o , 343
postoperative care, 343–344,
345 –349
studies, 328–329
surgical technique, 331–333,
334 –342

Descemet’s membrane, 34, 44, 153,
154, 328
Descemet’s window, prolene suture
in, 396
Developmental glaucoma. See
Congenital glaucoma
DeWecker scissor, usage o , 353
Di uprednate (Durezol), 219
Digital ocular compression, 363
Diode laser, role o , 67
Dipive rin, 315
Direct gonioscopy, 22–24
advantages o , 22–23, 23
def ned, 22
disadvantages o , 23, 24
instruments, 22
Koeppe lens, 22, 23
Disc Damage Likelihood Scale
(DDLS), 188 , 192
Disc hemorrhage, 184
Doppler ocular coherence tomography,
149
advantages o , 149
description o , 149
limitations o , 149
purpose o , 149
Doppler shi , 146
Dorzolamide, 305t
Drainage device, 376–391
aqueous misdirection, 389
choroidal detachment, 388
choroidal e usion, 388
complications o , 386–387
conjunctival erosion, 390
corneal decompensation, 390
corneal paracentesis, 379
implant migration, 391
implant occlusion, 389
latina or ripchord suture, 385
ligature suture, 385
nonabsorbable sutures, 379
nonrestrictive, 376, 377
pars plana tube shunt, 384
patch gra , 384
postoperative care or, 386
restrictive, 376, 378
ripchord suture, 385
scleral f xation, 381
superotemporal quadrant, 379
suprachoroidal hemorrhage, 388
surgical technique, 379–380
track creation, 383
tube f xation, 384
tube insertion, 383
tube malposition, 391
tube trimming, 382
Dynamic contour tonometry (DCT),
21, 21
Dynamic gonioscopy, 44, 45 . See also
Gonioscopy
Dysesthetic bleb, 429
Dysgenesis, 164
E Fornix-based ap, 352, 353, 355
Early Mani est Glaucoma Trial
(EMGT), 88
Ehlers-Danlos syndrome, 292
Eight-ball hyphema, 258
Electroretinography, 95
Encapsulated blebs, 61, 62
Encapsulation, 430, 431
treatment and outcomes o , 430
Encephalotrigeminal angiomatosis. See
Sturge-Weber syndrome
(SWS)
End diastolic velocity (EDV), 142
Endocyclophotocoagulation, 193
Endophthalmitis, 253
Endophthalmitis Vitrectomy Study, 424
Endoscopic cyclophotocoagulation
(ECP), 403, 409, 410
Enhanced corneal compensator (ECC),
71, 102
Epinephrine, 305t, 315
Epiphora, 154
Episcleral venous pressure, 5
measurement o , 12
normal values or, 6
Epstein–Barr virus, 235
Eserine, 302, 304
Esnoper V-2000, 332, 340
Essential iris atrophy, 284, 285
Esterman f eld, 190
Ex oliation syndrome (XFS), 56, 57 ,
182, 200–201
clinical examination o , 200–201,
201 –203
epidemiology o , 200
history o , 200
pathophysiology o , 200
treatment o , 201
Ex-PRESS mini glaucoma shunt, 373
surgical technique or, 374, 374 –375
and trabeculectomy, 350–375, 351
Eye, anatomy o , 52
F congenital, 152–173
Failed bleb, 61, 62
Fast Fourier trans ormation, 146
Filtering bleb, 61, 62 , 63
Fluocinolone acetonide implant, 212
Fluorescein, 5
dye, 138
Fluorescein SLO angiography, 138, 139
description o , 138
purpose o , 138
Fluorescent treponemal antibody
absorption (FTA-ABS)
test, 236
INDEX 4 3 9
Fluorophotometer, 5, 12
usage o , 5
5- uorouracil (5-FU), 222, 329, 361
Fourth phacomatosis, 296
Fovea, def ned, 122
Frequency-doubling perimetry (FDP),
132, 132 –135
Frequency-doubling technology
(FDT), 92, 93 –94
limitations o , 92
role o , 92
usage o , 92
Fuchs’ heterochromic iridocyclitis,
227–228, 229
and cataract, 228
course o , 228
diagnostic evaluation o , 227–228
di erential diagnosis o , 227
epidemiology o , 227
etiology o , 227
and glaucoma, 228
history o , 227
laboratory studies o , 228
management o , 228
ophthalmic examination o , 227–228
Functional reserve, 132
G
Ganglion cell, 125
Giant blebs, 424–426, 426
bleb dysesthesia, 425–426
corneal patching, 425, 428
in ections and corneal patches usage,
424–425, 427
treatment o , 424
Glaucoma, 2
alpha agonists or, 306, 306 –307
angle-closure, 40, 42 , 174, 221–223
associated with congenital
anomalies, 162–166
beta-blockers or, 308, 308 , 308t
blood ow in, 136–149
carbonic anhydrase inhibitors,
309–310, 310
chronic angle-closure, 270–281
combination agents or, 316, 316
CSLO and, 105
cyclodestructive procedures
or, 402–411 (See
also Cyclodestructive
procedures)
deep sclerectomy surgery or, 328–
349, 334 –342 , 345 –349
(See also Deep sclerectomy
surgery)
def nition o , 2, 174–175, 176
detection and RNFL analysis, 75
detection, OCT in, 110
 4 4 0   INDEX
Glaucoma (continued)
drainage device, 222, 376–391 (See
also Drainage device)
drop instillation or, 317, 317 –318
FDT or, 92
and Fuchs’ heterochromic
iridocyclitis, 228
hyperosmolar agents or, 311
ipsilateral, 172
laser trial, 322
lens-associated open-angle, 246–257
and lens-induced uveitis, 240–242
(See also Lens-induced
uveitis)
lens particle, 240, 250–251
lens protein, 247–248, 248 , 249
management o , 302–303
medical treatment o , 304–319, 305t
miotics or, 311, 311t
neovascular, 40, 43 , 216 , 228
occurrence o , 82
and optic nerve, 13
pathogenesis o , 4
phacolytic, 240, 247
phacomorphic, 240, 256, 257
pigmentary, 40, 41
primary, 150, 174
primary angle-closure, 270–281
primary open-angle, 150, 174–195
(See also Primary open-
angle glaucoma)
prostaglandins or, 313–314, 314
punctal occlusion or, 317, 319
risk actor or, 4, 82, 180t–181t, 402
and RTA, 119
Schlemm’s canal-based surgery,
392–401
scientif c history o , 2
secondary, 150, 152, 174
secondary angle-closure, 282–289
secondary open-angle, 196–207
secondary to elevated venous
pressure, 292–301
surgery in uveitis patient, 226
SWAP testing in, 90
sympathomimetic agents or, 315, 315
tissue damage development in, 179t
trabeculectomy in, 350, 351 (See
also Trabeculectomy)
traumatic, 258–269 (See also
Traumatic glaucoma)
uveitic, 208–245 (See also Uveitic
glaucoma)
Glaucoma diagnosis (GDx) test, 71
strengths o , 72
Glaucoma drainage device (GDD), 350,
376, 419
ultrasound imaging o , 435
Glaucoma drainage implants, 61, 63
Glaucoma graph, 192, 194
Glaucoma hemif eld testing, 84, 84
Glaucoma Laser Trial (GLT), 302
Glaucoma probability score (GPS),
105, 109
Glaucoma progression analysis (GPA),
88, 88
limitations o , 88
role o , 88
usage o , 88
Glaucoma surgery, late complications
o , 412–435
Glaucomatocyclitic crisis, 230–231
course o , 231
diagnostic evaluation o , 230–231
di erential diagnosis o , 230
epidemiology o , 230
etiology o , 230
history o , 230
laboratory studies o , 231
management o , 231
ophthalmic examination o , 230–231
Glaucomatous disc, 153
Glaucomatous optic nerve injury, 209
Glaucomatous optic neuropathy
(GON), 64, 82
Glaucomatous visual f eld de ects, 82, 83
Glaukom ecken, 279
Glycerin, 311
in uveitis treatment, 220
Goldmann applanation tonometer, 14, 15
usage o , 14
Goldmann equation, 5
modif cation o , 5
Goldmann goniolens, 30
Goldmann lens, 25, 321
Goldmann visual f eld, 122, 124
Goniolenses
characteristics o , 25t
types o , 26
Gonioscopy, 22–47, 36 , 182
angle anatomy, elements o , 32, 32 –33
angle classif cation, 38, 38 –39
angle structure in, 32, 32
in angular recession, 46, 47
in contusion trauma, 46, 46
in cyclodialysis, 46, 47
def ned, 22
direct, 22–24
dynamic, 44, 45
error actors in, 44, 44 –45
in ICE syndrome, 284
indirect, 25–27
in iridodialysis, 46, 47
o iris cyst, 32, 33
iris plane, 32
o iris processes, 34, 36 –37
iris root, 32
objectives o , 38
pigment deposition and, 40, 41 –43
purpose o , 22
o Schiemm’s canal, 32, 33
o Schwalbe’s line, 30, 34, 35
technique o , 30, 30 –31
in trauma, 46, 46 –47
and traumatic hyphema, 259
Goniosynechialysis, 221
Goniotomy, 154
procedures, 159
Goniotomy kni e, usage o , 221
Granulomatous uveitis, signs o , 217
Guarded f ltration surgery, 350
Guided progression analysis, 72, 74
def ned, 88
H
Haab’s striae, 153, 155
Haag-Streit 900 series slit-lamp
biomicroscope, 182
Heavy-molecular-weight (HMW)
protein, 247
Heidelberg retinal owmeter (HRF),
146, 146 –147
Heidelberg retina tomography (HRT),
67, 69 , 105
advantages o , 67–68
disadvantage o , 68
usage o , 67
Hematoxylin and eosin (H&E), 7
Hemodynamics measurement,
instruments or, 137
HeNe laser slit beam, 119
Herpes simplex uveitis, 232
Herpes simplex virus (HSV), 230
Herpes zoster uveitis, 232
Herpetic keratouveitis, 232–233
course o , 233
diagnostic evaluation o , 233
di erential diagnosis o , 232–233
epidemiology o , 232
etiology o , 232
f ltration surgery, 233
history o , 232
laboratory studies o , 233
management o , 233
ophthalmic examination o , 233
Herpetic uveitis, 234
Heterochromia, 227
“Hill o vision,” 122, 123
HLA-B27-associated anterior uveitis,
215 , 230
Hoskin anatomic classif cation, 152
Hoskins lens, 366
Humphrey f eld analyzer (HFA), 84, 133
Humphrey machine, 122
and AASVF test, 131
Humphrey visual f eld, 190
pa ern deviation, 88
Hyperosmolar agents, or glaucoma,
311
Hyperosmotic agents, 321

Hyphema, 258, 368
eight-ball, 263
layering, 262
small, 261
total, 262
traumatic, 261
Hypotony, 386, 413–415, 416
chronic, 369 , 387 , 413
delayed suprachoroidal
hemorrhages, 414–415
maculopathy, 268, 386, 413
shallow and at anterior chamber,
413–414, 413t, 417
I in uveitis treatment, 220
Idiopathic elevated episcleral venous
pressure (IEEVP), 299,
299 –300
Imbert-Fick law, 14
Implant migration, 391
Implant occlusion, 389
Indentation gonioscopy, 271
Indirect gonioscopy, 25–27
goniolenses, selection o , 25
multiple lenses or, 25, 25t
observer and obstacle, 27
open-angle conf guration, diagram
o , 26
Indocyanine, or phacomorphic
glaucoma, 256
Indocyanine green (ICG), 140
SLO angiography, 140, 141
description o , 140
purpose o , 140
Indomethacin, 231
In ammatory cells, 211. See also Uveitic
glaucoma
In ammatory glaucoma, 208
In iximab (Remicade), 238
Interstitial keratitis, 235
Intraocular pressure (IOP), 2, 4, 50, 136,
175, 192, 196, 197, 246, 292,
304, 328, 350, 376, 392
determination o , 5
importance o , 4
lowering o , 192t
Iridectomy, 302
surgical, 221, 357
Iridociliary cyst, 58, 59
Iridocorneal angle, 50
Iridocorneal (ICE) syndrome, 284
clinical examination o , 284
epidemiology o , 284
gonioscopy, 284
history o , 284
management o , 284
pathophysiology o , 284
Iridodialysis, gonioscopy in, 46, 47
Iridoschisis, 58, 60
Iridotrabeculodysgenesis, 152
INDEX 4 4 1
Iris Laser spots, comparison o spacing
atrophy, 233 o , 326
cysts, 32, 33 Laser trabeculoplasty, 192, 266,
hamartomas, 172 320–327
injury, 265 economics, 327
mesodermal dysgenesis o , 164 indications o , 320
neovascularization, 227 and intraocular pressure, 320
neovascularization o , 283 Latanoprost (Xalatan), 220, 304
nevus syndrome, 286 Late bleb leak, 371 , 419
processes, 34, 36 –37 Leishmaniasis, 235
Iris bombé, 278 Lens-associated open-angle glaucomas,
causes o , 221 246–257
recurrent, 225 clinical presentation o , 246t
Isosorbide, 305t, 311 Lens-associated uveitis (LAU), 246,
253–254, 255
iStent, 392, 399, 400 clinical examination o , 253–254
mechanism o action, 399 history o , 253
outcomes o , 399 pathophysiology o , 253
positioning, 401 special tests or, 254
postoperative appearance, 400 treatment o , 254
technique, 399 Lens extraction, e ect o , 280
iTrack microcatheter, 393, 396 Lens f ber, 252
Lens-induced uveitis, 240–242
J course o , 241
Juvenile idiopathic arthritis ( JIA), diagnostic evaluation o , 241
237–238 di erential diagnosis o , 241
course o , 238 epidemiology o , 240
diagnostic evaluation o , 237–238 etiology o , 240–241
di erential diagnosis o , 237 history o , 240
epidemiology o , 237 laboratory studies o , 241
etiology o , 237 management o , 242
history o , 237 ophthalmic examination o , 241
laboratory studies o , 238 Lens particle glaucoma, 240, 250–251
management o , 238 clinical examination o , 250–251
ophthalmic examination o , history o , 250
237–238 pathophysiology o , 250
Juvenile rheumatoid arthritis. See Juvenile special tests or, 251
idiopathic arthritis ( JIA) treatment o , 251
Lens protein glaucoma, 247, 248 , 249
K clinical examination o , 247
Kaplan-Meier survival curves, 421 history o , 247
Kelly punch, 414 pathophysiology o , 247
Keratic precipitates, 228, 230 special tests o , 247
Kissing choroidals, 413 treatment o , 247–248
Koeppe lens, 22, 23 Lens pseudoex oliation, 40, 41
Krukenberg spindle, 40, 41 , 182, 196, Levobunolol, 220, 305t, 308t
197 Lidocaine
in deep sclerectomy surgery, 331
L in glaucoma surgery, 352
Laser Doppler owmetry (LDF), 146 Lidocaine 1%, 353 , 354
limitations o , 146 Limbus-based ap, 352, 353, 355 , 359
Laser are photometry, 217 Lisch nodules, 172
Laser goniopuncture, 329 Lyme disease, 235
Laser iridotomy, 50, 56, 221, 236
and iris conf guration, 52 M
or sarcoidosis, 245 Mackay-Marg-type tonometer, 19, 20
Laser peripheral iridotomy (LPI), Macular thickness analysis scan, 76, 79
197, 276 Malignant glaucoma, 50, 54 –55 . See
e ect on anterior chamber angle, also Aqueous misdirection
280 syndrome
 4 4 2   INDEX
Mannitol, 311
in uveitis treatment, 220
Mar an syndrome, 167, 167 –168
characteristics o , 167
Mean transit time (M ), 138
Medical treatment
or glaucoma, 304–319, 305t
or uveitic glaucoma, 220–221 (See
also Uveitic glaucoma)
Mermoud predescemetic spatula, 332
Methazolamide, 305t
Methotrexate, 238
Metipranolol, 220
Microhemagglutination test Treponema
pallidum (MHA-TP), 236
Microhyphema, 258
Microlase, 404
Microspherophakia, 169, 169
Millipore f ltration, 247
Miotics, or glaucoma, 311, 311t
Mitomycin C (MMC), 332, 361, 361
application o , 361
handling o , 362
Monoamine oxidase (MAO), 306
Moorf eld’s regression analysis (M ),
67, 69 , 105, 108
Moxi oxacin, 343
Multi ocal choroiditis, 218
Multi ocal electroretinography
(m ERG), 95, 96
limitations o , 95
role o , 95
usage o , 95
Multi ocal visually evoked cortical
potential (m VEP), 97, 98
My cells, 132
Mycobacterium tuberculosis, 235
N pigment dispersion syndrome,
Narrow angle, def ned, 271
Nasal step de ect, 125, 128 , 129
Nasolacrimal duct, obstruction o , 154
Nd:YAG laser, 51, 221
Neodymium (Nd):YAG capsulotomy,
220
Neodymium (Nd):YAG laser, 324,
386, 404
Neovascular glaucoma (NVG), 40, 43 ,
216 , 228, 282–283
clinical examination o , 282–283
epidemiology o , 282
history o , 282
management o , 283
pathophysiology o , 282
Nerve bundles, glaucomatous damage
to, 126
Nerve f ber indicator (NFI), 71
Nerve f ber layer (NFL), 82
evaluation o , 82–119
photography, 99, 100 –101
limitations o , 99
role o , 99
usage o , 99
Neurof bromatosis (NF), 172, 173
orms o , 172
NF-1, 172
NF-2, 172
Neurof bromatosis (NF)-1, 172
Neurof bromatosis (NF)-2, 172
Neuroretinal rim, 67, 109
Noncontact transscleral CPC
technique, 404, 405 . See
also Cyclodestructive
procedures
Nonsteroidal anti-in ammatory, 386
O photography, 99, 100 –101
Octopus AASVF test, 122, 124
Octopus visual f eld, 190
Ocular coherence tomography (OCT),
149
Ocular hypertension, 386
Ocular hypertension treatment study
(OHTS), 67
Ocular pulsation, measurement o ,
144, 145
description o , 144
purpose o , 144
Ocular pulse amplitude, 21
Ocular syphilis, 235
diagnosis o , 236
Onchocerciasis, 235
One-eyed therapeutic trial, 304
Open-angle conf guration, diagram
o , 26
Open-angle glaucoma, 56, 57
ex oliation syndrome, 56, 57
pigmentary glaucoma, 56
56, 57
Open-angle mechanisms, in uveitic
glaucoma, 210–213. See also
Uveitic glaucoma
abnormal aqueous secretion, 210
aqueous humor proteins, 211
in ammatory cells, 211
prostaglandins, 211
steroid-induced ocular hypertension,
212–213
trabeculitis, 211–212
Optical coherence tomography (OCT),
75–76, 110, 111 –118 ,
271–272, 343
advantages o , 76
anterior chamber angle imaging with,
274 , 275
limitations o , 110
role o , 110
usage o , 110
Optic disc images, 65
Optic nerve, 13
damage, 13
evaluation o , 82–119, 153
automated perimetry, 84
con ocal scanning laser
ophthalmoscopy, 105,
106 –107
requency-doubling technology,
92, 93 –94
unctional tests, 82–98
glaucoma progression analysis,
88, 88
multi ocal electroretinography,
95, 96
optical coherence tomography,
110, 111 –118
retinal thickness analyzer, 119
scanning laser polarimetry, 102,
103 –104
short-wavelength automated
perimetry, 90, 91
structural tests, 99–119
Swedish interactive threshold
algorithms, 85, 86 –87
visual f eld index, 89, 89
visually evoked cortical potential,
97, 98
f eld, 121
gliomas, 172
H&E-stained histopathologic
section o , 13
imaging devices, principles and
clinical parameters o , 65t
Optic nerve head (ONH), 64, 65, 190
analysis, 75, 78
undus image, 71, 73
Out ow tracts, 8
Overf ltration, 365t, 366, 369
P
Palmberg sutures, 370
Palmberg transconjunctival sutures, 366
Panuveitis, 254
Partial-thickness ap, 356
Pascal tonometer. See Dynamic contour
tonometry (DCT)
Patch gra , 379
Peak systolic velocity (PSV), 142
Peribulbar anesthesia, 331
Perimetric testing, 122. See also
Psychophysical test
Perimetry, static and kinetic, 122, 123
Periocular steroid injection, in steroid
responder, 213
Peripheral anterior synechiae, 37 , 53 ,
214, 215 , 228, 271, 274 .
See also Uveitic glaucoma
Peripheral choroidal e usions, 417
Perkins tonometer, 18, 18

Peter’s anomaly, 164, 166
Phacoanaphylactic endophthalmitis, 240
Phacoanaphylactic uveitis, 240, 253
Phacoanaphylaxis. See Lens-associated
uveitis (LAU)
Phacoantigenic uveitis, 240
Phacolytic glaucoma, 240, 247
clinical examination o , 247
history o , 247
pathophysiology o , 247
special tests o , 247
treatment o , 247–248
Phacomorphic glaucoma, 50, 54 , 240,
256, 257
clinical examination o , 256
history o , 256
pathophysiology o , 256
treatment o , 256
Phacotoxic uveitis, 240
Photography, 99
NFL, 99, 100 –101
stereoscopic, 99, 100
Photophobia, 154
Physostigmine, 305t, 311t
Pigmentary glaucoma, 40, 41 , 56. See
also Open-angle glaucoma
Pigment deposition and gonioscopy, 40,
41 –43
angle-closure glaucoma, 40, 42
lens pseudoex oliation, 40, 41
neovascular glaucoma, 40, 43
pigmentary glaucoma, 40, 41
uveitis, 40, 42
Pigment dispersion syndrome (PDS),
56, 57 , 182, 196–197
clinical examination o , 196–197
epidemiology o , 196
history o , 196
and Krukenberg’s spindle, 198
pathophysiology o , 196
peripheral iris, 199
pigment deposition, 198 –199
transillumination de ects in, 198
treatment o , 197
Zentmeyer’s line, 199
Pigment storm, 196
Pilocarpine, 304
hydrochloride, 305t, 311t
nitrate, 305t, 311t
strengths, 312
Plateau iris, 50, 53 –54 , 277
diagnostic evaluation o , 277
slit-lamp examination and, 277
treatment o , 277
Plateau iris conf guration, 281
Plateau iris syndrome, 53 –54
Plexi orm neurof bromas, 172
Pneumotonometer, 5, 20, 20
Port wine stain, 296. See also Sturge-
Weber syndrome (SWS)
Posner-Schlossman Syndrome. See
Glaucomatocyclitic crisis
Posterior chamber intraocular lens
(PCIOL), 250
Posterior embryotoxon, 164
Posterior synechiae, 214–215, 216
Prednisolone acetate (Pred Forte), 219
Prednisone, 238
Primary acute angle closure, 276–281,
278
clinical examination o , 276
diagnostic evaluation o , 276
prognosis o , 276
symptoms o , 276
treatment o , 276
Primary angle-closure glaucoma,
270–281, 272t
background o , 270
clinical examination o , 271–272
epidemiology o , 271
gonioscopy and, 271
management o , 277
OCT and, 271–272
pathophysiology o , 270–271, 273
with relative pupillary block, 277
risk actors or, 271
UBM and, 271–272
Primary congenital glaucoma (PCG),
153–154, 155 –161
angle visualization, 158
characteristics o , 153
clinical examination o , 153–154
di erential diagnosis o , 154
epidemiology o , 153
history o , 153
management o , 154
unilateral, 153, 155
Primary open-angle glaucoma,
174–195
clinical examination o , 182–184
def nition o , 174–175, 176
epidemiology o , 177
external and biomicroscopic
examination, 182, 184
gonioscopy, 182
history o , 180
management o , 191
pathophysiology o , 178, 178
posterior pole, 182–184, 185 –189
risk actors o , 180t–181t
special tests, 190
treatment o , 191–195, 191t–193t
Primary open angle glaucoma (POAG),
2, 136, 392
Propionibacterium acnes, 253
Prostaglandin, 230
analogs, 220
or glaucoma, 313–314, 314
role o , 211
Pseudoex oliation, 252
INDEX 4 4 3
Pseudophakic malignant glaucoma,
51, 55
Pseudophakic pupillary block, 51, 55
Psychophysical test, 120–135
def ned, 120
description o , 122–124
requency-doubling perimetry, 132,
132 –135
optic nerve visual f elds, 125, 126 –131
purpose o , 121
short-wave automated perimetry,
132, 132 –135
Pulsatile ocular blood ow (POBF),
144, 145
Pulsed dye laser photocoagulation, 296
Punctal occlusion, or glaucoma, 317,
319
Pupillary block, 214
R
Ranibizumab, 363
Rapid plasma reagin (RPR), 236
Re erence plane, def ned, 67
Relative pupillary block, 49–50, 52 –53
def ned, 50
Resistive index (RI), 142
Retardation, 71
Retinal ganglion cell, 82
Retinal nerve f ber layer (RNFL), 64,
125, 178
analysis scanning, ast, 75, 77
deviation map, 71, 73
thickness map, 71, 73
and TSNIT graph, 71, 74
Retinal pigment epithelium (RPE), 75
Retinal sensitivity, 122
Retinal thickness analyzer (RTA), 119
limitations o , 119
role o , 119
usage o , 119
Retinal thickness mapping, 119, 119
Retrobulbar anesthesia, in Contact
transscleral CPC, 406
Retroillumination, with Koeppe lens, 23
Reverse pupillary block, 197
Rieger’s anomaly, 164
Rieger’s syndrome, 164, 165 –166
Ritch goniolen, 321
Round cupping, 156
Rubella virus, 227, 228
S
Sampaolesi’s line, 200
Sampaolesi’s sign, 40, 41
Sarcoidosis, 235, 243–245
course o , 244
diagnostic evaluation o , 243–244
di erential diagnosis o , 243
epidemiology o , 243
etiology o , 243
 4 4 4   INDEX
Sarcoidosis (continued)
history o , 243
laboratory studies o , 244
management o , 244–245
ophthalmic examination o , 243–244
visual loss in, 244
Sarcoidosis and active granulomatous
panuveitis, 218
Scanning electron microscopy, o
cadaver eyes, 326
Scanning laser ophthalmoscope (SLO)
angiography, 138–141, 139
Scanning laser polarimetry (SLP),
71–72, 102, 103 –104
limitations o , 102
role o , 102
usage o , 102
Schiemm’s canal, 32, 33
Schiotz tonometer, 5, 16, 17
Schlemm’s canal, 154, 299
based surgery, 392–401
canaloplasty, 392–393
indications o , 392
blood in, 300
Schwalbe’s line, 30, 34, 35 , 49, 164, 200
Scleral dissection, 394 –395
Scleral ap, 352
Scleral spur, 34
Seclusio pupillae, 214
Secondary angle-closure glaucoma,
282–289
Secondary open-angle glaucoma,
196–207
Seidel test, in bleb leak, 419
Selective laser trabeculoplasty (SLT),
324–325
e cacy, 324–325
mechanism o action, 324
technique, 324
Semiconductor diode laser, 404
Sha er’s classif cation, o angle, 38
Sha er-Weiss anatomic classif cation,
152
Shields lens, 405
Short-wavelength automated perimetry
(SWAP), 90, 91 , 132,
132 –135
limitations o , 90
role o , 90
usage o , 90
Slit lamp, 196
biomicroscopy, 48
usage o , 25
Small hyphema, 259, 261
Spaeth gonioscopic grading system, 182
Spaeth’s classif cation, o angle, 39
Spectral domain system OCT (SD-
OCT), 75, 76, 80 –81
Spectral retinal oximetry (SRO), 148,
148
description o , 148 role o , 85
purpose o , 148 usage o , 85
Stereophotography, 64–65, 66 Sympathomimetic agents, or glaucoma,
Stereoscopic photography, 99, 100 315, 315
limitations o , 99 Synechiae, posterior, 214–215, 216
role o , 99 Syphilitic interstitial keratitis, 235–236
usage o , 99 course o , 236
Steroid-induced glaucoma, 205t diagnostic evaluation o , 235–236
Steroid-induced ocular hypertension, di erential diagnosis o , 235
212–213. See also Uveitic epidemiology o , 235
glaucoma etiology o , 235
Steroid responder, periocular steroid history o , 235
injection in, 213 laboratory studies o , 236
Steroid-responsive glaucoma, 204–205, management o , 236
206 –207 ophthalmic examination o ,
clinical examination o , 204 235–236
epidemiology o , 204
history o , 204 T
pathophysiology o , 204 Tabular Data Section, 75, 77
treatment o , 204–205 Te on cannula, 333
Steroid-sparing agents, usage o , 219 Temporal wedge, 127
Stevens-Johnson syndrome, 309 Tenon cysts, 61
Stevens tenotomy scissors, usage o , 379 T-Flux implant, 329
Still’s disease, 237 Time domain system OCT (TD-
Stratus TD-OCT macular thickness OCT), 75, 76
analysis scan, 79 limitations o , 76
Stratus TD-OCT ONH analysis scan, 78 Timolol, 220, 316
Streptococcus sp., 425 Tomato-catsup undus, 170. See also
Stromal keratitis, 232 Sturge-Weber syndrome
Sturge-Weber syndrome (SWS), (SWS)
32, 170, 170 –171 , 292, Tonography, 5, 11
296–297, 297 –298 Tonometry, 14–21
clinical presentation o , 296 def ned, 14
etiology o , 296 Goldmann applanation, 14, 15
management o , 296–297 Mackay-Marg-type, 19, 20
visual loss, 296 Pascal, 21, 21
Subconjunctival injections, o Perkins, 18, 18
5- uorouracil, 329 pneumotonometer, 20, 20
Sub-Tenon’s anesthesia, 331 Schiötz, 16, 17
incision in conjunctiva or, 333 Tono-Pen©, 19, 19
Sub-Tenon’s injection, 212, 219 types o , 14
Sub-Tenon’s steroids, 411 Tono-Pen©, 19, 19
Superior vena cava syndrome, 292 Topical carbonic anhydrase inhibitors,
Superotemporal quadrant, 380 220
Suprachoroidal hemorrhage, 368 , Topical corticosteroid
388 , 415 or lens particle glaucoma, 251
delayed, 414–415 or postlaser in ammation
Surgical iridectomy, 236 prevention, 321
or sarcoidosis, 245 usage o , 219
in uveitic eyes, 221 (See also Uveitic Topical diclo enac, 343
glaucoma) Topical steroids, 247, 363
Suture Topographic change analysis (TCA),
latina, 385 67, 70 , 105, 109
lysis, 364 Topography standard deviation, usage
palmberg, 370 o , 67
Swan-Jacob lens, usage o , 154 Total hyphema, 258
Swann-Jacobs gonioprism, 397 Total internal re ection, 22
Swedish interactive threshold Trabectome, 392, 397, 398
algorithm (SITA), 85, handpiece and setup, 398
86 –87 , 122 mechanism o action, 397

outcomes o , 397
technique, 397
Trabecular meshwork-inducible
glucocorticoid response
(TIGR), 204
Trabecular meshwork (TM), 4, 5, 392
con ocal microscopy o , 9
Trabeculectomy, 154, 222, 350–375, 351
antimetabolites, intraoperative
application o , 361, 361 –362
complications o , 365–366, 365t,
367 –372
conjunctival bu onhole, 371
conjunctival closure, 359 –360
encapsulated bleb, 372
ornix-based ap, 355
internal tissue block removal, 357
late bleb leak, 371
limbus-based ap, 355
mechanical, 160
partial thickness ap, 356
postoperative care o , 363, 364
releasable sutures, 358
scleral ap, suturing the, 357
surgical iridectomy, 357
surgical technique, 352–353
suture, 161
topical anesthetic agents, 353
traction suture placement, 354
Trabeculitis, 211–212. See also Uveitic
glaucoma
Trabeculodescemetic membrane, 338
Trabeculodialysis, 221, 238
Trabeculodysgenesis, 152
Trabeculotome, 154
Traction suture, 381
Transcorneal ap revision, 431
Transcorneal sutures, 413, 415
Transpupillary transscleral CPC, 408,
408
Trapezoidal, 331
Trauma, gonioscopy usage in, 46, 46 –47
Traumatic cataract, 264 , 411t
Traumatic glaucoma, 258–269
Traumatic hyphema, 258–260
B-scan ultrasonography in, 259
epidemiology o , 258
gonioscopy and, 259
history and clinical examination
o , 259
pathophysiology o , 258–259
rebleeding in, 258
and slit-lamp examination, 259
special tests o , 259–260
treatment o , 260
Travoprost, 305t
Trend analysis so ware, 76
Treponema pallidum, 236
Triamcinolone, 219
TSNIT image, 75, 77
Tube shunt erosions, 434, 435
causes o , 434
treatment o , 434
Tube shunts, 193, 376
Tube versus Trabeculectomy Study, 376
Twin-prism separator, 64
U Viscocanalostomy, 223
Ultrasound biomicroscopy (UBM),
48–63, 217, 268, 271–272,
277
anterior chamber angle imaging
with, 274
characteristics o , 49t
o same eye, 281
usage o , 48
Unilateral optic nerve damage, 189
Unilateral primary congenital glaucoma,
153, 155
Unoprostone isopropyl, 305t
Uveal meshwork, def ned, 34
Uveitic glaucoma, 208–245, 210t
closed-angle mechanisms in,
214–215, 215 –216
diagnosis o , 217, 218
epidemiology o , 209
etiology o , 209–210
glaucomatous optic nerve injury, 209
management o , 219–221
medical therapy or, 220–221
open-angle mechanisms in, 210–213
postoperative complications o , 222
treatment, glaucoma drainage
devices in, 222
and vision loss, 209
Uveitis, 40, 42 , 208
anterior, 210t
granulomatous, 240, 243
granulomatous anterior, 220
incidence o , 209
intermediate, 210t
pediatric, 237
posterior, 210t
treatment, corticosteroids and,
209–210, 212
Uveitis–glaucoma–hyphema (UGH)
syndrome, 240
V Zentmeyer’s line, 199
Valacyclovir, 233
Van Herick-Sha er technique, 25, 28
INDEX 4 4 5
or angle depth estimation, 29
Vannas scissor, 352, 424
usage o , 353
Variable corneal compensation (VCC),
102
Vascular dysregulation, 136
Venereal Disease Research Lab
(VDRL), 236
Venous hypertension, 293
Vicryl suture, 380
Vicryl, usage o , 331
Visual evoked response imaging system
(VERIS), 95
Visual f eld de ects, 83 , 131
Visual f eld index (VFI), 89, 89
limitations o , 89
role o , 89
usage o , 89
Visual f eld testing, 122
Visually evoked cortical potential
(VECP), 97, 98
limitations o , 97
role o , 97
usage o , 97
Visual threshold, 122
Vogt-Koyanagi-Harada syndrome,
215, 243
Von Hippel’s corneal ulcer, 164
Von Recklinghausen’s
neurof bromatosis, 172
W
Watertight conjunctival closure, 374
Weill-Marchesani syndrome, 169
characteristics o , 169
Welch-Allyn ophthalmoscopes, 183
Westco scissors, 352
usage o , 331
Wilms’ tumor, 162
World Glaucoma Association Meeting,
136
Wound healing, actors in uence, 412t
X
Xylocaine 2%, 353
Z
Zeiss goniolens, 31
Zeiss lens, 25