OPHTHALMOLOGY

CLASSIFICATION OF GLAUCOMAS - denotes age of onset rather than

underlying mechanism
- e.g.:

 CONGENITAL GLAUCOMA
 PRIMARY CONGENITAL
 GLAUCOMA PRIMARY
INFANTILE GLAUCOMA or
JUVENILE GLAUCOMA
 JUVENILE OPEN ANGLE
GLAUCOMA or
SUMMARY DEVELOPMENTAL
• Rationale GLAUCOMA
• Childhood Glaucomas • more difficult since ONH is impossible to
• AAO Classification evaluate due to CORNEAL OPACITY
• Medical Management of Glaucoma • infants and young children develop OCULAR
• Lasers in Glaucoma ENLARGEMENT (DukeElder)
• Surgery • use of ELEVATED IOP as surrogate for
BACKGROUND diagnosis
o children can have elevated IOP
• Began with the term,“BUPHTHALMOS” without developing GON
used to describe 2ndary effect o (OCULAR HYPERTENSION or
of elevated IOP GLAUCOMA SUSPECT)

• Divided into:
1. SIMPLE BUPHTHALMOS
2. BUPHTHALMOS ASSOCIATED WITH
DEVELOPMENTAL ANOMALIES
CLASSIFICATION OF CHILDHOOD
• Evolved into HOSKINS classification divided into: GLAUCOMAS
1. TRABECULAR MESHWORK • CHILDHOOD GLAUCOMA RESEARCH
2. IRIS NETWORK (CGRN)
3. CORNEA o developed international classification
system
• SHAFFER-WEISS classification introduced: o represent end result of international
1. ISOLATED CONGENITAL (INFANTILE) collaboration on childhood glaucoma
GLAUCOMA
2. GLAUCOMAS ASSOCIATED WITH
CONGENITAL ANOMALIES
3. ACQUIRED GLAUCOMA

• WALTON proposed further modifications by listing

ALL DISORDERS associated with childhood
glaucoma

RATIONALE FOR CLASSIFYING THE

GLAUCOMAS
 CLASSIFICATION OF CHILDHOOD
GLAUCOMAS
• OVERLAPPING and VARIABLY • DEFINITION of childhood glaucoma is:
DEFINED NOMENCLATURE ✔ IOP related damage to the eye
 OPHTHALMOLOGY
✔ Covers glaucoma occuring at any point in
childhood
✔ Classification can evolve with ongoing
evaluation
✔Child with bilateral glaucoma can carry a
different diagnosis for each eye
• DEFINITION of TERMS:
1. CHILDHOOD AGE (based on national
criteria)
• US : younger than 18 years
• EU, UK, UNICEF : 16 years or
younger
2. GLAUCOMA :
• IOP-related damage to the eye
• at least 2 criteria required for
diagnosis:
• IOP > 21 mmHg; investigator
discretion if exam under
anesthesia data alone due to
variable effects of anesthesia on
all methods of IOP assessment
• OPTIC DISC CUPPING:
progressive increase in CDR, CD
asymmetry of ≥ 0.2 when the
optic discs are similar size, or
focal rim thinning
• CORNEAL FINDINGS: Haab
Striae or corneal diameter ≥ 11
mm in newborn, > 12 mm in
child < 1 year, or > 13 mm any
age
• PROGRESSIVE MYOPIA:
myopic shift, or an increase in
ocular dimensions out of keeping
with normal growth
• REPRODUCIBLE VF DEFECT:
consistent with GON with no
other observable reason for defect
3. GLAUCOMA SUSPECT
• no IOP-related damage to the
eye; at least 1 of the following
criteria:
• IOP > 21 mmHg on 2 separate
occasions
• SUSPICIOUS OPTIC DISC
APPEARANCE: increase CDR
for size of optic disc •
• SUSPICIOUS VF FOR
GLAUCOMA
• INCREASED CORNEAL
DIAMETER OR AXIAL
LENGTH (in setting of normal
IOP)
INTERNATIONAL CLASSIFICATION OF
CHILDHOOD GLAUCOMA
 PRIMARY CHILDHOOD GLAUCOMA
(PCG)
o Isolated angle anomalies, +/- mild
congenital iris anomalies
o Meets glaucoma definition, usually
with ocular enlargement
o Subcategories based on age at onset:
 NEONATAL or NEWBORN : 0-1
month
 INFANTILE : 1-24 months
 LATE-ONSET (RECOGNIZED)
: > 2 years
o Spontaneously arrested cases with
normal IOP but with typical signs of
PCG can be classified as PCG
 JUVENILE OPEN-ANGLE GLAUCOMA
(JOAG)
o No ocular enlargement
o No congenital ocular anomalies or
syndromes
o Open angle, normal appearance
o Meets glaucoma definition
 SECONDARY CHILDHOOD GLAUCOMA
o GLAUCOMA ASSOCIATED W/
NON-ACQUIRED OCULAR
ANOMALIES
 includes conditions of
predominantly ocular
anomalies present at birth that
may or may not be associated
with systemic signs
 Meets glaucoma definition
 List of common ocular
anomalies:
 Axenfeld Rieger anomaly
 Peters anomaly
 Microphthalmos
 Ectropion uvae
 Persistent fetal vasulature
 Oculodermal melanocytosis
 Mircorcronea
 Ectopia lentis
 Congenital iris hypoplasia
 Posterior polymorphous
dystrophy
 Aniridia
o GLAUCOMA ASSOCIATED W/ NON-
ACQUIRED SYSTEMIC ANOMALIES
 OPHTHALMOLOGY
 Includes conditions predominantly of  Open Angle Glaucoma : ≥ 50%
systemic disease present at birth that may open OR
be associated with ocular signs  Angle Closure Glaucoma : < 50%
 Meets glaucoma definition open or acute angle closure
 Glaucoma assoicated with nonaquired
systemic disease / syndrome: CLASSIFICATIONS OF THE GLAUCOMAS
 Trisomy 21 o PRIMARY GLAUCOMA
 Connective tissue disorders
 Marfan Syndrome  not associated with known ocular or
 Weil-Marchesani Syndrome systemic disorder that causes
 Stickler Syndrome
increased resistance to aqueous
 Metabolic disorders
 Homocystinuria Lowe Syndrome outflow
 Mucopolysaccharidoses  usually affects both eyes
 Phacomatoses
 Rubinstein-Taybi
 may be inherited
 Congenital Rubella
 Klippel Trenaurnay Weber o SECONDARY GLAUCOMA
 Neurofibromatoses
 associated with known ocular or
o GLAUCOMA ASSOCIATED W/
systemic disorders responsible for
ACQUIRED CONDITION
increased resistance to aqueous
 Meets glaucoma definition after
outflow
acquired condition is recognized
 often unilateral
 Glaucoma developing after cataract
 familial occurrence less common
surgery is EXCLUDED from this
NORMAL AQUEOUS
NORMAL AQUEOUS OUTFLOW
OUTFLOWcategory to highlight its
frequency and differences from other
conditions in the acquired category
 Based on Gonioscopy results:
 Open Angle Glaucoma : ≥ 50%
open OR
 Angle Closure Glaucoma : < 50%
open or acute angle closure
 List of common acquired conditions:
 Uveitis
 Trauma
 Steroid-indued Tumors
 Retinopathy of Prematurity
 Prior ocular surgery other than cataract
o GLAUCOMA FOLLOWING CATARACT
SURGERY
 Meets glaucoma definition ONLY
after cataract surgery is perfomed and CLASSIFICATIONS OF THE GLAUCOMAS
is subdivided into 3 catergories: o OPEN ANGLE GLAUCOMA
 CONGENITAL 1. PRIMARY OPEN ANGLE GLAUCOMA
IDIOPATHIC CATARACT 2. NORMAL TENSION GLAUCOMA
 CONGENITAL CATARACT 3. JUVENILE OPEN ANGLE GLAUCOMA
ASSOCIATED WITH 4. GLAUCOMA SUSPECT
OCULAR ANOMALIES, 5. SECONDARY OPEN ANGLE
NO PREVIOUS GLAUCOMA
GLAUCOMA
 ACQUIRED CATARACT,
NO PREVIOUS
GLAUCOMA

 Based on Gonioscopy results:

 OPHTHALMOLOGY
and consquently, production
of angiogenesis factors
(VEGF)
• release of VEGF leads to
formation of new vessels at
the pupillary border and iris
surface (NVI) and over the iris
angle (NVA)
 ultimately leads to formation of
FIBROVASCULAR
o ANGLE CLOSURE GLAUCOMA MEMBRANES which obstruct the
1. PRIMARY ACG WITH RELATIVE TM ->Secondary OAG
PUPILLARY BLOCK  as disease progresses, membranes
2. ACUTE ANGLE CLOSURE mature and contract, which tents
3. SUBACUTE ACG (INTERMITTENT the iris toward the TM resulting in
ANGLE CLOSURE) PERIPHERAL ANTERIOR
4. CHRONIC ANGLE CLOSURE SYNECHIAE -> Secondary ACG
5. SECONDARY ACG WITH PUPILARY
BLOCK
6. SECONDARY ACG W/O
PUPILLARY BLOCK
7. PLATEAU IRIS SYNDROME

o Based on MECHANISM OF OUTFLOW

OBSTRUCTION
 TRABECULAR
 RBC (Ghost Cell Glaucoma)
 Macrophages (Phacolytic
Glaucoma)
 Neoplastic cells (Neoplastic
Tumors)
 Pigment particles (Exfoliation
CLASSIFICATIONS OF THE GLAUCOMAS Glaucoma)
o Based on MECHANISM OF OUTFLOW  Protein (Uvietis, Lens-
OBSTRUCTION Induced Glaucoma)
 PRETRABECULAR (Membrane  Viscoelastic agents
Overgrowth)  Vitreous
- Fibrovascular membrane  Others (Steroid induced,
(Neovascular Glaucoma) Angle Recession, IOFB)
- Descemet-like membrane (ICE, PPD, o EXFOLIATION GLAUCOMA
trauma)  a.k.a. PSEUDOEXFOLIATION
- Epithelial overgrowth SYNDROME (PEX)
- Fibrous overgrowth  age-related systemic disease
- Inflammatory membrane (Fuch’s manifesting primarily in the eyes
heterochromic iridocyclitis)  accumulation of microscopic granular
o NEOVASCULAR GLAUCOMA AMYLOID PROTEIN fibers
 RETINAL ISCHEMIA (most (abnormalities of basement membrane
common mechanism) in epithelial cells)
• causes anterior segment  mainly idiopathic
changes (NVG / NVA) –  more prevalent in F > M
• predisposing conditions (PDR,  > 70 years of age
CRVO) cause retinal hypoxia  Scandinavia
 OPHTHALMOLOGY
 no specific ocular symptoms
 granular flakes of amyloid protein
resemble “DANDRUFF” and is visible on
slitlamp examination of the eye and can be
enmeshed in TM and block aqueous
outflow -> INCREASE IOP
o Based on MECHANISM OF OUTFLOW
OBSTRUCTION
 POSTTRABECULAR
 Carotico-Cavernous Fistula / common ocular conditions
Cavernous Sinus Thrombosis
 Retrobulbar tumors
 Thyroid ophthalmopathy
 SVC syndrome
 Mediastinal tumors
 Sturge-Weber syndrome
 Familial episcleral venous pressure
elevation
o CAROTICO-CAVERNOUS FISTULA
 abnormal communication between
INTERNAL or EXTERNAL
CAROTID ARTERIES and the
CAVERNOUS SINUS
 lesions may be classified based on: -
 ETIOLOGY
(Traumatic vs. Spontaneous)
 VELOCITY OF BLOOD
FLOW
(High vs. Low)
 ANATOMY
(Direct vs. Dural ; Internal
Carotid vs. External Carotid)
 CCF occur because of traumatic or
spontaneous RENTS in the walls of
the intracavernous ICA or its branches
- results in short-circuiting of arterial
blood into venous system
 2 TYPES OF ARTERIAL
DIVERSIONS:
 DIRECT CCF represents 70-
90% of all fistulas are
characterized by a direct
connection betweein
intracavernous segment of
ICA and the cavernous sinus
 DURAL AV SHUNTS
abnormal communications
between cavernous sinus and
one or more meningeal
branches of the ICA, ECA, or
both
 nearly all patients with direct CCF
experience progressive OCULAR
COMPLICATIONS if fistula is left
untreated
 INCREASING PROPTOSIS
 CONJUNCTIVAL
CHEMOSIS
 VISUAL LOSS
 CRVO, SECONDARY
GLAUCOMA •
 masquerade as conjunctivitis or other
 mortality is extremely rare due to
prompt diagnosis
ANGLE CLOSURE GLAUCOMA MECHANISMS
o ANTERIOR (“pulling”)
 Contracture of Membranes (NVG,
ICE, PPD)
 Consolidation of inflammatory
products
o POSTERIOR (“pushing”)
 With Pupillary Block
- Lens-induced glaucoma,
Pseudophakia, Posterior synechiae
 Without Pupillary Block
- Ciliary block (Malignant) glaucoma,
Phacomorphic glaucoma,
Retinoblastoma, iris cysts, anterior
rotation of ciliiary body (post PRP,
SB, CRVO)
DEVELOPMENTAL ANOMALIES OF THE
ANGLE
o INCOMPLETE DEVELOPMENT OF TM
 Congenital Glaucoma
 Axenfeld Rieger Syndrome
 Peters Anomaly
 Glaucomas associated with other
developmental anomalies
 OPHTHALMOLOGY
o IRIDOCORNEAL ADHESIONS and surgical treatment and decreased
 Axenfeld Rieger Syndrome disease progression to less than 15%
 Aniridia

o FACTORS TO CONSIDER IN COMPUTING

COMBINED MECHANISM GLAUCOMA FOR IOP:
o Combination of 2 or more forms of glaucoma 1. Structural damage : Optic Disc and
present either SEQUENTIALLY or RNFL
SIMULTANEOUSLY 2. Functional damage on White on
o IOP elevation can occur as a result of White Perimetry
EITHER or BOTH of the following: 3. Baseline IOP at which damage
1. The INTRINSIC RESISTANCE of occurred
the TM to aqueous outflow in open 4. Age
angle glaucoma; 5. Presence of additional Risk Factors
2. The DIRECT ANATOMIC o INDIVIDUALIZE TARGET IOP (Jample, et
OBSTRUCTION of the filtering al)
network by synechiae in angle closure  1 – ( Reference IOP + Visual Field
glaucoma score/ 100) x Reference IOP
o Treatment is MODIFIED based on the
proportion of open to closed angles and the RULEs OF THUMB
etiology of the angle closure component o Reduce IOP by :
 At least 25% for MILD Glaucoma
MEDICAL MANAGEMENT  At least 30% for MODERATE
o TREATMENT GOALS Glaucoma
1. To achieve target IOP and reduce IOP  > 40% in SEVERE Glaucoma
fluctuations with minimal possible o The higher the IOP, the larger the reduction
medications; required.
2. To administer glaucoma medications which o Target IOP is not fixed, not static.
have the least side effects on the quality of life o No sure fire method of estimating the Target
of the patient; IOP.
3. To achieve this treatment at an affordable o We cannot be certain as to what aspect of the
and sustainable cost for the patient; IOP actually causes the damage (peak IOP,
4. Monitor the structure and function of the fluctuations, short spikes, etc.)
optic nerve for further damage and adjust the
target IOP to a lower level is deterioration
occurs;
5. To treat non-IOP dependent systemic
factors (i.e., systemic hypertension, etc.) which
may contribute to the development and
worsening of GON
6. To educate and involve the patient (and
family) in the management of the disease
process.
SETTING OF TARGET IOP
o EARLY MANIFEST GLAUCOMA
TREATMENT STUDY (EMGTS)
 Showed that IOP reduction by at least
25% reduced progression from 62% to
45% in the treated group compared to
the untreated group
o COLLABORATIVE INITIAL GLAUCOMA
TREATMENT STUDY (CIGTS)
 Loweriing the IOP by 35%
demonstrated equivalence of medical
 OPHTHALMOLOGY
LASERS IN GLAUCOMA
LASER PERIPHERAL IRIDOTOMY
o LPI therapy of choice for PUPILLARY
BLOCK with ANGLE CLOSURE (relative or
absolute)
o Q switched NEODYNIUM (ND)-YAG
LASER causes tissue disruption by OPTICAL
BREAKDOWN and RESULTING
SHOCKWAVE
o Introduction of Nd:Yag laser for LPI was
advantageous:
 less total energy required
 highly effective with light-colored
eyes
 associated with lower closure rate
o Complications related to to use of higher laser
energy: -
 transient BOV (glare, ghost images)
 iris hermorrhage
 focal coreal opacity/corneal epithelial
disturbance
 transient rise in IOP
 focal cataract
SELECTIVE LASER TRABECULOPLASTY
o LESS DESTRUCTION/DAMAGE seen in
SLT is due to its ability to SELECTIVELY
PHOTOLYSE pigmented TM cells without
inducing photocoagulation and collateral
damage to nonpigmented cells
o SHORTER ( 3 nsec.) PULSE DURATION
than the thermal relaxation time of melanin (1
msec.)
o exact mechanism of action of SLT to explain
increased aqueous facility:
- MECHANICAL THEORY: tissue
contraction and scar formation
-CELLULAR THEORY:
increased cell division and repopulate
ion of TM
- BIOCHEMICAL THEORY:
release of chemical mediators (IL-1,
TNF) which stimulate remodelling of
EC matrix and increased permeability
GLAUCOMA LASER TRIAL (GLT)
o multicenter RCT published in 1990 involving
271 patients
o designed to asess efficacy and safety of argon
laser trabeculoplasty (ALT) as an alternative
treatment for controlling IOP in patients with
newly diagnosed, previously untreated POG
o each patient had one eye randomly assigned to
ALT (LF eye) and the other assigned to
Timolol Maleate 0.5% medication (MF eye)
o LF eyes had lower IOP and better VF and
ONH status than MF eyes
o LF eyes had 1.2 mmHg IOP reduction and 0.6
dB greater improvement in VF and slightly
less deterioration in optic disk compared to
MF eyes from entry into the GLT
ARTIFICIAL DRAINAGE SHUNTS
TUBE VERSUS TRABECULECTOMY
• multi center RCT landmark study published
in May 2012
• report 5 year treat,emt outcomes
• 17 clinical centers involving patients 18-85
years (212 eyes)
• compared results of surgeries performed on
eyes of patients who already had eye surgery
(trabeculectomy and/or cataract extraction)
with uncontrolled glaucoma with IOP 18-40
mmHg on MTMT versus patients where the
Baerveldt tube was used
• IOP after surgery (1 and 5 years)
• CONCLUSIONS:
 OPHTHALMOLOGY
- IOP after 1 year near identical (12.4
mmHg for trabeculectomy and 12.7
mmHg for Baerveldt shunt)
- similarity maintained after 5 years
- number of medis required for IOP
control was still similar

• MAJOR DIFFERENCES:
- >50% of trabeculectomies were still
controlling IOP compared to >70% of tubes
were still successful
- 3 times as many trabeculectomies
required re-operation
- Tube and Trabeculectomy fails at
~10% per year
• Tube shunt surgery had a higher success rate
than Trabeculectomy during 5 years of follow-
up