GLAUCOMA

Intraocular pressure

 The IOP is determined by the balance between the rate of

aqueous secretion and aqueous outflow.

 The latter is in turn related to the resistance encountered in

the outflow channels and to the level of episcleral venous
pressure.

 The rate of aqueous outflow is proportional to the

difference between the intraocular and episcleral venous
pressure.
 Intraocular pressure
Concept of normal intraocular pressure
 The distribution of IOP within the general population has a
range of 11–21 mmHg.

 Although there is no absolute pathological point, 21 mmHg is

considered the upper limit of normal and levels above this are
viewed with suspicion.

 However, in some patients glaucomatous damage occurs with

IOPs less than 21 mmHg (normal-tension or normal-pressure
glaucoma) whilst others remain unscathed with IOPs up to 30
mmHg (ocular hypertension).

 Although the actual level of IOP is important in the development

of glaucomatous damage, other factors are also significant.
 Intraocular pressure
Fluctuation
 Normal IOP varies with the time of day, heartbeat, blood
pressure level and respiration.
 The diurnal pattern varies, with a tendency to be higher in
the morning and lower in the afternoon and evening.
 Normal eyes manifest a mean diurnal pressure variation of
5 mmHg; ocular hypertensive or glaucomatous eyes,
however, exhibit a wider fluctuation.
 A single normal reading, particularly if taken during late
afternoon, may therefore be misleading and it may be
necessary to take several readings at different times of day
(‘phasing’).
 In clinical practice phasing during the morning hours may
be sufficient because 80% of patients peak between 8.00
a.m. and noon.
 Overview of glaucoma
 Definition
 It is difficult to define glaucoma precisely, as it
encompasses a diverse group of disorders.

 All forms of the disease have in common a potentially

progressive and characteristic optic neuropathy which is
associated with visual field loss as damage progresses, and
in which intraocular pressure is usually a key modifying
factor.

 On a molecular level, glaucoma of diverse aetiology is

linked by the presence of endothelial leucocyte adhesion
molecule-1 (ELAM-1), which indicates activation of a stress
response in trabecular meshwork cells.
 Overview of glaucoma
Epidemiology
 Glaucoma affects up to 2% of those over the age of 40
years globally, and up to 10% over the age of 80

 50% may be undiagnosed

 In a population of European or African ethnic origin,

primary open-angle glaucoma (POAG) is the most common
form

 On a worldwide basis, primary angle-closure constitutes up

to half of cases, with particularly high prevalence in
individuals of Far Eastern descent.
 Overview of glaucoma
Classification
 Glaucoma may be congenital (developmental) or acquired.

 Sub-classification into open-angle and angle-closure types

is based on the mechanism by which aqueous outflow is
impaired with respect to the anterior chamber angle
configuration.

 Distinction is also made between primary and secondary

glaucoma; in the latter a recognizable ocular or non-ocular
disorder contributes to elevation of IOP.
 Tonometry
 Goldmann tonometry
 Goldmann applanation tonometry (GAT) is
based on the Imbert–Fick principle, which
states that for an ideal, dry, thin-walled
sphere, the pressure (P) inside the sphere
equals the force (F) necessary to flatten its
surface divided by the area (A) of flattening
(i.e. P = F/A).
 Theoretically, average corneal rigidity and the
capillary attraction of the tear meniscus cancel
each other out when the flattened area has the
3.06 mm diameter contact surface of the
Goldmann prism (A ( ), which is applied to the
cornea with a variable amount of measurable
force from which the IOP is deduced.
 The Goldmann tonometer is shown in Figure B.
Disposable tonometer prisms and tonometer
caps have been introduced to counter fears of
infection from reusable prisms.
 Tonometry

 Technique
 a. The patient is positioned at the slit-lamp with the forehead firmly
against the headrest.
b. Topical anaesthetic and fluorescein are instilled into the conjunctival
sac.
c. With the cobalt blue filter and the brightest beam projected obliquely at
the prism, the prism is centred in front of the apex of the cornea.
d. The dial is preset between 1 and 2 (i.e. 10–20 mmHg).
e. The prism is advanced until it just touches the apex of the cornea (A
( ).
f. Viewing is switched to the ocular of the slit-lamp.
g. A pattern of two semicircle mires will be seen, one above and one below
the horizontal midline, which represent the fluorescein-stained tear film
touching the upper and lower outer halves of the prism.
h. The dial on the tonometer is rotated to align the inner margins of the
semicircles (B
( , right).
i. The reading on the dial, multiplied by 10, gives the IOP.
 Tonometry
 Other types of tonometry
 1. Pneumotonometers are also
based on the principle of
applanation but, instead of using a
prism, the central part of the
cornea is flattened by a jet of air;
non-portable (Fig. 1) or portable (A(
).

 2. Reichert ocular response

analyzer is a recently-developed
form of pneumotonometer which
measures IOP.

 3. Dynamic contour tonometry

(‘Pascal’) uses a solid state sensor
and a corneal contour-matching
surface to measure IOP.
 Tonometry
 4. Perkins applanation tonometer uses a
Goldmann prism adapted to a small light
source. It is hand-held (B
( ), and can therefore
be used in bed-bound or anaesthetized
patients.

 5. Tono-Pen® is a hand-held, self-contained,

battery powered, portable, miniaturized
electronic contact tonometer (C
( ).

 6. iCare® tonometer is a recently developed

small hand-held device based on a new
measuring principle, rebound or dynamic
tonometry, in which a very light probe makes
momentary contact with the cornea. The
instrument can be used for self-monitoring (D
( )
and screening in the community.

 7. Schiotz tonometer uses the principle of

indentation tonometry, in which the extent of
corneal indentation by a plunger of known
weight is measured.
 Gonioscopy

 1. Gonioscopy is a method of evaluating the anterior

chamber angle to provide information regarding the type of
glaucoma.

 It can also be utilized therapeutically for procedures such

as laser trabeculoplasty and goniotomy.

 2. Other means of anterior chamber angle assessment such

as high-frequency ultrasound biomicroscopy (UBM), and
anterior segment optical coherence tomography (OCT),
offer advantages in some aspects of angle analysis and may
be used to supplement visual gonioscopic findings.
 Identification of angle structures
 1. Schwalbe line is the most anterior structure, appearing as an
irregular opaque line. Anatomically it demarcates the peripheral
termination of Descemet membrane and the anterior limit of the
trabeculum
 2. The corneal wedge is useful in locating an inconspicuous
Schwalbe line
 3. The trabeculum extends from Schwalbe line to the scleral spur
 4. Schlemm canal may be identified in the non-pigmented angle as a
slightly darker line deep to the posterior trabeculum
 5. The scleral spur is the most anterior projection of the sclera
 6. The ciliary body stands out just behind the scleral spur
 7. Iris processes are small extensions of the anterior surface of the
iris
 8. Blood vessels running in a radial pattern at the base of the angle
recess are often seen in normal eyes. Pathological blood vessels run
randomly in various directions. As a general principle, any blood
vessel that crosses the scleral spur onto the trabecular meshwork is
abnormal.
 Evaluation of the optic nerve
head
 Normal optic nerve head
 Neuroretinal rim
 The neuroretinal rim (NRR) is the
tissue between the outer edge of
the cup and the optic disc margin.
The normal rim has an orange or
pink colour and a characteristic
configuration in most healthy eyes.

 Optic disc size

 Optic disc size is important in
deciding if a cup-disc (C/D) ratio is
normal. Normal median vertical
diameter for non-glaucomatous
discs is 1.50 mm in a Caucasian
population.
 Evaluation of the optic nerve
head
 Cup–disc ratio
 The C/D ratio indicates the
diameter of the cup
expressed as a fraction of
the diameter of the disc;
the vertical rather than the
horizontal ratio is generally
used in clinical practice.

Normal discs. (A) Small disc with a low

C/D ratio; (B) large disc with a higher
C/D ratio
Specific subtypes of glaucomatous damage. (A) Type 1 – focal
ischaemic; (B) type 2 – myopic; (C) type 3 – senile sclerotic;
(D) type 4 – concentrically enlarging
Retinal nerve fibre layer defects. (A) Superotemporal wedge-
shaped defect; (B) same eye seen with a green filter
 Perimetry
 The visual field can be represented
as a three-dimensional structure
akin to a hill of increasing sensitivity
(A).
 The outer aspect extends
approximately 50° superiorly, 60°
nasally, 70° inferiorly and 90°
temporally.
 Visual acuity is sharpest at the very
top of the hill (i.e. the fovea) and
then declines progressively towards
the periphery, the nasal slope being
steeper than the temporal. The
‘bottomless pit’ of the blind spot is
located temporally between 10° and
20°, slightly below the horizontal.
 A scotoma is an area of reduced
(‘relative’) or total (‘absolute’) loss
of vision which is surrounded by a
seeing area.
 Imaging in glaucoma
 1. Stereo disc photography
 2. Confocal scanning laser tomography
 3. Scanning laser polarimetry
 4. Optical coherence tomography (OCT) has become a
routine part of the management of macular and other
retinal disease. The following imaging strategies are
applicable to glaucoma:
a. Peripapillary retinal nerve fibre layer (RNFL). This
involves the acquisition of a circular scan of diameter 3.4
mm of the retina around the optic nerve head. Retinal
thickness is compared with normals. Sensitivity and
specificity are around 90%.
b. Optic nerve head (ONH). Radial cross-sectional
scans permit an objective and repeatable assessment of
disc morphology, with reasonable discriminatory value.
Primary open-angle glaucoma
Definition
 Primary open-angle glaucoma (POAG), also referred to as
chronic simple glaucoma, is a generally bilateral disease of
adult onset characterized by:
• An IOP >21 mmHg.
• Glaucomatous optic nerve damage.
• An open anterior chamber angle.
• Characteristic visual field loss as damage progresses.
• Absence of signs of secondary glaucoma or a non-
glaucomatous cause for the optic neuropathy.
POAG is the most prevalent type of glaucoma in individuals
of European and African ethnic origin. It affects both sexes
equally.
 Primary open-angle glaucoma
Risk factors

 1. IOP. The higher the IOP, the greater the likelihood of glaucoma.
 2. Age. It is more common in older individuals.
 3. Race. It is significantly (perhaps four times) more common,
develops at an earlier age, and may be more difficult to control in
black individuals than in whites.
 4. Family history of POAG. First-degree relatives of patients with
POAG are at increased risk.
 5. Diabetes mellitus. Many studies suggest a correlation between
diabetes and POAG.
 6. Myopia is associated with an increased incidence of POAG and
myopic eyes may be more susceptible to glaucomatous damage.
 7. Vascular disease. A range of systemic conditions linked to
vascular compromise may be associated: systemic hypertension,
cardiovascular disease, diabetes and vasospastic conditions such
as migraine. Poor ocular perfusion may be a risk factor for
glaucoma progression.
Primary open-angle glaucoma
Genetics
 Mutations at 15 loci in the human genome have so far been
identified as associated with POAG and are designated
primary open angle glaucoma-1A (GLC1A) to GLC1O.

 Four susceptible genes have been identified: the MYOC

gene (chromosome 1q21-q31), coding for the glycoprotein
myocilin that is found in the trabecular meshwork and other
ocular tissues, the OPTN gene on chromosome 10p, which
codes for optineurin, the WDR36 gene on chromosome
5q22, and the NTF4 gene on chromosome 19q13.3.

 Among them MYOC is the most frequently mutated gene in

POAG.
 Primary open-angle glaucoma
Steroid responsiveness
 A proportion of the population develops an elevation of IOP in
response to a course of topical steroid.

 This tendency is more marked in patients with POAG and their

close relatives.

 Intra- and periocular steroid administration, including

periocular application of steroid skin cream and nasal
administration, are also prone to elevate IOP. Systemic
steroids are much less prone to cause elevation of IOP, but
substantial, probably dose-dependent, rises can occur and
some authorities have advocated screening for all patients on
systemic steroids, perhaps those on dexamethasone in
particular.

 The precise mechanism of the ‘steroid response’ is uncertain,

but it may be mediated by an increase in trabecular meshwork
cell myocilin production.
 Primary open-angle glaucoma
Pathogenesis of glaucomatous optic neuropathy
 Retinal ganglion cell death in glaucoma occurs
predominantly through apoptosis (programmed cell death)
rather than necrosis.

 One or both of the following mechanisms may be involved:

 1. Direct mechanical damage to retinal nerve fibres at the
optic nerve head, perhaps as they pass through the lamina
cribrosa.
 2. Ischaemic damage, possibly due partly to compression of
blood vessels supplying the optic nerve head.
Insults via both mechanisms might lead to reduction in
axoplasmic flow, interference with the delivery of nutrients
or removal of metabolic products, deprivation of neuronal
growth factors, oxidative injury and the initiation of
immune-mediated damage.
 Primary open-angle glaucoma
Screening
 Universal population screening for glaucoma has not been
demonstrated to be cost-effective, and current practice
restricts screening to high-risk groups, such as older
individuals, those with a history of POAG in a close family
member over the age of 40, and people of black ethnicity.

 Population screening with tonometry alone is

unsatisfactory, since it will label as normal a significant
number of cases with other features of POAG such as
cupping and visual field loss.

 It is therefore prudent for routine screening eye

examinations to include visual field examination as well as
tonometry and ophthalmoscopy.
 Primary open-angle glaucoma
Diagnosis
 History
 1. Visual symptoms will usually be absent, unless damage is advanced.
Sometimes symptomatic central field defects may occur at an early
stage, in the presence of a relatively normal peripheral field.

 2. Previous ophthalmic history. Specific enquiry should be made about:

• Refractive status as myopia carries an increased risk of POAG, and
hypermetropia of primary angle-closure glaucoma (PACG).
• Causes of secondary glaucoma such as ocular trauma or
inflammation; previous eye surgery, including refractive surgery may
affect IOP reading.

 3. Family history
• POAG or related conditions such as OHT.
• Other ocular disease in family members.
 Primary open-angle glaucoma
 4. Past medical history. Asking specifically about the following:
• Asthma, heart failure or block, peripheral vascular disease:
contraindications to the use of beta-blockers.
• Head injury, intracranial pathology including stroke that may
cause optic atrophy or visual field defects.
• Vasospasm: migraine and Raynaud phenomenon.
• Diabetes, systemic hypertension and cardiovascular disease
may increase the risk of POAG.

 5. Current medication
• Steroids including skin cream and inhalants.
• Oral beta-blockers may lower IOP.

 6. Social history including smoking and alcohol intake, especially

if toxic/nutritional optic neuropathy is suspected.

 7. Allergies especially to any drugs likely to be used in glaucoma

treatment.
 Primary open-angle glaucoma
Examination
 1. Visual acuity is likely to be normal except in advanced glaucoma.
 2. Pupils. Exclude a relative afferent pupillary defect (RAPD); if
absent then subsequently develops - an indicator of substantial
progression.
 3. Colour vision assessment such as Ishihara chart testing if there
is any suggestion of an optic neuropathy other than glaucoma.
 4. Slit-lamp examination. Exclude features of secondary glaucomas
such as pigmentary and pseudoexfoliative.
 5. Tonometry, noting the time of day.
 6. Pachymetry for CCT.
 7. Gonioscopy.
 8. Optic disc examination should always be performed with the
pupils dilated, provided gonioscopy does not show critically narrow
angles.
 9. Perimetry should usually be performed prior to clinical
examination.
 10. Optic disc or peripapillary RNFL imaging.
 Primary open-angle glaucoma
Visual field defects
 1. The earliest changes suggestive
of glaucoma consist of increased
variability of responses in areas
that subsequently develop defects.
Alternatively there may be slight
asymmetry between the two eyes.

 2. Paracentral, small, relatively

steep depressions (A( and B)
constitute approximately 70% of
all early glaucomatous field
defects. Since the defects respect
the distribution of the retinal nerve
fibre layer they terminate at the
horizontal midline; defects above
and below the horizontal therefore
are not aligned with each other.
 Primary open-angle glaucoma
 3. Nasal (Rønne) step
represents a difference in
sensitivity above and below
the horizontal midline in the
nasal field.

 It is a common finding usually

associated with other defects (
A and B).
 Primary open-angle glaucoma
 4. Arcuate-shaped defects develop
as a result of coalescence of
paracentral scotomas.

 They typically develop between 10°

and 20° of fixation in areas that
constitute downward or, more
commonly, upward extensions from
the blind spot around fixation
(Bjerrum area).

 With time, they tend to elongate

circumferentially along the
distribution of arcuate nerve fibres
(Seidel scotoma) and may
eventually connect with the blind
spot (arcuate scotoma) reaching to
within 5° of fixation nasally (A
( and B
).
 Primary open-angle glaucoma
 5. Enlargement of scotomas due to
damage to adjacent fibres.
 6. Deepening of scotomas and
development of fresh defects.
 7. A ring scotoma develops when
arcuate defects in upper and lower
halves of the visual field join.
Misalignment between the two
often preserves the nasal step (
A and B).
 8. End-stage changes are
characterized by a small island of
central vision typically
accompanied by a temporal island.
The temporal island is usually
extinguished before the central.
 Management of POAG
 The primary aim of treatment is to prevent
functional impairment of vision within the
patient's lifetime by slowing the rate of ganglion
cell loss closer to that of the normal population
(approximately 5000/year).

 Currently the only proven method of achieving

this is the lowering of IOP.
 Management of POAG
 Patient instruction
 An explanation should be offered concerning the nature of
the disease, and an explanatory booklet provided.
 The timing of medication use should be specified, and the
patient educated in the technique of eye drop instillation.
 At follow-up visits the patient's proficiency at instilling
drops should be checked.
 In order to maximize drug contact time with the anterior
segment and to minimize systemic absorption the patient
should be instructed either to perform lacrimal sac
occlusion (by applying fingertip pressure at the medial
canthus) or to close the eyes for about 3 minutes after
instillation.
 Common or severe potential adverse effects should be
explained at the commencement of treatment and their
occurrence enquired about at follow-up visits.
 Management of POAG
 Treatment goals
 1. Target pressure. It is assumed that the pre-treatment
level of IOP has damaged the optic nerve and will continue
to do so. An IOP level is identified below which further
damage is considered unlikely (‘target pressure’). This is
identified taking into account the severity of existing
damage, the level of IOP, CCT, the rapidity with which
damage occurred if known, as well as the age and general
health of the patient. Therapy should maintain the IOP at or
below the target level. If not achievable by conservative
modalities, a decision is made regarding whether to
proceed with surgery or to continue monitoring with an
above-target IOP.

 2. Proportional reduction. An alternative strategy is to aim

for a reduction in IOP by a certain percentage – often 30%
– and then monitor, aiming for a further reduction if
progression occurs.
 Management of POAG
 3. Monitoring of the optic nerve and visual fields is
performed.

 In the event of further damage the target IOP is reset at a

lower level.

 Although there is no ‘safe’ level, progression is uncommon if

the IOP is <16 mmHg. It appears that each 1 mmHg
reduction in IOP leads to a 10% reduction in the rate of
nerve fibre loss.

 Lower target pressures therefore tend to be set in patients

with advanced disease.
 Management of POAG
 Medical therapy
 1. Commencing medical therapy
• Any chosen drug should be used in its lowest concentration.

• Ideally the drug with the fewest potential side-effects should be

used.

• Initial treatment is usually with one drug, usually a prostaglandin

analogue or beta-blocker.

 2. Review
• The interval to review after starting medication is set
according to the individual patient, but is usually 4–8 weeks.
• Response to the drug is assessed against the target IOP.
• If the response is satisfactory subsequent assessment is
generally set for a further 3–6 months.
 Management of POAG
 2. Review
• If there has been little or no response the initial drug is
withdrawn and another substituted.
• If there has been an apparently incomplete response
another drug may be added or a fixed combination
substituted.
• When two separate drugs are used the patient should be
instructed to wait five minutes before instilling the second
drug to prevent washout of the first.
• Sometimes it may be worthwhile allowing a further month
or two of treatment before altering a regimen, as response
may improve over time.
• Poor compliance or inadequate drop instillation technique
should be considered as a cause of unsatisfactory response.
• When drops are administered in the morning, it is good
practice always to enquire about whether today's dose has
been used prior to the examination.
 Management of POAG
 3. Perimetry. If IOP control is good and glaucomatous
damage mild or moderate with no substantial threat to
central vision, annual perimetry is generally sufficient.

 4. Gonioscopy should be performed annually in most

patients because the anterior chamber angle tends to
narrow with age.

 5. Optic disc examination should be performed at

appropriate intervals. Serial imaging may also provide
useful additional information.
 Management of POAG
 6. Causes of treatment failure:

• Inappropriate target pressure.

• Poor compliance with therapy occurs in at least 25% of

patients.

• Wide fluctuations in IOP frequently occur in patients treated

medically, and may be associated with progression.

• Patients may deteriorate despite apparently good IOP control.

Causes include occult compliance failure, undetected diurnal
variation, and possibly impaired optic nerve perfusion. The
possibility of an alternative pathology, particularly a
compressive lesion, should always be considered.
 Management of POAG
 Laser trabeculoplasty
 In argon laser trabeculoplasty (ALT) or selective laser
trabeculoplasty (SLT), argon or Nd:YAG laser is applied to the
trabeculum to enhance aqueous outflow and lower IOP.
 The following are the main indications:
1. Intolerance of topical medication including allergy.
2. Failure of medical therapy, as a less aggressive treatment
measure than surgery.
3. Avoidance of polypharmacy, usually with more than two
preparations. In this situation laser therapy may be
considered as a substitute for an additional drug.
4. Avoidance of surgery, for example in: • Patients in whom
laser may defer the need for surgery beyond life expectancy.
• Patients in whom filtration surgery carries a poorer
prognosis.
5. Primary therapy in accordance with patient preference, or
in patients who are unable or unwilling to comply with
medical therapy.
 Management of POAG
 Surgery
 Trabeculectomy is the surgical procedure most commonly
performed for glaucoma.
 The following are the main indications:
1. Failed medical therapy when laser trabeculoplasty is likely
to be inadequate or inappropriate.
2. Intolerance of or allergy to medical therapy when
trabeculoplasty is inappropriate.
3. Avoidance of polypharmacy, though the option of using
three or more medications should be discussed with the
patient.
4. Progressive deterioration despite seemingly adequate IOP
control.
5. Primary therapy. Advanced disease requiring a very low
target pressure may achieve a superior long-term outcome
from early surgery.
6. Patient preference. Occasionally patients express a strong
desire to be free of the commitment to chronic medical
treatment.
 Management of POAG
 Prognosis
 The great majority of patients diagnosed with POAG will not
become blind in their lifetime, but the rate of glaucoma
progression varies considerably.

• Untreated, the mean time for progression to blindness has

been estimated at 20 years.

• Older 20 year follow-up data for functional blindness in

one eye showed that this occurred in 25% of patients, with
blindness in both eyes in 10%, but recent figures are
considerably lower, with about 15% of patients' worse eyes
becoming blind.
 Primary angle-closure
glaucoma
 Introduction
 Overview
 The term ‘angle-closure’ refers to occlusion of the trabecular
meshwork (TM) by the peripheral iris (iridotrabecular contact –
ITC), obstructing aqueous outflow.

 Angle-closure can be primary, when it occurs in an anatomically

predisposed eye, or secondary to another ocular condition.

 Primary angle-closure glaucoma may be responsible for up to

half of all cases of glaucoma globally, with a particularly high
prevalence in individuals of Far Eastern descent.

 It is typically associated with greater speed of progression and

visual morbidity than POAG.
 Primary angle-closure
glaucoma
 Mechanism
 The mechanisms involved in
angle-closure can be categorized
according to the anatomical
level (anterior to posterior) at
which causative forces act.

 1. Pupillary block
• Failure of aqueous flow
through the pupil (relative
pupillary block – A), leads to a
pressure differential between
the anterior and posterior
chambers, with resultant
anterior bowing of the iris (B
( )
and ITC (C).
• Iridotomy equalizes anterior
and posterior chamber pressure,
provided the TM remains
sufficiently functional.
 Primary angle-closure
glaucoma
 2. Non-pupillary block relating to
the iris • Specific anatomical
factors (anteriorly positioned
ciliary processes), and a thicker
or more anteriorly-positioned iris.
• An element of pupillary block is
invariably present, but angle-
closure is not fully relieved by
iridotomy. • Associated with a
deeper anterior chamber •
Plateau iris configuration is
characterized by a flat central iris
plane in association with normal
central anterior chamber depth.
The angle recess is very narrow, Ultrasound biomicroscopy in
with a sharp iris angulation over plateau iris configuration shows
anteriorly positioned and/or loss of the ciliary sulcus due to
orientated ciliary processes (Fig.). anterior position of the ciliary
processes
 Primary angle-closure
glaucoma
 3. Lens-induced angle-closure
• those cases in which a sudden change in lens volume and/or position
leads to an acute or subacute IOP rise.

• Usually rapid progression of lens intumescence (phacomorphic

glaucoma) or anterior lens subluxation.

• The lens contributes to angle-closure in categories 1 and 2 and virtually

all pupillary block can be said to have a phacomorphic element that
increases with age as the lens enlarges.
 4. Retrolenticular causes
• Posterior segment causes of secondary angle-closure.

 5. ‘Combined mechanism’ describes the combination of angle-closure and

open-angle elements.
 Primary angle-
closure glaucoma
 Risk factors
 1. Age • The average age at presentation is about 60
years and the prevalence increases thereafter.
• Non-pupillary block forms of primary angle-closure
tend to occur at a younger age.
 2. Gender. Females are more commonly affected than
males.
 3. Race. As discussed above.
 4. Family history. First degree relatives are at increased
risk.
 5. Refraction. Eyes with ‘pure’ pupillary block are
typically hypermetropic.
 6. Axial length
• Short eyes tend to have shallow anterior chambers
(Fig.).
• Nanophthalmos - very short eye with a proportionally
large lens are at particular risk.
 Primary angle-closure
glaucoma
 Diagnosis
 Symptoms
 • Most patients with angle-closure are asymptomatic,
including a majority of those with intermittently or
chronically elevated IOP.
• Some patients present acutely (congestive glaucoma) with
haloes around lights due to corneal oedema, ocular pain and
headache.
• Other patients may have intermittent milder symptoms of
blurring (‘smoke-filled room’) unassociated with pain .
 • Precipitating factors include watching television in a
darkened room, reading, pharmacological mydriasis, acute
emotional stress and rarely systemic medication:
parasympathetic antagonists or sympathetic agonists (e.g.
inhalers, motion sickness patches and cold remedies).
 Primary angle-closure
glaucoma
 Signs
 1. Chronic presentation
• VA is normal unless damage is advanced.
• Anterior chamber is shallower in pupillary block than non-
pupillary block.
• Optic nerve signs depend on severity of damage.
• IOP elevation may be only intermittent.
• Gonioscopic abnormalities are as described above.
• ‘Creeping’ angle-closure is characterized by a gradual
band-like anterior advance of the apparent insertion of the
iris. It starts in the deepest part of the angle and spreads
circumferentially.
 Primary angle-closure
glaucoma
 2. Acute (congestive) angle-
closure
• VA usually 6/60.
• IOP is usually very high (50–
100 mmHg).
• Conjunctival hyperaemia
with violaceous circumcorneal
injection.
• Anterior chamber is shallow.
• Corneal epithelial oedema
(A).
• Unreactive mid-dilated
vertically oval pupil (B).
 Primary angle-closure
glaucoma
 3. Resolved acute (post-
congestive) angle closure
• Folds in Descemet
membrane (A), (if IOP has
been reduced rapidly), optic
nerve head congestion and
choroidal folds.
• Later there is iris atrophy
with a spiral-like
configuration, irregular pupil,
posterior synechiae (B) and
glaukomflecken (C).
• The optic nerve may be
normal or exhibit varying
degrees of atrophy (D).
 Primary angle-closure glaucoma
Treatment of acute and subacute presentation of PAC and PACG
 Hospital admission is usually required in an acute presentation
 1. Initial treatment
• The patient should assume a supine position to encourage the lens to
shift posteriorly under the influence of gravity.
• Acetazolamide 500 mg is given intravenously if IOP >50 mmHg, and
orally (not slow-release) if IOP is <50 mmHg.
• If treatment is intravenous an additional oral dose of acetazolamide 500
mg may be given.
• Topical apraclonidine 1%, timolol 0.5%, prednisolone 1% or
dexamethasone 0.1% to the affected eye, leaving 5 minutes between each
instillation.
• Pilocarpine 2–4% one drop to the affected eye, repeated after half an
hour, and one drop of 1% as prophylaxis into the fellow eye.
• Some practitioners omit pilocarpine in an acutely presenting eye with
very high IOP until a significant IOP fall has taken place, as the associated
ischaemia will compromise the action of pilocarpine on the pupillary
sphincter.
• Analgesia and an antiemetic may be required.
 Primary angle-closure
glaucoma
 2. Subsequent medical treatment
• Pilocarpine 2% q.i.d. to the affected eye and 1% q.i.d. to the
fellow eye.
• Topical steroid (prednisolone 1% or dexamethasone 0.1%) q.i.d.
if the eye is acutely inflamed.

 3. Following successful treatment with a clear cornea, reasonably

quiet anterior chamber, and preferably a normalized IOP, bilateral
laser iridotomy is performed. Topical steroids and any necessary
hypotensives are continued for at least a week.

 4. Subsequent management: observation, treatment of

persistently raised IOP as for POAG, iridoplasty or long-term low
dose pilocarpine if appositional closure persists. Trabeculectomy is
occasionally necessary for persistent IOP elevation despite a
successfully opened angle.
 Bibliography
 American Academy of Ophthalmology. Basic and Clinical Science Course
2012-2013, Section
 Hollo Gabor, Konstas G.P. Anastasios. Exfoliation syndrome: a systemic
disease. Exfoliation Syndrome and Exfoliative Glaucoma. Second Edition
European Glaucoma Society;10:87-97
 Kanski Jack J. Glaucoma. Clinical Ophthalmology: a systematic approach
– 7th ed. Butterworth-Heinemann International Editions 2011;10:311-
400
 Lama PJ. Systemic adverse effects of beta-adrenergic blockers: An
evidence-based assessment. Am J Ophthalmol 2002;134:749-760
 Shah Peter, Rosemary Robinson. Glaucoma. Oxford Handbook of
Ophthalmology. Second Edition Oxford University Press 2009;10:301-348
 Terminology and guidelines for glaucoma. 3rd Edition European
Glaucoma Society 2008, www.eugs.org