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Primary vs.
Secondary Angle
Closure
Glaucoma
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Original
article Annie K. Lim, MD, JoAnn
contributed A. Giaconi, MD
by:
JoAnn A. Giaconi, MD,
Koushik Tripathy, MD
All
(AIIMS), FRCS (Glasgow)
contributors:
and Sarwat Salim MD,
FACS
Assigned
editor:
Review: Not reviewed

Contents [hide]

1 Summary
2 Disease Entity
2.1 Disease
3 Terminology[1]
3.1 Etiology
3.2 Risk Factors
3.3 General Pathology
3.4 Pathophysiology
3.4.1 Primary
3.4.2 Secondary
4 Primary prevention
4.1 Screening
4.2 Laser peripheral iridotomy for
narrow angles
5 Diagnosis
5.1 History
5.2 Physical examination
5.3 Signs
5.4 Symptoms
6 Clinical diagnosis
6.1 Gonioscopy
6.2 Provocative tests
6.3 Imaging modalities
6.3.1 Ultrasound biomicroscopy
(UBM)
6.3.2 Anterior segment OCT (AS-
OCT)
6.3.3 Scheimpflug photography
6.4 Laboratory test
6.5 Differential diagnosis
7 Management
7.1 Medical therapy
7.1.1 Acute angle closure
glaucoma
7.1.2 Chronic angle closure
glaucoma
7.1.3 Peripheral iridotomy
7.1.4 Acute Angle Closure and
Fellow Eyes
7.1.5 Chronic Angle Closure and
Angle Closure Glaucoma
7.1.5.1 Persistent or
progressive rise in IOP after LPI
7.1.5.2 Complications of LPI
7.1.6 Iridoplasty
7.1.7 Cataract extraction
7.1.8 Filtration surgery
7.1.9 Glaucoma drainage implants
7.2 Other surgical procedures
7.3 Surgical follow up
7.4 Complications
8 Prognosis
8.1 Primary angle closure suspects
8.2 Acute angle closure
8.3 Chronic angle closure glaucoma
8.4 Secondary Angle Closure
9 Additional Resources
10 References

Summary
Angle closure glaucoma is a major cause of
blindness worldwide, with a particularly high
prevalence in some Inuit and Asian
populations. This disease has a familial
tendency and is associated with increasing
age and hyperopia. In angle closure
glaucoma, increased intraocular pressure is
caused by impaired outflow facility secondary
to appositional or synechial closure of the
anterior chamber drainage angle . In primary
angle closure glaucoma, the underlying
mechanism is primarily pupillary block, while
in secondary forms there are other underlying
causes that either push the iris forward from
behind or pull the iris forward to contact
trabecular meshwork. Both types of angle
closure glaucomas may cause acute dramatic
attacks or chronic asymptomatic disease. A
thorough history and eye examination are
essential in differentiating between primary
and secondary forms. This distinction is
crucial as the treatment of each form can vary
greatly. The mainstays of therapy are
medications that lower intraocular pressure
and laser peripheral iridotomy for any
component of pupillary block. Although angle
closure in all its forms is vision threatening,
early diagnosis and appropriate management
can stabilize disease and minimize vision loss.
This review will discuss risk factors, signs and
symptoms, diagnostic tests and imaging
modalities, pathophysiology, differential
diagnosis, and treatment modalities of angle
closure glaucoma.

Disease Entity
Disease
Angle closure glaucoma (ACG) results
from appositional or synechial closure of the
anterior chamber angle leading to reduction
in aqueous outflow facility, IOP elevation, and
subsequent damage to the optic nerve with
associated visual field loss. There are primary
and secondary forms of angle closure
glaucoma . In primary angle closure, the
mechanism causing disease is primarily
pupillary block, being either functional or
absolute, while in secondary forms there are
other underlying causes.

Terminology[1]
Primary angle-closure suspect (PACS) or
anatomic narrow angle – An eye in which the
anterior chamber angle recess has an
abnormally narrow angular width. The
peripheral iris is located close to, but not
touching, the posterior pigmented trabecular
meshwork (TM). No PAS are present. IOP,
optic nerve, and visual field are normal.

Primary angle closure (PAC) – An eye that

has a primary anatomic narrow angle and
evidence that trabecular obstruction by the
peripheral iris has occurred, such as
peripheral anterior synechiae (PAS), elevated
IOP, iris whorling or sectoral atrophy, and
excessive pigment deposition on the
trabecular surface. The eye does not have
glaucomatous damage of the optic nerve.

Primary angle closure glaucoma (PACG)-

iridotrabecular contact is present in three or
more quadrants of the drainage angle in the
presence of documented optic nerve damage
and visual field loss.

Secondary angle closure is caused by an

underlying identifiable pathologic etiology,
such as neovascularization or uveitis (see
“Differential Diagnosis” section). If angle
closure results in elevated IOP that causes
glaucomatous optic disc damage, it is
referred to as secondary angle closure
glaucoma.

Both primary and secondary angle closure

can result in acute angle closure attacks and
chronic angle closure leading to glaucoma.

Etiology
See Pathophysiology section below.

Risk Factors
A large number of risk factors have been
identified:

Hyperopia[2] [3]
Family history of angle closure[4] [5]
Advancing age[6] [7]
Female gender[7] [8]
Asian or Inuit descent[6] [9] [10] [11]
Shallow anterior chamber depth[12] [13]
Shorter axial length[14]
Thicker lens[15]

Risk factors for secondary angle closure are

related to the underlying diseases that cause
it. See Differential Diagnosis section for a list
of the various eye diseases that can cause
angle closure.

General Pathology
Not applicable

Pathophysiology
Primary
Primary angle closure glaucoma is caused by
relative pupillary block in the majority of
cases. In pupillary block, aqueous humor
encounters increased resistance as it flows
from the posterior to anterior chamber
through the iris-lens channel. Some degree of
relative pupillary block is present in most
phakic eyes. The risk of pupillary block is
highest with a mid-dilated pupil where there
appears to be maximum contact between the
iris and the lens. In eyes with angle closure,
other factors exacerbate the block, such as
the front lens surface being anterior to the
plane of iris insertion into the ciliary body
base. The increased pressure gradient across
the pupil causes the peripheral iris to bow
forward and cover some or all of the filtering
portion of the trabecular meshwork, resulting
in appositional angle closure. Peripheral
anterior synechiae form after prolonged or
repeated contacts of the peripheral iris with
TM. Another mechanism thought to be
important in primary angle closure is iris angle
crowding, which is caused by a thickened
peripheral iris filling the space between the
TM and angle recess under dark conditions.
[16] [17]

Eyes that experience angle closure are not

only anatomically different than normals —
they have shorter axial lengths, shallower
anterior chambers, thicker and relatively
anteriorly positioned lenses, and flatter
corneas[18] — but they are also
physiologically different. Thicker irides may
increase the posterior to anterior pressure
differential.[19] Dynamic factors in angle
closure eyes that can contribute to increased
pupillary block are the tendency to retain
more iris volume after dilation and choroidal
expansion causing forward lens movement.
[20] As imaging modalities, such as ultrasound

biomicroscopy and anterior segment optical

coherence tomography improve, these
dynamic factors will be better studied and
understood.

A less common cause of primary angle

closure is anterior non-pupillary block. This is
observed in eyes in which angle closure
progresses despite a patent iridotomy, for
example, as seen in plateau iris. Plateau iris
configuration is characterized by a normal
central anterior chamber depth, flat iris profile,
and crowding of the angle by the iris base.
There is a forward displacement of the iris
base by anteriorly located ciliary processes
that can lead to subsequent angle closure.
Plateau iris syndrome occurs when an eye
with plateau iris configuration develops a
closed angle.[21] Prominent last iris roll is
another mechanism of anterior nonpupillary
block in which a very thick iris with prominent
peripheral circumferential folds becomes
more pronounced and contacts trabecular
meshwork with dilation.[22]

Secondary
Secondary angle closure glaucoma is caused
by a myriad of other eye diseases (see
Differential Diagnosis section). There are
several secondary causes of angle closure
that involve relative and absolute pupillary
block. In phacomorphic glaucoma, the mass
effect of a thickened or intumescent cataract
pushes the iris forward and causes
pathological angle narrowing. Forward
displacement of the lens in ectopia lentis or
microspherophakia can also push the iris
forward and shallow the angle. Absolute
pupillary block occurs when there is no
movement of aqueous through the pupil
because of 360o posterior synechiae between
the iris and a crystalline lens, an intraocular
lens, capsular remnants, or the vitreous face.
In secondary angle closure glaucoma without
pupillary block, angle closure is due to either
a.) contraction of an inflammatory,
hemorrhagic, or vascular membrane in the
angle leading to PAS, or b.) forward
displacement of the lens-iris diaphragm, often
associated with ciliary body swelling and
anterior rotation.

Mechanisms that push the iris forward

from behind
Relative pupillary block (primary angle
closure)
Plateau iris configuration (primary angle
closure)
Absolute pupillary block – 360o
posterior synechiae secluding pupil
Aqueous misdirection or malignant
glaucoma
Ciliary body swelling, inflammation or
cysts
Choroidal swelling, effusions, or
detachments
Posterior segment tumors or space-
occupying substances (silicone oil or
gas bubble)
Contracting retrolental tissue as seen in
retinopathy of prematurity
Anteriorly displaced lens
Encircling retinal bands/buckles

Mechanisms that pull the iris forward

into contact with the trabecular
meshwork
Contraction of inflammatory membrane
or fibrovascular tissue
Iridocorneal endothelial (ICE) syndrome
with migration of corneal endothelium
Fibrous ingrowth
Epithelial downgrowth
Iris incarceration in traumatic or surgical
wound

Primary
prevention
Screening
Given the high prevalence and morbidity of
angle closure glaucoma in many countries,
there is a need for quick, inexpensive
screening methods that do not require highly
skilled operators.

Oblique flashlight test uses a penlight that is

held next to the temporal side of an eye with
the beam of light parallel to the iris and
shining across the anterior chamber. If there is
a shadow projected onto the nasal iris, the
angle is narrow because the iris is bowed
forward and blocking the path of light. If there
is no shadow, the anterior chamber angle is
considered open. Because of variability in
flashlight illumination and subjectivity in
assessment of the test result, however, this
test has a relatively low specificity.[23]

The Van Herick method uses a narrow slit

beam at 60o onto the cornea just anterior to
the limbus to evaluate the anterior chamber
depth. If the distance from the anterior iris
surface to the posterior corneal surface is less
than one-fourth the corneal thickness, the
angle may be narrow and should undergo
gonioscopy. Modification of this method by
creating additional grades, called the limbal
chamber depth test, outperformed central
anterior chamber depth (as measured by
optical pachymetry and ultrasound) and
autorefraction as a screening tool for the
detection of occludable drainage angles
identified by gonioscopy.[24]

Laser peripheral
iridotomy for narrow
angles
The goal of screening for narrow angles is to
identify patients at risk of developing angle
closure and to treat prophylactically with a
laser peripheral iridotomy (LPI). Patients with
narrow but open angles should be followed
for IOP elevation, progressive angle
narrowing, and development of PAS. There is
no evidence that iridotomy is indicated for
narrow but open angles with normal IOP;
however, LPI is fairly safe and can prevent a
potentially vision-threatening outcome so the
risks and benefits must be carefully
considered in each case and discussed with
the patient in details.

Indications for laser peripheral ridotomy

Elevation of previously normal IOP
Presence of potentially occludable
angle
PAS attributable to episodes of angle
closure
Progressive narrowing of the angle
Requirement for medication that may
provoke pupillary block (i.e. —
antidepressants, anticholinergics, etc.)
Symptoms that suggest prior or
intermittent subacute angle closure
Situation limiting a patient’s ability to
immediately receive ophthalmic care
(e.g.,frequent travel to less developed
countries where treatment may not be
readily available, lack of insurance, and
poor access to transportation)
Having another eye disorder that
requires frequent dilated eye exams
(e.g. diabetic retinopathy)
History of acute PAC in other eye

Diagnosis
Diagnosis of both primary and secondary
angle closure glaucoma is based on history
and eye exam. Gonioscopy is the gold
standard for evaluating the anterior chamber
angle, but imaging modalities assist in
quantifying and objectifying angle
characteristics. To distinguish between
primary and secondary causes, the clinician
must actively look for signs and symptoms of
possible secondary causes and rule each out
before the patient can be diagnosed
with primary angle closure glaucoma .

History
History of present illness – typical signs
and symptoms of acute or subacute
angle closure attacks (see Signs and
Symptoms sections) and if patient was
upset or in the dark when symptoms
started
Past ocular history
Trauma – can cause zonular
weakness or dehiscence allowing
lens to displace anteriorly
Incisional or laser surgery – can cause
anterior chamber inflammation or
predispose to epithelial/fibrous
ingrowth; may also lead to aqueous
misdirection (e.g. after LPI) or ciliary
body engorgement (e.g. after
extensive panretinal
photocoagulation).
History of prior retinal vein occlusion
– can cause angle neovascularization
or ciliary body engorgement rotating
lens forward
Past medical history – history of diabetes
or carotid stenosis disease that can cause
angle neovascularization
Medications – use of systemic
medications, such as sulfonamide,
topiramate, and phenothizaines, that may
cause ciliary body engorgement or
suprachoroidal effusion; use of
medications to treat allergy, bladder
dysfunction, or depression; use of
anticholinergics or sympathomimetics
that can dilate pupil
Family history of acute angle glaucoma

Physical examination
Refractive status – hyperopic eyes tend to
have shallower anterior chamber angles
which places them at risk for angle
closure
Pupil size and reactivity
Slit lamp exam
Conjunctiva – injection
Cornea – clarity, presence of edema,
evidence of surgical or traumatic
wounds
Anterior chamber – central and
peripheral depth, inflammation
Iris – areas of atrophy, mass,
neovascularization, or posterior
synechiae
Lens – thickness, phacodonesis,
subluxation, glaucomflecken
(necrosis of anterior lens
capsule; may indicate previous
attacks)
Intraocular pressure measurement,
preferably with applanation prior to
gonioscopy
Gonioscopy of both eyes with indentation
to evaluate for appositional versus
synechial angle closure
Evaluation of fundus and optic nerve –
dilation is often not advisable in primary
angle closure attack until an iridotomy
has been performed and/or the acute
attack has resolved as dilation can
exacerbate the condition. In contrast,
dilation may be permissible as the
appropriate treatment in certain forms of
secondary angle closure. The fundus
should be examined for underlying
causes leading to the angle closure.

Signs
Signs of a primary anatomic narrow angle on
slit lamp exam can be subtle and include a
shallow anterior chamber and an anteriorly
bowed iris.

Both primary and secondary forms of angle

closure can cause acute angle closure
attacks. The intraocular pressure usually
exceeds 40 mmHg and may rise to as high as
80 mmHg. The conjunctiva is significantly
injected. The cornea develops stromal and
microcystic edema in response to the acute
rise in eye pressure and decompensation of
the endothelial pump mechanism. Iris
sphincter ischemia leads to a fixed, mid-
dilated pupil. Sectoral iris atrophy may occur,
releasing pigment into the anterior chamber
that dusts the corneal endothelium and
anterior lens capsule. The overall anterior
chamber is shallow. The center is usually
formed, but the mid-peripheral iris bows
forward and may touch the peripheral cornea.
Often there is anterior chamber inflammation.
The fundus is typically difficult to examine
because of corneal edema. If visualized, the
optic nerve head may be hyperemic and
edematous. The angle is often difficult to
examine with gonioscopy because of corneal
edema, but if visualized, reveals contact of
the peripheral iris with cornea.

In the aftermath of an acute attack, pupillary

distortion may result if there is permanent
sphincter damage and/or iris atrophy. Anterior
lens capsule opacities, known as
glaukomflecken, may result from damage to
the anterior lens epithelium from high
intraocular pressure. The angle may have
permanent synechiae formation. Intraocular
pressure may be low if the ciliary body is so
ischemic that aqueous humor production is
compromised. As the ciliary body recovers,
normal aqueous humor production resumes
and the intraocular pressure rises.

ACG exhibits signs of optic neuropathy in the

typical glaucomatous pattern with increased
cupping of the optic nerve and retinal nerve
fiber layer dropout.

Summary of Clinical Findings in Various

Types of Secondary Angle Closure
Glaucoma
Neovascularization of the angle – blood
vessels from iris that cross scleral spur
to arborize along trabecular meshwork,
PAS
Anterior chamber inflammation – keratic
precipitates, posterior synechiae, iris
bombé, inferior PAS (as opposed to
primary angle closure where PAS tend
to develop superiorly)
Iridocorneal endothelial (ICE) syndrome
– beaten-bronze corneal endothelium,
corneal edema, high PAS that can
extend anterior to the Schwalbe line,
iris atrophy, corectopia
Drug induced (e.g. systemic topiramate)
– acute bilateral disease, acute myopic
shift, uniformly shallow chamber with
anterior iris and lens displacement,
ciliochoroidal effusion or detachment
Aqueous misdirection (malignant
glaucoma) – flattening of central and
periperhal anterior chamber, anterior
displacement of lens (cystralline or
intraocular lens) or vitreous face, clear
“aqueous” zones in vitreous
Lens-induced angle closure – thick
cataract, unstable or subluxed lens
Nanophthalmos – small but normal eye
with short axial length, microcornea,
large lens, and thickened sclera;
choroidal effusion
Retinopathy of prematurity or persistent
hyperplastic primary vitreous –
contracting retrolental tissue
Iris or ciliary body mass lesions or cysts
– irregular contour of iris or
neovascularization, hyphema or
vitreous hemorrhage, episcleral sentinel
vessel, ciliary body mass through
dilated pupil or on ultrasound
biomicroscopy
Posterior segment mass or large serous
retinal detachment – signs of each on
funduscopic exam
Epithelial and/or fibrous downgrowth –
wound dehiscence or gape, epithelial
cysts in anterior chamber adjacent to
wound, gray sheetlike membrane
covering anterior segment structures
that whitens with argon laser
Pseudophakic or aphakic pupillary
block – pupilary obstruction or
synechiae to anterior hyaloid surface,
the intraocular lens, or posterior
capsule
Ciliary body engorgement associated
with retinal vascular occlusion or
panretinal photocoagulation – anterior
rotation of iris and lens; retinal signs of
each underlying etiology, e.g. retinal
hemorrhages for vein occlusion and
extensive retinal laser scars from
photocoagulation.
Retinal surgery
Encircling scleral buckle – relatively
deep central anterior chamber with
peripheral iris flattening, choroidal
effusion
Pars plana vitrectomy - pupillary
block from expansile gases pushing
iris and lens forward, silicone oil in
the anterior chamber, non-patent
iridotomy

Symptoms
Patients with anatomic narrow angles without
acute angle closure are typically
asymptomatic in both the primary and
secondary forms. Similarly, primary and
secondary chronic angle closure patients
often experience no symptoms unless they
develop end-stage glaucoma, in which case
they may complain of decreased vision or
reduced peripheral vision.

Acute angle closure, on the other hand,

usually presents with dramatic symptoms
from the quick rise in intraocular pressure.
Patients complain of blurred vision, rainbows,
halos around lights, or even transient loss of
vision. They often have intense pain that may
be localized to the eye, may follow the
trigeminal distribution, or may be described
as diffuse discomfort. Nausea and vomiting
are common.

Subacute or intermittent angle closure attacks

are brief episodes of angle closure that
resolve spontaneously. Patients experience
the above symptoms of acute angle closure,
but on a milder scale. They will typically
experience some blurring of the vision or
halos with mild to moderate eye pain, brow
ache, or headache. These attacks are often
resolved by entering a well lit room which may
cause miosis or sleep as sleep-induced
miosis ameliorates the lesser degree of
pupillary block in these patients.

Clinical diagnosis
Gonioscopy
The key to diagnosis of anatomic narrow
angle or angle closure is gonioscopy, which is
still the gold standard method of angle