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Open-angle glaucoma: Epidemiology, clinical

presentation, and diagnosis


Author: Deborah S Jacobs, MD
Section Editor: Jonathan Trobe, MD
Deputy Editor: Jane Givens, MD

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is
complete.

Literature review current through: May 2019. | This topic last updated: Nov 07, 2018.

INTRODUCTION

Glaucoma is a group of eye diseases traditionally characterized by elevated


intraocular pressure (IOP). However, glaucoma is more accurately defined as an
optic neuropathy than a disease of high pressure. In open-angle glaucoma, optic
nerve damage results in a progressive loss of retinal ganglion cell axons, which is
manifested initially as visual field loss and, ultimately, irreversible blindness if left
untreated [1].

This topic will discuss the epidemiology, clinical presentation, and diagnosis of
open-angle glaucoma in adults. Glaucoma in children, angle-closure glaucoma,
and treatment and prevention of open-angle glaucoma are discussed elsewhere.
(See "Overview of glaucoma in infants and children" and "Angle-closure glaucoma"
and "Open-angle glaucoma: Treatment".)

CLASSIFICATION

There are different types of glaucoma, generally categorized by the anterior


chamber (iridocorneal) angle (figure 1) and the underlying etiology, if known:
● Open-angle glaucoma is an optic neuropathy characterized by progressive
peripheral visual field loss followed by central field loss in a typical pattern. It
is usually but not always in the presence of elevated intraocular pressure
(IOP). Increased aqueous production and/or decreased outflow are possible
mechanisms for elevated intraocular pressure (figure 2). The optic nerve or
"disc" takes on a hollowed-out appearance on ophthalmoscopic examination,
which is described as "cupping." Cupping is associated with the loss of
ganglion cell axons.

● Angle-closure glaucoma is characterized by narrowing or closure of the


anterior chamber angle. The normal anterior chamber angle provides drainage
for the aqueous humor (the fluid that fills the eyeball). When this drainage
pathway is narrowed or closed, inadequate drainage leads to elevated IOP and
damage to the optic nerve (figure 3). Acute angle-closure glaucoma occurs in
eyes with a certain anatomical predisposition. It presents as a painful red eye
and must be treated within 24 hours to prevent permanent blindness. (See
"Angle-closure glaucoma".)

● Developmental glaucoma occurs in infants and children and is discussed


elsewhere. (See "Overview of glaucoma in infants and children" and "Primary
infantile glaucoma".)

● Both open-angle and angle-closure glaucoma can be divided into primary and
secondary forms. Secondary glaucoma has many subtypes with elevated IOP
resulting from uveitis, trauma, glucocorticoid therapy, vasoproliferative
retinopathy, or ocular syndromes such as pigment dispersion or
pseudoexfoliation (the deposition of white fluffy material within the anterior
segment of the eye).

● Glaucoma can also be categorized based on timing (ie, acute, subacute, and
chronic).
● Mixed mechanism glaucoma refers to glaucoma with several etiologies (eg,
open-angle glaucoma complicated by superimposed angle-closure, or open-
angle glaucoma with superimposed uveitis).

EPIDEMIOLOGY

After cataracts, glaucoma is the second leading cause of blindness in the world
[2]. It is a leading cause of irreversible blindness and the leading cause of
blindness among African-Americans [3,4]. Open-angle glaucoma is the most
common type of glaucoma among populations of European or African descent,
whereas angle-closure glaucoma is more common among populations of Asian
descent [2,3]. Worldwide in 2015, there were an estimated 57.5 million people with
open-angle glaucoma, and this number is projected to increase to 65.5 million by
2020 [5]. It is estimated that there are 2.8 million people with open-angle
glaucoma in the United States in 2010 [6] and that the number will increase to 3.4
million in 2020 [7].

Patients with open-angle glaucoma report decreased quality-of-life and difficulties


with daily functioning, including driving [8,9]. Patients with glaucoma are also more
likely to report falls and motor vehicle collisions [10]. One meta-analysis found no
association between open-angle glaucoma and all-cause mortality [11].

Risk factors — The major risk factors for developing open-angle glaucoma include
age, black race, family history, and elevated intraocular pressure (IOP) [12-14].

● Age – The incidence of open-angle glaucoma increases with age, particularly


in patients of Caucasian and African ancestry [15-17]. The prevalence of
open-angle glaucoma is <1 percent in individuals under 55 years of age,
approaches 2 percent at age 65, and reaches approximately 4 percent at age
80 [7]. The rate of blindness from open-angle glaucoma also increases with
age [4].
● Race – Race is an important risk factor for development and progression of
open-angle glaucoma [18]. The estimated prevalence of open-angle glaucoma
is approximately three times higher in blacks compared with whites [7]. The
age-adjusted rate of blindness from glaucoma among blacks was 6.6 times
that among whites, with blindness beginning 10 years earlier in blacks [4]. The
incidence of open-angle glaucoma in Latinos is higher than in non-Hispanic
whites, though lower than in Afro-Caribbeans [19]. Non-Hispanic white women
comprise the largest group with open-angle glaucoma in the United States in
2011, but it is anticipated that this will shift to Hispanic men over the next
several decades [20].

● Family history – Family history is a significant risk factor for open-angle


glaucoma in several population studies [21-24]. The Baltimore Eye Survey
found that the relative risk of open-angle glaucoma increased 3.7- and 2.2-
fold for individuals with an affected sibling or parent, respectively [21]. Several
early-onset glaucoma syndromes are inherited as Mendelian dominant or
recessive traits; open-angle glaucoma, however, has a complex inheritance
pattern, with the likelihood that multiple genes interact with environmental
factors [22].

● Elevated intraocular pressure – There is a large body of literature illustrating


the association between elevated IOP and both development and progression
of open-angle glaucoma [24-29].

As an example, the Early Manifest Glaucoma Trial followed 255 patients with
a diagnosis of open-angle glaucoma over a mean of eight years; mean IOP
was a significant risk factor for progression of glaucoma (hazard ratio [HR]
1.11, 95% CI 1.06-1.17), even when IOP was within the "normal" range of 8 to
22 mmHg [30].

Nearly 40 percent of patients with otherwise characteristic open-angle


glaucoma will not have an elevated IOP [14]. These patients constitute a
subgroup commonly referred to as low-tension or normal-tension glaucoma.
While a small proportion of open-angle glaucoma in the United States may be
classified as normal tension, the majority of patients with open-angle
glaucoma in Asia have normal tension glaucoma [31]. By contrast, many
patients with elevated pressures never develop the optic nerve and field
changes characteristic of glaucoma [25,26]. Thus, although high pressure is
clearly associated with open-angle glaucoma, it is neither necessary nor
sufficient for the diagnosis and is therefore termed a "risk factor" for the
condition.

● Hypertension – Observational studies suggest an association between


hypertension and primary open-angle glaucoma. A 2014 systematic review
and meta-analysis of 60 observational studies found that the risk for primary
open-angle glaucoma was increased in patients with hypertension compared
with those without (relative risk [RR] 1.16, 95% CI 1.05-1.28); nearly all studies
noted an association between hypertension and increased intraocular
pressure [32].

● Diabetes – Observational studies also suggest an association between


diabetes and primary open-angle glaucoma. A 2014 systematic review and
meta-analysis of 47 observational studies found that the risk for primary
open-angle glaucoma was increased for patients with diabetes compared
with those without (RR 1.48, 95% CI 1.29-1.71) [33]. The risk increased 5
percent each year after the diagnosis of diabetes.

● Other factors – Other possible risk factors for developing open-angle


glaucoma include myopia, pseudoexfoliation, low diastolic perfusion
pressure, cardiovascular disease, a history of prior vitreoretinal surgery, and
hypothyroidism [34-40].

Although risk factors for the development of open-angle glaucoma have been well
documented, risk factors for progression of open-angle glaucoma are less certain
[41-43]. Results conflict whether fluctuation in IOP is predictive of glaucoma
progression [28,29]. Even in patients with documented findings of glaucoma on
comprehensive eye examination (eg, visual field deficits, optic disc changes), it is
unclear which patients go on to develop loss of visual acuity and blindness [44].

PATHOGENESIS

The pathogenesis of primary open-angle glaucoma is not clear. Optic nerve axon
loss may be related to ganglion cell susceptibility, microcirculatory deficiency at
the optic nerve head, or extracellular matrix factors [12,45]. These factors may
play a combined role: circulatory or extracellular matrix factors could account for
both high pressures and axon loss; variation in axon susceptibility might explain
why the disease state does not correlate well with elevated IOP [46]. It is not clear
if elevated intraocular pressure (IOP) is caused by factors related to aqueous
production, aqueous outflow, or anatomic or physiologic features of the trabecular
meshwork and other outflow structures. Systemic factors may also play a role, as
there is some evidence that cardiac autonomic dysfunction, as measured by heart
rate variability, may correlate with the presence of normal pressure glaucoma [47].

Mutations in the myocilin gene (MYOC) have been identified in about 4 percent of
adults with open-angle glaucoma and in more than 10 percent of cases of juvenile
open-angle glaucoma, a rare autosomal dominant condition with glaucoma onset
between 3 and 40 years of age [12]. MYOC mutations alter the myocilin protein.
Myocilin-associated glaucoma is characterized by elevated IOP, with IOP >40
mmHg in some patients with juvenile open-angle glaucoma. Myocilin is produced
in the ciliary body and trabecular meshwork, but its precise role in regulating IOP is
unknown.

CLINICAL PRESENTATION
Individuals with open-angle glaucoma rarely experience symptoms. Thus, open-
angle glaucoma is generally detected incidentally during comprehensive
ophthalmic examination. This is in contrast to angle-closure glaucoma in which
patients present with symptoms and signs including loss of visual acuity, pain,
conjunctival erythema, and corneal edema. (See "Angle-closure glaucoma", section
on 'Clinical presentation'.)

High elevations of intraocular pressure (IOP), up to 40 mmHg in patients with


open-angle glaucoma, generally cause no pain, redness, or visual symptoms.
There is no loss of visual acuity as long as central vision is preserved. Central
visual field loss is a late manifestation of open-angle glaucoma, usually preceded
by ganglion cell loss and optic nerve damage. Some patients are unaware of field
loss even when it has progressed to central "tunnel vision" of 10 to 20 degrees.
Visual field loss cannot be recovered once it has occurred.

The mean progression rate from a full field of vision to blindness takes
approximately 25 years in untreated patients [48]. However, it is important to note
that the median progression rate is much slower (approximately 70 years), since
only a small minority of patients progress rapidly to blindness. Nonetheless, in a
Swedish study of 592 glaucoma patients who had died between 2006 and 2010,
250 (42 percent) had at least one eye with blindness (as defined by the World
Health Organization criteria) and 16.4 percent were bilaterally blind at their last eye
visit; the median age for developing bilateral blindness was 86 years [49].

SCREENING

Intraocular pressure (IOP) testing (tonometry) remains the most available and
best-studied screening test for glaucoma. However, many persons with primary
open-angle glaucoma do not have increased IOP and not all persons with elevated
IOP will develop glaucoma, so screening with tonometry alone may be inadequate
[50]. Furthermore, there is no "gold standard" test for identifying glaucoma. It
remains controversial which (if any) populations should be screened, what
screening tests should be performed, and with what frequency. (See 'Diagnosis'
below.)

Evidence for treating elevated intraocular pressure — There are no available


studies that directly address whether screening prevents visual impairment or
worsened quality of life [50]. A meta-analysis of 10 trials demonstrated that
medical treatment for ocular hypertension reduced the risk of visual field defects
(odds ratio [OR] 0.62, 95% CI 0.47-0.81) [51]. Another meta-analysis of five
randomized trials in adults with elevated IOP found that topical IOP-lowering
medication, compared with placebo, decreased the risk of open-angle glaucoma
(hazard ratio [HR] 0.56, 95% CI 0.39-0.81) [52]. The study estimated that 12
subjects with elevated IOP would need to be treated to prevent one case of
glaucoma within five years of treatment.

Since the prevalence of elevated IOP in the general population is approximately 4


percent among individuals age ≥40 years [53] and 12 patients would be needed to
treat to prevent one case of glaucoma, 300 individuals would need to be screened
to prevent one case of glaucoma within five years of treatment. However, primary
open-angle glaucoma is a heterogeneous disease and does not progress in all
patients or may progress at a rate that would not interfere with vision or quality of
life in a person's lifetime.

Cost-effectiveness — In the absence of randomized trials assessing screening


strategies, several studies have attempted to determine the cost-effectiveness of
glaucoma screening, with some using Markov modelling [54-57]. One study in the
United Kingdom found that neither IOP measurement alone nor comprehensive
eye examination were cost-effective in subjects age ≥40 years but that screening
in older or other higher risk populations might be cost-effective [56]. Another study
in Finland found that screening with combined fundus examination, visual field
testing, and IOP measurement in persons age 50 to 89 was cost-effective for
preventing visual disability (EUR €32,603 per one year of avoided visual disability)
and quality-adjusted life years (QALY; €9023 per one QALY gained by screening)
[57]. There is a great deal of uncertainty in the assumptions of these models,
particularly with regard to which is the optimal screening protocol, the harms and
benefits associated with early detection, and the thresholds for different
treatments in relation to cost (determining follow-up, adherence, and additional
testing) [54,56,57].

Recommendations of others — There is significant variation in screening


recommendations from different organizations, partly since no randomized trials
have evaluated screening strategies for the prevention of open-angle glaucoma
[58].

● The American Academy of Ophthalmology (AAO) recommends


comprehensive eye examinations for patients with risk factors for glaucoma
(eg, African-Americans and Hispanics) by an ophthalmologist [59]. The
frequency depends on age: every one to two years in patients <40 years, one
to three years in patients age 40 to 54 years, one to two years in patients aged
≥55 years. For patients without risk factors, the AAO suggests a
comprehensive eye exam every 5 to 10 years in patients <40, two to four years
in patients 40 to 54 years, one to three years in patients 55 to 64 years, and
one to two years in patients ≥65 years. Screening only by measuring IOP is not
appropriate since a substantial number of patients with glaucomatous visual
field changes have normal IOP [25].

Field testing is performed by the ophthalmologist once the patient falls into
the diagnostic category of "glaucoma suspect" based upon the other risk
factors. (See 'Risk factors' above.)

● The US Preventive Services Task Force (USPSTF) found insufficient evidence


to recommend for or against screening adults for glaucoma [60]. While they
felt there was good evidence that screening could detect increased IOP and
early open-angle glaucoma, and that early treatment reduces the development
and progression of visual field defects, they also felt that the evidence was
insufficient to determine the extent to which screening would reduce
impairment in vision-related function or quality of life. They noted that harms
associated with treatment for increased IOP and early open-angle glaucoma
include local eye irritation and an increased risk for cataracts.

● The Canadian Task Force on the Periodic Health Examination also concluded
that there was insufficient evidence to recommend for or against screening
for glaucoma in the periodic health examination. Referral of high-risk
individuals to a specialist was deemed "clinically prudent" [61].

Synthesis — Given the risk of blindness with untreated glaucoma, the


effectiveness of treatment, and that early open-angle glaucoma is asymptomatic,
we suggest that individuals over age 40 be screened for glaucoma. Earlier
screening may be appropriate for individuals with significant risk factors for
glaucoma. Although the randomized trials cited in this section performed IOP-
based screening only, we suggest screening with a comprehensive eye
examination since individual tests such as fundus examination or measurement of
IOP alone will likely fail to detect many cases of glaucoma [62-64].

DIAGNOSIS

Glaucoma is diagnosed in patients with characteristic nerve damage on fundus


examination (picture 1A-B) and visual field testing, typically in the presence of
elevated intraocular pressure (IOP). Some authorities consider either
characteristic optic nerve change OR visual field defects as sufficient criteria for
diagnosis of open-angle glaucoma [65,66].

The American Academy of Ophthalmology (AAO) Preferred Practice Pattern


defines primary open-angle glaucoma as a chronic, generally bilateral, and often
asymmetrical disease, which is characterized (in at least one eye) by all of the
following [14]:

● Evidence of optic nerve damage from either or both of the following: optic
disc or retinal nerve fiber layer structural abnormalities (eg, thinning, cupping,
or notching of the disc rim, progressive change, nerve fiber layer defects);
reliable and reproducible visual field abnormalities (eg, arcuate defect, nasal
step paracentral scotoma, generalized depression) in the absence of other
causes or explanations for a field defect.

● Adult onset.

● Open, normal appearing anterior chamber angles.

● Absence of known (eg, secondary) causes of open-angle glaucoma.

Who should be referred for comprehensive eye examination? — Patients with


abnormal cupping on funduscopic examination or risk factors (eg, age >40) should
be referred to an ophthalmologist or optometrist for a comprehensive eye
examination. Any patient with high IOP detected during community-based
screening or spectacle/contact lens evaluation should also be referred for a
comprehensive eye examination. The best available data support examination by
an ophthalmologist as the most accurate way to detect glaucoma [67]. (See 'Risk
factors' above.)

Diagnostic tests

Fundus examination — The primary care clinician should be attentive to the


presence of cupping seen in the fundus. Cupping describes a hollowed-out
appearance of the optic nerve or "disc" on fundus examination. A cup whose
diameter is greater than 50 percent of the vertical disc diameter is indicative of
glaucoma.
Although cupping has the highest sensitivity and specificity of any other finding on
eye examination, there is no single cutoff criteria that yields sufficiently high
sensitivity and specificity to make cupping a useful diagnostic test [68]. One study
found that ophthalmologists, using direct ophthalmoscopy, detected less than
one-half of cases of glaucoma [62]. Combining cupping with other criteria
increased diagnostic yield.

Other findings on fundus examination indicative of glaucoma include thinning or


notching of the disc rim, progressive change of the size or shape of the cup, and
asymmetry of the cup-to-disc ratio between the eyes [68,69].

Visual field testing — Open-angle glaucoma ideally should be diagnosed before


there is significant visual field loss. However, confrontational field testing, using
the examiner's fingers, is not useful in the detection of glaucoma. Automated
perimetry is an important diagnostic tool that is much more reliable at detecting
visual field loss in glaucoma compared with confrontational field testing (figure 4)
[70].

There are several types of automated perimetry technologies, including standard


threshold automated perimetry, frequency-doubling technology perimetry, and
short-wavelength automated perimetry [63]. Automated perimetry has become the
standard of care for optometric and ophthalmic practice in the detection and
monitoring of glaucoma, although there is a role for careful manual perimetry in
some cases, particularly in patients with advanced field loss or dementia. Reliable
field testing requires comprehension and cooperation on the part of the patient.
Dementia and other mental or physical problems may preclude testing in certain
individuals, forcing the clinician to rely upon other variables in diagnostic and
therapeutic decision-making.

Visual field testing can be time consuming and of variable specificity and
sensitivity, depending on user characteristics and the type of test being used.
Newer technologies to measure visual fields with greater reliability are in
development. (See 'Newer technologies' below.)

Intraocular pressure — Elevated IOP alone does not establish the diagnosis of


open-angle glaucoma [14,71]. One-third to one-half of individuals with glaucoma
field defects have intraocular pressures ≤21 mmHg when first detected (normal
IOP 8 to 21 mmHg) [72]. In addition, over 90 percent of adults with pressures >21
mmHg have no optic nerve damage. However, patients with elevated IOP should
be referred to an ophthalmologist given their higher risk of open-angle glaucoma.
(See 'Risk factors' above.)

A prospective population study of risk factors associated with glaucomatous field


loss found that, during a period of five years, 99 percent of eyes with an initial
pressure <20 mmHg continued to be free of glaucomatous field defects,
compared with 93 percent of eyes with an initial pressure ≥20 mmHg [73]. The
sensitivity for the diagnosis of open-angle glaucoma by IOP measurement was
47.1 percent and the specificity over 90 percent at an IOP cutoff point of >21
mmHg [64]. The presence of either increased IOP (>21 mmHg) or increased
vertical cup/disc ratio (≥0.5) increased the sensitivity to 61 percent but decreased
the specificity to 84 percent. There was no cutoff value for IOP that had
reasonable sensitivity and specificity as a screening tool for the diagnosis of open-
angle glaucoma.

Ophthalmologists and optometrists can measure IOP by applanation tonometry,


pneumotonometry, or air-puff tonometry. Applanation tonometry is a method that
determines the IOP from the force required to flatten (applanate) a constant area
of the cornea (picture 2). Applanation tonometry is most accurate and less subject
to artifact. All tonometry methods require specialized equipment and skill and
thus fall out of the realm of the primary care clinician. Due to the effects of central
corneal thickness on the mechanics of applanation tonometry, falsely higher
measurements occur in patients with thicker corneas and falsely lower
measurements occur in those with thinner corneas [74]. This can be partially
corrected with pachymetry. (See 'Pachymetry' below.)

Schiotz tonometry is a handheld device that is relatively inexpensive but requires


frequent use for reliable results. Generalists who practice in populations that do
not have access to optometric or ophthalmic care can learn Schiotz tonometry
and use it in conjunction with the optic disc examination in deciding whom to treat
or refer. In developed countries, it is less common for primary care providers to
measure IOP.

There is some evidence that wearing a tight necktie may temporarily increase IOP
[75]. The clinical implications of these findings require further study. Regardless, it
is prudent to ask patients to loosen collars and ties prior to measuring IOP.

Pressure parameters for referral — There are no standard criteria for referral


to an ophthalmologist for patients with elevated pressures. Primary open-angle
glaucoma rarely presents with IOP >30 mmHg, which is more common among
patients with angle-closure glaucoma or secondary (rather than primary) open-
angle glaucoma. (See "Angle-closure glaucoma" and "Overview of the red eye".)

The following represent indications for ophthalmologic referral based on clinical


practice experience:

● IOP >40 mmHg – Emergency referral

● IOP 30 to 40 mmHg – Urgent referral (within 24 hours) if no symptoms


suggesting acute glaucoma

● IOP 25 to 29 mmHg – Evaluation within one week

● IOP 23 to 24 mmHg – Repeat measurement to confirm and/or referral for


comprehensive eye examination
These indications are not absolute and should be interpreted in the context of
patient history and examination findings. As an example, a patient with an IOP of
28 and advanced open-angle glaucoma including field loss and cupping may
represent an emergency because of risk of imminent field loss, while a patient
with a healthy nerve could withstand an IOP at that level for weeks with little risk
of further nerve damage.

Pachymetry — Pachymetry is the measurement of corneal thickness; it can be


performed by ultrasound or other methods. Patients with thin corneas are at
higher risk for the development of open-angle glaucoma [76,77]. Ophthalmologists
may perform pachymetry in patients with suspected or diagnosed open-angle
glaucoma to further evaluate their risk for development or progression of open-
angle glaucoma [78]. Additionally, corneal thickness affects the results of
applanation tonometry, and pachymetry may adjust for this effect. (See
'Intraocular pressure' above.)

Newer technologies — Several newer technologies have been developed to


evaluate the optic disc, retinal nerve fiber layer, and visual field. These may aid in
the early detection of glaucoma, as well as other eye diseases.

● Optical coherence tomography – Optical coherence tomography (OCT),


Heidelberg retinal tomography (HRT), and scanning laser polarimetry are
noninvasive imaging techniques that analyze light reflected off the fundus
[79,80]. The need for pupil dilation varies with the particular device and the
part of the fundus being studied. These tests are well-tolerated by patients.
The devices generate a digital image and quantification of specific features of
optic nerve head anatomy.

One study comparing OCT, HRT, and scanning laser polarimetry with
conventional qualitative assessment of stereoscopic photographs of the optic
disc showed that the three newer technologies performed as well as, but not
better than, stereoscopic photographs [81]. Accuracy of results from
stereoscopic images is, however, dependent on the experience and skill of the
interpreter, whereas the newer technologies provide more quantitative data
that is less user-dependent.

Additionally, in highly myopic but otherwise healthy eyes where there may be
visual field defects and the optic nerve may have an abnormal appearance,
OCT may provide the clinician an additional tool to help distinguish these
findings from true glaucomatous changes [82].

● Visual field testing with brain computer interface – A portable brain-


computer interface (wearable, wireless, dry electroencephalogram and
electrooculogram systems) that assesses electrical brain responses to visual
field stimulation has shown promise as an alternative and potentially more
reliable means of diagnosing glaucoma compared with standard visual field
testing [83].

INFORMATION FOR PATIENTS

UpToDate offers two types of patient education materials, "The Basics" and
"Beyond the Basics." The Basics patient education pieces are written in plain
language, at the 5th to 6th grade reading level, and they answer the four or five key
questions a patient might have about a given condition. These articles are best for
patients who want a general overview and who prefer short, easy-to-read
materials. Beyond the Basics patient education pieces are longer, more
sophisticated, and more detailed. These articles are written at the 10th to 12th
grade reading level and are best for patients who want in-depth information and
are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We
encourage you to print or e-mail these topics to your patients. (You can also locate
patient education articles on a variety of subjects by searching on "patient info"
and the keyword(s) of interest.)

● Basics topics (see "Patient education: Age-related vision loss (The Basics)"
and "Patient education: Open-angle glaucoma (The Basics)")

SUMMARY AND RECOMMENDATIONS


● Open-angle glaucoma is an optic neuropathy characterized by progressive
peripheral visual field loss followed by central field loss in a typical pattern. It
is usually but not always in the presence of elevated intraocular pressure
(IOP). Increased aqueous production and/or decreased outflow are possible
mechanisms for elevation of intraocular pressure (figure 2). (See
'Classification' above.)

● Open-angle glaucoma is a leading cause of irreversible blindness in the world.


The major risk factors for developing open-angle glaucoma include age, black
race, family history, and elevated IOP. (See 'Epidemiology' above.)

● Individuals with open-angle glaucoma rarely experience symptoms. Some


patients are unaware of field loss even when it has progressed to central
"tunnel vision." Thus, open-angle glaucoma is generally detected incidentally
on comprehensive ophthalmic examination. (See 'Clinical presentation'
above.)

● We suggest that all individuals over age 40 be screened for glaucoma by


referral for comprehensive eye examination to an ophthalmologist or an
optometrist skilled in the assessment of the optic nerve and knowledgeable
about glaucoma (Grade 2B). Screening with individual tests (eg, fundus
examination or measurement or intraocular pressure alone) is insufficient to
evaluate for the presence of glaucoma. Earlier screening may be appropriate
for individuals with significant risk factors for glaucoma. (See 'Screening'
above.)

● Patients with abnormal cupping on fundus examination should be referred for


a comprehensive eye examination. (See 'Who should be referred for
comprehensive eye examination?' above.)

● Glaucoma is diagnosed in patients with characteristic nerve damage on


fundus examination and on visual field testing (picture 1A-B), typically in the
presence of elevated IOP. Some authorities consider either characteristic
optic nerve change OR visual field defects as sufficient criteria for diagnosis
of open-angle glaucoma. (See 'Diagnosis' above.)

REFERENCES

1. Weinreb RN, Khaw PT. Primary open-angle glaucoma. Lancet 2004; 363:1711.

2. Kingman S. Glaucoma is second leading cause of blindness globally. Bull


World Health Organ 2004; 82:887.

3. Tham YC, Li X, Wong TY, et al. Global prevalence of glaucoma and projections
of glaucoma burden through 2040: a systematic review and meta-analysis.
Ophthalmology 2014; 121:2081.

4. Sommer A, Tielsch JM, Katz J, et al. Racial differences in the cause-specific


prevalence of blindness in east Baltimore. N Engl J Med 1991; 325:1412.

5. Kapetanakis VV, Chan MP, Foster PJ, et al. Global variations and time trends in
the prevalence of primary open angle glaucoma (POAG): a systematic review
and meta-analysis. Br J Ophthalmol 2016; 100:86.

6. Quigley HA, Broman AT. The number of people with glaucoma worldwide in
2010 and 2020. Br J Ophthalmol 2006; 90:262.
7. Friedman DS, Wolfs RC, O'Colmain BJ, et al. Prevalence of open-angle
glaucoma among adults in the United States. Arch Ophthalmol 2004; 122:532.

8. Ang GS, Eke T. Lifetime visual prognosis for patients with primary open-angle
glaucoma. Eye (Lond) 2007; 21:604.

9. Richman J, Lorenzana LL, Lankaranian D, et al. Relationships in glaucoma


patients between standard vision tests, quality of life, and ability to perform
daily activities. Ophthalmic Epidemiol 2010; 17:144.

10. Haymes SA, Leblanc RP, Nicolela MT, et al. Risk of falls and motor vehicle
collisions in glaucoma. Invest Ophthalmol Vis Sci 2007; 48:1149.

11. Akbari M, Akbari S, Pasquale LR. The association of primary open-angle


glaucoma with mortality: a meta-analysis of observational studies. Arch
Ophthalmol 2009; 127:204.

12. Kwon YH, Fingert JH, Kuehn MH, Alward WL. Primary open-angle glaucoma. N
Engl J Med 2009; 360:1113.

13. Czudowska MA, Ramdas WD, Wolfs RC, et al. Incidence of glaucomatous
visual field loss: a ten-year follow-up from the Rotterdam Study.
Ophthalmology 2010; 117:1705.

14. Prum BE Jr, Rosenberg LF, Gedde SJ, et al. Primary Open-Angle Glaucoma
Preferred Practice Pattern(®) Guidelines. Ophthalmology 2016; 123:P41.

15. Mukesh BN, McCarty CA, Rait JL, Taylor HR. Five-year incidence of open-angle
glaucoma: the visual impairment project. Ophthalmology 2002; 109:1047.

16. Leske MC, Heijl A, Hyman L, et al. Predictors of long-term progression in the
early manifest glaucoma trial. Ophthalmology 2007; 114:1965.

17. Chauhan BC, Mikelberg FS, Balaszi AG, et al. Canadian Glaucoma Study: 2.
risk factors for the progression of open-angle glaucoma. Arch Ophthalmol
2008; 126:1030.

18. Khachatryan N, Medeiros FA, Sharpsten L, et al. The African Descent and
Glaucoma Evaluation Study (ADAGES): predictors of visual field damage in
glaucoma suspects. Am J Ophthalmol 2015; 159:777.

19. Varma R, Wang D, Wu C, et al. Four-year incidence of open-angle glaucoma


and ocular hypertension: the Los Angeles Latino Eye Study. Am J Ophthalmol
2012; 154:315.

20. Vajaranant TS, Wu S, Torres M, Varma R. The changing face of primary open-
angle glaucoma in the United States: demographic and geographic changes
from 2011 to 2050. Am J Ophthalmol 2012; 154:303.

21. Tielsch JM, Katz J, Sommer A, et al. Family history and risk of primary open
angle glaucoma. The Baltimore Eye Survey. Arch Ophthalmol 1994; 112:69.

22. Wiggs JL. Genetic etiologies of glaucoma. Arch Ophthalmol 2007; 125:30.

23. Zegers RH, Reinders EF, de Smet MD. Primary open-angle glaucoma: the
importance of family history and role of intraocular pressure. Med J Aust
2008; 188:312.

24. Ekström C. Risk factors for incident open-angle glaucoma: a population-based


20-year follow-up study. Acta Ophthalmol 2012; 90:316.

25. Heijl A, Leske MC, Bengtsson B, et al. Reduction of intraocular pressure and
glaucoma progression: results from the Early Manifest Glaucoma Trial. Arch
Ophthalmol 2002; 120:1268.

26. Nemesure B, Honkanen R, Hennis A, et al. Incident open-angle glaucoma and


intraocular pressure. Ophthalmology 2007; 114:1810.

27. Miglior S, Zeyen T, Pfeiffer N, et al. Results of the European Glaucoma


Prevention Study. Ophthalmology 2005; 112:366.
28. The Advanced Glaucoma Intervention Study (AGIS): 7. The relationship
between control of intraocular pressure and visual field deterioration.The
AGIS Investigators. Am J Ophthalmol 2000; 130:429.

29. Martínez-Belló C, Chauhan BC, Nicolela MT, et al. Intraocular pressure and
progression of glaucomatous visual field loss. Am J Ophthalmol 2000;
129:302.

30. Bengtsson B, Leske MC, Hyman L, et al. Fluctuation of intraocular pressure


and glaucoma progression in the early manifest glaucoma trial.
Ophthalmology 2007; 114:205.

31. Cedrone C, Mancino R, Cerulli A, et al. Epidemiology of primary glaucoma:


prevalence, incidence, and blinding effects. Prog Brain Res 2008; 173:3.

32. Zhao D, Cho J, Kim MH, Guallar E. The association of blood pressure and
primary open-angle glaucoma: a meta-analysis. Am J Ophthalmol 2014;
158:615.

33. Zhao D, Cho J, Kim MH, et al. Diabetes, fasting glucose, and the risk of
glaucoma: a meta-analysis. Ophthalmology 2015; 122:72.

34. Girkin CA, McGwin G Jr, McNeal SF, et al. Hypothyroidism and the
development of open-angle glaucoma in a male population. Ophthalmology
2004; 111:1649.

35. Miglior S, Torri V, Zeyen T, et al. Intercurrent factors associated with the
development of open-angle glaucoma in the European glaucoma prevention
study. Am J Ophthalmol 2007; 144:266.

36. Xu L, Wang H, Wang Y, Jonas JB. Intraocular pressure correlated with arterial
blood pressure: the beijing eye study. Am J Ophthalmol 2007; 144:461.

37. Grødum K, Heijl A, Bengtsson B. Risk of glaucoma in ocular hypertension with


and without pseudoexfoliation. Ophthalmology 2005; 112:386.
38. Topouzis F, Harris A, Wilson MR, et al. Increased likelihood of glaucoma at the
same screening intraocular pressure in subjects with pseudoexfoliation: the
Thessaloniki Eye Study. Am J Ophthalmol 2009; 148:606.

39. Marcus MW, de Vries MM, Junoy Montolio FG, Jansonius NM. Myopia as a
risk factor for open-angle glaucoma: a systematic review and meta-analysis.
Ophthalmology 2011; 118:1989.

40. Mansukhani SA, Barkmeier AJ, Bakri SJ, et al. The Risk of Primary Open-Angle
Glaucoma Following Vitreoretinal Surgery-A Population-based Study. Am J
Ophthalmol 2018; 193:143.

41. Júlvez LP, Del Castillo Sánchez JB, Feijoo JG, Rubio-Terrés C. Methodologic
quality of studies on prognostic factors for primary open-angle glaucoma
progression measured by visual field deterioration. J Glaucoma 2010; 19:587.

42. Chan TCW, Bala C, Siu A, et al. Risk Factors for Rapid Glaucoma Disease
Progression. Am J Ophthalmol 2017; 180:151.

43. Ernest PJ, Schouten JS, Beckers HJ, et al. An evidence-based review of
prognostic factors for glaucomatous visual field progression. Ophthalmology
2013; 120:512.

44. Susanna R Jr. Unpredictability of glaucoma progression. Curr Med Res Opin
2009; 25:2167.

45. Kountouras J, Zavos C, Chatzopoulos D. Primary open-angle glaucoma:


pathophysiology and treatment. Lancet 2004; 364:1311.

46. Ray K, Mookherjee S. Molecular complexity of primary open angle glaucoma:


current concepts. J Genet 2009; 88:451.

47. Wierzbowska J, Wierzbowski R, Stankiewicz A, et al. Cardiac autonomic


dysfunction in patients with normal tension glaucoma: 24-h heart rate and
blood pressure variability analysis. Br J Ophthalmol 2012; 96:624.

48. Heijl A, Bengtsson B, Hyman L, et al. Natural history of open-angle glaucoma.


Ophthalmology 2009; 116:2271.

49. Peters D, Bengtsson B, Heijl A. Lifetime risk of blindness in open-angle


glaucoma. Am J Ophthalmol 2013; 156:724.

50. Summaries for patients. Screening for glaucoma: U.S. Preventive Services
Task Force recommendation statement. Ann Intern Med 2013; 159:I.

51. Vass C, Hirn C, Sycha T, et al. Medical interventions for primary open angle
glaucoma and ocular hypertension. Cochrane Database Syst Rev 2007;
:CD003167.

52. Maier PC, Funk J, Schwarzer G, et al. Treatment of ocular hypertension and
open angle glaucoma: meta-analysis of randomised controlled trials. BMJ
2005; 331:134.

53. Henderson P, Labbe T, Kass MA. Ocular hypertension. In: Ophthalmology, 3rd
ed, Yanoff M, Duker JS (Eds), Mosby Elsevier, 2009.

54. Tuulonen A. Cost-effectiveness of screening for open angle glaucoma in


developed countries. Indian J Ophthalmol 2011; 59 Suppl:S24.

55. Hernández R, Rabindranath K, Fraser C, et al. Screening for open angle


glaucoma: systematic review of cost-effectiveness studies. J Glaucoma 2008;
17:159.

56. Burr JM, Mowatt G, Hernández R, et al. The clinical effectiveness and cost-
effectiveness of screening for open angle glaucoma: a systematic review and
economic evaluation. Health Technol Assess 2007; 11:iii.

57. Vaahtoranta-Lehtonen H, Tuulonen A, Aronen P, et al. Cost effectiveness and


cost utility of an organized screening programme for glaucoma. Acta
Ophthalmol Scand 2007; 85:508.

58. Hatt S, Wormald R, Burr J. Screening for prevention of optic nerve damage
due to chronic open angle glaucoma. Cochrane Database Syst Rev 2006;
:CD006129.

59. Feder RS, Olsen TW, Prum BE Jr, et al. Comprehensive Adult Medical Eye
Evaluation Preferred Practice Pattern(®) Guidelines. Ophthalmology 2016;
123:P209.

60. Moyer VA, U.S. Preventive Services Task Force. Screening for glaucoma: U.S.
Preventive Services Task Force Recommendation Statement. Ann Intern Med
2013; 159:484.

61. Canadian Task Force on the Periodic Health Examination. Canadian guide to c
linical preventive health care, Canada Communication Group, Ottawa 1994. p.
932.

62. Wood CM, Bosanquet RC. Limitations of direct ophthalmoscopy in screening


for glaucoma. Br Med J (Clin Res Ed) 1987; 294:1587.

63. Zein WM, Bashshur ZF, Jaafar RF, Noureddin BN. The distribution of visual
field defects per quadrant in standard automated perimetry as compared to
frequency doubling technology perimetry. Int Ophthalmol 2010; 30:683.

64. Tielsch JM, Katz J, Singh K, et al. A population-based evaluation of glaucoma


screening: the Baltimore Eye Survey. Am J Epidemiol 1991; 134:1102.

65. Johnson CA, Keltner JL, Cello KE, et al. Baseline visual field characteristics in
the ocular hypertension treatment study. Ophthalmology 2002; 109:432.

66. Ocular Hypertension Treatment Study Group and the European Glaucoma
Prevention Study Group. The accuracy and clinical application of predictive
models for primary open-angle glaucoma in ocular hypertensive individuals.
Ophthalmology 2008; 115:2030.
67. Hollands H, Johnson D, Hollands S, et al. Do findings on routine examination
identify patients at risk for primary open-angle glaucoma? The rational clinical
examination systematic review. JAMA 2013; 309:2035.

68. Harper R, Reeves B. The sensitivity and specificity of direct ophthalmoscopic


optic disc assessment in screening for glaucoma: a multivariate analysis.
Graefes Arch Clin Exp Ophthalmol 2000; 238:949.

69. Qiu M, Boland MV, Ramulu PY. Cup-to-Disc Ratio Asymmetry in U.S. Adults:
Prevalence and Association with Glaucoma in the 2005-2008 National Health
and Nutrition Examination Survey. Ophthalmology 2017; 124:1229.

70. Johnson LN, Baloh FG. The accuracy of confrontation visual field test in
comparison with automated perimetry. J Natl Med Assoc 1991; 83:895.

71. Omoti AE, Okeigbemen VW, Waziri-Erameh JM. Current concepts in the
diagnosis of primary open angle glaucoma. West Afr J Med 2009; 28:141.

72. Leske MC. The epidemiology of open-angle glaucoma: a review. Am J


Epidemiol 1983; 118:166.

73. Armaly MF, Krueger DE, Maunder L, et al. Biostatistical analysis of the
collaborative glaucoma study. I. Summary report of the risk factors for
glaucomatous visual-field defects. Arch Ophthalmol 1980; 98:2163.

74. Brandt JD, Beiser JA, Kass MA, Gordon MO. Central corneal thickness in the
Ocular Hypertension Treatment Study (OHTS). Ophthalmology 2001;
108:1779.

75. Teng C, Gurses-Ozden R, Liebmann JM, et al. Effect of a tight necktie on


intraocular pressure. Br J Ophthalmol 2003; 87:946.

76. Kass MA, Heuer DK, Higginbotham EJ, et al. The Ocular Hypertension
Treatment Study: a randomized trial determines that topical ocular
hypotensive medication delays or prevents the onset of primary open-angle
glaucoma. Arch Ophthalmol 2002; 120:701.

77. European Glaucoma Prevention Study (EGPS) Group, Miglior S, Pfeiffer N, et


al. Predictive factors for open-angle glaucoma among patients with ocular
hypertension in the European Glaucoma Prevention Study. Ophthalmology
2007; 114:3.

78. Herndon LW, Weizer JS, Stinnett SS. Central corneal thickness as a risk factor
for advanced glaucoma damage. Arch Ophthalmol 2004; 122:17.

79. Hazin R, Hendrick AM, Kahook MY. Primary open-angle glaucoma: diagnostic
approaches and management. J Natl Med Assoc 2009; 101:46.

80. Li G, Fansi AK, Boivin JF, et al. Screening for glaucoma in high-risk populations
using optical coherence tomography. Ophthalmology 2010; 117:453.

81. Badalà F, Nouri-Mahdavi K, Raoof DA, et al. Optic disk and nerve fiber layer
imaging to detect glaucoma. Am J Ophthalmol 2007; 144:724.

82. Baek SU, Kim KE, Kim YK, et al. Development of Topographic Scoring System
for Identifying Glaucoma in Myopic Eyes: A Spectral-Domain OCT Study.
Ophthalmology 2018; 125:1710.

83. Nakanishi M, Wang YT, Jung TP, et al. Detecting Glaucoma With a Portable
Brain-Computer Interface for Objective Assessment of Visual Function Loss.
JAMA Ophthalmol 2017; 135:550.

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