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Literature review current through: May 2019. | This topic last updated: Nov 07, 2018.
INTRODUCTION
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
● 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].
Risk factors — The major risk factors for developing open-angle glaucoma include
age, black race, family history, and elevated intraocular pressure (IOP) [12-14].
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].
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'.)
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.)
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 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].
DIAGNOSIS
● 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.
Diagnostic tests
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.)
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.
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].
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)")
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