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Glaucoma Clinical Optic Disc Evaluation

Clinical Optic Disc Evaluation in Glaucoma

A l e x a n d r e S C R e i s , 1,2 A n d r e w T o r e n 3 a n d M a r c e l o T N i c o l e l a 4

1. Research Fellow, Department of Ophthalmology and Visual Sciences, Dalhousie University, Halifax, Nova Scotia, Canada;
2. Research Fellow, Department of Ophthalmology, University of Sao Paulo, Brazil; 3. Resident; 4. Associate Professor,
Department of Ophthalmology and Visual Sciences, Dalhousie University, Halifax, Nova Scotia, Canada

Abstract
Examination of the optic nerve head (ONH) is essential for the diagnosis of glaucoma and assessment of its progression. Slit-lamp
biomicroscopy with a handheld lens is the best method of ONH examination since it provides good stereopsis and magnification. ONH stereo
photographs are complementary and may identify findings missed on slit-lamp examination. As a result of its subjective nature, a standardised
approach should be utilised for clinical ONH evalaution, including an assessment of ONH size and careful evaluation of the neuroretinal
rim contour, the presence of retinal nerve fibre layer (RNFL) defects and optic disc haemorrhages. Other aspects, such as peripapillary
chorioretinal atrophy, vessel alterations and asymmetry between fellow eyes, might help differentiate normal from glaucomatous eyes.
Progressive changes in the appearance of the ONH or RNFL are best identified with optic disc photographs or automated devices. The findings
of clinical ONH evaluation are of greater value when corroborated with other aspects of clinical examination and clinical test.

Keywords
Optic disc, open-angle glaucoma, ophthalmoscopy, stereo photography, retinal nerve fibre layer, clinical examination, optic disc haemorrhage

Disclosure: The authors have no conflicts of interest to declare

Received: 15 October 2011 Accepted: 7 March 2012 Citation: European Ophthalmic Review, 2012;6(2):92–7
Correspondence: Marcelo T Nicolela, Department of Ophthalmology and Visual Sciences, Dalhousie University, 1276 South Park Street, Room 2035, Halifax, NS, Canada,
B3H 2Y9. E: [email protected]

The detection of structural damage to the optic nerve head (ONH) is techniques and clinical signs linked to glaucomatous damage and
central to the diagnosis of glaucoma and is extremely important for their relevance to patient care.
monitoring patients at risk of glaucoma or with established disease.
Glaucoma, by definition, is an optic neuropathy and therefore specific The Normal Optic Nerve Head
attention must be directed to the examination of the optic nerve. The The ONH, or optic disc, is the location where the axons from the
ONH is the site at which the dropout of retinal ganglion cells is retinal ganglion cells converge to exit the eye through the scleral
identified most easily with current clinical techniques and is postulated canal. Besides axons, the ONH consists of blood vessels, glia and
as the primary site for damage in glaucoma.1–3 Careful evaluation of the connective tissue. The size of the scleral canal governs the size of the
ONH and peripapillary tissues can usually identify early glaucomatous ONH: eyes with small canals have small optic discs (commonly seen
damage before detectable visual field loss occurs.4,5 in high hyperopia) and those with large canals have large discs
(commonly seen in high myopia). Jonas et al. measured the size of the
The main difficulties in the clinical assessment of the ONH relate to its scleral canal in 107 enucleated human donor eyes.6 They found a high
inherent subjectivity, and to the overlapping spectrum and large inter-individual variability with an average area of 2.59 mm2, ranging
diversity in the appearance of normal and diseased discs. Early from 0.68 to 4.42 mm2. The edges of the scleral canal define the optic
progressive glaucomatous changes in the ONH are subtle and may be disc margin, which is clinically visible as a whitish circular band at
missed without careful serial examinations of the individual’s optic the edge of the optic disc. The ONH is usually vertically oval, with
disc. Additionally, there is currently no quick, simple, inexpensive, an average dimension of 1.92 ± 0.29 mm (0.96–2.91 mm) vertically,
specific, sensitive and objective method of ONH analysis by which and 1.76 ± 0.31 mm (0.91–2.61 mm) horizontally, and a surface area
glaucoma is reliably diagnosed and progression detected. of 2.69 ± 0.70 mm2 (0.80–5.54 mm2).6 Jonas and Papastathopoulos
proposed that in routine practice, the clinician need not measure the
Clinicians should be aware that optic disc evaluation requires not only exact numerical value of the disc size, but instead use a quick, crude
an understanding of the normal disc appearance and the pathological estimate of whether the size of the disc in question is of average size,
process of glaucoma, but also training and clinical experience. It is smaller than average or larger than average (Figure 1).7
essential to associate the findings of the ONH evaluation with those of
the clinical history, clinical examination (such as refractive error, The optic cup is a central pale depression in the ONH not occupied
presence of afferent pupillary defect and intraocular pressure [IOP]) by neural tissue. The pale colour of the cup is a result of exposure
and visual field tests. This review article highlights the most relevant of the lamina cribrosa and loss of glial tissue. There is a physiological
aspects of clinical ONH evaluation: the relevant anatomy, examination relationship between optic disc size and cup size, so that large optic

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discs have large cups, and small discs should have small, or absent, Figure 1: Examples of the Normal Sizes of Optic Discs
cups.8 The neuroretinal rim is the tissue in between the outer edge
of the cup and the optic disc margin. Normally, circumlinear blood A B C

vessels rest on the neuroretinal rim tissue. Therefore, in most cases,

the boundaries of the cup are best determined by following the
trajectory of these vessels inside the ONH. The normal healthy
rim should have an orange or pink colour and no localised areas
of thinning. In addition, in normal eyes the inferior rim is usually
thicker than the superior rim, which is thicker than the nasal rim,
A: Small (disc area, 1.3 mm2); B: Medium (disc area, 2.4 mm2); C: Large (disc area, 3.8 mm2).
and the temporal rim is the thinnest (this is known as the ‘ISNT’ The disc areas were measured with confocal scanning laser tomography.
rule – Figure 2.9
Figure 2: Normal Optic Disc (A) and Glaucomatous
The distribution of the axons as they pass across the retina to enter Optic Disc (B)
the ONH is the key for the interpretation of visual field loss in relation
to the ONH cupping in glaucoma.10,11 The retinal nerve fibre layer A B
(RNFL) is composed of axons of retinal ganglion cells, which converge
to the ONH with three fundamental features:12 S

• the papillomacular bundle, which has a direct course from the T N

macula to the ONH;
• the fibres arising from the nasal retina, which also follow a
I
relatively straight course entering the nasal aspect of the ONH; and
• the peripheral nerve fibres arising from the retina temporal to the
macula, which have an arcuate course around the papillomacular
bundle to reach the superior and inferior aspects of the ONH. A: The outer dashed line outlines the optic disc margin; the inner dashed line outlines the
optic disc cup. The inferior (I) neuroretinal rim is thickest, followed in decreasing order of
thickness by the superior (S), nasal (N) and temporal (T) rims, obeying the ‘ISNT rule’;
Moreover, a horizontal raphe separates the axons in the superior
B: There is an overall thinning of neuroretinal rim tissue and prominent thinning in the
retina from those in the inferior retina. inferotemporal sector (particularly between 5 and 6 o’clock, where the rim is almost
absent), so not obeying the ‘ISNT rule’.

Examination Techniques
There are several techniques to examine the ONH clinically, including that 84 % of 128 cases of ODHs were detected on disc photographs
direct ophthalmoscopy, indirect ophthalmoscopy and slit-lamp only and not on the clinical examination.15
biomicroscopy with a contact lens (such as a Goldman lens), handheld
lens (+66, +78 or +90 dioptre [D] aspheric lenses) or a Hruby lens. The Evaluation of Reproducibility and Accuracy
use of a slit lamp with a handheld lens is, in the authors’ view, Several studies evaluated the accuracy of the subjective ONH
the preferred method. It provides a good balance between the examination, as well as the intra- and interobserver agreement in
quality of stereopsis and magnification, besides being comfortable to optic disc evaluation. As expected, the intraobserver reproducibility
patients and easy to incorporate in the routine slit-lamp examination. is consistently higher than that of the interobserver (kappa [range]
Slit-lamp biomicroscopy with a contact lens provides a better view 0.69–0.96 versus kappa [range] 0.20–0.84, respectively) in studies
and has the advantage of providing a non-inverted image. However, to evaluate agreement among observers in the estimation of optic
it is less comfortable to patients, requires the use of a coupling gel disc parameters.16,17 Similarly, substantial variability exists in the
and takes a longer time to perform. The clinical examination of a interpretation of optic disc change over time, even among expert
glaucoma patient should include pupil dilation as this allows a better observers, with kappa values ranging from 0.50 to 0.96 for
stereoscopic view and adequate RNFL examination.13 intraobserver and from 0.55 to 0.81 for interobserver agreement.18–24
Observers reading photographs in the context of major clinical
The direct ophthalmoscope is portable, cheap and offers a magnified trials are generally reported to have low interobserver variability and
view, although it only provides a monocular non-stereoscopic view. excellent reproducibility.18–20 However, a number of other studies found
The indirect ophthalmoscope is also portable and is useful to a poor-to-moderate agreement among glaucoma experts when they
examine children and unco-operative patients. In some cases it may independently assessed disc changes over time.21–24
be the only method to examine adults with severe lens opacity.
However, the low magnification of the indirect ophthalmoscope is a Most of these studies concentrated on the distinction between
serious disadvantage. glaucoma from normal discs25–29 and the detection of progression.27.30–35
The results depend on the expertise and experience of the
Optic disc photographs provide complementary information to the examiners,29,34,36 the ethnicity and optic disc phenotypes,37 quality of
clinical examination, as well as an objective record for future stereo photography33 and presence of pupillary dilation.13 How these
comparison. Photographic documentation, preferably stereo factors impact the accuracy of a single clinician’s examination in a
photography, is highly recommended since features such as the non-research setting remains unknown, although this is crucial to
presence of RNFL defects and optic disc haemorrhages (ODHs) patient care. In general, the sensitivity of the clinical examination for
can easily be missed during the clinical examination.14 For instance, the detection of early to moderate glaucoma (with early visual
data from the Ocular Hypertension Treatment Study (OHTS) showed field defects) is good, as its sensitivity to detect progression in

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Glaucoma Clinical Optic Disc Evaluation

Figure 3: Examples of Glaucomatous Optic Disc Features • identification of the optic disc margin;
• estimating the size of the neuroretinal rim;
A B • inspecting the RNFL; and
• noting additional features such as peripapillary atrophy
and ODHs.

Optic Disc Margin

Determination of the optic disc margin is one of the most important
aspects of the clinical examination, although it can be very difficult in
individuals with high myopia (Figure 3C) and eyes with significant
peripapillary atrophy (Figure 3D). The optic disc margin is defined
as the innermost border of reflective tissue that is internal to any
pigmented tissue and within which only neural tissue is present.43,44
C D
It should be the first aspect of a systematic optic nerve examination,
since variations in disc size alter significantly the estimation
of glaucomatous damage. A large optic disc might appear to be
glaucomatous because large discs normally have large cups and
apparently thin neuroretinal rims (although the total area of the
neuroretinal rim is usually larger in large discs). However, a small
optic disc might hide neuroretinal rim loss, as sometimes even a
small cup in a small disc is abnormal.

With a slit-lamp beam measurement scale, the size of the disc

A: Inferotemporal focal absence of neuroretinal rim tissue (notch) and superotemporal in vertical and horizontal dimensions can be calculated using
focal thinning of the neuroretinal rim tissue (notching) with the corresponding retinal
nerve fibre layer defect (between arrowheads); B: Diffuse rim loss, with concentric cup conversion scales for the corresponding lens (×1.0 for +60 D, ×1.1 for
enlargement and no localised areas of loss or pallor; C: Myopic disc with crescent +78 D and ×1.3 for +90 D lens).45 The disc size can also be estimated
temporal and inferior neuroretinal rim tissue; D: Shallow cupping extending to the optic
disc margin, marked areas of peripapillary atrophy and choroidal sclerosis. The dotted with the direct ophthalmoscope. The 50 aperture of the Welch–Allyn
line indicates the border between the beta zone (internally) and the alpha zone ophthalmoscope produces a circular spot with a diameter of 1.5 mm
(externally – indicated by arrowheads).
and an area of 1.77 mm2, which is slightly smaller than an optic disc
of average size.46
early-to-moderate disease. The majority of these studies emphasise
the need for a standardised methodology in assessing optic disc Neuroretinal Rim
photographs, which can increase the likelihood of detecting change Loss of neuroretinal rim tissue should be examined carefully for both
over time. diffuse and focal loss, which may be evident as thinning, notching
and nasal cupping. Kinking of the blood vessels within the ONH helps
Imaging instruments for the detection of glaucoma, such as optical to identify the margin of the rim tissue. In myopic eyes or those with
coherence tomography, scanning laser polarimetry and confocal shallow cupping it can be quite difficult (Figures 3C and 3D). The
scanning laser tomography (CSLT), perform as well as, but not better inherent variability in size and shape of the optic disc among normal
than, qualitative evaluation of optic disc stereo photographs by experts individuals and patients with glaucoma impairs the clinician’s ability to
to detect early perimetric glaucoma.25–29 The use of such devices should determine rim loss with high accuracy. Detection of rim loss over time
be considered when expert advice is not available. Some studies has a higher specificity than cross-sectional detection of glaucoma,
that compared CSLT to glaucoma specialists in detecting ONH changes since detection over time does not depend on the inter-individual
over time suggest that the automated technology at least meets and variability of optic disc appearance.
possibly exceeds the sensitivity of the subjective ONH assessment.22,38
Automated imaging devices offer exciting opportunities to identify Jonas et al originally described the ISNT rule.6 This morphometric
glaucoma progression more accurately, which might improve even characteristic is not followed in patients with glaucoma.9,47 However,
more with greater technological advances on the quality of the images one study found that early glaucomatous eyes retained the same
and better tools to analyse the information provided. However, optic neuroretinal rim area configuration as seen in control eyes with the
disc photography currently remains the gold standard in evaluating ISNT rule, which suggests that the loss of neuroretinal rim in most
glaucoma progression.39,40 early glaucoma subjects occurs in a diffuse manner so that they retain
the same neuroretinal area configuration of normal subjects.48
A Standardised Approach
It is essential that the examiner have a strategy and a standardised Cup-to-disc Ratio
approach in mind when examining the ONH. It is necessary to acquire Estimation of the size of the cup is usually made by comparison with
the most information in the shortest possible time, as the examination the size of the disc, and given as the ratio of the vertical diameter
can be uncomfortable for the patient. A recent publication by O’Neill of the cup to the vertical diameter of the disc (vertical cup to-disc
et al. showed that glaucoma subspecialists adopt a more systematic ratio or CDR). This is the most frequently performed assessment by
behaviour when examining the optic nerve and RNFL compared with clinicians of the overall glaucomatous optic disc damage. However,
that of trainees.41 At least four main aspects need to be included in a the CDR has only limited value in the identification of glaucoma, in
systematic examination:42 part because of the wide variation in the size of the optic disc and

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Clinical Optic Disc Evaluation in Glaucoma

Figure 4: Patient with Recurrent Optic Disc Haemorrhage in the Inferotemporal Sector where there is a Significant
Thinning of the Neuroretinal Rim Tissue

Baseline 1 year 2 years 3 years 4 years

consequently of the cup in the normal population. In addition, there is in only 15 % of the normal eyes.56 In that same study, both alpha
a significant variability among glaucoma specialists in the evaluation of and beta zones were larger in glaucoma patients, but only the beta
CDR from stereoscopic photographs.16,35 Tielsch et al.49 demonstrated zone was more frequent in glaucoma patients than in healthy
that 17 to 19 % of CDR estimates made by two different glaucoma controls. Tezel et al. suggested that the presence, as well as the
specialists differed by 0.2 disk diameters or more. When adjusted for area and extension, of peripapillary atrophy along the optic disc
disc size the CDR has been shown to have an improved diagnostic border, especially of the beta zone variety, is associated with
ability in early glaucoma.50 subsequent progression to glaucomatous damage.57,58 However, See
et al. showed no difference in the rates of change of peripapillary
A careful observation of rim contour as opposed to cup size or CDR atrophy area as measured with CSLT between glaucoma patients
is a better way to detect glaucomatous optic disc damage. Matching and healthy controls.59
of clues inside and outside the optic disc is also useful, such as
confirming the presence of a RNFL defect in an area where the Optic Disc Haemorrhages
neuroretinal rim is suspicious (Figure 3A). The association between ODHs and glaucoma, particularly in
glaucomatous eyes with lower levels of IOP, is well established.60–62
Retinal Nerve Fibre Layer The presence of ODHs is a sign of the development of glaucoma15 and
Assessment of the RFNL requires the detection of subtle clues. This is a strong indicator of functional and structural progression.61,63–67
made possible through good technique and practice. Red-free light Siegner and Netland reported close to an 80 % rate of optic
is best for evaluation of the RNFL as the short wavelength light brings disc progression following an ODH in patients with glaucoma, with
the anterior layer into better focus. There is considerable variation progression occurring on average two years after the haemorrhage.68
in the RNFL among the general population, but usually there is In patients with ocular hypertension the rate of conversion to
considerable symmetry between the two eyes of the same patient.51,52 glaucoma seems to be lower. Only 14 % of patients from the OHTS
The classic localised defect of the RNFL associated with glaucoma is with disc haemorrhage developed an open-angle glaucoma endpoint,
seen as a darkened wedge that extends from a corresponding which occurred after a mean follow-up of approximately one year.15
thinning in the neuroretinal rim tissue (Figure 3A). Diffuse RNFL
defects can also be seen in glaucoma, although they are difficult Identifying disc haemorrhages requires meticulous inspection by the
to detect with biomicroscopy.10 examiner. Stereo photographs help to identify small haemorrhages
near blood vessels. Disc haemorrhages usually occur at the
Peripapillary Chorioretinal Atrophy inferotemporal margin (Figure 4), and there is considerable spatial
Peripapillary chorioretinal atrophy is significantly larger and occurs correlation between ODHs and neuroretinal rim tissue notches,
more often in glaucomatous eyes than in normal eyes, or in eyes RNFL defects62 and visual field loss.69 Often they recur in the
with ocular hypertension.53,54 Furthermore, it is more often seen in same area of the disc until a notch is formed, and then occur
glaucomatous eyes with shallow cupping than in glaucomatous at other areas of the disc where the rim is still normal.60,62,70,71 They
eyes with deep and steep excavation.55 Peripapillary atrophy is divided are transient and usually disappear after 1–6 months.72 The presence
into the central beta zone and the peripheral alpha zone. The alpha of ODHs is probably an important feature in monitoring treatment
zone is characterised by an irregular hypo- and hyperpigmentation, response. A recent report from the Early Manifest Glaucoma Trial
associated with thinning of the chorioretinal tissue layer. Features reported that IOP-reducing treatment was unrelated to the presence
of the beta zone are marked atrophy of the retinal pigment or frequency of disc haemorrhages.61 However, most other studies
epithelium and choriocapillaris and thinning of the chorioretinal suggest that IOP-lowering treatment decreases the frequency
tissues with good visibility of the large choroidal vessels and of ODHs.73–75
sclera.56 If both zones are present, the beta zone is always closer
to the optic disc than the alpha zone. The alpha zone and beta Other Glaucoma Features
zone have to be differentiated from the scleral crescent in eyes Besides the features mentioned above, there are other optic disc
with high myopia and from the inferior scleral crescent in eyes with features that the clinician should be alert to in both the diagnosis
tilted optic discs. and follow-up of patients with glaucoma. Primary open-angle
glaucoma (POAG) is usually bilateral, but frequently asymmetric, and
Jonas et al. reported that some alpha zone occurred in almost every a CDR asymmetry of 0.2 or greater has long been held to be
normal eye (85 %), in contrast with beta zone, which was present suggestive of glaucoma.76 However, data from the Blue Mountains

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Figure 5: Glaucomatous Progression over 10 Years these individuals converted to glaucoma on the basis of optic disc
change alone.85 In the European Glaucoma Prevention Study, the
A B cumulative probability for conversion to glaucoma in the placebo
group was 14 % over 60 months, but only 37 % of these converted
on the basis of optic disc changes.23 This difference in optic disc
progression rates is probably influenced by the different criteria used
to determine optic disc progression.

Optic disc progression is reported as thinning of the neuroretinal

rim tissue in either a diffuse or localised pattern (Figure 5). The
position and location of the deflection of blood vessels should
be compared across all available photographs to uncover changes
that may indicate neuroretinal rim loss in a specific sector. Baring
A: Normal appearance of an optic disc with increased peripapillary atrophy;
B: Generalised thinning of the neuroretinal rim tissue, markedly in the inferior quadrant. of the circumlinear vessel or progressive nasalisation of vessels
There is nasalisation of the blood vessels and an increase in the chorioretinal peripapillary can also occur with progression (Figure 5). 86,87 Changes in the optic
atrophy area.
disc are more easily observed in early cases of glaucoma where
the dynamic range for change is greater. In more advanced stages
population study showed that cup-to-disc asymmetry is significantly of disease, the optic disc may be too damaged for the examiner
associated with asymmetry of optic disc size and that asymmetry to establish further thinning of the neuroretinal rim. When looking
alone was not useful in identifying patients with glaucoma. 77 for progression, clinicians should be cognisant of the most probable
Therefore, when assessing the asymmetry of cup or neuroretinal rim locations to show rim area loss in glaucoma: the inferotemporal
between eyes it is important to examine whether or not the optic is the first to present loss, followed by the superotemporal,
disc size and shape are symmetrical. Asymmetry of CDRs should be temporal, inferonasal, superonasal and finally the nasal sector.
correlated to asymmetry in other parts of the clinical examination as However, individual cases can show progression that deviates from
well (i.e., IOP, visual field sensitivity, quantitative measurements of this sequence. 88,89
the optic nerve or RNFL).
Methods to assess changes of the optic disc over time include the
Normally, circumlinear blood vessels rest on neuroretinal rim use of optic disc drawing comparisons, sequential optic disc
tissue within the optic disc. Blood vessel changes over time usually photographs (mono or stereo) and quantitative parameters on
occur as a result of cupping and excavation, and might be automated devices, such as the CSLT. Glaucomatous changes in
particularly helpful in identifying progressive glaucomatous the optic disc occur slowly over several years, and even very
nerve damage. Common glaucomatous changes in vessels include well drawn representations or detailed descriptions of the optic
vessel bayoneting and baring of the circumlinear blood vessels. disc are insufficient to detect small structural progression. Optic disc
Bayoneting refers to the sharp 90° turn, or occasionally more than photographs remain the gold standard for documentation and
90°, turn that a blood vessel develops as it dips into an acquired monitoring of optic disc appearance, although automated devices
pit of neuroretinal rim tissue loss and then emerges out onto the such as the CSLT can probably provide equal or superior information
disc edge. Baring of circumlinear vessels refers to the unsupported on structural changes of the optic disc over time when the results are
appearance that ONH vessels acquire when no neuroretinal rim is analysed correctly.22,38,90 Objective recording of the appearance of the
directly in contact with them. Nasalisation of the ONH vessels optic disc is therefore essential to monitor a patient with glaucoma
is described as one of the ophthalmoscopic signs of glaucomatous or suspected of having the disease.
damage. 78 However, as the blood vessels commonly enter and
leave the eye along the nasal border of the cup, they will appear Automated imaging devices might have other practical advantages
nasalised when the cup is large, either physiologically or as a result over disc photographs for the assessment of progression: faster and
of glaucoma. 79,80 easier image acquisition permits more frequent examinations to
allow estimates of the rate of change; and the possibility to utilise
Visibility of the lamina cribrosa pores (lamina dot sign) is more objective and statistical ways to evaluate changes in the optic
common in eyes with glaucomatous damage to the optic disc than in disc and RNFL structure. Further improvements in hardware to
normal eyes.81 This association, however, is almost entirely because capture data with increasingly higher resolution, and in software
of an increased visibility associated with larger vertical CDR and optic to analyse the data using increasingly complex methods, may allow
disk size82 The size and shape of lamina cribrosa pores has been the clinician to make quicker and more informed clinical decisions
reported to predict glaucomatous visual field loss,83 although the in the near future.22
strength of this sign is variable.84
Conclusion
Detection of Progression Early structural damage in glaucoma remains a hallmark of the
The rate of optic nerve change is extremely variable among different diagnosis of glaucoma and detection of progression. The interpretation
patients. It is difficult to define rates of change because of the is subjective and requires complex consideration of numerous factors,
generally slow nature of the disease. Additionally, there is no emphasising the importance of a high-quality clinical examination.
universally accepted method to assess change. For instance, in the While newer technologies show promise in offering better detection of
OHTS, the cumulative probability for conversion from ocular glaucomatous change over time, the subjective examination remains
hypertension to glaucoma was 9.5 % over 60 months and 67 % of vital in managing glaucoma in clinical practice. n

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