The best method readily available to the clinician for performing this examination is high plus lens fundus biomicroscopy. Optimal magnification can be achieved by using a +60D lens which provides 1.5 times the magnification of a 90D lens. During this examination the patient's pupils must be maximally dilated with a combination of mydriatic agents such as 1% Tropicamide and 2.5% Phenylephrine.
EYE CANCER.pptx prepared by Neha kewat digital learning
Evaluating the optic nerve head in glaucoma
1. Evaluating the Optic Nerve Head in Glaucoma
The best method readily available to the clinician for performing this examination is
high plus lens fundus biomicroscopy. Optimal magnification can be achieved by using
a +60D lens which provides 1.5 times the magnification of a 90D lens. During this
examination the patient's pupils must be maximally dilated with a combination of
mydriatic agents such as 1% Tropicamide and 2.5% Phenylephrine.
It is important to remember that the image seen through a high plus lens is a virtual
image, and will be inverted with the right on the left, and the top on the bottom.
The normal optic nerve diameter varies in size from 1.2 mm to 2.5 mm with the
average being 1.88 mm vertically and 1.77 mm horizontally. The area of the disc varies
in normal patients from 0.92 to 5.54 square millimeters.
This variation in normal optic disc sizes can affect the cup/disc ratio in two ways. First
in a larger disc, there is more area for the nerve fibers to fill, allowing for a larger cup.
Secondly, the size of the disc is used as the denominator of the ratio. It has been
suggested, that patients with larger optic disc size may have an increased
susceptibility to glaucoma. However, a recent study found no correlation between the
size of the optic nerve and the likelihood of visual field loss. Nevertheless, the
importance of a large or small C/D can only be determined when considered in the
context of the size of the optic nerve head. Therefore, it is essential to evaluate the
size of the optic disc in all patients who are glaucoma suspects. An easy way to do this
clinically is to use the direct ophthalmoscope. A relatively normal sized optic nerve
head will be approximately equal to the spot size projected onto the retina through the
small aperture of the scope.
Knowing that each optic nerve head is normal in size also allows the examiner to be
sure that any asymmetry in C/D ratios between the two eyes of a patient is due to a
difference in the number of axons. Asymmetry in C/D size between the two eyes of a
normal patient has been shown to be rare. Cup-to-disc ratios differ by 0.2 or less in
96% of normal eyes, so asymmetry of more than 0.2 in a patient with symmetrical disc
sizes and no anisometropia, greatly increases the suspicion of glaucoma.
A study of cup to disc ratios performed in the 1960's indicated that only 7% of the
normal population had C/D ratios of 0.5 or greater. This study also indicated that 86%
of normal C/D ratios were below 0.4. Because of this study, anyone with a C/D greater
than 0.4 was automatically considered a glaucoma suspect.
Recent studies have changed our views. Evaluation of the contour of the cup with
stereoscopic viewing and image analyzers has shown that the average C/D ratio is
quite a bit larger than previously thought. In a recent study of normal individuals, the
average horizontal C/D ratio was found to be 0.47 in Caucasians and 0.57 in African-
Americans. The average vertical meridians found in this study, were 0.49 for
Caucasians and 0.56 for African-Americans. Another recent study supports these
results with averages of 0.51 in the horizontal and 0.43 in the vertical meridian. This
investigation indicates that in order to include 84% of the population, C/D ratios less
2. than 0.74 horizontally and 0.64 vertically should be considered normal. Also, in
this study, patients with steeply bordered cups were found to have even larger average
C/D's, with a horizontal average of 0.65 and a vertical average of 0.57.
These studies also show that when determining the amount of cupping, it is very
important to evaluate the contour and not the pallor of the cup. This is because the
optic nerve head damaged by glaucoma typically has cupping which is larger than the
pallor, whereas the normal eye has cupping equal to the area of pallor.
Because it is recognized that the average size of a normal C/D ratio is larger than
previously accepted and that a large cup/disc size is not definitive as a diagnosis of
glaucoma, less attention is being placed on the size of the cup, and more is being
focused on the appearance and configuration of the neural rim tissue found
between the cup and the edge of the disc.
The rim tissue is often the first area to show changes in glaucoma, and must be
examined very critically during an optic nerve head evaluation. The normal neuroretinal
rim tissue is uniformly pink in color indicating good vascular perfusion. Because there
is a round cup located in a vertically elongated oval optic disc, the width of the neural
rim tissue varies by quadrant. In the normal eye, the Inferior quadrant has the widest
rim tissue with the Superior portion second in width. The nasal tissue is slightly
thinner than the superior tissue and the tissue in the Temporal quadrant is the
thinnest. This variation in rim sizes will cause large physiologic cups to appear
elongated horizontally. A disc with the normal configuration of rim tissue despite a
large cup/disc ratio can be seen in Figure 1. It is very important to remember that, the
superior rim tissue will appear inferior and the inferior tissue will appear on top in your
view. The reversal will also affect the nasal and temporal rim, which will be switched in
your view.
Figure 1: Normal rim tissue in a disc with a large cup.
The rim tissue will thin as nerve fibers atrophy. This results in pallor in the area of
atrophy and a decrease in the size of the rim tissue over time. If the nerve fiber loss is
generalized, the atrophy of nerve fibers will cause an overall decrease in the width of
3. rim tissue and an increase in the size of the cup. This generalized atrophy can be seen
in Figure 2, and is typical in moderate to advanced glaucoma, with corresponding
visual field loss. Because these changes are obvious only in the later stages of the
condition, the increase in cup size is not very helpful in making a diagnosis of
glaucoma early in the disease process.
Figure 2: Generalized atrophy which is typical in moderate to advanced glaucoma.
The focal nerve fiber loss in early glaucoma is more subtle and requires close
observation to detect. In early glaucoma, the inferior rim is usually affected first,
with the superior rim a close second. The next tissue to be damaged is typically the
temporal rim, with the nasal rim the last affected. Thinning in one focal area of the
disc can cause a "notch" to develop in the rim tissue over time. Since the inferior and
superior rim tissues are affected first, notching is typically seen in one of these
quadrants (I & S). When evaluating the optic nerve, it is helpful to have the results of a
visual field test performed on the same day readily available. This allows the
comparison of areas of potential visual field defects to the nerve fiber responsible for
that area of the field. It is estimated that 20% of the nerve fibers must be atrophied to
cause a visual field defect of 5 dB and 40% to cause a 10 dB loss. Because of this,
visual field results are best when used in conjunction with the optic nerve head and
nerve fiber layer evaluation.
4. Figure 3: Notching of the inferior optic rim tissue.
Figure 4: Subtle changes of the optic nerve head in the 11 o'clock position
Figure 3 shows obvious notching of the inferior optic rim tissue. Figure 4 shows a much
more subtle area of notching in the 11 o'clock position. This notching would be very
easy to overlook without the aid of the visual field. Figure 5 shows the visual field for
the patient in figure 4. Although the patient performed poorly on this visual field,
making the results of questionable value, it is interesting to note that there is a
corresponding change in the patient's inferior nasal visual field, as would be expected
from the optic nerve head appearance. When using the visual field as a tool to help
evaluate the optic nerve rim tissue, it is essential to remember that the superior rim
tissue consists of those fibers responsible for the inferior visual field. Because this
visual field reversal matches the reversal in the view of the high plus lens, the area of
rim tissue affected in the high plus lens view will be in the same quadrant as the
visual field loss.
5. Figure 5: Visual field of the patient seen in Figure 4 with corresponding visual field
When both the inferior and superior rim tissues are damaged in glaucoma, vertical
elongation of the optic cup occurs. This can be seen in Figure 6. This common vertical
elongation of an optic nerve head with glaucomatous damage will have corresponding
inferior and superior visual field defects.
Figure 6: Vertical elongation of the optic cup
6. Another optic nerve change which has significant diagnostic and therapeutic
importance is hemorrhaging. A small disc hemorrhage, known as a splinter or Drance
hemorrhage, is commonly associated with normal tension glaucoma. These
hemorrhages typically appear blot-like when located on the disc, and more flame
shaped if they are in close proximity to the disc. The occurrence of a disc hemorrhage
such as the one seen in Figure 7, should make you suspicious of glaucoma. Although
hemorrhages such as these can also be found in patients with a history of recent
posterior vitreous detachment, branch retinal vein occlusion, or diabetic retinopathy, it
is very rare for them to occur in the normal population. The examiner must be
particularly vigilant in cases where a splinter hemorrhage is noted in a patient with a
history of branch retinal vein occlusion or diabetes as these patients are already at an
increased risk of developing glaucoma. If other risk factors are present, the
appearance of a disk hemorrhage is a strong indicator for the initiation of glaucoma
treatment. They are more likely to occur on the temporal side of the disc, and can be
found either superior temporal or inferior temporal with equal frequency. Splinter
hemorrhages have been shown to precede nerve fiber layer and visual field changes in
some patients. Although the hemorrhages can resolve in as short as 2 weeks or as
long as 35 weeks, the average time to resolution is 10 weeks. It is quite common for an
area of notching to develop after the resolution of one of these hemorrhages, and it is
also common for the hemorrhages to recur in the same area, or within 30 degrees of
the original location. When a Drance hemorrhage is found in a patient who has
already been diagnosed, and is being treated for glaucoma, it indicates an
unfavorable prognosis, and the need for more aggressive therapy.
Figure 7: Splinter hemorrhage in the superior temporal quadrant
Peripapillary atrophy is also an important diagnostic consideration in glaucoma.
Peripapillary atrophy appears as a zone of chorioretinal atrophy with large choroidal
vessels and sclera visible around the optic nerve head. Further from the optic nerve
head, a less pronounced area of irregular hyper- and hypopigmentation can be seen.
As demonstrated in Figure 8, it is most common for this to occur on the temporal side
7. of the disc. The area with sclera and large choroidal vessels visible as is seen in Figure
6, is called the central zone or "zone Beta" and has been shown to be more frequent
and more extensive in eyes with high tension and normal tension glaucoma. In
addition, the location and extent of peripapillary atrophy has been shown to correlate to
visual field loss in both types of glaucoma. A recent study showed that in low
tension glaucoma, the appearance of the zone Beta peripapillary atrophy was
actually correlated more closely with visual field loss than the appearance of the
optic nerve itself. It is believed that peripapillary atrophy is not caused by
glaucomatous damage, but instead indicates that a patient is at an increased risk to
develop glaucoma. Although its etiology is uncertain, peripapillary atrophy seems to be
an indication that this area of the retina has a poor blood supply. To understand this, it
is important to realize that studies have shown a thinned or absent choroid with no
choroidal filling in zone Beta during the choroidal filling phase of fluorescein
angiography. Since the prelaminar portion of the optic nerve head also relies on the
choroid for its blood supply, a compromised choroid could cause ischemia of the optic
nerve head in this area, making the axons more susceptible to damage. In eyes with
small cup to disc ratios, the appearance of peripapillary atrophy may be a more
sensitive indicator of glaucomatous optic nerve damage than cup-to-disc ratios. The
appearance of peripapillary atrophy should raise the suspicion of glaucoma, and be
used in conjunction with other test results when making clinical decisions on the
diagnosis and management of glaucoma.
The examination of the optic nerve head should also include an evaluation for the
presence of acquired pits of the optic nerve. Acquired pits of the optic nerve (APON)
appear as sharply localized depressions of the lamina cribrosa with a loss of laminar
architecture. They are usually found in areas of pallor, and extend to the outer edge of
the disc. They are more likely to be located in the inferior temporal quadrant, with the
second most common location the superior quadrant. Although, it is not known whether
these pits are related to intraocular pressure, a study in 1990, indicates they are more
common in patients with low tension glaucoma than normals. Like peripapillary
atrophy, APON may represent an area of the optic nerve head that is more susceptible
to axonal damage.
Figure 8: Peripapillary atrophy on the temporal side of the disc (glaucoma)
8. Although a single evaluation of the optic nerve head can be very useful in the detection
of glaucoma, most glaucoma diagnoses require an observed change in the optic
nerve head over time. Examples include rim tissue changes, the appearance of
lamina cribrosa which was not previously visible, the shifting of a retinal blood vessel
on the optic nerve head, or the development of baring of a circumlinear vessel.
Because these changes typically occur gradually over several years, and because they
can be quite subtle, no optic nerve head evaluation can be considered complete
without documentation of the nerve head appearance with high magnification stereo
photographs, detailed drawings of specific areas of concern, and good written
descriptions.
Another method of monitoring and documenting the configuration of the optic nerve
head is by using computer assisted imaging. These instruments measure the vertical
and horizontal extent, depth, volume and contour of the cup. Although data is stored
and easily recalled for evaluation of changes over time, these instruments have not
achieved widespread use, possibly due to the large financial investment required.
Since the definitive diagnosis of glaucoma requires that the optic nerve be affected, the
evaluation of the optic nerve head is probably one of the most important tests to
perform when evaluating a patient for glaucoma. When carefully performed, the
stereoscopic evaluation of the optic nerve has high sensitivity and specificity for the
correct diagnosis of glaucoma, but like all other tests for glaucoma, it is best when
used in conjunction with the results of many other tests.
9. Evaluating the Nerve Fiber Layer
Evaluation of the nerve fiber layer is another useful tool to aid in the early diagnosis of
glaucoma. This is because nerve fiber layer defects can occur before visual field
changes are found. The evaluation of the nerve fiber layer can be done with a bright
light source like the binocular indirect ophthalmoscope, or at the biomicroscope with a
high plus lens. A clear condensing lens should be used in either case. Red free light,
which is absorbed by the pigment of the retinal pigment epithelium and the choroid, is
used to provide a dark background. The normal nerve fiber layer reflects light and
appears as a whitish haze over the darker underlying retinal structures. There will be
a striated appearance to the nerve fibers, with thicker nerve fiber layers appearing
brighter. Because the nerve fiber layer is thickest in the superior and inferior arcades
closest to the disc, this area should be the brightest portion of the view. There will be
less brightness in the thinner papillomacular region and the nasal side of the disc.
Symmetry between the reflections in the superior and inferior arcades and between
each of the patient's eyes is expected. A normal appearing nerve fiber layer can be
seen in Figure 9. This is the same eye as is pictured in Figure 1.
Figure 9: Normal appearing nerve fiber layer
When a patient has suffered nerve damage from glaucoma, darker areas or streaks
will appear in the nerve fiber layer. Dark areas which are slightly larger than arterioles
and reach the disc following the normal course of the nerve fiber layer are called slit
defects. They represent retrograde degeneration of the axons due to focal damage of
the optic nerve at the lamina. These can occur in approximately 10% of normal
patients. Wedge defects are caused by atrophy of many ganglion cells in the same
area of the optic nerve. These defects start at the disc as narrow lines and expand as
they get further from the disc. A wedge defect can be seen between 4 and 6 o'clock in
Figure 10. Notching of the neural rim tissue, as well as a visual field defects are
often associated with wedge defects. The most common type of defect, diffuse
atrophy, typically occurs in the superior and inferior arcades. The nerve fiber layer in
these areas loses its consistency and looks like it has been combed or raked with
darker and lighter areas. In severe cases nerve fiber layer reversal can occur in
which the normal pattern of superior and inferior brightness with increasing dimness
10. towards the papillomacular bundle is lost and the papillomacular area becomes the
brightest structure. Nerve fiber layer reversal is associated with thinning of the neural
rim and a diffuse depression or constriction of the visual field. An example of nerve
fiber layer reversal is seen in Figure 11.
Figure 10: A wedge defect in the nerve fiber layer between 4 and 6 o'clock
Figure 11: Nerve fiber layer reversal
Although it can be difficult to see the changes in the nerve fiber layer, especially in
lightly pigmented individuals, the technique provides additional early information for
determining if a patient has glaucoma. Black and white photography of the nerve fiber
layer provides more contrast of the tissue and improves the ability to compare the
health of the nerve fiber layer over time.