7 Steps

Step 1: Cup
Slit-lamp measurements of the vertical and horizontal cup-to-disc ratios should be determined.
Asymmetry of 0.2 or more between the two optic nerves raises suspicion for possible glaucoma.
Care must be taken to note whether the cup was identified by change in color or contour (see
Step 2). Visibility of lamina cribosa fenestrations in the cup is known as the laminar dot sign.

Step 2: Color
The pallor associated with glaucomatous changes in the cup chronologically follows the thinning
of the neuroretinal rim and its color. Therefore, close attention should be given to the areas
where vessels appear to be kinking in addition to that of pallor as the former would more
accurate in identifying the cup. This discrepancy between the cup and pallor becomes more
apparent when saucerization is present. Saucerization occurs when a shallow cup leads to the cup
margin with the remaining central pallor intact.

Step 3: Contour
The contour of the neuroretinal rim may reveal subtle features consistent with glaucomatous
change. Focal notching, or thinning, of the neuroretinal rim may exist. This occurs
particularly after resolution of splinter hemorrhages. Focal notching will lead to localized loss of
retinal nerve fiber that can be demonstrated using the red-free filter on the slit-lamp or be directly
measured for with ancillary testing such as the GDx. This loss in the retinal nerve fiber may
proceed changes in the optic nerve located if discovered early in the disease process. Eventual
progression of focal notching leads to a sharpened rim where sections of the neuroretinal rim are
virtually non-existent. In contrast to these localized areas, concentric atrophy results in a
generalized loss of tissue. Besides glaucoma, normal age-related changes in the retinal nerve
fiber do occur and can mimic similar atrophy.

Step 4: ISNT Rule

The four quadrants of the neuroretinal rim follow a characteristic pattern of thickness in normal
individuals as described by the ISNT rule. The ISNT rule represents the thickness of these
quadrants in decreasing order: inferior (I), superior (S), nasal (N), and temporal (T). Any
violation of the ISNT rule raises suspicion of glaucomatous changes. This is especially important
if thinning is noted inferiorly and superiorly as these areas are usually the first to change in
glaucoma.
Step 5: Disc Size
The dimensions of the disc should be measured along its long axis making sure to account for the
magnification factor of the lens being used1.1 and 1.3 for the 78 and 90 diopter lenses,
respectively. Other methods for determing size includes using the 5 degree aperture on the direct
ophthalmoscope that closely approximates the size of a normal disc. Also, confocal scanning
laser ophthlamoscopes can provide detailed measurements as part of their outpatient during
examination. Care needs to be taken not to include the zones around the disc in the measurement
such as the scleral lip or the zones of alpha and beta atrophy (see Step 7).

Step 6: Vessel Caliber

Vessel tortuosity denotes chronic central vessel occlusion with presence of collateral vasculature.
Sharply bending bayoneting vessels can be located at sharpened rims. Baring of circumlinear
vessels that once resided at the rim margin can be seen moving away from the border. Presence
of splinter hemorrhages can signify signal glaucomatous changes and usually leave behind focal
notching of the rim once they resolve approximately 15 to 18 months after initially presenting.
These hemorrhages can be easily overlooked if the examiner does not take care to look for them.
They rarely occur in non-glaucomatous eyes and should be noted if discovered especially as they
last for a brief period of time (usually between 2 to 4 months). They commonly exist in the
superotemporal or inferotemporal rim. In particular, patient with normal tension glaucoma are at
increased risk (between 3 to 5 times more likely) to have them. However, care needs to be taken
if disc hemorrhages are noted as the differential includes other causes such as posterior vitreous
detachment, trauma, hypertension, or use of anticoagulation medications like coumadin.
Step 7: Peripapillary Atrophy (PPA)
Evaluation of the peripapillary architecture and related atrophy does aid in glaucoma assessment.
Two distinct zones of atrophyzone alpha and betacan surround the optic disc. The inner
zone beta represents loss of retinal pigment epithelium and choriocapillaris leaving intact choroid
vasculature. Zone beta atrophy is more often seen in glaucomatous nerves. Its size significantly
correlates with other factors used to determine the severity of glaucoma such as perimetric
defects and help differentiate between classes of chronic open angle glaucoma. These areas of
atrophy are predominated located temporally but can encircle in the optic nerve. Zone alpha
atrophy is more common in normal eyes and is located on the outer surface of zone beta if
present. It contains areas of hyper or hypopigmentation with thinning of the choriocapillaris. The
difference in location between the two types of atrophy translates to differing visual field defects.
Of note, care must be taken when examining patients that are myopic or have tilted optic nerves.
In these individuals, scleral crescents, in particually inferiorly, can be present and must not be
confused with zone alpha or beta atrophy.

Variability in Examination
Examination of the optic nerve demonstrates both interobserver and intraobserver variability.

Interobserver Variability. Interobserver variability occurs when two or more observes vary in
their evaluation on one common entity. When evaluating the optic nerve (and the progression of
glaucoma), different clinicians will give varying measurements in their examination. This leads
to different examination and ultimately practice patterns as these subjective parameters are used
in clincial decision making.

Intraobserver Variability. Intraobserver variability arises from the variation of evaluation from
one observer about the same entity. Similarly to interobserver variability, intraobserver
variability when evaluating the optic nerve results in different assessments of glaucomatous
nerves even though the judgement comes from the same observer. These varying assessments of
the optic nerve result in different practice patterns as clinicial decisions are based on these
findings.

Conclusion
Repetition and adherence to these (or similar steps) can dramatically aid any ophthalmologist.
This is especially important when facing the common dilemma of diagnosing glaucoma or
monitoring its progression in cases that are not obvious.
(Optic Nerve Photos: Courtesy of Sarwat Salim, MD,FACS, University of Tennessee and
George Spaeth, MD, Wills Eye Hospital)