SURVEY OF OPHTHALMOLOGY VOLUME 43 • NUMBER 4 • JANUARY–FEBRUARY 1999

MAJOR REVIEW

Ophthalmoscopic Evaluation of the Optic Nerve Head

JOST B. JONAS, MD, WIDO M. BUDDE, MD, AND SONGHOMITRA PANDA-JONAS, MD

Department of Ophthalmology and Eye Hospital, University Erlangen-Nürnberg, Erlangen, Germany

Abstract. Optic nerve diseases, such as the glaucomas, lead to changes in the intrapapillary and para-
papillary region of the optic nerve head. These changes can be described by the following variables: size
and shape of the optic disk; size, shape, and pallor of the neuroretinal rim; size of the optic cup in rela-
tion to the area of the disk; configuration and depth of the optic cup; ratios of cup-to-disk diameter and
cup-to-disk area; position of the exit of the central retinal vessel trunk on the lamina cribrosa surface;
presence and location of splinter-shaped hemorrhages; occurrence, size, configuration, and location of
parapapillary chorioretinal atrophy; diffuse and/or focal decrease of the diameter of the retinal arterioles;
and visibility of the retinal nerve fiber layer (RNFL). These variables can be assessed semiquantitively by
ophthalmoscopy without applying sophisticated techniques. For the early detection of glaucomatous
optic nerve damage in ocular hypertensive eyes before the development of visual field loss, the most
important variables are neuroretinal rim shape, optic cup size in relation to optic disk size, diffusely or
segmentally decreased visibility of the RNFL, occurrence of localized RNFL defects, and presence of
disk hemorrhages. (Surv Ophthalmol 43:293–320, 1999. © 1999 by Elsevier Science Inc. All rights
reserved.)

Key words. cup/disk ratio • neuroretinal rim • optic cup • optic disk • optic disk
hemorrhages • optic disk pallor • parapapillary atrophy • peripapillary scleral ring •
retinal nerve fiber layer • retinal vessel diameter

I. Optic Disk Size In the same epidemiologic investigation,227 with the

exclusion of very tall and very small people, disk area
A. BIOLOGIC AND DEMOGRAPHIC CORRELATES increased by 0.02 mm2 with each 10-cm increase in
The optic disk area is not constant among individ- body length, whereas in a smaller nonepidemiologic
uals but shows an interindividual variability of about study, disk area was independent of body size and
0.80 mm2 to almost 6.00 mm2, or about 1:7, in a nor- body weight.112 Within a range of 25 to 15 diopters
mal white population (Figs. 1–5).21,25,61,118,227,268,278 (D) of refractive error, optic disk size was statistically
There are normal eyes with rather small optic disks, independent of ametropia in previous studies,25,118,278
and there are normal eyes with very large optic whereas in a recent epidemiologic investigation,227
disks.24 The optic disk area is independent of age be- disk area linearly increased by 1.2% 6 0.15% for
yond an age of about 3 to 10 years.21,25,118,227,278 In re- each diopter increase toward myopia. All studies
gard to gender, body length, and refractive error, agree that the optic disk is significantly larger in eyes
the results of several studies are partially contradic- with high myopia122,227,278 and that it is significantly
tory. Some investigations suggested that optic disk smaller in eyes with marked hyperopia (more than
size does not vary between women and men,25,118,278 15 D) than in eyes with a normal refractive error.
whereas in a recent epidemiologic study, mean optic Size of the optic disk varies with race.40,172,269,278
disk area was 3.2% larger in men than in women.227 Whites have relatively small optic disks, followed in

293
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294 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

Fig. 1. Small, otherwise normal, optic disk. There is no Fig. 2. Medium-sized normal optic disk with temporal flat
cupping, because of the smallness of the optic disk, physio- sloping of the optic cup. Note the physiologic shape of the
logic unsharpness, and slight prominence of the disk bor- neuroretinal rim and the medium size of the optic cup in
der. There is no parapapillary atrophy, the diameter of the physiologic relation to the size of the optic disk. The optic
retinal arterioles is unremarkable, and the visibility of the cup depth is shallow to medium. There is no parapapillary
retinal nerve fiber layer is excellent. The black arrows atrophy, the diameter of the retinal arterioles is unremark-
point toward the peripapillary scleral ring. able, and the visibility of the retinal nerve fiber layer is ex-
cellent. The black arrows point toward the peripapillary
scleral ring.

Fig. 3. Medium-sized normal disk with circular steep cup-

ping in the temporal disk region. Note the physiologic Fig. 4. Slightly large, otherwise normal, optic disk. Note
shape of the neuroretinal rim and the medium size of the the physiologic shape of the neuroretinal rim and the size
optic cup in physiologic relation to the size of the optic of the optic cup in physiologic relation to the size of the
disk. The optic cup depth is medium to deep. There is no optic disk. The optic cup depth is medium to deep. There
parapapillary atrophy, the diameter of the retinal arteri- is no parapapillary atrophy, the diameter of the retinal ar-
oles is unremarkable, and the visibility of the retinal nerve terioles is unremarkable, and the visibility of the retinal
fiber layer is average. The black arrows point toward the nerve fiber layer is average. The black arrows point toward
peripapillary scleral ring. the peripapillary scleral ring.
OPHTHALMOSCOPY OF OPTIC NERVE HEAD 295

Fig. 6. Large optic disk in a highly myopic and otherwise

normal eye (“secondary macrodisk”). Note the abnormal
disk shape, the shallow disk cupping, and the physiologic
Fig. 5. Large, otherwise normal, optic disk (“primary shape of the neuroretinal rim. The black arrows point to-
macrodisk”). Note the physiologic shape of the neuroreti- ward the optic disk border.
nal rim and the physiologically large and deep optic cup
(“pseudoglaucomatous but physiologic macrocup in pri-
mary macrodisk”). There is no parapapillary atrophy, and
the diameter of the retinal arterioles is unremarkable. The
black arrows point toward the peripapillary scleral ring.
The macrodisks can be divided into primary mac-
rodisks and secondary acquired macrodisks (Figs. 4–
6). The size of primary macrodisks is independent of
age after the first years of life118 and is independent
increasing disk size by Mexicans, Asians, and Afri- of or only slightly dependent on refractive error. Pri-
can-Americans. One may infer that disk size in- mary macrodisks can be subclassified into “asymp-
creases with the ethnically determined pigmenta- tomatic primary macrodisks,” without any morpho-
tion. A recent study on whites did not find a logic or functional defects, and “symptomatic
relationship between disk size and iris color,27 how- primary macrodisks,” with morphologic and func-
ever, suggesting that, within an ethnic group, disk tional defects, such as pits of the optic disk134 and
size and pigmentation are not correlated. the morning-glory syndrome.128 Primary macrodisks
The optic disk measurements vary according to are marginally correlated with the size of the cornea:
the method of measurement applied. Mean optic the larger the optic disk, the larger the cornea and
disk area of non–highly myopic whites examined in the larger the anterior corneal curvature radius.125
various studies ranged between 2.1 mm2 and about In contrast to the primary macrodisks, the acquired
2.8 mm2.2,24,25,27,30,37,40,117,118,153,172,212,227,259,269,278,279 Fu- or secondary macrodisks increase in size after birth
ture investigations will determine which method of and occur in eyes with high myopia.122 They can be
measurement comes closest to the real disk dimen- further subclassified into eyes with primary high my-
sions and by which correction factors the measure- opia caused by reasons yet unknown and eyes with
ments of the other methods have to be adapted.17 secondary high myopia caused by congenital glau-
coma. In both subtypes of secondary macrodisks,
B. MICRODISKS AND MACRODISKS
disk size is positively correlated with the myopic re-
Based on the gaussian-like distribution curve of fractive error.98,122 Because the myopic refractive er-
the optic disk area, very small disks, or “microdisks,” ror can increase with age in highly myopic patients,
and very large disks, or “macrodisks,” can be defined the size of secondary macrodisks in highly myopic
morphometrically: a microdisk as being smaller than eyes partially increases with age.
the mean minus twofold standard deviations and a
macrodisk as being larger than the mean plus two- C. MORPHOGENETIC AND
fold standard deviations (Figs. 1–5).118,153 Only 2.3% PATHOGENETIC IMPLICATIONS
of a standard population are statistically expected to The interindividual variability in optic disk size is
exceed each of these limits. morphogenetically and pathogenetically important.
296 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

It is morphogenetically important, because eyes duces a more pronounced displacement of the lam-
with large optic disks as compared with eyes with ina cribrosa in large optic disks than in small optic
small optic nerve heads have a larger neuroretinal nerve heads.28,40 Inside the optic disk, the susceptibil-
rim area,21,24,25,37,64,118,153,227,245,278 more optic nerve fi- ity for neuroretinal rim loss is higher in regions with
bers,148,213 less nerve fiber crowding per square milli- a long distance to the exit of the central retinal ves-
meter of disk area,148 a higher count and a larger to- sel trunk than in sectors with a short distance.101
tal area of lamina cribrosa pores,129 a higher ratio of These factors indicate a higher risk for glaucoma-
interpore connective tissue area to total lamina crib- tous damage in eyes with large disks.
rosa area,129 a higher count of cilioretinal arteries,113 The factors that favor a relationship between disk
and a higher count of retinal photoreceptors193 and size and glaucoma susceptibility are countered by
retinal pigment epithelium cells192 in combination factors that oppose it. Nonarteritic anterior ischemic
with a larger retinal surface area and longer horizon- optic neuropathy18,119 and optic disk drusen116,254 oc-
tal and vertical diameters of the globe.198 cur more frequently in small optic nerve heads than
The optic disk size variability is pathogenetically in large optic disks. For both entities, similar patho-
important, because some optic nerve anomalies and genetic mechanisms have been discussed as have
diseases are correlated with optic disk size. Optic been proposed for glaucoma, i.e., a perfusion prob-
disk drusen,116,183,254 pseudopapilledema,115,235 and lem for ischemic optic neuropathy and a blockage of
nonarteritic anterior ischemic optic neuropathy18,119 the orthograde axoplasmic flow in the case of optic
occur significantly more often in small optic disks. disk drusen.178,270 In the optic disk, the optic nerve fi-
Pits of the optic disk134,107 and the morning-glory bers are more crowded in eyes with small optic nerve
syndrome128 are more common in large optic nerve heads than in eyes with large disks.129 A dense ar-
heads. Eyes with arteritic anterior ischemic optic rangement of the nerve fibers in small optic disks
neuropathy119 and eyes with retinal vessel occlusions73,258 may suggest that the lamina cribrosa mechanically
have optic disks with a statistically normal size. deformed by the glaucomatous process may more
In glaucoma, the optic disk is normal in size in pri- easily press the optic nerve fibers in small optic disks
mary open-angle glaucoma (POAG),33,120 including than in large optic nerve heads. Eyes with small optic
the juvenile-onset type of POAG111 and the age-related disks have been reported to possess a slightly smaller
atrophic type of POAG,110 and in secondary open-angle number of optic nerve fibers than eyes with large op-
glaucoma (SOAG) caused by primary melanin dis- tic nerve heads.148,213 This suggests that eyes with
persion syndrome (“pigmentary glaucoma”).100 Two small optic disks have a smaller anatomic reserve ca-
studies141,271 suggested that in SOAG caused by pseu- pacity. Other studies have suggested that the higher
doexfoliation of the lens (“pseudoexfoliative glau- glaucoma susceptibility in the inferior and superior
coma”) the optic disk is slightly smaller than in POAG. disk regions as compared with the temporal and na-
This finding, however, can be attributed to a bias in sal disk sectors201,224,272 is associated with a higher
the selection of the patients. In all glaucomatous percentage of pore area to disk area.43,129,189,220 This
eyes with high myopia, including the highly myopic ratio increases with decreasing optic disk size.129 All
type of POAG, the optic disk is abnormally large be- these factors would predispose eyes with small optic
cause of secondary macrodisks acquired as a result disks to glaucomatous optic nerve damage.
of high myopia.98 In an intraindividual bilateral comparison of non–
The interindividual variability of the optic disk highly myopic white patients with NPG, however, the
area raises the question of whether disk size is corre- eye with the larger optic disk, as compared with the
lated with glaucoma susceptibility. There are several contralateral eye with the smaller optic nerve head,
factors that favor a relationship between large disk showed neither a more marked nor a less pro-
size and increased glaucoma susceptibility. The nounced glaucomatous optic nerve damage.149 A
larger optic disk size,40,172,269 in combination with re- similar result was obtained in another study on pa-
portedly higher glaucoma susceptibility,171,173,243 in the tients with POAG.103 The results of both studies indi-
African-American population group compared with cate that for a given patient the degree of the glau-
whites led to the hypothesis that eyes with large optic comatous optic nerve atrophy was not markedly
disks are more prone to glaucomatous optic nerve fi- associated with optic disk size. A reanalysis of the
ber loss than eyes with small optic disks. This as- study96 in which patients with NPG had a signifi-
sumption was supported by several studies in which cantly larger optic disk than POAG patients revealed
optic disk area was significantly larger in non–highly that optic disk size decreased significantly with in-
myopic patients with normal-pressure glaucoma creasing glaucomatous visual field defect.60 This sug-
(NPG) than in patients with POAG.30,96,271 From gests that the results of previous cross-sectional stud-
purely mechanical factors, one can deduce that the ies reporting an unusually large disk size in NPG may
pressure gradient across the lamina cribrosa pro- at least partially be due to a selection artifact.30,96,271
OPHTHALMOSCOPY OF OPTIC NERVE HEAD 297

In the routine practice of referring ophthalmolo- The ophthalmoscopic approximative measure-

gists, the chance of detecting glaucoma in patients ment of the optic disk structures is an alternative to
with NPG is dependent on the size of the optic cup: those sophisticated and time-consuming techniques.
the larger the cup, the higher the probability that The horizontal and vertical disk diameters are mea-
the ophthalmologist will suspect glaucoma despite sured ophthalmoscopically using a standard Gold-
normal intraocular pressure (IOP) measurements. mann three-mirror contact lens and a commercial
Because of the physiologic correlation between cup slit lamp with adjustable beam length.143 The length
size and disk area,21,24,25,37,64,118,153,227,278 patients with of the beam, which is adjusted to the diameter of the
early NPG have a higher chance of detection if their optic disk, is read on the scale of the slit lamp. These
disk is large. If, however, NPG is advanced, the glau- values have to be multiplied by a correction factor to
comatous enlargement of the optic cup will be suffi- get the same results as would be obtained by measur-
cient for the detection of glaucoma even if the optic ing the optic disk on photographs and compensat-
disk is small. In early NPG, this leads to a preferred ing for the ocular and camera magnification factor.
referral of patients with large disks to the study cen- The correction factor depends on the standard val-
ters and leads to a decrease of disk area with increas- ues of the laboratory and has to be calculated for
ing optic nerve damage in cross-sectional studies of each laboratory separately. Based on the direct mea-
patients with early and advanced NPG.149 surements of the horizontal and vertical disk diame-
Summarizing all these factors, one might infer ters, one calculates the optic disk area by applying
that their effects compensate each other. This agrees the modified formula of an ellipse (area 5 Pr/4 3
with the results of Dandona et al,43 who report that horizontal diameter 3 vertical diameter).130 In a pre-
morphometric data of the lamina cribrosa, includ- vious study, the error for the ophthalmoscopic de-
ing the ratio of pore area to lamina area, were not termination of disk diameters was 6.4% 6 4.8%
significantly different in blacks and whites, despite (mean 6 S.D.)143 and was independent of the refrac-
the optic disk being larger in blacks. tive error of the examined eye for a range of 25 D to
15 D. The error for the optic disk area is calculated
D. OPTIC DISK DIAMETERS by applying the modified formula of an ellipse using
the horizontal and vertical disk diameters as mea-
Similar to the area of the optic disk, the horizontal sured by ophthalmoscopy, as 9.3% 6 6.7%.143
diameter of the optic disk is not interindividually Instead of the Goldmann contact lens, other oph-
constant, but rather demonstrates a variability of thalmoscopic devices can be used, such as the Zeiss
1:2.9.118 Despite this, the optic disk diameter has four-mirror contact lens,255,256 a simple modified
been taken as an interindividual size unit to compare ophthalmoscope,72 or the Volk 60-D lens with a cor-
intraocular lesions, such as malignant melanomas. It rection factor of about 1.0 and the Volk Superfield
has been suggested that, in studies comparing differ- lens with an adaptation factor of about 1.5.196
ent individuals with each other, fundus lesions should Independent of the technique used, it is impor-
be measured in absolute size units (millimeters) with tant to measure the optic disk as all area inside of
correction of the photographic magnification, instead the peripapillary scleral ring. The peripapillary scleral
of using the interindividually variable disk diameter ring itself does not belong to the optic disk. This is
as a measurement unit. of utmost importance for all optic disk measure-
ments, because the inclusion of the peripapillary
E. MEASUREMENT OF THE OPTIC DISK SIZE scleral ring into the optic disk area markedly en-
One can determine the measurement of the optic larges the area of the neuroretinal rim and decreases
disk and its structures, such as the neuroretinal rim, the cup/disk ratios.
optic cup, and alpha and beta zones of parapapillary
atrophy, using optic disk photographs,23,24,227 by plot- II. Optic Disk Shape
ting the outlines of the disk structures on paper and The optic disk has a slightly vertically oval form,
measuring the outlines planimetrically,2,5,7,25,27,52,92,100– with the vertical diameter being about 7–10% larger
126,271,272,275
or with the help of computerized semi- than the horizontal one (Figs. 1–5).118 The maximal
automatic optic disk analyzers, using scanning laser disk diameter is nearly identical with the vertical di-
tomography of the optic nerve head,17,29–31,163,199,234 laser ameter, and the horizontal diameter is almost equal
polarimetry of the retinal nerve fiber layer (RNFL),282–284 to the minimal diameter. The ratio between mini-
and other techniques.34,36,37,40,177 Compared with the mal to maximal disk diameter ranged in previous
clinical assessment of the optic nerve head,169 the investigations between 0.64 and 0.98, corresponding
semiautomatic or automatic techniques have the ad- to an interindividual variability of 1:1.53.118,142 The
vantages of higher reproducibility and lower inter- ratio between the horizontal to vertical disk diame-
observer variability. ters varied between 0.70 and 1.37 (variability of
298 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

1:1.96). Thus, just by numbers, the interindividual that glaucoma susceptibility is mostly independent
variability in optic disk shape measured by the ratio of the shape of the optic disk.142 As a single variable,
of horizontal to vertical disk diameter and the ratio the optic disk shape is not markedly important for
of minimal to maximal disk diameter is less marked diagnosis and pathogenesis of glaucoma. This is
than the interindividual variability in area of the op- valid for eyes with a myopic refractive error of less
tic disk. than 28 D. Considering the relationship between
The disk form is not correlated with age, sex, right distance of the central retinal vessel trunk exit on
and left eye, and body weight and height.118,142 An the lamina cribrosa and location of glaucomatous
abnormal optic disk shape is significantly correlated neureoretinal rim loss,97,101 however, evaluation of
with increased corneal astigmatism and amblyopia the optic shape becomes important, because the op-
(Fig. 7).124 In a recent study, the amount of corneal tic disk shape influences the distance between neu-
astigmatism was significantly (p , 0.001) correlated roretinal rim at the disk border and the central reti-
with an increasingly elongated optic disk shape. Cor- nal vessel trunk exit.
neal astigmatism was significantly (p , 0.01) highest In highly myopic eyes, the optic disk is signifi-
in eyes with tilted disks. It was significantly (p 5 cantly more oval and elongated in configuration and
0.006) smallest in eyes with an almost circular disk more obliquely oriented than in any other group.98
shape. Amblyopia was significantly (p , 0.05) associ- The abnormal shape of the optic disk is significantly
ated with an elongated optic disk shape and high (p , 0.05) more pronounced in eyes with a myopic
corneal astigmatism. Especially in young children, if refractive error of more than 212 D than in eyes
an optic disk with abnormal shape is found in rou- with a refractive error ranging between 28 D and
tine ophthalmoscopy, keratometry or skiascopy should 212 D.98 This suggests that the myopic stretching
be performed to rule out corneal astigmatism and to leading to the secondary macrodisk in highly myopic
prevent amblyopia.124 The orientation of the longest eyes does not exert a similar traction on the optic
disk diameter can indicate the axis of corneal astig- disk in all directions, but that the optic disk is pulled
matism. Furthermore, eyes with a tilted optic disk more strongly in some meridians than in others.
can exhibit visual field defects mimicking a temporal One may speculate whether the marked and irregu-
hemianopsia, which may be caused by or may be as- lar stretching of the optic disk in highly myopic eyes
sociated with a hypopigmentation of the fundus in is one of the reasons why some of these eyes have a
the nasal inferior fundus region (Fig. 7). relatively high susceptibility for glaucomatous optic
In individuals with a myopic refractive error of less nerve fiber loss, as shown by glaucomatous optic
than 28 D, normal eyes and glaucomatous eyes do nerve damage in the presence of normal IOP
not differ significantly (p . 0.20) in optic disk measurements.203
shape.142 Within the POAG group, optic disk shape is
not correlated with neuroretinal rim area and mean
perimetric defect, either interindividually or in an III. Neuroretinal Rim Size
intraindividual bilateral comparison. This suggests As the intrapapillary equivalent of the retinal
nerve fibers and optic nerve fibers, the neuroretinal
rim is one of the main targets in the ophthalmo-
scopic evaluation of the optic nerve.2,5,23–25,33,37,65,118,
120,122,153,181,182,245,259,272,278
The neuroretinal rim size is
not interindividually constant but demonstrates,
similar to the optic disk and cup, a considerably high
interindividual variability. It is correlated with the
optic disk area: the larger the disk, the larger the
rim.2,23,25,37,64,118,245,278 The increase of rim area with
enlarging disk area is most marked for eyes with no
disk cupping, medium pronounced for eyes with a
temporal flat sloping of the optic cup, and is least
marked in eyes with a circular steep disk cupping.118
The correlation between rim area and disk area
corresponds with the positive correlation between
optic disk size, optic nerve fiber count,148,213 and
number and total area of the lamina cribrosa pores.129
Fig. 7. Fundus photograph of an eye with tilted disk, infe-
rior scleral crescent, and hypopigmentation of the fundus It points toward a greater anatomic reserve capacity
in the nasal inferior region (and accompanying corneal in eyes with large optic disks as compared with eyes
astigmatism). with small optic disks.
OPHTHALMOSCOPY OF OPTIC NERVE HEAD 299

Possible reasons for the interindividual size vari- to the higher differential light sensitivity in perime-
ability of the rim are a different nerve fiber count, try in the inferior visual field than in the superior vi-
16,95,131,148,174,175
a different relationship between embry- sual hemisphere.
ologically formed and regressed retinal ganglion cell In glaucoma, neuroretinal rim is lost in all sectors
axons,207,226 different density of nerve fibers within of the optic disk with regional preferences, depend-
the optic disk,148 different lamina cribrosa architec- ing on the stage of the disease.15,19,23,69,75,83,87,89,91,106,157,
ture,43,129,189,220 different diameters of retinal gan- 201,224,223,272
In eyes with modest glaucomatous dam-
glion cell axons,148,131,213 different proportion of glial age, rim loss is found predominantly at the infero-
cells on the whole intrapapillary tissue,179,189 and/or temporal and superotemporal disk regions (Fig. 8).
other factors. In eyes with moderately advanced glaucomatous at-
The nerve fibers within the neuroretinal rim are rophy, the temporal horizontal disk region is the lo-
retinotopically arranged. Studies have shown that ax- cation with relatively the most marked rim loss. In
ons from ganglion cells close to the optic disk lie very advanced glaucoma, the rim remnants are lo-
more centrally in the optic disk, whereas axons from cated mainly in the nasal disk sector, with a larger
cells in the retinal periphery lie at the optic nerve rim portion in the upper nasal region than in the
head margins.180,221 This corresponds to the nerve fi- lower nasal region.106 This sequence of disk sectors
ber distribution in the RNFL.221 (inferotemporal, superotemporal, temporal horizon-
tal, nasal inferior, nasal superior) correlates with the
IV. Neuroretinal Rim Shape progression of visual field defects, with early peri-
In normal eyes, the neuroretinal rim shows a char- metric changes in the nasal upper quadrant of the
acteristic configuration. It is based on the vertically visual field and a last island of vision in the temporal
oval shape of the optic disk and the horizontally oval inferior part of the visual field in eyes with almost ab-
shape of the optic cup. The neuroretinal rim is usu- solute glaucoma.38,51,70,74 This indicates that for an
ally broadest in the Inferior disk region, followed by early diagnosis of glaucoma, the temporal inferior
the Superior disk region, the Nasal disk area, and fi- and the temporal superior disk sectors should espe-
nally the Temporal disk region (the ISNT rule, as cially be checked for glaucomatous changes. It
termed by Werner).118 The characteristic shape of should be kept in mind, however, that the glaucoma-
the rim is of utmost importance in the diagnosis of tous loss of neuroretinal rim is mostly diffuse, occur-
early glaucomatous optic nerve damage in ocular hy- ring in all sectors of the optic disk, with some prefer-
pertensive eyes prior to the development of visual ential locations for slightly more pronounced damage,
field defects in white-on-white perimetry. The physi- depending on the stage of the disease.106
ologic shape of the neuroretinal rim is associated Previous studies have shown that the following fac-
with the following: 1) the diameter of the retinal ar- tors may be associated with the pattern of glaucoma-
terioles, which are significantly wider in the infer- tous neuroretinal rim loss.
otemporal arcade than in the superotemporal
arcade137,147; 2) the visibility of the retinal nerve fiber 1. The physiologic configuration of the rim is
bundles, which are significantly more often better broader at the inferior and superior disk poles
detectable in the inferotemporal region than in the than at the nasal and temporal poles.118
superotemporal region138,147; 3) the location of the 2. The morphology of the inner surface of the
foveola 0.53 6 0.34 mm inferior to the optic disk lamina cribrosa shows larger pores and a
center138; 4) the morphology of the lamina cribrosa higher ratio of pore to interpore connective
with the largest pores and, relatively, the least tissue area in the inferior and superior regions
amount of interpore connective tissue in the inferior as compared with the temporal and nasal
and superior regions as compared with the temporal regions. A high ratio of pore area to total area
and nasal sectors43,129,189,220; and 5) the distribution of is considered to predispose to glaucomatous
the thin and thick nerve fibers in the optic nerve just nerve fiber loss.43,129,179,189,220
behind the globe, with the thin fibers in the tempo- 3. Glaucomatous backward bowing of the lamina
ral part of the nerve.131,148,174 cribrosa to the outside, mainly in the inferior and
Although the neuroretinal rim is broadest in the superior disk regions, is shown on scanning
inferior part of the optic disk, the neuroretinal rim electron microscopic photographs of glaucomatous
area above a horizontal line drawn through the cen- eyes.211
ter of the foveola is larger than below this line. This 4. The lamina cribrosa is thicker in the disk
is explainable by the location of the foveal center periphery, where the nerve fiber bundles have
temporal inferior to the optic disk138 and by the loca- a slightly more bent course through the lamina
tion of the blind spot temporal and inferior to the cribrosa45 and where they are lost earlier than
fixation center in the visual field. This corresponds in the center of the optic disk.
300 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

Fig. 8. Glaucomatous optic disks with abnormal shape of

the neuroretinal rim. Note that the rim width is more or
less even in all disk sectors, which contradicts the ISNT
rule. The long white arrows in the top left photograph
point toward the localized retinal nerve fiber layer defect.
The black arrows point toward the peripapillary scleral ring.

5. Thin and thick retinal nerve fibers are region- 6. The distance from the central retinal vessel
ally distributed. Thin nerve fibers come from trunk exit on the lamina cribrosa plays a role.101
the foveola, passing mainly through the tempo- The larger the distance to the central retinal
ral aspect of the optic disk,131,148,180,190,191,221 and vessel trunk exit on the lamina cribrosa, the
are less susceptible to glaucoma218 than thick more pronounced the loss of neuroretinal rim
nerve fibers, which originate predominantly in and the perimetric defect in the corresponding
the fundus periphery, lead to the inferior, visual field quadrant.286 Taking into account
superior and nasal disk regions, and are more the slightly eccentric location of the retinal
glaucoma-sensitive. This may explain why glau- vessel trunk exit in the nasal upper quadrant of
comatous rim loss occurs later in the temporal the vertically oval optic disk,129 one can infer
horizontal disk sector with predominantly thin that the progressive sequence of rim loss in
nerve fibers than in the temporal inferior or glaucoma is partially dependent upon the
temporal superior disk sectors containing thin distance of the region to the retinal vessel
and thick axons. It is contradictory that in far trunk exit. As a corollary, glaucomatous eyes
advanced glaucoma rim remnants are usually with an atypical location of the retinal vessel
located in the nasal disk sector. There, prefer- trunk exit or an unusual optic disk form were
entially thick retinal ganglion cell axons leave found to exhibit an abnormal glaucomatous
the eye. rim configuration (Fig. 9).101
OPHTHALMOSCOPY OF OPTIC NERVE HEAD 301

VI. Optic Cup Size in Relation to the Optic

Disk Size
The optic cup is the excavation in the optic nerve
head. The border between the optic cup and the
neuroretinal rim is determined by contour and not
by pallor. For the evaluation of the optic cup, it is
useful to clinically examine the optic nerve head ste-
reoscopically, e.g., during slit-lamp examination us-
ing ophthalmoscopic lenses, or to have stereo optic
disk photographs. Especially in eyes with shallow
disk cupping, such as in highly myopic eyes with
glaucoma, assessment of the location of kinking of
vessels can be helpful for the determination of the
border of the optic cup.
Parallel to the optic disk and the neuroretinal rim,
the optic cup also shows a high interindividual vari-
ability (Fig. 1–5).21,24,25,37,118,153,227,278 Similar to macro-
disks, very large cups, or macrocups, may be defined
as being larger than the mean plus twofold standard
Fig. 9. Glaucomatous optic disk (right eye) with abnor- deviations. One has to take into account, however,
mal shape of the neuroretinal rim (broadest part in the
temporal superior disk region) and abnormal position of that the distribution of the optic cup area is not
the central retinal vessel trunk exit in the temporal supe- gaussian. The macrocups can be subdivided into pri-
rior disk sector. Note that the parapapillary atrophy is larg- mary macrocups and acquired, or secondary, macro-
est in the nasal region in spatial correlation to the most cups. Primary macrocups occur in primary macro-
marked neuroretinal rim loss in the nasal disk sector. The disks153; they appear pseudoglaucomatous but are
black arrows point toward the peripapillary scleral ring.
physiologic (Fig. 5). They are constant in size after
the first years of life. Acquired, or secondary, macro-
cups can be further subclassified. The secondary
In contrast to glaucomatous optic neuropathy, highly myopic macrocup is found in highly myopic
nonglaucomatous optic nerve damage is not always eyes with secondary macrodisks caused by the myo-
associated with neuroretinal rim loss.75,76 Conse- pic stretching of the optic nerve head (Fig. 6).122
quently, the shape of the neuroretinal rim is not The other type of secondary macrocups is caused by
markedly altered (Fig. 10). the glaucomatous loss of neuroretinal rim.
In normal eyes, the areas of the optic disk and op-
tic cup are correlated with each other: the larger the
V. Neuroretinal Rim Pallor optic disk, the larger the optic cup.21,24,25,37,118,153,227,279
Increasing pallor of the optic disk and, especially, In small optic disks, cupping normally does not oc-
of the neuroretinal rim is a typical sign of optic cur. Large optic disks usually have a large optic cup.
nerve damage.76,88,177,184,185,230,231,236–239,241,274,277 The in- In the morphologic diagnosis of glaucoma, this feature
crease in pallor of the neuroretinal rim may be has to be considered. Early or moderately advanced
more marked in eyes with nonglaucomatous optic glaucomatous optic nerve damage may erroneously
neuropathy than in eyes with glaucoma. In other be overlooked in small optic disks with relatively low
words, if the neuroretinal rim looks rather pale, the cup-to-disk ratios, if one does not consider that small
probability of a nonglaucomatous optic neuropathy optic disks normally have no optic cup (Fig. 11).105
is higher than that of glaucoma. Pallor of the neu- The glaucomatous eyes with small optic disks and
roretinal rim is, thus, one among other variables to pseudonormal but glaucomatous minicups often
differentiate between glaucomatous and nonglauco- show glaucomatous abnormalities in the parapapillary
matous optic neuropathy. In glaucoma, the overall region, such as a decreased visibility of the RNFL,2,6,249
pallor of the optic disk as sum of optic cup and diffusely diminished diameter of the retinal arterioles63,
133,137
neuroretinal rim increases mainly due to the en- and/or focally reduced arteriole diameter,197,219,
229
largement of the optic cup. and parapapillary chorioretinal atrophy.13,77,135,205,
285
Pallor of the optic disk can quantitatively be mea- In contrast, a large optic cup in a large optic disk
sured using sophisticated techniques39,177,184,185,230,231, should not lead to the diagnosis of glaucoma if the
241,274,277
or it can be estimated by a simple ophthal- other intrapapillary variables are normal, mainly the
moscopic examination. configuration of the neuroretinal rim.153
302 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

Fig. 11. Small glaucomatous optic disk: pseudonormal but

Fig. 10. Medium-sized optic disk with nonglaucomatous glaucomatous minicupping in minidisk. Note the small optic
optic nerve atrophy. Note the normal shape of the neuroret- cup despite the smallness of the optic disk. The abnormal
inal rim and the size of the optic cup in physiologic relation shape of the neuroretinal rim contradicts the ISNT rule. The
to the size of the optic disk. The optic cup depth is medium visibility of the retinal nerve fiber layer is decreased. The
to shallow. There is no marked parapapillary atrophy, and black arrows point toward the peripapillary scleral ring.
the visibility of the retinal nerve fiber layer is decreased. The
black arrows point toward the peripapillary scleral ring.

nonglaucomatous optic nerve damage. In addition

In contrast to glaucoma, the optic cup does not to parapapillary atrophy,104 disk pallor, and depth of
markedly enlarge in eyes with nonglaucomatous op- the optic cup, the increase in cup area is, thus, an
tic nerve damage.136,152 Accordingly, the neuroretinal important marker to differentiate between glauco-
rim does not pronouncedly decrease in eyes with matous and nonglaucomatous optic nerve damage.

Fig. 12. Glaucomatous optic disk of a patient with focal normal-pressure glaucoma. Disk hemorrhages are present in the
left photograph at the 2:00 o’clock and the 5:30 o’clock positions, in spatial correlation with the neuroretinal rim notch
and the localized retinal nerve fiber layer defects, which are indicated by the black arrows in the right photograph. Note
the physiologic a zone (as indicated by the white arrowheads in the left photograph) and the absence of a beta zone. The
black arrows in the left photograph point toward the peripapillary scleral ring.
OPHTHALMOSCOPY OF OPTIC NERVE HEAD 303

VII. Configuration and Depth of the ulation from 0.0 to almost 0.9.118 Because of the cor-
Optic Cup relation between disk area and cup area,21,24,25,37,118,
153,227,279
In normal eyes, the shape of the optic cup is hori- cup/disk ratios are low in small optic nerve
zontally oval; the horizontal diameter is about 8% heads and are high in large optic disks. An unusually
longer than the vertical diameter.118 The combina- high cup/disk ratio, therefore, can be physiologic in
tion of the horizontally oval shape of the optic cup eyes with large optic nerve heads (Figs. 4 and 5),153
and the vertically oval shape of the optic disk ex- whereas an average cup/disk ratio is uncommon in
plains the configuration of the normal neuroretinal normal eyes with small optic disks. In the diagnosis
rim, which has its broadest parts in the inferior and of glaucomatous optic nerve damage, this interindi-
superior disk regions and its smallest parts in the vidual variability of cup/disk ratios and their depen-
temporal and nasal region of the optic disk.118 dence on the optic disk size has to be accounted for.
In addition to its area, the optic cup is ophthalmo- Eyes with physiologically high cup/disk ratios in
scopically described by its depth. In normal eyes, the macrodisks should not be overdiagnosed and con-
optic cup depth depends on the cup area and, indi- sidered glaucomatous, and eyes with increased IOP,
rectly, on the disk size: the larger the optic cup, the small optic nerve heads, and average or low cup/
deeper it is.59 In glaucoma, the optic cup deepens disk ratios should not be underdiagnosed and re-
depending on the type of glaucoma and the level of garded to be only “ocular hypertensive” (Figs. 4, 5,
IOP. Semiquantitative studies have shown that the and 11).105 Using an optic disk grid may be helpful
deepest optic cups can be found in glaucomatous for the estimation of the cup/disk ratios.85,86
eyes with high minimal values of IOP, such as juve- As a ratio of cup diameter to disk diameter, the
nile-onset POAG and SOAG caused by traumatic re- cup/disk ratios are independent of the magnifica-
cession of the anterior chamber angle.35,111,144,252,253 tion by the optic media of the examined eye, and of
Of all types of open-angle glaucoma, the optic cup is the fundus camera or other instrument, and meth-
most shallow in eyes with the highly myopic type of ods to correct for the ocular and camera magnifica-
POAG and eyes with the age-related atrophic type of tion do not have to be applied. The quotient of the
POAG.35,67,71,98,251–253 In glaucoma, the depth of the horizontal to vertical cup/disk ratios is also indepen-
optic cup is slightly associated with the degree of dent of the size of the optic cup and disk.
parapapillary atrophy102: the deeper the optic cup, IX. Position of the Exit of the Central
the smaller the parapapillary atrophy. This holds Retinal Vessel Trunk on the Lamina
true especially for the juvenile-onset type of POAG Cribrosa Surface
with high minimal and maximal IOP measurements
and for the focal type of NPG. Both types of glau- As already pointed out, the local susceptibility for
coma can show relatively steep and deep disk cup- glaucomatous neuroretinal rim loss partially de-
ping and an almost unremarkable parapapillary pends on the distance to the exit of the central reti-
atrophy.109,111 nal vessel trunk on the lamina cribrosa surface (Fig.
9)101: the longer the distance to the central retinal
vessel trunk exit, the more marked the glaucomatous
VIII. Cup/Disk Ratios loss of neuroretinal rim and the loss of visual field in
Because of the vertically oval optic disk and the the corresponding visual field quadrant. The location
horizontally oval optic cup, the cup/disk ratios in of the central retinal vessel trunk exit can, therefore,
normal eyes are significantly larger horizontally than be one of several factors influencing local glaucoma
vertically.118 The horizontal cup/disk ratio is smaller susceptibility. Assuming that cilioretinal arteries play
than the vertical one in less than 7% of normal eyes,118 a role similar to that of the central retinal trunk in
indicating that the quotient of the horizontal to ver- predisposing to local susceptibility for losing neuroret-
tical cup/disk ratios is usually higher than 1.0. This inal rim and to develop spatially related parapapil-
is important for the diagnosis of glaucoma, in which, lary atrophy, the findings of a recent study101 with re-
in the early to medium advanced stages, the vertical spect to the neuroretinal rim loss confirm a previous
cup/disk diameter ratio increases faster than the investigation. In that study it was found that eyes with
horizontal one, leading to an increase of the quotient open-angle glaucoma and a temporal cilioretinal ar-
of horizontal to vertical cup/disk ratios to values tery retained longer central visual field (and tempo-
lower than 1.0.120,121 ral neuroretinal rim) than open-angle glaucomatous
As ratio of cup diameter to disk diameter, the eyes without a temporal cilioretinal artery.168 The lo-
cup/disk ratios are dependent on the size of the op- cation of the central retinal vessel trunk exit on the
tic disk and cup (Fig. 1–5). The high interindividual lamina cribrosa and an abnormal shape of the optic
variability of the optic disk and cup diameters ex- disk should, therefore, be noted in glaucomatous eyes
plain why the cup/disk ratios range in a normal pop- with an unusual configuration of the neuroretinal rim.
304 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

The relationship between distance to the central backward bowing of the lamina cribrosa and the
retinal vessel trunk exit and location of glaucoma- more marked may be the compression of the chorio-
tous damage is valid also for parapapillary atrophy: capillaris in the parapapillary region. Another clini-
the longer the distance to the central retinal vessel cal finding, however, does not fit with this hypothe-
trunk exit, the more enlarged the parapapillary atro- sis. Eyes with juvenile open-angle glaucoma and high
phy in glaucomatous eyes compared with normal IOP usually have a rather deep and steep disk cup-
eyes.97 This is in agreement with the spatial relation- ping and often have an unremarkable parapapillary
ship between glaucomatous neuroretinal rim loss in- atrophy.111 This discrepancy may be explained by the
side of the optic disk and enlargement of parapapil- assumption that parapapillary atrophy needs a longer
lary atrophy outside of the optic disk border.102,132 time to develop compared with neuroretinal rim
Pathogenetically, one may infer that the retinal loss; therefore, in glaucomatous eyes with high IOP,
vessel trunk could act as a stabilizing element against deep and steep disk cupping, and relatively fast de-
glaucomatous changes in the lamina cribrosa. It velopment of optic nerve damage, neuroretinal rim
could render more difficult a mechanical distortion loss may occur earlier than parapapillary atrophy
and backward bowing of the lamina cribrosa in glau- can develop. A different hypothesis could be that va-
coma. This hypothesis is supported by photographs soactive substances or other substances with result-
of a W-shaped lamina cribrosa in glaucomatous ing tissue damage act on both sides of the optic disk
eyes.211 The lamina cribrosa is more condensed and border, leading to loss of neuroretinal rim inside the
bowed more to the back in the inferior and superior optic disk and to enlargement of parapapillary atrophy
disk regions than close to the center of the lamina outside of the optic nerve head.60 Another possibil-
cribrosa, where the retinal vessels emerge. If the ves- ity, a biomechanical construct for optic disk cupping
sel trunk is, as usual, decentered into the superona- in glaucoma, could be that the IOP-related stress
sal quadrant of the optic disk, the inferotemporal could lead to similar changes on both sides of the
disk region without support by the vessel trunk is optic disk border.28 The pathogenetic reason for the
larger than the superonasal disk sector. Conse- finding that glaucomatous parapapillary atrophy par-
quently, the inferotemporal sector can be deformed tially depends on the distance to the central retinal
to a greater extent than the superonasal disk region. vessel trunk exit, remains unclear so far and may be
This could explain the greater frequency of neu- elucidated in further studies.
roretinal rim notches in the inferotemporal disk re-
gion than in the superotemporal sector in eyes with X. Optic Disk Hemorrhages
a normal disk shape and a normal position of the
A. GENERAL FINDINGS
vessel trunk exit.120 As an alternative to this mechani-
cal theory, one could also speculate that the vascular Splinter-shaped or flame-shaped hemorrhages at
supply to the adjacent tissue is better in close vicinity the border of the optic disk are a hallmark of glauco-
of the retinal vessel trunk than in the periphery. A vi- matous optic nerve atrophy (Fig. 12).53,54 Very rarely,
tally important participation of branches of the cen- found in normal eyes,79,123,150,156,159,232 disk hemor-
tral retinal vessels in the nourishment of the optic rhages are detected in about 4–7% of eyes with glau-
nerve fibers in the lamina cribrosa, however, has not coma.8,22,50,68,82,158,161,244 Their frequency increases from
been demonstrated yet.170 an early stage of glaucoma to a medium advanced
The theory of the central retinal vessel trunk act- stage and decreases again toward a far advanced
ing as a stabilizing element against the deformation stage.150 One study suggested that disk hemorrhages
of the lamina cribrosa, however, does not easily ex- are not found in disk regions or eyes without detect-
plain the relationship between the central retinal able neuroretinal rim.150 In early glaucoma, they are
vessel trunk exit and the location of parapapillary at- usually located in the inferotemporal or superotem-
rophy. Accordingly, it has remained unclear, so far, poral disk regions. They are associated with localized
why a spatial relationship exists between loss of neu- RNFL defects, neuroretinal rim notches, and circum-
roretinal rim inside of the optic disk and enlarge- scribed perimetrical loss (Fig. 12).8,50,80,146,150,261
ment of chorioretinal atrophy in the corresponding
parapapillary region. One explanation could be B. DIAGNOSTIC IMPORTANCE
that, by the backward bowing of the lamina cribrosa, The diagnostic importance of disk hemorrhages is
Bruch’s membrane at the optic disk border is also based on their high specificity. In two epidemiologic
drawn backward, leading to a compression of the studies, frequency of disk hemorrhages in nonglau-
parapapillary choriocapillaris with resulting damage comatous eyes was about 1%.79,159 This high specific-
of the retinal pigment epithelium and deep retinal ity of about 99% points toward a helpful role in the
layers. The greater the distance to the central retinal early diagnosis of glaucoma. Because they are only
vessel trunk is, the more pronounced may be the rarely found in normal eyes, disk hemorrhages usu-
OPHTHALMOSCOPY OF OPTIC NERVE HEAD 305

ally indicate the presence of glaucomatous optic tients developing disk hemorrhages than could have
nerve damage, even if the visual field is unremark- been expected given the initial prevalence.
able,1,6,9,11,20,162,262 and suggest progression of glau- One can raise further questions about whether
coma.1,6,9,11,50,54,162,228,244,246,261 Glaucoma, however, is the differences in frequency of disk bleeding in the
not the only optic nerve disease in which optic nerve various glaucoma types are caused by varying
disk hemorrhages can be found. Additionally, be- amounts of blood per hemorrhage and different
cause of the low prevalence of disk hemorrhages in rates of absorption of blood. The disk hemorrhages
eyes with glaucoma8,22,50,68,82,90,158,161,244 and, thus, low are visible for about 8 days to 12 weeks after the ini-
sensitivity, they are not at all sufficient as a single tial bleeding.80 One cannot exclude the possibility
variable to separate normal eyes from eyes with early that a high IOP may stop bleeding relatively early,
glaucoma, which explains why they are not a useful resulting in a small disk hemorrhage in high-pres-
tool in screening examinations for glaucoma. About sure glaucomatous eyes, and that a low IOP may stop
2 months after the initial bleeding, a localized defect the bleeding relatively late, leading to a large disk
of the RNFL or a broadening of a localized RNFL hemorrhage in eyes with NPG. This would favor a
defect can often be detected correlating with a cir- faster absorption of disk hemorrhages in eyes with
cumscribed scotoma in the visual field.80 high-pressure glaucoma than in eyes with NPG. If
this holds true, the differences in IOP between the
C. DIFFERENCES BETWEEN THE VARIOUS various types of open-angle glaucomas will not be
GLAUCOMA TYPES causative for a preferred development of disk hem-
Because frequency of optic disk hemorrhages dif- orrhages in eyes with NPG, but they will be responsi-
fers between the various types of the open-angle ble for a longer visibility of the bleeding and could
glaucomas, assessment of disk bleedings can be mimic a higher incidence of the hemorrhages.
helpful for classification of the glaucoma type. Disk One also has to consider that the disk hemor-
hemorrhages were found most often in patients with rhages described in these and other studies might
focal NPG. Frequency of detected disk bleedings was represent the extreme of large disk bleeding in a
lower in patients with juvenile-onset POAG, age- possible spectrum of hemorrhages, including also
related atrophic POAG, and highly myopic POAG. microscopical bleeding undetectable on ophthal-
35,50,67,98,110,111,150,158,253
Disk hemorrhages, however, can moscopy. This would mean that all figures concern-
be found in all types of the chronic open-angle glau- ing the frequencies of bleeding would relate to only
comas, suggesting that the pathomechanism associ- a fraction of the total of disk hemorrhages.
ated with disk hemorrhages may be present in all
these glaucoma types. D. PATHOGENETIC ASPECTS
There are factors, however, that limit the practical With regard to the pathogenesis of disk hemor-
importance of the reported differences in the fre- rhages, one could argue that the rapid movements
quencies of disk hemorrhages in the various types of of the lamina cribrosa tear the superficial blood ves-
glaucomas. The differences between the groups sels of the optic disk, resulting in a break and subse-
were for the most part not statistically significant. quent circumscribed bleeding. This is contradicted
Since the selection of glaucoma patients in the hos- by the clinical experience that eyes normally do not
pital-based studies is partially dependent on the ap- show splinter-like or flame-shaped disk hemorrhages
pearance of the optic disk, one cannot exclude with after a contusion with a very marked and short elevation
certainty that the frequency of the disk hemorrhages of IOP.160 The question arises whether disk hemor-
artefactually is high in the studies. It is possible that rhages have their origin in the arterioles, venules, or
clinic-based glaucoma groups used in many studies the capillaries of the peripapillary radial network on the
have a larger number of disk hemorrhages than a surface of the peripapillary retina.12 The commonly
population-based sample, as patients may have been held belief that disk hemorrhages indicate an ischemic
referred into the care system because a disk hemor- event may be contradicted by the fact that they are
rhage was detected. Those patients with NPG are never associated with a cotton-wool spot, which is
likely to be under care because of optic disk ab- the typical sign for an ischemic infarct in the RNFL.
normalities, including hemorrhages, because they
would not have been identified through elevated XI. Parapapillary Chorioretinal Atrophy
IOP. This could result in a bias, with NPG patients
presenting with a falsely high frequency of disk hem- A. HISTORICAL REMARKS
orrhages compared with patients with high-pressure Around the beginning of the 20th century, oph-
glaucoma. Some evidence for this hypothesis is pre- thalmologists such as Elschnig and Bücklers turned
sented in a longitudinal study by Diehl et al,48 which, their attention to an association between glaucoma
on follow-up examination, revealed fewer NPG pa- and parapapillary chorioretinal atrophy.26,56,57 It was
306 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

called “halo glaucomatosus” when it totally encircled In normal eyes, both the a zone and b zone are
the optic disk in eyes with end-stage glaucoma. largest and most frequently located in the temporal
Later, Primrose, Hayreh, Wilensky and Kolker, horizontal sector, followed by the inferior temporal
Anderson, Airaksinen and other investigators con- area and the superior temporal region.102,135 They
firmed the observations describing the occurrence are smallest and most rarely found in the nasal para-
of parapapillary atrophy in eyes with glaucoma.7,13,32, papillary area. The a zone is present in almost all
58,66,71,77,81,102,132,135,155,166,187,204–206,225,233,264–266,285
Heijl and normal eyes and is, thus, more common than the b
Samander found a spatial correlation between the zone (mean frequency in normal eyes, about 15%–
parapapillary chorioretinal atrophy and the location 20%).102,132,135 The a zone and the b zone have to be
of the most marked visual field loss.81 Anderson differentiated from the myopic scleral crescent in
noted that the presence or extent of a crescent cor- eyes with high myopia and from the inferior scleral
related with the glaucomatous disk damage.13 crescent in eyes with “tilted optic disks.” The myopic
scleral crescent present in highly myopic eyes differs
B. ALPHA ZONE AND BETA ZONE histologically from the glaucomatous b zone in non–
Ophthalmoscopically, parapapillary chorioretinal highly myopic eyes. In the region of the myopic cres-
atrophy has been divided into a central b zone and a cent, only the inner limiting membrane and under-
peripheral a zone (Fig. 13).102,132,135 A peripheral lying RNFL or its remnants cover the sclera,47 whereas
zone (a zone) is characterized by an irregular hy- in the glaucomatous b zone, Bruch’s membrane and
popigmentation and hyperpigmentation and inti- the choroid are interposed between the remnants of
mated thinning of the chorioretinal tissue layer. On the retina and the sclera.127,165
its outer side it is adjacent to the retina, and on its
inner side it is in touch with a zone characterized by C. PARAPAPILLARY ATROPHY IN GLAUCOMATOUS
visible sclera and visible large choroidal vessels AND NONGLAUCOMATOUS OPTIC NERVE DAMAGE
(b zone) or with the peripapillary scleral ring, re- Size, shape, and frequency of both zones do not
spectively. Features of the inner zone (b zone) are differ significantly between normal eyes and eyes
marked atrophy of the retinal pigment epithelium with nonglaucomatous optic nerve atrophy.104,151 Both
and of the choriocapillaris, good visibility of the zones are significantly larger and the b zone occurs
large choroidal vessels and the sclera, thinning of more often in eyes with glaucomatous optic nerve at-
the chorioretinal tissues, and smooth margins to the rophy than in normal eyes.102,132,135,264 Size of both
adjacent a zone on its peripheral side and to the zones and frequency of the b zone are significantly
peripapillary scleral ring on its central side. If both correlated with variables indicating the severity of
zones are present, the b zone is always closer to the the glaucomatous optic nerve damage, such as neu-
optic disk than the a zone. roretinal rim loss, decrease of retinal vessel diame-
In indirect and direct clinical-histologic compari- ter, reduced visibility of the retinal nerve fiber bun-
sons, the b zone correlates with a complete loss of dles, and perimetric defects. A large b zone or “halo
retinal pigment epithelium cells and a markedly di- glaucomatosus” is often associated with a marked de-
minished count of retinal photoreceptors.58,127,165 Ac- gree of fundus tessellation, a shallow glaucomatous
cordingly, the circle of Zinn-Haller can be visualized disk cupping, a relatively low frequency of disk hem-
in some eyes in vivo in the area of parapapillary atro- orrhages and detectable localized defects of the
phy.200 The a zone is the equivalent of pigmentary ir- RNFL, a mostly concentric loss of neuroretinal rim,
regularities in the retinal pigment epithelium. Thus, and normal or almost normal IOP measurements.102,110
the b zone corresponds psychophysically to an abso- The location of parapapillary chorioretinal atrophy
lute scotoma and the a zone to a relative scotoma. is spatially correlated with the neuroretinal rim
14,176,260
It is unclear whether the observed thinning loss in the intrapapillary region.102,132 It is larger in
of the uvea in eyes with glaucoma,164,186 suggesting a that sector with the more marked loss of neuroreti-
decreased uveal blood flow, is pathogenetically con- nal rim. Accordingly, it is relatively largest in that
nected with the development of parapapillary chorio- quadrant that has the longest distance to the exit
retinal atrophy in glaucomatous eyes. Indocyanine of the central retinal vessel trunk on the lamina
green angiography showed areas of hypofluores- cribrosa.97
cence in the peripapillary region in late-phase angio- The general opinion about the association be-
grams in about two thirds of eyes with glaucoma tween parapapillary atrophy and glaucoma, how-
compared with 20% of control eyes. These hypofluo- ever, has been divided.44,187,217 Derick et al reported
rescent areas were discussed to be either the result that, in monkeys with experimental glaucoma, para-
of blockage of background fluorescence by pigment papillary atrophy did not markedly enlarge after IOP
or caused by an absence of vascular tissue in the level had been elevated and that the presence of peripap-
of the choriocapillaris.188 illary crescent was not significantly associated with
OPHTHALMOSCOPY OF OPTIC NERVE HEAD 307

Fig. 13. Glaucomatous optic disks with parapapillary atrophy: alpha zone (white arrowheads), beta zone (white arrows).
Note the focal narrowing of the retinal arteriole in the top left photograph (large black arrow). The small black arrows
point toward the peripapillary scleral ring.

the development of glaucomatous optic disk cup en- larger and the b zone was found significantly more
largement.44 This contradicts the results of a recent often in the affected eyes than in the contralateral
investigation in which parapapillary atrophy, espe- nonglaucomatous eyes. Eliminating the effect of sys-
cially the b zone, was significantly larger, and the b temic parameters, such as age, atherosclerosis, and
zone occurred significantly more often in monkey arterial blood pressure in these intraindividual inter-
eyes after an induced increase in IOP.78 One possi- eye comparisons, these correlations suggest an asso-
ble reason for the discrepancy between these two ciation between parapapillary chorioretinal atrophy
studies on experimental glaucoma may be that the and the degree of glaucomatous optic nerve atro-
follow-up time in the investigation by Derick et al phy.102 This agrees with significant correlations on
was considerably shorter than that in the other increasing frequency and enlarging area of parapap-
study; thus, in Derick’s study, parapapillary atrophy illary atrophy with decreasing area of neuroretinal
may not have had enough time to develop. rim,102,132,208,264,275 diminishing visibility of RNFL,139
In clinical studies as well as in investigations on ex- increasing visual field defect,13,14,102,108,132,199 decreased
perimental high-pressure glaucoma in monkeys, side temporal contrast sensitivity as determined by a full-
differences in parapapillary atrophy were signifi- field flicker test,92 and decreasing diameter of the
cantly correlated with side differences in neuroretinal retrobulbar part of the optic nerve as measured sono-
rim area and mean visual field defect.102 In unilateral graphically.46 In recent follow-up studies by Tezel et
glaucoma, parapapillary atrophy was significantly al, progression of parapapillary atrophy, especially
308 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

the b zone, was described as an early glaucomatous E. CONCLUSION

finding in some patients with ocular hypertension.266 In conclusion, parapapillary chorioretinal atrophy
Accordingly, presence and size of parapapillary atro- is one among other morphologic variables to detect
phy were related to the development of subsequent glaucomatous abnormalities in the optic nerve head.
optic disk or visual field damage in ocular hyperten- In a ranking list of optic disk variables for the detec-
sive patients.257,265 In a study on patients with NPG, tion of glaucomatous optic nerve damage, it is a vari-
disk hemorrhages were closely associated with the able of second order. As one among other parame-
size of parapapillary atrophy, underlining the impor- ters, it is useful for the differentiation of the various
tance of parapapillary atrophy in the morphologic types POAG and SOAG. In contrast to glaucomatous
diagnosis of glaucomatous optic neuropathy.261 eyes, eyes with nonglaucomatous optic nerve atro-
In contrast to glaucomatous optic neuropathy, phy, including eyes after nonarteritic anterior isch-
nonglaucomatous optic nerve damage does not lead emic optic neuropathy, have a normal parapapillary
to an enlargement of parapapillary atrophy (Fig. atrophy.104,151 Evaluation of the parapapillary atro-
14).104,151 This indicates that parapapillary atrophy is phy can, therefore, be helpful in the differential di-
one among other optic disk variables to differentiate agnosis of glaucomatous versus nonglaucomatous
between glaucomatous and nonglaucomatous optic optic nerve damage.
nerve damage.

XII. Diameter of Retinal Arterioles

D. PARAPAPILLARY ATROPHY IN THE VARIOUS
TYPES OF OPEN-ANGLE GLAUCOMAS A. DIFFUSE ARTERIOLE NARROWING

Parapapillary chorioretinal atrophy is helpful for Diffuse narrowing of the retinal vessels has been
the papillomorphologic differentiation of various described for glaucomatous and nonglaucomatous
types of open-angle glaucomas. The open-angle optic neuropathies (Fig. 14).63,104,151,133,137 In glau-
glaucomas are a heterogenous group of diseases that coma, the vessel diameter reduces with decreasing
vary in the level of IOP, age of the patients, preva- area of the neuroretinal rim, diminishing visibility of
lence of arterial hypotension, refractive error, and the RNFL and increasing visual field defects.137 Be-
atrophic appearance of the posterior fundus. These cause the reduction of the vessel caliber is also
forms of open-angle glaucomas differ in the appear- found in eyes with nonglaucomatous optic nerve
ance of the optic disk, including the presence and damage, such as descending optic nerve atrophy63,104
area of parapapillary chorioretinal atrophy. and nonarteritic anterior ischemic optic neuropa-
The b zone of parapapillary atrophy is signifi- thy,151 one can infer that a generalized reduction of
cantly larger in eyes with highly myopic POAG98 than the vessel diameter is typical of optic nerve damage
in eyes with age-related atrophic POAG,110 in which but not characteristic of glaucoma. From a pathoge-
the b zone is significantly larger than in eyes with netic point of view, it suggests that vessel reduction
SOAG caused by pseudoexfoliation of the lens does not cause glaucomatous optic nerve fiber loss
(pseudoexfoliative glaucoma),141 primary melanin but, at least partially, is secondary to a reduced de-
dispersion syndrome (pigmentary glaucoma),100 and mand in the superficial layers of the retina.
non–highly myopic POAG. The b zone is signifi-
cantly the smallest in juvenile-onset POAG,111 B. FOCAL ARTERIOLE NARROWING
The findings in patients with focal NPG are con- Rader et al recently drew attention to focal nar-
tradictory. Some studies suggested that parapapillary rowing of the retinal arterioles in the intrapapillary
atrophy is larger in size in NPG than in POAG264; and peripapillary region of eyes with glaucoma (Fig.
other investigations found that eyes with NPG and 13 top left) or nonarteritic anterior ischemic optic
eyes with POAG do not markedly differ in parapapil- neuropathy.219 Similar observations were made by
lary atrophy.152 In a recent study in which eyes with Ratkin and Drance229 and others.197 The degree of
the focal type of NPG were separated from other focal narrowing of the retinal arterioles increased
glaucomatous eyes with normal IOP, the b zone was significantly with age in normal eyes.195,197 Corrected
significantly smaller in eyes with focal type of NPG for age, it was significantly higher in eyes with optic
(Fig. 12) than in eyes with POAG and elevated nerve atrophy than in normal eyes. Eyes with glau-
IOP.109 In the same study, eyes with focal NPG and coma and the eyes with nonglaucomatous optic
eyes with juvenile-onset POAG did not vary signifi- nerve damage did not vary significantly in the sever-
cantly in the b zone, despite marked differences in ity of focal narrowing. Focal arteriole narrowing was
IOP. Of all glaucoma types examined in this study, slightly more pronounced in eyes with NPG and eyes
except focal NPG, the b zone increased significantly with nonarteritic anterior ischemic optic neuropathy
with decreasing mean IOP. than in the other groups.197 These differences, how-
OPHTHALMOSCOPY OF OPTIC NERVE HEAD 309

Fig. 14. Optic disks with nonglaucomatous optic nerve damage: alpha zone of parapapillary atrophy (white arrowheads).
Note the decreased visibility of the retinal nerve fiber layer, the reduced caliber of the retinal arterioles, and the absence
of normal parapapillary atrophy (no beta zone). There is no marked disk cupping. The black arrows point toward the peri-
papillary scleral ring.

ever, were not marked. In the glaucoma group, the teriole narrowing, considerable hemodynamic changes
degree of focal narrowing of the retinal arterioles may occur.
was significantly more pronounced if the optic nerve The finding that focal arteriole narrowing was
damage was more advanced. A recent study compar- mostly independent of parapapillary atrophy197
ing fundus photographs and fluorescein angiograms points against the hypothesis that vasoconstrictive
with each other showed that focal narrowing of the factors in the parapapillary region could lead to a fo-
retinal arterioles in the parapapillary region of eyes cal vasospasm in the retina and simultaneously to
with optic neuropathies represents a real stenosis of atrophic changes in the deep retinal layers, retinal
the vessel lumen and is not attributable to an oph- pigment epithelium, and the choroid. The finding
thalmoscopic artifact.194 Considering that the vessel that focal vessel attenuation was observed in glauco-
diameter can be reduced by 50% or more in focal ar- matous eyes and in eyes with nonglaucomatous optic

Fig. 15. A glaucomatous minidisk with pseudonormal but glaucomatous minicup with markedly reduced visibility of the
retinal nerve fiber layer and reduced caliber of the retinal arterioles is shown on the left, in contrast to the normal mini-
disk with very good visibility of the retinal nerve fiber layer shown on the right.
310 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

nerve atrophy leads one to infer that focal narrowing tected by examining eyes with clear optical media
of the retinal arterioles is part of a panoply of and a normal fundus pigmentation. Minor defects
changes characteristic of any optic nerve damage. Its are better seen on RNFL photographs, especially in
occurrence in both groups indicates that focal vessel patients who do not cooperate well. In juvenile
narrowing is not specific for glaucoma and that it subjects, the evaluation of the RNFL is more diffi-
does not play a major specific role in the pathogene- cult than in adults, because the reflectivity of the in-
sis of the disease. This hypothesis is further favored ner limiting membrane is considerably higher in
by the fact that reduced blood perfusion, as in cen- children.
tral retinal artery occlusion or nonarteritic anterior Several techniques for the clinical evaluation of
ischemic optic neuropathy,104,151 does not markedly the RNFL in glaucoma have been reported. Among
decrease the neuroretinal rim area. In glaucoma, them are red-free ophthalmoscopy,280,281 nerve fiber
however, the neuroretinal rim area is reduced.6,23 layer photography with black-and-white film,10,250
This finding points against a deficiency of retinal computerized images to measure the relative height
blood perfusion as the reason for the glaucomatous of the peripapillary nerve fiber layer surface,34,36 po-
loss of neuroretinal rim and optic nerve fibers. larimetric determinations of the RNFL thickness,
234,267,282–284
densitometry of the reflectance of the
RNFL,55 photogrammetric measurements of the RNFL
XIII. Evaluation of the Retinal Nerve thickness,202,240,263 and measurement of the RNFL
Fiber Layer contour in the peripapillary region by confocal laser
scanning tomography systems.29,31 Sommer248 noted
A. OPHTHALMOSCOPIC EVALUATION that adding polarized light can improve the visual-
The RNFL contains the retinal ganglion cell axons ization of the RNFL. Airaksinen emphasized the use
covered by astrocytes and bundled by processes of of a wide-angle fundus camera, using high-resolu-
Müller cells. It can be assessed ophthalmoscopically280,281 tion, fine-grain, black-and-white films with a blue
on wide-angle, red-free photographs4,10,84,249 or by us- monochromatic interference filter (wavelength 495
ing sophisticated techniques, such as scanning laser nm).4,10 The same filter and camera can be used for
tomography or laser polarimetry.234,267,282–284 For oph- conventional fundus fluorescein angiography. Re-
thalmoscopic evaluation, it is helpful to use green cently, Tuulonen et al showed that the RNFL can
light. In eyes with opaque media, a yellow lens color- also be evaluated in photographs taken with a non-
ation, and a low degree of pigmentation of the reti- mydriatic fundus camera.273 With only color fundus
nal pigment epithelium, the RNFL is less visible than photographs available, the RNFL detectibility can be
in eyes with clear media and deeply pigmented reti- improved by reproducing the color slides through a
nal pigment epithelium. green filter on black-and-white film, as reported by
The retinal nerve fibers or, better, the retinal Hoyt93,94 and Frisén.62 Because of the higher resolu-
nerve fiber bundles are ophthalmoscopically detect- tion power of a low-sensitive, black-and-white film as
able as bright and fine striations in the inner retinal compared with a color film, the detectibility of the
layer fanning off the optic disk to the retinal periph- RNFL is inferior on reproduced color fundus photo-
ery.4,10,93,94,99,249,280,281 According to electron micro- graphs compared with photographs taken with the
scopic studies,222 these fine striations represent tissue method described by Airaksinen.
canals, in which processes of the Müller cells gather
the axons together into bundles with a diameter of B. CLINICAL FINDINGS IN NORMAL EYES
about 20 mm. In the temporal and nasal parapapil- In normal eyes, visibility of the RNFL is regionally
lary region, the striations are finer and consist of one unevenly distributed. Dividing the fundus into eight
fiber bundle per stripe only. In the temporal inferior regions, the nerve fiber bundles are most visible in
and temporal superior fundus regions, the striations the temporal inferior sector, followed by the tempo-
are broader and have several bundles per stripe.222 ral superior area, the nasal superior region, and fi-
The bright stripes lying between the dark lines are nally the nasal inferior sector.138,147 It is least visible
formed by the processes of the Müller cells, with op- in the superior, inferior, temporal horizontal, and
tical properties other than the retinal nerve fibers nasal horizontal regions. Accordingly, the diameters
themselves. The retinal nerve fibers have only a little of the retinal arterioles are significantly widest at the
tendency to leave their bundle. temporal inferior disk border, followed by the tem-
By ophthalmoscopy, the RNFL can be examined poral superior disk region, the nasal superior area,
through a dilated pupil using green light. The use of and finally the nasal inferior disk region.147 This is
a Goldmann contact lens provides a better detect- consistent with the location of the foveola below a
ability of the RNFL than an indirect ophthalmo- horizontal line drawn through the center of the op-
scope. Most wedge-shaped RNFL defects can be de- tic disk138 and with the configuration of the neu-
OPHTHALMOSCOPY OF OPTIC NERVE HEAD 311

roretinal rim, which is broadest at the temporal infe- tic nerve damage, RNFL defects are usually no
rior disk border, followed by the temporal superior longer detectable because of the pronounced loss of
disk region.118 The sectors’ sequence concerning the nerve fibers in all fundus sectors. Localized RNFL
best visibility of the RNFL correlates with the sectors’ defects occur more often in eyes with the focal type
sequence in respect to rim configuration and retinal of NPG than in eyes with the age-related atrophic
artery caliber. Physiologically, it points toward an an- type of open-angle glaucoma, the highly myopic type
atomic and nutritional relationship. Visibility of the of open-angle glaucoma, and the juvenile-onset type
RNFL decreases with age.138,147 It correlates with an of POAG.98,110,111,146 In their vicinity at the optic disk
age-related reduction of the optic nerve fiber count, border, one often finds notches of the neuroretinal
with an annual loss of about 4,000 to 5,000 fibers per rim, sometimes an optic disk hemorrhage, and a
year out of an original population of presumably 1.4 parapapillary chorioretinal atrophy that is more
million optic nerve fibers.16,148,174 These features of marked in that sector than in other sectors.9,146,150
the normal RNFL are important for diagnosis of Localized RNFL defects are often found 6 to 8 weeks
RNFL changes secondary to optic nerve damage in after optic disk bleeding.9 They point toward a local-
the diseased eye. ized type of optic nerve damage.
With respect to different sectors of the fundus, local-
ized RNFL defects are most often found in the tempo-
C. CLINICAL FINDINGS IN GLAUCOMATOUS EYES ral inferior sector, followed by the temporal superior
By definition, glaucomatous optic nerve atrophy is sector.146 In the nasal fundus region, localized RNFL
associated with an optic nerve fiber loss and, thus, defects are rarely seen.146 This may be attributable to
decreased visibility of the RNFL (Figs. 15–17). This the fact that the RNFL in normal eyes is less detectable
nerve fiber loss can occur in a diffuse way or in the in the nasal fundus than in the temporal inferior and
form of localized defects. temporal superior fundus areas.138,147 In fundus areas
in which the RNFL is physiologically thin, localized de-
1. Localized Retinal Nerve Fiber Layer Defects fects are harder to find than in areas with a thick
In 1973 Hoyt et al93,94 were the first to report on RNFL. It is unclear whether the morphology of the
the significance of localized RNFL defects (Fig. 17 lamina cribrosa, with larger pores in the inferior and
right) in glaucomatous eyes. Localized defects of the superior sectors and smaller pores in the temporal and
RNFL are defined as wedge-shaped and not spindle- nasal regions, also plays a role in the development of
like defects, running toward or touching the optic localized RNFL defects.129,210,220
disk border. If they are pronounced, they can have a The importance of localized defects of the RNFL
broad basis at the temporal raphe of the fundus. for the diagnosis of glaucoma has been shown in
Typically occurring in about 20% of all glaucoma many studies. Airaksinen9 described clearly detect-
eyes,146 they can also be found in eyes with an atro- able wedge-shaped defects of the RNFL in eyes with
phy of the optic nerve caused by other factors, such increased IOP and normal visual field. These eyes
as optic disk drusen, toxoplasmotic retinochoroidal showed later localized perimetric changes when the
scares, ischemic retinopathies with cotton-wool spots area of concern was examined.6
of the retina, or after long-standing papilledema or Experimental studies have shown that localized
optic neuritis caused by multiple sclerosis.42,145 Be- RNFL defects can ophthalmoscopically be detected
cause the localized RNFL defects are not present in if more than 50% of the thickness of the RNFL is
normal eyes, they almost always signify a pathologic lost.209 This can be explained by the “sandwich” ar-
abnormality.146 This is important for subjects with rangement of the retinal nerve fiber bundles in the
ocular hypertension, in which a localized RNFL de- RNFL. The first glaucomatous defects concern mainly
fect points to optic nerve damage even in the ab- retinal ganglion cells close to the temporal raphe of
sence of perimetric abnormalities.9,126,185,215,247 One the retina. Their axons are located in the deep and
has to consider, however, that localized RNFL de- middle layer of the RNFL. If these axons are lost, the
fects are not pathognomonic for glaucoma, because configuration of the surface of the RNFL is only
they also occur in other types of optic nerve atro- slightly changed, because the axons over the lost fi-
phy.42,145 Because of their relatively low frequency in bers still cover the defect under them. The localized
eyes with optic nerve damage, their sensitivity to in- RNFL defects have to be differentiated from slit-like
dicate optic nerve atrophy is not very high. or groove-like (pseudo) defects, which often do not
In glaucomatous eyes, the frequency of localized extend to the optic disk border and do not have a
RNFL defects increases significantly from “early” broad base close to the temporal raphe of the fun-
glaucoma to medium advanced glaucomatous dam- dus. This includes a so-called cleavage of the RNFL,
age and then decreases again with very marked glau- which can mimic a true defect of the RNFL, espe-
comatous changes.146 In eyes with very advanced op- cially in high myopia.41
312 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

Fig. 16. A normal minidisk with very good visibility of the retinal nerve fiber layer is shown on the left, in contrast to the
glaucomatous optic disk shown on the right, which depicts a reduced visibility of the retinal nerve fiber layer in a minidisk
with reduced caliber of the retinal arterioles.

2. Diffuse Loss cerning the best RNFL visibility.”138,139,147 If the RNFL

In addition to localized RNFL defects, a diffuse is markedly more detectable in the temporal supe-
loss of retinal nerve fibers occurs in eyes with dam- rior fundus region than in the temporal inferior sec-
age of the optic nerve. This leads to a decreased visi- tor in an eye without fundus irregularities, it points
bility of the RNFL. After sectioning of the optic toward a loss of RNFL, mainly in the temporal infe-
nerve in the orbit of monkeys, Quigley214 observed rior fundus region. This variable can be examined
loss of the visibility of the RNFL starting 1 month af- on ophthalmoscopy without applying sophisticated
ter the operation and complete 4 weeks later. Oph- techniques. It is also helpful to evaluate whether the
thalmoscopically, the diffuse RNFL loss is more diffi- retinal vessels are clearly and sharply detectable. The
cult to detect than a localized defect. It is helpful to retinal vessels are normally embedded in the RNFL.
use the variable “sequence of fundus sectors con- In eyes with a diffuse RNFL loss, the retinal vessels

Fig. 17. A normal macrodisk with good visibility of the retinal nerve fiber layer and normal configuration of the neu-
roretinal rim is shown on the left, in contrast to the normal-sized optic disk shown on the right, which depicts a markedly
reduced visibility of the retinal nerve fiber layer—including a localized defect of the retinal nerve fiber layer (white arrow-
heads) and an abnormal configuration of the neuroretinal rim, with notching at the 4:30 o’clock position correspond-
ing to the localized retinal nerve fiber layer. The black arrows in the left photograph point toward the peripapillary
scleral ring.
OPHTHALMOSCOPY OF OPTIC NERVE HEAD 313

are covered only by the inner limiting membrane, the optic cup in relation to the size of the optic disk,
resulting in a better visibility and a sharper image of diffusely or segmentally decreased visibility of the
the large retinal vessels. This is an important variable RNFL, and occurrence of localized RNFL defects
in the diagnosis of optic nerve damage. and disk hemorrhages.
Pathogenetically, it is unclear whether the local- If the rim is not markedly broader in the inferior
ized defects and the diffuse loss of the RNFL repre- and superior disk regions as compared with the tem-
sent two pathomechanisms or whether they are two poral disk region, a glaucomatous loss of rim tissue
extremes of the same process.49 The contrast be- may be suspected in the inferior and superior disk
tween localized and diffuse RNFL loss, the varying regions. In other words, if the neuroretinal rim is
frequencies of localized RNFL defects in different more or less even in width in all disk sectors, glauco-
types of glaucoma,98,110,111,146 and the association be- matous optic nerve damage can be suspected (Fig.
tween localized RNFL defects and optic disk hemor- 8). In the evaluation of the shape of the neuroreti-
rhages,9,146,150 however, lead to the assumption of the nal rim in glaucomatous eyes, one must account for
latter. It is also unknown whether all nerve fibers the fact that the rim configuration depends on the
within a nerve fiber bundle are lost simultaneously distance to the exit of the central retinal vessel trunk
or whether there is a gradual loss of fibers within a on the lamina cribrosa surface. To cite an example,
bundle, resulting in a progressive thinning of the in glaucoma eyes with the vessel trunk abnormally
nerve fiber bundle. exiting in the superotemporal disk quadrant, the
For glaucoma, many studies have shown that dis- neuroretinal rim is often smallest in the inferonasal
turbances of the RNFL are correlated with other disk region (Fig. 9).
variables indicating the degree of glaucomatous op- In eyes with small disks, the neuroretinal rim can
tic nerve damage.2–4,52,140,154,167,216,242,272,276 not clearly be delineated from the optic cup, thus,
Considering its importance in the assessment of the shape of the rim can not be determined well. In
anomalies and diseases of the optic nerve and the these eyes, the variable “cup size in relation to disk
feasibility of its ophthalmoscopic evaluation, the size” is the most important intrapapillary factor to
RNFL should be examined during every routine detect glaucomatous optic nerve damage (Fig. 11).
ophthalmoscopy. This holds true especially for pa- In eyes with small optic disks, the question arises
tients with early damage of the optic nerve. The im- whether an optic disk of this size is physiologically
portance of evaluating the RNFL is indicated further capable of having an optic cup.
in studies in which glaucomatous damage of the op-
tic nerve could have been detected earlier by exami- XV. Differentiation of Glaucomatous
nation of the RNFL than by conventional computer- versus Nonglaucomatous Optic Neuropathy
ized perimetry. It is of utmost importance for the Glaucomatous and nonglaucomatous optic neur-
detection of glaucoma in eyes with a pseudonormal opathy have in common both a decreased diameter
but glaucomatous minicup in minidisks,105 and it is of the retinal arterioles, including the occurrence of
useful to classify an eye with a pseudoglaucomatous focal arteriole narrowing, and a reduced visibility of
but normal large cup in a large disk as well.153 In eyes the RNFL. Localized RNFL defects can be found in
with advanced optic nerve atrophy, other examina- glaucoma and in many types of nonglaucomatous
tion techniques, such as perimetry, may be more optic nerve damage, such as in optic disk drusen and
helpful for the follow-up of optic nerve damage. long-standing papilledema. Compared with non-
Evaluation of the RNFL is also very useful in eyes glaucomatous optic nerve atrophy, the optic cup en-
with nonglaucomatous optic nerve damage. The larges and deepens in glaucomatous optic neuropa-
combination of decreased visibility of the RNFL, a thy, and, in a complementary manner, the neuroretinal
reduced caliber of the retinal arterioles, and an in- rim decreases. In addition to glaucoma, an enlarge-
creased pallor of the optic disk with an unremark- ment of the optic cup and a loss of neuroretinal rim
able size and shape of the optic disk, neuroretinal may be found in patients after arteritic anterior is-
rim, and parapapillary atrophy characterizes non- chemic optic neuropathy and in a few patients with
glaucomatous optic nerve atrophy and distin- intrasellar or suprasellar tumors. Because parapapil-
guishes it from the glaucomatous type of optic nerve lary atrophy does not usually occur in eyes with non-
damage. glaucomatous optic nerve damage, it is helpful for
the differentiation of glaucomatous versus nonglau-
XIV. Early or Preperimetric Diagnosis of comatous optic neuropathy.
Glaucomatous Optic Nerve Damage
For the early detection of glaucomatous optic Methods of Literature Search
nerve damage in ocular hypertensive eyes before the A search of the MEDLINE database was con-
development of visual field loss, the most important ducted with the following search words: optic disk,
variables are shape of the neuroretinal rim, size of neuroretinal rim, optic cup, peripapillary scleral
314 Surv Ophthalmol 43 (4) January–February 1999 JONAS ET AL

ring, parapapillary atrophy, optic disk hemorrhages, 19. Becker B: Cup/disk ratio and topical corticosteroid testing.
Am J Ophthalmol 70:681–685, 1970
and optic disk morphometry. The search covered 20. Bengtsson B: Optic disk haemorrhages preceding manifest
the years from 1970 to 1997. Additional sources in- glaucoma. Acta Ophthalmol 68:450–454, 1990
cluded publications cited in other articles. Criteria 21. Bengtsson B: The variation and covariation of cup and disk
diameters. Acta Ophthalmol 54:804–818, 1976
for inclusion or exclusion of articles were originality, 22. Bengtsson B, Holmin C, Krakau CET: Disk haemorrhages
importance for the ophthalmoscopic evaluation of and glaucoma. Acta Ophthalmol 59:1–14, 1981
the optic nerve head without using sophisticated ex- 23. Betz P, Camps F, Collignon-Brach J, et al: Biometric study
of the disk cup in open-angle glaucoma. Graefes Arch Clin
amination techniques, and new findings in the anat- Exp Ophthalmol 218:70–74, 1982
omy and pathomorphology of the optic disk with 24. Betz P, Camps F, Collignon-Brach D, Weekers R: Photo-
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tion physiologique de la papille. J Fr Ophthalmol 4:193–
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review. neuroretinal rim of the optic nerve in normal eyes. Am J
Ophthalmol 103:497–504, 1987
26. Bücklers M: Anatomische Untersuchung über die Bezie-
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Gelbfärbung der Linse ophthalmoskopisch nachweisbar X. Optic disk hemorrhages

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560, 1990
D. Pathogenetic aspects
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Am J Ophthalmol 81:341–345, 1976 XII. Diameter of retinal arterioles
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lation of visual field defect with exit point of central retinal
A. Diffuse arteriole narrowing
vein in NTG (abstract). Invest Ophthalmol Vis Sci 36(Suppl): B. Focal arteriole narrowing
S79, 1995 XIII. Evaluation of the retinal nerve fiber layer
A. Ophthalmoscopic evaluation
Outline B. Clinical findings in normal eyes
C. Clinical findings in glaucomatous eyes
I. Optic disk size
1. Localized retinal nerve fiber layer defects
A. Biologic and demographic correlates
2. Diffuse loss
B. Microdisks and macrodisks
XIV. Early or preperimetric diagnosis of glaucomatous
C. Morphogenetic and pathogenetic implications
optic nerve damage
D. Optic disk diameters
XV. Differentiation of glaucomatous versus
E. Measurement of the optic disk size
nonglaucomatous optic neuropathy
II. Optic disk shape
XVI. Methods of literature search
III. Neuroretinal rim size
IV. Neuroretinal rim shape
V. Neuroretinal rim pallor
VI. Optic cup size in relation to the optic disk size
VII. Configuration and depth of the optic cup Supported by Deutsche Forschungsgemeinschaft (SFB 539).
VIII. Cup/disk ratios Reprint address: Dr. J. Jonas, Universitäts-Augenklinik, Schwa-
IX. Position of the exit of the central retinal vessel trunk bachanlage 6, 91054 Erlangen, Germany. E-mail: Jost.Jonas@
on the lamina cribrosa surface augen.med.uni-erlangen.de