IIH, also known as pseudotumor cerebri, is a disorder of elevated
intracranial pressure of unknown cause. The diagnosis is mainly based
on the exclusion of other pathologic conditions responsible for
papilledema and/or headaches (according to modified Dandy
criteria
28 ). Thus, lumbar puncture documenting raised CSF
pressure without abnormalities in protein level or cell count, and
normal neuroimaging studies (CT and MRI)
18 19 are
necessary.
The possibility of visual loss is very real and demands a regular
monitoring of patients with IIH for detecting functional
15 and morphologic
8 23 24 changes of the optic nerve. Despite
some different evidences in the past,
7 9 10 11 15 25 26 a recent report on patients with IIH with
asymmetric papilledema has indicated that the amount of visual loss
qualitatively correlates with the severity of disc edema, implying that
visual loss in IIH is caused by papilledema and not by a retrolaminar
mechanism.
42 Therefore, either for diagnosis or follow-up,
accurate examination of disc swelling may provide additional valuable
information to the perimetric evaluation. In the modern clinical
setting, accordingly to optic disc conditions, morphologic assessment
is performed by common but very subjective methods such as direct
ophthalmoscopy
8 and stereoscopic slit lamp biomicroscopy
using the 60-, 78-, or 90-D lens,
43 44 or by the more
detailed fluorescein angiography,
6 retinal nerve
fiber layer photographs,
12 planimetry,
45 46 stereophotogrammetry,
47 and,
mainly, recent computer-assisted technologies such as optical coherence
tomography, scanning laser polarimetry, or confocal scanning laser
tomography.
39 The use of echography in the objective
evaluation of the optic nerve head has been advocated by some
investigators,
1 2 3 4 5 and the accuracy and reproducibility of
US readings have been assessed in relation to those of confocal
scanning laser tomography by the HRT in disc cupping.
4
We studied the reliability of US readings of disc elevation in patients
with IIH evaluating the degree of agreement with measurements obtained
with the HRT. The same evaluation was conducted in a group of subjects
with ophthalmoscopic evidence of variable degree of physiologic disc
excavation to verify our reliability by comparing these data with those
from a recent report.
4 The US measurements of disc
elevation significantly correlated with the HRT readings, although a
stronger correlation existed in disc cupping. The amounts of
correlations, however, were not high for a comparison of measuring
techniques, especially in the disc-swelling condition. Indeed,
examination of the correlation coefficients (
r = 0.62
and
r = 0.84, for disc swelling and cupping,
respectively) reveals that the two variables can only account for
nearly 38% and 71%, respectively, of each other’s variance
(
r 2). When evaluating the agreement
instead of the strength of the relationship,
41 a larger
discrepancy between the methods was obtained for swelling (0.24 ±
0.59 mm, mean ± 2 SD) than for cupping evaluation (0.05 ±
0.3 mm), with a tendency for the US measurements to be higher than the
HRT measurements with increasing magnitude of the examined structure.
In contrast, the agreement decreased at both measurement extremes. The
amount of the discrepancies in the two groups differed significantly
(
P < 0.05), suggesting that the optic disc conditions
might contribute to the different degree of agreement. Specifically, in
our opinion it may be explained, at least in part, by the reference
planes used for measurements of cupping and papilledema in the two
methods.
In cupping, the tomographic MxCD parameter measures the greatest
vertical distance from the HRT curved surface (i.e., the peripapillary
inner retinal surface) to the bottom of the excavation (the lamina
cribrosa, approximately).
40 The US method evaluates the
distance between the profile of the inner retinal surface and the
lamina cribrosa.
2 4 Therefore, in cupping evaluation, both
methods use similar planes.
In papilledema, the tomographic MxHC parameter measures from the
maximum elevation of the disc to the curved surface (i.e., the retinal
surface), because the lamina cribrosa is no more recognizable than it
is in cupping. US readings are taken from the top of the
elevated disc to the lamina cribrosa, which is still identifiable
because of its higher reflectivity than the surrounding tissues. The
distance between the inner retinal surface and the lamina cribrosa
could account in part for the difference between the measurements
obtained with US and HRT. The individual amount of such distance
depends on the physiologic variability among optic disc excavations
existing before the development of the edema.
However, there was much variation in the discrepancies among patients
with IIH
(Fig. 3) . In addition, the evidence of variation even among a
few subjects of the cupping group
(Fig. 4) suggests that the different
agreement may be accounted for by factors other than the difference in
the reference planes. Nevertheless, the 95% limits of agreement found
in cupping depth evaluation were similar to those from a recent
report
4 (0.05 ± 0.3 versus 0.087 ± 0.328 mm),
confirming the reliability of our procedure.
Analysis of individual data shown on the plots of
Figures 3 and 4 with
respect to the identity line indicates that there was a trend in the
bias—that is, a tendency for the mean difference to increase as the
magnitude of the measurements increased, in both conditions. Such
behavior may depend on measuring errors owed to US or HRT accuracy or
both.
The ideal perpendicular US scanning plane to the disc should pass
through the lens, but it is not commonly used because refraction of the
sound beam at the lens surfaces and nonpredictable angle of incidence
on posterior pole occur. Therefore, the probe is placed as close as
possible to the corneal limbus displaying the maximal vitreous length.
In this case, the lens is avoided, but minimal obliquity may be
introduced in the optic disc.
To the best of our knowledge, although sufficient accuracy of the HRT
was reported on volumetric measurements of both excavations and
elevations,
35 only one study concerning the accuracy of a
predecessor of the HRT on depth measurements has yet been
published,
48 and the accuracy on height measurements has
never been investigated. Moreover, no study specifically evaluated
accuracy of the HRT on both disc elevations and excavations of
increasing magnitude. Variable accuracy may account for an additional
measurement error especially affecting, in this study population,
height measurements, which were larger than depth measurements.
Additionally, even though we verified that the contour line was placed
on flat retina avoiding the presence of humps on the contour line
height variation diagram (i.e., small areas of local retinal edema
under the contour line), mild retinal edema uniformly involving even
the periphery of the tomographic image may be responsible for a slight
underestimate of the MxHC parameter in large-sized papilledema.
Good reproducibility was found in cupping evaluation either for
tomographic MxCD measurements (5.91%), which agrees with previous
studies in which coefficient of variation ranged from 4% to
9%,
32 34 49 50 or for US readings (7.93%). The similar
US coefficient of variation found in swelling evaluation (7.63%)
indicates that US assessment of swollen discs can be considered a
highly reproducible method in observing patients with IIH and
papilledema. Thus, besides the well-known role of echography in
indirectly estimating CSF pressure by the determination of optic nerve
sheaths diameters,
21 22 the present study suggests a
further potential usefulness of the technique in patients with IIH with
ophthalmoscopic evidence of disc swelling. Serial contiguous B-scans
(three horizontal and three vertical) of the optic disc from nasal to
temporal and from superior to inferior margins should be recorded and
used for measurements and follow-up. Obviously, longitudinal studies
and improvements in both techniques, which may ameliorate correlation
between the methods, are needed.
The differences between US and HRT readings in papilledema
introduce a new parameter that may provide a good estimate of the full
amount of disc edema: the true disc edema coefficient (TDEC). It
represents the ratio between the ophthalmoscopically nonvisible edema,
that is the space included between the planes of inner retina and
lamina cribrosa (measurable as US-HRT), equivalent to the preexisting
optic disc cup filled by the nerve fiber swelling, and the total height
of the disc edema ultrasonographically measured: TDEC =
(US-HRT)/US. Therefore, it indicates the contribution of the
ophthalmoscopically nonapparent edema to the whole papilledema.
TDECs higher than 0.5 suggest a greater amount of nonapparent than
apparent edema, and vice versa.
To date, the relationship between functional damage and the amount of
whole papilledema (as ultrasonographically measurable) has never been
investigated. Moreover, because quantitative associations between
functional and funduscopic assessment of papilledema in IIH have never
been provided, there is no evidence of whether functional damage is
related mostly to the ophthalmoscopically apparent or nonapparent
edema. The potential ability of TDEC to separate different degrees of
tissue edema, from disc cup disappearance to disc elevation, may be
helpful in this purpose and, when associated with CSF pressure level
assessment, relevant to the understanding of the pathophysiology of
visual loss in IIH. Therefore, even the evaluation of TDEC changes
during the resolution of papilledema may help analyze the relationships
between papilledema and CSF pressure. Additionally, US evaluation of a
possible anterior bowing of the lamina cribrosa related to direct
transmission of elevated CSF pressure, currently attainable by new
generation B-scanners, may provide further findings regarding this
issue. Indeed, previous CT and MRI studies in patients with chronically
high CSF pressure have postulated that the true edema has only a
contributing role in disc elevation, indicating a bulging of the
terminal optic sheath subarachnoid space into the posterior aspect of
the globe at the optic nerve head as a major factor.
51 52
Clinical implications for the TDEC may also be considered. For
instance, patients with similar funduscopic disc elevation
(Fig. 5) may differ in US measurements, and consequently in TDEC, suggesting a
more serious condition and different clinical management for patients
with higher values. Specifically, in spite of equal HRT heights,
patients may show higher US values and TDEC, because of greater
ophthalmoscopically nonapparent edema related to a more posterior
lamina cribrosa position. Nonetheless, even the usefulness of TDEC
needs a less variable discrepancy between these techniques.
In conclusion, the present study, although indicating reproducibility
of US readings and a correlation between US and HRT measurements, shows
a significant discrepancy between the results of the methods of
papilledema evaluation. The weakness of the agreement may be due in
part to different reference planes. Further studies are needed to
determine whether coupling HRT and US assessment of disc elevation may
be clinically useful in the management of patients with IIH and
papilledema. The use of US may be recommended in those patients in
ophthalmologic centers where the HRT is not available and in presence
of marked media opacities.
The authors thank Benedetto Falsini for helpful suggestions in
preparing the manuscript.