125

CHAPTER 12

THE OPTIC DISC IN CONGENITAL GLAUCOMA

12.1 Introduction
My research in primary congenital glaucoma was divided into four periods:
1960 - 1970: Intraocular pressure: normal and pathological.
1970 - 1980: Echometry in the diagnosis and in the follow-up.
1980 - 1990: Functional results in congenital glaucomas: computerized perimetry
(Octopus and high-pass resolution perimetry).
1991 - 1996: Study of the optic disc on the same patients with confocal tomography.

12.2 Intraocular Pressure

In 1967 I showed for the first time that intraocular pressure in the newborn up to 6
years of age, has a mean of 10 mmHg, instead of 15 mmHg like the adult [1] (Sampaolesi
1967).
In America, in 1969 Carvalho and Calixto [2] were the first authors to confirm this
finding, followed by Radtke and Cohan in the USA in 1975 [3]. Tucker and coworkers
confirmed our results as well in 1992. In Europe, Tarkkanen, Reibaldi, Fledelius, Gold-
mann, Dominguez and others also confirmed the same values.
The results we obtained are summarized in figures 12.1 and 12.2. Figure 12.1 shows
a normal range band for intraocular pressure between birth and 6 years of age. The intra-
ocular pressure increases with age. Figure 12.2 displays the intraocular pressure values
obtained in different age groups.

12.3 Diagnosis by echometry

In 1970 I started to measure the axial length with echometry, initially in the eyes of
healthy children. In 1971 I started performing echometry in eyes with congenital glau-
coma and I found that when the intraocular pressure values were doubtful because they
were at the top limit of the normal range, echometry was a very helpful new parameter.
This led us to undertake a systematic study of echometry in normal children with the
purpose of obtaining the mean values and their scatter according to age.
Conversely to what occurs in intraocular pressure readings, the axial length is not af-
fected by general anesthetics.
In 1974, at the Glaucoma Symposium held in Würzburg, I drew the attention for the
first time to the extraordinary value of echometry for the diagnosis of congenital glau-
126

Fig. 12.1

Fig. 12.2

coma as a better parameter than intraocular pressure [5]. Thereafter, I published several
research papers on the subject [6, 7, 8, 9, 10, 11]. Reibaldi in 1982 [12], Betinjane, in
1980 [13] and in 1982 [14], Tarkkanen in 1983 [15], Fledelius in 1983 [16] and Spaeth,
in 1983 [17], proved the value of echometry in the early diagnosis of congenital glau-
coma and its superiority over the tonometry.
Results: We studied a group of 100 normal eyes of up to 84 months of age (7 years).
In figure 12.3 the ordinate represents the axial length in mm and the abscissa represents
the age expressed in months on a logarithmic scale. The dotted area is the area of nor-
mality. Figure 12.4 represents 79 eyes with congenital glaucoma, where all cases fall
above the normal range band.
 127

Fig. 12.3 Fig. 12.4

12.4 Surgery
Until 1968 we performed Barkan’s goniotomy [18] with which we were successful in
65% of the cases. Since then, we have been performing trabeculectomy according to
Harms [19, 20, 21] or Paufique’s technique [22], which have been successful in regulating
intraocular pressure in 98% of the cases.
There are two types of pathological chamber angles in children: type 1 and type 2.
The angle belonging to type 1 has thin pathological mesodermal remnants, while type 2
angles have thick mesodermal remnants (apparent high insertion of the iris). It is impos-
sible to distinguish the ciliary body band in both types of chamber angle.
Faced with type 1 chamber angles in eyes with an axial length not larger than 23 mm
within the first month of age, we decide to perform trabeculotomy and in type 2 chamber
angles with an axial length above 23 mm, the decision is combined surgery (trabeculot-
omy plus trabeculectomy). During the last 25 years most of the centers specialized in
children have left goniotomy aside and turned to trabeculotomy because this technique is
simpler and its results is better. It is also easier to learn by residents.

12.5 Echometry in the follow-up

Buschmann and Bluth, in 1974 [23, 24], were the first who conducted a follow-up of
congenital glaucomas with echometric measurements.
Echometry is very useful in the follow-up of congenital glaucomas, specially if it is
necessary to indicate a new surgery (reoperation).
There are four different types of evolution after surgery (figure 12.5):
1) When the axial length does not increase and, with time, falls within the normal range
of evolution. It is a satisfactory result and the best evolution one can expect.
128

Fig. 12.5

2) When the axial length continues to increase as revealed by subsequent checkups and
it deviates even further from the normal range. This is the worst type of evolution.
Reoperation is always necessary.
3) The axial length continues to increase parallel to the normal range. In these cases
reoperation is almost always necessary. The decision depends on the other parame-
ters.
4) There is a fourth type of evolution which was described by Massim and Pellat [25].
The axial length remains for some time at pathological levels running parallel to the
normal range band, then increasing and, finally, back parallel to the normal band.
In these cases it is also necessary to perform a reoperation but the other parameters
should also be taken into consideration.
When the axial length continues to grow and the intraocular pressure rises to patho-
logical level, the indication for surgery is very simple. But when the intraocular pressure
is high but stays within the normal range, echometry is of great value to indicate a new
surgery. Carvalho and coworkers in 1979 [26] also studied the results of goniotomy and
trabeculotomy.

12.6 Material
We have studied three different populations which had pediatric glaucomas during
childhood [6, 7, 8, 9]. The first group (19 eyes), consisted of primary congenital glauco-
mas operated only once. The intraocular pressure was regulated and the axial length of
the eye stopped its enlargement after surgery. Surgery was successful.
The second group (12 eyes) consisted of children with primary glaucomas in which
the intraocular pressure was regulated and the axial length of the eye stopped its enlarge-
ment after 2 to 6 reoperations.
The third group (29 eyes) consisted of late congenital glaucomas, goniodysgenesis
and juvenile open angle glaucoma. As these diseases manifested later, generally between
 129

Fig. 12.6

4 and 6 years of age, the axial length of the eyes did not grow despite the ocular hyper-
tension because the sclera was no longer elastic. The intraocular pressure was regulated
with medical therapy or surgery (table 12.1).
Material Cases Follow- Male Female
up (yrs.)
Group 1: Primary congenital 19 12-28 13 6
glaucomas operated once
Group 2: Reoperated primary 12 7-22 10 2
congenital glaucomas
Group 3: Goniodysgenesis 29 11-23 15 14
Total: 60 7-24 38 22
Control group: Normal individuals [25] 110 50 60
(age: 5-25 yrs.)
Table 12.1
The follow-up of the three groups was performed with echometry and applanation
tonometry. Ten to 28 years after surgery, the results were assessed by means of the analy-
sis of the following parameters: axial length, myopia, visual acuity, visual field and optic
disc appearance (scanning laser tomography). The optic disc was studied with the Hei-
delberg Retina Tomograph HRT (software version 1.11).
The axial length is different in the three groups. After surgery, group 1 remained at
the values reached before surgery; in group 2 (reoperated cases), the axial length was
bigger, and in group 3 it fell within the normal range because the sclera does not enlarge
after 5 years of age.
The myopia is also different in the three groups. Group 2 was highly myopic, group
1 mildly myopic and the group 3 was almost emmetropic.
The best visual acuity was found in group 3, the worst in group 2; in group 1, it was
considerably good.
The only visual field damage that can be found in children is diffuse sensitivity de-
pression, i.e. pathological MD values (figure 12.6).
130

12.8 Study of the optic nerve in congenital glaucomas

12.8.1 Method
We studied the optic discs in the three groups with the Heidelberg Retina Tomo-
graph (HRT). Three images were acquired for each eye and the mean topography was
obtained. The standard deviation of the mean topography must always be lower than 30
µm. The HRT was developed by Heidelberg Engineering GmbH (Heidelberg, Germany)
and uses the principle of confocal laser scanning microscopy [30]. The basic clinical
work with this device was performed by Burk and coworkers at Heidelberg University,
Germany [27, 28, 29].
In addition to the study of the optic disc parameters, the profiles of the optic discs of
each group were studied to a scale.
12.8.2 Parameters
We will now explain the alterations found in each parameter for each group, com-
pared to the parameters of a group of 110 normals, ranging from 5 to 25 years of age
[31].
One way analysis of variance (ANOVA) was used for overall comparison among the
four groups and post hoc comparisons were done with Tukey's test. Data was considered
as statistically significant if p < 0.05. All tests were performed with the software Graph-
Pad InStat (version 2.05).
In tables 12.2 and 12.3, "p" represents the results obtained with the ANOVA, in-
cluding normals. Table 12.2 shows the differences between groups 1, 2 and 3, while table
12.3 shows the differences of each group with the control (normal group). In both tables,
*, ** and *** indicate p < 0.05; < 0.01 and < 0.001, respectively. NS indicates there was
no significance.
Figures 12.7a and 12.7b show the analysis of the parameters disc area and cup/disc
area ratio. Figures 12.8a-d show the analysis of the parameters related to the disc cup-
ping: mean cup depth, maximum cup depth, cup area and cup volume. Figures 12.9a and
12.9b analyze the parameters rim volume and rim area. Figures 12.10a and 12.10b show
the cup shape measure and contour line height variation.
In each parameter, the difference between the three groups (table 12.2) and the dif-
ference between each group and the normal group (table 12.3) were analyzed.
Disc area (figure 12.7a)
In the three groups, statistically significant differences were found between groups 1
and 2 (p < 0.05) and between groups 2 and 3 (p < 0.01). No significant difference was
found between groups 1 and 3. Compared to the normal group, significant difference was
found only for group 2 (p < 0.001).
Cup/disc area ratio (figure 12.7b)
In the three groups, statistically significant differences were found between groups 1
and 2 (p < 0.001) and between groups 1 and 3 (p < 0.01). No significant difference was
found between groups 2 and 3. Compared to the normal group, significant differences
were found for groups 2 and 3 (p < 0.0001).
 131

Table 12.2

Table 12.3

Mean cup depth (figure 12.8a)

The difference between the three groups was statistically significant only between
groups 1 and 2 and between groups 2 and 3 (p < 0.001). Compared to the normal group,
all three groups were significantly different (p < 0.001).
Maximum cup depth (figure 12.8b)
As with the previous parameter, the differences were statistically significant only
between groups 1 and 2 and between groups 2 and 3 (p < 0.001). All three groups were
significantly different from the normal group (p < 0.001).
Cup area (figure 12.8c)
In the three groups, statistically significant differences were found between groups 1
and 2 (p < 0.001), between groups 1 and 3 (p < 0.05), and between groups 2 and 3 (p <
0.01). No significant difference was found between groups 2 and 3. Compared to the
normal group, significant differences were found for groups 2 and 3 (p < 0.001).
132

Fig. 12.7: Disc area (a) and cup/disc area ratio (b). Bars are mean + SEM.
(a) *, **, ***: p < 0.05, p < 0.01 and p < 0.001 vs. group 2;
(b) **, ***: p < 0.01 and p < 0.001 vs. group 1; ###: p < 0.001 vs. normal group.

Fig. 12.8: Mean cup depth (a), maximum cup depth (b), cup area (c), and cup vol-
ume (d). Bars are mean + SEM.
(a), (b) and (d) ***: p < 0.001 vs. group 2; ###: p < 0.001 vs. normal group;
(c) **, ***: p < 0.01 and p < 0.001 vs. group 2; ###: p < 0.001 vs. normal group.

The cup areas of the eyes of group 2 were larger than the areas of group 1 and group
3 eyes. The cup areas of groups 1, 2 and 3 were larger than those of normal eyes. The
largest area was found in group 2 (reoperated cases).
 133

Fig. 12.9: Rim volume (a) and rim area (b). Bars are mean + SEM.
(a) *, **, ***: p < 0.05, p < 0.01 and p < 0.001 vs. group 1;
(b) *, **: p < 0.05 and p < 0.01 vs. group 3.

Cup volume (figure 12.8d)

In the three groups, statistically significant differences were found only between
groups 1 and 2 and between groups 2 and 3 (p < 0.001). Compared to the normal group,
significant differences were found only for groups 2 and 3 (p < 0.001).
In group 2, the cup volume was larger than in group 1, group 3, and in normal eyes.
Rim volume (figure 12. 9a)
In the three groups, statistically significant differences were found only between
groups 1 and 2 (p < 0.05) and between groups 1 and 3 (p < 0.001). Compared to the nor-
mal group, a significant difference was found only for group 1 (p < 0.01).
The rim volume was larger in group 1 (glaucomas operated only once) than in group
2 (reoperated congenital glaucomas)
Rim area (figure 12.9b)
In the three groups, a statistically significant difference was found only between
groups 1 and 3 (p < 0.01). Compared to the normal group, a significant difference was
found only for group 3 (p < 0.05).
The smallest rim areas were found in eyes belonging to group 3.
Cup shape measure (figure 12.10a)
In the three groups, statistically significant differences were found only between
groups 1 and 2 and between groups 2 and 3 (p < 0.05). Compared to the normal group, a
significant difference was found only for group 2 (p < 0.01).
Height variation contour (figure 12.10b)
The differences between the three groups were statistically significant. Compared to
the normal group, a significant difference existed only for group 2 (p < 0.05).
134

Fig. 12.10: Cup shape measure (a) and height variation contour (b). Bars are mean +
SEM (SEM of normal group N in (a) is 0.007);
(a) *, **: p < 0.05 and p < 0.01 vs. group 2;
(b) *: p < 0.05 vs. normal group.

This indicates that even though in congenital glaucomas there is a reduction in the
number of nerve fibers, there is no localized depression in any quadrant or octant, as it
occurs in adults. Therefore, in children there are no disc notchings or scotomatous de-
fects.
12.8.3 Profiles
In addition to these parameters, we computed the mean profile of the optic nerve
head in each group with Turbocad software (version 2.0). In order to compare the profiles
to the mean profile of a normal disc, we studied a control group of 110 normal individu-
als between 5 and 25 years of age.
Figure 12.11 shows the mean profile of group 1 as compared to a normal mean pro-
file. They are almost identical, because as surgery succeeded in regulating the intraocular
pressure quickly, the optic nerve did not suffer.
In comparing their surfaces, it can be observed that the increase in the cup area is
due to an increase in the total disc area and not to a reduction of the neuroretinal rim area.
Upon its distension, Elschnig’s Ring becomes larger and, therefore, the total disc area
becomes larger than in normal eyes.
Figure 12.12 shows the image of an optic disc belonging to group 1, as obtained
with the HRT.
Figure 12.13 shows the mean profile of group 2, which differs significantly from the
normal mean profile. It also shows a great reduction in the volume of the neuroretinal rim
and a great increase in the cup volume.
With regard to the surface, there is a great increase in the cup area and a reduction in
the neuroretinal rim area. The total disc area is greatly enlarged by the distension of El-
schnig’s Ring. It should be kept in mind that this cup increase occurs at the expense of a
 135

Fig. 12.11

Fig. 12.12

reduction of the tilted neuroretinal rim (in blue color), while the flat neuroretinal rim (in
green color) remains almost unchanged.
Figure 12.14 shows the image of an optic disc belonging to group 2, obtained with
the HRT.
Figure 12.15 shows the mean profile of group 3, which is almost the same as the
normal profile. Unlike in groups 1 and 2, the total disc area is the same as in the normal
group. This correlates with the fact that there is no distension of Elschnig’s Ring because
ocular hypertension affects the eye only after 5 years of age. This behavior is the same as
in glaucomas of adults, where the cup area enlargement is proportional to the neuroretinal
rim area reduction.
Figure 12.16 shows an optic disc belonging to group 3, obtained with the HRT.
136

Fig. 12.13

Fig. 12.14

The red point of each profile graph indicates the end of the cup and the beginning of
the hyaloid duct (according to histopathologic determinations, it starts when the cup sur-
face is less or equal to 62 µm2). In group 3, unlike in groups 1 and 2, the end of the cup
remains almost at the same depth as in the normal disc, since in this group the eye is no
longer elastic. In the other two groups, the end of the cup is considerably displaced poste-
riorly.
12.8.4 Visual field
The visual field examinations were performed in the three groups with the Octopus
2000, Octopus 1-2-3, and with high-pass resolution perimeters. The results were pub-
lished in the proceedings of the 1990 International Perimetry Society Meeting held in
Malmö, Sweden [32], and in the 1994 International Perimetry Society Meeting held in
Washington, D.C. [33].
 137

Fig. 12.15

Fig. 12.16

Optic disc deterioration is associated with a marked visual field damage, which
manifests many years later. These visual field damages are worse in group 2.
As already stated, children regulating their intraocular pressure before 5 years of age
with surgery did not have scotomatous defects, because they do not have localized fiber
defects. We found only diffuse defects, specially in group 2.
In one of papers we presented in Washington, we studied the correlation of MD
(mean defect) obtained with the Octopus perimeter, with global deviation obtained with
high-pass resolution perimetry; it was found to be significant (r = 0.80, p < 0.001). How-
ever, there was no significant correlation between MD and NC (neural capacity).
In our opinion, these psychophysical tests measure different aspects of the visual
field system.
138

At the 1990 International Perimetry Society Meeting in Malmö and still today, we
think we have been the first to study the visual field in children with differential light
sensitivity perimetry (Octopus 2000 and Octopus 1-2-3 perimeters) and with high-pass
resolution perimetry. Both methods are capable to obtain reliable results in children be-
tween 5 and 10 years of age.
In order to be sure of the accuracy of our results, we used a reliability factor always
lower than N=10, as an exclusion criterion. Both perimetric methods showed that there
are no scotomatous defects in the visual fields of children with congenital glaucoma. The
only visual field damage that can be found in children is diffuse sensitivity depression,
e.g., pathological MD values.

12.9 Visual field, optic nerve parameters and axial length. Correlation and com-
parative study
In all the graphs which will follow, the axial length enlargement in the growing child
is represented on the abscissa, on the left ordinate the axial length is expressed in mm,
and on the right ordinate the different HRT parameters have been included.
We will first analyze three parameters related to RNFL thickness: neuroretinal rim
area, neuroretinal rim volume and RNFL thickness, relative to the axial length.
Correlation between axial length and neuroretinal rim area (figure 12.17):
The graph shows that the NRR area starts to change late. It must be kept in mind that
the change in the NRR area does not occur in parallel to the increase in the optic disc
area.
Correlation between axial length and neuroretinal rim volume (figure 12.18):
Conversely to what occurs with the NRR area, the NRR volume becomes dramati-
cally reduced as the axial length grows. When during the first six months of age the axial
length reaches 25 mm, the NRR volume decreases to 0.32 mm3, i.e., to the lowest normal
limit.
Correlation between axial length and RNFL thickness (figure 12.19):
The RNFL thickness changes in the same way as the NRR volume does. Both pa-
rameters measure the RNFL at different locations.
In short, the NRR area starts to change very late since it does not change signifi-
cantly when the optic disc enlarges. Besides, the NRR volume and the RNFL thickness
are good parameters for the early detection of optic nerve damage.
We will now analyze three optic nerve head parameters: disc area, cup area and cup
volume, relative to the axial length and the mean defect (MD) of the visual field.
Correlation between axial length and disc area (figure 12.20):
In the beginning, the disc area remains at normal values, but when the axial length
reaches 25 mm it starts to increase. The disc area and the visual field are related to each
other.
Correlation between axial length and cup area (figure 12.21):
The figure shows that the cup area starts to increase when the axial length surpasses
24 mm, and then increases quickly at axial length 25 mm and higher.
 139

Fig. 12.17

Fig. 12.18

Fig. 12.19
140

Fig. 12.20

Fig. 12.21

Correlation between axial length and cup volume (figure 12.22):

The cup volume starts to change immediately when the axial length increases, as the
above mentioned parameters do.
Correlation between diffuse damage of the visual field (MD) and axial length (figure
12.23):
When the axial length grows beyond 25 mm, severe visual field loss usually devel-
ops.
In short, when the axial length starts to grow, the disc area, cup area and cup volume
also enlarge. The correlation between these parameters and the axial length is very high.
The disc area and cup volume are the best parameters for the early detection of optic
nerve and visual field damage in congenital glaucoma.
 141

Fig. 12.22

Fig. 12.23

12.10 Comparison between optic nerve damage in congenital glaucoma and in

adulthood open angle glaucoma
Optic nerve damage caused by ocular hypertension in congenital glaucoma is com-
pletely different from the damage occurring in adulthood open angle glaucoma. In adults,
the cup area and especially the cup volume are parameters changing late in the evolution
of glaucoma (see chapter 14). Figures 12.24 and 12.25 show the optic discs of a child and
of an adult, where this difference is clearly seen.
142

Fig. 12.24

Fig. 12.25

12.11 Estimation of the disc area on the basis of axial length values
We have developed a formula which enables us to estimate the disc area from the
axial length in normal eyes (figure 12.26). We assume that the disc diameter is propor-
tional to half the axial length and, therefore, can be obtained by multiplying the axial
length with a constant K (the tangens of angle ß). As shown in figure 12.26, the disc area
is then equal to the square of the axial length, multiplied by the constant Q = 0.0035.
If the axial length reaches 26.6 mm and the disc area is 2.52 mm2, the visual field is
severely damaged.
The values of echometry reveal the disc surface and thus, this technique enables us
to perform surgery before functional defects occur. It should be kept in mind that on chil-
dren from birth to five years of age, it is impossible to perform perimetry to determine
whether or not there are any defects.
 143

Fig. 12.26

12.12 Differential diagnosis between a congenital glaucomatous optic disc and a

megalopapilla
One of the most common diagnostic mistakes in daily practice is caused by megalo-
papillas. Direct ophthalmoscopy or biomicroscopy of a megalopapilla reveals marked
pallor and a great cupping. We have examined children between 4 and 14 years of age
who were ready to undergo surgery for congenital glaucoma. Upon thorough study, these
cases had normal axial length and corneal diameter and, if perimetry was possible, nor-
mal visual field and no signs of congenital glaucoma.
Figures 12.27 and 12.28 were obtained from an 11-year-old boy who was ready to
undergo glaucoma surgery. Figure 12.27 shows the large size of the optic disc, which
covers the whole surface of a 10 degree tomography (topographical image and summa-
tion image). Figure 12.28 reveals a normal contour line, a normal neuroretinal rim (in
blue and green) and, on the top right, normal stereometric parameter values. The other
studies performed on this boy also gave normal results.

Fig. 12.27 Fig. 12.28

144

Fig. 12.29 Fig. 12.30

Figures 12.29 and 12.30 were obtained from an 18-year-old female patient with be-
nign hypophysis adenoma and a mildly damaged visual field. She was ready for surgery
due to the appearance of her optic disc, but this was a megalopapilla case. Figure 12.29
shows a topographical and a summation image. Figure 12.30 shows a section at the level
of the optic disc evidencing a very marked cupping which logically leads to the thought
of an optic disc atrophy or a glaucomatous cupping. All the parameters of this optic nerve
were normal.

12.13 Conclusions
Leydhecker and Goldmann’s hypothesis stating that the optic nerve deteriorates
many years before visual field damage takes place is now being confirmed by clinical
experience. In 85% of cases it has been found that about 10 years elapse between optic
nerve deterioration and visual field damage [30, 34].
In children, the optic nerve starts deteriorating as soon as ocular hypertension oc-
curs, while the visual field starts to deteriorate only 10 years later. This is the reason why
congenital glaucoma must be subjected to surgery as soon as diagnosed, and if the axial
length continues to grow despite surgery, it is necessary to reoperate. There is still plenty
of time until visual field damage takes place.
A retrospective study allowed us to better understand the optic disc damage preced-
ing visual field loss. Conversely to what happens in adults, as in children the anteroposte-
rior axis of the eye enlarges, Elschnig´s Ring also enlarges with a resulting increase in the
optic disc area. (This is the only pathologic case in which Elschnig's Ring enlarges, ex-
cept for congenital malformations.) This structural change at the level of the optic disc
also affects the parameters measuring the optic nerve. The main parameters for the optic
discs of congenital glaucomas, unlike what happens in adults, are cup volume, cup area
and disc area. When the axial length reaches or surpasses 25 mm before six months of
age, these parameters become pathological and functional deterioration starts.
Congenital glaucoma is a glaucoma of good prognosis. It is critical to prevent the
optic nerve head from further deterioration, by stopping eye enlargement caused by ocu-
lar hypertension which must be reduced surgically.
 145

Fig. 12.31

The following is an example in which echometry saved the eye of a child with con-
genital glaucoma just in time because the optic nerve can deteriorate severely before vis-
ual field damages start.
An 8-month-old boy was operated for congenital glaucoma. The right eye regulated
its intraocular pressure, and its axial length remained the same. At 2 and 4 years of age,
the axial length of the left eye started to grow. Surgery was indicated at the age of 4
years, but it was delayed by his family until he was 6 years. Upon an examination per-
formed at 10 years of age, the eye which regulated its intraocular pressure and kept its
axial length stable had -3 diopters of myopia, visual acuity of 20/30 and normal visual
field and optic nerve, while the contralateral reoperated eye had -6 diopters of myopia,
visual acuity of 20/30, severely damaged optic nerve and normal visual field (figure
12.31).
146

Bibliography
1. Sampaolesi R, Reca R, Carro A: Presión ocular en el niño hasta los 5 años bajo
anestesia con Pentrane (Metoxifluorane). Arch Oftalmol B Aires 1967, XKII: 180-
185.
2. Carvalho CA, Calixto N: Semiologia do glaucoma congenito. XV Cong Bras
Oftalmol Porto Alegre 1969, pp. 105-174.
3. Radtke ND, Cohan BE: Intraocular pressure measurement in the newborn. Am J
Ophthalmol 1974;78:501.
4. Tucker SM, Enzenauer RW, Levin AV et al: Corneal diameter, axial length and
intraocular pressure in premature infants. Ophthalmology 1992;99:1296.
5. Sampaolesi R, Reca R, Armando E: Normaler intraocularer Druck bei Kindern bis
zu 5 Jahren mit und ohne Allgemeinnarkose: Seine Witchtigkeit für die Frühdiag-
nose des angeborenen Glaukoms. Glaucoma Symposium, Würzburg, Germany,
1974, pp. 278-289.
6. Sampaolesi R: Ocular echometry in the diagnosis of congenital glaucoma. Ophthal
Proc Series, 1981;29:177-189.
7. Sampaolesi R, Carusso R: Ocular echometry in the diagnosis of congenital glau-
coma. Arch Ophthalmol 1982;100:574-577.
8. Sampaolesi R: Ocular echometry in the diagnosis of congenital glaucoma and its
evolution. In: Krieglstein GK, Leydhecker W (eds.): Glaucoma Update II, pp. 175-
184. Springer Verlag, 1983.
9. Sampaolesi R: Echométrie oculaire, un noveau parametre dans le diagnostic et le
controle de l'évolution du glaucome congenital. Bull et Mem S.F.O. 1984;95:401-
409.
10. Sampaolesi R: Congenital glaucoma. Long-term results of surgery. In: Krieglstein
GK (ed.): Glaucoma Update III. Springer-Verlag, Berlin, Heidelberg, 1987, pp. 154-
161.
11. Sampaolesi R: Echometry in congenital glaucoma: long-term results after 10 to 17
years of surgery. Ultrasonography in Ophthalmol 12; pp. 181-191. Kluwer Aca-
demic Publishers, Dordrecht, 1990.
12. Reibaldi A: Biometric ultrasound in the diagnosis and follow-up of congenital glau-
coma. Annales of Ophthalmology 707 (1982).
13. Carvalho CA, Betinjane AJ: Valores da biometria ultrasonografica obtidos em
olhos normais de pacientes ate 18 meses de idade. Rev Bras Oftalmol 1980;39:479-
482.
14. Betinjane AF: Contribuçao ao estudo da biometria ultrasonografica no glaucoma
congenito. Tese Doc Livre Fac Med USP (1982).
 147

15. Tarkkanen A, Uusitalo R, Mianowicz J: Ultrasonographic biometry in congenital

glaucoma. Acta Ophthalmol 1983;61:618-623.
16. Fledelius H: Personal communication.
17. Spaeth G: Discussion to Sampaolesi’s ocular echometry and the diagnosis of con-
genital glaucoma and its evolution. In: Krieglstein GK, Leydhecker W (eds.): Glau-
coma Update II, p. 184. Springer-Verlag, Berlin, 1983.
18. Barkan O: New operation for chronic glaucoma: restoration of physiological func-
tion by opening Schlemm’s canal under direct magnified vision. Am J Ophthalmol
1936;19:951-966.
19. Harms H: Glaukom-Operationen am Schlemm’schen Kanal. Sitzungsber der 114.
Versammlung des Vereins RheinWestf. Augenärzte (1966).
20. Harms H: Trabeculotomy results and problems. Adv Ophthalmol 1970; vol 27: 95-
96 (Karger, Basel, New York).
21. Harms H, Dannheim R: Trabeculotomy. Results and problems. Microsurgery in
glaucoma, 2nd Int Symp Ophthalmol, Microsurgery Study Group, Bürgen-stock
1968. Adv Ophthalmol 1970, vol. 22: 121-131 (Karger, Basel, München, New
York).
22. Paufique L, Sourdille PH, Ortiz-Olmedo AH: Technique et résultats de la tra-
beculotomie ab externo dans le traitement du glaucoma congénital. Bull et Mem
SFO 1969, pp. 54-65 (Masson et Cie. Editeurs, Paris, 1970).
23. Buschmann W, Bluth K: Regelmäßige echographische Messung der Achsenlänge
des Auges zur Kontrolle der Druckregulierung bei Hydrophthalmie. Klin Mbl
Augenheilk 1974;165:878-886.
24. Buschmann W, Bluth K: Eine echographische Methode zur Verlaufskontrolle
angeborener Glaukome. Graefes Arch Ophthalmol 1974;192:313-329.
25. Massin M, Pellat B: Ultrasonic biometry in congenital glaucoma, a clinical study.
Personal communication. Hópital National des Quinz-Vingts, 28 Rue de Charenton
75571, Paris Cedex 12, France, 1982.
26. Carvalho CA, Betinjane AJ, Camargo ML: Results of goniotomy and trabeculot-
omy as the initial surgical procedure in the treatment of congenital glaucoma. In:
Krieglstein GK, Leydhecker W (eds): Glaucoma Update, pp. 33-38 (Springer-
Verlag, Berlin), 1979.
27. Burk ROW, Rohsrschneider K, Kruse FE, Völcker HE: Laser Scanning Tomo-
graphie der Papille, In: Gramer E (ed) Glaukom. Diagnostik und Therapie. Interna-
tionales Glaukomsymposium, Würzburg 1988. Enke, Stuttgart, 1990, pp. 113-119.
28. Burk ROW, König J, Rohrschneider K, Noack H, Völcker HE , Zinser G:
Analysis of three-dimensional optic disk topography by laser scanning tomography.
Parameter definition and evaluation of parameter inter-dependence. In: Nasemann J,
148

Burk ROW (eds.): Scanning laser ophthalmoscopy and tomography. Quintessenz,

München, 1990, pp. 161-176.
29. Burk ROW, Rohrschneider K, Noack H, Völcker HE: Volumetrische Papillen-
analyse mit Hilfe der Laser-Scanning-Tomographie. Parameterdefinition und Ver-
gleich von Glaukom-und Kontrollpapillen. Klin Mbl Augenheilk 1991;198:522-529.
30. Zinser G, Wijnaendts-van-Resandt RW, Ihrig C: Confocal laser scanning mi-
croscopy for ophthalmology. Proc SPIE 1988;1028:127-132.
31. Sampaolesi R, Sampaolesi JR: Tomografía confocal de la retina y del nervio óp-
tico. Relato Anual 1995. Arch Oftalmol B Aires 1997; LXX: 77-86.
32. Sampaolesi R, Casiraghi JF: Computerized visual fields in pediatric glaucoma.
Perimetry Update 1990/91: pp. 455-464. Kugler Publications, Amsterdam, 1991.
33. Mills RP, Wall M (eds): Perimetry Update 1994/95, pp. 185-188. Kugler Publica-
tions, Amsterdam 1995.
34. Boeglin RJ, Caprioli J: Contemporary clinical evaluation of the optic nerve in
glaucoma. In: Caprioli J and Stamper RL (eds): Ophthalmology Clinics of North
America. Contemporary Issues in Glaucoma 1991; vol. 4, pp. 711-731 (W.B. Saun-
ders Company, Philadelphia, 1991.
149