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Graefe’s Arch Clin Exp Ophthalmol (2000)

238:207–213 © Springer-Verlag 2000 C L I N I C A L I N V E S T I G AT I O N

Gesine Meyer Trabeculotomy in congenital glaucoma


Oliver Schwenn
Norbert Pfeiffer
Franz Grehn

Received: 17 November 1998 Abstract Background: Congenital 25/27 eyes (92.6%) after 1 year, in
Revised version received: 8 July 1999 glaucoma is a potentially blinding 15/18 eyes (83.3%) after 2 years, in
Accepted: 8 July 1999 disease that requires surgical therapy. 8/12 (66.7%) eyes after 3 years, in
This paper describes the outcome of 4/8 eyes (50%) after 4 years and in
trabeculotomy in primary congenital 4/4 (100%) eyes after 5 years of fol-
glaucoma. Methods: Thirty-nine low-up. Complications included hy-
eyes of 22 children with congenital potony (three eyes), subchoroidal
glaucoma who underwent trabeculot- bleeding (one eye ), detachment of
omy with or without a simultaneous Descemet’s membrane (one eye) and
trabeculectomy between 1992 and macular pucker (one eye in which la-
1997 were retrospectively analyzed. ter mitomycin C was used). Visual
Results: Mean follow-up was acuity (VA) was tested with various
24.7±17.9 months. A mean of 1.3 op- methods in 35 eyes. VA was within
erations per eye were performed. The the normal nomogram range in
mean IOP at the end of follow-up 12 eyes and below the normal range
(n=39) was 17.7±6.0 mmHg; in in 23 eyes at the end of follow-up.
8 eyes (20.5%) the IOP was >21 Axial length measurements showed
G. Meyer (✉) · F. Grehn mmHg, in 31 eyes (79.5%) it was normalization according to the age
Department of Ophthalmology, ≤21 mmHg. The mean difference be- nomogram in 22 of 35 eyes.
University of Würzburg, tween pretreatment IOP and IOP at Conclusion: This study shows that
Josef-Schneider-Strasse 11,
D-97080 Würzburg, Germany the end of follow-up (n=39) was trabeculotomy is an effective surgical
Fax:+49-931-201-2245 –10.5±9.4 mmHg (–37.2%). Success procedure in congenital glaucoma
D. Schwenn · N. Pfeiffer rates were calculated: IOP was with satisfactory success rates up to
Department of Ophthalmology, ≤21 mmHg in 36/39 eyes (92.3%) 5 years of follow-up.
University of Mainz, Mainz, Germany after 1/2 year of follow-up, in

Introduction dure for adult and developmental glaucoma. Later, in


1966, Harms [21] described a modified technique of
Congenital glaucoma is caused by developmental abnor- trabeculotomy which has became popular in Europe in
malities of the trabecular meshwork which impair the the past three decades. Modifications have been de-
outflow of aqueous humor. In the management of con- scribed by several authors [20, 29, 32, 33]. The main ad-
genital glaucoma, medical treatment is not successful in vantage of trabeculotomy over goniotomy is that trabec-
the long term. Therefore, surgical therapy is required. ulotomy can be performed even if the cornea is hazy and
Surgery improves outflow facility by opening the abnor- the chamber angle cannot be seen. In addition, this tech-
mal trabecular meshwork. Two procedures are generally nique allows better control of the surgical procedure if
used: trabeculotomy and goniotomy. Trabeculotomy was the eye has undergone previous surgery or if iris strands
first described in 1960 independently by Allen and Buri- are present.
an [4] and Burian [9] and by Smith [42, 43] as a proce-
208

Fig. 1 Surgical technique of trabeculotomy

Fig. 2 Intraocular pressure (IOP) at the end of follow-up (n=39).


Patients and methods The abszissa shows the preoperative IOP, the ordinate shows the
postoperative IOP of individual eyes. Triangles show results after
We retrospectively analyzed a consecutive series of 39 eyes of 1 operation, circles show results after 2 operations. One eye re-
22 children with primary congenital glaucoma or Axenfeld-Rieger ceived topical 5-FU during surgery
syndrome who underwent trabeculotomy with or without a simulta-
neous trabeculectomy between 1992 and 1997. These patients were
under the care of the University Eye Hospital Würzburg and/or the was placed underneath the conjunctiva for 5 min before dissecting
University Eye Hospital Mainz, Germany. Thirteen patients were of the scleral flap, and the site was then rinsed with 30 ml bal-
male, nine female. Twenty patients had bilateral congenital glauco- anced salt solution (BSS).
ma and two patients had unilateral congenital glaucoma. Three of Preoperative and follow-up examinations included the following
these patients (six eyes) had Axenfeld-Rieger syndrome. parameters: IOP was measured by hand-held Perkins applanation
A total of 51 operations were performed (mean 1.3 opera- tonometry under ketamine or inhalation anesthesia, or without gen-
tions/eye). Twenty-seven of 39 eyes underwent one operation and eral anesthesia if possible. Axial length was measured either with
12 eyes underwent two operations. Thirty-seven of these opera- the Ocuscan 400 (Sonometrics Systems) or the Grieshaber Biomet-
tions were trabeculotomies, and 14 were trabeculotomies com- ric System (Switzerland). Follow-up measurements were done with
bined with trabeculectomy. Topical 5-fluorouracil was used in one the same system in each case. Visual acuity was tested with Teller
of the combined operations. acuity cards, E-test or geometrical signs (Lea Hyvärinen test). Or-
The surgical technique of trabeculotomy was as follows thoptic examinations were done according to the routine schedule.
(Fig. 1): A limbus-based conjunctival flap was used in either a
12 o’ clock position or in one of the superior quadrants. A scleral
flap of 3×4 mm and half scleral thickness was dissected and a ra- Results
dial incision was cut into the floor of the sclera until the outer wall
of Schlemm’s canal was opened. The outer wall was then cut with
Vannas scissors parallel to the limbus in both directions to facili- The mean age at the first operation was 15.7 months
tate the introduction of the trabeculotomy probe. A trabeculotomy (range 0.2–75.5 months). The mean interval between two
probe of the Mackensen type was used (Geuder Instruments, Hei- operations in the same eye was 12.5 months (range
delberg, Germany). After the trabeculotomy probe was introduced 0.7–48.2 months). Mean follow-up was 24.7±17.9 months
into Schlemm’s canal, the tip of the probe was rotated into the an-
terior chamber with moderate force. This was performed in both (range 3.5–61.5 months).
directions. The scleral flap was then closed with two to five 10/0
nylon stitches.
In the 14 operations in which trabeculotomy was supplemented Intraocular pressure
by trabeculectomy, trabeculotomy was completed as described
above. The inner wall of Schlemm’s canal was then perforated
into the anterior chamber at the site of the operation. The four out- Intraocular pressure was measured by hand-held applana-
er scleral corners of the cross-like incisions were excised using tion tonometry (Perkins Tonometer). The mean pretreat-
Vannas scissors. A small iridectomy was done to avoid iris pro- ment IOP (n=39) was 28.2±6.7 mmHg. Thirty-four eyes
lapse. The scleral flap was closed with two to five 10/0 nylon
stitches in a way that allowed moderate filtration. (87.2%) had an IOP >21 mmHg, five (12.8%) an IOP ≤21
In one case we used topical 5-fluorouracil (40 mg/ml): a small mmHg. Seventeen of these eyes were examined under ket-
sponge (Merocel) of 8×8 mm size soaked with the antimetabolite amine anesthesia, 20 eyes under inhalation anesthesia and
209

Table 1 Number of eyes and rates of IOP regulation according to


two different success criteria (IOP ≤21 mmHg; IOP ≤21 mmHg
and IOP drop ≥20%) after one or two operations

n IOP ≤21 mmHg IOP ≤21 mmHg and IOP-drop


(eyes) ≥20% (eyes)

1/2 year 39 36 (92.3%) 35 (89.7%)


1 year 27 25 (92.6%) 24 (88.9%)
2 years 18 15 (83.3%) 14 (77.8%)
3 years 12 8 (66.7%) 7 (58.3%)
4 years 8 4 (50.0%) 4 (50.0%)
5 years 4 4 (100%) 3 (75.0%)
aThree out of four eyes that were seen both at 4 years and 5 years
did not fulfil the success criteria at 4 years, but were subsequently
operated and fulfilled the success criteria at 5 years

after 2 years, 58.3% after 3 years, 50% after 4 years and


75% after 5 years. Three of four eyes that were seen both
at 4 years and 5 years did not fulfil the success criteria at
4 years, but were subsequently operated on and fulfilled
the success criteria at 5 years.

Corneal edema and corneal diameter

Twenty-seven of 39 eyes showed preoperative corneal


Fig. 3 Mean IOP± standard deviation before treatment and at the edema and 12 of 39 eyes had a clear cornea. The mean
end of follow-up (n=39)
pretreatment horizontal corneal diameter (n=35) was
13.1±1.1 mm (range 10.8–14.6 mm). The mean horizon-
2 eyes without general anesthesia. The mean IOP of all tal corneal diameter at the end of follow-up (n=29) was
eyes (n=39, follow-up 3.5–61.5 months) at the end of fol- 13.8±0.9 mm (range 11.5–15.2).
low-up was 17.7±6.0 mmHg. In eight eyes (20.5%) the
IOP was >21 mmHg, in 31 (79.5%) eyes the IOP was
≤21 mmHg (Fig. 2). However, in an additional three eyes Axial length
IOP reduction was <20% of preoperative IOP. When a
combined criterion of IOP ≤21 mmHg and an IOP drop of The mean pretreatment axial length (n=35) was 22.3±
≥20% was used, the success rate at the end of follow-up 2.6 mm (range 18.3–27.6 mm). Compared to the 95%
was 71.8% (28 of 39 eyes). Two of the six eyes with Ax- percentile of the nomogram the axial length was shorter
enfeld-Rieger syndrome did not meet the IOP criteria of in one eye (2.9%), longer in 21 eyes (60.0%) and within
≤21 mmHg plus IOP drop of ≥20%. the 95% percentiles in 13 eyes (37.1%). The mean axial
In 26 of these eyes postoperative examination was length at the end of follow-up was 23.9±2.3 mm (n=35).
performed under anesthesia with ketamine, in 7 eyes un- Axial length growth was proportional to the nomogram
der inhalation anesthesia and in 6 eyes without general in 8 eyes, accelerated in 13 eyes and reduced in 14 eyes
anesthesia. The mean difference between pretreatment (Fig. 4). The absolute axial length at the end of follow-
IOP and IOP at the end of follow-up (n=39) was up was within the 95% percentiles of the nomogram in
–10.5±9.4 mmHg (–37.2%, Fig. 3). In 6 of 39 eyes a lo- 11 eyes, beyond the 95% percentile in 22 eyes, and be-
cal antiglaucomatous therapy was applied preoperatively. low the 95% percentile in 2 eyes.
At the end of follow-up seven eyes were under local an- When normalization of axial length growth was taken
tiglaucomatous therapy. as an additional criterion for success, the success rate at
Table 1 shows the success rates. When IOP ≤21 mmHg the end of follow-up dropped to 54.5% (18 of 33 eyes).
was taken as a success criterion, the success rates were
92.3% after 1/2 year, 92.6% after 1 year, 83.3% after
2 years, 66.7% after 3 years, 50% after 4 years and Optic disc
100% after 5 years. When success was defined by
IOP ≤21 mmHg plus an IOP drop of ≥20%, the success At the end of follow-up, the optic disc of 22 eyes showed
rates were 89.7% after 1/2 year, 88.9% after 1 year, 77.8% a glaucomatous excavation; 17 eyes had normal discs.
210

Table 3 Complications after surgery. Most of the severe compli-


cations relate to the four children of one Turkish family with un-
usually severe congenital glaucoma (hypotony with choroidal de-
tachment, Descemet detachment, epiretinal gliosis after MMC
trabeculectomy)

Complications Number
of eyes

• Paracentral subchoroidal bleeding 1


• Ηypotony
– with transient shallowing of anterior chamber 2
– with choroidal detachment 1
• Descemet detachment 1
• Εpiretinal gliosis after additional trabeculectomy 1
+MMC

Complications

Complications occurred in six eyes. Paracentral subchoroi-


dal bleeding was seen in one eye of a black child after
combined trabeculotomy and trabeculectomy. It resolved
within 2 months. The reason for this bleeding was most
Fig. 4 Axial length growth at the end of follow-up (n=35). Pro- likely a postoperative hypotony. Transient hypotony with
portional means that axial length growth paralleled the nomogram shallowing of the anterior chamber was seen after two op-
according to age. Slowed-down means that axial length growth ap- erations. Unusual complications occurred in three of four
proached the nomogram. Accelerated means that axial length Turkish sisters with congenital glaucoma, in whom trabec-
growth exceeded the gradient of the nomogram
ulotomy was performed: Hypotony with choroidal detach-
ment occurred in the right eye of the eldest sister. In this
Table 2 Distribution of refrac- case a puncture of the suprachoroidal fluid was performed.
tion of 21 eyes at the end of Refraction Number of eyes Later on this eye needed a trabeculectomy with mitomycin
follow-up C. In one eye of the second sister a detachment of Descem-
>–6 dpt. 4
–2 – –6 dpt. 6 et’s membrane occurred after trabeculotomy despite cor-
–2 – +2 dpt. 7 rect probing of Schlemm’s canal. Revision, subsequent
+2 – +6 dpt. 3 lensectomy for mature cataract with phacolysis and cyclo-
>+6 dpt. 1 photocoagulation were required. The third sister had per-
sistent high IOP after two trabeculotomies in her right eye.
She therefore needed an additional trabeculectomy with
Visual acuity and orthoptic status mitomycin C. About 3 months after this operation she de-
veloped epiretinal gliosis and a pars plana vitrectomy with
Visual acuity could be tested monocularly in 25 eyes and membrane peeling became necessary (see Table 3).
binocularly in 10 eyes. 19 eyes were tested with Teller
acuity cards, 13 eyes with E-test and 3 eyes with geomet-
rical signs (Lea Hyvärinen test). At the end of follow-up Discussion
visual acuity according to the nomogram (n=35) was nor-
mal in 12 eyes and below normal range in 23 eyes. Re- In congenital glaucoma, the IOP is elevated due to devel-
fraction by retinoscopy could be tested in 21 eyes. Twelve opmental abnormalities of the trabecular meshwork re-
of these eyes were myopic, 7 eyes were hyperopic and 2 sulting in impairment of aqueous outflow. Surgical treat-
eyes were emmetropic (see Table 2). Astigmatism oc- ment has therefore concentrated on procedures that open
curred in 12 of 21 eyes (≥1 diopter in 9 eyes). Six patients the trabecular meshwork ab externo or ab interno, i.e.
had anisometropia of more than 2 diopters. trabeculotomy or goniotomy, respectively.
Orthoptic data were available in 21 patients at the end In the university eye hospitals of Würzburg and
of follow-up. Strabismus (exotropia or esotropia) oc- Mainz, trabeculotomies in congenital glaucoma (primary
curred in 8 patients. Six patients had nystagmus, 5 of and Axenfeld-Rieger syndrome) were performed in a
them in combination with strabismus. consecutive series of 39 eyes between 1992 and 1997.
Patching was performed in 9 of 21 patients: in 5 pa- The present study describes efficacy in terms of IOP de-
tients for strabismus, in 3 patients for anisometropia and crease, normalization of corneal diameter and axial
in 1 patient for both. length, visual outcome and rate of complications.
211

In our series of 39 eyes the mean IOP at the end of trabeculotomies (mean follow-up 5.6 years). Trabeculot-
follow-up was 17.7±6.0 mmHg. As IOP measurements omy was also successful in four of eight eyes when used
in small children usually have to be done under general as a secondary procedure after a different type of opera-
anesthesia (as in most cases of our series), IOP may be tion had been unsuccessful.
altered by the anesthetics and may be measured too high Luntz [28] obtained a success rate of 90% (26 of 29
(ketamine) or too low (inhalation anesthesia). When IOP operations) after one trabeculotomy (mean follow-up
≤21 mmHg was taken as success criterion, the success 2 years). This study included no patients with Axenfeld-
rates were 92.3% after 1/2 year, 92.6% after 1 year, Rieger syndrome. The study of Quigley [38] described 28
83.3% after 2 years, 66.7% after 3 years, 50% after operations resulting in a success rate of 80% (17 of 22
4 years and 100% after 5 years. When calculating the eyes) with a single trabeculotomy after 1-year follow-up.
success rate at the end of follow-up of all eyes, 79.5% of At a glance the results of most of these authors [22,
eyes fulfilled the above success criterion. When IOP 28, 33] seem to be slightly better than our results. How-
≤21 mmHg plus an IOP drop of ≥20% was used as suc- ever, it has to be considered that in our study cases with
cess criterion, the success rate at the end of follow-up Axenfeld-Rieger syndrome were included. Furthermore,
was 71.8% (28 eyes). We also followed axial length, cor- there was also a Turkish family with four sisters who all
neal diameter and refraction under general anesthesia. had remarkably severe atypical congenital glaucoma,
With IOP ≤21 mmHg, IOP drop of ≥20% and normal- probably of pseudodominant inheritance. The study of
ized axial length growth as combined criterion, the suc- Quigley [38],who also included cases with Axenfeld-
cess rate at the end of follow-up was only 54.5% (18 of Rieger syndrome and other secondary glaucomas, seem
33 eyes). to better correspond to the conditions of our study. All
The latter criterion, however, overestimates the failure eyes with primary glaucoma were successfully treated in
rate, because some eyes tend to develop myopia despite his study.
normalized IOP. The ≥20% IOP drop criterion may also In severe or refractory cases of congenital glaucoma,
lead to overestimation of failures, because many preop- trabeculotomy may be supplemented by trabeculectomy
erative IOP measurements were made under inhalation with or without the use of antimetabolites [2, 7, 15, 30,
anaesthesia, which leads to a considerable artificial de- 31, 41].
crease in IOP, whereas postoperative control measure- We used additional trabeculectomy in cases of Axen-
ments were mostly done in ketamine anaesthesia, which feld-Rieger syndrome, in eyes that had excessive en-
provides normal or raised IOP readings [36]. In contrast largement of the globe and in patients who underwent a
to adult primary open-angle glaucoma the ≤21 mmHg second operation. Racial factors were also an indication
success rate criterion seems appropriate for congenital for combined surgery (in one of the Turkish sisters).
glaucoma, where many of the eyes have not yet devel- 5-fluorouracil was used in one eye during reoperation.
oped optic nerve damage. A surgical problem may occur when the probe is in-
Visual acuity measurements are difficult in congenital troduced in the subciliary space and the ciliary muscle
glaucoma, with a mean presenting age of 15.7 months is erroneously disinserted from the scleral spur. A cyclo-
and a mean follow-up of 24.7 months. Therefore, the fi- dialysis or disinsertion of the iris base instead of trab-
nal rate of amblyopia in these cases cannot be deter- eculotomy then results. This was reported in two of
mined at the present follow-up. McPherson’s cases [33] but occurred in none of our
Trabeculotomy is considered an adequate procedure cases. Moderate bleeding into the anterior chamber is a
in congenital glaucoma by many other surgeons [22, 28, regular event in correct trabeculotomy due to reflux of
29]. A number of articles on trabeculotomy have been blood from Schlemm’s canal, which is a continuation of
published during the past decades in primary and sec- episcleral veins. If the IOP drops to below the episcleral
ondary congenital glaucoma [1, 5, 11, 12, 14, 22, 26, 28, venous pressure during the procedure, blood flows back
34, 38–41] and in adults [8, 10, 16, 23–25, 44–46]. into Schlemm’s canal and enters the anterior chamber
Harms and Dannheim [22] found a postoperative IOP through the trabeculotomy site due to the lack of venous
≤22 mmHg more than 3 months after uncomplicated pri- valves.
mary trabeculotomy in all of 18 eyes with primary con- Detachment of Descemet’s membrane is also one of
genital glaucoma and in 4 of 7 eyes which either had the possible complications of trabeculotomy and oc-
secondary congenital glaucoma or had already under- curred in one of our cases. Quigley [38] reported two
gone other types of antiglaucomatous operations. cases of Descemet detachment. Prolapse of the iris oc-
Authors of other studies analyzed eyes with Axen- curred in nine cases of the series of Luntz [28]. Howev-
feld-Rieger syndrome separately from eyes with primary er, no prolapse of the iris was seen in our series. Postop-
congenital glaucomas. In the study of McPherson and erative subchoroidal bleeding (one operation) and hypot-
Berry [33], 19 of 23 eyes (83%) were controlled (IOP ony with transient shallowing of the anterior chamber
<21 mmHg) with one primary trabeculotomy and 22 of (two operations) or with choroidal detachment (one op-
23 eyes (96%) were controlled with up to three eration) occurred in our series (see Table 3).
212

Comparison between trabeculotomy and goniotomy cornea or lens is low and the operation can even be
has shown equal efficacy of both procedures in congeni- done in cases where the cornea is cloudy [3]. The tra-
tal glaucoma [5, 33]. becular meshwork can be cleaved reliably if the trabec-
A comparison of the two techniques in similar groups ulotomy probe is correctly introduced into Schlemm’s
of patients with congenital glaucoma was made by canal. In contrast, goniotomy may incise various struc-
McPherson and Berry [33]. Both types of operation re- tures of the chamber angle, as has been shown by his-
sulted in a similar lowering of IOP, although a higher tology [27]. In goniotomy it may be helpful to remove
number of operations per eye were performed in goniot- the corneal epithelium in cases of corneal cloudiness
omy eyes (2.6 operations) than in trabeculotomy eyes [13].
(1.6 operations). In the study of Anderson [5] the first On the other hand, some authors emphasize that
eye was randomly assigned to either goniotomy or goniotomy, due to its transcorneal access does not dam-
trabeculotomy and the other eye to the other procedure. age the conjunctiva and sclera [19]. This is of impor-
If the IOP was controlled in the first eye, it was also con- tance if filtration surgery later becomes necessary.
trolled in the fellow eye by the other operation. If, how- Miller and Rice [37] showed that previous surgery in-
ever, either goniotomy or trabeculotomy was unsuccess- volving the conjunctiva significantly increased the risk
ful in the first eye, the other eye also failed after the oth- of failure of trabeculectomy in congenital glaucoma.
er operation. This is a strong argument for the proposi- On the other hand, Akimoto et al. [3] emphazise that
tion that the individual situation of the glaucoma influ- the area of conjunctiva involved in trabeculotomy is so
ences the outcome more than does the type of operation. small that trabeculectomy can easily be performed sub-
On the other hand, comparisons also depend largely on sequently.
the individual experience of the surgeon with one or the In a modified technique trabeculotomy can be done
other procedure. over 360°. In this technique a thread is placed into
In our own retrospective study of goniotomy with a Schlemm’s canal. By pulling the suture into the anterior
mean follow-up of 51.6 months, the success rate at the chamber Schlemm’s canal is opened over 360°. The
end of follow-up of all eyes was 70.6% according to the study of Beck and Lynch [6] shows success in 87%
IOP criteria used in the present study and 59.4% if nor- (13 of 15) of the eyes that underwent surgery by this
malization of axial length growth was also a criterion technique, whereas the report of four cases by Gloor
[35]. showed a high rate of complications [17].
A clinical study of patients who underwent gonioto- Our study shows that trabeculotomy is an effective
my for primary congenital glaucoma at the University surgical technique for the treatment of congenital glau-
Eye Hospital Würzburg in the period from 1965 to 1983 coma. However, it must be emphasized that adequate
[18] focussed particularly on the visual outcome after follow-up examinations have to be done, especially in
goniotomy. After a mean follow-up of 11 years (3–28 cases of unilateral congenital glaucoma, where the risk
years), visual acuity was ≥0.4 in over 50% of 60 eyes of amblyopia and anisometropia is a major problem.
and the visual field was normal in 74.3% of 35 eyes. Regular orthoptic examinations are therefore mandatory,
The main advantage of trabeculotomy is the external and patching should be started if necessary.
microsurgical approach. Thus the risk of injuring the

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URL:http://dx.doi.org/10.1007/s00417
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