DOI: 10.5301/ejo.5000083
Eur J Ophthalmol 2012 ; 22 ( 4): 620-625
ORIGINAL ARTICLE
Optic disc pit maculopathy: the value
of small-gauge vitrectomy, peeling, laser treatment,
and gas tamponade
Stanislao Rizzo, Claudia Belting, Federica Genovesi-Ebert, Emanuele Di Bartolo, Federica Cresti,
Laura Cinelli, Luca Allegrini
Azienda Ospedaliera Universitaria Pisana–Cisanello, Eye Surgery Clinic, Pisa - Italy
PURPOSE. To report the outcome of 10 patients with optic pit maculopathy (OPM) and evaluate the role
of small-gauge vitrectomy, gas endotamponade, and additional laser photocoagulation treatment.
METHODS. We retrospectively investigated 10 patients who underwent small-gauge, sutureless vitrectomy for OPM, detachment of the posterior hyaloid, internal limiting membrane (ILM) peeling, endolaser photocoagulation on the temporal margin of the optic disc, and gas tamponade. Preoperative and
postoperative best-corrected visual acuity (BCVA) was recorded and optical coherence tomography
(OCT) imaging was performed.
RESULTS. Seven out of 10 patients gained at least 2 lines of vision; 2 patients gained 1 line of vision.
Visual improvement occurred more than 3 months after surgery. One myopic patient developed a
macular hole postoperatively, resulting in a poor functional result even though complete retinal attachment was achieved. The functional outcome did not always correlate well with the OCT imaging, in
which complete retinal reattachment was observed in 5 out of 10 eyes.
CONCLUSIONS. The therapeutic approach should include both small-gauge vitrectomy and ILM peeling
to relieve vitreoretinal traction, as well as laser photocoagulation of the temporal margin of the optic
disc in order to prevent vitreous fluid from entering the subretinal/intraretinal space. In addition, the
patients should be told that visual recovery can take a long time.
KEY WORDS. Gas endotamponade, Laser photocoagulation, Optic disc pit maculopathy, Small-gauge,
Sutureless vitrectomy
Accepted: October 23, 2011
INTRODUCTION
Optic pit anomaly is a rare congenital condition that appears with a prevalence of 1:11,000 individuals, caused by
a closure anomaly of the embryonic fissure (1). Although
optic pits are typically unilateral, bilateral pits are seen in
15% of cases (2). Serous macular elevations have been
estimated to develop in 25% to 75% of eyes with optic
pits, leading to a serous detachment of the neuroretina
in the papillomacular bundle (2, 3). More detailed optical
coherence tomography (OCT) imaging has shown that the
maculopathy consists of a splitting of the inner retinal lay620
ers into a schisis-like structure, in addition to an accumulation of subretinal fluid (4).
Although the pathogenesis is still not fully understood, what
is known is that this fluid passes from the optic pit into the
subretinal space, and studies in recent years have demonstrated that it originates from the vitreous (5, 6), rather
than from cerebrospinal fluid (3). With the advent of the use
of high-resolution OCT images vitreomacular traction has
also been visible in some cases (7). In fact, in some eyes
OPM is associated with a macular hole (8).
However, doubt exists regarding the treatment. Spontaneous reattachment (9) and recurrences are part of the natu-
© 2011 Wichtig Editore - ISSN 1120-6721
Rizzo et al
ral course of this disease, seen in approximately 25% of
cases. But long-standing OPM leads to significant reduction, and sometimes permanent loss of vision (10). Laser
photocoagulation at the margin of the optic nerve head has
been successful in some cases (5). Posterior buckling has
been applied to relieve vitreoretinal traction (7). In addition,
vitrectomy with and without peeling of the internal limiting
membrane (ILM) has been described (11, 12). Nonetheless, there is doubt about the value of vitrectomy and laser
treatment and studies on a large number of cases are not
available. Vitrectomy with internal gas tamponade and additional laser photocoagulation has recently been shown to
produce long-term improvement in visual acuity (13). The
initial intent of this treatment was to compress the retina
at the edge of the disc to enhance the effect of laser treatment. However, Lincoff et al have postulated that internal
gas tamponade also functions to mechanically displace
subretinal fluid away from the macula, allowing a shallow,
inner layer separation to persist, which is associated with a
mild scotoma and relatively good visual acuity (14).
The purpose of this study was to investigate the results
of small-gauge vitrectomy, posterior vitreous detachment,
peeling of the ILM, laser of the optic nerve margin, and gas
tamponade in 10 eyes with OPM.
were collected. Snellen visual acuity was converted into a
logarithm of minimal angle of resolution (logMAR) and for
the statistical analysis the Student t test was applied.
Surgical procedure
MATERIALS AND METHODS
All patients underwent small-gauge vitrectomy. Surgery
was performed by one surgeon (S.R.). The instrumentation used was 25-gauge vitrectomy system by Bausch &
Lomb®, Rochester, NY, USA; 23-gauge or 25-gauge vitrectomy system by Alcon®, Fort Worth, TX, USA. Three cannula-trocar systems were inserted transconjunctivally into the
eye at an oblique angle (15). The conjunctiva was displaced
above the designated sclerotomy to avoid alignment of the
conjunctival and the scleral entry site. The trocars were
then removed and the cannulas served as an entry site to
facilitate the exchange of the instruments. Detachment of
the posterior hyaloid was induced if not yet present, and a
complete as possible vitrectomy was performed. The ILM
was peeled without use of a dye. We performed endolaser
photocoagulation to the papillomacular bundle along the
temporal edge of the nerve head. Then the globe was filled
with long-acting gas. Surgery was completed by removal
of the cannulas without suturing the conjunctiva and the
sclera. Sclerotomies were inspected for possible wound
leakage and vitreous incarceration. The patient was asked
to keep a prone position for 1 week.
This study is a retrospective, noncomparative case series,
approved by the local ethics committee.
RESULTS
Main outcome measures
The main outcomes were best-corrected visual acuity,
anatomic outcome recorded by funduscopy, fundus photographs, and OCT imaging.
Patients and methods
We performed a computer-based investigation to identify
all patients who had undergone pars plana vitrectomy for
the treatment of optic disc pit maculopathy at our institution
between January 2005 and March 2009. Demographic data
were collected from the medical records. The preoperative
and postoperative best-corrected visual acuity (BCVA), the
anatomic outcome, and functional examinations as fundus
photographs and OCT images (Carl Zeiss®, Jena, Germany)
We identified 10 eyes of 10 patients that underwent smallgauge vitrectomy for OPM during the study period (Tab. I).
Seven patients were female, 3 were male. Mean age was
28 years (range 10-67 years). In 2 patients the optic pit was
bilateral. The right eye was affected in 5 patients, the left
eye in 5 patients. None of the patients were affected by a
systemic disease or were under regular medical therapy at
the time of surgery. The time period between perceived alteration of vision and presentation at our clinic ranged from
1 day (patient 1, monocular) to 36 months (patient 8), mean
9 months. Except for patient 1, all patients had a duration
of symptoms of at least 2 months.
Preoperative BCVA ranged from 1.5 to 0.3 logMAR units
(mean 0.86, SD=0.48) (Tab. II). Refraction error in these
eyes ranged from –2.50 D to +7.00 D. Follow-up was 14 to
60 months (mean 27 months).
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Small-gauge vitrectomy for optic disc pit maculopathy
Fundus examination showed a serous retinal detachment
associated with an optic pit anomaly in all eyes. In patient
8 (duration of symptoms 36 months) significant changes
of the retinal pigment epithelium were seen. Color fundus
photographs were taken with a standard fundus camera.
The OCT examination confirmed the presence of subretinal
fluid in all eyes. In 3 patients, OCT images showed a splitting of the retina with a schisis-like, intraretinal accumulation of fluid, in addition to the presence of subretinal fluid.
In patient 6 the OPM was associated with a macular hole
(duration of symptoms 12 months). Vitreous traction was
observed in one eye.
Seven patients underwent 23-gauge vitrectomy; in 3 eyes,
25-gauge vitrectomy instrumentation was used. Vitrectomy was uneventful in all eyes. No hypotony, endophthalmitis, or any other serious complications were noted in
the postoperative period. One patient (number 7, 57 years
old) presented cataract formation and progression following vitrectomy and required surgery for cataract extraction
and intraocular lens implantation. It is worth noting that
especially among the young patients the crystalline lens
remained clear.
Postoperative BCVA ranged from 1.6 to 0.1 (mean 0.41,
SD=0.45) (Tab. II). With respect to preoperative values,
TABLE I - PATIENT DEMOGRAPHICS AND CLINICAL FINDINGS
No.
Age, y
Sex
Eye
Bilateral/unilateral optic
pit anomaly
Refraction
Duration of
symptoms
Follow-up, mo
1
43
F
Left
Unilateral
+7.00 D
5d
60
2
59
F
Left
Unilateral
–2.50 D
>3 mo
48
3
29
F
Left
Unilateral
–2.50 D
18 mo
36
4
67
F
Left
Bilateral
–1.75 D
9 mo
18
5
26
F
Right
Unilateral
Emmetropic
2 mo
19
6
10
F
Left
Unilateral
Emmetropic
12 mo
19
7
57
F
Right
Bilateral
+3.00 D
4 mo
18
8
28
M
Right
Unilateral
+1.25 D
36 mo
17
9
22
M
Right
Unilateral
Emmetropic
2 mo
16
10
20
M
Right
Unilateral
Emmetropic
4 mo
14
TABLE II - PREOPERATIVE/POSTOPERATIVE BCVA AND OCT FINDINGS
Patient no.
BCVA
pre
Preoperative OCT
BCVA
post
Postoperative OCT
Further surgery
1
1.5
Schisis-like structure, SRF
0.2
+
No
2
1.5
1.6
+, New MH
3
0.5
Schisis-like structure,
SRF
Vitreoretinal traction, SRF
0.1
+
Vitrectomy +
heavy silicone oil
No
4
0.7
SRF
0.5
+
No
5
0.5
SRF
0.3
*
No
6
1.0
Macular hole, SRF
0.1
MH closed
No
7
1.5
SRF
0.5
*
Cataract extraction
8
0.4
RPE changes, SRF
0.3
*
No
9
0.3
SRF
0.1
+
No
10
0.7
Schisis-like structure, SRF
0.4
*
No
+ = Complete retinal reattachment; * = partial retinal reattachment; BCVA = best-corrected visual acuity in logMAR units; CF = counting fingers; MH = macular hole;
OCT = optical coherence tomography; RPE = retinal pigment epithelium; SRF = subretinal fluid.
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© 2011 Wichtig Editore - ISSN 1120-6721
Rizzo et al
there was a significant improvement (p<0.05, Student t
test). Visual improvement was recorded in 9 out of 10 eyes
and occurred more than 3 months after surgery. Four patients gained 3 or more lines in vision, 5 eyes gained 1 or
2 lines in vision, and 1 eye lost visual acuity (due to the
formation of a macular hole after vitrectomy for OPM).
The retina reattached completely in 4 eyes, as confirmed
by OCT imaging. In 4 eyes the subretinal fluid was only partially absorbed, resulting in a slow improvement in vision.
In some eyes the OCT findings did not correlate well to
the functional outcome. In patient 6, who was affected by
OPM and additional macular hole, the retina was attached
and the macular hole was closed, reaching a BCVA of 0.1
logMAR units. As mentioned above, patient 2 presented
the formation of a macular hole 1 month after surgery and
underwent a second vitrectomy with heavy silicone oil
tamponade. After heavy silicone oil removal the macular
hole appeared closed and the retina completely attached
but visual acuity was not better than counting fingers.
DISCUSSION
Although this disease has a good prognosis, persistent and
untreated OPM can lead to secondary macular changes
and pigmentary degeneration, irreversible reduced central
vision, and central field defects (1, 10). Indeed, patient 8,
whose symptoms had lasted 3 years, showed RPE changes when coming to our observation. He improved only one
line in vision, although postoperatively the subretinal fluid
was reabsorbed. Therefore we do not think that waiting for
a spontaneous resolution is justified, even if this has been
described in the literature (16).
Since there are contradictory reports about the pathogenesis, the treatment approach is also still under debate,
since no treatment has clearly proved more advantageous
than the others. Studies on a large number of patients, in
particular case-controlled, do not exist. In literature, case
reports are mostly described (12, 16-19). Georgalas et al
found visual improvement after vitrectomy, ILM peeling,
and gas tamponade without laser photocoagulation in 2
cases (12). Snead et al (17) and Gandorfer et al (18) performed vitrectomy, laser photocoagulation, posterior hyaloid peeling, and gas tamponade with success in one case.
Jalil et al performed successful vitrectomy and drainage
of subretinal fluid in one case (19). However, the limited
number of patients and the fact that spontaneous resolu-
tion of the pathology also exists does not provide sufficient
evidence in regard to the surgical approach. In contrast to
the present literature, we investigated 10 eyes and visual
improvement was achieved in 9 eyes. The case number is
small, but relatively important, considering the rarity of the
disease.
The rationale for vitrectomy lies in the fact that tractional
forces could explain the delay of macular detachment in
young adulthood and the frequency of treatment failure after laser photocoagulation and gas tamponade. The presence of vitreoretinal traction on the macula in the literature
is controversial. In the largest published series of this condition, Theodossiadis et al found that OPM was caused by
vitreomacular traction in 28 out of 38 patients (7). They performed macular buckling procedure to relieve vitreomacular traction and obtained anatomic and functional success
in 9 out of 9 eyes (20). The fact that longstanding OPM can
result in a macular hole demonstrates that probably the
same tractional force is active in both pathologies (8). In
fact, patient 6 presented a macular hole in addition to OPM
and had a history of 12 months. In contrast, Karacorlu et al
investigated 12 patients: none of the patients had macular
hole or vitreoretinal traction (21).
The other reported option is laser photocoagulation. Assuming that the subretinal fluid passes from the optic pit,
it would be desirable to create a better chorioretinal adhesion at the edge of the pit (5). Brockhurst also observed
the presence of a tiny hole at the edge of the optic pit from
where the fluid passed into the subretinal space in 2 eyes
(5). He achieved retinal reattachment in 5 eyes after repeated laser coagulation along the disc margin. He also found
that laser treatment did not compromise the visual acuity
or worsen the scotoma, also because the optic pit itself, as
well as the long-standing detachment, could cause a visual field defect. The damage caused by photocoagulation
on the nerve fiber layer is much reduced in the presence of
subretinal fluid. Therefore prophylactic laser treatment at
the edge of the pit in the absence of OPM should not be
considered (5). Laser treatment should be performed with
caution but can be repeated if the OPM persists (5).
There are only 2 recent studies on a large number of patients (both retrospective and noncomparative). Hirakata et
al investigated 11 eyes and performed vitrectomy, induction of posterior vitreous detachment, and gas tamponade
without laser treatment (22). Complete retinal attachment
over 1 year postoperatively was obtained and visual improvement was seen in 7 out of 11 eyes. No recurrences
© 2011 Wichtig Editore - ISSN 1120-6721
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Small-gauge vitrectomy for optic disc pit maculopathy
occurred. Our study confirmed that the visual improvement
can take a very long time. The other study is by GarcíaArumí et al, who performed vitrectomy, laser photocoagulation, and gas endotamponade in 11 eyes (23). Reabsorption of the macular detachment was seen in all cases
and resulted in an increase of BCVA. García-Arumí pointed
out the importance of performing photocoagulation after vitrectomy, but before fluid-gas exchange, in order to
minimize the laser-induced damage to the papillomacular
bundle (23). Our findings are broadly consistent with these
2 studies.
In our study, good visual results were obtained by smallgauge vitrectomy, mechanical detachment of the posterior
hyaloid, ILM peeling, gas tamponade to relieve abnormal
vitreoretinal traction over the macula, and additional laser
photocoagulation at the edge of the pit for the closure of
the defect. Nine out of 10 patients had a significant improvement in BCVA, even if OCT images showed complete
retinal attachment in only 5 out of 10 eyes. The functional
outcome may not correspond well to the anatomic outcome. This was also described by Yip et al, who found
that there is not a good correlation between BCVA and
OCT imaging in central serous chorioretinopathy (24).
We cannot exclude the hypothesis that the release of continued vitreoretinal traction achieved by surgical induction
of PVD could be sufficient on its own to reduce the new fluid accumulation in the inner layer separation, presumably
coming from the optic disc pit, as shown in the Hirakata
et al series (22). However, laser photocoagulation at the
optic pit margin may help to maintain adhesion over time;
as shown by our follow-up, ranging from 14 to 60 months
(mean 27 months).
In conclusion, our data concerning patients who underwent small-gauge vitrectomy, ILM peeling, laser photocoagulation, and gas endotamponade are in the range of other studies and concur with the previous results achieved
by García-Arumí et al using a similar technique and standard 20-gauge vitrectomy instrumentation. Therefore we
can conclude that it may be the current preferred approach
to treat OPM.
Further studies are required to evaluate this technique,
although the implementation of large-series studies remains a challenge because of the rarity of cases of optic
disc pit maculopathy.
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The authors report no proprietary interest or financial support.
Address for correspondence:
Stanislao Rizzo, MD
Via Diaz 86
55100 Lucca
Italy
[email protected]
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