Abstract
Purpose
To discuss the anatomical and functional results in cases of optic disc pit maculopathy (ODP-M) with a follow-up of at least 11 years after scleral buckling procedure (SBP).
Methods
We studied 12 eyes with ODP-M treated with SBP, in a long-term follow-up of 12.8 ± 1.5 years after surgery. All patients underwent best-corrected visual acuity (BCVA) measurement, slit-lamp biomicroscopy, fundus photography, fluorescein angiography, indocyanine green angiography, B-scan ultrasonography, and optical coherence tomography at baseline and 6-12 months, 2 years and at least 11 years postoperatively.
Results
Complete macular reattachment was noticed between 6 and 12 months postoperatively. The BCVA improved significantly at the first postoperative examination. Further improvement was noticed at the second examination, while BCVA remained almost stable at the last examination. Foveal restoration of ellipsoid layer (inner segment/outer segment) was noted in 10 out of 12 cases. The existing vitreous strands remained unchanged during the follow-up. Vitreous traction gradually disappeared (4/5 eyes). Circulation in short/long posterior ciliary arteries was unaffected, while neither recurrences nor complications were observed during the follow-up period. Association of the scleral sponge to the scleral sheath of the optic nerve remained unchanged during the follow-up.
Conclusions
A total of 12.8 ± 1.5 years after treatment, all the studied cases retained the successful anatomical and functional results that they had 2 years postoperatively, without inducing cataract during the follow-up period. The SBP seems to act equally well as a barrier either obstructing the entrance of fluid from the vitreous cavity or blocking the circulation of subarachnoid cerebrospinal fluid into the retina.
Keywords
Introduction
Optic disc pit (ODP) is a rare congenital abnormality of the optic nerve head (1, 2). It typically appears as a small, hypopigmented excavation of the optic disc, approximately 500 μm in size. Most commonly it is located at the temporal or inferotemporal segment of the optic disc, although rarely it can be found centrally or along the nasal side of the optic disc (1, 2). It has no sex predilection (1). Optic disc pit can be associated with arcuate scotomas and enlarged blind spot, especially when it is large.
Optic disc pit remains asymptomatic or causes reduction of vision due to serous macular detachment in a frequency ranging from 25% to 75%. In cases of macular detachment, visual acuity is usually 20/80 or less in the affected eye (2). Optical coherence tomography (OCT) studies have shown that the macular elevation may have bilateral structure, such as schisis-like separation and outer layer detachment (3, 4), or macular elevation without schisis (5), while recent findings showed splitting of retinal layers by intraretinal fluid at multiple layers (6). The macular detachment especially in the long-standing cases can be associated with lamellar or full-thickness macular holes, cystoid changes, and retinal pigment epithelium atrophy, resulting in irreversible deterioration in visual acuity (2, 7-8-9).
The origin of the fluid and the pathophysiology of the ODP maculopathy (ODP-M) are controversial. Various reports have suggested either vitreous or cerebrospinal fluid as the cause of macular elevation. In both cases, the fluid enters into the macular layers through the ODP (10, 11). The purpose of this retrospective study was to evaluate the anatomical and functional results in patients with ODP-M who were treated with the scleral buckling procedure (SBP) and followed up for 12.8 ± 1.5 years after the surgical intervention. Additionally, the study aimed to compare the results of the last examination with those of the first and especially the second year postoperatively.
Materials and Methods
Study design
We retrospectively studied 12 consecutive patients (12 eyes) with ODP-M who were successfully treated with SBP between 1998 and 2003 and followed up for at least 11 years. The study was performed at the 1st Department of Ophthalmology, University of Athens, and the 2nd Department of Ophthalmology, Henry Dunant Hospital. At that time, 14 patients were operated by G.P.T. with the SBP. In one eye, intraretinal fluid reappeared, having another unsuccessful operation with the same technique, while one patient was lost to follow-up. From the original study, we excluded the case that was unsuccessfully treated and the patient who was lost to follow-up. The investigation was conducted according to the Declaration of Helsinki and all participants provided informed consent after the purpose of the study had been explained to them. The medical ethics committee of both hospitals approved the study.
Examination techniques
All patients underwent complete clinical examination, including measurement of best-corrected visual acuity (BCVA) by means of a Snellen chart, slit-lamp biomicroscopy, fundus imaging, intraocular pressure measurement, OCT examination, fluorescein angiography, indocyanine green angiography (ICG), and B-scan ultrasonography. For statistical purposes, BCVA was transformed to the logarithm of the minimum angle of resolution (logMAR). All patients were examined via time-domain OCT (Stratus OCT, Carl Zeiss Meditec, Dublin, California, USA). Spectral-domain OCT was used in all cases after 2008 (Spectralis Heidelberg OCT, Heidelberg Engineering, Heidelberg, Germany; and Cirrus OCT, Carl Zeiss Meditec).
Surgical technique
In the SBP, a 7.5 × 5.5-mm scleral sponge was placed in the area of the sclera, which corresponds to the macula and at the axis of the 12-6 o'clock meridian. The fixation of the upper end of the sponge was made at the posterior edge of the insertion of the superior oblique muscle, while the lower end was stretched and fixated at the temporal side of the belly of the inferior rectus muscle. To determine the correct position of the sponge and to avoid close contact of the explant with the optic nerve, B-scan ultrasonography was performed during surgery. Whenever the sponge was in close contact to the optic nerve, it was repositioned. No additional cryopexy, laser, gas, or encircling band was used (12).
Parameters assessed
The BCVA was assessed before and after ODP-M surgery. Other parameters, such as the type of macular elevation at presentation (i.e., schisis, retinal detachment, and mixed type), the location of the ODP on the optic disc, the communication between the ODP and subretinal fluid under the fovea, and the existence of lamellar or full-thickness macular holes were evaluated. In addition, the condition of the vitreous on the optic disc and macula, including the presence of vitreous traction or vitreous strands, and the status of inner segment/outer segment (IS/OS–ellipsoid zone) photoreceptors layer during follow-up were studied.
Specifically, the photoreceptor IS/OS junction line (ellipsoid zone) was evaluated in the fovea in an inner circle of 1-mm diameter and this line was identified as intact, disrupted, or absent. When the IS/OS junction line (ellipsoid zone) was continuous, it was characterized as intact, when it was interrupted by gaps shorter than 200 μm as disrupted, and if the gaps were 200 μm or longer as absent. Evaluation of the IS/OS junction line (ellipsoid zone) was confirmed using a minimum of 2 horizontal or vertical crosshair scan lines. The same masked OCT specialist performed each evaluation.
Follow-up protocol
All patients were examined postoperatively the first day, the first week, and monthly for 6 months. Annual examination was performed thereafter. For the purpose of the present clinical investigation, we included the baseline study and 3 postoperative examinations: the first 6-12 months (8.4 ± 2.2 months) after surgery, the second 2 years after surgery, and the last 11-15 years (12.8 ± 1.5 years) postoperatively.
Statistical analysis
Statistical analysis was performed using SPSS 20.0 statistical software (SPSS Inc., Chicago, Illinois, USA). Descriptive statistics, including the mean values, median, standard deviations, and percentages, were used to describe the baseline characteristics. The Wilcoxon matched-pairs signed-rank test or the McNemar test were used for comparisons at the different time points of the follow-up for ordinal and nominal data, respectively, as appropriate. A p value less than 0.05 was defined as statistically significant. Given that multiple comparisons were performed, the Bonferroni correction was adopted, i.e., the threshold for statistical significance was equal to 0.05/4 (0.0125). Additionally, potential correlation between the BCVA and the IS/OS (ellipsoid zone) condition was assessed using Spearman's rank correlation coefficient (Spearman's rho).
Results
In the present retrospective study, 12 patients (12 eyes) were included. Clinical data of patients before and after surgery are shown in Table I. At presentation, the mean ± SD age of patients enrolled was 23.3 ± 6.4 years. Five were male (41.7%) and 7 were female (58.3%). The mean BCVA was 1.09 ± 0.36 logMAR (range 0.60-1.90). The macular elevation was characterized as schisis in 2 cases (16.7%), retinal detachment in 1 case (8.3%), and of mixed type (retinal detachment and schisis) in 9 cases (75%). The location of the ODP was at the temporal side of the optic disc in all cases. Communication between the ODP and macular elevation was observed in 5 cases (41.7%). Lamellar macular hole coexisted in 4 cases (33.3%) and full-thickness macular hole in 1 case (8.3%). The mean postoperative follow-up period was 12.8 ± 1.5 years.
Clinical data in patients with optic disc pit maculopathy treated with scleral buckling procedure before and after surgery
IS/OS = inner segment/outer segment; VA = visual acuity; VMT = vitreomacular traction.
Visual acuity is recorded in logMAR scale.
At baseline, vitreomacular traction (VMT) was observed in 5 cases (41.7%), while presence of vitreous strands emerging from the ODP without any association with the macular area was noticed in 4 cases (33.3%). In the cases where the VMT was present, it was extended between the optic disc and the macular area (papillomacular traction). Vitreomacular traction was present in 2 cases with lamellar macular hole and in the 1 case with full-thickness macular hole (13). There was a trend to statistically significant difference as to the percentage of VMT between the baseline and the last examination (p = 0.046), while there was no statistically significant difference in the other examinations. Specifically, at the first postoperative examination (6-12 months after surgery), 4 cases (33.3%) had VMT, compared to the 5 (41.7%) at baseline (p = 0.564). Vitreomacular traction still existed in 2 cases (16.6%, p = 0.157) at the second examination (2 years after surgery) and in 1 case (8.3%, p = 0.317) at the last examination after surgery. During the follow-up, the VMT that was alleviated due to the SBP became faded and loosened. The presence and the shape of the existing vitreous strands in 4 cases remained unchanged at first (p>0.999), second (p>0.999), and last examination (p>0.999), as well as between the baseline and the final examination (p = 0.317). All patients presented complete resolution of fluid at the first postoperative examination (6-12 months after surgery). No fluid reaccumulation was noticed in the second and last examination (Figs. 1-3). The lamellar macular hole in 4 cases and the full-thickness macular hole in 1 case were sealed off due to SBP.

Case 5. Optic disc pit maculopathy in a 21-year-old woman. (

Case 7. (

Case 7. (
Best-corrected visual acuity improved significantly at the first postoperative examination (6-12 months after surgery) compared to baseline (p = 0.012). Further improvement was noticed at the second postoperative examination (2 years after treatment) in comparison with the first one, which was not statistically significant (p = 0.256). The BCVA remained almost stable and unchanged at the last examination compared to the second one (p = 0.659), while there was a statistically significant improvement of the BCVA between the baseline and the last examination of the follow-up (p = 0.005).
Preoperatively, the IS/OS photoreceptor junction line (ellipsoid zone) was intact in 4 of the 12 cases (33.3%), interrupted in 6 cases (50%), and absent in 2 cases (16.6%) (Tab. I). At the first postoperative examination (6-12 months), the IS/OS photoreceptor junction line (ellipsoid zone) was intact in 5 out of 12 (41.7%), interrupted in 5 cases (41.7%), and absent in 2 cases (16.6%), presenting no statistically significant difference compared to baseline (p = 0.743). At the second examination, the IS/OS photoreceptor junction line (ellipsoid zone) condition significantly improved in comparison with the first (p = 0.0028). Specifically, the IS/OS photoreceptor junction line (ellipsoid zone) was intact in 8 cases (66.8%), interrupted in 2 cases (16.6%), and absent in 2 cases (16.6%). No further improvement was noticed between the second and the last postoperative examination (p>0.999). Since IS/OS line (ellipsoid zone) is well-delineated with time-domain OCT, the comparison between the original examinations with time-domain OCT and the last examination with SD-OCT was accurate (14).
Significant correlation between BCVA and the integrity of IS/OS photoreceptor junction line (ellipsoid zone) existed at the first (Spearman's rho = −0.372, p = 0.001), second (Spearman's rho = −0.597, p = 0.004), and last examination of the follow-up (Spearman's rho = −0.722, p = 0.008). In the studied cases, postoperatively and especially in the second and last examination, the size of the surface and the borders of the ODP are more distinguishable and well-defined (Fig. 4).

Case 7. (
The ICG during the follow-up did not reveal any circular disturbances in long/short posterior ciliary arteries due to sponge fixation. This observation became evident from the first to the last examination of the follow-up and reassured that the correct sponge fixation did not influence the blood circulation of long/short posterior ciliary arteries at least 11 years after treatment. Cataract formation was not observed in operated eyes in the first and second year observation or in the last examination of the follow-up (12.8 ± 1.5 years).
Intraoperative hemorrhage located at the point of the sponge fixation was found immediately after surgery in 1 case and was absorbed within the first postoperative month. Lamellar macular hole and full-thickness macular hole vanished due to scleral buckling application.
Discussion
The exact mechanism responsible for the development of ODP-M has not been clarified. Among the most prevailing theories are those that support the origin of the fluid from the vitreous cavity or from the subarachnoid space (SAS) (11, 15-16-17). Recent studies induced by swept-source OCT have shown that in eyes with congenital ODPs, the pits were defects of the lamina cribrosa at the site of the pit and herniation of the nerve tissue into the pits (18). These investigations confirmed previous histologic findings that were performed in cadaver eyes with congenital ODPs (19, 20). Ohno-Matsui et al (18), with the help of the swept-source OCT, were also able to see the SAS just posterior to the bottom of the ODP. Even though any direct communication was not observed, it could be possible that a communication of fluid between ODP and SAS exists through such a thin pia matter. The authors noted a highly reflective tissue between the herniated retinal tissue, with thickness ranging from 88 to 125 μm. The study also discovered vitreous fibers that were connected to the herniated retinal tissue (18).
Further reports related to the origin of the fluid revealed subretinal migration of gas and intracranial migration of silicone oil, suggesting that there is a movement of fluid between the vitreous cavity and the subarachnoid space (21). Moreover, Jain and Johnson (11), in a recent review, suggested that the most plausible sources of fluid are the vitreous cavity and SAS, depending on particular conformation of the congenital disc pathologic features in each affected eye. The concept of liquefied movement of vitreous into the subretinal space through the ODP has been also supported (15, 22). Additionally, it is hypothesized that vitreous has a further participating role in the development of ODP-M due to the traction it exerts on the ODP (11, 23, 24) and in the peripapillary area (22).
Given that the mechanism and pathology of ODP-M had not been clarified, many surgical techniques have been applied, varying in anatomical and functional outcome. The proposed techniques include xenon and argon laser photocoagulation (7, 25), vitrectomy with the induction of posterior vitreous detachment (26), gas tamponade with or without photocoagulation (27, 28), vitrectomy alone or with various adjuncts, vitrectomy with inner layer fenestration (29), and scleral buckling (30, 31). In recent years, several investigators have performed vitrectomy with various adjuncts (26, 32-33-34-35-36-37-38-39-40). However, because many reports of surgical combinations seem to converge on a maximum approach combining vitrectomy, laser photocoagulation, and gas tamponade to optimize surgical outcomes, the effect of vitrectomy itself is unclear (29).
We have reported that SBP seems to be an effective surgical modality for managing ODP-M (12, 30, 31). The beneficial effect after scleral buckling application was based upon the assumption that the pit may function as a leak (41) and this action is probably due to the combination of traction on the pit associated with the existence of fluid components from the vitreous cavity or from SAS (11, 18, 22). The SBP seems to respond equally well to the treatment of macular detachment in the case where fluid originates from the vitreous cavity or if it emanates from the SAS. In the case where the fluid comes from the vitreous cavity, the vitreous traction of the optic disc probably facilitates the entrance of fluid through the ODP into the retina. In this case, the SBP elevates the macular surface and posterior hyaloid from an inward to outward vector obstructing the entrance of fluid into the intraretinal space. Simultaneously, it alleviates the vitreous traction of the macula in cases where vitreous traction is exerted on the macula. In the case where the fluid emanates from SAS, the sponge placement adjacent to the temporal side of the optic nerve collapses the course of the ODP canal, obstructing the flow of fluid. We supported this assumption mainly based on the histologic findings of cadaver eyes with ODPs, where it was found that the scleral canal forms the temporal wall of the ODP and that the pit is extended further posteriorly approaching the pia matter (20).
In all 12 studied eyes, the optic pit was located at the temporal side of the optic disc. Complete resolution of fluid from the intraretinal layers was observed between 6 and 12 months after surgery. The fluid absorption started from the nasal part of the elevation near the optic disc and extended to the rest of the macular elevation. This finding strengthens our observation that the buckle acts as a barrier obstructing the entrance of fluid from the ODP. The observation of Hirakata et al (42) regarding the dark appearance of the ODP after treatment was confirmed in our study. Hirakata et al support the view that the dark appearance of the ODP can be attributed to the eradication of the preexisting vitreous adhesion on the optic disc. In our cases, however, the dark appearance of the ODP and its delineated margins were detected, while the vitreous strands emerging from the ODP were present before treatment and during the whole period of the observation (Fig. 2). The same also happened with the alleviated VMT due to the SBP. This different appearance of the ODP between the baseline and the second as well as the last examination could probably be attributed to the absence of fluid into the entrance of the ODP in the successfully treated cases rather than due to the vitreous disappearance of the optic disc.
As far as the time frame of BCVA improvement, it became evident that BCVA presented significant improvement within the first year (6-12 months) after surgery, further improved at the 2-year postoperative examination, and remained almost stable thereafter. Noticeably, no recurrence occurred between the second and the last examination of the follow-up, while BCVA and IS/OS junction line (ellipsoid zone) condition remained stable. The stable condition of the operated eyes in the second postoperative year could predict an uneventful evolution for the further period of observation.
The preoperative vitreous traction that existed in 5 out of 12 cases (41.7%) vanished in all but one case due to the elevation of the papillomacular area, which was caused by the SBP. The appearance of vitreous strands before and after surgery, which remained unchanged, suggested that their presence probably does not influence the successful course of the operated eyes. The SBP responded equally well in the treatment of ODP-M independently of the type of macular elevation (schisis and retinal detachment, retinal detachment alone). Circulatory disturbances of the short/long posterior ciliary arteries were not observed from the first to the last examination of the follow-up, probably due to the elasticity of the applied sponge and the young age of the operated patients.
Even though it was supported that SBP obstructs the flow fluid into the retina, the correct positioning of the sponge in the macular area presented difficulties. As a result, the technique has been mostly replaced by the apparently easier pars plana vitrectomy. However, the recent findings with swept-source OCT (18, 43) and the latest postulated information about the origin of the fluid renew previous theories about the existing communication between the vitreous and SAS (11). Taking into account these recent findings in association with the results of our study, we support that the SBP causes a permanent obstruction of the flow of the fluid, which circulates into the canal of the optic nerve pit, occluding the entrance of the fluid into the retinal layers, without causing damage as the laser application induces. Based on the rationale of proposed treatment modalities, it seems that SBP is more appropriate in blocking the circulation of fluid from the SAS through the ODP into the retina.
The SBP is an option for repairing the ODP-M that may obviate the lens changes associated with vitrectomy, which may be an attractive benefit in younger phakic patients. The observation between the second year and the last examination of the long follow-up of 12.8 years did not show further changes regarding lens opacities.
Limitations of the study include the relatively small number cases and that the study does not compare equal numbers of patients treated with different surgical procedures in the same period of time. Another potential limitation pertains to the fact that SBP requires appropriate experience and has a long learning curve. However, the study describes the effectiveness of SBP in the treatment of ODP-M in light of recent discoveries with swept-source OCT, for the longest follow-up period to date (12.8 ± 1.5 years).
In conclusion, ODP-M is a perplexing disease, where we know the entrance of fluid into the retinal layers, but hypothesize about its origin. The SBP seems to respond equally well obstructing the entrance of fluid into the retinal layers when the fluid comes from the vitreous cavity or if it originates from SAS. In the last instance, the application of sponge adjacent to the temporal side of the scleral sheath canal of the optic nerve pit collapses the lumen of the canal and does not permit fluid circulation from the SAS, something that cannot be accomplished by current surgical modalities.
During the longest follow-up period so far of 12.8 ± 1.5 years, stability of BVCA, the status of IS/OS line (ellipsoid zone), and unaffected circulation in the long/short posterior ciliary arteries, studied by ICG, were noted. The observed anatomical and functional results of the last examination were similar to those 2 years after treatment. The second postoperative year could be considered of particular importance for further prognosis of the disease after SBP. Regarding the difficulties of sponge placement in the posterior pole (44), SBP has a long learning curve. The results from the present study in association with the long follow-up support that SBP is an effective technique for treatment of ODP-M.
Footnotes
Financial support: No financial support was received for this submission.
Conflict of interest: None of the authors has conflict of interest with this submission.
