Purpose: The aim of this study was to analyze the impact of OCT characteristics on the natural course of optic disc pit maculopathy (ODP-M). Methods: This was a single-center, retrospective, observational case series. Patients with ODP-M were included in the study. Baseline demographic characteristics, central macular thickness (CMT), IPP, subretinal fluid (SRF), subretinal precipitations, outer retinal fluid (ORF), and outer retinal layer hole were evaluated. The changes in the OCT characteristics were analyzed with respect to the best-corrected visual acuity (BCVA) from baseline to the last visit. Results: Twenty-two eyes of 22 patients were evaluated with a mean follow-up time of 37.6 (median 22) months. The mean BCVA was 0.31 logMAR at baseline and 0.28 logMAR at the final visit (p = 0.521). Baseline BCVA was significantly related to CMT (β coefficient 0.001, p = 0.002). Mean BCVA increased in 7 patients (group 1), remained stable in nine (group 2), and decreased (group 3) in 6 patients. No significant difference was found between the groups regarding the baseline BCVA, CMT, and extent of retinal fluid. In patients with subretinal deposits, BCVA remained stable in 3 patients and worsened in one. ORF was recorded in all patients. In patients with SRF, the mean change of BCVA during follow-up differed significantly, depending on the presence (0.07 logMAR) or absence (−0.125 logMAR) of SRF (p = 0.019). Conclusion: Among the OCT characteristics, SRF was a negative prognostic factor in ODP-M. Most ODP-M patients showed improved or stable vision over 3 years of follow-up. Therefore, observation may be considered in patients with reasonable vision and without SRF even when they present with remarkable OCT findings.

Optic disc pit (ODP) is an optic disc cavity anomaly that is generally congenital but can develop secondary to some diseases such as glaucoma and myopia [1, 2]. Although mostly unilateral,bilateral involvement has been reported in 15% of the patients [3]. The prevalence of ODP is 2/10,000 [4]. A prospective study found its incidence to be approximately one in two million [5].

ODP maculopathy (ODP-M) develops in 25–75% of patients and resolves spontaneously in approximately 25% [6‒8]. Most of the previous studies in the pre-optic coherence tomography (OCT) era reported a poor prognosis in patients with ODP-M if left untreated [7, 8]. Therefore, surgery has been recommended for patients with ODP-M.

The advance in OCT imaging enables detailed examination of the optic disc and macula. Consequently, the knowledge about ODP and ODP-M is also expanded. Few studies based on OCT imaging have reported visual improvement or stable visual acuity in some patients with ODP-M in the long-term follow-up [5, 9, 10]. Almost all OCT studies paid special attention to finding a possible prognostic factor for surgical success by analyzing the preoperative OCT features in ODP-M. Subretinal deposits, intrapapillary proliferation (IPP), and the extent of retinal fluid have been reported as factors associated with disease severity and negatively affecting surgical prognosis [11‒13]. On the other hand, little is known about the prognostic role of these morphological features in the natural course of ODP-M. In our study, we aimed to investigate the change of the OCT characteristics in the natural course of ODP-M and their effects on the visual acuity.

All patients who presented to the Department of Ophthalmology of the University of Tübingen between January 2008 and January 2021 and were diagnosed with ODP were included in this retrospective analysis. Approval was obtained from the Local Ethics Committee (283/2021BO2), and this study was conducted in line with the Declaration of Helsinki.

A complete ophthalmological examination including Spectral domain-OCT (Spectralis, Engineering GmbH, Heidelberg, Germany) was performed. Patients with a previous laser treatment or vitrectomy, a follow-up of less than 6 months and without an Early Treatment Diabetic Retinopathy Study (ETDRS) macular thickness map were excluded. Best-corrected visual acuity (BCVA) was tested with a Snellen chart and converted into logarithm of the minimum angle of resolution (logMAR) for further statistical analysis. ODP features were identified in color and red-free fundus photographs. The size of the ODP was measured in clock hours, and the location was noted regard to the optic disc center. ODP-M was diagnosed if fluid in the macular region was identified on OCT examination. Retinal fluid was classified as subretinal fluid (SRF), outer retinal fluid (ORF), or intraretinal fluid according to its location in the retinal layer. If intraretinal fluid and ORF were found, it was classified as multilayer fluid (MLF) (shown in Fig. 1). Subretinal precipitates, IPP, and outer retinal layer hole were recorded (shown in Fig. 2, 3). Patients were divided into three groups as improved BCVA (group 1), stable BCVA (group 2), and decreased BCVA (group 3), if a change of BCVA of at least 0.1 logMAR was recorded from the baseline to the last visit.

Fig. 1.

OCT images of a patient followed up with ODP-M. a OCT image characterized by SRF (white arrow head), ORF (white open arrow), and schisis of the innermost layer (white arrow) (BCVA 0.3 logMAR). b Twenty two months after diagnosis, complete resolution of the schisis, and partly regression of the intraretinal and SRF. An outer retinal hole (white arrows) has developed (BCVA 0.3 logMAR). c Follow-up examination 44 months after the diagnosis showed complete resolution of the retinal fluid (BCVA = 0.1 logMAR).

Fig. 1.

OCT images of a patient followed up with ODP-M. a OCT image characterized by SRF (white arrow head), ORF (white open arrow), and schisis of the innermost layer (white arrow) (BCVA 0.3 logMAR). b Twenty two months after diagnosis, complete resolution of the schisis, and partly regression of the intraretinal and SRF. An outer retinal hole (white arrows) has developed (BCVA 0.3 logMAR). c Follow-up examination 44 months after the diagnosis showed complete resolution of the retinal fluid (BCVA = 0.1 logMAR).

Close modal
Fig. 2.

OCT images of a patient followed up with the diagnosis of ODP-M. a OCT image characterized by SRF, ORF, and elongated photoreceptor outer segments with subretinal deposit (white arrow) (BCVA 0.1 logMAR). b Six months after diagnosis, OCT image characterized by SRF, ORF, and elongated photoreceptor outer segments (white arrow) (BCVA 0.1 logMAR). c Eleven months after diagnosis, OCT image characterized by minimal ORF (BCVA = 0.0 logMAR).

Fig. 2.

OCT images of a patient followed up with the diagnosis of ODP-M. a OCT image characterized by SRF, ORF, and elongated photoreceptor outer segments with subretinal deposit (white arrow) (BCVA 0.1 logMAR). b Six months after diagnosis, OCT image characterized by SRF, ORF, and elongated photoreceptor outer segments (white arrow) (BCVA 0.1 logMAR). c Eleven months after diagnosis, OCT image characterized by minimal ORF (BCVA = 0.0 logMAR).

Close modal
Fig. 3.

OCT image of a patient with ORF due to ODP-M. The patient has IPP (white open arrow) and a suspected link between pit and retina (white arrows) (VA = 0.0 logMAR).

Fig. 3.

OCT image of a patient with ORF due to ODP-M. The patient has IPP (white open arrow) and a suspected link between pit and retina (white arrows) (VA = 0.0 logMAR).

Close modal

The macular thickness map with ETDRS circles was used for the measurement of the central macular thickness (CMT) and the extent of the fluid (Heidelberg Eye Explorer version 1.9.13.0, Spectralis Viewing Module 6.5.2.0, Heidelberg Engineering, Germany). The ETDRS retinal thickness map consisted of three concentric circles with a diameter of 1, 3, and 6 mm [14].

Descriptive and statistical analysis was performed using IBM SPSS Statistics 21. Age, follow-up time, BCVA, and baseline CMT as mean and median, gender, clinical features, fluid change, and other OCT findings were presented as percentages. Dependent variables were evaluated with the Wilcoxon signed rank test, independent variables with the Kruskal-Wallis test, and categorical variables with the χ2 test. The relationship between the variables was analyzed by multiple linear regression models. Statistical significance was accepted as a p value of 0.05 or less.

ODP was identified in 152 eyes of 133 patients. In 72 (54.1%) patients with ODP, the OCT examination revealed an ODP-M. Thirty-eight (52.7%) patients had ODP-M in the right eye, and 6 patients had bilateral ODP-M (8.3%). The following patients were excluded: 31 patients with less than 6 months of follow-up, 5 patients with previous laser treatment or vitrectomy, and 14 patients without baseline OCT or macular thickness map. In addition, one eye of four patients with bilateral ODP-M was randomly removed to include not more than one eye per patient in further analysis. In total, 22 eyes of 22 patients who met the inclusion criteria were included in the analysis.

The mean age of the patients was 36.2 years (median 32) and 11 (50%) of them were female. In 13 patients (59%), the right eye was involved. The mean follow-up time was 37.6 months (median 22). The demographic and clinical characteristics of the patients are given in Table 1.

Table 1.

Baseline characteristics of patients with ODP-M

 Baseline characteristics of patients with ODP-M
 Baseline characteristics of patients with ODP-M

The mean BCVA was at baseline 0.31 logMAR (median 0.20) and at the final visit 0.28 logMAR (median 0.10). The change in the BCVA was not found to be statistically significant (p = 0.521). In 7 patients, the mean BCVA increased from 0.37 logMAR (median 0.40) to 0.10 logMAR (median 0.00). BCVA did not change in 9 patients. In 6 patients, it decreased from 0.35 logMAR (median 0.25) to 0.53 logMAR (median 0.35). According to stepwise multiple regression analysis, no parameter was found related to baseline BCVA, except for the CMT (β coefficient 0.001, p = 0.002). In the regression analysis, no parameter related to the change in BCVA was found.

Table 2 summarizes the baseline BCVA, ETDRS macular thickness mapping, and the OCT features. There was no significant difference between the groups in baseline BCVA, CMT, and extent of retinal fluid, as identified on the ETDRS macular thickness map regions. BCVA improved in 3 of 13 patients with IPP, did not change in six, and decreased in 4 patients. Mean BCVA improved from 0.26 logMAR to 0.23 logMAR and from 0.38 logMAR to 0.35 logMAR in patients with and without IPP, respectively. SRF was present in 3 of the 13 patients with IPP and in 5 of the 9 patients without IPP. New SRF developed in 2 patients of the IPP group during the follow-up and SRF disappeared in 2 patients without IPP. No new IPP occurred during the follow-up. In 2 of 3 patients with outer retinal layer holes, vision worsened, and these patients were operated on. Spontaneous vision improvement of 0.3 logMAR was observed in the third patient. In patients with subretinal deposits, BCVA remained stable in three and worsened in 1 patient. The mean BCVA decreased from 0.37 logMAR (median 0.10) to 0.40 logMAR (median 0.10) in these patients. In patients without subretinal deposits, it increased from 0.30 logMAR (median 0.25) to 0.25 logMAR (median 0.15). Posterior vitreous detachment was found in 4 patients at the baseline, and none of the patients developed posterior vitreous detachment during the follow-up.

Table 2.

Baseline BCVA and OCT features by visual acuity change groups in patients with ODP-M

 Baseline BCVA and OCT features by visual acuity change groups in patients with ODP-M
 Baseline BCVA and OCT features by visual acuity change groups in patients with ODP-M

The analysis of retinal fluid distribution revealed MLF without SRF in 9 patients, the most common form, followed by ORF with SRF in five and ORF only in 5 patients (Table 3). At baseline, 8 patients had SRF with foveal involvement. The mean time for the persistence of SRF was 29.1 months (median 8.8). Mean BCVA in patients with SRF was at baseline 0.38 (median 0.30) logMAR and at final visit 0.45 (median 0.20) logMAR (p = 0.461). In one of the 8 patients with SRF at baseline, the vision improved, remained stable in four, and deteriorated in 3 patients. In one of the 3 patients with vision decrease, SRF completely disappeared, but an IS-OS defect was seen. The other patients had an increase in SRF. In 2 patients, new SRF developed during the follow-up. One patient with SRF at the baseline and 2 patients with newly developed SRF were treated surgically. In 14 patients without SRF at baseline, BCVA increased in 6 (42.9%) patients, remained stable in 5 (35.7%), and deteriorated in 3 (21.4%). In these patients, the mean BCVA was 0.27 logMAR (median 0.20) at baseline and 0.18 logMAR (median 0.10) at the final visit (p = 0.142). No significant difference in the baseline BCVA of the patients was seen with and without SRF at baseline (p = 0.702). However, during the follow-up period, there was a significant difference in change of BCVA between 10 patients who had at least in one visit with SRF (mean −0.07, median −0.05) and 12 patients without SRF (mean 0.12, median 0.05) (p = 0.019) (shown in Fig. 4; Table 4).

Table 3.

Retinal fluid distribution by visual acuity change groups in patients with ODP-M

 Retinal fluid distribution by visual acuity change groups in patients with ODP-M
 Retinal fluid distribution by visual acuity change groups in patients with ODP-M
Table 4.

Characteristics of patients classified by the presence of SRF

 Characteristics of patients classified by the presence of SRF
 Characteristics of patients classified by the presence of SRF
Fig. 4.

Change of BCVA depending on the presence of SRF (mean, SD). During the follow-up period, there was a significant difference in change of BCVA between 12 patients without SRF (mean 0.12, median 0.05) and 10 patients with SRF (mean −0.07, median −0.05) (p= 0.019).

Fig. 4.

Change of BCVA depending on the presence of SRF (mean, SD). During the follow-up period, there was a significant difference in change of BCVA between 12 patients without SRF (mean 0.12, median 0.05) and 10 patients with SRF (mean −0.07, median −0.05) (p= 0.019).

Close modal

The presented study investigated the vision change, retinal fluid, and OCT characteristics of the macula in patients with ODP-M in a mean follow-up of 38 months. The baseline BCVA, CMT, presence of subretinal deposits, IPP, and extent of the fluid were not found as determinative in the natural course. Patients with ODP-M and SRF had a tendency for vision decrease.

The mean age at presentation of our cohort was 36 years, similar to previous studies [5, 6, 9, 10]. Consistent with other reports, in 91% of the patients, the location of the optic pit was in the temporal, temporal superior, or temporal inferior region of the optic disc [5‒7, 9]. The ODP-M was found in 54% of the patients with ODP. The BCVA at presentation (0.31 logMAR) was similar with OCT-based studies but higher than some pre-OCT-era studies [5, 9, 10]. This discrepancy is probably due to the earlier detection of the maculopathies in asymptomatic patients with better vision using OCT.

Spontaneous resolution in ODP-M is known [15‒17]. However, long-term natural course studies of the ODP-M are limited. Except for one, all observational studies of the pre-OCT era reported visual deterioration [6‒8, 18]. On the other hand, OCT-based studies for ODP-M reported more often stable or improved macular morphology and vision [5, 9, 10]. In the prospective study by Steel et al. [11], 53 patients were followed for 12 months, and 81% of the patients remained visually stable or their vision improved. Tzu et al. [10] followed 8 patients (median 20 months) and noted stable vision in half of them. Bloch et al. [9] followed 31 patients with OCT for an average of 37 months (median 22) and found stable visual acuity of the patients. Similarly, 72% of the patients in our study had a stable or improved vision over a mean follow-up of 38 months (median 22).

Steel et al. [11] investigated the extent of retinal fluid relative to the temporal major vascular arcade. They concluded that vitrectomy was more successful if the fluid did not exceed the temporal major arcade [11]. We used the ETDRS macular mapping to provide precise information for the extent of macular fluid and found that baseline CMT and extent of the fluid in the macula were not a determinant in the natural course of visual acuity.

It is thought that IPP could be related to the complete resolution of ODP-M. One study reported that IPP was seen in all patients and increased over time [12]. In the presented study, IPP was detected in 60% of the patients who had better baseline BCVA and a lower rate of SRF.

Subretinal deposits were thought to be an indicator of chronicity in ODP-M patients. In such eyes, the initial visual acuity was low with a poor prognosis. Chatziralli et al. [13] reported an increase in vision in this group of patients after vitrectomy or scleral buckling. However, macular hole development was noted in some patients, and the increase in vision was limited [13]. In line with this study, we also found low BCVA in patients with subretinal deposits at the presentation and no negative effect on the visual prognosis during the follow-up.

In our study, the most common form of retinal fluid was MLF without SRF (40.9%), as reported by Bloch et al. [9] In some studies, the most common fluid type was MLF associated with SRF [5, 19]. We recorded ORF in all patients at the presentation. This can be considered as an indicator that the retinal fluid is progressing from the outer retinal layers to other layers [19]. The presence of SRF is thought to worsen the prognosis [20]. In our collective, only 8.3% of patients without SRF (one out of twelve) and 50% with SRF (five out of ten) had worsening of the vision. The reduction of vision was 0.07 logMAR in patients with SRF, similar to the previous studies [5, 9]. On the other hand, patients without SRF showed an improvement of −0.125 logMAR over the same time period. In one study, spontaneous retinal fluid resolution developed in 5 patients without SRF with foveal involvement [5]. In another study, six of the patients with foveal SRF had spontaneous complete resolution [9]. In our study, a spontaneous retinal fluid resolution was seen in 4 of 22 patients. Two of these patients had SRF with foveal involvement.

Our study has several limitations, inherent to the retrospective design. We could not determine the time-dependent change of the fluid characteristics because of the different intervals of the follow-up examinations. Also, the amount and change of IPP could not be evaluated quantitatively. Another limitation of the study is certainly the small number of patients. Due to the strict criteria, only 22 patients with ODP-M were included in this analysis. Thus, generalization of the results should be made with caution. On the other hand, the homogenous composition of our collective is the strength of this study, enabling an objective analysis of the findings.

Observation of ODP-M patients with OCT, including ETDRS macular mapping, can facilitate the dynamic and precise assessment of the macula. The presence of SRF in ODP-M is associated with a significant worsening of vision. BCVA of patients without SRF stays stable or may improve even in the presence of severe OCT changes over the time period of 3 years. Considering this, a decision to surgically intervene in ODP-M patients without SRF should be made cautiously and on an individual basis.

Approval was obtained from the Local Ethics Committee of the University of Tuebingen, Germany, (283/2021BO2), and this study was conducted in line with the Declaration of Helsinki. This study has been granted an exemption by the Ethics Committee from requiring written informed consent.

The authors have no conflicts of interest to declare.

The authors have no funding sources to declare.

Study design was done by Ayse Güzin Taslıpınar Uzel and Faik Gelisken. Acquisition, analysis, and interpretation of data were done by Ayse Güzin Taslıpınar Uzel, Faik Gelisken, Karl Ulrich Bartz-Schmidt, and Jonas Neubauer. Drafting of the manuscript was done by Ayse Güzin Taslıpınar Uzel, Faik Gelisken, and Jonas Neubauer. Critical revision was done by Ayse Güzin Taslıpınar Uzel, Faik Gelisken, Karl Ulrich Bartz-Schmidt, and Jonas Neubauer.

Data generated or analyzed during this study are mostly included in this article. Further inquiries can be directed to the corresponding author.

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