Retrospective long-term surgical results of internal limiting membrane-sparing vitrectomy for pediatric optic disc pit maculopathy

Introduction

Optic disc pit is a kind of congenital disc abnormality presented with a coloboma of the optic nerve head. Optic disc pit appears as an independent, oval and grey-white depression located at the infertemporal margin of the optic disc (1). Severe decreased visual acuity can occur as a result of complicated optic disc pit maculopathy (ODPM), especially in those with subretinal fluid (SRF) involving the macula (1,2).

For patients presenting with optic disc pit accompanied by persistent macular SRF and significant visual acuity impairment, surgical intervention may be warranted (3,4). Pars plana vitrectomy (PPV), supplemented with various adjuvant therapies such as laser therapy, gas tamponade, and internal limiting membrane (ILM) peeling, has emerged as the standard surgical approach in the management of OPDM (5-8).

However, the usefulness of ILM peeling in ODPM still remains controversial, especially in pediatric patients. Babu et al. reported in their study that ILM peeling can be beneficial for multilayer retinoschisis resolution for patients with ODPM (9), while other studies observed an association between ILM peeling and the appearance of complicated full-thickness macular hole (10-12). Few studies specifically investigated the long-term anatomical and functional outcomes of vitrectomy without ILM peeling in pediatric patients with OPDM. This may be attributable to the rarity of such cases. The purpose of this study was to determine the outcomes of ILM-sparing vitrectomy for the treatment of rare cases of OPDM in pediatric patients. We present this article in accordance with the STROBE reporting checklist (available at https://tp.amegroups.com/article/view/10.21037/tp-2024-560/rc).

Case presentation

Medical records of four patients (four eyes), who were diagnosed with OPDM and underwent ILM-sparing vitrectomy between January 2019 and July 2022 in the Pediatric Retina Department of Beijing Tongren Hospital, were retrospectively reviewed. All surgical procedures were performed by a single experienced surgeon, Dr. H.L., using a microscope-integrated intra-operative (optical coherence tomography (OCT) system (Rescan 700, Zeiss, Oberkochen, Germany). This study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Beijing Tongren Hospital Ethics Committee Board (No. 20200523) and informed consent was taken from all the legal guardians after a complete description of the study.

The diagnosis of ODPM in this study was based on the presence of an optic disc pit in combination with SRF involving the macular fovea. A retrospective review was conducted on the complete follow-up records of four patients. Best corrected visual acuity (BCVA), fundus photographs, OCT, and central macular thickness (CMT) were included. BCVA was measured using the logarithm of the minimal angle of resolution (LogMAR). CMT was measured in micrometers (µm) using OCT. BCVA and CMT were analyzed using descriptive statistics (mean, standard deviation, range). The changes in BCVA and CMT were compared and analyzed before surgery and at 1, 3, 6, 12, 18, and 24 months postoperatively to assess visual function and anatomical outcomes at different stages. OCT images were acquired using various equipment models due to the randomized assignment of device types for preoperative and postoperative follow-up assessments in each case (Spectralis SD-OCT, Heidelberg Engineering, Heidelberg, Germany; RTVue XR OCT, Optovue, Fremont, CA, USA; VG 200D, Intalight Ltd., China).

PPV was performed for persistent SRF involving the macular fovea with decreased visual acuity. During the surgery, PPV combined with laser coagulation at the lateral edge of the optic pit was performed; posterior vitreous detachment was achieved, but the ILM was not peeled off. Fluid-air exchange with C₃F₈ tamponade was used in all cases. Main outcomes included pre- and postoperative BCVA and CMT; also, the mean duration for the resolution of subretinal and intraretinal fluid in the macular region, as assessed via OCT imaging.

Baseline clinical characteristics

This study included four eyes of four pediatric patients. Among those four children, three were male and one was female. The mean age was 7.75±3.77 years (range 5–13 years). The average duration from the onset of visual impairment symptoms to surgical intervention was 3.50±3.00 months. Patients were followed for a mean of 29.75±4.57 months after surgery (range 25–35 months). All the patients had no prior treatment before presenting to our hospital. Preoperative clinical characteristics are summarized in Table 1.

All the 4 eyes demonstrated macular detachment with SRF, retina schisis and macular edema of varying severity on OCT images; none of the eyes had posterior vitreous detachment on OCT or fundus examinations; two of the four eyes (case 1 and case 3) also had simultaneous choroidal colobomas (Figures 1,2); and two of the four eyes (case 2 and case 4) also had preoperative out-layer lamellar macular hole (Figures 3,4); and one eye had pre-papillary residual condensed vitreous (Figure 2).

Figure 1 Anatomical improvement after ILM-sparing vitrectomy combined with laser, gas tamponade for case 1 (a 13-year-old girl). This figure includes preoperative (A) and postoperative fundus photographs (C: 1 month postoperative; E: 6 months postoperative; G: 12 months postoperative); preoperative (B) and postoperative (D: 1 month postoperative; F: 6 months postoperative; H: 12 months postoperative) OCT images with corresponding BCVA and CMT. White dotted circles indicate multiple choroidal colobomas, white arrows indicate laser induced scar presented with localized hyperreflective signals; red dotted circles indicate optic disc pit; green arrows indicate the scanning locations and directions of the OCT images in the right column. BCVA, best-corrected visual acuity; CMT, central macular thickness; ILM, internal limiting membrane; OCT, optical coherence tomography; POM, postoperative months; Preop, preoperative.

Figure 2 Anatomical improvement after ILM-sparing vitrectomy combined with laser, gas tamponade for case 3 (a 5-year-old boy). This figure includes preoperative (A) and postoperative (C: 3 months postoperative; E: 12 months postoperative) infrared fundus photograph; preoperative (B) and postoperative (D: 3 months postoperative; F: 12 months postoperative) OCT scans with corresponding BCVA and CMT. White arrow indicates pre-papillary residual condensed vitreous; red dotted circles indicate optic disc pit; The green lines indicate the scanning locations and directions of the OCT images in the right column. BCVA, best-corrected visual acuity; CMT, central macular thickness; ILM, internal limiting membrane; OCT, optical coherence tomography; POM, postoperative months; Preop, preoperative.

Figure 3 Anatomical improvement after ILM-sparing vitrectomy combined with laser, gas tamponade for case 2 (a 6-year-old boy). This figure includes preoperative (A) and postoperative (C: 6 months postoperative; E: 12 months postoperative) infrared fundus photograph; preoperative (B) and postoperative (D: 6 months postoperative; F: 12 months postoperative) OCT scans with corresponding BCVA and CMT. White dotted circle indicates preoperative lamellar out-layer macular hole. BCVA, best-corrected visual acuity; CMT, central macular thickness; ILM, internal limiting membrane; OCT, optical coherence tomography; POM, postoperative months; Preop, preoperative.

Figure 4 Anatomical improvement after ILM-sparing vitrectomy combined with laser, gas tamponade for case 4 (a 5-year-old boy). This figure includes preoperative (A) and postoperative (C: 3 months postoperative; E: 6 months postoperative; G: 12 months postoperative) infrared fundus photograph; preoperative (B) and postoperative (D: 3 months postoperative; F: 6 months postoperative; H: 12 months postoperative) OCT scans with corresponding BCVA and CMT. White dotted circle indicates preoperative lamellar out-layer macular hole.The green arrows in E&G indicate the scanig locations and directions of the OCT images in F&H. BCVA, best-corrected visual acuity; CMT, central macular thickness; ILM, internal limiting membrane; OCT, optical coherence tomography; POM, postoperative months; Preop, preoperative.

Visual acuity outcomes

Mean preoperative BCVA was LogMAR 1.18±0.67 (range 0.3–1.7). BCVA began to improve within the first month postoperatively, even in the absence of significant fluid reduction on OCT, and best BCVA gains were seen 12 months postoperatively (0.49±0.30), and BCVA was kept stable between 12 with 24 months postoperatively (Figure 5A).

Figure 5 Visual acuity and macular retinal thickness after vitrectomy with inner retinal fenestrations. (A) This graph shows the BCVA with standard deviation from baseline to 24 months postoperatively. (B) This graph shows the CMT from baseline to 24 months postoperatively. BCVA, best-corrected visual acuity; CMT, central macular thickness; ILM, internal limiting membrane.

Anatomical outcomes

Progressive postoperative improvements of CMT were seen from a mean preoperative CMT of 969.75±56.28 µm to a mean CMT of 265.25±22.10 µm at 12 months postoperatively and kept stable with the next 12 months of follow-up period (Figure 5B).

All four eyes had complete resolution of SRF with retina schisis disappearance and macular edema resolution, ending with total macular detachment resolved at the last visit (Figures 1-4). The time required for the complete absorption of SRF on OCT images varies among individuals. In case 3, SRF was fully absorbed within the first two months, whereas in case 1, SRF had not been fully absorbed even six months after surgery. On average, the complete absorption time is 5.13±2.17 months postoperatively. The laser-induced lesions along the temporal margin of the optic disc pit are characterized by localized hyperreflective signals within the retinal nerve fiber layer on OCT imaging (Figure 1), and an adhesion response at this site becomes evident as early as three weeks postoperatively, marking the initial area where SRF begins to resolve. The sequence of resolution was observed to begin with the absorption of SRF, followed by the restoration of the retinoschisis, with macular edema being the final component to resolve.

Recurrence of macular detachment or intraretinal fluid was not observed. Two of the patients had transient high intraocular pressure 1–2 weeks postoperatively, and none of the patients experienced any surgical complications, including cataract, recurrent retinal detachment, or full-thickness macular hole during the follow-up period.

Discussion

The present study investigated the long-term efficacy of ILM-sparing vitrectomy for treating ODPM in pediatric patients. Despite the rarity of ODPM, this maybe the first paediatric-only case series, to our knowledge, to address the surgical management of ODPM by using ILM-sparing vitrectomy and our findings demonstrated promising anatomical and visual outcomes including resolution of SRF, intraretinal fluid and macular edema, as well as improvement of postoperative CMT and BCVA.

ILM peeling may not be a necessary procedure in vitrectomy for pediatric patients with ODPM. Increasing evidence supported the efficacy and necessity of PPV combined with gas tamponade in treating ODPM with progressive vision loss and SRF involving the macula. However, the need for ILM peeling during PPV remains controversial, particularly in pediatric cases. Previous studies have reported that ILM peeling may facilitate the resolution of retinoschisis in the macular region, as it is thought to further relieve traction at the vitreoretinal interface (13,14). Conversely, other research has suggested that ILM peeling does not improve the anatomical or visual outcomes in ODPM patients (15) and may even increase the risk of complications, such as full-thickness macular hole formation (10). The findings of our study indicated that in pediatric ODPM patients, ILM peeling is not required during PPV to achieve favorable, stable, long-term anatomical and functional outcomes.

Previous studies on the resolution of retinal fluid in ODPM patients following vitrectomy, including both adults and children, have shown variable outcomes. One study reported complete fluid resolution in only 31.25% of cases, likely due to insufficient follow-up, with a median resolution time of 526 days (16). Other studies with longer follow-ups reported higher reattachment rates of 71.4% and 90.9%, with resolution times averaging 395 and 304 days, respectively (17,18). Another study involving 32 ODPM patients found a median reattachment time of 416 days with a mean follow-up time of 768 days (19). These studies underscore the typically long recovery time after vitrectomy. In contrast, our study demonstrated a 100% macular reattachment rate in pediatric patients, with a significantly shorter mean resolution time of 5.13 months (about 154 days). Similarly, another study involving six pediatric patients treated with the inner retinal fenestration technique also reported a 100% reattachment rate, but with a longer mean time of at least 12 months of complete resolution of retinal fluid (20). Those results observed in our study suggests that ILM-sparing does not adversely affect the rate or duration of anatomical recovery in pediatric ODPM patients; also that pediatric patients may tend to exhibit better anatomical outcomes and faster recovery times compared to adult ODPM patients following surgery; additionally, more rapid absorption of macular retinal fluid observed in our case series, compared to other pediatric study, may be attributable partly to the adjunctive laser photocoagulation applied to the temporal margin of the optic disc pit during surgery, postoperative OCT imaging (Figure 1D) consistently demonstrated that neuroepithelial reattachment initiated at the laser-treated areas, supporting the efficacy of combined laser photocoagulation in accelerating fluid resolution.

Studies on adult ODPM patients treated with ILM-sparing vitrectomy show significant CMT reduction: one study reported a decrease from 796 to 276 µm after 18.6 months (16), another from 750 to 277 µm over 44 months (17), and a third to 235 µm after 33.4 months (21). By comparison, our pediatric study showed even greater improvement, with mean preoperative CMT dropping from 969.75±56.28 to 265.25±22.10 µm at 12 months. Similarly, a study of six pediatric patients with inner retinal fenestration showed CMT reductions from 637.83±209.09 to 311.00±40.36 µm at 12 months (12). These results suggest that pediatric patients treated by ILM-sparing vitrectomy may achieve greater reductions in CMT and faster anatomical recovery compared to adults following surgical intervention for ODPM.

In addition to anatomical improvements, our pediatric patients with OPDM achieved substantial gains in BCVA. Notably, BCVA began to improve immediately postoperatively, even in the absence of significant fluid reduction on OCT, a finding consistent with observations in other pediatric case series employing different surgical modalities (20), as well as in adults undergoing vitrectomy with or without ILM peeling (22,23). Our pediatric cohort achieved a final mean BCVA of 0.1±0.08 (LogMAR) at postoperative 12 months, which is superior to the mean postoperative BCVA of 0.16±0.29 (LogMAR) reported in previous studies at 1 year (20), also better than BCVA outcomes in adult populations (14,24). These findings suggest that pediatric patients may possess a greater capacity for visual recovery even with persistent SRF compared to adults. Additionally, ILM-sparing vitrectomy appears to be more effective for achieving optimal BCVA compared to inner retinal fenestration. This is particularly significant in pediatric cases, where ongoing visual development underscores the potential impact of early intervention on long-term visual function.

Importantly, no cases of recurrent macular detachment or intraretinal fluid were observed during the follow-up period, indicating a low recurrence rate post-surgery. Moreover, none of the patients experienced serious complications such as full-thickness macular hole, cataract development, or secondary retinal detachment, which are potential risks in procedures involving ILM peeling. The absence of these complications further supports the notion that ILM-sparing vitrectomy may be a safer alternative in pediatric ODPM cases, where preserving the integrity of the retinal layers may be particularly crucial for long-term visual outcomes.

This study’s limitations include its retrospective design and small sample size, which restrict the ability to generalize the findings and preclude the use of a control group for comparing the efficacy of different surgical approaches, such as vitrectomy combined with laser and gas tamponade, with or without ILM peeling. Additionally, while the follow-up period averaged 29.75±4.57 months, it may not have been sufficient to detect late-onset complications or recurrences.

Conclusions

This retrospective study evaluated the long-term efficacy of ILM-sparing vitrectomy combined with laser and gas tamponade for pediatric ODPM. Findings indicate that ILM-sparing vitrectomy is an effective way that can resolve maculopathies and restore BCVA in pediatric patients with ODPM with minimal complications. Larger studies with extended follow-up are needed to confirm these outcomes and further optimize surgical techniques for this complex condition.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the STROBE reporting checklist. Available at https://tp.amegroups.com/article/view/10.21037/tp-2024-560/rc

Peer Review File: Available at https://tp.amegroups.com/article/view/10.21037/tp-2024-560/prf

Funding: The study was supported by Clinical Medicine Development of Special Funding in Beijing Tongren Hospital, Capital Medical University (No. TRZDYXZY201703); The Priming Scientific Research Foundation for the Junior Researcher in Beijing Tongren Hospital, Capital Medical University (No. 2021-YJJ-ZR-010). The funders had no role in the study design, data collection, analysis, interpretation of data, or writing of the manuscript.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tp.amegroups.com/article/view/10.21037/tp-2024-560/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. This study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Beijing Tongren Hospital Ethics Committee Board (No. 20200523) and informed consent was taken from all the legal guardians after a complete description of the study.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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