Etiology and Management of Pediatric Macular Holes

Optical Coherence Tomography (OCT) is part of the routine examination of all vitreomacular disorders. Spectral domain technology has provided images with better resolution, which are also easier to interpret reliably. In most of these pathologic cases, it is now possible to visualize the status of the posterior hyaloid, its connection to the retinal surface, and its thickness and reflectivity. OCT plays an essential role in the diagnosis and preoperative assessment of macular holes. In impending macular holes, it shows minute irregularities of the foveal curvature, and/or tiny intrafoveal tractional retinal cysts. In full thickness macular holes, OCT clearly shows defects in the retinal tissue and allows full thickness macular holes to be distinguished from macular lamellar holes or pseudoholes. The quality of the OCT scan profiles also allows accurate measurement of macular hole diameter, which is a major indicator of the chances of successful surgery and may affect the choice of surgical technique. Furthermore, OCT allows assessment of the quality of macular hole closure. Although the presence of epiretinal membranes may also be diagnosed on red-free and blue reflectance photographs, OCT has the advantage of showing the degree of retinal thickening in the fovea, as well as the presence of any intrafoveal cysts or vitreomacular traction. Diagnosis of the vitreomacular traction syndrome has also been greatly facilitated by the use of OCT, which shows, more clearly than ultra sound or biomicroscopy, the elevation of the foveal surface due to the focal traction of a thick and hyper-reflective posterior hyaloid. Finally, OCT has allowed the identification of myopic foveoschisis, a hitherto unknown but relatively common complication related to myopic staphyloma. This vision - threatening complication may benefit from surgery, depending on its OCT characteristics. In conclusion, although OCT is still a technique in evolution, but it already provides invaluable information on vitreomacular disorders and plays an essential part in their diagnosis and management.

Background: A macular hole is a full-thickness defect of retinal tissue involving the anatomic fovea, thereby affecting central visual acuity. Pars plana vitrectomy and gas tamponade is a recognised modality of treatment for macular hole.Larger holes are more likely to remain open after repair and late reopening after an initially closed macular hole is seen in macular holes larger than 400 μm.Aims and Objective: To evaluate the anatomical and functional outcome of pars plana vitrectomy with internal limiting membrane peeling for chronic stage 3 macular hole.Materials and Methods: Records of 15 patients with stage 3 chronic macular holes operated from 1st January 2013 to 30th June 2013 and completed 1 year of follow up were retrospectively evaluated and included in the study. Preoperative best distance corrected visual acuity (BCVA), preoperative macular hole size, final BCVA and macular hole status at 1 year follow up were recorded. Macular hole closure and visual improvement was calculated. Correlation of macular hole closure and visual improvement with various macular hole parameter was estimated.Results: Eleven (73.3%) macular holes closed at 1 year follow-up. Mean BCVA improved from 1.2 ± 0.27 to 0.89 ± 0.36 logarithm of minimum angle of resolution at 1 year (p<0.001). Visual improvement was seen in only eight (53.3%) eyes. Both macular hole closure and visual improvement showed no correlation with minimum linear diameter, base diameter and hole height.Conclusion: Chronic stage 3 macular hole can be closed successfully in majority of patients with fairly good visual improvement. Macular hole parameters of stage 3 holes may not have any correlation with the anatomical and visual outcome.Asian Journal of Medical Sciences Vol.7(6) 2016 43-46

Classical or temporal internal limiting membrane (ILM) flap transposition with air or gas tamponade are current trends with the potential to improve surgical results, especially in cases with large macular holes. A prospective case series included patients with idiopathic macular holes or persistent macular holes after 23-G pars plana vitrectomy (PPV) and ILM peeling with gas tamponade. In all patients, 23-G PPV and ILM peeling with ILM flap transposition with gas tamponade and postoperative face-down position was performed. In 7 of 9 eyes, temporal ILM flap transposition combined with pedicle ILM flap could be successfully performed and macular holes were closed in all eyes after surgery. The remaining 2 eyes were converted to pedicle ILM flap transposition with macular hole closure after surgery. Three eyes were scheduled as pedicle ILM flap transposition due to previous ILM peeling. In 2 of these eyes, the macular hole could be closed with pedicle ILM flap transposition. In 3 eyes, free ILM flap transposition was performed and in 2 of these eyes macular hole could be closed after surgery, whereas in 1 eye a second surgery, performed as pedicle ILM flap transposition, was performed and led to successful macular hole closure. Use of ILM flaps in surgical repair of macular hole surgery is a new option of treatment with excellent results independent of the diameter of macular holes. For patients with persistent macular holes, pedicle ILM flap transposition or free ILM flap transposition are surgical options.

Objective To compare the outcomes of vitrectomy and internal limiting membrane peeling for the treatment of macular hole retinal detachment(MHRD) in high myopia and rhegmatogenous retinal detachment with macular hole (RRD+ MH). Methods In this retrospective case-control study, 51 eyes of 51 consecutive patients of retinal detachment with macular hole received standard pars plana vitrectomy with internal limiting membrane peeling and 16% C3F8/silicon oil tamponade. They were divided into two groups. Thirty eyes of MHRD with axial high myopia (the axial length longer than 26.0 mm) were in research group (high myopia group) and 21 eyes of 21 patients with RRD+ MH were in control group (RRD+ MH group). Anatomical closure of macular holes using optical coherence tomography (OCT) and final best-corrected visual acuity (BCVA) were compared between the two groups. Results Anatomical closure of the macular hole confirmed by OCT was achieved in 29 of 30 eyes (96.67%) in the high myopia group, and in 19 of 21 eyes (90.48%) in the RRD+ MH group. There was no statistically significant difference between the two groups (χ2=0.10, P=0.75). In the high myopia group, the mean LogMAR VA was (1.13±0.50) at the final follow-up after the surgery, and it was (1.87±0.55) before the surgery. In the RRD+ MH group, it was(2.00±0.54) before surgery and(1.16±0.51) after surgery. There was no significant statistically difference between the two groups in visual increase (p=0.66). Conclusion Vitrectomy and internal limiting membrane peeling with C3F8/silicone oil tamponade is effective for the treatment of macular hole induced retinal detachment in high myopia and rhegmatogenous retinal detachment with macular hole, with high closure rate of the macular hole and significant visual improvement. Key words: Retinal detachment, high myopia, macular hole; Retinal detachment, rhegmatogenous; Vitrectomy; Peeling, internal limiting membrane

A macular hole is an anatomic opening in the retina that develops at the fovea. Macular holes can be seen in highly myopic eyes or following ocular trauma, but the great majority are idiopathic. Pars plana vitrectomy was introduced to treat full-thickness macular holes, which if left untreated have a poor prognosis since spontaneous closure and visual recovery are rare.Vitrectomy is a surgical technique involving the removal of the vitreous body that fills the eye. The surgeon inserts thin cannulas into the eyes through scleral incisions to relieve traction exerted by the vitreous or epiretinal membranes to the central retina and to induce glial tissue to bridge and close the hole. The primary objective of this review was to examine the effects of vitrectomy for idiopathic macular hole on visual acuity. A secondary objective was to investigate anatomic effects on hole closure and other dimensions of visual function, as well as to report on adverse effects recorded in included studies. We searched the Cochrane Eyes and Vision Group Trials Register (4 March 2015), the Cochrane Central Register of Controlled Trials (CENTRAL; 2015, Issue 2), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE (January 1946 to March 2015), EMBASE (January 1980 to March 2015), Latin American and Caribbean Health Sciences Literature Database (LILACS) (January 1982 to March 2015), the Web of Science Conference Proceedings Citation Index-Science (CPCI-S) (January 1980 to March 2015), the ISRCTN registry (www.isrctn.com/editAdvancedSearch), ClinicalTrials.gov (www.clinicaltrials.gov) and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 4 March 2015. We included randomised controlled trials comparing vitrectomy (with or without internal limiting membrane peeling) to no treatment (that is observation) for macular holes. We used standard methodological procedures expected by Cochrane. Two review authors independently extracted the data. We estimated best corrected visual acuity and macular hole closure at 6 to 12 months of follow-up. Three studies provided data on the comparison between vitrectomy and observation in eyes with macular hole and visual acuity less than 20/50. Two studies, conducted in the USA and published in 1996 and 1997, used a similar protocol and included participants with stage II macular hole (42 eyes randomised, 36 analysed, number of participants not reported) or participants with stage III/IV hole (129 eyes of 120 participants, 115 eyes in analyses). The third study, conducted in the UK and published in 2004, included 185 eyes of 174 participants with full-thickness macular hole (41 eyes with stage II holes and 74 eyes with stage III/IV holes in analyses). Studies were of good quality for randomisation and allocation concealment, whereas visual acuity measurement was unmasked.At 6 to 12 months, visual acuity was improved by about 1.5 Snellen lines (-0.16 logMAR, 95% confidence intervals -0.23 to -0.09 logMAR, 270 eyes, moderate-quality evidence). The chances of macular hole closure at 6 to 12 months were greatly increased using vitrectomy, yielding an odds ratio of 31.4 (95% confidence intervals 14.9 to 66.3, 265 eyes, high-quality evidence; raw sum data: 76% vitrectomy, 11% observation). Vitrectomy was beneficial both in smaller (stage II) and in larger (stage III/IV) macular holes.The largest study reported that cataract surgery was needed in about half of cases at two years after operation and that retinal detachment occurred in about 5% of operated eyes. Vitrectomy is effective in improving visual acuity, resulting in a moderate visual gain, and in achieving hole closure in people with macular hole. However, these results may not apply to modern surgery due to technological improvements in vitrectomy techniques.

PurposeTo evaluate the outcome of pneumatic vitreolysis (PVL) for vitreomacular traction (VMT) with or without full thickness macular hole (MH) < 400 µm.MethodsForty-seven eyes of 47 patients were included who had undergone PVL for VMT with or without MH. Main outcome measures were release of VMT, MH closure, best-corrected visual acuity (BCVA) and adverse events.ResultsThirty-three patients had isolated VMT and 14 patients VMT with a MH. Four weeks after PVL, the overall VMT release rate was 35/47 (74.5%): 25/37 (67.6%) in phakic and 10/10 (100%) in pseudophakic eyes (p = 0.03). Four of 14 MH (28.6%) were closed. Twenty-two of 47 (46.8%) eyes required a subsequent PPV: 12/33 (36.4%) in the VMT only group and 10/14 (71.4%) in the VMT with MH group. Mean BCVA improved from 0.48 (± 0.24) to 0.34 (± 0.23) logMAR at 6 months in patients with VMT alone (p < 0.001), and from 0.57 (± 0.27) to 0.41 (± 0.28) logMAR in patients with VMT and MH (p = 0.008). Adverse events included new formation of a large MH in 4/33 (12.1%) eyes, failure of MH closure in 10/14 (71.4%) eyes, progression of mean minimum linear diameter (MLD) MH size from baseline 139 (± 67) to 396 (± 130) µm (p < 0.001) and development of a retinal detachment in 4/47 (8.5%) eyes.ConclusionWhile PVL leads to a high VMT release rate particularly in pseudophakic eyes, it is associated with a relatively high incidence of MH formation, MH size progression and retinal detachment.

The inverted flap (IF) technique has recently been introduced in macular hole (MH) surgery. The IF technique has shown an increase of the success rate in the case of large MHs and in MHs associated with high myopia. This study reports the anatomical and functional results in a large series of patients affected by MH treated using pars plana vitrectomy and gas tamponade combined with internal limiting membrane (ILM) peeling or IF. This is a retrospective, consecutive, nonrandomized comparative study of patients affected by idiopathic or myopic MH treated using small-gauge pars plana vitrectomy (25- or 23-gauge) between January 2011 and May 2016. The patients were divided into two groups according to the ILM removal technique (complete removal vs. IF). A subgroup analysis was performed according to the MH diameter (MH < 400 µm and MH ≥ 400 µm), axial length (AL < 26 mm and AL ≥ 26 mm), and the presence of chorioretinal atrophy in the macular area (present or absent). We included 620 eyes of 570 patients affected by an MH, 300 patients underwent pars plana vitrectomy and ILM peeling and 320 patients underwent pars plana vitrectomy and IF. Overall, 84.94% of the patients had complete anatomical success characterized by MH closure after the operation. In particular, among the patients who underwent only ILM peeling the closure rate was 78.75%; among the patients who underwent the IF technique, it was 91.93% (P = 0.001); and among the patients affected by full-thickness MH ≥400 µm, success was achieved in 95.6% of the cases in the IF group and in 78.6% in the ILM peeling group (P = 0.001); among the patients with an axial length ≥26 mm, success was achieved in 88.4% of the cases in the IF group and in 38.9% in the ILM peeling group (P = 0.001). Average preoperative best-corrected visual acuity was 0.77 (SD = 0.32) logarithm of the minimum angle of resolution (20/118 Snellen) in the peeling group and 0.74 (SD = 0.33) logarithm of the minimum angle of resolution (20/110 Snellen) in the IF group (P = 0.31). Mean postoperative best-corrected visual acuity was 0.52 (SD = 0.42) logarithm of the minimum angle of resolution (20/66 Snellen) in the peeling group and 0.43 (SD = 0.31) logarithm of the minimum angle of resolution (20/53 Snellen) in the IF group (P = 0.003). Vitrectomy associated with the inverted ILM flap technique seems to be effective surgery for idiopathic and myopic large MHs, improving both functional and anatomical outcomes.

Secondary macular hole(MH) formation after vitrectomy is rare and its risk factors and pathogenesis are not clearly understood. This retrospective study was conducted to identify the risk factors of this complication and assess outcomes at 2 tertiary centres. The primary outcomes were the clinical characteristics associated with development of secondary MH, which included the primary diagnosis for initial vitrectomy, features on optical coherence tomography, and adjuvant surgical techniques used during the initial surgery. Secondary outcomes included the change in best-corrected visual acuity(BCVA), clinical factors associated with the need for re-operations for MH closure and prognostic factors for the visual outcomes. Thirty-eight eyes out of 6,354 cases (incidence 0.60%) developed secondary MH after undergoing vitrectomy for various vitreoretinal disorders over an 11-year period, most frequently after initial surgery for retinal detachment(RD) (9 eyes) and secondary epiretinal membrane (6 eyes). The mean age was 57.1 years (range: 17.8–76.7), and the mean follow-up was 51.1 months (range: 6.8 to 137.6). Prior to secondary MH formation, development of ERM was the most common OCT feature (19 eyes, 50%), and no cases of cystoid macular oedema (CME) were observed. A greater proportion of eyes with secondary MH had long axial lengths (32% ≥26 mm vs 5% of eyes ≤22 mm). MH closure surgery was performed in 36 eyes and closure was achieved in 34 (success rate 94%, final BCVA 20/86), with ≥3-line visual gain in 18 cases. BCVA at MH onset (OR = 0.056, P = 0.036), BCVA at post-MH surgery month 3 (OR = 52.671, P = 0.011), and axial length ≥28 mm (OR = 28.487, P = 0.030) were associated with ≥3-line visual loss; a history of macula-off RD (OR = 27.158, P = 0.025) was associated with the need for multiple surgeries for MH closure. In conclusion, secondary MH occurs rarely but most commonly after vitrectomy for RD. Patients with axial length ≥28 mm and poor BCVA at 3 months post-operation may have limited visual prognosis; those with a history of macula-off RD may require multiple surgeries for hole closure.

This study evaluates the efficacy and usefulness of vitrectomy with internal limiting membrane peeling and autologous blood clot covering without gas tamponade in the treatment of macular holes (MHs). All patients with a full-thickness MH with a minimum diameter of <600 µm and a base diameter of <1,200 µm underwent pars plana vitrectomy and internal limiting membrane peeling with autologous blood covering the MH at the end of the surgery. No fluid-air exchange or gas tamponade was performed. Postoperatively, all patients were instructed to adopt supine position overnight and thereafter any comfortable posture. A total of 18 eyes of 18 consecutive patients were included. The mean age of the patients (12 women and 6 men) was 59.06 ± 14.31 years (range, 21-81 years). The MHs composed of 13 idiopathic MHs, 2 MHs with high myopia (axial length > 26.5 mm), 2 traumatic MHs, and 1 MH associated with diabetic macular edema. Among them were five large MHs (minimum diameter > 400 µm). Complete MH closure was achieved in all eyes at the end of the follow-up period (range, 3-14 months). Visual acuity was significantly improved from preoperative 0.89 ± 0.41 logarithm of the minimum angle of resolution (20/155 Snellen) to 0.42 ± 0.33 logarithm of the minimum angle of resolution (20/53 Snellen) at the final visit (P < 0.001). The novel surgical protocol using vitrectomy, internal limiting membrane peeling, and autologous blood clot covering at the end of the MH surgery with limited diameters achieved highly effective closure and visual improvement and eliminated the gas tamponade and thus the associated adverse effects and the need for postoperative face-down positioning.

PurposeTo observe the macular pigment (MP) appearances in eyes with macular hole (MH) and clarify the origin of the appearances. The mechanisms underlying the development of MH are discussed based on the observation of MP.MethodsThis observational case series included 33 eyes of 31 patients with MH who underwent vitrectomy. The MP optical density was measured using the two-wavelength fundus autofluorescence technique. The exact localization of MP was evaluated by comparing MP distribution images and optical coherent tomography B-scan images.ResultsMP was missing at the MH. The area of the MP defect corresponded with the area of the defect of outer plexiform layer. MP was present in the retinal flap in stage 2 MH that included glia (Müller cells) and plexiform layers and in the operculum in stage 3 MH, which mainly comprised Müller cells. Cystic spaces in the outer plexiform layer surrounding stage 3 and 4 MHs showed a honeycomb appearance on MP images. MP reappeared to form an irregularly shaped pigment plane after surgical closure of MH. The MP optical volume did not change before and after surgery. Fellow eyes with a central dip in MP distribution subsequently developed MH.ConclusionsThe characteristic appearances of MP at the MH were attributed to MP in the plexiform layers and Müller cell cones. A central dip of MP distribution might be a sign of Müller cell cone damage that proceeds with MH formation.Translational RelevanceObservation of MP was useful for understanding the mechanisms of MH formation.

We aim to review scientific literature concerning published studies on autologous retinal transplantation to treat macular hole patients. The following databases were searched: Medline and Medline Non-Indexed Items, Embase (1990–2020), Ovid Medline® (1990 to November 2020), Embase (1990 to November 2020), Ovid Medline® and Epub Ahead of Print, in-Process and Web of Science (all years). Search keywords included “autologous”, “retinal transplant”, “autologous neurosensory retinal free flap” “transplantation”, “macular hole”, and “macular hole surgery”. Eighteen case series and single case reports were reviewed. Preoperative and final best-corrected visual acuity, microperimetry and multifocal electroretinogram findings, macular hole closure rate, preoperative and postoperative ellipsoid zone, and external limiting membrane defects were obtained and analyzed. Indications of autologous retinal transplantation for macular hole surgery included refractory macular holes, conventional techniques, and large macular holes. The number of cases included in the different case series ranged from 2 to 130 cases, and prior macular hole surgeries of the patients ranged from 0 to 3. Overall, the published case series of autologous retinal transplants have reported a macular hole closure rate of 66.7 to 100%, as well as a significant improvement in best-corrected visual acuity. The most frequently reported complications included considerable intraoperative bleeding and postoperative dislocation of the graft. The presence of functionality in the graft area has also been documented by microperimetry and multifocal electroretinogram. In conclusion, the autologous retinal transplantation technique for macular hole patients has emerged as another surgical option, with a high macular hole closure rate and visual improvement.

PurposeWe analyzed the anatomical and visual outcomes after surgical treatment of idiopathic macular holes with pars plana vitrectomy, internal limiting membrane (ILM) peeling using Brilliant Blue dye, and silicone oil tamponade without postoperative posturing.MethodsThis was a retrospective interventional study of 10 eyes in eight patients who underwent surgical treatment of idiopathic macular holes using pars plana vitrectomy, ILM peeling using Brilliant Blue dye, and silicone oil tamponade without postoperative posturing. The preoperative staging of macular holes and postoperative anatomic outcomes were assessed using spectral-domain optical coherence tomography.ResultsAll patients were women with a mean age of 66.86 ± 4.8 years. In two patients, bilateral macular holes were present and both eyes were operated on. Stage 2 macular hole was diagnosed in three eyes, three eyes had stage 3, and four eyes had stage 4 macular holes. Anatomical success and closure of the macular hole was achieved in nine eyes (90%) after one operation. In one eye, the macular hole was closed after reoperation. The preoperative mean best-corrected visual acuity (BCVA) was 0.15 decimal units (0.8 logMAR units). Until the end of the follow-up period, BCVA was 0.25 decimal units (0.6 logMAR units). Visual acuity was improved in seven patients (70%). In two patients (20%), visual acuity remained at the same level, and in one eye (10%), visual acuity decreased. Postoperatively, all patients reported a significant reduction of metamorphopsia.ConclusionInitial results after 20G pars plana vitrectomy with peeling of the ILM, use of dye (Brilliant Blue), and tamponade with silicone oil without postoperative posturing gave good anatomical and functional outcome in terms of visual acuity and reduction of metamorphopsia. Taking into account the age of the patients, this method, which does not require prolonged postoperative face-down posturing, was well tolerated by the patients. Because the anatomical and visual outcome as well as the rate of postoperative complications are comparable to those when gas is used as a tamponading agent, silicone oil tamponade can also be safely used as a first option in surgery of macular holes. However, a longer period of follow-up of the operated eyes, as well as a larger group of operated eyes, will be required to identify long-term outcomes of this surgical treatment.

The reattachment rate, macular hole (MH) closure rate, visual acuity improvement and redetachment rate of MH retinal detachment (MHRD) of high myopia are not satisfactory owing to long axis oculi, posterior scleral staphyloma and macular atrophy. At present, minimally invasive vitrectomy surgery combined with the internal limiting membrane flap technique has become popular in the treatment of MHRD, as it can promote MH closure, and significantly improve the outcome of MHRD. However if this method can improve the postoperative visual function is still controversial. The advantage of this technique is that the loosened internal limiting membrane is applied to cover the MH surface to form a scaffold structure similar to the basement membrane. It can stimulate Muller cell gliosis more effectively, and promote tissue filling in the MH which results in MH closure. It can also promote retinal reattachment and reduce the likelihood of retinal redetachment. This technique is expected to be a standard surgical method for the treatment of MHRD of high myopia in the future. The inserted internal limiting membrane flap technique is relatively easy to perform, induces stable flaps by simple procedures, and can be an essential complement procedure of the inverted internal limiting membrane flap technique. In order to reduce the recurrence rate in the future, it is necessary to further define the indications of different surgical methods and the predictive effects of MH healing mode on the success rate and visual function recovery. Key words: Myopia, degenerative/complications; Retinal perforations/surgery; Retinal detachment/ surgery; Vitreoretinal surgery; Epiretinal membrane; Editorial

Terson syndrome refers to intraocular haemorrhage that occurs due to subarachnoid bleeding associated with an acute increase in intracranial pressure. No previous study has reported a delayed macular hole (MH) secondary to Terson syndrome. A 17-year-old boy visited our department and presented with vitreous bleeding and a history of subarachnoid haemorrhage. Sub-internal limiting membrane (ILM) haemorrhage with ILM detachment and intraretinal haemorrhage were detected during pars plana vitrectomy. Additionally, a delayed MH was detected 1 week after the surgery. There was no sign of MH closure during a 2-month follow-up. Subsequently, an MH massage was performed to close the MH. Our findings suggest that a delayed MH can occur secondary to Terson syndrome. Elevated hydrodynamic pressure and hydrostatic pressure, which are caused by sub-ILM and intraretinal haemorrhages of the fovea, contribute to the formation of an MH. Additionally, ILM peeling may cause damage to the macula and facilitate the formation of MHs. Although the MH may close by itself, early surgical intervention is recommended when there is no sign that the MH will close spontaneously because a prolonged MH can lead to retinal damage.

Chin, Eric K. MD; Almeida, David R.P. MD, MBA, PhD; Sohn, Elliott H. MD Author Information