Long-term anatomical and functional outcomes of surgical treatment of retinal complications in children and adolescents with Stickler syndrome between 2004 and 2021.

BACKGROUND: Stickler syndrome (STL) is an inherited progressive connective tissue collagen disorder. STL is the most common hereditary cause of retinal complications, retinal tears, and the development of retinal detachment (RD) in childhood. The aim of the study was to evaluate the long-term anatomical and functional results of surgical treatment of retinal complications in children and adolescents affected by STL. METHODS: A retrospective, single-center study was performed a cohort of children with STL who underwent retinal surgery between 2004 and 2021. RESULTS: The study group consisted of nine children; the mean age at the time of the retinal tear with/without retinal detachment was 7.2 (2-10) years, and the mean follow-up period was 9.6 (5-16) years. Pathogenic variants COL2A1 (5 children) and COL11A1 (3 children) were confirmed in our cohort. In total, we operated on 13 eyes, 11 eyes with complicated RD and two eyes with multiple retinal defects, but without RD. At the end of the follow-up period, an attached retina was achieved 77% (10 eyes) with or without silicone oil tamponade: cryopexy alone was successful in one eye (10%), scleral buckling (EB) in five eyes (50%), and vitrectomy with silicone oil tamponade combined with EB in four eyes (40%). The mean number of surgeries was 2.3 per eye. The resulting best corrected visual acuity ranged from 0.03 to 0.1 in one eye, from 0.16 to 0.4 in two eyes, and from 0.5 to 1.0 in 7 eyes. CONCLUSION: Repair of retinal tears with/without retinal detachment in patients with Stickler syndrome often requires multiple surgeries with combinations of cryopexy, scleral buckling, and/or vitrectomy with silicone oil tamponade. Treatment of the ocular complications arising from STL requires long-term comprehensive care.

Combined pulses of light and sound in the retina with nutraceuticals may enhance the recovery of foveal holes.

The present manuscript stems from evidence, which indicates that specific wavelength produce an activation of the autophagy pathway in the retina. These effects were recently reported to synergize with the autophagy-inducing properties of specific phytochemicals. The combined administration of photo-modulation and phytochemicals was recently shown to have a strong potential in eliciting the recovery in the course of retinal degeneration and it was suggested as a non-invasive approach named "Lugano protocol" to treat age-related macular degeneration (AMD). Recent translational findings indicate that the protective role of autophagy may extend also to acute neuronal injuries including traumatic neuronal damage. At the same time, very recent investigations indicate that autophagy activation and retinal anatomical recovery may benefit from sound exposure. Therefore, in the present study, the anatomical rescue of a traumatic neuronal loss at macular level was investigated in a patient with idiopathic macular hole by using a combined approach of physical and chemical non-invasive treatments. In detail, light exposure was administered in combination with sound pulses to the affected retina. This treatment was supplemented by phytochemicals known to act as autophagy inducers, which were administered orally for 6 months. This combined administration of light and sound with nutraceuticals reported here as Advanced Lugano's Protocol (ALP) produced a remarkable effect in the anatomical architecture of the retina affected by the macular hole. The anatomical recovery was almost complete at roughly one year after diagnosis and beginning of treatment. The structural healing of the macular hole was concomitant with a strong improvement of visual acuity and the disappearance of metamorphopsia. The present findings are discussed in the light of a synergism shown at neuronal level between light and sound in the presence of phytochemicals to stimulate autophagy and promote proliferation and neuronal differentiation of retinal stem cells.

Persistent macular holes - what is the best strategy for revision?

PURPOSE: This study aims to analyze the success rate and functional outcome after revision surgery of persistent idiopathic full-thickness macular holes in a large patient cohort and to identify the optimal tamponade strategy and the value of new adjunctive manipulation techniques for persistent macular hole (pMH) closure. METHODS: Retrospective, comparative, non-consecutive case series of all revisional surgeries for idiopathic pMH between 2011 and 2019 at the Eye Clinic Sulzbach were identified. Of 1163 idiopathic MH surgeries, 74 eyes of 74 patients had pMH. Of those, group 1 (n = 38) had vitrectomy with tamponade alone (20% sulfur hexafluoride gas, 15% hexafluoroethane gas, silicone oil 5000, Densiron®), while group 2 (n = 36) included tamponade with adjuvant manipulation (internal limiting membrane (ILM) translocation, subretinal fluid injection, epiretinal amniotic membrane, free retina graft, or autologous blood). Main statistical outcomes were anatomic closure rate, visual acuity (VA), minimum linear diameter (MLD), and base diameter (BD). RESULTS: Overall total anatomical success rate was 81.1% and mean VA improved 3.5 lines from LogMAR 1.03 ± 0.30 to 0.68 ± 0.38 (p < .001). Preoperative MLD or BD had no effect on total anatomic success (p = 0.074, p = 0.134, respectively). When comparing the two groups, slightly better anatomic success rates were achieved in group 1 (84.2%) compared to that in group 2 (77.8%) (p = 0.68). Final VA in group 1 (LogMAR 0.67 ± 0.39) outperformed group 2 (LogMAR 0.86 ± 0.38) (p = 0.03). CONCLUSIONS: Revisional surgery for persistent idiopathic MH with tamponade alone had comparable anatomical closure but better VA outcomes, compared to tamponade with adjuvant manipulation.

Anatomical and functional outcomes one year after vitrectomy and retinal massage for large macular holes.

Purpose: The aim of this study was to report results of macular hole closure, visual benefit and longitudinal changes in foveal architecture over 1 year following macular hole surgery with retinal massage. Methods: Records of patients with full thickness macular hole (FTMH) with minimum basal diameter of 550 µ who underwent vitrectomy, internal limiting membrane (ILM) peeling and retinal massage were drawn up. Retinal massage was performed after dye assisted ILM peeling, using a 27G flute needle with a long and soft silicone tip under air in a centripetal direction around the FTMH. At the end, 10% C3F8 gas was used as tamponade. The foveal contour at 1 year follow up was recorded based on its cross sectional appearance on OCT and was classified into U, V and irregular types of closure as previously described. Results: Forty-one eyes of 41 patients with a mean age of 70.4 ± 6.9 years were included. The mean preoperative vision was 0.99 ± 0.07 logMAR and mean maximum basal diameter of the FTMH was 835 ± 208 µ. Macular hole closure was seen in all patients at 1-month follow up. The BCVA improved to 0.5 ± 0.1 log MAR at 6 months (P < 0.001) and then stabilized. The U shaped closure was the commonest pattern (n = 22, 54%), followed by V-shaped closure (n = 16, 39%) while irregular closure was seen in 3 eyes (7%). Conclusion: Retinal massage after ILM peeling is a very simple, and as yet under-utilized manoeuvre that may improve hole closure rates and lead to potentially greater improvement in vision compared to existing techniques for large holes.

Surgical outcomes of macular hole and epimacular membrane treatment in patients with intraoperative amaurosis under sub-Tenon's anesthesia.

OBJECTIVE: Some patients have been found to develop intraoperative amaurosis under sub-Tenon's anesthesia. We explored whether these patients have poor surgical outcomes during mid- to long-term postoperative follow-up. METHODS: In this case series, 74 of 85 patients with macular diseases who underwent phacoemulsification combined with vitrectomy under sub-Tenon's anesthesia developed intraoperative amaurosis. The surgical outcomes at the 2- and 4-month follow-ups in these patients were investigated and compared with the outcomes in patients without amaurosis using best-corrected visual acuity (BCVA), optical coherence tomography (OCT), and pattern visual evoked potential (PVEP). RESULTS: Both BCVA and the OCT-based macular structure in patients with intraoperative amaurosis showed significant postoperative improvement comparable with that of patients without amaurosis. The presence of intraoperative amaurosis was not associated with either macular hole closure or macular edema regression. PVEP revealed no significant changes in the wave latency or amplitude before and after surgery. CONCLUSION: Intraoperative amaurosis following sub-Tenon's block is commonly seen but does not predict a poor surgical prognosis. When a patient develops amaurosis during surgery, the surgeon should increase patient comfort through verbal communication rather than perform an additional intervention to help relieve the patient's anxiety.

Comparing the inverted internal limiting membrane flap with autologous blood technique to internal limiting membrane insertion for the repair of refractory macular hole.

PURPOSE: To compare the anatomical and functional outcomes of the inverted internal limiting membrane (ILM) flap technique and the autologous blood clot (ABC) to ILM insertion technique for the repair of refractory macular hole (RMH). METHODS: Fifty-two consecutive patients (52 eyes) diagnosed with RMH with a minimum diameter more than 700 µm were enrolled. All patients underwent vitrectomy and gas tamponade. Of these, 25 patients undergo inverted ILM flap combination with ABC (Group A). The remaining 27 patients underwent ILM insertion (Group B). The main outcome measures were macular hole (MH) closure, foveal configuration, logarithm of the minimum angle of resolution (logMAR), best-corrected visual acuity (BCVA), superficial foveal avascular zone (FAZ) and superficial parafoveal vessel density. RESULTS: Three months post-operation, MH was successfully anatomic closed in 24 of 25 eyes (96%) in Group A and 25 of 27 eyes (92.5%) in Group B (P = 0.599). A concave foveal configuration of MH closure was observed in 23 of 25 eyes (92%) in Group A and 2 of 27 eyes (7.4%) in Group B (P < 0.001). Mean BCVA (logMAR) had improved from 1.31 ± 0.61 to 0.68 ± 0.40 in Group A (P < 0.001) and from 1.34 ± 0.39 to 1.29 ± 0.62 in Group B (P = 0.584) at 3 months. Average superficial FAZ area and superficial parafoveal vessel density were 0.29 ± 0.08 mm2 and 51.41 ± 2.79% in Group A and 0.73 ± 0.15 mm2 and 43.77 ± 2.71% in Group B, respectively. There was a significant difference in both the average superficial FAZ area and parafoveal vessel density between Groups A and B (P < 0.001 for both). CONCLUSION: Anatomical foveal configuration, mean BCVA (logMAR), mean superficial FAZ and parafoveal vessel density outcomes for the inverted ILM flap combined with ABC approach were better than the outcomes obtained with ILM insertion in the treatment of RMH. This approach may promote better long-term, vision function outcomes for patients diagnosed with RMH.

Incidence and impact factors of intraoperative loss of light perception under sub-Tenon's anesthesia in patients with macular diseases.

PURPOSE: To investigate the incidence and impact factors of intraoperative loss of light perception (LP) under sub-Tenon's anesthesia in patients with macular diseases. METHODS: Eighty-five consecutive patients received standard phacoemulsification combined pars plana vitrectomy (PPV) under sub-Tenon's anesthesia. At several checkpoints during the surgery (the end of phacoemulsification, the end of vitrectomy, and the end of surgery), participants were interviewed about whether they had LP or not after removing the influence of contralateral eye and the photo-bleaching effect. In patients treated with retinal photocoagulation, visual experience on laser flashes was evaluated. RESULTS: Under routine draping, no patients reported loss of LP at all the checkpoints. When the contralateral eye was tightly covered, the rates of LP loss were 84.7%, 97.6%, and 87.1% at the end of phacoemulsification, the end of vitrectomy, and the end of surgery, respectively. When the photo-bleaching effect was also removed, the rates of LP loss were 61.2%, 82.4%, and 56.5% at each checkpoint, respectively, and there were 87.1% (74/85) of patients reporting visual loss in at least one checkpoint. In addition, 76.9% (50/65) of patients could not feel laser flashes during retinal photocoagulation. CONCLUSION: Intraoperative loss of LP under sub-Tenon's anesthesia was a relatively common and reversible event. The conduction block of optic nerve by anesthetic mainly contributed to the visual loss during surgery. Photo-bleaching effect also has some effect on the LP evaluation. Surgeons need to inform and counsel the patients about the intraoperative loss of LP, to prevent any sudden panic attacks in them.

Five-year nationwide incidence of macular hole requiring surgery in Korea.

BACKGROUND/AIMS: To estimate the incidence and demographics of macular hole (MH) requiring surgery in Korea. METHODS: Patients who underwent surgery for MH in Korea from 2011 to 2015 with the diagnostic code for MH and the surgical code for vitrectomy were retrospectively identified using the Korean national health claims database. The average incidence rate of MH during the 5-year study period was estimated by applying the direct method of standardisation using the 2015 census data as a reference population. RESULTS: A total of 7301 patients with MH requiring surgery were identified. The average incidence of MH requiring surgery was 3.14 (95 % CI, 3.07 to 3.21) per 100 000 person-years . The incidence in women (4.29 per 100 000 person-years; 95% CI, 4.17 to 4.40) was significantly higher than that in men (2.00 per 100 000 person-years; 95% CI, 1.92 to 2.07; p<0.001). The incidence rate of MH increased exponentially with increasing age between the ages of 35 years and 69 years and was highest among patients aged 65-69 years. The female-to-male ratio for the incidence of MH was 2.15:1. CONCLUSIONS: This study represents the largest nationwide population-based investigation of the incidence of MH, using a database that covers the entire population of South Korea. MH is the disease of elderly peaked 70 years old and more common in women.

Vitrectomy with silicone oil tamponade for retinal detachment associated with giant retinal tears: Favourable outcomes without adjuvant scleral buckling.

PURPOSE: To evaluate the outcomes of pars plana vitrectomy with silicone oil tamponade in the management of retinal detachment associated with giant retinal tears. METHODS: We reviewed 45 eyes of 42 patients with primary retinal detachment associated with giant retinal tears over 10 years at a tertiary referral centre. Patients underwent 23-gauge vitrectomy without adjuvant scleral buckling by a single surgeon and had follow-up at least 6 months after silicone oil removal. RESULTS: Mean follow-up was 37 ± 35 months. Seven eyes (16%) had grade C proliferative vitreoretinopathy, and 16 (36%) had a giant retinal tear ≥180° at baseline. The primary reattachment rate was 84%, and the overall final anatomical success rate was 98%. The mean Snellen visual acuity equivalent at the final visit was 20/58. Final visual acuity ≥20/40 was achieved in 64%. The mean duration of silicone oil tamponade was 10.5 ± 4 weeks. By the final visit, silicone oil had been removed from 44 eyes (98%). CONCLUSION: The high rates of anatomical and functional success support management of giant retinal tears-associated retinal detachment with vitrectomy without adjuvant scleral buckling. Removal of silicone oil at the earliest possible time helps to avoid complications such as keratopathy, glaucoma and visual loss without apparent reason.

AUTOLOGOUS LENS CAPSULAR FLAP TRANSPLANTATION COMBINED WITH AUTOLOGOUS BLOOD APPLICATION IN THE MANAGEMENT OF REFRACTORY MACULAR HOLE.

PURPOSE: To report results of lens capsular flap transplantation (LCFT) and autologous whole blood application in refractory macular hole (MH) treatment. METHODS: Seven phakic and three aphakic eyes with persistent MH after standard surgery with internal limiting membrane peeling were studied. Lens capsule flap was acquired from the same eye in eight cases (seven phakic and one aphakic). The fellow eye was used in two aphakic eyes without sufficient lens capsule. The fellow eye underwent simultaneous phacoemulsification. All eyes underwent complete vitrectomy, LCFT into the MH, whole blood application, and 15% perfluoropropane (C3F8) tamponade. The patients were instructed to maintain a face-down or prone position for two weeks postoperatively. Structural and functional changes were evaluated. RESULTS: The mean preoperative MH diameter was 1,472.78 ± 736.88 µm. The MH was completely closed in nine eyes: eight eyes receiving same-eye LCFT and one receiving fellow-eye LCFT. In the other fellow-eye LCFT recipient, the MH was partially closed. Visual acuity improved from 1.84 ± 0.49 logarithm of the minimum angle of resolution (median Snellen acuity: 20/1,750, range: 20/4,000-20/125) preoperatively to 1.34 ± 0.59 logarithm of the minimum angle of resolution (median Snellen acuity: 20/450, range: 20/4,000-20/63) postoperatively (P = 0.009). CONCLUSION: Lens capsular flap transplantation and autologous whole blood application may improve anatomical and visual outcomes in refractory MH cases. The lens equator and fellow eye may be promising sources of LCF.

Strategy for the management of complex retinal detachments: the European vitreo-retinal society retinal detachment study report 2.

OBJECTIVE: To study the outcome of the treatment of complex rhegmatogenous retinal detachments (RRDs). DESIGN: Nonrandomized, multicenter, retrospective study. PARTICIPANTS: One hundred seventy-six surgeons from 48 countries spanning 5 continents reported primary procedures for 7678 RRDs. METHODS: Reported data included clinical manifestations, the method of repair, and the outcome. MAIN OUTCOME MEASURES: Failure of retinal detachment repair (level 1 failure rate), remaining silicone oil at the study's conclusion (level 2 failure rate), and need for additional procedures to repair the detachments (level 3 failure rate). RESULTS: The main categories of complex retinal detachments evaluated in this investigation were: (1) grade B proliferative vitreoretinopathy (PVR; n = 917), (2) grade C-1 PVR (n = 637), (3) choroidal detachment or significant hypotony (n = 578), (4) large or giant retinal tears (n = 1167), and (5) macular holes (n = 153). In grade B PVR, the level 1 failure rate was higher when treated with a scleral buckle alone versus vitrectomy (P = 0.0017). In grade C-1 PVR, there was no statistically significant difference in the level 1 failure rate between those treated with vitrectomy, with or without scleral buckle, and those treated with scleral buckle alone (P = 0.7). Vitrectomy with a supplemental buckle had an increased failure rate compared with those who did not receive a buckle (P = 0.007). There was no statistically significant difference in level 1 failure rate between tamponade with gas versus silicone oil in patients with grade B or C-1 PVR. Cases with choroidal detachment or hypotony treated with vitrectomy had a significantly lower failure rate versus treatment with scleral buckle alone (P = 0.0015). Large or giant retinal tears treated with vitrectomy also had a significantly lower failure rate versus treatment with scleral buckle (P = 7×10(-8)). CONCLUSIONS: In patients with retinal detachment, when choroidal detachment, hypotony, a large tear, or a giant tear is present, vitrectomy is the procedure of choice. In retinal detachments with PVR, tamponade with either gas or silicone oil can be considered. If a vitrectomy is to be performed, these data suggest that a supplemental buckle may not be helpful. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Subconjunctival lidocaine before laser treatment: a randomized trial.

PURPOSE: The aim of the study was to determine whether subconjunctival anesthesia is effective at reducing pain associated with laser retinopexy. DESIGN: This was a single-center, prospective, patient-masked, randomized, controlled trial. PARTICIPANTS: In the primary study group, 65 patients were recruited. Thirty-two patients (32 eyes) received anesthetic injection, and 33 patients (33 eyes) received the sham injection. The crossover study group consisted of 28 patients (56 eyes). METHODS: Between February 2008 and April 2008, all patients who were consecutively booked to undergo panretinal photocoagulation (PRP) or peripheral laser retinopexy (PLR) were invited to participate in the study. Patients were randomized to receive subconjunctival lidocaine injection or a sham injection before their intended laser retinal treatment. These patients were defined as our primary study group. Patients who required a second laser treatment received the opposite injection in a masked fashion before laser retinal treatment. These patients were defined as our crossover group. Patients in both the primary study group and the crossover group were masked to the treatment given. After the laser treatment, patients completed a pain questionnaire. MAIN OUTCOME MEASURES: The primary outcome was incidence of pain. The secondary outcome was severity of pain. RESULTS: Primary study group: In the anesthetic treatment group, 19 patients (59%) experienced pain compared with 32 patients (97%) in the sham treatment group (P<0.001). Among the patients who did experience pain, the average pain score was 3.6 in the anesthetic treatment group and 4.1 in the sham treatment group (P=0.55). Crossover study group: In the anesthetic treatment group, 12 patients (43%) experienced pain compared with 28 patients (100%) in the sham treatment group (P<0.001). In patients who did experience pain, the average pain score was 3.3 in the anesthetic treatment group and 4.6 in the sham treatment group. Twenty patients (71%) preferred anesthetic to sham treatments, 1 patient (4%) preferred sham over anesthetic, and 7 patients (25%) thought both laser treatments were the same. CONCLUSIONS: Subconjunctival 2% lidocaine significantly reduces the incidence of pain in patients who receive PRP or PLR.

The use of silicone oil-RMN3 (Oxane HD) as heavier-than-water internal tamponade in complicated inferior retinal detachment surgery.

BACKGROUND: Conventional silicone oil provides suboptimal support of the inferior retina. In this study we evaluated the efficacy of Oxane HD in the management of complex retinal detachments involving lower quadrants of the retina. METHODS: A prospective, interventional, comparative study. Eighteen patients were recruited. Treatment outcomes were compared with a historical control group of 14 patients. Patients with grade C3 PVR or greater and inferior retinal breaks, recurrent inferior retinal detachments (with or without PVR) and giant retinal tears were included. In those patients who re-detached under heavy silicone oil (n = 4), retro-oil epiretinal membranes (ERMs) were obtained at the time of subsequent surgery to analyse the immunopathological response to oxane HD. Immunohistochemistry was used to detect glia, retinal pigment epithelium cells (RPE), macrophages, T lymphocytes, or neural elements in the tissue using well-characterised monoclonal antibodies. RESULTS: Retinal attachment of the posterior pole following removal of silicone oil was achieved in 66.6% of the treatment group (n = 12) and 64.3% of controls (n = 9) (p = 1.0). Post-operative PVR developed in five patients in the treatment group (27.8%) and five control patients (35.8%). Following removal of silicone oil, residual oil was observed in 27.8% of the treatment group and 7.1% of controls. Median visual acuity, 3 months following removal of silicone oil, was 2.0 (IQR 0.9-2.0) in the treatment group and 1.0 (IQR 0.6-1.8) in the control group. Complications in the treatment group included, hypotony (n = 3), uveitis (n = 2), glaucoma (n = 1). All ERMs analysed demonstrated microscopic appearances typical of PVR. The membranes were fibrocellular in nature, contained RPE and glial cells, and variable amounts of intracellular and extracellular pigment. In addition, all had a dense infiltrate of vacuolated (presumed oil-filled) macrophages. CONCLUSION: We failed to observe an advantage following the use of Oxane HD in the treatment of inferior retinal detachments. Moreover, Oxane HD was difficult to remove and was associated with a higher incidence of complications.

Use of autologous whole blood during internal limiting membrane peeling and macular hole surgery is protective for indocyanine green toxicity.

OBJECTIVE: To compare indocyanine green (ICG)-assisted internal limiting membrane (ILM) peeling with and without autologous whole blood (WB) protection during macular hole repair surgery. MATERIAL AND METHODS: We retrospectively reviewed 50 eyes with an idiopathic macular hole. Group 1 contained 22 eyes that underwent ILM peeling with WB protection and group 2 contained 28 eyes that underwent ILM peeling without WB. Anatomic and visual results were compared. RESULTS: After surgery, macular hole closure was achieved in 96% of the group 1 eyes and in 93% of the group 2 eyes. The preoperative mean best-corrected visual acuity (BCVA) was logarithm of the minimum angle of resolution (logMAR) 0.95 in group 1 and logMAR 0.89 in group 2 (p = .544). The postoperative mean BCVA was logMAR 0.57 in group 1 and logMAR 0.92 in group 2 (p = .017). CONCLUSION: Macular hole surgery protected with WB facilitates improved visual recovery in ICG-assisted ILM peeling.