Quantitative assessment of intervortex anastomosis in central serous chorioretinopathy and fellow eyes: Does the size of anastomotic vessels matter for the diagnosis?

PURPOSE: To evaluate the frequency and size of intervortex anastomosis at the posterior pole on en-face spectral domain optical coherence tomography (SD-OCT) images in central serous chorioretinopathy (CSC) cases and their fellow eyes and its associations with choroidal morphology. METHODS: Sixty-five treatment-naive eyes of 65 patients with CSC, 65 fellow eyes, and 55 eyes of healthy age-matched participants were included. The presence of intervortex anastomosis at the watershed zone and asymmetry of the choroidal vessels between the superior and inferior macula were evaluated using 6 × 6 mm en-face SD-OCT. The diameter of the widest Haller vessel and the diameter of the widest anastomotic Haller vessel passing through the watershed zone were measured on en-face SD-OCT images. The choroidal vascularity index (CVI) was assessed using ImageJ software. RESULTS: Intervortex vein anastomosis on the horizontal watershed zone was detected in 75.4% diseased eyes, 61.5% in fellow eyes, and 36.4% in healthy age-matched controls (p < 0.001). The mean CVI was significantly higher in both diseased (74.3 ± 2.3%) and fellow (73.8 ± 2.2%) eyes of CSC cases than in healthy controls (72.5 ± 2.3%) (p = 0.002, p = 0.013, respectively). In the cases with intervortex vein anastomosis, the diameter of the widest anastomotic Haller vessel passing through the watershed zone was 0.40 ± 0.10 mm in diseased eyes, 0.35 ± 0.11 mm in fellow eyes, and 0.30 ± 0.09 mm in healthy age-matched controls (p = 0.001). CONCLUSIONS: Intervortex anastomosis might be seen as a variation in normal eyes, however, its frequency and the size of anastomotic vessels are significant higher in not only CSC but also in fellow eyes.

Outcomes after giant peripheral retinotomy and anterior flap retinectomy for rhegmatogenous retinal detachments with advanced proliferative vitreoretinopathy using small gauge vitrectomy.

PURPOSE: To analyze the visual and anatomical outcomes for eyes with rhegmatogenous retinal detachment (RRD) and advanced proliferative vitreoretinopathy (PVR) undergoing giant peripheral retinotomy (GPR) using 25-gauge pars plana vitrectomy (PPV). METHODS: In this retrospective multi-center study, patients with RRD with either anteroposterior or circumferential retinal shortening and advanced PVR requiring more than 90-degree GPR with/without relaxing retinotomy were included. Subjects of either gender, any age group, and with complete surgical notes were included. Outcome measures of the study included anatomical success (i.e. complete retinal re-attachment) at 6 months using survival analysis, visual outcomes, and post-operative complications. RESULTS: Forty-one eyes of 41 patients (33 males) with a mean age of 44.9 ± 21.4 years were included. At 6 months follow-up, anatomical success was seen in 29 eyes (70.7%) with a cumulative re-attachment rate of 66% (95% confidence interval = 48 = 79%). All re-detachments occurred at ≤6 months with a peak at 4-6 months (n = 9). Twenty-three eyes (56%) achieved ambulatory vision (5/200) or better. Direct perfluorocarbon liquid-silicone oil exchange was performed in 20 eyes. Intra-operative complications included persistent retinal folds (2 eyes), subretinal air (1 eye), and subretinal bleed (1 eye). Eleven eyes (26.8%) developed secondary glaucoma (2 eyes required a drainage device), and hypotony of ≤6 mmHg was noted in 3 eyes (7.3%). Corneal decompensation was noted in 8 eyes (19.5%), and 3 eyes (7.3%) underwent re-surgery for re-RRD. CONCLUSION: After GPR using small gauge PPV, two-thirds achieve anatomical success, and over half have ambulatory vision, but overall post-operative complications can occur in more than half of the eyes.

Navigated yellow subthreshold micropulse laser for refractory cystoid macular edema following rhegmatogenous retinal detachment surgery.

Purpose: To report the efficacy and safety of navigated 577nm yellow subthreshold micropulse laser (YSML) treatment in a case of refractory cystoid macular edema (CME) following combined phaco-vitrectomy for rhegmatogenous retinal detachment (RRD). Observations: A 69-year-old male patient complained a slow and progressive visual loss in the right eye (RE) since two months. A complete ophthalmological evaluation was performed. Best corrected visual acuity (BCVA) was hand motion and slit lamp examination revealed a nuclear cataract and a total macula-off RRD in the RE. Patient underwent a combined phaco +25 gauge pars plana vitrectomy (PPV) with 5000 cSt silicon oil (SO) tamponade. At the 3-month follow up BCVA was 20/250, retina was completely flat but a macular proliferative vitreoretinopathy (PVR) was detected with swept source optical coherence tomography (SS-OCT) and a second 23 G PPV with PVR peeling and SO removal was performed. At 1 month visit from the second surgery retina was flat and BCVA was 20/200 due to a persistent CME. Oral carbonic anhydrase inhibitors and topical steroids were administered for 2 months without any improvements. At this point, YSML was applied with a macular grid pattern and at three months follow up visit SS-OCT showed a complete resolution of CME, BCVA was 20/100 and these anatomical and functional outcomes were maintained at 6 months follow-up. Conclusions and importance: YSML treatment may be considered a safe and effective treatment strategy for the management of refractory CME following complex RRD surgery cases.

POLYPOIDAL LESIONS ASSOCIATED WITH CHOROIDAL NEVI.

PURPOSE: To investigate eyes with polypoidal lesions associated with choroidal nevi, their multimodal imaging characteristics, and long clinical follow-up. METHODS: Multicenter, retrospective case series study of patients with polypoidal lesions overlying choroidal nevi. Demographic and clinical information were recorded. Multimodal imaging including color fundus photography, optical coherence tomography, optical coherence tomography angiography, fundus fluorescein angiography, indocyanine angiography, and A- and B-scan ultrasonography were analyzed for nevus and polypoidal lesion characteristics. RESULTS: Fourteen eyes (14 patients; mean age: 70.3 ± 6.7 years) with polypoidal lesions overlying choroidal nevi were included. The mean follow-up duration was 50.0 ± 27.9 months (range 12-108). All nevi were pigmented on color fundus photography, flat on ultrasonography with a mean basal diameter of 3.8 ± 0.4 mm. In all but one eye, optical coherence tomography showed a shallow irregular pigment epithelium detachment overlying the nevus. A total of 11/14 eyes (78.6%) had exudative activity, 9 eyes received intravitreal anti-vascular endothelial growth factor injections, and one eye required intravitreal anti-vascular endothelial growth factor combined with photodynamic therapy. Mean visual acuity was 20/32 at baseline and 20/50 at final visit. CONCLUSION: We present the largest known cohort of eyes with polypoidal lesions associated with choroidal nevi with up to 9 years follow-up. The exudative degree of the polypoidal lesion in this condition is variable and treatment decisions should be taken on an individual basis. We hypothesize that choroidal ischemia because of altered choroidal vasculature rather than Haller layer hyperpermeability plays a role in the formation of polypoidal lesions overlying nevi.

Exudative Progression of Treatment-Naïve Nonexudative Macular Neovascularization in Age-Related Macular Degeneration: A Systematic Review With Meta-Analyses.

PURPOSE: To systematically review and report the rate of exudative progression over time in patients with nonexudative macular neovascularization (MNV) in age-related macular degeneration (AMD). DESIGN: Systematic review with prevalence meta-analyses and individual participant meta-analysis. METHODS: We searched 10 literature databases on March 26, 2023, for studies of consecutive patients with treatment-naïve nonexudative MNV in AMD. The primary outcome of interest was time from diagnosis to exudative progression. We conducted meta-analyses on the prevalence of exudative progression at 1 and 2 years. Where possible, we extracted individual participant data from studies and conducted an individual participant meta-analysis and explored the exudative progression using a time-to-event curve. RESULTS: We identified 16 eligible studies with a total of 384 eyes with nonexudative MNV. Exudative progression had occurred in 20.9% (95% CI 13.1%-29.8%) of eyes at 1 year and in 30.7% (95% CI 21.8%-40.4%) at 2 years. Similar results were observed in the individual participant meta-analysis, showing exudative progression in 18.9% (95% CI 13.5%-26.3%) of eyes at 1 year and 31.3% (95% CI 24.2%-40.0%) at 2 years. Risk factors for a fast exudative progression were the presence of subretinal lipid globules, large MNV areas, rapid MNV growth, growth in pigment epithelium detachment height and width, appearance of a branching pattern, and development of a hyporeflective halo around the MNV. CONCLUSIONS: Nonexudative MNVs in AMD are at high risk of exudative progression. Recognition of these lesions may allow for better individualized follow-up regimens in which closer monitoring may facilitate earlier diagnosis of exudative progression.

An Italian case series' description of thiamine responsive megaloblastic anemia syndrome: importance of early diagnosis and treatment.

BACKGROUND: Individuals with thiamine-responsive megaloblastic anemia (TRMA) mainly manifest macrocytic anemia, sensorineural deafness, ocular complications, and nonautoimmune diabetes. Macrocytic anemia and diabetes may be responsive to high-dosage thiamine treatment, in contrast to sensorineural deafness. Little is known about the efficacy of thiamine treatment on ocular manifestations. CASES PRESENTATION: Our objective is to report data from four Italian TRMA patients: in Cases 1, 2 and 3, the diagnosis of TRMA was made at 9, 14 and 27 months. In 3 out of 4 subjects, thiamine therapy allowed both normalization of hyperglycemia, with consequent insulin suspension, and macrocytic anemia. In all Cases, thiamine therapy did not resolve the clinical manifestation of deafness. In Cases 2 and 3, follow-up showed no blindness, unlike Case 4, in which treatment was started for megaloblastic anemia at age 7 but was increased to high doses only at age 25, when the genetic diagnosis of TRMA was performed. CONCLUSIONS: Early institution of high-dose thiamine supplementation seems to prevent the development of retinal changes and optic atrophy in TRMA patients. The spectrum of clinical manifestations is broad, and it is important to describe known Cases to gain a better understanding of this rare disease.

Peripapillary fluid: Obvious and not so obvious!

Intraretinal or subretinal fluid in the peripapillary area can be clinically visualized in conditions such as peripapillary choroidal neovascularization, optic disc pit maculopathy, and optic nerve head tumors and granulomas. Optical coherence tomography (OCT) helps to visualize peripapillary fluid in many other chorioretinal conditions such as peripapillary pachychoroid syndrome, posterior uveitis, central retinal vein occlusion, malignant hypertension, hypotonic maculopathy as well as neuro-ophthalmological conditions such as glaucoma, microcystic macular edema and disc edema due papilledema, non-arteritic anterior ischemic optic neuropathy, neuroretinitis, and diabetic papillopathy. Often, the differential diagnosis of peripapillary fluid is a bit tricky and may lead to misdiagnosis and improper management. We describe a diagnostic algorithm for peripapillary fluid on OCT and outline the salient features and management of these conditions.

Two-port dry vitrectomy for rhegmatogenous retinal detachment: a pilot study.

OBJECTIVE: To evaluate the safety and efficacy of a new surgical technique for the management of primary rhegmatogenous retinal detachment (RRD), consisting of localized PPV near the retinal break(s), without infusion line, associated with a drainage of subretinal fluid and cryoretinopexy. METHODS: Multicentric prospective study conducted at the University Hospital of Cagliari and IRCCS Fondazione Policlinico Universitario A. Gemelli, Roma. Twenty eyes affected by RRD with the causative retinal break(s) in the superior meridians were enrolled between February 2022 and June 2022. Patients with cataract ≥3, aphakia, significant posterior capsule opacification, giant retinal tears, retinal dialysis, history of trauma and PVR ≥C2 were excluded. All eyes underwent a two-port 25-gauge PPV with localized removal of the vitreous surrounding retinal break(s), followed by 20% SF6 injection and cryopexy. The surgical time was recorded for each procedure. Best-corrected visual acuity (BCVA) was measured at baseline and postoperative 6 months. RESULTS: Primary anatomic success at 6 months was achieved by 85% of patients. No complications occurred, except for three (15%) retinal re-detachments. The average surgical time was 8.61 ± 2.16 min. Overall, the difference between pre- and last postoperative mean BCVA was statistically significant (p = 0.02). CONCLUSIONS: Two-port dry PPV demonstrated safety and efficacy for the treatment of RRD, reaching an 85% of anatomical success rate. Although further studies are necessary to confirm the efficacy and long-term benefit of this treatment, we believe that this surgical technique could be considered a valid and safe alternative for the management of primary RRD.

Pars Plana Vitrectomy in Inherited Retinal Diseases: A Comprehensive Review of the Literature.

Inherited retinal diseases (IRDs) are a group of clinically and genetically heterogeneous disorders that may be complicated by several vitreoretinal conditions requiring a surgical approach. Pars plana vitrectomy (PPV) stands as a valuable treatment option in these cases, but its application in eyes with such severely impaired chorioretinal architectures remains controversial. Furthermore, the spreading of gene therapy and the increasing use of retinal prostheses will end up in a marked increase in demand for PPV surgery for IRD patients. The retinal degeneration that typically affects patients with hereditary retinal disorders may influence the execution of the surgery and the expected results. Considering the importance of PPV application in IRD-related complications, it is fundamental to try to understand from the literature what is adequate and safe in posterior eye segment surgery. Use of dyes, light toxicity, and risk of wounding scar development have always been themes that discourage the execution of vitreoretinal surgery in already impaired eyes. Therefore, this review aims to comprehensively summarize all PPV applications in different IRDs, highlighting the favorable results as well as the potential precautions to consider when performing vitreoretinal surgery in these eyes.

Clinical Applications of Optical Coherence Tomography Angiography in Inherited Retinal Diseases: An Up-to-Date Review of the Literature.

Optical coherence tomography angiography (OCT-A) is a valuable imaging technique, allowing non-invasive, depth-resolved, motion-contrast, high-resolution images of both retinal and choroidal vascular networks. The imaging capabilities of OCT-A have enhanced our understanding of the retinal and choroidal alterations that occur in inherited retinal diseases (IRDs), a group of clinically and genetically heterogeneous disorders that may be complicated by several vascular conditions requiring a prompt diagnosis. In this review, we aimed to comprehensively summarize all clinical applications of OCT-A in the diagnosis and management of IRDs, highlighting significant vascular findings on retinitis pigmentosa, Stargardt disease, choroideremia, Best disease and other less common forms of retinal dystrophies. All advantages and limitations of this novel imaging modality will be also discussed.

Two-Port "Dry Vitrectomy" as a New Surgical Technique for Rhegmatogenous Retinal Detachment: Focus on Macula-on Results.

We evaluated a new surgical technique for treating primary rhegmatogenous retinal detachment (RRD), consisting of localized vitrectomy near the retinal break associated with drainage of subretinal fluid without infusion. Twelve eyes of twelve patients with primary RRDs with macula-on superior, temporal, and/or nasal quadrants' RRD with retinal breaks between 8 and 4 o'clock, pseudophakic or phakic eyes, were enrolled. All eyes underwent a two-port 25-gauge vitrectomy with localized removal of the vitreous surrounding the retinal break(s), followed by a 20% SF6 injection and cryopexy. The difference between pre-operative (T0) and post-operative mean BCVA at 6 months follow-up (T6) was not statistically significant (0.16 logMAR vs. 0.21 logMAR; p = 0.055). Primary anatomic success at 6 months was achieved by 86% of patients. No other complications, except for two retinal re-detachments linked to an incorrect head position of the patients, were recorded. Although further studies are necessary to evaluate the treatment's efficacy, we believe our technique could be considered a valid alternative for managing primary RRD.

Yellow Subthreshold Micropulse Laser in Retinal Diseases: An In-Depth Analysis and Review of the Literature.

Yellow subthreshold micropulse laser (YSML) is a retinal laser capable of inducing a biologic response without causing thermal damage to the targeted tissue. The 577-nm YSML is delivered to the retina abiding by different protocols in which wavelength, power, duration, spot size and number of spots can be properly set to achieve the most effective and safe treatment response in various chorioretinal disorders. The ultrashort trains of power modulate the activation of the retinal pigment epithelium cells and intraretinal cells, such as Müller cells, causing no visible retinal scars. Subthreshold energy delivered by YSML stimulates the production of the heat-shock proteins, highly conserved molecules that protect cells against any sort of stress by blocking apoptotic and inflammatory pathways that cause cell damage. YSML treatment allows resorption of the subretinal fluid in central serous chorioretinopathy and intraretinal fluid in various conditions including diabetic macular edema, postoperative cystoid macular edema and other miscellaneous conditions. YSML also seems to modulate the development and progression of reticular pseudodrusen in dry age-related macular degeneration. The aim of this review is to discuss and summarize the safety and efficacy of YSML treatment in retinal diseases.

Multimodal Imaging Based Predictors for the Development of Choroidal Neovascularization in Patients with Central Serous Chorioretinopathy.

This study evaluated predictors for choroidal neovascularization (CNV) associated with central serous chorioretinopathy (CSCR) based on multimodal imaging. A retrospective multicenter chart review was conducted on 134 eyes of 132 consecutive patients with CSCR. Eyes were classified as per the multimodal imaging-based classification of CSCR at baseline into simple/complex CSCR and primary episode/recurrent/resolved CSCR. Baseline characteristics of CNV and predictors were evaluated with ANOVA. In 134 eyes with CSCR, 32.8% had CNV (n = 44) with 72.7% having complex CSCR (n = 32), 22.7% having simple (n = 10) and 4.5% having atypical (n = 2). Primary CSCR with CNV were older (58 vs. 47, p = 0.00003), with worse visual acuity (0.56 vs. 0.75, p = 0.01) and of longer duration (median 7 vs. 1, p = 0.0002) than those without CNV. Similarly, recurrent CSCR with CNV were older (61 vs. 52, p = 0.004) than those without CNV. Patients with complex CSCR were 2.72 times more likely to have CNV than patients with simple CSCR. In conclusion, CNV associated with CSCR was more likely in complex CSCR and older age of presentation. Both primary and recurrent CSCR are implicated in CNV development. Patients with complex CSCR were 2.72 times more likely to have CNV than patients with simple CSCR. Multimodal imaging-based classification of CSCR supports detailed analysis of associated CNV.

Iatrogenic ophthalmic artery occlusion after platelet-rich plasma dermal filler documented with ultra-widefield imaging.

PURPOSE: To report a case of iatrogenic ophthalmic artery occlusion (OAO) secondary to platelet-rich plasma (PRP) dermal filler injection for facial rejuvenation documented with ultra-widefield imaging. METHODS: Case report. RESULTS: A 45-year-old woman developed a sudden and painful vision loss in the left eye (LE) after a dermal filler injection of PRP in the left glabellar region. She immediately received intravenous corticosteroids with no improvements. Two weeks later a complete ophthalmological examination including visual acuity (VA), fundus examination, ultra-widefield fundus autofluorescence and fluorescein angiography, and optical coherence tomography was performed. A diagnosis of iatrogenic OAO in the LE with profound ocular ischemia was made and VA remained no light perception. Monthly follow-up visits were scheduled to ascertain the onset of any ocular complication. CONCLUSIONS: Dermal filler injections of PRP can lead to rare but devastating side effects with permanent visual loss. Considering that there is currently no validated treatment strategy, prevention could be the real key of iatrogenic OAO management.

Vitelliform maculopathy: Diverse etiologies originating from one common pathway.

Vitelliform lesions (VLs) are associated with a wide array of macular disorders but are the result of one common pathway: retinal pigment epithelium (RPE) impairment and phagocytic dysfunction. VLs are defined by the accumulation of yellowish subretinal material. In the era of multimodal advanced retinal imaging, VLs can be further characterized by subretinal hyperreflectivity with optical coherence tomography and hyperautofluorescence with fundus autofluorescence. VLs can be the result of genetic or acquired retinal diseases. In younger patients, VLs usually occur in the setting of Best disease. Additional genetic causes of VL include pattern dystrophy or adult-onset vitelliform macular dystrophy. In older patients, acquired VLs can be associated with a broad spectrum of etiologies, including tractional, paraneoplastic, toxic, and degenerative disorders. The main cause of visual morbidity in eyes with VLs is the onset of macular atrophy and macular neovascularization. Histopathological studies have provided new insights into the location, nature, and lifecycle of the vitelliform material comprised of melanosomes, lipofuscin, melanolipofuscin, and outer segment debris located between the RPE and photoreceptor layer. Impaired phagocytosis by the RPE cells is the unifying pathway leading to VL development. We discuss and summarize the nature, pathogenesis, multimodal imaging characteristics, etiologies, and natural course of vitelliform maculopathies.

Imaging characteristics of bilateral CSCR cases:12 months follow up.

PURPOSE: To report the disease pattern, progression and imaging characteristics in eyes with bilateral central serous chorioretinopathy (CSCR). METHODS: This was a retrospective case review of bilateral CSCR patients with active disease in at least one eye. Multimodal imaging including fundus photography, fundus autofluorescence, optical coherence tomography (OCT), fluorescein and indocyanine angiography (FA/ICGA) was done at baseline and follow-up visits. Disease classification was done using recently described classification criteria. The degree of asymmetry in the disease distribution pattern at baseline and disease progression during follow-up visit with a minimum duration of 12 months was studied. RESULTS: Among 103 CSCR patients, 36 patients (34.95%) with mean age of 53.6 ± 10.5 years had bilateral CSCR at baseline. Five patients (13.9%) had asymmetrical disease i.e. simple in one eye and complex in fellow eye. The remaining 31 patients had symmetric disease (simple, 2; complex 29). Mean duration of follow up was 17.58 ± 13.84 months. There was no significant difference between both eye parameters at last follow up (best corrected visual acuity, BCVA; central macular thickness, CMT; and subfoveal choroidal thickness, SFCT) (all p > 0.05). At last follow up, 22 eyes (2 simple and 20 complex) remained active whereas none of the eyes converted from simple to complex CSCR. CONCLUSION: Bilateral disease was more commonly seen with complex CSCR in contrast to simple CSCR. Moreover, disease distribution in complex CSCR had symmetric pattern if bilateral disease was present. None of the simple CSCR eyes converted to complex type.