Intraretinal Cystoid Spaces Research Articles

Risk factors for failing sub-Tenon’s triamcinolone acetonide for uveitic macular edema

BackgroundSub-Tenon’s triamcinolone acetonide (STA) is less effective than intravitreal corticosteroids in the treatment of uveitic macular edema (ME), but does have some relative advantages, including substantially lower cost and decreased risk of post-injection ocular hypertension. It would be useful for clinicians to know which eyes may respond well to STA and not necessarily require intravitreal therapy. The objective of this study is to identify risk factors for failing STA for the treatment of uveitic ME.Main bodyA retrospective cohort study was performed. Medical records were reviewed of patients who underwent STA for the treatment of uveitic ME between January 1, 2013, and July 31, 2022, at the University of Colorado Hospital. Uveitic ME was defined by a central subfield thickness (CST) greater than 320 μm or the presence of intra-retinal cystoid spaces on optical coherence tomography (OCT), or by the presence of petaloid macular leakage on fluorescein angiography (FA). Data collected included age, race/ethnicity, sex, history of diabetes mellitus, anatomic classification of uveitis, use of corticosteroids, use of immunomodulatory therapy, presence of intra-retinal fluid on OCT, CST on OCT, and presence of petaloid macular leakage on FA. STA failure was defined as the need for additional therapy within 12 weeks of STA due to persistent or worsening uveitic ME. One hundred eighty eyes from 131 patients were included. Forty-two eyes (23.3%) were considered treatment failures. In univariate and multivariable analysis, higher baseline CST was associated with a higher likelihood of failing STA (OR 1.17 for each 30 μm increase in CST, P = 0.016).ConclusionsSTA, while not as potent as intravitreal corticosteroids for the treatment of uveitic ME, was still an effective therapy, particularly for patients with lower baseline CST. Given its lower side effect profile and cost compared to intravitreal treatments, clinicians could consider STA as an initial treatment for mild uveitic ME.

OCT biomarkers as predictors of visual improvement in diabetic macular edema eyes receiving dexamethasone implants

BackgroundSeveral optical coherence tomography (OCT) biomarkers have been proposed as predictors for functional and anatomical outcomes in Diabetic Macular Edema (DME). This study aims to examine the impact of these OCT features on the visual acuity improvement of patients with DME after long-acting Dexamethasone intravitreal implants (DEX-I) injection. Furthermore, the safety and impact of DEX-I on clinical parameters, including intraocular pressure (IOP) were assessed.MethodsIn this retrospective observational study, we reviewed the medical records of naïve and non-naïve eyes with DME who received at least one DEX-I. The primary endpoint was visual acuity improvement of ≥ 5 ETDRS letters at 1 month and 4 months after treatment. Secondary outcomes were the changes in OCT biomarkers and the impact of DEX-I on IOP at 1 and 4 months of follow-up. Linear panel regression analysis was used to test for differences in central subfield thickness (CST) over time and it was stratified according to biomarkers at baseline. Finally, a logistic regression analysis was used to identify factors predicting visual improvement at 1 and 4 months.ResultsWe included 33 eyes of which 63.6% were at an advanced stage of DME. Overall, CST, cube average thickness (CAT), cube volume (CV), and intraretinal cystoid spaces > 200 μm (ICS) decreased following DEX-I injection (p < 0.001). Additionally, a thicker CST at baseline was observed in eyes with better visual improvement at one month (p = 0.048). After logistic regression analysis, CST was retained as the only predictor for visual improvement at one month (p = 0.044). Furthermore, panel regression analysis identified a relation between subfoveal neuroretinal detachment (SND) at baseline and CST increase at four months. Lastly, only 15.2% of the eyes necessitated topical medication for IOP reduction, with no differences observed when stratifying between naïve and non-naïve eyes.ConclusionOur analyses suggest that a ticker baseline CST may serve as a positive predictor of early visual improvement and SND presence at baseline may be a negative prognostic factor for CST increase 4 months after DEX-I injection. Other well-known biomarkers, such as disorganization of the inner retinal layers (DRIL) and hyperreflective foci (HF), did not demonstrate prognostic value on visual outcomes, at least within the first four months following the injection.

TOPOGRAPHIC ASSESSMENT OF INTRARETINAL CYSTOID SPACE AND ITS PROGNOSTIC VALUES IN IDIOPATHIC EPIRETINAL MEMBRANE.

To assess the topographical distribution of intraretinal cystoid space (IRC) and its prognostic value in idiopathic epiretinal membrane (iERM). One hundred twenty-two eyes of iERM that had been followed up for 6 months after membrane removal were included. Based on the baseline IRC distribution, the eyes were divided into Groups A, B, and C (absence, IRC within 3 mm, and 6 mm from the fovea, respectively). The best-corrected visual acuity (BCVA), central subfield macular thickness (CSMT), ectopic inner foveal layer, and microvascular leakage (ML) were assessed. Fifty-six eyes (45.9%) had IRC, of which 35 (28.7%) were in Group B and 21 (17.2%) in Group C at baseline. Compared with group B, group C showed worse BCVA, thicker CSMT, and a greater association with ML (OR = 5.415; P = 0.005) at baseline; and also presented with worse BCVA, thicker CSMT, and wider distribution of IRC postoperatively. A wide distribution of IRC was an unfavorable baseline factor in achieving good visual acuity (OR = 2.989; P = 0.031). Widely distributed IRCs were associated with advanced disease phenotype as poor BCVA, thick macula, and baseline ML in iERM and also showed a poor visual outcome after membrane removal.

Intravitreal Ranibizumab and Dexamethasone Implant Injections as Primary Treatment of Diabetic Macular Edema: The Month 24 Results from Simultaneously Double Protocol

Purpose To compare efficacy of simultaneously administered intravitreal dexamethasone implant (DEX implant) and ranibizumab (simultaneous double protocol) injections with ranibizumab monotherapy for diabetic macular edema (DME) at month 24. Methods This is a prospective, consecutive, clinical interventional study. Naïve eyes with DME were randomized into two groups: 34 eyes received simultaneous double-protocol therapy and 34 eyes received ranibizumab monotherapy. The primary efficacy endpoint was change in visual acuity in month 24. The secondary efficacy endpoints were to evaluate gain of ≥15 letters, morphological changes and central foveal thickness. Decreased vision from DME (study eye BCVA, 20/40 or worse Snellen equivalent using ETDRS testing), the presence of DME with ≥300 μm foveal intraretinal cystoid spaces (within 1000 μm of the centre of the fovea), subfoveal neuroretinal detachment (SND), intraretinal hyperreflective foci (HRF, within 500 μm of the centre of the fovea) or foveal lipid exudates and external limiting membrane (ELM) and ellipsoid zone (EZ) disruption (within 500 μm of the centre of the fovea) on SD-OCT were eligible to enrol. Results The mean baseline BCVA was 48 ± 23 letters in double protocol group and 52 ± 14 letters ranibizumab monotherapy group (p = 0.416). The mean number of ETDRS letters changed from baseline at 12 months versus change from baseline at month 24 in double protocol group and ranibizumab monotherapy group were +21.6 versus +20.2 and +9.6 versus +9.1, respectively. At the month 24 time point, 65.4% of patients in double protocol group and 26.2% of patients in ranibizumab monotherapy group had gained ≥15 ETDRS letters in BCVA from baseline (p < 0.001). The external limiting membrane (ELM) and ellipsoid zone (EZ) integrity were better in the double protocol group in comparison to ranibizumab monotherapy group at month 24. In addition, there was no statistically significant increase in the proportion of patients with epiretinal membrane in double protocol group at month 24, by the contrast with ranibizumab monotherapy group (p = 0.06 and p = 0.04 in the double protocol and ranibizumab monotherapy groups, respectively). From baseline to month 24, the mean central foveal thickness (CFT) was 672 ± 293 μm reduced to 278 ± 84 μm in double protocol group and was 631 ± 279 μm reduced to 356 ± 108 μm in ranibizumab monotherapy group (p < 0.001 and p < 0.001). From baseline to month 24, 38% (13/34) of eyes in double protocol group and 18% (6/34) of eyes in ranibizumab monotherapy group had at least 5 mmHg of IOP elevation (p = 0.012). Two grades or more increased cataract density were detected 27% (6/22) of eyes in the double protocol group and in 12.5% (3/24) of eyes in the ranibizumab monotherapy group from baseline to month 24 (p = 0.032). Conclusion According to the improvements in visual acuity and morphological changes achieved at month 24, the simultaneous double protocol therapy can be an effective treatment option for DME with inflammatory biomarkers on OCT or/and decreased visual acuity.

Clinical characteristics of patients with epiretinal membrane-Foveoschisis

PurposeThe purpose of this study is to investigate the clinical and morphological characteristics of epiretinal membrane (ERM)-Foveoschisis.MethodsMedical charts of 2088 patients diagnosed with idiopathic ERM were screened and eyes with ERM-Foveoschisis were included. All eyes underwent a complete ophthalmological examination including spectral domain optical coherence tomography (SD-OCT). OCT features and best corrected visual acuity (BCVA) were analysed. ERM-Foveoschisis was defined as open, closed, elevated or flat based on the OCT features. Ellipsoidal zone (EZ) abnormality, intraretinal cystoid spaces, central foveal thickness (CFT), posterior vitreous detachment (PVD) and lens status were assessed.ResultsOne hundred-sixty-six patients (175 eyes) (72% female, mean age 70.46 years) were included. Incidence of ERM-Foveoschisis was 6.7%. Open type was seen in 86.8% and had a significantly better mean BCVA than closed type (p = 0.01). No statistically significant difference of mean BCVA was noted between the elevated and flat types. Mean BCVA was significantly lower in eyes with EZ abnormality (p = 0.03) and eyes with intraretinal cystoid spaces (p = 0.02). Patients with ‘closed’ ERM-Foveoschisis showed a significant higher median CFT than ‘open’ ERM-Foveoschisis (respectively, 364 µm and 176 µm, p < 0.001). A total of 81.9% eyes had PVD.ConclusionWe differentiated four morphological types of ERM-Foveoschisis based on the OCT examination. Closed ERM-Foveoschisis presented with a higher CFT and lower BCVA than the open type. ERM-Foveoschisis with cystoid intraretinal spaces presented with a lower BCVA. The impact of the morphological types of the ERM-Foveoschisis on the clinical course and for therapy decision requires further long-term studies.

Retinal Thickness and Morphology Changes on OCT in Youth with Type 2 Diabetes: Findings from the TODAY Study.

To evaluate changes in retinal thickness and morphology using OCT in youth with type 2 diabetes (T2D) and to identify systemic biomarkers correlating with these changes. Retrospective subgroup analysis of a prospective study. Participants who underwent OCT imaging in the Treatment Options for Type 2 Diabetes in Adolescents and Youth (TODAY) trial and its follow-up study TODAY2. In 2010-2011 (TODAY) and 2017-2018 (TODAY2), 6×6-mm macular volume OCT scans were acquired, segmented, and analyzed to generate total retinal thickness, inner retinal thickness, and outer retinal thickness. The main retinal morphologies graded were intraretinal cystoid spaces, subretinal fluid, and posterior vitreous detachment (PVD). Changes in total and individual retinal layer thickness and development of abnormal vitreomacular morphology between TODAY and TODAY2. Participants had a mean age of 17.9±2.4 years and glycated hemoglobin (HbA1c) of 8.2±2.8% in TODAY and a mean age of 25.0±2.4 years and mean HbA1c of 9.5±2.8% in TODAY2. Longitudinally between assessments, there were overall decreases in outer retinal thickness from 167.2±11.5 microns to 158.4±12.8 microns (P<0.001) and in photoreceptor thickness from 30.3±2.9 microns to 29.8±4.1 microns (P=0.04) in the central subfield, while in the inner subfield, we noted a decrease in outer retinal thickness from 150.5±10.1 microns to 144.9±10.5 microns (P<0.001) and an increase in inner retinal thickness from 136.9±11.5 microns to 137.4±12.6 microns (P=0.01). Multivariate analysis showed that in the center subfield, HbA1c increases were associated with increases in total retinal thickness (r: 0.67, P=0.001), whereas fasting glucose was positively correlated with inner retinal thickness (r: 0.02, P=0.02). In the inner subfield, both systolic (r:-0.22, P<0.001) and diastolic (r:-0.22, P=0.003) blood pressures were negatively correlated with total retinal thickness. There was an increase in PVD (18.9%) and cystoid spaces (4.2%). Youth with T2D develop retinal thickness changes on OCT, including increases in total retinal and inner retinal thickness in the center subfield that correlate with HbA1c and fasting glucose, respectively. Taken together with the increased prevalence of abnormal vitreomacular morphology in this cohort at risk, these findings emphasize the importance of controlling risk factors to prevent the development of sight-threatening retinal complications.

THREE-DIMENSIONAL QUANTIFICATION OF INTRARETINAL CYSTOID SPACES ASSOCIATED WITH FULL-THICKNESS MACULAR HOLE.

To evaluate intraretinal cystoid spaces in patients with idiopathic macular hole (MH). Retrospective cohort study included consecutive patients with full-thickness MH who underwent successful MH surgery and 12 months of follow-up. Custom software was applied to preoperative optical coherence tomography scans to generate fluid volume. Inner fluid volume was defined as cystoid spaces in the inner nuclear layer, and outer fluid volume was defined as cystoid spaces in Henle fiber layer of the outer nuclear layer. Thirty-nine eyes from 39 participants were included. Postoperative 12-month visual acuity correlated with both inner fluid volume and minimum MH size (both P < 0.05) but not outer fluid volume. Inner fluid volume positively correlated with minimum MH size ( P = 0.0003). After accounting for minimum MH size with multivariable analysis, inner fluid volume effect on VA remained significant ( P = 0.025). After dividing inner fluid volume into tertiles, mean baseline visual acuity was 20/50 in eyes with small inner fluid volume, and was 20/125 in eyes with large inner fluid volume ( P = 0.0039). Mean postoperative 12-month visual acuity was 20/20 in eyes with small inner fluid volume compared with 20/32 in eyes with large inner fluid volume ( P = 0.019). Increased inner fluid volume was associated with worse postoperative VA.

Non-vasogenic cystoid maculopathies.

Besides cystoid macular edema due to a blood-retinal barrier breakdown, another type of macular cystoid spaces referred to as non-vasogenic cystoid maculopathies (NVCM) may be detected on optical coherence tomography but not on fluorescein angiography. Various causes may disrupt retinal cell cohesion or impair retinal pigment epithelium (RPE) and Müller cell functions in the maintenance of retinal dehydration, resulting in cystoid spaces formation. Tractional causes include vitreomacular traction, epiretinal membranes and myopic foveoschisis. Surgical treatment does not always allow cystoid space resorption. In inherited retinal dystrophies, cystoid spaces may be part of the disease as in X-linked retinoschisis or enhanced S-cone syndrome, or occur occasionally as in bestrophinopathies, retinitis pigmentosa and allied diseases, congenital microphthalmia, choroideremia, gyrate atrophy and Bietti crystalline dystrophy. In macular telangiectasia type 2, cystoid spaces and cavitations do not depend on the fluid leakage from telangiectasia. Various causes affecting RPE function may result in NVCM such as chronic central serous chorioretinopathy and paraneoplastic syndromes. Non-exudative age macular degeneration may also be complicated by intraretinal cystoid spaces in the absence of fluorescein leakage. In these diseases, cystoid spaces occur in a context of retinal cell loss. Various causes of optic atrophy, including open-angle glaucoma, result in microcystoid spaces in the inner nuclear layer due to a retrograde transsynaptic degeneration. Lastly, drug toxicity may also induce cystoid maculopathy. Identifying NVCM on multimodal imaging, including fluorescein angiography if needed, allows guiding the diagnosis of the causative disease and choosing adequate treatment when available.

CLINICAL FEATURES AND PROGNOSIS IN IDIOPATHIC EPIRETINAL MEMBRANES WITH DIFFERENT TYPES OF INTRARETINAL CYSTOID SPACES

To observe the characteristics and prognosis of different types of intraretinal cystoid spaces in idiopathic epiretinal membranes (iERMs). Two hundred and seven consecutive patients with symptomatic iERM who underwent vitrectomy between September 2016 and December 2019 were included. According to spectral-domain optical coherence tomography images, intraretinal cystoid spaces were classified into cystoid macular edema (CME) and microcystic macular edema (MME). Other optical coherence tomography characteristics, including ectopic inner foveal layers, central foveal thickness, and interdigitation zone integrity, were also evaluated. Intraretinal cystoid spaces were presented in 30.1% of the iERMs, 21.5% were CME-type, 66.2% were MME-type, and 12.3% were combined-type. Compared with CME, eyes with MME-type and combined-type showed a significantly lower best-corrected visual acuity (BCVA) preoperatively and postoperatively. Cystoid macular edema is always presented in earlier stages (92.9%) and has no significant effects on BCVA (PStage I = 0.927, PStage II = 0.985). Conversely, MME is the primary type in advanced stages associated with a longer duration of symptoms (P = 0.037) and lower preoperative BCVA (P = 0.008). After surgery, cystoid spaces were newly occurred in 33 eyes (21.6%), with no effects on BCVA (P = 0.668). In the multiple regression analysis, the presence of MME was a risk factor for preoperative BCVA (P = 0.001). However, it is not an independent predictor for the postoperative VA. Our research further proved that MME is an adverse factor for preoperative and postoperative VA in iERMs. Moreover, we underlined the importance of distinguishing between CME and MME, which may affect prognosis differently.

Evaluation of the retinal and choroidal microvasculature changes in cases of sarcoid and tuberculosis-associated posterior uveitis using OCT angiography

PurposeUsing optical coherence tomography angiography (OCTA) to evaluate retinal microvascular changes in sarcoid and tuberculous (TB) posterior uveitis.MethodsCross-sectional observational study includes 30 eyes. FFA and OCTA images were acquired. OCTA images were analyzed for areas of capillary hypo-perfusion, disorganization of the superficial and deep capillary plexuses (SCP and DCP) and intraretinal cystoid spaces and for measuring the size of the foveal avascular zone and vessel density (VD) in the SCP and DCP.ResultsA total of 11 eyes were associated with TB and 19 with sarcoidosis. By OCTA, 100% had areas of capillary non-perfusion, 36.7% choroidal voids, 30% disorganization of the SCP and DCP and 26.6% intraretinal cystoid spaces. The VD of the DCP was significantly lower in the TB group. On comparing OCTA and FFA, parafoveal ischemia was detected more frequently on OCTA and macular edema more frequently on FFA (P = < 0.001). The BCVA was not significantly correlated with the VD of the SCP or DCP.ConclusionOCTA can be used in detection of early microvascular changes, segmenting retinal layers and localizing abnormalities. The presence of these changes may aid in the diagnosis of TB and sarcoid uveitis, for prognosis, follow-up and may be the only choice when FFA is contraindicated.

Impact of OCT scan-patterns in identifying morphological features of lamellar macular holes and macular pseudoholes.

To evaluate the impact of the optical coherence tomography (OCT) scan patterns on the detection of the features associated with lamellar macular hole (LMH) and macular pseudohole (MPH). This is a retrospective analysis of 100 consecutive eyes with LMH (n=41) and MPH (n=59) having at least three of the following OCT features, which include mandatory criteria for the diagnosis of LMH and MPH: Epiretinal membrane, epiretinal proliferation, verticalization, intraretinal cystoid spaces, foveoschisis, irregular foveal contour, foveal cavity with undermined edges, and ellipsoid line disruption. Primary outcome measurement was the detection frequency of the features in three different OCT scan patterns: 1) volume scan; 2) six radial scans (R6); and 3) vertical and horizontal radial scans (R2). Of the total eight features, the maximal detection frequency was found as 4.45±1.45, 4.35±1.47, and 3.70±1.59, by the volume, R6 and R2, respectively. R2 was inferior to the other patterns in detection of the total features (P<0.001), whereas R6 and volume patterns were found comparable (P=0.312). The physician should be aware that the selection of the OCT-scan pattern may influence the detection of mandatory morphological criteria for the diagnosis of LMH and MPH.

Predictive value of the characteristics of intraretinal cystoid spaces on early response to antivascular endothelial growth factor treatment in patients with cystoid diabetic macular edema.

To evaluate whether baseline spectral-domain optical coherence tomography characteristics of intraretinal cystoid spaces predict visual outcomes in patients receiving intravitreal antivascular endothelial growth factor injection therapy (bevacizumab 1.25mg/0.05ml) for diabetic cystoid macular edema. The relationship between the properties of the cystoid spaces before injection and anatomic and functional results after injection were investigated in patients who received three consecutive intravitreal bevacizumab injections for cystoid macular edema. The best-corrected visual acuity for functional success and central subfield thickness for anatomical success were evaluated. The relationship of the location of the cystoid spaces with the integrity of photoreceptors and inner retinal layers was also evaluated. In 36 eyes of 36 patients, the mean best-corrected visual acuity significantly improved (p=0.002), and mean central subfield thickness decreased after injections (p=0.003). The improvement in best-corrected visual acuity was limited in eyes with outer nuclear layer cysts (p=0.045). Intracystic reflectivity was higher in eyes that poor best-corrected visual acuity than in eyes with successful visual outcomes (p=0.028). The disrupted ellipsoid zone was present in 13 (59.0%) of 22 eyes with outer nuclear layer cysts, whereas in only 1 of 14 eyes (7.1%) without outer nuclear layer cysts (p=0.009). Disorganization of retinal inner layers was present in 15 of 22 (68.1%) eyes with outer nuclear layer cysts, whereas only 2 of 14 (14.2%) without outer nuclear layer cysts had disorganization of retinal inner layers (p=0.013). Characteristics of intraretinal cystoid spaces may predict prognosis in patients with diabetic cystoid macular edema, and visual gain may be limited in the eyes with outer nuclear layer cysts.

One-year outcome of cystoid macular degeneration in central serous chorioretinopathy

To study structural and functional outcomes of cystoid macular degeneration (CMD) in chronic central serous chorioretinopathy (CSCR). This retrospective study included 26 eyes having chronic CSCR with CMD who underwent either observation, photodynamic therapy (PDT), micropulse laser, or eplerenone therapy. Various optical coherence tomography parameters were analyzed at baseline and 1 year. Number of eyes that maintained or gained vision after treatment was 63.1%, compared to a loss of 2.1 ± 1.1 lines in observation group. Sub-foveal large choroidal vessel responded to PDT (p = 0.03); while CMT (p = 0.035) and intra-retinal cystoid spaces (0.037) responded to eplerenone. Longer duration of the symptoms and round cystoid spaces were associated with a decrease in CMT (p = 0.03) and decrease in cystoid spaces size (p = 0.02) respectively on follow up. Treatment of eyes with CMD prevents further deterioration of vision. Round configuration of intra-retinal cystoid space has a better anatomical outcome.

Retinal Dialysis Revealed by Swept-Source OCT

We present a case of a peripheral retinal dialysis treated with laser photocoagulation imaged using far peripheral swept-source (SS)-OCT (Optos Silverstone, Optos). A, Ultra-widefield pseudocolor fundus photography of the left eye shows the inferotemporal retinal dialysis with the pigmented scars corresponding to the previous barricade laser treatment. Green lines (B and C) indicate the locations scanned by SS-OCT. B-scans (B) and (C) demonstrate vitreoretinal traction (arrowhead) with retinal elevation, the presence of intraretinal cystoid spaces and complete absence of the retina more distally (asterisk), consistent with a dialysis (Magnified version of Fig A-C is available online at www.aaojournal.org).

Clinical Heterogeneity in Autosomal Recessive Bestrophinopathy with Biallelic Mutations in the BEST1 Gene

Autosomal recessive bestrophinopathy (ARB) has been reported as clinically heterogeneous. Eighteen patients (mean age: 22.5 years; 15 unrelated families) underwent ophthalmological examination, fundus photography, fundus autofluorescence, and optical coherence tomography (OCT). Molecular genetic testing of the BEST1 gene was conducted by the chain-terminating dideoxynucleotide Sanger methodology. Onset of symptoms (3 to 50 years of age) and best-corrected visual acuity (0.02–1.0) were highly variable. Ophthalmoscopic and retinal imaging defined five phenotypes. Phenotype I presented with single or confluent yellow lesions at the posterior pole and midperiphery, serous retinal detachment, and intraretinal cystoid spaces. In phenotype II fleck-like lesions were smaller and extended to the far periphery. Phenotype III showed a widespread continuous lesion with sharp peripheral demarcation. Single (phenotype IV) or multifocal (phenotype V) vitelliform macular dystrophy-like lesions were observed as well. Phenotypes varied within families and in two eyes of one patient. In addition, OCT detected hyperreflective foci (13/36 eyes) and choroidal excavation (11/36). Biallelic mutations were identified in each patient, six of which have not been reported so far [c.454C>T/p.(Pro152Ser), c.620T>A/p.(Leu207His), c.287_298del/p.(Gln96_Asn99del), c.199_200del/p.(Leu67Valfs*164), c.524del/p.(Ser175Thrfs*19), c.590_615del/p.(Leu197Profs*26)]. BEST1-associated ARB presents with a variable age of onset and clinical findings, that can be categorized in 5 clinical phenotypes. Hyperreflective foci and choroidal excavation frequently develop as secondary manifestations.

Long-term results of autologous plasma as adjuvant to pars plana vitrectomy in the treatment of high myopic full-thickness macular holes.

To analyse the feasibility and efficacy of a novel autologous plasma rich in growth factor (PRGF) preparation as adjuvant to pars plana vitrectomy and internal limiting membrane peel in high myopic full-thickness macular hole (FTMH). Single-centre, single-surgeon retrospective chart review of consecutive patients with high myopic FTMH who underwent surgery with a minimum follow-up of 12 months. Patients were divided in group 1 (naïve) and group 2 (persistent). Quantitative and qualitative variables were analysed, compared among groups and correlated with best corrected visual acuity (BCVA). Postoperatively, FTMH resolved in 28/31 eyes in group 1 (90%) and in 10/11 eyes in group 2 (91%), without significant differences (p = 0.954). None of the preoperative anatomical variables analysed showed significant association with preoperative BCVA. Intraoperatively, no significant complications were registered. Postoperatively, BCVA improved significantly in the studied population (p < 0.001). Preoperative factors associated with better postoperative BCVA were the presence of intraretinal cystoid spaces (p = 0.028) and elevated FTMH borders (p = 0.005). Preoperative dome-shaped macula was associated with significantly worse postoperative BCVA (p = 0.049). The use of PRGF as adjuvant to vitrectomy showed to be reproducible, straightforward and efficient, with primary anatomical success rate comparable to other surgical approaches and lower risk of complications.

Retinal structural and vascular changes in posterior microphthalmos

BackgroundPosterior microphthalmos (PM) is a rare developmental disorder characterised by high hyperopia, short axial length, presence of retinal papillomacular fold and relatively normal anterior segment findings. The study objective is to describe the retinal structural and vascular changes in eyes with PM with spectral domain optical coherence tomography, optical coherence tomography angiography and multicolour imaging.MethodsIn this retrospective, comparative case series, 10 eyes of five patients with PM as cases and 10 eyes of five age‐ and sex‐matched controls were included. Structural changes, namely inner and outer retinal layer thicknesses, were measured using optical coherence tomography. Multicolour imaging findings were noted. Perifoveal vascular changes with qualitative and quantitative assessments were analysed using optical coherence tomography angiography.ResultsThe foveal dip was absent in all 10 eyes (100 per cent) with PM. There was an elevated retinal papillomacular fold in six eyes (60 per cent) and intraretinal cystoid spaces in two eyes (20 per cent) with PM. The inner retinal layers were thicker in PM. On multicolour imaging, foveal avascular zone and retinal wrinkles were identified in eyes with retinal papillomacular fold in blue and green reflectance images. Perifoveal vascular changes in optical coherence tomography angiography included foveal area size reduction in superficial and deep vascular networks. The foveal capillary vessel densities were higher in PM compared to the controls in both superficial (46.3 ± 3.7 per cent; p = 0.000) and deep (54.7 ± 3.5 per cent; p = 0.000) capillary plexuses. Flow areas in superficial (p = 0.693) and deep (p = 0.088) capillary plexuses were not statistically relevant.ConclusionThe study suggests that in PM, retinal foreshortening occurs in one meridian leading to secondary changes such as loss of foveal dip, posterior bowing of the outer layers of the eye, thickening of the inner retina and ultimately, retinal papillomacular fold formation. The findings of the study need further validation in a larger series of patients with PM.

The Area and Number of Intraretinal Cystoid Spaces Predict the Visual Outcome after Ranibizumab Monotherapy in Diabetic Macular Edema.

Visual outcomes in diabetic macular edema (DME) after anti-vascular endothelial growth factor therapy vary across individuals. We retrospectively reviewed the clinical records for 46 treatment-naive eyes of 46 patients with DME who underwent intravitreal ranibizumab (IVR) monotherapy with a pro re nata regimen for 12 months. Overall, mean best-corrected visual acuity (BCVA) improved. Multivariate analyses adjusted for age and baseline BCVA showed that the area ratio, compared with the retinal area, and the number of intraretinal cystoid spaces evaluated on OCT (optical coherence tomography) images at baseline positively correlated with LogMAR BCVA and the extents of ellipsoid zone and external limiting membrane disruption at 12 months, and negatively correlated with central retinal thickness at the time of edema resolution. Therefore, a high area ratio and large number of intraretinal cystoid spaces resulted in a disorganized outer retinal structure at 12 months, a thin and atrophic retina after edema resolution, and a worse visual outcome. The area ratio and number of intraretinal cystoid spaces on initial OCT images were predictors of the visual outcome after IVR therapy in DME irrespective of baseline age and BCVA. The factors were related to retinal neurodegenerative changes in DME and could help in obtaining proper informed consent before treatment.

Distinctive Mechanisms and Patterns of Exudative Versus Tractional Intraretinal Cystoid Spaces as Seen With Multimodal Imaging

To determine clear-cut distinctions between tractional and exudative intraretinal cystoid spaces subtypes. Retrospective, multicenter, observational case series. A cohort of patients diagnosed with intraretinal cystoid spaces and imaged with optical coherence tomography (OCT), fluorescein angiography (FA), blue fundus autofluorescence (BFAF), en face OCT, and OCT angiography (OCT-A) was included in the study. All images were qualitatively and quantitatively evaluated. In this study were included 72 eyes of 69 patients. Exudative intraretinal cystoid spaces (36/72 eyes, 50%) displayed a "petaloid" morphology as seen with en face OCT, FA, and BFAF. Tractional intraretinal cystoid spaces (24/72 eyes, 33.3%), displayed a radial "spoke-wheel" en face OCT pattern. There was no leakage with FA and BFAF did not reveal specific patterns. Eyes with full-thickness macular hole (FTMH, 12/72 eyes, 16.7%) displayed a "sunflower" en face OCT appearance. FTMH showed OCT, OCT-A, and BFAF features of both exudative and tractional cystoid spaces, but without any FA leakage. Inner nuclear layer (INL) thickness was significantly lower in tractional cystoid spaces (P < .001). There were a greater number of INL cystoid spaces in both the exudative and FTMH subgroups (P= .001). The surface area of INL cystoid spaces was significantly lower in the tractional subgroup (P < .001). There was a significant reduction of the microvascular density in eyes with exudative vs tractional (P= .002) and FTMH (P < .001) subgroups. Exudative and tractional intraretinal cystoid spaces displayed characteristic multimodal imaging features and they may represent 2 different pathologic conditions with equally different clinical implications.

PATIENTS WITH EPIRETINAL MEMBRANES DISPLAY RETROGRADE MACULOPATHY AFTER SURGICAL PEELING OF THE INTERNAL LIMITING MEMBRANE

Intraretinal cystoid spaces are commonly found after surgical peeling of epiretinal membranes. In this study, we explored whether these cysts were associated with ganglion cell loss and thus might be a manifestation of retrograde maculopathy. The latter is a nonvascular edema with a characteristic morphology that is often found in the inner nuclear layer (INL) of patients with optic neuropathy. In this retrospective case series, we identified consecutive patients who underwent surgical epiretinal membrane peeling. We determined the frequency of microcystic macular edema (MME), defined by vertical cystoid spaces in the INL, and we measured the thickness of individual macular layers before and after surgery. Epiretinal membrane peeling resulted in an improvement of visual acuity and a reduction of retinal thickness by about 15%. In total, 35% of patients with MME before surgery showed no sign of MME postoperatively, whereas edema persisted after surgery in 65% of patients. Interestingly, 29% of the patients without MME before surgery developed MME after surgery. Overall, we found MME in 35% of patients before peeling and in 42% after peeling. After surgery, the mean ganglion cell layer thickness was reduced compared with healthy control eyes. Ganglion cell layer thickness correlated inversely with thickness of the INL. Compared with patients without MME, individuals with MME had a thinner ganglion cell layer and a thicker INL in the affected eye. Our findings indicate that peeling of epiretinal membranes and internal limiting membranes is associated with atrophy of ganglion cells and thickening of the INL. The latter is associated with the presence of MME. Altogether, we assume that surgical treatment of epiretinal membranes induces a variant of a retrograde maculopathy.