IMPLICATIONS OF COMPLETE POSTERIOR VITREOUS DETACHMENT IN EYES WITH CENTRAL RETINAL VEIN OCCLUSION.

BACKGROUND/PURPOSE: To evaluate the status of the posterior vitreous hyaloid on presenting optical coherence tomography images of the macula and its relationship to clinical characteristics, treatment patterns, and outcomes in eyes with central retinal vein occlusion. METHODS: This is a retrospective longitudinal cohort study of consecutive patients with acute, treatment-naive central retinal vein occlusion diagnosed between 2009 and 2021 who had at least 12 months of follow-up. Clinical characteristics, treatment patterns, and outcomes were analyzed between eyes stratified based on the presence or absence of a complete posterior vitreous detachment (PVD) on optical coherence tomography at presentation. RESULTS: Of 102 acute, treatment-naive central retinal vein occlusions identified, 52 (51%) had complete PVD at presentation and 50 (49%) did not. Central subfield thickness was significantly lower in those with complete PVD (12 months: 284.9 ± 122.9 µ m vs. 426.8 ± 286.4 µ m, P < 0.001; last follow-up: 278 ± 127.9 vs. 372.8 ± 191.0 µ m, P = 0.022). One-year intravitreal injection burden was significantly less for those with a complete PVD than those without (5.1 ± 3.6 injections vs. 6.7 ± 3.3 injections, P = 0.013). CONCLUSION: Central retinal vein occlusion with complete PVD on presentation had significantly lower central subfield thickness and 1-year injection burden. Assessment of the vitreomacular interface in central retinal vein occlusion may serve as a prognostic imaging biomarker.

Occlusive retinal vasculitis: novel insights into causes, pathogenesis and treatment.

PURPOSE OF REVIEW: Occlusive retinal vasculitis (ORV) has a large differential diagnosis and varied therapeutic approaches. This review highlights existing and novel causes and treatment options for ORV. RECENT FINDINGS: Mutations in CAPN5, TREX1, and TNFAIP3 have been associated with dominantly inherited forms of ORV. Various intraocular therapeutics have been shown to occasionally cause ORV; the most recent medications associated with ORV are vancomycin and brolucizumab. In cases of ORV linked to Behçet's disease, clinical trials support the use of tumor necrosis factor alpha inhibitors. SUMMARY: Identification of the underlying etiology of ORV is critical to help guide treatment. Treatment in ORV involves both treatment any underlying infection or autoimmune condition, cessation of the any offending causative agent and local treatment of neovascular complications.

SHORT-TERM OUTCOMES AFTER INTERIM TREATMENT WITH BROLUCIZUMAB: A Retrospective Case Series of a Single Center Experience.

PURPOSE: To examine outcomes of eyes with neovascular age-related macular degeneration that were switched to brolucizumab because of an unsatisfactory response to bevacizumab, ranibizumab, and/or aflibercept and then switched back because of the presence or risk of intraocular inflammation. METHODS: Retrospective case series of 51 eyes. Visual acuity and retinal anatomy on optical coherence tomography were recorded at the first brolucizumab injection (T1), the final brolucizumab injection (T2), and 6 months following the final brolucizumab injection (T3). RESULTS: At T2, 41 eyes (41/51%, 80%) had decreased subretinal fluid (31 eyes), intraretinal fluid (12 eyes), or pigment epithelial detachment height (12 eyes). At T3, decreased subretinal fluid was sustained in 17 eyes (17/31%, 55%), decreased intraretinal fluid was sustained in eight eyes (8/12%, 67%), and decreased pigment epithelial detachment height was sustained in eight eyes (8/12%, 67%). Mean logarithm of the minimum angle of resolution visual acuity at T1, T2, and T3 was 0.396 (∼20/50), 0.441 (∼20/55), and 0.468 (∼20/59), respectively. During the brolucizumab treatment period, 11 eyes (11/51%, 22%) developed intraocular inflammation, including one case of retinal vasculitis. CONCLUSION: Interim treatment with brolucizumab resulted in anatomical improvements in 41 eyes (41/51%, 80%) that were maintained in 22 of these eyes (22/41%, 54%) for at least 6 months after switching back to the original anti-vascular endothelial growth factor therapeutic. There were no corresponding significant changes in visual acuity.

Multimodal imaging in infectious and noninfectious intermediate, posterior and panuveitis.

PURPOSE OF REVIEW: Given the heterogeneity of uveitis, markers of inflammation vary from patient to patient. Multimodal imaging has proven itself to be critical for accurate evaluation for disease activity and treatment response in uveitis. RECENT FINDINGS: Ultra-widefield (UWF) fluorescein angiography and autofluorescence (AF) as well as optical coherence tomography angiography (OCTA) have provided insights into disease pathogenesis and monitoring not previously appreciated. In addition to structural retinal imaging, OCT can be used to assess the choroid, the posterior cortical vitreous and the retinal vasculature in eyes with uveitis. SUMMARY: Multimodal ocular imaging in eyes with uveitis is critical for disease diagnosis and assessing response to treatment. UWF fluorescein angiography can detect retinal vasculitis even in the absence of overt vascular sheathing. UWF AF can help detect more chorioretinal lesions than clinically visible. OCT can be used to assess the posterior cortical vitreous, retina, large retinal vessels and choroid in uveitis. The use of multimodal imaging will likely be needed to determine clinical trial endpoints in studies evaluating therapeutics for uveitis.

Local treatment of infectious and noninfectious intermediate, posterior, and panuveitis: current concepts and emerging therapeutics.

PURPOSE OF REVIEW: Local therapeutics play an important role in the management of infectious and noninfectious uveitis (NIU) as well as certain masquerade syndromes. This review highlights the established therapeutics and those under investigation for the management of uveitis. RECENT FINDINGS: An injectable long-acting fluocinolone acetonide insert was recently approved by the Food and Drug Administration for the treatment of NIU affecting the posterior segment. Intravitreal methotrexate, sirolimus, and anti-vascular endothelial growth factor (VEGF) agents are being evaluated for efficacy in NIU. Intravitreal foscarnet and ganciclovir are important adjuncts in the treatment of viral retinitis as are methotrexate and rituximab for the management of vitreoretinal lymphoma. SUMMARY: Local injectable steroids with greater durability are now available for NIU but comparative efficacy to other treatment modalities remains to be determined. Local steroid-sparing immunosuppressive agents are undergoing evaluation for efficacy in NIU as are anti-VEGF agents for uveitic macular edema. Local antivirals may improve outcomes in cases of viral retinitis. Local chemotherapeutics can help induce remission in vitreoretinal lymphoma.

Biologics for the treatment of noninfectious uveitis: current concepts and emerging therapeutics.

PURPOSE OF REVIEW: There is mounting evidence supporting the use of biologic therapeutics for the management of noninfectious uveitis (NIU). This review highlights: biologics with documented efficacy in NIU; agents with ongoing evaluation for efficacy in uveitis; and therapeutics for which investigation for efficacy in NIU is warranted. RECENT FINDINGS: The tumor necrosis factor-alpha (TNF-α) inhibitor adalimumab has recently gained approval by the Food and Drug Administration for the treatment of noninfectious intermediate, posterior, and panuveitis. There is mounting evidence supporting the use of tocilizumab and rituximab in NIU. There is developing interest in evaluating the interleukin (IL)-23 inhibitors for efficacy in NIU. SUMMARY: The TNF-α inhibitors adalimumab and infliximab have the greatest body of data supporting their use in NIU. These agents are considered second-line therapy for most forms of NIU but may be considered first-line therapy for uveitis associated with Behçet's disease and juvenile idiopathic arthritis. The B-cell inhibitor rituximab and the IL-6 inhibitor tocilizumab also have documented efficacy in NIU. Tocilizumab and interferon therapy may be particularly efficacious in the management of uveitic macular edema. The IL-23 inhibitors and janus kinase inhibitors are agents whose efficacy in NIU will likely be determined in the near future.

USE OF THE ISCHEMIC INDEX ON WIDEFIELD FLUORESCEIN ANGIOGRAPHY TO CHARACTERIZE A CENTRAL RETINAL VEIN OCCLUSION AS ISCHEMIC OR NONISCHEMIC.

PURPOSE: To understand the relationship between baseline ischemic index (IsI) values on ultra-widefield fluorescein angiography and classification as ischemic central retinal vein occlusion (CRVO). METHODS: Single-center retrospective cohort study of CRVO patients imaged using ultra-widefield fluorescein angiography from which IsI values were calculated. An ischemic CRVO was defined as those eyes with an afferent pupillary defect and counting fingers acuity or worse or neovascularization during the first year of follow-up. Logistic regression was performed to characterize the relation between the IsI and clinical outcomes. RESULTS: Sixty eyes of 60 treatment-naive CRVO patients with baseline ultra-widefield fluorescein angiography and ≥1 year of follow-up were identified. Those with an IsI ≥35% were significantly more likely to have an ischemic CRVO during the first year of follow-up than those with an IsI <35% (83.3 vs. 13.9%, odds ratio 111, P < 0.0001). Baseline and final logarithm of the minimum angle of resolution acuity were worse in eyes with an IsI ≥35% (1.18 vs. 0.46, P < 0.001 and 1.26 vs. 0.45, P < 0.001, respectively) despite similar baseline and final central subfield thickness (P = 0.1-0.23). CONCLUSION: A baseline IsI of ≥35% on ultra-widefield fluorescein angiography in eyes with treatment-naive CRVO was sensitive (90%) and specific (92.5%) for classification as an ischemic CRVO during the first year of follow-up.

CENTRAL RETINAL VEIN OCCLUSION IN YOUNG INDIVIDUALS: A Comparison of Risk Factors and Clinical Outcomes.

PURPOSE: To compare the presentation and outcomes of patients younger than 50 years versus patients aged 50 years and older with central retinal vein occlusion (CRVO). METHODS: This single-center retrospective study included patients with CRVO presenting between January 2009 and July 2016. Charts were reviewed and data were abstracted. Presenting and final clinical parameters, treatment burden, and predisposing factors for CRVO were compared between the two groups. RESULTS: Thirty-six patients younger than 50 years and 233 patients aged 50 years and older at the time of CRVO onset were included. At presentation, younger patients had better visual acuity than older patients (20/80 vs. 20/224, P = 0.001) and a lower incidence of cystoid macular edema (54 vs. 79%, P = 0.001). Twenty-one of 36 (58%) younger patients had at least one identifiable nontraditional risk factor for CRVO. At final follow-up, younger patients received fewer total intravitreal injections (3.8 ± 5.8 at 34.2 months) compared with older patients (6.5 ± 8.8, at 37.6 months, P = 0.03) and had better final acuity (20/85 vs. 20/289, P = 0.004, respectively). CONCLUSION: Younger patients had better baseline and final acuities, a lower incidence of cystoid macular edema at presentation, and received fewer intravitreal injections than older patients. Workup for etiology of CRVO in younger patients may reveal nontraditional risk factors for CRVO.

PERIVASCULAR THICKENING ON OPTICAL COHERENCE TOMOGRAPHY AS A MARKER OF INFLAMMATION IN BIRDSHOT RETINOCHOROIDITIS.

PURPOSE: In this study, we hypothesized that thickening along the major arcade vessels is a noninvasive marker of inflammation in eyes with birdshot retinochoroiditis (BRC). METHODS: In this single-center retrospective study, patients with BRC were identified. Perivascular thickening was categorized as mild, moderate, or severe, based on a set of standard reference retinal thickness maps derived from representative spectral domain optical coherence tomography volume scans. The assigned perivascular severity thickness category was then compared with other inflammatory markers and optical coherence tomography measurements. These parameters were also examined in eyes with intermediate uveitis to assess the diagnostic specificity of perivascular thickening. RESULTS: In eyes with BRC, greater perivascular thickening was associated with increased vitreous haze (P = 0.009) and retinal vascular leakage on fluorescein angiography (P = 0.0001). Perivascular thickening was correlated with central subfield thickness and total macular volume on optical coherence tomography. Controlling for central subfield thickness and total macular volume, the odds of higher severity level of perivascular thickening were nine times greater in eyes with BRC than those with intermediate uveitis (P < 0.0001). Eyes with BRC and active inflammation were more likely to have moderate or severe perivascular thickening (P = 0.02). CONCLUSION: Perivascular thickening, determined by optical coherence tomography, may be a useful noninvasive biomarker of inflammation in eyes with BRC.

The role of cystoid macular edema as a marker in the progression of non-paraneoplastic autoimmune retinopathy.

PURPOSE: To determine the relationship between cystoid macular edema (CME) and disease severity and progression in non-paraneoplastic autoimmune retinopathy (npAIR). METHODS: A retrospective study was conducted on patients seen between 2008 and 2016 with npAIR as defined by electroretinogram (ERG) dysfunction, visual field changes, presence of antiretinal antibodies, a negative malignancy workup, and no other apparent cause for visual dysfunction. Optical coherence tomography (OCT) scans were reviewed for each patient. A minimum follow-up of 1 year was necessary for study inclusion. The presence or absence of CME and the length of the preserved EZ on the centermost line scan of the SD-OCT images was recorded at each visit. The main outcome measure assessed was the rate of EZ loss (EZ final - EZ initial / days follow-up) over time, a marker for disease progression. RESULTS: Thirty-two eyes (16 patients) were included with an average follow-up of 42 months. Twenty-one eyes (66%) had CME on initial presentation and final follow-up (group 1), eight eyes (25%) did not have CME on presentation or final follow-up (group 2), and three eyes (9%) did not have CME on presentation but developed CME during follow-up (group 3). Group 1 eyes had a lower maximal a-wave amplitude (59.0 vs. 220.9 mV, p = 0.012) and lower maximal b-wave amplitude (88.1 vs 256.9 mV, p = 0.017) on baseline ERG compared to Group 2 eyes. The rate of EZ loss over time was significantly greater for group 1 with CME compared to group 2 without CME both at 12 months (- 1.26 µm/day vs. - 0.26 µm/day, p = 0.022) and at final follow-up (- 1.03 µm/day vs. - 0.08 µm/day, p = 0.012). CONCLUSIONS: CME was associated with decreased ERG amplitudes and greater velocity of EZ loss, suggesting that CME is a useful biomarker of more severe and more progressive disease in npAIR.

SUBRETINAL TRANSPLANTATION OF AN AUTOLOGOUS RETINAL FREE FLAP FOR CHRONIC RETINAL DETACHMENT WITH PROLIFERATIVE VITREORETINOPATHY WITH AND WITHOUT MACULAR HOLE.

PURPOSE: To describe the surgical technique for subretinal transplantation of an autologous retinal free flap in cases of chronic retinal detachment with proliferative vitreoretinopathy with and without a macular hole. METHODS: Descriptive case series. RESULTS: Two patients with recurrent retinal detachment with proliferative vitreoretinopathy were referred for surgical evaluation. The first patient had high myopia and a macular hole. Acuity at the last time of sustained retinal reattachment was 20/400 for the first patient and counting fingers for the second patient and, in both cases, declined to light perception in the months after redetachment. Despite membrane/internal limiting membrane peeling, surgical repair required an inferior retinectomy in both cases. A retinal free flap was placed subfoveally. In the first patient, the flap was placed with the photoreceptors facing bare retinal pigment epithelium. In the second patient, the flap was inverted so its photoreceptors faced residual macular photoreceptors. The retina was flattened over the flap. Postoperatively, vision improved to 20/160 and 20/400 in the first and second patient respectively. CONCLUSION: Subretinal placement of a retinal flap is possible in eyes with retinal detachment and proliferative vitreoretinopathy with and without macular hole. Whether such a flap results in superior visual and anatomical outcomes remains to be seen.

Ocular manifestations of inflammatory bowel disease.

PURPOSE OF REVIEW: Extraintestinal manifestations (EIMs) of inflammatory bowel disease (IBD) are numerous and can often involve the eye. This review highlights the ocular complications associated with IBD including the critical role the ophthalmologist can play in the diagnosis of IBD, the pathogenesis of IBD, its ocular complications, and the treatment of ocular inflammation associated with IBD. RECENT FINDINGS: Polygenic and environmental influences, as well as gut microbial dysbiosis, have been implicated in the pathogenesis of IBD. IBD and its EIMs appear to respond well to TNFα-targeted biologics. SUMMARY: IBD is thought to be caused by polygenic and environmental influences, including a dysbiotic gut microbiota. It is a systemic immune-mediated disease with varying types of ocular manifestations that can precede, occur simultaneously, or follow intestinal involvement. The diagnosis of IBD can be confused with other seronegative spondyloarthropathies as well as Behçet's disease. Treatment of IBD-associated ocular inflammation can range from corticosteroids to steroid-sparing immunosuppression such as azathioprine or methotrexate. Refractory disease can respond well to TNFα inhibitors.

EFFECT OF SYSTEMIC BETA-BLOCKERS, ACE INHIBITORS, AND ANGIOTENSIN RECEPTOR BLOCKERS ON DEVELOPMENT OF CHOROIDAL NEOVASCULARIZATION IN PATIENTS WITH AGE-RELATED MACULAR DEGENERATION.

PURPOSE: Recent studies have suggested that the use of systemic beta-blockers, angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers can induce regression of choroidal neovascularization in rodent models. The purpose of this study is to evaluate if these agents have a protective effect against the development of choroidal neovascularization in patients with age-related macular degeneration. METHODS: In this single-center retrospective case-control study, the charts of 250 patients with neovascular age-related macular degeneration were compared with those of 250 controls with dry age-related macular degeneration. Charts were reviewed for current and past use of beta-blockers, angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers. Frequency tables were generated, and associations were examined using chi-square tests, t-tests, and multivariate logistic regression. RESULTS: There was no statistically significant difference between rates of beta-blocker use (P = 0.57), angiotensin-converting enzyme inhibitors use (P = 0.20), or angiotensin receptor blockers use (P = 0.61) between the 2 groups. Additionally, there was no statistically significant difference between rates of use of combinations of the above drugs between the two groups. CONCLUSION: Although there is growing evidence that beta-blockers, angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers can induce regression of choroidal neovascularization in rodent models, these medications do not seem to confer a protective effect against the development of choroidal neovascularization in patients with age-related macular degeneration.

Acute, Treatment-Naïve Branch Retinal Vein Occlusion in Younger Individuals: Risk Factors and Clinical Outcomes.

Purpose: To compare the risk factors and clinical outcomes in patients younger than 50 years with acute, treatment-naïve branch retinal vein occlusion (BRVO) with outcomes in patients 50 years or older. Methods: Patients diagnosed with acute, treatment-naïve BRVO at Duke Eye Center over a 9.5-year period who had BRVO with onset 3 months or less before presentation, BRVO with macular involvement, and 12 months or more of follow-up were included. Demographic data, presenting clinical features, risk factors, treatment patterns, and clinical outcomes were extracted during a retrospective review of medical records. Results: Of 302 patients identified, 23 were younger than 50 years (younger group) and 279 were 50 years or older (older group). Compared with older patients, younger patients had similar rates of hypertension (P = .275), diabetes mellitus (P = 1.000), smokers (P = .787), and open-angle glaucoma (P = .628). The younger group had a lower rate of hyperlipidemia than the older group (35% vs 59%) (P = .028). The 2 groups had similar presenting logMAR visual acuities (VAs) in the BRVO eye (P = .131). At the final follow-up, younger patients had significantly better logMAR VA in the BRVO-affected eye than older patients (mean 0.51 ± 0.65 vs 1.01 ± 1.20) (P = .016). The 2 groups had similar treatment burdens at 1 year (P = .516) and at the final follow-up (P = .782). Conclusions: Younger patients with acute, treatment-naïve BRVO have similar risk factors and treatment patterns as older patients, except for a lower rate of hyperlipidemia. Younger patients with BRVO may have similar presenting VA as older BRVO patients but better final VA, suggesting that age may be a potential prognostic factor.

Characterization of autoimmune eye disease in association with Down's syndrome.

BACKGROUND: Autoimmunity and deficiency of the transcription factor autoimmune regulator protein (AIRE) are known associations with Down syndrome (DS). Lack of AIRE abrogates thymic tolerance. The autoimmune eye disease associated with DS has not been characterized. We identified a series of subjects with DS (n = 8) and uveitis. In three consecutive subjects, we tested the hypothesis that autoimmunity to retinal antigens might be a contributing factor. SUBJECTS/METHODS: This was a multicentred, retrospective case series. Deidentified clinical data of subjects with both DS and uveitis were collected via questionnaire by uveitis-trained ophthalmologists. Anti-retinal autoantibodies (AAbs) were detected using an Autoimmune Retinopathy Panel tested in the OHSU Ocular Immunology Laboratory. RESULTS: We characterized eight subjects (mean age 29 [range, 19-37] years). The mean age of detected uveitis onset was 23.5 [range, 11-33] years. All eight subjects had bilateral uveitis (p < 0.001 based on comparison to published university referral patterns), with anterior and intermediate uveitis found in six and five subjects respectively. Each of three subjects tested for anti-retinal AAbs was positive. Detected AAbs included anti-carbonic anhydrase II, anti-enolase, anti-arrestin, and anti-aldolase. DISCUSSION: A partial deficiency in the AIRE on chromosome 21 has been described in DS. The similarities in the uveitis presentations within this patient group, the known autoimmune disease predisposition in DS, the recognized association of DS and AIRE deficiency, the reported detection of anti-retinal antibodies in patients with DS in general, and the presence of anti-retinal AAbs in three subjects in our series supports a causal association between DS and autoimmune eye disease.

Resolution of outer retinal abnormalities in eyes with vitreomacular traction without macular hole in the OASIS trial.

BACKGROUND/AIMS: To describe the clinical impact of external limiting membrane (ELM) disruption, ellipsoid zone (EZ) disruption and subretinal fluid (SRF) seen on optical coherence tomography (OCT) in eyes with vitreomacular traction (VMT) without macular hole (MH) in the Ocriplasmin for Treatment for Symptomatic Vitreomacular Adhesion Including Macular Hole study. METHODS: Phase 3b randomised double-blind sham-controlled multicentre study including 144 eyes with VMT without MH. Eyes were randomised to receive a single intravitreal injection of ocriplasmin or sham injection and were followed for 24 months. Eyes were analysed for presence, course and clinical impact of ELM disruption, EZ disruption and SRF on OCT. RESULTS: ELM disruption, EZ disruption and SRF were present in 32.6%, 52.2% and 45.8% of ocriplasmin-treated eyes and 39.6%, 42.6% and 37.5% of sham-treated eyes at baseline. VMT resolution was associated with resolution of ELM and EZ disruption and SRF. A small number of eyes had persistent ELM disruption, EZ disruption and/or SRF at the seventh visit or later (17 months or later) following medical or surgical VMT resolution. Resolution of ELM disruption, EZ disruption and/or SRF was associated with an improvement of visual acuity from baseline. Following VMT resolution, ELM recovery usually preceded EZ recovery and SRF resolution. CONCLUSIONS: ELM disruption, EZ disruption and/or SRF are present in a significant percentage of eyes with VMT without MH. Release of VMT is usually associated with outer retinal recovery and an associated improvement in visual acuity. ELM recovery typically precedes EZ recovery and SRF resolution following VMT release.

Therapeutic Outcomes of Non-Infectious Scleritis Treated with Tumor Necrosis Factor-Alpha Inhibitors.

PURPOSE: We determine the efficacy of tumor necrosis factor-α (TNF) inhibitors in establishing scleritis quiescence. METHODS: We conducted a multicenter retrospective chart review of patients with non-infectious scleritis treated with a TNF inhibitor for at least 6 months. The primary endpoint was scleritis quiescence at 6 months. Secondary endpoints included scleritis quiescence at 12 months, TNF inhibitor effects on concurrent doses of systemic corticosteroids and visual acuity outcomes at 6 and 12 months. RESULTS: At 6 months, 82.2% (37/45) of subjects obtained scleritis quiescence with TNF inhibition. At 12 months, 76.2% (32/42) of subjects remained quiescent. Baseline daily corticosteroid use (21.5 ± 21.6 mg) decreased to 5.4 ± 8.3 mg by 6 months (p < 0.0001) and 2.8 ± 6.1 mg by 12 months (p < 0.001). There was no significant difference between the baseline and 6-month BCVA (p = 0.52). CONCLUSIONS: TNF inhibitors are an effective scleritis therapy with significant systemic corticosteroid sparing effect.

A Comparison of Peel-Induced Maculopathy Following ILM Peeling Using a Microvacuum Pick Versus Forceps.

OBJECTIVE: To compare peel-induced maculopathy (PIM) using surgical forceps versus the microvacuum pick (MVP). METHODS: Consecutive eyes undergoing internal limiting membrane (ILM) peeling using either the MVP or forceps were assessed. En face optical coherence tomography (OCT) images at the level of the nerve fiber layer were generated for 6-month postoperative visit. The percentage of the imaged area showing PIM was termed the PIM index. PIM severity was additionally measured using a qualitative PIM severity scale. RESULTS: Seventy-four consecutive eyes underwent ILM peeling with either the MVP (36/74; 49%) or forceps (38/74; 51%). At month-6 postoperatively, the mean PIM index for forceps was 7.7% vs 4.7% for the MVP (P < 0.001, R2 = 0.15). At 6 months, 26/38 eyes (68.5%) in the forceps group had either moderate or severe PIM compared to 12/36 eyes (33.3%) in the MVP group (P = 0.001). CONCLUSIONS: ILM peeling with the MVP resulted in lower PIM severity compared to forceps. [Ophthalmic Surg Lasers Imaging Retina 2023;54:37-42.].

Longitudinal Assessment of the Choroidal Vascularity Index in Eyes with Branch Retinal Vein Occlusion-Associated Cystoid Macular Edema.

INTRODUCTION: Cystoid macular edema (CME) is the most common cause of central vision loss in eyes with branch retinal vein occlusion (BRVO eyes). In recent literature, choroidal vascularity index (CVI) has been proposed to be an enhanced depth imaging optical coherence tomography (EDI-OCT) metric that may help characterize choroidal vascular changes in the setting of retinal ischemia, and potentially prognose visual outcomes and treatment patterns for patients with BRVO-related CME. This study sought to further characterize choroidal vascular changes in BRVO by comparing the CVI, subfoveal choroidal thickness (SFCT), and central subfield thickness (CST) in BRVO eyes with CME compared to unaffected fellow eyes. METHODS: This was a retrospective cohort study. Subjects included treatment-naïve BRVO eyes with CME diagnosed within 3 months of onset of symptoms and unaffected fellow eyes. EDI-OCT images were collected at baseline and at the 12-month follow-up visit. CVI, SFCT, and CST were measured. Demographics, treatment patterns, and best-corrected visual acuity (VA) were abstracted. Median CVI, SFCT, CST, and VA were compared between the two cohorts. Longitudinal relationships between these variables were analyzed. RESULTS: A total of 52 treatment-naïve eyes with BRVO and CME and 48 unaffected fellow eyes were identified. Baseline CVI was lower in eyes with BRVO than in fellow eyes (64.7% vs. 66.4%, P = 0.003). At 12 months, there was no difference in CVI between BRVO eyes and fellow eyes (65.7% vs 65.8%, P = 0.536). In BRVO eyes, there was a strong correlation between reduced CST and improved VA over the 12-month study period (r = 0.671, P < 0.001). CONCLUSION: There are differences in CVI in treatment-naïve BRVO eyes with CME at presentation compared to fellow eyes, but these differences resolve over time. Anatomic changes in macular thickness in BRVO eyes with CME may be correlated with VA outcomes.

Our study evaluated a novel ocular optical coherence tomography imaging metric, the choroidal vascularity index, in eyes that developed cystoid macular edema, a condition which can significantly impair acuity of central vision, after being diagnosed with branch retinal vein occlusion. In each patient, we compared the choroidal vascularity index in eyes that developed treatment-naïve, newly diagnosed branch retinal vein occlusion with cystoid macular edema to the non-diseased fellow eye. We made comparisons at the time of diagnosis (baseline) and at the 12-month follow up, and analyzed changes over time. We found that at the baseline visit, branch retinal vein occlusion eyes with cystoid macular edema had a significantly lower choroidal vascularity index than their unaffected fellow eyes, but that the differences between eyes resolved by the 12-month follow-up visit. Our findings suggest that choroidal vascularity may be compromised in the acute phase of branch retinal vein occlusion, but that this phenomenon resolves over time. Future research should further evaluate whether imaging characteristics of choroidal vascularity may be associated with changes in anatomic and visual outcomes in retinal diseases.

INTERMEDIATE UVEITIS WITH RETINAL DETACHMENT IN A PATIENT WITH POMPE DISEASE.

PURPOSE: To describe the ocular findings in a patient with glycogen storage disease II (Pompe disease). METHODS: Case report. RESULTS: A 14-year-old boy with Pompe disease was referred for evaluation of a retinal detachment in the left eye. Indirect ophthalmoscopy revealed bilateral fibrotic snowbanks and an inferior rhegmatogenous retinal detachment extending into the macula. Fluorescein angiography revealed mild diffuse perivascular leakage in both eyes. The retinal detachment was repaired with scleral buckling and cryotherapy. Workup for the etiology of the intermediate uveitis was unrevealing. CONCLUSION: Enzyme replacement therapy has improved the survival of individuals with Pompe disease. With greater patient longevity, new ocular associations may continue to emerge. Whether intermediate uveitis is an ocular association of Pompe disease remains to be determined.