OUTCOMES OF INFECTIOUS PANUVEITIS ASSOCIATED WITH SIMULTANEOUS MULTI-POSITIVE OCULAR FLUID POLYMERASE CHAIN REACTION.

PURPOSE: To evaluate features of infectious panuveitis associated with multiple pathogens detected by ocular fluid sampling. METHODS: Single-center, retrospective, consecutive case series of patients with aqueous/vitreous polymerase chain reaction testing with >1 positive result in a single sample from 2001 to 2021. RESULTS: Of 1,588 polymerase chain reaction samples, 28 (1.76%) were positive for two pathogens. Most common pathogens were cytomegalovirus (n = 16, 57.1%) and Epstein-Barr virus (n = 13, 46.4%), followed by varicella zoster virus (n = 8, 28.6%), Toxoplasma gondii (n = 6, 21.4%), herpes simplex virus 2 (n = 6, 21.4%), herpes simplex virus 1 (n = 6, 21.4%), and Toxocara (n = 1, 3.6%). Mean initial and final visual acuity (logarithm of the minimum angle of resolution) were 1.3 ± 0.9 (Snellen ∼20/400) and 1.3 ± 1.1 (Snellen ∼20/400), respectively. Cytomegalovirus-positive eyes (n = 16, 61.5%) had a mean final visual acuity of 0.94 ± 1.1 (Snellen ∼20/175), whereas cytomegalovirus-negative eyes (n = 10, 38%) had a final visual acuity of 1.82 ± 1.0 (Snellen ∼20/1,320) ( P < 0.05). Main clinical features included intraocular inflammation (100%), retinal whitening (84.6%), immunosuppression (65.4%), retinal hemorrhage (38.5%), and retinal detachment (34.6%). CONCLUSION: Cytomegalovirus or Epstein-Barr virus were common unique pathogens identified in multi-PCR-positive samples. Most patients with co-infection were immunosuppressed with a high rate of retinal detachment and poor final visual acuity. Cytomegalovirus-positive eyes had better visual outcomes compared with cytomegalovirus-negative eyes.

VALSALVA RETINOPATHY: Clinical Features and Treatment Outcomes.

PURPOSE: To report the clinical presentation and outcomes in patients with Valsalva retinopathy. METHODS: This was a retrospective case series of patients diagnosed with Valsalva retinopathy between June 1, 2010, and May 31, 2020. Clinical notes, operative reports, fundus photography, and optical coherence tomography images were reviewed. RESULTS: This study comprised 58 eyes of 58 patients. The most common causes were lifting (34.4%), vomiting (20.6%), straining (20.6%), and coughing (17.2%). Mean best-corrected visual acuity at diagnosis was 20/163. The most frequently involved vitreoretinal compartment was the subhyaloid space (42.3%) followed by the intraretinal (32.7%), intravitreal (23.1%), and subretinal (13.4%) spaces. Mean best-corrected visual acuity of all patients was 20/59 at 3 months, 20/48 at 6 months, and 20/22 at 1 year. Mean time to clearance of hemorrhage on clinical examination was 99.0 ± 18.7 days in patients who underwent observation and 4.5 ± 3.5 days after surgery in patients who received pars plana vitrectomy. CONCLUSION: Valsalva retinopathy is generally associated with a favorable visual prognosis. Most eyes perform well with observation although pars plana vitrectomy may be indicated in patients requiring rapid resolution of hemorrhage.

ACUTE- AND DELAYED-ONSET ENDOPHTHALMITIS AFTER OPEN GLOBE INJURIES: Clinical Features and Visual Acuity Outcomes.

PURPOSE: The purpose of the study was to report the clinical features and best-corrected visual acuity outcomes in patients with acute- and delayed-onset endophthalmitis after open globe injuries. METHODS: The study included a retrospective, comparative, consecutive case series of patients with endophthalmitis after open globe injury between January 2016 and October 2020 at the Bascom Palmer Eye Institute. RESULTS: Acute-onset endophthalmitis accounted for 16 of 20 cases (80%), and all cases were diagnosed at the initial examination. Delayed-onset endophthalmitis cases, occurring more than 2 weeks after injury, accounted for 4 of 20 cases (20%) and were because of Zone 1 wound leaks and infections. Factors associated with endophthalmitis included presence of a retained intraocular foreign body (11/20 [55%]) and delay of presentation >24 hours (15/20 [75%]) ( P < 0.001 and 0.002, respectively). The mean presenting best-corrected visual acuity was logMAR 1.64 (20/800), and the mean best-corrected visual acuity at the last follow-up was logMAR 1.22 (20/300). CONCLUSION: In patients with open globe injury-related endophthalmitis, visual acuity outcomes are generally poor. Despite intravitreal antibiotics at primary closure, delayed-onset endophthalmitis cases may develop in the setting of compromised Zone 1 wound integrity.

NATIONWIDE DEMOGRAPHIC DISPARITIES IN UNITED STATES EMERGENCY DEPARTMENT VISITS IN PATIENTS WITH RHEGMATOGENOUS RETINAL DETACHMENT.

BACKGROUND/PURPOSE: Within the evolving landscape of healthcare in the United States (US), delineating the demographic nuances and financial implications of emergent conditions, such as rhegmatogenous retinal detachment (RRD), is paramount. This study seeks to analyze the demographic and hospital billing amount/cost of service disparities in RRD visits to emergency departments (EDs) nationwide. METHODS: We conducted a retrospective, cross-sectional, population-based study using International Classification of Diseases, 10th revision , and Current Procedural Terminology codes in the 2016 to 2019 Nationwide Emergency Department Sample databases to identify RRD visits. The analysis included demographics, hospital billing amount, and cost of service of RRD ED management. RESULTS: A total of 12,492 RRD encounters were identified with men constituting 64% and a prominent age group being 50 to 64 years (49.3%). Most patients (90%) were managed in metropolitan teaching hospitals, predominantly in the southern U.S. region (56.1%). Private insurance covered 45% of patients. Same-day RRD repair odds increased in November and December. Whites had a higher likelihood for same-day treatment. Hospital billing amount rose from $23,600 in 2016 to $30,354 in 2019, with stable mean total cost of service. Rhegmatogenous retinal detachment ED visit incidence did not show seasonal variation ( P = 0.819). CONCLUSION: Most patients with RRD in U.S, EDs were middle-aged men, with Whites more likely to receive same-day repair. There was no sex bias observed in same-day repair decision-making. Although hospital billing amount increased over the study period, total cost of service remained stable. The incidence of RRD ED visits showed no seasonal variation.

INTRAOCULAR INFLAMMATION INCIDENCE AFTER INTRAVITREAL BROLUCIZUMAB INJECTION FOR EXUDATIVE AGE-RELATED MACULAR DEGENERATION.

PURPOSE: We evaluated the clinical outcomes of intraocular inflammation (IOI) of eyes with neovascular age-related macular degeneration (AMD) injected with brolucizumab in our tertiary referral center. METHODS: A retrospective case series for which clinical records of all eyes that received intravitreal brolucizumab at Bascom Palmer Eye Institute between December 1, 2019, and April 1, 2021, were reviewed. RESULTS: There were 345 eyes of 278 patients who received 801 brolucizumab injections. IOI was detected in 16 eyes of 13 patients (4.6%). In those patients, baseline Logarithm of Minimu Angle of Resolution (logMAR) best-corrected visual acuity was 0.32 0.2 (20/42), while it was 0.58 0.3 (20/76) at IOI presentation. The mean number of injections among eyes experiencing IOI was 2.4, and the interval between the last brolucizumab injection and IOI presentation was 20 days. There was no known case of retinal vasculitis. Management of IOI included topical steroids in seven eyes (54%), topical and systemic steroids in five eyes (38%), and observation in one eye (8%). Best-corrected visual acuity returned to baseline and inflammation resolved in all eyes by the last follow-up examination. CONCLUSION: Intraocular inflammation after brolucizumab injection for neovascular AMD was not uncommon. Inflammation resolved in all eyes by the last follow-up visit.

EVALUATION OF RETINAL DISPLACEMENT FOLLOWING PRIMARY SCLERAL BUCKLING FOR MACULA-INVOLVING RHEGMATOGENOUS RETINAL DETACHMENT.

PURPOSE: To evaluate the incidence and degree of retinal displacement following scleral buckling surgery for macula-involving rhegmatogenous retinal detachment. METHODS: Retrospective interventional case series comprised of patients treated with primary scleral buckling procedure without gas tamponade for macula-involving rhegmatogenous retinal detachment and imaged postoperatively with fundus autofluorescence imaging between June 1, 2016 and July 25, 2021. Clinical notes, operative reports, fundus autofluorescence photographs, and optical coherence tomography images were reviewed. The presence and degree of retinal displacement were recorded. RESULTS: Twelve eyes of 11 patients were included. One (8%) eye with an epiretinal membrane demonstrated 0.1 mm of retinal displacement along the superior arcade and in the superotemporal periphery. The remainder of eyes (92%) did not show any identifiable signs of retinal displacement. CONCLUSION: Retinal displacement does not seem to be a frequent complication of primary scleral buckling surgery for macula-involving rhegmatogenous retinal detachment.

TRENDS IN INTRAVITREAL CORTICOSTEROID AGENT USE BY US OPHTHALMOLOGISTS IN MEDICARE BENEFICIARIES AND ASSOCIATION WITH PHYSICIAN-INDUSTRY INTERACTIONS.

PURPOSE: To report trends of intravitreal corticosteroid use and explore the relationship between career experience, reported industry payments, and prescribing habits. METHODS: A retrospective review of ophthalmologists who administered intravitreal dexamethasone implants (DEX) and triamcinolone acetonide (TA) injections between August 2013 and December 2017. RESULTS: A total of 1,070 US ophthalmologists were reimbursed by Medicare for 522,804 DEX injections and 2.6 million TA injections. There was a significant positive trend in the number of DEX (P = 0.01), but not TA, injections per year. Mid-career and late-career physicians performed significantly greater total injections on average compared with early-career physicians (both P < 0.001). Early-career physicians performed a greater proportion of DEX injections than late-career physicians (P = 0.006). Industry payments were positively associated with the proportion of DEX used and inversely correlated with the proportion of TA administered (P < 0.001). On multivariate analysis, years in practice, number of payments, and total value of payments were significantly associated with the number of DEX injections administered (all P < 0.001). CONCLUSION: From 2013 to 2017, the use of DEX increased, whereas TA use remained stable. There was a positive association between DEX use and physician-industry interactions, which may be explained by seniority and experience. This study does not define a causal relationship.

GENDER DIFFERENCES IN SELF-REPORTED PROCEDURAL VOLUME AMONG VITREORETINAL FELLOWS.

PURPOSE: A prior study revealed discrepancies in self-reported surgical numbers between male and female ophthalmology residents. This study further investigates the gender differences in self-reported procedural volume among vitreoretinal surgery fellows and examines the differences for surgical, medical, and total self-reported procedural volume between male and female vitreoretinal fellows. METHODS: A retrospective review of case logs submitted to the American Society of Retina Specialists by first-year and second-year vitreoretinal fellows from July 1, 2018, to June 30, 2019, was performed. Fellows who reported fewer than 100 pars plana vitrectomies were excluded. A total of 133 fellows were included. RESULTS: Overall, 37 of 57 (65%) first-year fellows and 59 of 76 (78%) second-year fellows were male. An average of 1,120 procedures were self-reported among all vitreoretinal fellows. In the group of second-year fellows at the completion of fellowship, men reported more total procedures (1,171 [864-1,600] vs. 1,005 [719-1,257]; P = 0.072). Women reported statistically significant fewer endolaser (P = 0.018), internal limiting membrane peel (P = 0.042), and cryoretinopexy (P = 0.002) procedures compared with men. When splitting the data by total surgical versus medical procedures, men reported more procedures than women both surgically (1,077 [799-1,490] vs. 925 [622-1,208]; P = 0.085) and medically (72 [41-116] vs. 56 [20-94]; P = 0.141), although these differences were not statistically significant. CONCLUSION: There is a trend for female vitreoretinal fellows to report fewer surgical procedures than their male counterparts, raising concerns for gender gaps in vitreoretinal surgical training. Further research is needed to verify this discrepancy and identify potential barriers that female vitreoretinal surgeons are facing in training.

ANALYSIS OF THE VITREORETINAL SURGICAL FELLOWSHIP APPLICANT POOL: Publication Misrepresentations and Predictors of Future Academic Output.

PURPOSE: To evaluate characteristics associated with misrepresentation of publication record, future career placement, and subsequent academic output among vitreoretinal surgical fellowship applicants. METHODS: A retrospective review of 337 vitreoretinal surgical applicants between 2015 and 2018 was conducted. Publications reported in the applications were verified using PubMed, Google, and Google Scholar. Applications were considered misrepresented if there was no record of the publication or if there was an inconsistency in authorship. Applicants were followed after graduation and their employment position and postgraduation publications were recorded. The main outcome measures were the number of unverifiable publications, postfellowship job placement, and postgraduate peer-reviewed publications. RESULTS: Of the 377 applicants, 309 (82.0%) listed peer-reviewed publications. Of those with a publication, 32 (10.4%) had misrepresentations. A reported desire to pursue an academic career was associated with a future career in academic medicine, whereas Alpha Omega Alpha status was correlated with a future career in private practice. Alpha Omega Alpha status, a reported desire to pursue an academic career, and the number of peer-reviewed publications before fellowship were positively correlated with higher numbers of peer-reviewed publications after fellowship. CONCLUSION: Unverifiable authorship among vitreoretinal surgical fellowship applicants is significant, affecting nearly one in 10 applicants with peer-reviewed publications. A reported desire to pursue academic medicine as listed on the fellowship application is a useful indicator for a future career in academics, and for increased number of peer-reviewed publications after fellowship.

Long-term Visual Outcomes and Causes of Vision Loss in Chronic Central Serous Chorioretinopathy.

PURPOSE: To evaluate the long-term visual outcomes and causes of vision loss in chronic central serous chorioretinopathy (CSC). DESIGN: Retrospective, longitudinal study. PARTICIPANTS: A total of 133 participants (217 eyes) with chronic CSC. METHODS: A retrospective review of clinical and multimodal imaging data of patients with chronic CSC managed by 3 of the authors between May 1977 and March 2018. Multimodal imaging comprised color photography, fluorescein angiography, indocyanine green angiography, fundus autofluorescence (FAF), and OCT. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA) at the final visit; change in BCVA between first visit and 1-, 5-, and 10-year follow-up visits; and causes of vision loss at final visit. RESULTS: Data from 6228 individual clinic visits were analyzed. Mean age of patients at the first visit was 60.7 years, and mean period of follow-up from first to last visit was 11.3 years. The cohort included 101 male patients (75.9%). At the final visit, 106 patients (79.7%) maintained driving-standard vision with BCVA of 20/40 or better in at least 1 eye, and 17 patients (12.8%) were legally blind with BCVA of 20/200 or worse in both eyes. Mean BCVA at first visit was not significantly different from mean BCVA at 1- or 5-year follow-up visits (both P ≥ 0.65) but was significantly better than the mean BCVA at the 10-year follow-up visit (P = 0.04). Seventy-nine percent of eyes with 20/40 or better vision at the first visit maintained the same level of vision at the 10-year follow-up visit. Ninety-two percent of eyes with 20/200 or worse vision at the first visit maintained the same level of vision at the 10-year follow-up visit. Cystoid macular degeneration, choroidal neovascularization (CNV), outer retinal disruption on OCT, and FAF changes were associated with poorer vision at the final visit (all P ≤ 0.001). Multivariable analysis revealed that greater age at first visit was associated with greater BCVA change at the 10-year follow-up visit (P = 0.001). CONCLUSIONS: Chronic CSC can be a sight-threatening disease leading to legal blindness. Age at presentation and outer retinal changes on multimodal imaging were associated with long-term BCVA changes and may be predictors of long-term visual outcomes.

Cost Evaluation of Early Vitrectomy versus Panretinal Photocoagulation and Intravitreal Ranibizumab for Proliferative Diabetic Retinopathy.

PURPOSE: To evaluate costs and cost-utility of early vitrectomy (pars plana vitrectomy [PPV]) compared with panretinal photocoagulation (PRP) and intravitreal ranibizumab (IVR) for proliferative diabetic retinopathy (PDR) without diabetic macular edema. DESIGN: A decision analysis model of cost-utility. PARTICIPANTS: There were no participants. METHODS: A decision analysis was based on results from the Diabetic Retinopathy Clinical Research Network Protocol S comparing treatment of PRP with IVR (0.3 mg) in PDR without incident macular edema to model the total 2-year costs and outcomes for each treatment scenario. These values were compared with the 2-year hypothetical costs of early PPV for PDR. Centers for Medicare and Medicaid Services data were used to calculate associated modeled costs in a hospital/facility-based and nonfacility setting. Cost-utility was calculated on the basis of the preserved visual utility and estimated life years remaining. In addition, costs for lifetime treatment were modeled for all scenarios and used to calculate lifetime quality-adjusted life years (QALY) costs for each scenario. Sensitivity analyses were performed to evaluate the impact of the model's assumptions. MAIN OUTCOME MEASURES: Cost of treatment, utility, and cost per QALY. RESULTS: The modeled cost per QALY of treatment for PDR for 2 years of utility in the facility (nonfacility) setting was $163 988 ($102 559) in the PRP group, $436 992 ($326 424) in the IVR group, and $181 144 ($107 965) in the PPV group. Sensitivity analysis showed that both IVR and PPV groups would have equivalent costs per QALY over the first 2 years if 78% (facility) and 80% (nonfacility) of patients in the PPV group required additional treatment with IVR (at the dose of 10.1 injections as in Protocol S). Beyond 2 years, the cost per QALY in the facility (nonfacility) setting was calculated as $61 695 ($21 752) in the PRP group, $338 348 ($239 741) in the IVR group, and $63 942 ($22 261) in the PPV group. CONCLUSIONS: Early PPV as a strategy for treatment of PDR without macular edema demonstrates cost-utility similar to management with PRP and more favorable cost-utility compared with IVR in the short term. This advantage over IVR continues when lifetime costs are factored.

Cost-Utility of Evaluation for Posterior Vitreous Detachment and Prophylaxis of Retinal Detachment.

PURPOSE: To evaluate the costs and cost-utility of examination for posterior vitreous detachment (PVD) and treatment of associated pathology, and of managing various other peripheral retinal disorders to prevent retinal detachment (RD). DESIGN: A decision analysis model of cost-utility. PARTICIPANTS: There were no participants. METHODS: Published retrospective data on the natural course of PVD, retinal tears, and lattice degeneration were used to quantitate the visual benefits of examination and treatment. Center for Medicare and Medicaid Services data were used to calculate associated modeled costs in a hospital/facility-based and nonfacility/ambulatory surgical center (ASC)-based setting. Published standards of utility for a given level of visual acuity were used to derive costs and quality-adjusted life years (QALYs). MAIN OUTCOME MEASURES: Cost of evaluation and treatment, utility of defined health states, QALY, and cost per QALY. RESULTS: The modeled cost of evaluation of a patient with PVD and treatment of associated pathology in the facility/hospital (nonfacility/ASC)-based setting was $65 to $190 ($25-$71) depending on whether a single or 2-examination protocol was used. The cost per QALY saved was $255 to $638/QALY ($100-$239/QALY). Treatment of a symptomatic horseshoe tear resulted in a net cost savings of $1749 ($1314) and improved utility, whereas treatment of an asymptomatic horseshoe tear resulted in $2981/QALY ($1436/QALY). Treatment of asymptomatic lattice degeneration in an eye in which the fellow eye had a history of RD resulted in $4414/QALY ($2187/QALY). CONCLUSIONS: Evaluation and management of incident acute PVD (and symptomatic horseshoe tears) offer a low cost and a favorable cost-utility (low $/QALY) as a result of the minimization of the cost and morbidity associated with the development of RD, thus justifying current practice standards.

INTERNAL LIMITING MEMBRANE PEELING DURING PARS PLANA VITRECTOMY FOR RHEGMATOGENOUS RETINAL DETACHMENT: Cost Analysis, Review of the Literature, and Meta-analysis.

PURPOSE: To evaluate the visual and potential economic impact of primary internal limiting membrane (ILM) peeling in primary treatment of rhegmatogenous retinal detachment. METHODS: A PubMed search was performed to extract data regarding the rate of epiretinal membrane formation and the rate of secondary pars plana vitrectomy with membrane peel after repair of rhegmatogenous retinal detachment with or without ILM peeling. Data were aggregated and analyzed in a meta-analysis. This information was used to perform a cost analysis to determine the economic ramifications of primary ILM peeling. RESULTS: Six included studies compared the outcomes of eyes receiving pars plana vitrectomy for rhegmatogenous retinal detachment repair with and without primary ILM peel. The cumulative rate of epiretinal membrane formation was 29% (86/295) in the eyes without ILM peel and 3% (8/289) in the eyes with ILM peel. The cumulative rate of secondary pars plana vitrectomy/membrane peel was 16% (22/141) in the eyes without ILM peel and 0% (0/158) in the eyes with ILM peel. The weighted summary point estimate odds ratio was 0.083 (95% confidence interval 0.042-0.164), indicating a statistically significant protective effect across the 6 studies of ILM peeling and the development of epiretinal membrane. Based on published data, the average dollars saved by conducting a primary ILM peel was $615 in a facility setting and $364 in an ambulatory surgical center. CONCLUSION: Published, mainly retrospective, data suggest that primary ILM peel in rhegmatogenous retinal detachment repair may have a significant reduction in the rate of postoperative epiretinal membrane and may lessen the need for secondary pars plana vitrectomy/membrane peel. Furthermore, from an economic perspective, the reduction in the need for secondary surgery may justify the higher cost with primary ILM peel.

ULTRA-WIDEFIELD IMAGING OF POSTERIOR SEGMENT PATHOLOGY IN THE SETTING OF THE BOSTON KERATOPROSTHESIS.

PURPOSE: To evaluate the ability to visualize and document posterior segment pathology through the Boston keratoprosthesis (KPro) using the Optos P200Tx ultra-widefield (UWF) scanning laser ophthalmoscope. METHODS: A retrospective chart review was conducted of patients who underwent Boston Type I keratoprosthesis implantation and who subsequently were imaged with an UWF system. Ultra-widefield images were reviewed to evaluate for vitreoretinal pathology and were compared with the clinical examination. RESULTS: In this series of 10 patients (10 eyes), 100% of vitreoretinal pathology found on clinical examination was detectable using the Optos system. In 4 cases (40%), UWF imaging provided superior detection of pathology compared with the clinical examination by imaging through retroprosthetic membranes (3 cases) and by detection of a retinal detachment (one case). In 1 case (10%), B-scan ultrasonography was needed to characterize vitreoretinal pathology that could not be definitively distinguished on UWF imaging and was difficult to detect on clinical examination. Ultra-widefield imaging detected the following vitreoretinal pathologies in KPro eyes: retinal hemorrhage, epiretinal membrane, retinal detachment, proliferative diabetic retinopathy, and choroidal folds. CONCLUSION: Ultra-widefield imaging provides a high-resolution view of the posterior pole and periphery despite the limitations of imaging through the narrow optic of Boston Type I keratoprosthesis, and it may improve visualization through retroprosthetic membranes. Detection and documentation of vitreoretinal complications in the setting of a permanent keratoprosthesis may be enhanced using UWF imaging.

Feasibility and clinical utility of ultra-widefield indocyanine green angiography.

PURPOSE: To evaluate the feasibility and clinical utility of a novel noncontact scanning laser ophthalmoscope-based ultra-widefield indocyanine green angiographic system. METHODS: Ultra-widefield indocyanine green angiographic images were captured using a modified Optos P200Tx that produced high-resolution images of the choroidal vasculature with up to a 200° field. Ultra-widefield indocyanine green angiography was performed on patients with a variety of retinal conditions to assess utility of this imaging technique for diagnostic purposes and disease treatment monitoring. RESULTS: Ultra-widefield indocyanine green angiography was performed on 138 eyes of 69 patients. Mean age was 58 ± 16.9 years (range, 24-85 years). The most common ocular pathologies imaged included central serous chorioretinopathy (24 eyes), uveitis (various subtypes, 16 eyes), age-related macular degeneration (12 eyes), and polypoidal choroidal vasculopathy (4 eyes). In all eyes evaluated with ultra-widefield indocyanine green angiography, high-resolution images of choroidal and retinal circulation were obtained with sufficient detail out to 200° of the fundus. CONCLUSION: In this series of 138 eyes, scanning laser ophthalmoscope-based ultra-widefield indocyanine green angiography was clinically practical and provided detailed images of both the central and peripheral choroidal circulation. Future studies are needed to refine the clinical value of this imaging modality and the significance of peripheral choroidal vascular changes in the diagnosis, monitoring, and treatment of ocular diseases.

Multifocal choroiditis without panuveitis: clinical characteristics and progression.

PURPOSE: To describe the clinical characteristics and progression of patients with multifocal choroiditis lesions who had minimal or no evidence of anterior uveitis and/or vitritis. METHODS: Retrospective, observational, single-center consecutive case series. Clinical histories, examination, and multimodal imaging findings were analyzed. RESULTS: Sixty-five eyes of 41 patients were identified. The mean age at diagnosis was 38.4 years (median, 35 years; range, 15-81 years), and 70.7% of the patients were women. Involvement was bilateral in 21 patients (51.2%) at presentation. The 60-month bilateral event-free survival was 75.0% (95% confidence interval, 49.8-91.2%). The mean visual acuity was 20/46 (median, 20/25; range, 20/20 to count fingers at 2 feet) at presentation and 20/42 (median, 20/25; range, 20/20-5/400) at the last recorded visit. The 60-month "20/50 or worse" event-free survival was 100%. Between the first presentation and final follow-up (a mean duration of 92.6 months; range, 0-343 months), 46.7% of the eyes developed new or larger chorioretinal spots and 32.6% developed new or recurrent choroidal neovascularization. The 60-month choroidal neovascularization event-free survival was 68.1% (95% confidence interval, 39.2-85.4%). CONCLUSION: Patients with multifocal choroiditis lesions, but with minimal or no anterior uveitis or vitritis, tended to be young women. Approximately half of the patients presented with bilateral involvement, which is less than has been reported in most case series of multifocal choroiditis with panuveitis. One quarter of all unilaterally affected patients will develop bilateral involvement by 60 months.

RETINAL VASCULAR DISEASE IN LIMB-GIRDLE MUSCULAR DYSTROPHY.

PURPOSE: To report bilateral retinal vascular occlusive disease in limb-girdle muscular dystrophy (LGMD). METHODS: Case report. RESULTS: A 34-year-old Asian woman was referred for evaluation and management of central retinal vein occlusion. Ultra-wide-field fluorescein angiography showed resolving initial peripheral retinal vein occlusion in one eye and peripheral venular segmental staining in the fellow asymmetric eye. Genetic testing established the diagnosis of LGMD. CONCLUSION: Similar to other forms of muscular dystrophy, LGMD is caused by genetic abnormalities in sarcolemma proteins, a key structural component that connects the intracellular cytoskeleton of a myofiber to the extracellular matrix. Like other muscular dystrophies, LGMD may be associated with retinal vascular abnormalities noted. In this case, retinal vascular smooth muscle dysfunction was seen in LGMD, analogous to reported vascular abnormalities in other muscular dystrophies such as facioscapulohumeral dystrophy and Duchenne muscular dystrophy.