Successful Treatment of Clinically Diagnosed Cancer-Associated Retinopathy with Intravitreal Dexamethasone Implant Followed by 0.18 mg Fluocinolone Implant without Systemic Immunosuppression.

PURPOSE: To report a case of clinically diagnosed cancer-associated retinopathy (CAR) successfully treated with intravitreal corticosteroid implants without systemic immunosuppression. METHODS: Case report with multimodal imaging. RESULTS: An 80-year-old man without known systemic malignancy presented with debilitating shimmering, hemeralopia and rapidly progressive bilateral vision loss following uncomplicated cataract surgery. Mild vitritis, extensive photoreceptor loss, mottling of retinal pigmentary epithelium (RPE), and mild vascular attenuation were found in both eyes. Full field electroretinogram (ffERG) showed severe bilateral rod-cone dysfunction. Infectious etiologies and vitreoretinal lymphoma were ruled out. During cancer workup, intravitreal corticosteroid treatment was offered. Significant anatomical improvement with reconstitution of ellipsoid zone, improved RPE irregularities and functional improvement, were observed 3 weeks after bilateral intravitreal dexamethasone implants (Ozurdex). 2 months later, patient received bilateral intravitreal 0.18mg fluocinolone acetonide implants (YUTIQ). Later, a colonic adenocarcinoma was found (pathologic stage pT3 pN0). Patient recovered well from surgery and no chemotherapy was needed. 9 months since bilateral intravitreal fluocinolone acetonide implants (11 months since bilateral intravitreal dexamethasone implants), best corrected vision maintained at 20/25-2 OD, 20/20 OS without ongoing treatments. Bilateral reconstitution of ellipsoid zones and nearly resolution of RPE irregularities remained stable. Repeat ffERG demonstrated improved cone response OS and stable diminished rod response OU. Patient reports resolution of ocular symptoms. CONCLUSION: The sustained improvements with intravitreal corticosteroid monotherapy suggest potential advantages using local therapy over systemic treatment. Long term follow-up is warranted. Further research is needed to evaluate the efficacy of using 0.18mg fluocinolone implant (YUTIQ) to treat CAR.

Optic Disc Edema Is an Under-Recognized Feature of Birdshot Chorioretinitis.

BACKGROUND: Optic disc edema is a feature of many ophthalmic and neurologic conditions. It remains an underappreciated feature of birdshot chorioretinitis (BSCR), leading to delay in diagnosis and treatment. The purpose of our study was to identify clinical features that are concomitant with optic disc edema and suggest a diagnosis of BSCR. METHODS: Retrospective multicenter case series of 29 patients who were referred to a neuro-ophthalmologist or uveitis specialist for evaluation of disc edema and were ultimately diagnosed with BSCR. RESULTS: Fifty-four eyes of 30 patients, from the practices of 15 uveitis specialists, met the eligibility criteria. In addition to disc edema, concomitant features in all patients included vitritis, chorioretinal lesions, and retinal vasculitis. Visual recovery to 20/40 or better occurred in 26 of 29 patients. Visual acuity remained 20/100 or worse in 2 patients previously diagnosed with idiopathic intracranial hypertension, 1 patient previously diagnosed with optic neuritis, and 1 patient for whom treatment was delayed for years, leading to optic disc atrophy. CONCLUSIONS: Optic disc edema is a presenting feature in some cases of BSCR. A diagnosis of BSCR should be considered when disc edema occurs with vitritis, chorioretinal inflammation, and retinal vasculitis. Patients should be referred to a uveitis specialist for treatment.

Acanthamoeba-associated retinitis successfully treated with intravitreal and systemic antimicrobials.

Purpose: To describe a case of unilateral Acanthamoeba-associated retinitis in the absence of concomitant corneal infection in an immunocompetent host without risk factors. Observations: A 37-year-old woman presented with unilateral multifocal retinitis with minimal vitritis. Anterior segment was normal. Conventional diagnostics of bacterial, fungal, viral, Toxoplasma and Toxocara etiologies all returned negative. Empiric treatments were unsuccessful, including oral valacyclovir, oral fluconazole, as well as intravitreal injection of vancomycin and ceftazidime. Metagenomic deep sequencing (MDS) identified Acanthamoeba genomic fragments in the vitreous sample. Multiple intravitreal voriconazole injections were performed and achieved partial suppression of lesion growth. Subsequent dual therapy of oral voriconazole and trimethoprim-sulfamethoxazole led to resolution of the lesions and vision improvement without further injections. Conclusions and importance: This is an unusual case of unilateral Acanthamoeba-associated retinitis without concomitant corneal infection, diagnosed via unbiased DNA and RNA deep sequencing, with other etiologies ruled out by conventional approaches. Treatment with systemic and intravitreal therapy led to a successful resolution of retinitis and vision improvement. Our case demonstrates the potential of MDS as an unbiased diagnostic tool for rare ocular pathogens and the therapeutic effect of oral voriconazole with trimethoprim-sulfamethoxazole for Acanthamoeba intraocular infection.

Late diagnosis of Cutibacterium acnes (formerly Proprionibacterium acnes) endophthalmitis and the importance of dual testing with bacterial culture and polymerase chain reaction.

PURPOSE: To describe two cases of C. acnes endophthalmitis that reinforce the importance of performing both bacterial culture and 16s polymerase chain reaction when the causative pathogen is unclear or difficult to culture, such as C. acnes. A case of C. acnes endophthalmitis complicated by sub-buckle scleral perforation is illustrated with intraoperative photography. METHODS: Two-case series. RESULTS: Case 1 describes a case of C. acnes endophthalmitis in a longstanding pseudophakic patient following multiple vitrectomies for recurrent retinal detachment, complicated by sub-buckle scleral perforation. Bacterial culture revealed C. acnes while 16s PCR was negative. Conversely, Case 2 demonstrates a case of chronic endophthalmitis diagnosed one year following cataract surgery. PCR (with repeat tap for confirmation) revealed C. acnes with a negative culture. CONCLUSION: When the causative pathogen of endophthalmitis is unclear, dual testing of microbial culture and C. acnes 16s PCR improves the diagnostic yield of investigations for fastidious pathogens. C. acnes can present as an indolent or virulent endophthalmitis.

Diagnosis and Characteristics of Presentation of Tubulointerstitial Nephritis and Uveitis Syndrome During the COVID-2019 Pandemic.

PURPOSE: To compare the diagnosis and clinical features of tubulointerstitial nephritis and uveitis syndrome (TINU) before and during the COVID-19 pandemic. METHODS: Retrospective chart review. RESULTS: Before the COVID-19 pandemic (March 2017 to March 2019), 1/561 (0.18%) new patient was diagnosed with TINU. During the pandemic (March 2020 to March 2022), 15/581 (2.58%) new patients were diagnosed with TINU. We found a significant increase in TINU cases during the pandemic (P=0.0005). Various posterior segment findings were observed in 2/3 (66.7%) patients before the pandemic and 13/15 (86.7%) patients during the pandemic, including disc edema, chorioretinal scars, disc leakage, and peripheral vascular leakage. CONCLUSION: This is the first study reporting an increased number of TINU during the COVID-19 pandemic. With most of the American population now exposed to COVID-19, a large multi-center epidemiological study would be helpful to investigate any association of COVID-19 disease or vaccination with TINU in recent years.

A diet rich in fermentable fiber promotes robust changes in the intestinal microbiota, mitigates intestinal permeability, and attenuates autoimmune uveitis.

Therapeutic approaches for noninfectious uveitis have expanded greatly over the past 10 years, but are limited by potential side effects and limited efficacy. Thus, therapeutic approaches that include less toxic, potentially preventative strategies to manage noninfectious uveitis are essential areas of study. Diets rich in fermentable fiber are potentially preventative in various conditions such as metabolic syndrome and type 1 diabetes. We studied the effects of various fermentable dietary fibers in an inducible model of experimental autoimmune uveitis (EAU) and found that they differentially modulated uveitis severity. A high pectin diet was the most protective, reducing clinical disease severity through the induction of regulatory T lymphocytes and the suppression of Th1 and Th17 lymphocytes at peak ocular inflammation in either intestinal or extra-intestinal lymphoid tissues. The high pectin diet also promoted intestinal homeostasis as shown by changes in intestinal morphology and gene expression, as well as intestinal permeability. Pectin-induced modulation of intestinal bacteria appeared to be associated with protective changes in immunophenotype in the intestinal tract, and correlated with reduced uveitis severity. In summary, our current findings support the potential for dietary intervention as a strategy to mitigate noninfectious uveitis severity.

Two Cases of Chronic Central Serous Chorioretinopathy Successfully Treated with Systemic Interferon Alpha.

Chronic central serous chorioretinopathy (CSCR) is a sight threatening disease that can lead to legal blindness. Verteporfin photodynamic therapy is the main treatment for chronic CSCR, however, there has been a critical worldwide shortage of verteporfin. Other medical treatments have been attempted with variable efficacy. Interferons have shown efficacy in treating uveitis and associated macular edema. We report 2 cases of treatment refractory chronic CSCR successfully treated with subcutaneous injection of interferon alpha with significant anatomical and functional improvement. To our knowledge, this is the first report observing the therapeutic potential of systemic interferon alpha in the treatment of chronic CSCR. A large randomized controlled clinical trial would help to better evaluate the safety and efficacy of systemic PEG-IFNα2a in treating chronic CSCR, and further define the optimal dose, treatment interval and duration.

Randomized evaluation of a school-based, trauma-informed group intervention for young women in Chicago.

This study explores whether a school-based group counseling program for adolescent girls, implemented at scale, can mitigate trauma-related mental health harms. In a randomized trial involving 3749 Chicago public high school girls, we find that participating in the program for 4 months induces a 22% reduction in posttraumatic stress disorder symptoms and find significant decreases in anxiety and depression. Results surpass widely accepted cost-effectiveness thresholds, with estimated cost-utility well below $150,000 per quality adjusted life year. We find suggestive evidence that effects persist and may even increase over time. Our results provide the first efficacy trial of such a program specifically designed for girls, conducted in America's third largest city. These findings suggest the promise of school-based programs to mitigate trauma-related harms.

A 48-YEAR-OLD WOMAN WITH CHRONIC, PROGRESSIVE BILATERAL VISION LOSS AND DYSPNEA.

PURPOSE: To describe a unique case of isolated, bilateral serous retinal detachments associated with primary pulmonary arterial hypertension. METHODS: Case report. RESULTS: A 48-year-old woman with primary pulmonary arterial hypertension presented with bilateral vision loss. She was found to have bilateral serous retinal detachments in the macula with accumulation of the fibrinous material. Optical coherence tomography demonstrated intraretinal and subretinal fluid, hyperreflective material in the subretinal space, and choroidal engorgement. Fluorescein angiography demonstrated pooling in the maculas, an area of blockage corresponding with the area of subretinal exudative material, and a petalloid pattern of leakage in the maculas without evidence of retinal vascular leakage. Her ocular symptoms improved with aggressive medical management of her pulmonary arterial hypertension with the addition of eplerenone. CONCLUSION: Primary pulmonary arterial hypertension results in chronically elevated systemic venous pressure, leading to both systemic and ocular symptoms. It is important to consider this systemic condition in the differential diagnosis of serous retinal detachments to provide adequate multidisciplinary management.

Consensus-based recommendations for optical coherence tomography angiography reporting in uveitis.

BACKGROUND/AIMS: To establish a consensus in the nomenclature for reporting optical coherence tomography angiography (OCTA findings in uveitis. METHODS: The modified Delphi process consisted of two rounds of electronic questionnaires, followed by a face-to-face meeting conducted virtually. Twenty-one items were included for discussion. The three main areas of discussion were: wide field OCTA (WF-OCTA), nomenclature of OCTA findings and OCTA signal attenuation assessment and measurement. Seventeen specialists in uveitis and retinal imaging were selected by the executive committee to constitute the OCTA nomenclature in Uveitis Delphi Study Group. The study endpoint was defined by the degree of consensus for each question: 'strong consensus' was defined as >90% agreement, 'consensus' as 85%-90% and 'near consensus' as >80% but <85%. RESULTS: There was a strong consensus to apply the term 'wide field' to OCTA images measuring over 70° of field of view, to use the terms 'flow deficit' and 'non-detectable flow signal' to describe abnormal OCTA flow signal secondary to slow flow and to vessels displacement respectively, to use the terms 'loose' and 'dense' to describe the appearance of inflammatory choroidal neovascularisation, and to use the percentage of flow signal decrease to measure OCTA ischaemia with a threshold greater than or equal to 30% as a 'large area'. CONCLUSIONS: This study sets up consensus recommendations for reporting OCTA findings in uveitis by an expert panel, which may prove suitable for use in routine clinical care and clinical trials.

PSEUDOPANUVEITIS AS A HARBINGER FOR SYSTEMIC LEUKEMIA RECURRENCE.

PURPOSE: To describe a patient with a history of pre-B-cell acute lymphoblastic leukemia in remission, who developed recurrent alternating intraocular leukemia manifesting with pseudohypopyon, uveal mass, and serous retinal detachment. In multiple instances, this constellation of ocular findings preceded systemic leukemia recurrence. METHOD: Case report. RESULTS: A 29-year-old man with a history of pre-B-cell acute lymphoblastic leukemia, in remission after a hematopoietic stem cell transplant, presented with pseudohypopyon, uveal lesions, and serous retinal detachment of the right eye. Comprehensive workup for infectious and inflammatory etiologies was unremarkable, and a bone marrow biopsy revealed systemic recurrence of leukemia. One year later, while again in remission, the patient developed a pseudohypopyon, uveal mass, and serous retinal detachment of the other eye. Repeat bone marrow biopsy showed impending leukemia relapse, which occurred 1 month later. Orbital radiation resulted in complete ocular resolution. CONCLUSION: The constellation of pseudohypopyon, serous retinal detachment, and uveal mass (pseudopanuveitis) should be recognized as a harbinger for systemic pre-B ALL recurrence.

Impact of the COVID-19 pandemic on uveitis patient care.

BACKGROUND: The COVID-19 pandemic has significantly changed practice of medicine and patient care worldwide. The impact of the pandemic on patients with uveitis is unknown. We developed the COVID-19 Practice Patterns Study Group to evaluate the effect of the pandemic on uveitis patient care. METHODS: This is a multicentre, cross-sectional survey of uveitis specialists practising worldwide. A web-based survey was distributed through the mailing lists of international uveitis societies to assess modifications in patient care, and use of immunomodulatory therapies (IMTs),aswell as considerations regarding COVID-19 vaccination. RESULTS: A diverse group consisting of 187 uveitis specialists from six continents participated in this survey. Most of these experts noted a disruption in clinical management of patients, including clinic closures or decrease in volume, patients missing in-person visits due to the fear of infection and difficulties obtaining laboratory testing. Most participants initiated (66.8%) and continued (93.3%) IMTs based on clinical presentation and did not modify their use of immunosuppressives. In cases of reported exposure to COVID-19 infection, most participants (65.3%) recommended no change in IMTs. However, 73.0% of the respondents did recommend holding all or select IMTs in case of COVID-19 infection. COVID-19 vaccine was recommended universally by almost all the specialists and 52% stated that they would counsel patients regarding the decreased immunogenicity and effectiveness of the vaccine in immunocompromised patients. CONCLUSIONS: Uveitis patient care has changed significantly since the beginning of the pandemic. The recommendations will continue to evolve as new data on IMTs and vaccination become available.

Creating an inclusive community for BIPOC faculty: women of color in academia.

Institutions of higher education are increasingly diverse with more women and people of color hired, but there remains much work to be done to ensure that underrepresented faculty feel supported in their careers, evaluated fairly for tenure and/or promotion, and made to feel appreciated and valued in their institutions. This perspective paper will review how interpersonal and institutional prejudice disadvantage women of color in academia in aspects of their professional responsibilities including teaching, mentoring students, research, and service. The combination of these challenges explains why women of color are not rewarded in the same way and do not advance as quickly in their academic careers compared to white men. After considering the subtle and direct ways that biases cause harm to women of color, we present recommendations to better support women of color in faculty roles with the goal of combating prejudice pertaining to both racial/ethnic and gender biases.

Antimetabolite Drugs Exhibit Distinctive Immunomodulatory Mechanisms and Effects on the Intestinal Microbiota in Experimental Autoimmune Uveitis.

Purpose: The purpose of this study was to investigate the effect of antimetabolite drugs on T-cell responses and intestinal microbial composition in autoimmune uveitis. Methods: Experimental autoimmune uveitis (EAU) was induced in C57BL/6J mice treated with 0.00625 mg/mL methotrexate (MTX) or 0.625 mg/mL mycophenolate mofetil (MMF) in drinking water for 4 weeks prior to immunization and 2 weeks thereafter. The effector T cell (Teff) and regulatory T cell (Treg) populations were identified using flow cytometry. The 16S rRNA gene sequencing was applied for gut microbiome characterization. DESeq2 analysis was used to discriminate relative abundances of taxa and PLS-DA to integrate cytometric and microbiome data between groups. Results: Both MTX and MMF abrogated uveitis in EAU without clinical signs of toxicity as compared to water-fed controls. MTX reduced Teff and Treg expansion in peripheral tissues and eyes. MTX decreased alpha diversity, increased Akkermansia, and reduced Lachnoclostridium abundances. Conversely, MMF enhanced Tregs in the mesenteric lymph node and the eyes. In parallel, MMF increased the gut alpha diversity, including an increased abundance of Lachnospiraceae NK4A136 group and a decreased abundance of Lachnospiraceae UCG-001. A significant congruent correlation among intestinal microbial changes, T-cell responses, and clinical scores was observed for both antimetabolites. Conclusions: Although MTX and MMF both abrogated uveitis in EAU, they showed different effects on T-cell subsets and the intestinal bacterial composition. This work indicates unique immunomodulation by each drug and is the first to demonstrate potential microbiota-related mechanisms.

A Review of the Role of the Intestinal Microbiota in Age-Related Macular Degeneration.

Blindness from age-related macular degeneration (AMD) is an escalating problem, yet AMD pathogenesis is incompletely understood and treatments are limited. The intestinal microbiota is highly influential in ocular and extraocular diseases with inflammatory components, such as AMD. This article reviews data supporting the role of the intestinal microbiota in AMD pathogenesis. Multiple groups have found an intestinal dysbiosis in advanced AMD. There is growing evidence that environmental factors associated with AMD progression potentially work through the intestinal microbiota. A high-fat diet in apo-E-/- mice exacerbated wet and dry AMD features, presumably through changes in the intestinal microbiome, though other independent mechanisms related to lipid metabolism are also likely at play. AREDS supplementation reversed some adverse intestinal microbial changes in AMD patients. Part of the mechanism of intestinal microbial effects on retinal disease progression is via microbiota-induced microglial activation. The microbiota are at the intersection of genetics and AMD. Higher genetic risk was associated with lower intestinal bacterial diversity in AMD. Microbiota-induced metabolite production and gene expression occur in pathways important in AMD pathogenesis. These studies suggest a crucial link between the intestinal microbiota and AMD pathogenesis, thus providing a novel potential therapeutic target. Thus, the need for large longitudinal studies in patients and germ-free or gnotobiotic animal models has never been more pressing.

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.

Swept-source optical coherence tomography objective composite activity score for uveitis.

PURPOSE: To develop an objective intraocular inflammation composite score. METHODS: Cross-sectional study. Non-invasive image acquisition and processing were conducted from April 2017 to April 2019. Inflammation-grade stratified eyes from patients with active, inactive uveitis and healthy controls were recruited. After clinical assessment, four anterior and posterior segment image acquisition protocols per eye, using swept-source optical coherence tomography (SS-OCT), were performed at inclusion. Eight imaging biomarkers in three domains: anterior, intermediate and posterior were studied. They were ranked and selected by discriminatory power and correlation with clinical scores. A final SS-OCT-derived composite uveitis activity score (SS-UAS) was developed through multiple linear regression. RESULTS: We studied 224 eyes with uveitis (165 active and 59 inactive) from 165 patients (mean age 46.6 SD 15.5 years; 55.3% women) and 38 eyes from 19 healthy controls (mean age 43.6 SD 17.1; 47% women). The selected SS-OCT-derived biomarkers to build the final score were anterior chamber hyper-reflective dots (anterior), high-definition relative vitreous intensity (intermediate) and the averaged thickened retinal index (posterior). Swept-source (SS)-UAS was highly discriminant between active and inactive, and between active and healthy eyes (means 2.06 SD 1.86, 0.93 SD 0.44, and 0.96 SD 0.38, respectively, both p -, Mann-Whitney U). Construct validity (Cronbach's alpha = 0.7), internal consistency, criterion validity and reliability (concordance correlation coefficient intra-rater = 0.99, 95% CI: 0.98-0.99; inter-rater = 0.98, 95% CI: 0.96-0.99) were favourable. CONCLUSIONS: Global intraocular inflammation can potentially be staged and scored objectively, continuously, consistently and in a valid manner through the combined processing of SS-OCT scans.

Risk Factors and Disease Course for Blood-Brain Barrier Disruption-Associated Maculopathy.

Importance: Blood-brain barrier disruption (BBBD) is a systemic therapy for malignant central nervous system (CNS) tumors that has been linked to poorly understood pigmentary maculopathy. Objectives: To examine the rate of and risk factors for the development of BBBD-associated maculopathy and to assess whether there can be visually significant progression after completion of systemic therapy. Design, Setting, and Participants: In this retrospective case series, data from February 1, 2006, through December 31, 2019, were collected from patients treated with osmotic BBBD at a single tertiary referral center who had subsequent ophthalmic evaluation. Exposures: Treatment with BBBD therapy for any malignant CNS tumor. Main Outcomes and Measures: Rate and potential risk factors for developing BBBD-associated maculopathy and changes in visual acuity and retinal imaging characteristics after completion of BBBD therapy. Results: Of 283 patients treated with BBBD and chemotherapy for a CNS malignant neoplasm, 68 (mean [SD] age, 46.0 [17.9] years; 25 [38.5%] female) had an ophthalmic examination after starting systemic therapy. After excluding 3 patients because of bilateral media opacities, pigmentary maculopathy was present in 32 of 65 patients (49.2%) treated with BBBD. The number of BBBD treatment sessions, but not age, CNS malignant cancer type, or systemic chemotherapy agent, was associated with maculopathy development (odds ratio, 1.30; 95% CI, 1.12-1.50; P = .001). After completion of BBBD therapy, progressive enlargement of geographic atrophy occurred in 5 eyes of 3 patients, and choroidal neovascularization developed in 1 eye. Conclusions and Relevance: In this case series, an association was found between BBBD-related maculopathy and the number of BBBD treatment sessions, suggesting a dose-dependent effect. In some cases, maculopathy progression, including enlargement of geographic atrophy, occurred years after completion of systemic therapy. These findings may have important implications for patient education and ophthalmic monitoring.

Maculopathy Associated With Osmotic Blood- Brain Barrier Disruption and Chemotherapy in Patients With Primary CNS Lymphoma.

BACKGROUND AND OBJECTIVE: To describe the incidence, characteristics, and risk factors of a pigmentary maculopathy in patients with primary central nervous system (CNS) lymphoma treated with blood-brain barrier disruption (BBBD) therapy. PATIENTS AND METHODS: This retrospective chart review included patients with biopsy-proven primary CNS lymphoma treated with or without BBBD therapy who underwent an ophthalmic examination after starting systemic treatment. Clinical data and all available retinal imaging were analyzed. RESULTS: Fifty-eight patients met inclusion criteria. Twenty-one of 36 patients treated with BBBD therapy had a bilateral pigmentary maculopathy. None of the 22 patients treated with conventional chemotherapy had similar changes. Additional findings in patients treated with BBBD included geographic retinal pigment epithelium atrophy, subretinal fluid, and in one case, choroidal neovascularization. Some cases of maculopathy resulted in reduced visual acuity. The presence of a pigmentary maculopathy was associated with a higher number of BBBD treatment sessions (20.1 vs 13.3, P = .016), but not vitreoretinal lymphoma involvement or intravitreal methotrexate injections. CONCLUSION: In this cohort, 58.3% of patients with primary CNS lymphoma treated with BBBD and chemotherapy were found to have a bilateral pigmentary maculopathy. This maculopathy can result in reduced visual acuity and is associated with the number of BBBD treatment sessions. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:S5-S12.].