Ocular Findings of Cryptococcal Meningitis in Previously Healthy Adults.

BACKGROUND: Patients with cryptococcal meningitis (CM) often have ocular manifestations; although data are describing these findings in nonimmunosuppressed, previously healthy individuals are scarce. METHODS: A retrospective chart review was performed for previously healthy patients with CM who underwent a complete ophthalmological examination within a 5-year period at the National Institutes of Health. Demographics, CSF parameters, findings on initial ophthalmological examination, and MRI abnormalities were analyzed. RESULTS: Forty-four patients within a median of 12 weeks after CM diagnosis were included in our study; 27 patients (61%) reported abnormal vision on presentation. Seventy-one percent of patients were not shunted at the time of their initial eye examination. The most common ocular abnormalities were visual field defects in 21 (66%), decreased visual acuity in 14 (38%), and papilledema in 8 (26%) patients. Intraocular pressure was within normal range in all patients. Cranial nerve defects were identified in 5 patients and optic neuropathy in 2 patients. Patients who had hydrocephalus or did not receive a ventriculoperitoneal shunt were not noted to have worse ocular abnormalities. CONCLUSIONS: The most common ocular findings in our cohort of nontransplant, non-HIV cryptococcal meningitis patients were visual field defects, decreased visual acuity, and papilledema. Our results emphasize the need for a comprehensive eye examination in patients with CM who may not always report a change in vision on presentation.

CHOROIDAL LESIONS UNRESPONSIVE TO FLUOCINOLONE ACETONIDE INTRAVITREAL IMPLANT IN BIRDSHOT CHORIORETINOPATHY.

PURPOSE: To report the persistence of choroidal lesions despite fluocinolone acetonide intravitreal implants and their resolution with oral prednisone treatment. METHODS: Retrospective chart review of a birdshot chorioretinopathy patient at a tertiary referral clinic. RESULTS: Indocyanine angiography revealed resolution of choroidal lesions with oral prednisone and recurrence after discontinuation of oral prednisone. CONCLUSION: Choroidal lesions responded to oral prednisone despite bilateral active fluocinolone acetonide intravitreal implant in a birdshot chorioretinopathy patient.

Neuro-Behcet Disease and Ocular Inflammation: A Case Report and Literature Review.

Purpose: To describe a case of panuveitis with occlusive vasculitis leading to the diagnosis of neuro-Behcet disease (NBD) and discuss the relationship between uveitis and NBD.Methods: Case report with a literature review of ocular inflammation in NBD.Results: A 26-year-old woman with a seven-month history of recurrent cerebral venous sinus thromboses (CVST) and right-sided hemiparalysis secondary to rhombencephalitis presented with bilateral panuveitis and occlusive retinal vasculitis. Systemic evaluations were negative for hypercoagulability and infection. Although HLA-51 negative, the diagnosis was consistent with NBD.Conclusion: NBD is a rare subset of BD with a limited number of studies and patients. However, uveitis is more common in adults with parenchymal disease; may predate the development of neurological symptoms. The most common locations of ocular inflammation were posterior and panuveitis. MRI/V of the brain can identify enhancing lesions in the rhombencephalon or CVST in patients with uveitis with neurological findings.

Accuracy of intravitreal injection volume for aflibercept pre-filled syringe and BD Luer-Lok one-milliliter syringe.

BACKGROUND: To evaluate the accuracy of intravitreal injection volume of the pre-filled syringe (PFS) in which aflibercept is packaged compared to the BD Luer-Lok 1-mL syringe. METHODS: Ophthalmologists injected their typical intravitreal volume for aflibercept using either the PFS or BD Luer-Lok 1-mL syringe for 5 times each. The injected fluid was weighed using a micro-scale and converted to volume. The volume of fluid injected was also evaluated when the 0.05 mL line on the PFS was lined up to the tip or base of the dome-shaped plunger. RESULTS: Injection volume was measured for 12 physicians. The average injected fluid volume was 74.22 ± 15.87 µL for PFS and 53.42 ± 4.61 µL for the BD Luer-Lok 1-mL syringe (p < 0.0001). The average deviation in volume injected for the PFS was higher compared to the BD Luer-Lok 1-mL syringe (11.36 µL vs. 3.35 µL, p < 0.0001). When the PFS was lined up with the tip of the dome-shaped plunger at the 0.05-mL line, the average injected volume was 71.03% higher. CONCLUSIONS: The intravitreal injection volume and variability using the new PFS were significantly higher than the volume injected using the BD Luer-Lok 1-mL syringe previously used, potentially leading to higher rates of visually significant elevation of intraocular pressures.

Patient Clinical Outcomes in Standalone Versus a Combined Ophthalmology-rheumatology Uveitis Clinic.

BACKGROUND: To evaluate uveitis care outcomes in standalone versus a combined ophthalmology-rheumatology clinic. METHODS: Participants were patients aged 18 years and older with a minimum 12-month history of chronic uveitis prior to being referred to the combined uveitis clinic at Kresge Eye Institute and who were treated in the combined clinic for at least 6 months. Best corrected visual acuity (BCVA), objective markers of inflammation, and achieving targeted dose of immunomodulatory therapy (IMT) were compared in the cohort of uveitis patients 6 months prior to and after the initial evaluation in the combined clinic. RESULTS: Sixty-six percent of study participants were female with a mean age of 51.5 years. BCVA improved from 0.58 logMAR (Snellen: ~20/74) at the initial combined clinic visit to 0.50 logMAR (Snellen: ~20/63) 6 months after the first combined visit (p = 0.0137). The establishment of the combined uveitis clinic led to higher frequency of patients at target dose of IMT: an increase from 49.0% at 6 months prior to the combined visit to 70.1.4% and 79.8% at the initial combined visit and 6 months after the combined visit, respectively. CONCLUSION: A combined model of management for chronic uveitis patients wherein rheumatological services are coupled with ophthalmic care leads to improvement in patient clinical outcomes and achieving target therapy.

Multiple Evanescent White Dot Syndrome Presenting After Initiation of Empagliflozin, a Sodium-Glucose Cotransporter-2 Inhibitor.

Multiple evanescent white dot syndrome (MEWDS) is an inflammatory condition of the retina that typically presents unilaterally with multiple gray-white spots in the outer retina or retinal pigmented epithelium and granular changes within the fovea. We report a case of new-onset MEWDS in a patient closely after the initiation of empagliflozin, a sodium-glucose cotransporter inhibitor medicine for his type II diabetes mellitus.

Disease Relapse After Drug-Free Remission in Ocular Mucous Membrane Pemphigoid.

PURPOSE: To quantitate the risk of relapse of ocular and extraocular disease among patients with mucous membrane pemphigoid (MMP) who had undergone drug-free remission. DESIGN: Retrospective, comparative, interventional case series. METHODS: There were 167 patients with biopsy-proven MMP who were seen at the Wilmer Eye Institute between November 1984 and December 2019. Among the 167 patients, 119 patients had ocular involvement and 103 of those patients received systemic treatment for MMP. The main outcome measures were the incidence of ocular remission, incidence rate of disease relapse after remission, and risk factors for disease relapse. RESULTS: Over a median follow-up time of 7 years, 74 of 103 treated patients (71.8%) experienced drug-free remission (incidence rate = 0.28/person-year [PY], 95% confidence interval [CI] 0.22-0.35/PY). Most patients (80/103, 77.7%) received cyclophosphamide therapy. Thirteen of the 74 patients (17.6%) had disease relapse after remission: 4 with ocular disease only, 4 with extraocular disease only, and 5 with both. The rate relapse of ocular MMP was 0.020/PY (95% CI 0.009-0.038/PY), and the rate of relapse of MMP at any site (ocular or extraocular site) was 0.029/PY (95% CI 0.015-0.050/PY). The use of cyclophosphamide was associated with a greater chance of remission (hazard ratio [HR] = 3.84, P < .0001) and a lower risk of relapse (HR = 0.32, P = .05) compared with other immunosuppressive drugs except for rituximab. Five patients experienced drug-free remission after rituximab therapy and none of them had relapse (median follow-up after remission = 3.6 years). When use of cyclophosphamide or rituximab was compared with all other treatments, the risk of MMP relapse at any site (HR = 0.17, P = .02) and of ocular MMP (HR = 0.11, P = .007) were significantly lower. CONCLUSIONS: Rates of relapse of MMP after drug-free remission are low but not zero; therefore, monitoring of patients remains necessary. Relapses were not observed among those patients treated with rituximab who had remission; however, follow-up duration in those patients was shorter than the whole MMP cohort and the sample size was small.

Optical Coherence Tomography Angiography Changes in Choroidal Vasculature following Treatment in Punctate Inner Choroidopathy.

Purpose: To describe the changes seen on optical coherence tomography angiography [OCTA] in patients with PIC following immunosuppressive therapy.Methods: We reviewed serial OCTA scans from five consecutive PIC patients (5 eyes) with at least 3 months of follow-up, who underwent imaging before and after immunosuppressive therapy. Using ImageJ, superficial and deep retinal vasculature were analyzed for vessel area and foveal avascular zone. Choriocapillaris layer was analyzed for flow signal loss.Results: Five out of five patients received an orbital floor triamcinolone acetonide injection as the initial treatment for periods of activity. Mean choriocapillaris (CC) flow void area obtained after immunosuppressive therapy was significantly lower than the mean CC flow void area obtained prior to treatment (Pre-treatment: 0.270 vs Post-treatment: 0.144; p = .0068). In 2 out of 2 patients with longitudinal visual field testing, CC flow voids were spatially associated with visual field defects, and immunosuppressive therapy was associated with reduced CC flow void area and improved visual function.Conclusion: OCTA can detect alterations in choriocapillaris flow. Longitudinal follow-up demonstrates a centripetal restoration of choriocapillaris flow in response to immunosuppressive therapy. OCTA may be a useful adjunct for monitoring and evaluating treatment of PIC.

Principal Cause of Poor Visual Acuity after Neovascular Age-Related Macular Degeneration: Age-Related Eye Disease Study 2 Report Number 23.

PURPOSE: To analyze the principal cause for poor vision in eyes with best-corrected visual acuity (BCVA) of 20/200 or worse 2 years after neovascular age-related macular degeneration (nAMD). DESIGN: Prospective cohort study of participants enrolled in a clinical trial of oral supplements. PARTICIPANTS: Age-Related Eye Disease Study 2 (AREDS2) participants whose eyes began anti-vascular endothelial growth factor (VEGF) therapy for incident nAMD and had data available at 2 years. METHODS: Participants underwent refracted BCVA testing, ophthalmoscopic examination, and fundus photography at baseline and annual visits. Self-reports of anti-VEGF injections were collected. MAIN OUTCOME MEASURES: Principal cause of BCVA of 20/200 or worse at 2 years, detected on fundus photography grading. RESULTS: Of the 594 eligible eyes, the number with BCVA of 20/200 or worse at 2 years was 56 (9.4%). Mean BCVA was 14.9 letters (standard deviation [SD], 12.3 letters; Snellen equivalent, 20/500), versus 70.1 letters (SD, 12.8 letters; Snellen equivalent, 20/40) in the other group. Of the 55 eyes with fundus photography available at 2 years, 33 (60.0%) had central macular atrophy and 22 (40.0%) had central subretinal fibrosis assessed as the principal cause for poor vision. The group with poor BCVA had a higher proportion of non-White participants (8.9% vs. 1.7%; P = 0.006), lower BCVA 2 years earlier (mean, 38.0 letters [SD, 26.7 letters; Snellen equivalent, 20/160] vs. 71.8 letters (SD, 11.9 letters; Snellen equivalent, 20/40]; P < 0.0001), higher proportion with macular atrophy 2 years earlier (26.8% vs. 12.3%; P = 0.003), higher proportion with macular hemorrhage (25.5% vs. 13.2%; P = 0.014), and fewer anti-VEGF injections (7.6 vs. 10.2; P = 0.001). CONCLUSIONS: Visual acuity data and fundus photography were obtained in a clinical trial environment, but were related to anti-VEGF therapy given in routine clinical practice. At 2 years after starting anti-VEGF therapy, almost 1 in 10 eyes showed BCVA at the level of legal blindness. From fundus photography grading, the cause of poor vision appeared to be macular atrophy in 60% and subretinal fibrosis in 40%. These data may be useful in understanding the long-term limits to good vision in nAMD.

Profound bilateral post-partum retinal circulation ischemia in two diabetic mothers with pre-eclampsia.

PURPOSE: We report 2 cases of young diabetic mothers with pre-eclampsia and no previously documented visual deficits prior to pregnancy who were found to have profound retinal circulation loss during the postpartum period. OBSERVATIONS: Fluorescein angiogram in both cases documented profound ischemia of retinal and macular circulation with resulting severe vision loss, leading to legal blindness. CONCLUSIONS AND IMPORTANCE: While Pre-eclampsia and Eclampsia are well known clinical entities with potentially grave effects on both the mother and child, there is little information about the possible combined effects of diabetes and Pre-eclampsia/Eclampsia on the retinal circulation. We aim to raise awareness about this devastating association with a need for aggressive monitoring and prompt treatment of similar patients to potentially avoid poor visual outcomes.

Incidence and Clinical Characteristics of Ocular Involvement in Mucous Membrane Pemphigoid.

Purpose: To describe the risk of developing ocular mucous membrane pemphigoid (MMP) or a new extraocular site of MMP, and to identify risk factors for new involvement. Methods: Retrospective chart review of 162 biopsy-proven MMP patients. Results: At presentation, 109 of 162 MMP patients (67.3%) had ocular involvement and 53 patients did not. Of the 53 patients without ocular involvement at presentation followed up to 22 years, the risk of developing ocular MMP was 0.014 per person-year (PY, 95% confidence interval [CI]: 0.005/PY, 0.034/PY). The risk of developing any new location of extraocular MMP was 0.020/PY (95% CI: 0.007/PY, 0.043/PY). Smoking was a risk factor for developing an additional extraocular MMP location (hazard ratio [HR] = 4.09, p = 0.04). Conclusions: Patients presenting with extraocular MMP are at risk for developing ocular MMP, and all MMP patients are at risk for developing secondary extraocular MMP locations, although the rates were low.