Ophthalmologic Presentations of Incontinentia Pigmenti.

Purpose: To characterize treatments and outcomes in incontinentia pigmenti. Methods: Cases of incontinentia pigmenti were consecutively identified from a retina practice. Inclusion criteria were patients with incontinentia pigmenti with at least 6 months of follow-up. All patients had a full ophthalmic examination, including imaging with widefield fundus photography and widefield fluorescein angiography. Eyes with areas of avascular retina were treated with laser photocoagulation (except for 1 eye with mild changes). Results: Thirty-six eyes of 18 patients with incontinentia pigmenti were included. The median age at presentation was 11 months. On presentation, 7 eyes had a visual acuity (VA) of 20/40 or better and 3 eyes had VA of 20/50 to 20/100. The remaining 26 eyes could fix and follow or had at least light perception (LP) VA given the patients' young age. Of the 36 eyes, 20 (56%) had retinal involvement. The mean follow-up for treated patients was 6.9 years. Seventy-four percent of treated eyes required 1 laser session only. No eye that received laser treatment subsequently developed a retinal detachment. Of the 26 eyes with initial fix-and-follow or LP VA, 12 had Snellen or Allen VA testing at follow-up. Nine of these eyes had a follow-up VA of 20/40 or better. Of 10 eyes with a Snellen or Allen VA recorded at the initial visit, 9 had a final VA that was the same or improved. Conclusions: Laser photocoagulation was effective in treating patients with retinal manifestations of incontinentia pigmenti. Except for 1 eye, VA remained stable at the final follow-up.

Survey of Retina Specialists' Attitudes and Practice Patterns of Screening and Management of Candida Endophthalmitis.

PURPOSE: To understand retina specialists' attitudes and practice patterns for screening for and managing Candida endophthalmitis and any impact of the COVID-19 pandemic on these practice patterns. METHODS: A survey was developed on Survey Monkey and distributed to ASRS regular members via email in April 2021. RESULTS: The survey had 231 respondents, of whom 169 (73%) performed inpatient consultations. Ninety percent responded that they do not recommend routinely screening asymptomatic patients with candidemia. For unresponsive patients with candidemia that lack visible signs of endophthalmitis, 65% did not recommend routine screening.However, 85% reported their affiliated hospitals did not have a policy to defer such screening consultations; this proportion did not significantly change when asked if a policy was implemented in response to the COVID-19 pandemic.For patients with a dilated examination without signs of endophthalmitis, 89% surveyed recommended continuing systemic antifungals and reconsult PRN, while the remainder recommended repeat examinations until off antifungals. For initial management of Candida endophthalmitis, 55% indicated systemic antifungals only; 43% indicated systemic antifungals with tap & inject, and 2% indicated systemic antifungals with vitrectomy and intravitreal antifungals. CONCLUSION: A discordance exists between the prevailing opinion among retina specialists against routine Candida endophthalmitis screening and hospital consultation policies, which were not significantly impacted by the COVID-19 pandemic. In cases of diagnosed endogenous endophthalmitis, slightly more than half of surveyed retina specialists would not initiate additional treatment beyond systemic antifungals while just under half surveyed recommended initial tap and inject in addition to systemic antifungals.

AI Deployment on GBM Diagnosis: A Novel Approach to Analyze Histopathological Images Using Image Feature-Based Analysis.

BACKGROUND: Glioblastoma (GBM) is one of the most common malignant primary brain tumors, which accounts for 60-70% of all gliomas. Conventional diagnosis and the decision of post-operation treatment plan for glioblastoma is mainly based on the feature-based qualitative analysis of hematoxylin and eosin-stained (H&E) histopathological slides by both an experienced medical technologist and a pathologist. The recent development of digital whole slide scanners makes AI-based histopathological image analysis feasible and helps to diagnose cancer by accurately counting cell types and/or quantitative analysis. However, the technology available for digital slide image analysis is still very limited. This study aimed to build an image feature-based computer model using histopathology whole slide images to differentiate patients with glioblastoma (GBM) from healthy control (HC). METHOD: Two independent cohorts of patients were used. The first cohort was composed of 262 GBM patients of the Cancer Genome Atlas Glioblastoma Multiform Collection (TCGA-GBM) dataset from the cancer imaging archive (TCIA) database. The second cohort was composed of 60 GBM patients collected from a local hospital. Also, a group of 60 participants with no known brain disease were collected. All the H&E slides were collected. Thirty-three image features (22 GLCM and 11 GLRLM) were retrieved from the tumor volume delineated by medical technologist on H&E slides. Five machine-learning algorithms including decision-tree (DT), extreme-boost (EB), support vector machine (SVM), random forest (RF), and linear model (LM) were used to build five models using the image features extracted from the first cohort of patients. Models built were deployed using the selected key image features for GBM diagnosis from the second cohort (local patients) as model testing, to identify and verify key image features for GBM diagnosis. RESULTS: All five machine learning algorithms demonstrated excellent performance in GBM diagnosis and achieved an overall accuracy of 100% in the training and validation stage. A total of 12 GLCM and 3 GLRLM image features were identified and they showed a significant difference between the normal and the GBM image. However, only the SVM model maintained its excellent performance in the deployment of the models using the independent local cohort, with an accuracy of 93.5%, sensitivity of 86.95%, and specificity of 99.73%. CONCLUSION: In this study, we have identified 12 GLCM and 3 GLRLM image features which can aid the GBM diagnosis. Among the five models built, the SVM model proposed in this study demonstrated excellent accuracy with very good sensitivity and specificity. It could potentially be used for GBM diagnosis and future clinical application.

Hyperbaric oxygen therapy for central retinal artery occlusion: Visual acuity and time to treatment.

Purpose: To evaluate the effectiveness of hyperbaric oxygen (HBO2) therapy as a treatment for central retinal artery occlusion (CRAO). Methods: A total of 38 patients who underwent HBO2 for non-arteritic CRAO were identified. Patients with arteritic CRAO, branch retinal artery occlusion, ophthalmic artery occlusion, and other diagnoses were excluded from the analysis. The main outcome measured was the change in visual acuity at the most recent follow-up exam compared to the visual acuity at presentation before the initiation of HBO2 therapy. Results: The overall visual acuity after HBO2 compared with the visual acuity at presentation showed a mean improvement of 0.5 logMAR from 2.2 to 1.7 logMAR (p=0.0003). Patients who presented with hand motion and light perception vision had a mean improvement of 0.4 logMAR (p=0.06) and 0.8 logMAR (p=0.004) after HBO2, respectively. An average visual acuity improvement of 0.5 logMAR (p=0.01) was observed when patients underwent HBO2 earlier than 24 hours of symptom onset. This mean improvement increased to 0.9 logMAR (p=0.009) if HBO2 was initiated within eight hours. Conclusions: HBO2 may be an effective treatment for non-arteritic CRAO, especially if patients are treated early and present with salvageable vision. The time to treatment and the presenting visual acuity may be predictive factors on the visual prognosis following HBO2. Further studies with a prospective design and more patients are necessary to determine the long-term outcomes and the optimal protocol for HBO2 in CRAO patients.

Ocular adverse events associated with chimeric antigen receptor T-cell therapy: a case series and review.

BACKGROUND/AIMS: Chimeric antigen receptor T-cell (CAR T) therapy has been shown to improve the remission rate and survival for patients with refractory haematological malignancies. The aim of this study is to describe ocular adverse effects associated with CAR T therapy in patients with haematological malignancies. METHODS: This is a retrospective, single-institution, case series. Patients aged 18 years or older who received standard of care CAR T therapy for relapsed/refractory large B-cell lymphoma with a documented ophthalmic evaluation were included. The primary outcome was clinician ophthalmic examination findings. RESULTS: A total of 66 patients received CAR T-cell therapy from February 2018 to October 2019 with 11 receiving an ophthalmic examination. Eleven patients (n=22 eyes) who received CAR T-cell therapy were included in review. The median time from CAR T-cell infusion date to ocular examination was 57.5 days. The median patient age at the time of examination was 60.5 years. Ten patients had subjective symptoms prompting ophthalmic examination. Two patients reported floaters and photopsias. One patient had worsening ocular graft-versus-host disease. Two patients were identified with possible reactivation of viral infections, including herpes zoster ophthalmicus and regressing acute retinal necrosis. CONCLUSIONS: The increasing use of CAR T therapy for malignancies underscores the importance of ophthalmologists and oncologists understanding the potential toxicities associated with its use, particularly ocular toxicities and when to refer for an ophthalmic examination.

Silicone oil tamponade in managing recalcitrant endophthalmitis after cataract surgery secondary to Clostridium intestinale.

A woman presented two weeks after uncomplicated cataract surgery with decreased visual acuity from endophthalmitis. One week after initial management with intravitreal antibiotics, her visual acuity decreased further, undergoing pars plana vitrectomy with intravitreal and intravenous antimicrobial coverage with preliminary improvement. Three days after vitrectomy, her vision decreased with recurrent inflammation. Initial cultures grew Clostridium intestinale She underwent repeat vitrectomy with silicone oil tamponade with no subsequent recurrence. The silicone oil was removed after 4 months and her visual acuity returned to 20/20 after 1 month and through 1 year of follow-up. Postoperative endophthalmitis is rare, with cases due to Clostridium species particularly destructive. In this first reported case of C. intestinale endophthalmitis, conventional management did not achieve lasting quiescence until silicone oil tamponade was employed. Pars plana vitrectomy with silicone oil tamponade should be considered in the management of recurrent endophthalmitis or endophthalmitis secondary to a recalcitrant microbe.

Topical Dexamethasone as an Adjuvant to Mineralocorticoid Receptor Antagonists for the Treatment of Recalcitrant Central Serous Chorioretinopathy.

BACKGROUND AND OBJECTIVE: A previous report demonstrated efficacy of mineralocorticoid antagonist with adjuvant topical dexamethasone (MRA+DEX) in resolving subretinal fluid (SRF) in a chronic central serous chorioretinopathy (CSCR) patient. This pilot study investigates the use of MRA+DEX to treat recalcitrant, chronic CSCR patients. STUDY DESIGN: Retrospective review of chronic, recalcitrant CSCR patients unresponsive to MRA alone who were treated with MRA+DEX and followed for up to 3 months. Apical SRF thickness and visual acuity were measured. RESULTS: Ten eyes of eight chronic, recalcitrant patients were included with an average follow-up of 109 days. Mean percent reduction in apical fluid thickness at one month and at last follow-up after adding dexamethasone (DEX) was 33% and 52%, respectively. Five eyes (50%) achieved complete resolution of SRF. Three eyes (30%) showed partial response and two (20%) eyes had no response. There was no significant change in visual acuity. CONCLUSIONS: MRA+DEX decreased SRF in some recalcitrant, chronic CSCR patients. Large prospective studies are needed to evaluate the utility of MRA+DEX in these chronic CSCR patients. [Ophthalmic Surg Lasers Imaging Retina 2022;53:659-665.].

Corrigendum to "Sarcoidosis presenting as Wallenberg syndrome and panuveitisË® Respir. Med. Case Rep. 24 (2018) 16-18.

[This corrects the article DOI: 10.1016/j.rmcr.2018.03.002.].

Vitreous microparticles contain apoptotic signals suggesting a diabetic vitreopathy.

AIM: To evaluate differences in microparticle profiles in vitreous samples between diabetic and non-diabetic eyes undergoing vitrectomy. METHODS: Un-masked cross-sectional series of 34 eyes undergoing vitrectomy. Vitreous specimens were collected and processed to evaluate for membrane integrity (DAPI), apoptosis (Annexin-V), and endothelial-cell origin (V-Cadherin). A BD LSR II flow cytometer was used for analysis and standardized sub-micron-sized beads were used for size comparison. RESULTS: Thirty-four specimens underwent analysis. Greater levels of Annexin-V were found on microparticles from specimens in which blood had entered the vitreous (n=12) compared to those without blood (n=22; 52.3%±30.7% vs 19.6%±27.2%, P=0.002). Patients with diabetes having surgery with hemorrhage (n=7) had greater expression of Annexin-V than those without hemorrhage (n=8; 62.1%±31.7% vs 18.9%±20.9%, P=0.009). However, in patients with non-diabetic vitreous hemorrhage, the level of Annexin-V expression was not significantly different compared to other disease processes (38.6%±25.7%, n=5 vs 20.0%±30.9%, n=14, P=0.087). CONCLUSION: Increased expression of the apoptotic marker, Annexin-V is detected on vitreous microparticles in diabetes-related vitreous hemorrhage. When evaluating vitreous hemorrhage in patients without diabetes, the apoptotic signal is not significantly different. Vitrectomy in patients with diabetes, and improvement in visual outcomes, may be related to the removal of a serum-derived, pro-apoptotic vitreous. Further investigation is warranted in order to identify the molecular characteristics of microparticles that regulate disease.

Vitreoretinal Lymphoma: Optimizing Diagnostic Yield and Accuracy.

PURPOSE: To determine whether the addition of adjunctive tests, including immunohistochemistry (IHC), cytokine analysis, flow cytometry, and IgH gene rearrangement testing, achieves improved diagnostic parameters compared with cytologic smears alone in the detection of vitreoretinal lymphoma (VRL). To determine which of these tests or combination of tests provide the greatest diagnostic utility. DESIGN: Retrospective review to assess diagnostic value. METHODS: This single university-affiliated tertiary care center study included data from 237 vitreous biopsies performed between 1999 and 2017 in patients with suspected VRL. From 1999 to 2008-2009, cytologic smears were the sole test performed (84 cases). The protocol initiated in 2008-2009 added the 4 additional diagnostic tests (153 cases). The sensitivity, specificity, positive predictive value, negative predictive value, diagnostic accuracy, and diagnostic yield were calculated. Parameters were calculated for tests individually, for all 5 combined, and all possible 2-, 3-, and 4-test combinations. For cytologic smears, diagnostic parameters were calculated both before and after the addition of adjunctive tests to our protocol and for the entire cohort. RESULTS: Of the 237 vitreous biopsies, 50 samples (21%) were from patients with confirmed central nervous system lymphoma and/or actively treated central nervous system, systemic, or intraocular lymphoma. Diagnostic yields (95% CI) were 90% (85%-93%) for smears, 82% (72%-89%) for IHC, 91% (85%-96%) for cytokine analysis, 76% (67%-84%) for IgH gene rearrangement, and 50% (40%-60%) for flow cytometry. For smears, the sensitivity pre-protocol was 73% (39%-94%), compared with 87% (69%-96%) post-protocol. IgH gene rearrangement was the only test exhibiting low sensitivity (40%). The combination of smears, IHC, and cytokine analysis exhibited the highest diagnostic parameters, with sensitivity 92%, specificity 98%, and diagnostic yield 100%. CONCLUSIONS: The combination of cytologic smears, IHC, and cytokine analysis seems to be a reasonable and sufficient protocol for the diagnosis of suspected VRL. IgH gene rearrangement and flow cytometry may be the most expendable tests from our protocol.

Diabetic Retinopathy Screening with Automated Retinal Image Analysis in a Primary Care Setting Improves Adherence to Ophthalmic Care.

PURPOSE: Retinal screening examinations can prevent vision loss resulting from diabetes but are costly and highly underused. We hypothesized that artificial intelligence-assisted nonmydriatic point-of-care screening administered during primary care visits would increase the adherence to recommendations for follow-up eye care in patients with diabetes. DESIGN: Prospective cohort study. PARTICIPANTS: Adults 18 years of age or older with a clinical diagnosis of diabetes being cared for in a metropolitan primary care practice for low-income patients. METHODS: All participants underwent nonmydriatic fundus photography followed by automated retinal image analysis with human supervision. Patients with positive or inconclusive screening results were referred for comprehensive ophthalmic evaluation. Adherence to referral recommendations was recorded and compared with the historical adherence rate from the same clinic. MAIN OUTCOME MEASURE: Rate of adherence to eye screening recommendations. RESULTS: By automated screening, 8.3% of the 180 study participants had referable diabetic eye disease, 13.3% had vision-threatening disease, and 29.4% showed inconclusive results. The remaining 48.9% showed negative screening results, confirmed by human overread, and were not referred for follow-up ophthalmic evaluation. Overall, the automated platform showed a sensitivity of 100% (confidence interval, 92.3%-100%) in detecting an abnormal screening results, whereas its specificity was 65.7% (confidence interval, 57.0%-73.7%). Among patients referred for follow-up ophthalmic evaluation, the adherence rate was 55.4% at 1 year compared with the historical adherence rate of 18.7% (P < 0.0001, Fisher exact test). CONCLUSIONS: Implementation of an automated diabetic retinopathy screening system in a primary care clinic serving a low-income metropolitan patient population improved adherence to follow-up eye care recommendations while reducing referrals for patients with low-risk features.

Impact of Intraoperative Ocular Lubricants on Corneal Debridement Rate During Vitreoretinal Surgery.

PURPOSE: To compare surgical parameters among patients receiving Viscoat (sodium chondroitin sulfate 4%-sodium hyaluronate 3%) or Goniosol (hydroxypropyl methylcellulose 2.5%) as topical lubricants for retinal surgery. METHODS: This was a retrospective analysis of patients undergoing retinal surgery between March 2013 and March 2018 using Goniosol or Viscoat as adjuvants. Primary outcome measures were rate of corneal debridement and operative time between groups, compared using χ 2 and t-tests, respectively. RESULTS: Compared to Viscoat (n=319), the Goniosol group (n=210) had more frequent intraoperative corneal debridement (21.4% vs 0, p<0.05) and longer surgical times (98 vs 78 minutes, p<0.05). Patients in the Viscoat group had higher rates of complex procedures (34.8% vs 26.7%, p<0.05), but were younger (50.7 vs 55.0 years, p<0.05) and more likely to be phakic (83.4% vs 70.5%, p<0.05). CONCLUSION: These findings suggest potential advantages of using Viscoat over Goniosol for corneal lubrication to aid visualization during vitreoretinal surgery.

TRAUMATIC MACULAR HOLE CLOSURE AND VISUAL IMPROVEMENT AFTER TOPICAL NONSTEROIDAL ANTIINFLAMMATORY DRUG TREATMENT.

PURPOSE: To report a case of pediatric traumatic macular hole that closed with visual improvement after treatment with topical ketorolac. METHODS: Retrospective case report. RESULTS: A 15-year-old girl presented with persistent left blurred vision after being hit with a soccer ball 2 months before. Visual acuity was 20/40 with a full-thickness macular hole with cystoid macular edema. After treatment with ketorolac 0.4% four times a day for a month, the hole closed with resolution of the cystoid macular edema but some remaining subretinal fluid. The ketorolac was tapered over the following month, and the subretinal fluid resolved during the subsequent months. At 10 months after initial presentation, patient's vision was 20/20 with a normal foveal contour, no subretinal fluid, and minimal ellipsoid zone disruption. CONCLUSION: Topical nonsteroidal antiinflammatory drug treatment may play a role in the resolution of traumatic macular holes with cystoid macular edema.

Successful treatment of an exudative choroidal hemangioma with oral propranolol in a 10-year-old boy.

A 10-year-old boy was referred for a circumscribed choroidal hemangioma with underlying exudative detachment of the left eye. To avoid general anesthetics required for laser-based therapy in a child, we began a trial of oral propranolol. The patient's exudative detachment resolved, with resulting improvement in visual acuity, and remained quiescent for 3 years.

Sarcoidosis presenting as Wallenberg syndrome and panuveitis.

Sarcoidosis is a multi-system disease with neurological involvement being one of the more rare manifestations. We report a case of a patient who presented with the lateral medullary syndrome and panuveitis as her initial manifestation of sarcoidosis. The patient's course was further complicated by renal involvement. Lacrimal gland and renal biopsies showed noncaseating granulomas without evidence of infection, establishing the diagnosis. Intracranial vertebral artery involvement was confirmed by brain imaging. Bilateral hilar lymphadenopathy with upper lobe predominant nodules on chest imaging was consistent with asymptomatic pulmonary involvement. Systemic steroid therapy is indicated for treatment of ocular sarcoidosis, with standard stroke management indicated for the treatment of lateral medullary syndrome.