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Ella H Leung, Daniel J Oh, Shannon E Alderson, Joshlynn Bracy, Mia McLeod, Litzi I Perez, Alexander Bottini, David Chin Yee, Krishna Mukkamala Georgia Retina, Atlanta, GA, USACorrespondence: Ella H Leung, 833 Campbell Hill St NW, Suite 300, Marietta, GA, 30060, USA, Tel +1 770-218-1888, Email eleung@garetina.comPurpose: To evaluate the initial efficacy and safety of intravitreal faricimab in eyes previously treated for neovascular age-related macular degeneration (nARMD).Patients and methods: A retrospective review of all patients with nARMD previously treated with anti-vascular endothelial growth factor (anti-VEGF) injections who received at least 3 intravitreal faricimab injections with at least 3 months of follow-up.Results: A total of 190 eyes were included. Patients received a mean of 34.2± 23 anti-VEGF injections over 182.41± 128 weeks prior to switching to faricimab. Patients then received a mean of 6.99± 2.3 faricimab injections with an average 34.88± 8.2 weeks of follow-up. The mean best corrected visual acuities improved from 0.33± 0.32 logMAR &ap;20/43 to 0.27± 0.32 logMAR &ap;20/37 (P=0.0022). The central subfield thickness (CST) improved from 312± 87μm to 287± 71μm (P< 0.0001). At the last clinical visit, 24% had no subretinal fluid or intraretinal fluid on optical coherence tomography. The mean dosing interval between the last two consecutive faricimab injections (7.64± 6.2 weeks) was significantly longer than that for ranibizumab (5.16± 2.0 weeks, P< 0.001) or aflibercept (5.57± 3.6 weeks, P< 0.001). No patients developed idiopathic intraocular inflammation.Conclusion: Intravitreal faricimab was associated with improved vision and CSTs, even in treatment-resistant nARMD eyes. The mean last dosing interval for faricimab was longer than for ranibizumab or aflibercept. No significant adverse events were directly attributed to faricimab during the study.Keywords: faricimab, intravitreal injection, neovascular age-related macular degeneration, intraocular inflammation, anti-vascular endothelial growth factor

Background and Objective: To compare the risk factors for the development and progression of pigmentary retinopathy in patients exposed to pentosan polysulfate sodium (PPS)., Materials and Methods: Retrospective cohort study of patients exposed to PPS with at least two follow-up visits with multimodal imaging., Results: A total of 97 patients were included (33 with PPS-associated retinopathy and 64 without). The average follow-up was 29.4 months, overall cumulative dose was 1,220 ± 910 g (1,730 ± 870 vs 959 ± 910; P < 0.0001), and total PPS duration was 12.1 ± 7.1 years (16.0.2 ± 6.1 vs 10.1 ± 6.9; P < 0.0001). The best-corrected visual acuity remained stable during follow-up. At presentation, the average area of the retinopathy in the worse eye was 54.1 ± 50 mm 2 in the PPS-retinopathy group, worsening at a rate of 6.10 ± 10 mm 2 /year. Patients who developed choroidal neovascular membranes (CNVMs) had faster rates of retinopathy progression (11.6 ± 12 vs 3.53 ± 7.6 mm 2 /year, P = 0.036). No patient had the exact same gene mutation., Conclusion: PPS-associated pigmentary retinopathy can continue to progress over time, even after discontinuing the medication. CNVM development may be associated with faster rates of retinopathy progression. [ Ophthalmic Surg Lasers Imaging Retina 2023;54:388-394.] .

Purpose: To evaluate the risk factors and fundus findings of patients with potential PPS-associated retinopathy., Patients and Methods: A retrospective chart review was performed of patients exposed to PPS who had a dilated fundus examination at a large retina-only practice from 2018-21. Multimodal images were evaluated by masked reviewers., Results: A total of 148 patients were included, of whom 33 (22%) had PPS-associated retinopathy, and 115 (78%) did not. The mean age was 60.3 years old, and the mean follow-up was 11.8 months. The PPS-associated retinopathy group had higher mean cumulative doses of PPS (1600g±849 vs 864g±852, P < 0.0001, Mann-Whitney test) and longer duration of PPS use (13.6 years vs 7.48, P < 0.0001). There was no statistically significant difference based on a history of kidney or liver disease or the dosage per day for the weight, body mass index, body surface area, or lean body weight. Of the patients with PPS-associated retinopathy whose genetic results were available, 15 of 16 (93%) were heterozygous for variants of uncertain significance., Conclusion: A longer duration of PPS use and higher cumulative dosage of PPS were associated with an increased risk of developing PPS-associated pigmentary retinopathy. The role of genetic mutations in patients exposed to PPS is still to be determined., Competing Interests: The authors report no conflicts of interest in this work., (© 2021 Leung et al.)

A male infant born at 23 weeks gestation with a birthweight of 660 grams presented with retinopathy of prematurity (ROP) that began progressing at 44 weeks. He subsequently developed Zone III, Stage 3, pre-plus disease in both eyes (OU), as well as scattered exudates in the macula, dragged vessels temporally, and an exudative retinal detachment temporally in the left eye after a period of regressing and stable ROP. After bilateral laser photocoagulation, there was regression of the neovascularization, resolution of the exudative detachment, and eventual stabilization of disease OU by 12 months postmenstrual age. [ Ophthalmic Surg Lasers Imaging Retina . 2021;52:403-406.] .

Purpose: To compare the antibiotic susceptibilities and visual acuity (VA) outcomes in endophthalmitis caused by methicillin-resistant (MRSA) versus methicillin-sensitive S. aureus (MSSA)., Methods: The records of 34 cases of S. aureus endophthalmitis at The New York Eye and Ear Infirmary from Jan 1997 to June 2011 were reviewed. Antibiotic susceptibility profiles over time and VA at presentation and at 3, 6, and ≥12 months were recorded. S. aureus isolates were grouped based on oxacillin resistance., Results: Of the 34 cases, 15 (44 %) were MRSA and 19 (56 %) MSSA. Median presenting VA was hand motions (logMAR 4.0) in both the MRSA and MSSA groups. There was no statistically significant difference in VA between the MRSA and MSSA groups at 3, 6, or ≥12 months. No MRSA isolates were resistant to vancomycin or gentamicin. While over 85 % of MRSA isolates tested for fourth-generation fluoroquinolones were resistant, just 10 % MSSA isolates tested were resistant. There was a trend suggesting an increase in the proportion of MRSA isolates compared to MSSA isolates over the course of the study period., Conclusions: There was no statistical difference in short- or long-term VA outcomes between the MRSA and MSSA groups at any time point. Resistance to fourth-generation fluoroquinolones was present in over 85 % of MRSA isolates, but just 10 % of MSSA isolates. An increasing proportion of MRSA amongst S. aureus isolates was noted over the course of the study period.

Purpose: To describe a new classification of stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR)., Design: Retrospective case series and literature review., Participants: A total of 17 patients from 5 institutions., Methods: Detailed case history, multimodal imaging, and genetic testing were reviewed for patients with macular schisis without a known predisposing condition. Patients with a stellate appearance centered on the fovea with correlating confirmed expansion of the outer plexiform layer (OPL) by optical coherence tomography (OCT) were included. Exclusion criteria included a family history of macular retinoschisis, a known genetic abnormality associated with retinoschisis, myopic traction maculopathy, epiretinal membrane, vitreoretinal traction, optic or scleral pit, or advanced glaucomatous optic nerve changes., Main Outcome Measures: Clinical features, anatomic characteristics, and visual acuity., Results: A total of 22 eyes from 16 female patients and 1 male patient with foveomacular schisis were reviewed from 5 institutions. Initial visual acuity was ≥ 20/50 in all eyes (mean, 20/27), but visual acuity in a single eye decreased from 20/20 to 20/200 after the development of subfoveal fluid. The refractive status was myopic in 16 eyes, plano in 3 eyes, and hyperopic in 2 eyes. Three eyes had a preexisting vitreous separation, and 19 eyes had an attached posterior hyaloid. Follow-up ranged from 6 months to >5 years., Conclusions: In this largest known series of patients with SNIFR, all patients demonstrated splitting of the OPL in the macula with relatively preserved visual acuity (≥ 20/40) except in a single patient in whom subretinal fluid developed under the fovea., (Copyright © 2014. Published by Elsevier Inc.)

Purpose: The purpose of this study is to report a case of Kyrieleis plaques (segmental retinal periarteritis) associated with cytomegalovirus (CMV) retinitis., Methods: A 47-year-old female with recently diagnosed human immunodeficiency virus and a CD4 count of 55 cells/µl presented with decreased vision and floaters in her left eye. Ophthalmic examination revealed an advancing border of white granular CMV retinitis extending into the macula. Intraocular aqueous specimen contained 420,000 copies/ml of CMV DNA by polymerase chain reaction. The patient was treated with intravitreal foscarnet and oral valganciclovir., Results: Kyrieleis plaques involving the retinal arteries were noted on presentation and increased during the first 6 weeks of treatment as the retinitis faded. The plaques on fluorescein angiography did not leak fluorescein dye and slowly faded over 5 months., Conclusions: Kyrieleis plaques can be seen in the setting of CMV retinitis. These plaques can be differentiated from vascular sheathing and frosted branch angiitis by its occurrence only in the retinal arteries and the absence of leakage of fluorescein dye.

A rare variant of adenoid cystic carcinoma is the dedifferentiated sarcomatoid form, which has previously been reported in the hard and soft palate, maxillary sinus, submandibular glands, and nasal cavity. The authors report the first case of a dedifferentiated sarcomatoid adenoid cystic carcinoma occurring in the lacrimal gland, that of a 52-year-old man. The patient presented with a 4-month history of diplopia, decreased vision, and right upper eyelid swelling. Radiographic imaging showed a soft tissue mass in the extraconal compartment superolateral to the right eye. The patient subsequently underwent surgical debulking. Histologic examination of the tissue revealed classic cribiform adenoid cystic carcinoma and a sarcomatous component consisting of malignant spindle cells and fusiform cells arranged in whorls. Dedifferentiation is a well-established phenomenon in salivary gland tumors that is associated with aggressive behavior and poor prognosis; however, the exact nature of such dedifferentiated neoplasms remains unclear.

Background: Choroideremia is an X-linked recessive disorder characterized by vision loss with progressive atrophy of the retinal photoreceptors, retinal pigment epithelium (RPE), and choriocapillaris. Ectodermal dysplasia is a heterogeneous group of disorders characterized by a deficiency of two or more ectodermal derivatives. We report on the phenotypic and genetic characteristics of a 29-year-old woman with both choroideremia and ectodermal dysplasia., Materials and Methods: Observational case report with physical and ophthalmic examination, fluorescein angiography (FA), visual field testing, electroretinography, and cytogenetic analysis. This study adhered to the tenets of the Declaration of Helsinki and The New York Eye and Ear Infirmary Institutional Review Board guidelines., Results: Physical and ocular examination revealed hypotrichosis, hypohidrosis, full dentures, meibomian gland hypoplasia, and a decrease in corneal tear film. Visual acuity was hand motions in the right eye and 20/50 in the left eye. Fundus examination and fluorescein angiography were consistent with advanced choroideremia and revealed diffuse bilateral RPE and chorioretinal atrophy with sparing of the fovea. Visual field testing had less than 10-degree central islands in both eyes. Scotopic electroretinogram (ERG) was flat with a small flicker response. Cytogenetic analysis showed a complex translocation involving chromosomes X, 1, and 3: 46,X,t(X;1;3)(q13;q24;q21),inv(9)(p11q13). Selective inactivation of the normal X chromosome was present in blood and skin. Chromosomal analyses of the proband's family (mother and two brothers) were normal., Conclusion: An X-autosome chromosomal translocation combined with non-random inactivation of the normal X-chromosome in a woman resulted in the phenotypic findings of choroideremia and ectodermal dysplasia.

Purpose: The purpose of this study was to report recurrent hemolacria, or "bloody tears," as a sign of scleral buckle (SB) infection., Methods: This is an interventional case series of three eyes of three patients with hemolacria after SB placement., Results: Two men and one woman were treated for recurrent hemolacria after SB placement for a rhegmatogenous retinal detachment. Two patients had an encircling silicone sponge placed, one 6 years and the other 3 years before presentation. The third patient had a segmental solid silicone element placed 3 months before presentation. Two of the patients reported between 6 to 10 episodes of hemolacria occurring for "years" before referral and diagnosis. In all three patients, hemolacria originated from an occult conjunctival fistula overlying or adjacent to an exposed SB. Microbiological cultures grew Staphylococcus aureus in two eyes and polymicrobial growth in the other. Hemolacria resolved with explantation of the SB in two patients and with long-term continuous topical antibiotics in the other patient., Conclusion: Hemolacria can be a sign of a SB infection and should raise a high level of suspicion for the presence of an occult conjunctival fistula with exposure of the underlying scleral buckling element when frank exposure is not seen.