Revision of Initial Referral Diagnosis following Genotypic Confirmation of Familial Exudative Vitreoretinopathy.

PURPOSE: To describe common referral diagnoses of FEVR (Familial Exudative Vitreoretinopathy) DESIGN: Retrospective case series. SUBJECTS: Patients with phenotypic FEVR evaluated by the pediatric retina service at Bascom Palmer Eye Institute from March 1, 2019 to May 31, 2023. METHODS: A retrospective detailed review of history, imaging, and genetic testing was performed for all patients included in the study. MAIN OUTCOME MEASURES: The primary outcome measures included genetic evaluation, clinical imaging and characteristics. Secondary outcome measures were age and stage of disease at diagnosis. RESULTS: Fifty-five patients with phenotypic and genetically confirmed FEVR were identified. Of these, the initial diagnosis was incorrect upon referral in 33 cases (60%). The referral diagnoses included Coats disease (n =2, 7%), toxoplasmosis (n=2, 7%), retinal detachment (n=6, 18%), persistent fetal vasculature (PFV) (n =5, 15%), suspected inherited retinal disease (n=2, 7%), myopia (n=3, 9%), retinopathy of prematurity (ROP) with preterm gestational age (n=7, 21%), and ROP at moderate to late preterm gestational age (n=6, 18%). The mean age at diagnosis was 8.78 years and 6.42 years for patients with revised diagnosis versus initial diagnosis of FEVR, respectively (p =0.4). There was no correlation between age of diagnosis and stage of disease (p =0.8). CONCLUSIONS: A high index of suspicion for FEVR must be maintained given the heterogeneity of phenotypes. Atypical cases of PFV, Coats disease, and ROP should prompt fluorescein angiography and genetic evaluation.

Ocular Dirofilariasis: A Clinicopathologic Case Series and Literature Review.

Purpose: Ocular dirofilariasis is an uncommon zoonotic infection that is usually associated with a carnivore host. In this case series and literature review, we investigate the clinical presentation, management, and histopathology of ocular dirofilariasis. Methods: The database at the Florida Lions Ocular Pathology Laboratory was searched for surgical specimens at the Bascom Palmer Eye Institute under approval of the Institutional Review Board. Patients with a histopathologic diagnosis of dirofilariasis between the years 1962 and 2022 from the Florida Lions Ocular Pathology Laboratory database were included (n = 3). A systematic PubMed search was conducted by two independent authors to identify published cases of ophthalmic dirofilariasis worldwide. Keywords were used to identify articles, and exclusion criteria were applied. Results: Three patients, two males and one female, were identified from the Florida Lions Ocular Pathology Laboratory database with a diagnosis of ocular dirofilariasis. The mean age was 46.7 years (with a range 33-57 years). There were two eyelid lesions (Cases 1 and 3) and one involving the subconjunctival space (Case 2). All three organisms were excised and presumptively identified as Dirofilaria tenuis. All 3 patients were managed with curative surgical removal and recovered completely. Our review of the literature identified 540 published reports and 142 published reports with 186 cases that met the exclusion criteria. Conclusion: We present a case series and literature review of ocular dirofilariasis. Knowledge of the incidence, risk factors, prevention, and diagnosis of this unique parasitic infection will help in proper management and prevent further ocular complications.

Choroideremia presenting as vision loss secondary to choroidal neovascularization.

BACKGROUND: Choroidal neovascularization (CNV) is a rare complication of choroideremia that occurs secondary to relative atrophy of the retinal pigment epithelium and eventual rupture of Bruch's membrane. The ideal management of CNV in choroideremia is unclear. MATERIALS AND METHODS: Case report. OBSERVATIONS: A 14-year-old male with no known ocular history presented to the eye emergency department complaining of a central scotoma in the right eye for 4 days. He had no past medical history and family history was unremarkable for known ocular disease. Visual acuity was 20/70 in the right eye and 20/30 in the left eye. Posterior segment exam revealed chorioretinal atrophy extending from the outer macula to the midperiphery in both eyes. There was CNV with associated subretinal hemorrhage in the right eye. Optical coherence tomography demonstrated the presence of CNV with subretinal fluid in the right eye and parafoveal outer retinal atrophy in both eyes. Genetic testing revealed a hemizygous exon 2 deletion on the CHM gene, pathogenic for choroideremia. The patient received a total of 3 injections 4 weeks apart followed by 1 injection 6 weeks later with resolution of the subretinal hemorrhage and reduction in CNV size with improvement in visual acuity to 20/20 at last follow-up exam. CONCLUSIONS AND IMPORTANCE: Choroidal neovascularization is a rare cause of central vision loss in patients with choroideremia. In this report, we demonstrate a good functional and anatomic response to intravitreal bevacizumab in a 14-year-old patient with undiagnosed choroideremia who presented with CNV-induced central vision loss.

CENTRAL BOUQUET HEMORRHAGE: Clinical and Multimodal Imaging Features.

PURPOSE: To describe the clinical characteristics, multimodal imaging features, and anatomic basis of a distinctive pattern of deep retinal hemorrhages located in the central fovea, a presentation referred to as "central bouquet hemorrhage." METHODS: Retrospective, observational, multicenter case series of eyes with central bouquet hemorrhage. Multimodal imaging features were reviewed and analyzed. RESULTS: Ten eyes from 10 patients (4 women and 6 men), with a mean age of 55.6 ± 21.7 years (range 25-84 years) were included. Underlying etiologies were neovascular age-related macular degeneration (40%), lacquer cracks in pathological myopia (30%), macular telangiectasia Type 2 (10%), proliferative diabetic retinopathy (10%), and ocular trauma associated with angioid streaks (10%). On ophthalmoscopy, all eyes with central bouquet hemorrhage displayed a deep retinal hemorrhage with round margins in the central fovea and associated with petaloid hemorrhages radiating in the surrounding Henle fiber layer. Cross-sectional optical coherence tomography showed a well-delineated round hyperreflective lesion involving the central foveal Henle fiber layer/outer nuclear layer in all cases. Accompanying hyperreflective hemorrhages tracking along the obliquely oriented Henle fiber layer were present in all eyes. Resolution occurred in all patients, either spontaneously (30%) or after treatment with intravitreal anti-vascular endothelial growth factor injections (70%), and was associated with partial visual acuity improvement (from 20/113 to 20/36). CONCLUSION: "Central bouquet hemorrhage" is a novel descriptive term describing a characteristic round pattern of intraretinal blood in the fovea associated with Henle fiber layer hemorrhage and encountered in a spectrum of macular disease.

Patients With Dragged Optic Disc Vessels and Retinal Folds: Clinical Features, Multimodal Imaging, and Histopathology.

BACKGROUND AND OBJECTIVE: To describe the multimodal imaging and histopathological features of patients with dragged optic disc vessels (DODV). PATIENTS AND METHODS: This is a retrospective, observational analysis using multimodal imaging of eyes with DODV in patients with retinal vascular diseases including familial exudative vitreoretinopathy, inflammatory disease, and others. In addition, two additional enucleated eyes with DODV underwent histopathological analysis. RESULTS: Of the 13 patients, eight were girls and five were boys. Mean age was 5 years (ranging from 4 months to 10 years old). Of the 15 eyes, 12 (80%) demonstrated temporal dragging and three (20%) had nasal dragging. Retinal vascular abnormalities were present in 11 of the fellow eyes. Multimodal imaging demonstrated features of DODV including direction of traction, outer retinal thickening, increased flow, and other features. Only two (13.3%) eyes required surgical intervention. Mean follow-up was 14 (range 3 to 30 months) months. At last follow-up all eyes remained stable. Last visual acuity ranged from 20/100 to counting fingers. As well, two enucleated globes of adults with retinopathy of prematurity underwent histopathologic evaluation, showing optic nerve fibers that extended from the optic nerve into the DODV. CONCLUSIONS: DODV is a sign of various late-stage retinal vascular diseases, associated with poor visual function. Multimodal imaging and histopathology can assist in understanding the disease pathology. [Ophthalmic Surg Lasers Imaging Retina 2023;54:634-642.].

Reusable Fundus Model for Laser Retinopexy Simulation and Training.

BACKGROUND AND OBJECTIVE: To describe a simulation technique for laser barrier retinopexy as an option for ophthalmologists in training to practice slit lamp and laser indirect ophthalmoscopy (LIO) retinopexy. MATERIALS AND METHODS: A commercially available fundus model and laser printer labels were used to simulate laser retinopexy of horseshoe tears (HST). At two time points, seven first-year residents and seven second-year residents were asked to complete three rows of laser burns around HSTs and the times were recorded. RESULTS: Second-year residents completed simulated retinopexies in less time than first-year residents on both the slit lamp (P = .001) and with LIO (P = .001). Second-year residents were also statistically more efficient when retested at the second time point (P = .003 and P = .005, slit lamp and LIO, respectively). CONCLUSION: This novel educational model is able to capture differences in training level and increased experience among ophthalmology trainees, and may be beneficial as part of a structured teaching program. [Ophthalmic Surg Lasers Imaging Retina 2022;53:606-609.].

Acute-onset postoperative endophthalmitis after cataract surgery performed by resident and attending physicians at a university teaching hospital.

PURPOSE: To evaluate acute-onset postoperative endophthalmitis following cataract surgery and to compare rates between resident and attending physician-performed cohorts. SETTING: Bascom Palmer Eye Institute, Miami, Florida. DESIGN: Retrospective chart review. METHODS: Records of patients diagnosed with endophthalmitis within 6 weeks of surgery performed by attending and resident physicians between January 1, 2015, and December 31, 2020, were reviewed. Total cataract volume was obtained from institutional records, and resident case totals were obtained from case logs. Endophthalmitis cases were obtained from billing records and confirmed with chart review. RESULTS: There were 22 cases of endophthalmitis among 32 505 cases (0.068%). Endophthalmitis occurred in 6 of 6447 (0.093%) resident cases and 16 of 26 058 (0.061%) attending cases ( P = .55). The most common bacterial isolates were coagulase-negative Staphylococcus (8/22, 36.3%) and Streptococcus species (3/22, 13.6%), with negative cultures in 10 (10/22, 45.5%). Initial treatment with vitreous tap and injection of intravitreal antibiotics was performed in 21 eyes (21/22, 95.4%) and vitrectomy with intravitreal antibiotic injection in one (1/22, 4.5%). Vitrectomy was performed secondarily in 9 patients (9/22, 40.9%). Corrected distance visual acuity (CDVA) at last follow-up was ≥20/40 in 13 eyes (13/22, 59%) and ≤hand motions in 3 eyes (3/22, 13.6%). CDVA (logMAR mean ± SD) was 1.22 ± 1.16 in resident and 0.49 ± 0.79 in attending cases ( P = .11). CONCLUSIONS: In the current study, acute-onset postoperative endophthalmitis developed infrequently following cataract surgery. The rates and visual outcomes of endophthalmitis were similar in resident and attending cases.

Adoption and Use of Social Media Platforms by Ophthalmology Programs during the COVID-19 Pandemic.

Purpose The aim of this study was to determine whether the coronavirus disease 2019 (COVID-19) pandemic influenced the presence of ophthalmology residency programs on social media platforms. Methods The presence of all accredited ophthalmology residency programs on Twitter and Instagram was assessed for January 2020 and August through March 2021 through an online search. The number of followers and posts per month during these periods was collected for each program. The presence of programs on social media was compared between the two time periods. Results The percentage of programs on Instagram rose from 18% in January of 2020 to 57% in March of 2021 ( p < 0.001). On Twitter, the percentage of programs rose from 24 to 37% ( p < 0.001). From January 2020 to March 2021, the mean number of Instagram followers increased from 265 to 649 ( p < 0.0001), while the mean number of Twitter followers increased from 421 to 532 ( p < 0.0001). The top ten ophthalmology residency programs in 2021 according to Doximity all had Instagram pages, while 57 (53%) of remaining programs had pages ( p = 0.005). Meanwhile, 7 top ten programs (70%) were on Twitter compared with 36 (34%) of the remaining programs ( p = 0.036). Conclusions The presence of ophthalmology residency programs on Instagram and Twitter increased significantly after the onset of the COVID-19 pandemic. Social media represents an evolving platform through which programs can connect with prospective residents.

Ocular trauma secondary to exercise resistance bands during the COVID-19 pandemic.

OBJECTIVE: To characterize injuries caused by exercise resistance bands. METHOD: Single-site retrospective case series of patients presenting to the Bascom Palmer Eye Institute emergency room with ocular injuries secondary to exercise resistance bands from March through September 2020. RESULTS: Eleven patients (9 males, 2 females, 14 eyes) were reviewed. Eight patients had a unilateral injury (3 right eyes, 5 left eyes) while 3 had bilateral injuries. Iritis was the most common presentation, seen in all 11 patients, followed by hyphema (9 patients, 82%), and vitreous hemorrhage (4 patients, 36%). Among affected eyes, the mean presenting visual acuity was approximately 20/100, improving to 20/40 on the last follow up (p = 0.06). However, 4 eyes (33%) had vision ≤20/60 at last follow up. CONCLUSIONS: Exercise resistance bands can cause a wide spectrum of ocular injuries, some leading to long-term vision loss. As such, we recommend that patients strongly consider using eye protection goggles or glasses while using resistance bands for exercise.

Rates of RNFL Thinning in Patients with Suspected or Confirmed Glaucoma Receiving Unilateral Intravitreal Injections for Exudative AMD.

PURPOSE: This study evaluated whether the rate of retinal nerve fiber layer (RNFL) thinning is faster in eyes receiving intravitreal injections than in fellow uninjected eyes among patients suspected of having or confirmed to have glaucoma and exudative age-related macular degeneration (AMD). DESIGN: Retrospective comparative cohort study. METHODS: Patients with a diagnosis of unilateral exudative AMD and confirmed to have or suspected of having glaucoma in both eyes receiving unilateral intravitreal injections were identified. Those with ≥3 RNFL optical coherence tomography scans and ≥6 injections were included in the study. Rates of RNFL thinning in the injected eye versus the uninjected eye were estimated using linear mixed models. The main outcome measurement was the differences in rates of RNFL thinning in the injected versus the fellow uninjected eye. The effects of postinjection elevation of intraocular pressure (IOP), injection frequency, and number of injections were also evaluated. RESULTS: A total of 53 patients met the inclusion criteria, receiving 26.4 ± 15.9 intravitreal injections. The average rate of RNFL thinning in uninjected eyes was -0.620 µm/year (P = .029). Injected eyes had an additional incremental loss of -0.385 µm/year, but this value was not statistically significant (95% confidence interval [CI]: -1.147 to 0.379 µm/year; P = .324). Subgroup analysis with only glaucoma patients (n = 33) also demonstrated a nonsignificant effect of injections (-0.568 µm/year; 95% CI: -1.454 to 0.319 µm/year; P = .212). Postinjection IOP elevation, injection frequency, and total number of injections were not associated with faster RNFL loss. CONCLUSIONS: Among exudative AMD patients with glaucoma or suspected of having glaucoma, the rate of RNFL thinning in eyes receiving intravitreal injections did not significantly differ from that of fellow uninjected eyes.

UV-Photokeratitis Associated with Germicidal Lamps Purchased during the COVID-19 Pandemic.

PURPOSE: To report photokeratitis caused by the improper use of germicidal lamps purchased during the COVID-19 pandemic. METHODS: Case series. RESULTS: Seven patients presented with acute ocular surface pain after exposure to UV-emitting germicidal lamps. Visual acuity was 20/30 or better in 13 of 14 eyes (93%). Anterior segment examination revealed varying degrees of conjunctival injection and diffusely distributed punctate epithelial erosions (PEEs) in every patient. No intraocular inflammation was identified across the cohort and all fundus examinations were normal. Treatment varied by provider and included artificial tears alone or in combination with antibiotic ointments and/or topical steroids. Five patients were followed via telehealth, one patient returned for an in-office visit, and one patient was lost to follow-up. Five of six patients endorsed complete resolution of symptoms within 2-3 days. CONCLUSIONS: Patients should follow manufacturer recommendations when using UV-emitting germicidal lamps and avoid direct exposure to the ocular surface.

Surgical Removal of Intraocular Parasite in a Patient With Diffuse Unilateral Subacute Neuroretinitis.

Diffuse unilateral subacute neuroretinitis (DUSN) is a rare ocular infectious disease caused by migrating larvae of a nematode. DUSN is often a diagnostic challenge as it is difficult to identify the pathogenic nematode on funduscopic examination because it moves frequently and often resides within the subretinal space. Herein, the authors present a patient with unilateral, chronic visual loss who was noted to have a live, motile worm within the vitreous cavity. Initial attempts to treat with laser photocoagulation were unsuccessful. In the accompanying video, the authors report the first case of a live intraocular parasite successfully removed with pars plana vitrectomy.

Known and novel ocular toxicities of biologics, targeted agents, and traditional chemotherapeutics.

PURPOSE: Increases in cancer with an aging population and the rapid development of new chemotherapeutics underscore the need for ophthalmologists to identify and manage potential ocular toxicities. This retrospective case series reports the ocular side effects of traditional and novel chemotherapeutic agents from a large center. METHODS: The medical records of 3537 adult patients 18 years and older who presented to an academic ophthalmology department on high-risk medications identified by ICD-9 search between January 2010 and February 2015 were reviewed. A cancer diagnosis, as well as a temporal association with chemotherapeutic use and ocular side effect, was deemed necessary for inclusion in the study. The main measures were ocular side effects in cancer patients taking chemotherapy, ocular imaging abnormalities, and the outcome of each side effect. RESULTS: Of the 161 oncology patients referred to the ophthalmology clinic for chemotherapeutic screening or ocular side effect, 31 (19.3%) were identified as having an ocular adverse reaction due to a novel or traditional chemotherapeutic medication. A novel flattening of the corneal curvature with hyperopic shift and corneal microcysts was identified in a patient taking the antibody-drug conjugate mirvetuximab soravtansine and was reversible with topical steroids. A bilateral medium-vessel choroidal vasculopathy with serous retinal detachment was seen with ipilimumab. The most frequent medication with ocular toxicity was interferon-α(2b) (IFN-α(2b)) (6/31, 19.4%); headache was typical in these patients (83.3%). Ibrutinib ocular toxicity was second most common (5/31, 16.1%), usually causing red or dry eye, while one patient developed branch retinal artery occlusion. Retinal abnormalities documented on OCT imaging occurred with IFN-α(2b), ipilimumab, binimetinib, and docetaxel, while rod-cone ERG abnormality was seen with cisplatin. Inflammatory conditions included anterior scleritis with zoledronic acid, focal eyelid inflammation with veliparib, bilateral chemosis with R-CHOP, iritis, and blepharospasm with IFN-α(2b). AION occurred with pemetrexed, and transient vision loss with hyperemic disc OS was seen with FOLFOX. Two patients (2/31, 6.5%) developed permanent vision loss. Six patients were lost to follow-up, and the clinical course was unknown (6/31, 19.4%). CONCLUSIONS AND RELEVANCE: Cases of permanent visual loss were observed; yet, in the majority of side effects, they improved with topical therapy and/or holding the medication. Further research is needed to elucidate the incidence and the pathophysiology of these side effects and maximize patient quality of life.