Intraocular schwannoma with extrascleral extension.

PURPOSE: To report a rare case of intraocular schwannoma with extrascleral extension in a patient with juvenile idiopathic arthritis and to review the literature for this topic. METHODS: Case report. RESULTS: A 19-year-old male with a history of juvenile idiopathic arthritis was referred for diagnosis and management of an episcleral mass, initially thought to be focal nodular scleritis. The ocular surface of the right eye revealed an elevated amelanotic episcleral nodule inferonasally, with thin strands of overlying sclera, feeding episcleral vessels, and measuring 11 mm × 11 mm in diameter and 5 mm in thickness. Ophthalmoscopic examination revealed a minimally pigmented ciliochoroidal tumor measuring 13 mm in diameter and 11.4 mm in total thickness and without associated subretinal fluid, orange pigment, or drusen. Ultrasound biomicroscopy and anterior-segment optical coherence tomography confirmed a solid mass with scleral disruption and extraocular extension. Shave biopsy revealed palisading spindle cells and interspersed eosinophilic fibrillary cytoplasmic processes, forming Verocay bodies. The specimen stained positive for S-100 and negative for Melan-A, consistent with benign schwannoma. Observation was recommended. CONCLUSION: Intraocular schwannoma is a rare, benign uveal tumor that can demonstrate extrascleral extension, mimicking inflammatory and malignant neoplastic processes. Clinical diagnosis is challenging, and tissue biopsy is required for definite diagnosis.

Visual acuity outcomes in Coats disease by classification stage in 160 patients.

PURPOSE: To assess visual outcomes of Coats disease by classification stage. METHODS: A retrospective review was conducted on consecutive patients with Coats disease, classified according to Shields classification and with available Snellen visual acuity before and after treatment. RESULTS: There were 160 eyes with Coats disease (stage 1 (n=2) vs stage 2A (n=17) vs stage 2B (n=22) vs stage 3A1 (n=26) vs stage 3A2 (n=40) vs stage 3B (n=42) vs stage 4 (n=9) vs stage 5 (n=2)). By comparison, more advanced stage showed greater frequency of poor presenting visual acuity (<20/200) (0% vs 0% vs 50% vs 35% vs 38% vs 83% vs 100% vs 100%, p<0.001) and higher mean intraocular pressure (17 vs 15 vs 15 vs 15 vs 15 vs 15 vs 37 vs 26, p<0.001). More advanced stage was less likely managed with laser photocoagulation (100% vs 87% vs 48% vs 62% vs 74% vs 35% vs 0% vs 0%, p<0.001) and more likely with cryotherapy (0% vs 47% vs 81% vs 81% vs 82% vs 88% vs 50% vs 100%, p=0.001). More advanced stage was associated with lower frequency of visual acuity ≥20/40 (100% vs 71% vs 5% vs 42% vs 23% vs 5% vs 0% vs 0%, p<0.001) and greater frequency of visual acuity <20/200 (0% vs 12% vs 36% vs31 % vs 45% vs 88% vs 0% vs 100%, p<0.001). CONCLUSION: Visual acuity in eyes with Coats disease parallels staging with more advanced stage demonstrating poorer visual acuity at presentation and final visit.

Circumscribed Choroidal Hemangioma: Visual Outcome in the Pre-Photodynamic Therapy Era versus Photodynamic Therapy Era in 458 Cases.

PURPOSE: To analyze visual outcomes after treatment of choroidal hemangioma in the pre-photodynamic therapy (PDT) era versus PDT era. DESIGN: Retrospective, comparative case series. PARTICIPANTS: A total of 458 patients with circumscribed choroidal hemangioma. METHODS: Comparison of hemangioma managed in the pre-PDT (1967-2001) era versus PDT (2002-2018) era. MAIN OUTCOME MEASURE: Visual acuity outcome. RESULTS: A total of 458 tumors were treated over this 51-year period. A comparison (pre-PDT [n = 220 cases] vs. PDT [n = 238 cases]) revealed PDT era patients were of older mean age (48.9 vs. 53.8 years, P = 0.002) and were more likely to have systemic hypertension (17.7% vs. 33.8%, P < 0.001), tumor location in the macula (57.4% vs. 67.5%, P = 0.01), subretinal fluid on OCT (33.3% vs. 70.7%, P = 0.01), and greater extent of overlying lipofuscin (P = 0.001). Findings of tumor basal diameter and thickness and fluorescein and indocyanine green angiography were no different in the 2 eras. Treatment (pre-PDT vs. PDT) included argon laser photocoagulation (42.1% vs. 0.4%), PDT (0% vs. 43.8%), transpupillary thermotherapy (0% vs. 0.4%), plaque radiotherapy (7.0% vs. 5.2%), external beam radiotherapy (1.4% vs. 1.3%), enucleation (0.9% vs. 0.4%), and observation (48.6% vs. 47.6%). After treatment, patients in the PDT era demonstrated better mean logarithm of the minimum angle of resolution visual acuity (1.28 vs. 0.51, P < 0.001) (Snellen equivalent 20/400 vs. 20/63, P < 0.001). Final visual acuity was ≥20/40 for those with entering vision of ≥20/40 (59.6% vs. 74.7%, P = 0.001) and for those with entering vision of 20/50-20/200 (25.4% vs. 47.3%, P < 0.001). CONCLUSIONS: Management of choroidal hemangioma in the PDT era has allowed for significantly better visual outcome compared with the pre-PDT era, with mean final visual acuity of 20/400 (pre-PDT era) versus 20/63 (PDT era).

Circumscribed choroidal hemangioma: Clinical features and outcomes by age category in 458 cases.

PURPOSE: To investigate features and outcomes of circumscribed choroidal hemangioma by patient age. METHODS: Retrospective review of circumscribed choroidal hemangioma from 3/29/1967-6/4/2018 based on age at presentation (≤20 vs. >20-50 vs. >50 years). RESULTS: There were 458 circumscribed choroidal hemangiomas diagnosed at mean age (13 vs. 41 vs. 64 years, p < 0.001). The youngest age group had worse presenting visual acuity (20/400 vs. 20/150 vs. 20/100, p < 0.001), larger tumor basal diameter (13.5 vs. 6.6 vs. 6.2, p < 0.001), greater tumor thickness (5.8 vs. 3.1 vs. 2.9, p < 0.001), closer distance to foveola (0.5 vs. 1.4 vs. 1.2, p = 0.03), and greater extent of subretinal fluid (4 quadrants, 26% vs. 8% vs. 2%, p < 0.001). The youngest patients were less likely to be treated with primary observation (39% vs. 39% vs. 56%) or photodynamic therapy (10% vs. 27% vs. 22%) and more likely to be treated with plaque radiotherapy (26% vs. 6% vs. 3%) or external beam radiotherapy (13% vs. 1% vs. 0%) (p < 0.001). The youngest patients required greater total number of treatments (mean 4 vs. 2 vs. 1, p < 0.001). At mean follow-up (44 vs. 68 vs. 60 months, p = 0.37), the youngest patients had worse visual acuity (20/400 vs. 20/200 vs. 20/100, p = 0.03), but no difference in visual acuity loss of 3 or more Snellen lines (27% vs. 13% vs. 16%, p = 0.55). CONCLUSION: Younger patients (≤20 years) with circumscribed choroidal hemangioma present with worse visual acuity and larger, more posterior tumors. Future studies are needed to improve early detection and treatment for this subgroup of patients.

Predictors of enucleation in Coats disease: analysis of 259 eyes of 259 patients at a single center.

PURPOSE: To evaluate the effects of clinical features associated with enucleation in eyes with Coats disease. METHODS: The medical records of all patients with Coats disease at the Ocular Oncology, Wills Eye Hospital from November 1, 1973, to July 31, 2018, were reviewed retrospectively. The clinical features pertaining to need for ultimate enucleation and time to enucleation were compared. RESULTS: The records of 351 eyes were reviewed, of which 259 had follow-up at our center and 32 (12%) were managed with enucleation. Reasons for enucleation included neovascular glaucoma (n = 24 [75%]), possible tumor (6 [19%]), and phthisis bulbi (2 [6%]). Compared to nonenucleated eyes, enucleated eyes had more extensive clock hour involvement of telangiectasia (P < 0.001), light bulb aneurysms (P < 0.001), exudation (P < 0.001), and subretinal fluid (P < 0.001). On adjusted analysis by binomial logistic regression, variables predictive of enucleation included presence of iris neovascularization (P = 0.01), ultrasonographic retinal detachment (P = 0.004), open-funnel retinal detachment (P = 0.04), closed-funnel retinal detachment (P = 0.01), ultrasonographic elevation of subretinal fluid by millimeters (P = 0.001), and angiographic extent of light bulb aneurysms by clock hours (P = 0.02). By Kaplan-Meier analysis of 4-year cumulative risk of enucleation, risk factors for enucleation included presence of iris neovascularization (hazard ratio [HR] 31.0; P < 0.001), ultrasonographic retinal detachment (HR 56.2; P < 0.001), open-funnel retinal detachment (HR 2.7; P = 0.01), and closed-funnel retinal detachment (HR 4.5; P < 0.001). CONCLUSIONS: Clinical features that predict risk of and time to enucleation in eyes with Coats disease include iris neovascularization, ultrasonographic presence and millimeter-elevation of retinal detachment, and angiographic extent of light bulb aneurysms.

Coats Disease: Clinical Features and Outcomes by Age Category in 351 Cases.

PURPOSE: To investigate features and outcomes of Coats disease by patient age. METHODS: Patients with Coats disease from 1973 to 2018 were evaluated based on age category at presentation (3 years or younger vs older than 3 to 10 years vs older than 10 years). RESULTS: There were 351 eyes of 351 patients with Coats disease diagnosed (2 vs 6 vs 27 years, P < .001). The youngest age group had more referral diagnoses of retinoblastoma (29% vs 15% vs 0%, P < .001), worse presenting visual acuity (< 20/200: 80% vs 67% vs 31%, P < .001), more advanced Coats disease stage (stage 3B: 65% vs 38% vs 10%, P < .001), and greater clock-hour extent of telangiectasia (7 vs 5 vs 4, P < .001), light bulb aneurysms (7 vs 4 vs 3, P < .001), exudation (10 vs 7 vs 5, P < .001), and subretinal fluid (10 vs 7 vs 4, P < .001). The oldest patients received a greater total number of treatments (3.3 vs 3.1 vs 4.4, P = .04), with more argon laser photocoagulation (37% vs 52% vs 73%, P < .001) and intravitreal anti-vascular endothelial growth factor (6% vs 9% vs 23%, P < .002) and less cryotherapy (74% vs 84% vs 58%, P < .001). At mean follow-up (70 vs 65 vs 38 months, P = .02), the youngest patients had poorer visual acuity outcome (< 20/200: 83% vs 64% vs 39%, P < .001), had less disease resolution (43% vs 65% vs 62%, P = .01), and were more likely to ultimately require enucleation (22% vs 10% vs 6%, P = .01). CONCLUSIONS: Younger patients (3 years or younger) with Coats disease present with worse visual acuity and more advanced disease stage, and are more likely to require ultimate enucleation. [J Pediatr Ophthalmol Strabismus. 2019;56(5):288-296.].

Factors Predictive of Subretinal Fluid Resolution in Coats Disease: Analysis of 177 Eyes in 177 Patients at a Single Center.

PURPOSE: The aim of this study was to investigate factors predictive of subretinal fluid (SRF) resolution in Coats disease. DESIGN: Retrospective cohort study. METHODS: Institutional review board-approved review of patients diagnosed with Coats disease demonstrating SRF (stage 3-5) at a single center from November 1973 to July 2018 with comparison of eyes that had resolution of SRF to those in which SRF persisted. RESULTS: There were 177 cases (154 males, 87%) of Coats disease diagnosed at a mean age of 8 years. After a mean follow-up of 62 months, SRF resolved in 110 (62%) and persisted in 67 (38%) eyes. Comparison (resolved SRF vs persistent SRF) revealed classification as stage 3A [63 (57%) vs 20 (29%)], stage 3B [47 (43%) vs 40 (60%)], or stage 4 [0 (0%) vs 7 (11%)] (P < 0.001). Eyes with resolved SRF presented with fewer clock hours of telangiectasia (mean: 5 vs 7 clock hours, P < 0.001), light bulb aneurysms (mean: 5 vs 7 clock hours, P < 0.001), exudation (mean: 7 vs 10 clock hours, P < 0.001), and extent of SRF (mean: 7 vs 10 clock hours, P < 0.001). Factors predictive of SRF resolution included absence of iris neovascularization on fluorescein angiography [odds ratio 0.05 (95% confidence interval 0.01-0.60), P = 0.02], and less elevated SRF by ultrasonography [odds ratio 0.84 (95% confidence interval 0.76-0.95), P = 0.004). For every 1-mm decrease in SRF, likelihood of SRF resolution increased by 16%. CONCLUSIONS: Resolution of SRF was achieved in the majority of eyes (62%) with stage 3 to 5 Coats disease. Predictors of SRF resolution included lack of neovascularization on fluorescein angiography and less elevation of SRF by ultrasonography.

Coats disease in 351 eyes: Analysis of features and outcomes over 45 years (by decade) at a single center.

Purpose: To assess features and outcomes of Coats disease over 5-decades. Methods: Retrospective review of Coats disease patients at a single center. Features and outcomes were compared based on decade of presentation. Results: There were 351 patients with Coats disease. The presenting median age (6 years), male sex (84%), and unilaterality (100%) did not change per decade. Coats disease classification did not change per decade with Stage 1 (1%), Stage 2 (21%), Stage 3 (68%), Stage 4 (6%), and Stage 5 (1%). Clinical features that changed per decade (1970s vs. 1980s vs. 1990s vs. 2000s vs. 2010s) included 1980s features of more eyes with exudation in all 4 quadrants (22% vs. 58% vs. 44% vs. 33% vs. 27, P = 0.01) and total exudative retinal detachment (33% vs. 53% vs. 39% vs. 27% vs. 21%, P < 0.001). Imaging features that changed per decade included 2010s greater fluorescein angiographic extent of retinal non-perfusion in mean clock hours (4 vs. 4 vs. 3 vs. 5 vs. 6, P = 0.003), and 1980s greater mean height of retinal detachment ultrasonographically (5 vs. 12 vs. 5 vs. 5 vs. 4 mm, P < 0.001). Treatment features that changed per decade included 1980s greater primary enucleation (11% vs. 16% vs. 3% vs. 4% vs. 1%, P = 0.001), and 2010s greater use of laser photocoagulation (55% vs. 33% vs. 38% vs. 40% vs. 72%, P < 0.001), sub-Tenon corticosteroid (0% vs. 4% vs. 5% vs. 8% vs. 29%, P < 0.001), and intravitreal anti-VEGF) (0% vs. 4% vs. 2% vs. 13% vs. 18%, P = 0.003). Outcomes that changed per decade included 2010s findings of more complete resolution of subretinal fluid (64% vs. 59% vs. 38% vs. 58% vs. 72%, P = 0.01) and less need for primary/secondary enucleation (17% vs. 27% vs. 14% vs. 13% vs. 6%, P = 0.04). Conclusion: Eyes with Coats disease in the 1980s demonstrated more advanced findings, often requiring enucleation. Over the decades, greater use of laser photocoagulation and injections has led to improved disease resolution with greater globe salvage.

Philadelphia Telemedicine Glaucoma Detection and Follow-up Study: Analysis of Unreadable Fundus Images.

PURPOSE: The purpose of this study was to ascertain determinants of unreadable fundus images for participants enrolled in the Philadelphia Telemedicine Glaucoma Detection and Follow-up Study. METHODS: Individuals were screened for glaucoma at 7 primary care practices and 4 Federally Qualified Health Centers using telemedicine. Screening (visit 1) included fundus photography, assessing family history of glaucoma, and intraocular pressure (IOP) measurements. Participants with an unreadable image in at least one eye were deemed unreadable and invited to return for a confirmatory eye examination (visit 2). RESULTS: A total of 906 participants completed the visit 1 eye screening and 17.1% (n=155/906) were "unreadable." In the multivariable logistic regression analysis, older age, male sex, smoking, and worse visual acuity were significantly associated with an unreadable fundus image finding at the eye screening (P<0.05). Of the 89 participants who were invited for the confirmatory eye examination solely for unreadable images and attended visit 2, 58 (65.2%) were diagnosed with at least one ocular pathology. The most frequent diagnoses were cataracts (n=71; 15 visually significant, 56 nonvisually significant), glaucoma suspects (n=27), and anatomical narrow angle (n=10). CONCLUSIONS: Understanding the causes of unreadable fundus images will foster improvements in telemedicine techniques to optimize the predictive accuracy, efficiency, and cost in ophthalmology. A high proportion of participants with unreadable images (65.2%) in our study were diagnosed with some ocular pathology, indicating that the finding of an unreadable fundus image warrants a referral for a comprehensive follow-up eye examination.