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PURPOSE To evaluate the features and long-term outcomes of hydroxyapatite (HA) orbital implant following enucleation in pediatric patients. METHODS A retrospective review was performed on clinical records of patients ≤18 years of age , managed with enucleation and HA implant placement. Recorded outcomes included implant-related complications, implant and prosthesis motility, and patient cosmetic satisfaction. RESULTS There were 525 children (531 sockets) with HA implant following enucleation. The mean age at HA implant placement was 3 years (median, 2 years; range, 4 days to 17 years). Indications for enucleation included retinoblastoma (n = 457, 86%), uveal melanoma (n = 17, 3%), medulloepithelioma (n = 8, 2%), and others (n = 49, 9%). The HA implant was uncoated (n = 370, 70%) or polymer coated (n = 161, 30%). For the 370 uncoated implants, wrapping was provided with scleral (n = 346, 94%) or bovine pericardium (n = 1

Purpose To evaluate the efficacy of intravitreal bevacizumab for prevention of macular edema after plaque radiotherapy of uveal melanoma. Design Retrospective, single-center, nonrandomized, interventional comparative study. Participants Patients with uveal melanoma treated with plaque radiotherapy were divided into 2 groups: a bevacizumab group and a control group. Intervention The bevacizumab group received intravitreal bevacizumab injection at the time of plaque removal and every 4 months thereafter for 2 years (total, 7 injections). The control group had no intravitreal bevacizumab injection. Both groups had periodic follow-up with ophthalmoscopy and optical coherence tomography (OCT). Main Outcome Measures Development of OCT-evident macular edema. Results There were 292 patients in the bevacizumab group and 126 in the control group. The median foveolar radiation dose was 4292 cGy (bevacizumab) and 4038 cGy (control; P = 0.327). The cumulative incidence of OCT-evident macular edema over 2 years (bevacizumab group vs. control group) was 26% versus 40% ( P = 0.004), respectively; that for clinically evident radiation maculopathy was 16% versus 31% ( P = 0.001), respectively; that for moderate vision loss was 33% versus 57% ( P P = 0.004), respectively. There was no statistically significant difference in clinically evident radiation papillopathy ( P = 0.422). Kaplan-Meier estimates at 2 years showed statistically significantly reduced rates of OCT-evident macular edema ( P = 0.045) and clinically evident radiation maculopathy ( P = 0.040) in the bevacizumab group compared with controls. Conclusions Patients receiving intravitreal bevacizumab injection every 4 months after plaque radiotherapy for uveal melanoma demonstrated OCT-evident macular edema, clinically evident radiation maculopathy, moderate vision loss, and poor visual acuity less frequently over a period of 2 years than patients not receiving the injections.

To assess the efficacy of interferon alpha-2b (IFNα2b) in the management of ocular surface squamous neoplasia (OSSN).This is a retrospective, nonrandomized interventional case series study of 80 patients with 81 tumors treated with IFNα2b eye drops and/or injection combined with surgical excision when necessary. The main outcome measure was complete response or partial response based on the American Joint Committee on Cancer classification.The OSSN was classified as Tis (n = 10, 12%), T1 (n = 13, 16%), T2 (n = 6, 7%), T3 (n = 51, 63%), and T4 (n = 1, 1%). IFNα2b was used as immunotherapy alone (n = 22, 27%) or combined with surgery (n = 59, 73%). Overall (n = 81), complete response was achieved in 90% Tis, in 100% T1, in 100% T2, in 94% T3, and in 100% T4. Specifically for immunotherapy (n = 22), IFNα2b alone achieved complete response in 75% (3/4) Tis, in 100% (8/8) T1, and in 70% (7/10) T3. Planned IFNα2b plus surgery (n = 59) achieved control in 100% (6/6) Tis, in 100% (5/5) T1, in 100% (6/6) T2, in 100% (41/41) T3, and in 100% (1/1) T4. Tumor recurrence was noted in 5% (4/81) of cases over a median follow-up of 1 year. Ocular side effects included conjunctival hyperemia (n = 4, 5%), ocular irritation (n = 3, 4%), superficial punctate keratitis (n = 3, 4%), and conjunctival follicles (n = 1, 1%). Systemic side effects included postinjection flu-like syndrome for 1 day (n = 7, 9%).IFNα2b, when appropriately combined with surgical excision for OSSN, provides complete control in 95% of cases overall, specifically in 90% Tis, in 100% T1, in 100% T2, in 94% T3, and in 100% T4.

To compare a single image with a computer-generated summarized image from the ultra-wide-field fluorescein angiogram (UWFFA) sequence for evaluation of ischemic index (ISI).UWFFA sequences from patients with diabetic retinopathy (DR) (n=5), branch retinal vein occlusion (BRVO) (n=5), and central retinal vein occlusion (CRVO) (n=5) were evaluated by six graders. A single image best illustrating retinal non-perfusion was compared to a summarized image generated by computerized superimposition of angiograms. Non-perfused and ungradable retinal areas were outlined and the ISI between the single and summarized images was compared.The mean ISI in the single versus (vs) summarized images was 17% vs 15% in BRVO (p=0.12), 48% vs 48% in CRVO (p=0.67), and 25% vs 23% in DR (p=0.005). Inter-grader agreement of ISI in single versus summarized images was 0.43 vs 0.40 in BRVO, 0.69 vs 0.71 in CRVO, and 0.53 vs 0.34 in DR.Computer-generated summarized images were similar to single images for grading ISI in BRVO and CRVO, but underestimated it in DR.

Purpose To describe the characteristics of choroidal nevus using the enhanced depth imaging (EDI) feature of spectral-domain optical coherence tomography (OCT). Design Retrospective, observational case series. Participants One hundred four eyes with choroidal nevus. Methods Spectral-domain EDI OCT was performed with a Heidelberg Spectralis HRA+OCT (Heidelberg Engineering, Heidelberg, Germany) using a custom scan acquisition protocol of up to 13 raster lines of 9-mm scan length with automatic real-time image averaging set at 100 images. The thickness of choroidal nevus was measured by combining Heidelberg's autosegmentation with manual segmentation. Main Outcome Measures Imaging features and thickness correlation of choroidal nevus by EDI OCT versus standard ultrasonography. Results Of 104 eyes with choroidal nevus imaged with EDI OCT, 51 (49%) displayed image detail suitable for study. The remaining 53 cases were suboptimal because of statistically identified factors of age older than 60 years ( P = 0.027), female gender ( P = 0.008), extramacular location of nevus ( P P = 0.002), mean distance from optic disc more than 4 mm ( P P = 0.006). Of the 51 suitable cases, mean nevus thickness was 685 μm (median, 628 μm; range, 184–1643 μm) by EDI OCT compared with 1500 μm (median, 1500 μm; range, 1000–2700 μm) by ultrasonography. The most common EDI OCT imaging features included partial (59%) or complete (35%) choroidal shadowing deep to the nevus, choriocapillaris thinning overlying the nevus (94%), retinal pigment epithelial (RPE) atrophy (43%), RPE loss (14%), RPE nodularity (8%), photoreceptor loss (43%), inner segment–outer segment junction (IS–OS) irregularity (37%), IS–OS loss (6%), external limiting membrane irregularity (18%), outer nuclear and outer plexiform layer irregularity (8%), and inner nuclear layer irregularity (6%). Overlying subretinal fluid was identified by EDI OCT (16%), ophthalmoscopic examination (8%), and ultrasonographic evaluation (0%). A comparison of pigmented versus nonpigmented nevus showed only 1 significant difference of more intense choroidal shadowing with pigmented nevus ( P = 0.046). Conclusions Imaging of choroidal nevus with EDI OCT enables precise measurement of tumor thickness with comparatively reduced thickness relative to ultrasonography. Using EDI OCT, 94% of choroidal nevi were found to have overlying choriocapillaris thinning. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Objective To evaluate the efficacy of interferon alpha-2b (IFNα2b) for extensive ocular surface squamous neoplasia (OSSN). Design Retrospective, interventional case series. Participants Eighteen eyes in 18 patients. Methods Each patient with giant OSSN (a single tumor ≥15 mm basal diameter or ≥6 limbal clock-hours) was managed with topical IFNα2b (1 million IU/ml) 4 times daily or with injection IFNα2b (a portion of 10 million IU/ml vial) with follow-up every 1 to 3 months. Main Outcome Measures Tumor response, recurrence, and treatment complications were evaluated. Results Eighteen patients with giant OSSN (median diameter [MD], 20 mm; median clock-hours [MCH], 6) were treated with topical IFNα2b (n = 12), injection IFNα2b (n = 3), or both (n = 3). The IFNα2b achieved complete tumor control (immunotherapy) in 13 eyes and partial tumor control with reduction in size (immunoreduction) in 5 eyes. Topical IFNα2b alone (n = 12) provided complete immunotherapy in 7 eyes (MD, 12 mm; MCH, 9) over a median period of 5 months and immunoreduction by 74% in 5 eyes (MD, 20 mm; MCH, 3), allowing for subsequent surgical excision (n = 3), photodynamic therapy (n = 1), or cryotherapy (n = 1) for tumor control. Injection IFNα2b alone (n = 3; median, 1 injection) provided complete control of giant tarsal conjunctival OSSN (MD, 20 mm) over a 1-month period. A combination of topical and injection IFNα2b (n = 3; median, 3 injections) completely resolved larger tumors (MD, 30 mm; MCH, 6) over a 6-month period. Complications of IFNα2b included transient flu-like symptoms (n = 3), corneal epithelial defect (n = 2), and conjunctival hyperemia (n = 1). During a median follow-up of 11 months, there were no tumor recurrences, but 2 new tumors appeared at a remote site from the original tumor, requiring operative intervention. Conclusions In 72% of giant OSSNs IFNα2b achieved complete control (immunotherapy); there was a reduction in size (immunoreduction) in 28% of giant OSSNs. Financial Disclosure(s) The authors have no proprietary or commercial interest in any of the materials discussed in this article.

Purpose To assess the efficacy of less than 3 cycles of intra-arterial chemotherapy (IAC) for retinoblastoma. Design Retrospective, nonrandomized, interventional case series. Participants Eight patients. Intervention Intra-arterial chemotherapy. Main Outcome Measures Tumor control and globe salvage. Results Eight patients received fewer than 3 cycles of IAC for retinoblastoma because there was complete tumor control with no residual viable tumor (n = 7) or poor response (n = 1) with little hope that further therapy would benefit the patient. In 3 cases, additional vascular compromise precluded further IAC. The treatment was primary in 6 cases and secondary after failure of other treatment in 2 cases. The 8 eyes were classified (International Classification of Retinoblastoma) as group C (n = 2), group D (n = 3), group E (n = 1), and secondary treatment (n = 2). At initial examination, the main tumor showed a mean basal diameter of 16 mm, a thickness of 8.6 mm, vitreous seeds (n = 2), subretinal seeds (n = 6), and iris neovascularization (n = 1). Three patients were treated with a single cycle of IAC, and 5 patients were treated with 2 cycles of IAC. After IAC, complete tumor response was found in 7 eyes (88%) and partial response was found in 1 eye (13%). Over a mean of 13 months follow-up, there was intraretinal tumor recurrence (n = 1), subretinal seed recurrence (n = 1), and no case of vitreous seed recurrence. Globe salvage was achieved in 2 of 2 group C eyes (100%), 3 of 3 group D eyes (100%), 0 of 1 group E eye (0%), and 1 of 2 secondary treatment eyes (50%). Globe salvage was achieved in 6 of 8 eyes (75%), and 2 of 8 eyes (25%) required enucleation. Conclusions One or 2 cycles of IAC can be sufficient for selected eyes with group C or D retinoblastoma, with remarkable tumor control. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

BACKGROUND AND OBJECTIVE: This study assessed the effect of introduction of diabetic retinopathy screening using non-mydriatic digital fundus photography (nDFP) in an urban academic family practice clinic on patient compliance to recommended dilated eye examination (REE). PATIENTS AND METHODS: Two hundred four patients with diabetes mellitis who were noncompliant to annual REE were screened for diabetic retinopathy using nDFP. The images were electronically transmitted to the retinal specialist to triage them for follow-up REE based on the photographic findings. Retrospective review of their medical records compared the compliance to REE before and after nDFP screening. RESULTS: Compliance to REE increased from 9% before screening to 66.5% after screening ( P < .0001). Good quality fundus images were obtained in 93% of the eyes. CONCLUSION: Introduction of nDFP for diabetic retinopathy screening in the family practice clinic can result in a dramatic increase in compliance to REE among patients with diabetes mellitis previously poorly compliant to REE.

To evaluate morphologic features and the evolution of inner retinal dimples using spectral domain optical coherence tomography after internal limiting membrane peeling.A single-center retrospective study of spectral domain optical coherence tomography features in 43 eyes after full-thickness macular hole surgery with internal limiting membrane peeling without adjunctive internal limiting membrane staining.Postoperatively, a total of 210 dimples were noted in 37 eyes (86%), with a mean of 5.7 dimples per eye (median, 5; range, 1-20), most frequently located in the inferotemporal quadrant (n = 122, 58%, P0.0001). At initial detection, mean dimple dimensions were 199 μm wide (median, 183; range, 87-442 μm) and 19 μm deep (median, 17; range, 7-35 μm). During follow-up, mean maximum dimensions were 351 μm wide (median, 305; range, 219-494 μm) and 30 μm deep (median, 31; range, 15-55 μm). Dimple dimension reached a maximum at 12 months (median, 10; range, 2-22 months) followed by a modest decline. Mean preoperative and final postoperative visual acuity were 0.70 logMAR (median, 0.54; range, 0.09-1.4 logMAR) and 0.38 logMAR (median, 0.30; range, 0-1.4 logMAR), respectively.Inner retinal dimples are common after adjunct-free internal limiting membrane peeling and evolve in the postoperative period with enlargement over the first 12 months followed by reduction in size, and have no apparent impact on the visual acuity.

To evaluate the clinical features, treatment, and prognosis of patients with uveal metastasis from lung cancer.Retrospective chart review.There were 194 patients with a diagnosis of uveal metastasis from lung cancer.Radiotherapy, chemotherapy, enucleation, or observation.Ocular tumor control, final visual acuity, and tumor-related death.There were 374 uveal metastatic tumors originating from primary lung cancer in 229 eyes of 194 patients. Tumor location included choroid (88%), ciliary body (2%), and iris (10%), with bilateral involvement in 18%. Diagnosis of uveal metastasis preceded the diagnosis of primary lung cancer in 44% of patients. The choroidal metastatic focus had a mean basal diameter of 8 mm and mean thickness of 3 mm, and were mostly located posterior to the equator (91%). The choroidal metastasis commonly had yellow or orange color (98%), had plateau (61%) or dome (38%) configuration, and displayed associated subretinal fluid (85%). Choroidal tumors were multifocal in 49 cases (23%). Ciliary body tumors were commonly dome shaped (75%) with an episcleral sentinel vessel (75%). Iris tumors were multifocal in 2 cases (13%), had visible intrinsic vessels (97%), and were associated with tumor seeding in the angle (38%) or on the iris stroma (25%). The uveal metastases were treated with teletherapy (31%), chemotherapy (18%), brachytherapy (9%), chemotherapy combined with teletherapy or brachytherapy (14%), enucleation (3%), or observation (21%). At last visit, eyes with follow-up showed tumor regression (66%), stability (12%), growth (14%), recurrence (3%), or new metastasis (5%). Visual acuity improved or remained stable in 59% eyes. One-year mortality from the time of detection of uveal metastasis was 54%.Of 194 patients with uveal metastasis from lung cancer, 44% did not have a history of known lung cancer. Current methods of ocular treatment allow globe salvage in 92% of patients and improved/stable vision in 59% of patients. Systemic prognosis remains poor with tumor-related death in 54% of patients at 1 year.

Purpose To evaluate the outcomes of iris melanoma managed with plaque radiotherapy on the basis of the initial presence or absence of glaucoma. Design Retrospective, comparative case series. Participants A total of 144 patients. Intervention Custom-designed iodine-125 plaque radiotherapy delivering planned 8000 cGy to melanoma apex using transcorneal application. Main Outcome Measures Tumor control and treatment-related complications. Results Of 144 patients with iris melanoma, glaucoma was present at the initial visit in 58 (40%). Causes of elevated intraocular pressure included angle infiltration by melanoma in 50 patients (86%), angle neovascularization in 4 patients (7%), and hyphema in 4 patients (7%). At presentation, the eyes displaying iris melanoma with glaucoma (vs. without glaucoma) were statistically more likely to display angle tumor (66% vs. 43%), with minimal thickness (1.9 vs. 2.9 mm), and melanoma seeding in iris stroma (7 vs. 3 clock hours) and angle (5 vs. 2 clock hours). Plaque radiotherapy was performed in all cases. Kaplan–Meier estimates at 7 years post-treatment revealed no statistical differences in outcomes of local recurrence (14% vs. 15%), enucleation (14% vs. 11%), or metastasis (2% vs. 0%) comparing eyes with and without glaucoma. Of the entire group, multivariate analysis for factors predictive of recurrence included partial (vs. complete) anterior segment irradiation and postradiotherapy glaucoma. Factors related to enucleation included diabetes mellitus, poor initial visual acuity, higher radiation dose to tumor apex, and tumor recurrence. There were no factors predictive of metastasis. Conclusions Iodine-125 plaque radiotherapy provides adequate tumor control for iris melanoma with a low metastatic potential of 1% at 7 years. Iris melanoma with secondary glaucoma showed a statistically significant greater likelihood of flat tumor with iris and angle seeding and no difference in outcomes compared with eyes without glaucoma. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

PURPOSE: To study the effect of intraarterial chemotherapy (IAC) on retinoblastoma-induced retinal detachment. METHODS: Retrospective, noncomparative, interventional case series including 15 patients, with intraarterial (ophthalmic artery) chemotherapy as the intervention. Resolution of retinal detachment was the main outcome measure. RESULTS: Of 15 patients with retinoblastoma-induced retinal detachment managed with IAC, 7 (47%) presented with total retinal detachment and 8 (53%) with partial detachment. The eyes were classified as International Classification of Retinoblastoma group C (n = 2, 13%), group D (n = 4, 27%), and group E (n = 5, 33%). There were 4 eyes (27%) that received IAC as secondary treatment after the failure of other treatment methods. After IAC, all tumors showed regression. Of the 7 eyes with initial total retinal detachment, complete resolution of fluid was found in 3 (43%) and partial resolution in 4 (57%). Of the 8 eyes with initial partial retinal detachment, complete resolution of subretinal fluid was found in all 8 (100%), but 1 eye later developed recurrent subretinal fluid. The mean interval to complete resolution of subretinal fluid was 2 months. Globe salvage was achieved in 5 of 7 eyes (71%) with total retinal detachment and 6 of 8 eyes (75%) with partial retinal detachment. CONCLUSION: Eyes with retinoblastoma-induced total or partial retinal detachment showed complete resolution of detachment after IAC in 43% and 100%, respectively.

Background Analysis of 52 eyes with high-risk retinoblastoma managed with postenucleation adjuvant chemotherapy using vincristine sulfate, etoposide phosphate, and carboplatin showed no evidence of systemic metastasis in any case during a mean (range) follow-up of 66 (12-202) months. Purpose To determine the efficacy of postenucleation adjuvant chemotherapy with vincristine, etoposide, and carboplatin in the prevention of metastasis for patients with high-risk retinoblastoma. Methods Retrospective, nonrandomized, interventional case series of 52 eyes in 51 patients with high-risk retinoblastoma consisting of tumor invasion into the anterior segment, posterior uvea 3 mm or greater, postlaminar optic nerve, or any combination of posterior uvea and optic nerve involvement. Results Of 51 consecutive patients with high-risk retinoblastoma, there were 30 males (59%) and 21 females (41%), with a median age of 28 months at diagnosis. All 52 eyes were classified as group E. The main histopathologic risk factors included anterior segment invasion (7 [13%]), isolated massive posterior uveal invasion of 3 mm or greater (6 [12%]), isolated postlaminar optic nerve invasion (15 [29%]), or any posterior uveal invasion with any optic nerve involvement (24 [46%]). There was additional invasion into the sclera (3 [6%]) and extrascleral structures, including the orbit (1 [2%]). A single histopathologic high-risk factor was present in 32 eyes (62%), whereas 20 eyes (38%) manifested 2 or more high-risk characteristics. Based on previously published series, untreated high-risk retinoblastoma carries at least a 24% risk for metastatic disease. In the present series, using vincristine, etoposide, and carboplatin in all cases, there was no metastasis during a mean follow-up of 66 months (median [range], 55 [12-202] months). Conclusions Retinoblastoma with invasion into the postlaminar optic nerve and/or posterior uvea is at high risk for metastasis and death. In this study, postenucleation chemotherapy using vincristine, etoposide, and carboplatin was effective in preventing metastasis in every case (100%).

Purpose To evaluate iris melanoma in children versus adults. Methods Retrospective, nonrandomized clinical case series including all patients with a clinical diagnosis of iris melanoma managed at the Ocular Oncology Service at Wills Eye Institute over 40 years. Patients were divided into three age categories based on age at presentation: children (≤20 years), mid-adults (21-60 years), and older adults (>60 years). The clinical features, treatments, and outcomes were statistically analyzed based on patient age at presentation. The main outcome measures were melanoma features and related metastasis and death. Results Of 8,101 eyes with uveal melanoma, there were 317 (4%) with iris melanoma, including 24 (8%) children (≤20 years), 187 (59%) mid-adults (21-60 years), and 106 (33%) older adults (>60 years). There was no age-related difference in race, sex, tumor quadrant, thickness, pigmentation, associated corectopia, ectropion uveae, hyphema, or extraocular extension. Significant age-related differences were found with mean tumor basal diameter, tapioca appearance, mean intraocular pressure, secondary glaucoma, tumor seeding in angle, and mean number of clock hours of angle seeding. Multivariate analysis of factors predictive of metastasis included extraocular extension and high intraocular pressure. Factors predictive of death included increased tumor thickness and high intraocular pressure. There was no difference in metastasis or death by age group. Conclusions Iris melanoma shows significant clinical differences in children versus adults, with smaller tumor size, less tumor seeding in angle, and lower incidence of secondary glaucoma. There was no significant difference in metastasis or death by age group.

Purpose To evaluate plaque radiotherapy for management of residual or recurrent iris melanoma after surgical resection. Design Retrospective, nonrandomized interventional case series. Participants We included 32 patients with residual or recurrent iris melanoma after surgical resection. Intervention Custom designed iodine-125 plaque radiotherapy. Main Outcome Measures Tumor control, recurrence, poor visual acuity, enucleation, metastasis, and radiation complications. Results There were 32 eyes with residual (n = 12) or recurrent (n = 20) iris melanoma after surgical resection that were treated with iodine-125 plaque radiotherapy. The residual melanoma was evident clinically in 3 cases and histopathologically in 9; plaque radiotherapy was delivered at a mean interval of 2 months after resection. For the recurrent cases, the mean interval from initial tumor resection to detection of recurrence was 58 months, at which time plaque radiotherapy was applied. For all cases, the mean tumor basal diameter was 6 mm (range, 1–13) and thickness was 2 mm (range, 0.8–4.0) at the time of radiotherapy. Anterior chamber seeding was present in 26 (81%) eyes and glaucoma in 11 (34%) eyes. Visual acuity at presentation was good (20/20–20/50) in 27 (84%), intermediate (20/60–20/150) in 3 (9%), and poor (≤20/200) in 2 eyes (6%). At 6 years after plaque radiotherapy, outcomes included complete tumor control in 87%, poor visual acuity in 9%, enucleation in 13% (for reasons of tumor recurrence [n = 3] and severe glaucoma [n = 1]), and metastasis in 3%. At 6 years, radiation complications included corneal epitheliopathy in 6%, scleral necrosis in 3%, cataract in 53%, elevated intraocular pressure (from tumor or radiotherapy) in 19%, and macular edema in 6%. Conclusions Iodine-125 plaque radiotherapy is effective in the management of residual or recurrent iris melanoma after surgical resection, providing tumor control in 87% of patients at 6 years and avoiding enucleation in most cases. Financial Disclosure(s) The authors have no proprietary or commercial interest in any of the materials discussed in this article.