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Purpose To report the efficacy of photodynamic therapy (PDT) for management of retinal hemangioblastoma. Design Retrospective case series. Participants Seventeen patients with retinal hemangioblastoma treated with PDT. Methods The medical records of 17 patients with retinal hemangioblastoma treated with PDT were reviewed, and treatment outcomes were assessed. Photodynamic therapy was performed with 6 mg/m2 body surface area of verteporfin infused intravenously over 10 minutes activated by 50 J/cm2 laser light at 689 nm for 83 or 166 seconds. Main Outcome Measures Tumor control, subretinal and intraretinal fluid resolution, and visual outcome. Results Eighteen retinal hemangioblastomas in 17 eyes were treated with PDT. Median patient age was 31 years (mean, 36 years; range, 7–66 years), and median follow-up was 51 months (mean, 61 months; range, 2–144 months). Genetic testing confirmed von Hippel-Lindau disease in 8 of 17 patients (47%). The tumors were unilateral in all patients and unifocal in most patients (n = 13/17 [76%]). The tumor median basal diameter was 3.5 mm (mean, 3.4 mm; range, 1.5–6.0 mm) and median thickness was 2.1 mm (mean, 2.3 mm; range, 1.0–5.0 mm). The tumor location was juxtapapillary in 9 of 18 cases (50%). Associated findings included subretinal fluid (n = 14/17 [82%]) and macular edema (n = 12/17 [71%]). The median number of PDT sessions was 1.5 (mean, 1.8; range, 1.0–4.0). Standard duration of PDT (83 seconds) was used in all cases except 2, in which double duration (166 seconds) was used. Outcomes revealed tumor control in 13 of 18 tumors (72%), partial or complete resolution of subretinal fluid in 10 of 14 eyes (71%), and partial or complete resolution of macular edema in 7 of 12 eyes (58%), and stable or improved visual acuity in 12 of 17 eyes (71%). Photodynamic therapy-related transient exudative response was noted in 4 of 17 eyes (24%). Conclusions Photodynamic therapy is an effective treatment for both juxtapapillary and peripheral retinal hemangioblastomas, providing satisfactory rates of tumor control and visual stabilization and improvement. Patients should be monitored for PDT-related transient exudative response.

Isolated choroidal melanocytosis is a rare condition that appears to be a limited form of ocular melanocytosis. Ocular melanocytosis has been known to be associated with an increased risk of uveal melanoma, and more recently, a similar association has been suggested for isolated choroidal melanocytosis. We describe 3 cases of patients who developed unilateral, multifocal uveal melanoma in the setting of underlying isolated choroidal melanocytosis. All patients developed either two distinct tumors at presentation or a new discrete choroidal melanoma arising from the choroidal melanocytosis over 1 year following treatment of the original tumor by plaque brachytherapy. These cases provide additional evidence of the association between isolated choroidal melanocytosis and uveal melanoma and suggest increased risk of multifocal melanoma in patients with this condition.

Purpose: The aim of the study was to describe choroidal detachments and concurrent scleritis associated with necrotic choroidal metastasis or melanoma. Methods: We conducted a retrospective case series. Results: We report 4 patients with scleritis and choroidal detachment with an underlying malignant choroidal tumor. All patients underwent fine-needle aspiration biopsy for cytopathologic characterization of their choroidal tumor, and they all demonstrated evidence of tumor necrosis. Two patients were diagnosed with choroidal metastasis from lung and esophageal adenocarcinoma. Both patients ultimately expired from systemic metastasis. The remaining 2 patients were diagnosed with choroidal melanoma and were successfully treated with plaque radiotherapy. Conclusion: Choroidal detachment with concurrent scleritis can occur as a rare sequelae of tumor necrosis of an underlying choroidal malignancy.

The purpose of this review is to summarize the current indications for photodynamic therapy (PDT) in the treatment of retinal diseases and intraocular tumors. The recent introduction of new imaging modalities such as optical coherence tomography angiography and swept-source optical coherence tomography has enabled the quick and non-invasive identification of certain retinal conditions that benefit from PDT. Furthermore, recent reports in the literature have confirmed the role of PDT as an effective treatment modality for selected intraocular tumors. While the use of PDT has decreased since its introduction due to the advent of intravitreal anti-vascular endothelial growth factor therapy, there is still a role for this modality in several retinal conditions and intraocular tumors including central serous chorioretinopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy, choroidal neovascularization associated with choroidal nevus, choroidal hemangioma, choroidal metastasis, and retinal hemangioblastoma. PDT should be employed in the appropriate settings.

Intra-arterial chemotherapy (IAC) for the treatment of intraocular retinoblastoma has gained recognition as a method to improve ocular salvage; however, there is a paucity of evidence supporting treatment factors prognosticating ocular survival.All patients with retinoblastoma treated with IAC at a single institution between December 2008 and December 2019 were evaluated. Patient demographics, tumor classification, prior treatments, procedural data, other non-IAC therapies, adverse reactions, procedural complications, ocular outcomes, and overall survival were assessed via retrospective chart review. Factors suggestive of increased ocular survival were identified via univariate and multivariate analyses. The impact of accrued treatment experience was evaluated by grouping eyes by the respective year, IAC treatment was initiated.Forty-nine eyes of 43 patients were treated for retinoblastoma with IAC (256 total procedures). At least grade 3 neutropenia was observed following 19% of IAC procedures. The risk of neutropenia was not statistically different between single or multidrug IAC. Comparing those who received balloon-assisted intra-arterial chemotherapy (bIAC) in more than two-thirds of cycles to those who did not, the risk of arterial access site complications was not statistically different. Multivariate analysis revealed a significantly lower risk of enucleation associated with treatment era in years (hazard ratio [HR] = 0.52-1.00, p .05) and laser therapies (HR = 0.02-0.60, p .05).Ocular survival rates in patients treated with IAC for retinoblastoma at our institution have increased over time. Accrued treatment experience and programmatic changes have likely contributed. Larger, prospective series may lead to a better understanding of factors that consistently contribute to better ocular salvage.

Several neoplastic processes can involve the eye, either primarily or secondary to a systemic malignancy. The most common primary tumors of the eye include conjunctival and uveal melanoma, retinoblastoma, conjunctival and intraocular lymphoma, and ocular surface squamous neoplasia. Metastatic spread from systemic malignancies, especially of the breast and lung, also can involve the eye. A combination of ophthalmologic examination, ancillary testing, and cytologic/histopathologic evaluation leads to accurate diagnosis. Management consists of surgery, radiotherapy, chemotherapy, and immunotherapy delivered in various forms.

Purpose To determine factors predictive of visual outcome in patients with circumscribed choroidal hemangioma treated with photodynamic therapy (PDT). Design Retrospective case series. Participants Seventy-nine patients with circumscribed choroidal hemangioma treated with PDT. Methods Patients with circumscribed choroidal hemangioma treated with PDT were identified, and factors predictive of final visual acuity were assessed. Main Outcome Measures Factors predictive of final visual acuity of 20/40 or better versus 20/50 or worse. Results Seventy-nine eyes of 79 patients with circumscribed choroidal hemangioma were treated with PDT. All tumors were unilateral and posterior to the equator. Mean largest basal diameter was 5.7 mm (range, 2.0–10.0 mm); mean thickness was 3.0 mm (range, 1.4–4.5 mm). A total of 116 PDT sessions were performed (mean, 1.5 sessions; range, 1.0–7.0 sessions). Standard duration PDT was used in most cases (83 seconds; n = 110/116 [95%]). Mean follow-up was 43 months. Of 79 patients, 49 (62%) demonstrated good visual acuity (≥20/40) and 30 (38%) showed intermediate to poor visual acuity (≤20/50) after PDT. A comparison (final visual acuity, good vs. intermediate to poor) revealed a statistically significant difference in baseline features of photopsia (100% vs. 0%; P = 0.04), initial visual acuity of 20/40 or better (77% vs. 23%; P Conclusions In this comparative analysis, PDT was an effective treatment method for circumscribed choroidal hemangioma. Good final visual outcome (≥20/40) was correlated with good baseline visual acuity, smaller tumor size, lack of CME, and lack of treatment before PDT.

Purpose To describe the clinical and imaging characteristics, pathologic features, and management options of retinal pigment epithelium (RPE) adenoma/adenocarcinoma. Design Retrospective case series. Participants Fifty-one patients with RPE adenoma/adenocarcinoma. Methods Treatment options for the patients included observation, partial lamellar sclerouvectomy (PLSU), enucleation, and others. Main Outcome Measures Factors related to visual acuity (VA) outcomes (>2 Snellen lines loss, poor final VA [≤20/200], good final vision [≥20/40]), tumor growth, and need for enucleation. Results The mean patient age at diagnosis was 51 years, and the majority of patients were white (40/51, 78%) and female (34/51, 67%). Primary management included observation (29/51, 57%), PLSU (9/51, 18%), enucleation (4/51, 8%), or others (9/51, 18%). Outcomes revealed decreased VA (10/32, 31%), poor final VA (17/32, 53%), good final VA (11/32, 34%), tumor growth (12/25, 48%), and need for enucleation (7/51, 14%). By multivariable analysis, features predictive of decreased VA included increasing baseline tumor thickness (P = 0.01) and presence of vitreous hemorrhage (P = 0.05). Factors predictive of poor final VA included presence of exudative retinal detachment (P = 0.004), baseline VA 20/50 to 20/150 (P = 0.008), and baseline VA ≤20/200 (P = 0.01). Absence of feeding and/or draining vessel was predictive of good VA (P = 0.004). Tumor growth was associated with multiple treatments (P = 0.02). Increased tumor thickness (P = 0.03) and presence of exudative retinal detachment (P = 0.01) were predictive of enucleation. Conclusion RPE adenoma/adenocarcinoma can simulate choroidal melanoma. Greater tumor thickness, vitreous hemorrhage, exudative retinal detachment, and poor baseline VA predict worse visual outcome and greater risk for enucleation.

Purpose:To analyze risk of nevus transformation into melanoma per millimeter increment.Methods:Retrospective analysis of 3,806 choroidal nevi for transformation into melanoma per incremental millimeter thickness (flat [≤1.0 mm], thin [1.1–2.0 mm], thicker [2.1–3.0 mm], and thickest [>3.0 mm])Results

Purpose:To use multimodal imaging for identification of risk factors for choroidal nevus transformation into melanoma.Methods:Retrospective chart review of 3806 consecutive choroidal nevi with imaging and 2355 choroidal nevi with additional follow up to identify factors predictive of transformation

To identify factors predictive of post-management secondary glaucoma in eyes with iris melanoma.Patients with iris melanoma who were conservatively managed on the Ocular Oncology Service, Wills Eye Hospital from 1970 to 2016 were included. Charts were retrospectively reviewed, and binary logistic regression analysis was performed. Main outcome measures were factors predictive of post-management glaucoma, defined as intraocular pressure (IOP) 22 mmHg following melanoma treatment.Of 271 patients with iris melanoma, melanoma-related glaucoma was identified in 40 (15%) at presentation and post-management glaucoma developed in 75 (28%) at a mean of 103.7 months (range:1.0-120.0). Comparison (post-management glaucoma vs. no glaucoma) revealed patients with post-management glaucoma presented with worse visual acuity (20/50-20/150) (17% vs. 5%, p = 0.001), increased mean tumour basal diameter (5.1 mm vs. 4.3 mm, p = 0.004), greater melanoma-related increased IOP on presentation (24.1 mmHg vs. 16.2 mmHg, p 0.001), diffuse tumour shape (9% vs. 4%, p = 0.01), American Joint Committee on Cancer (AJCC) T4 category (7% vs. 2%, p = 0.03), and extraocular tumour extension (7% vs. 2%, p = 0.03). Risk factors for post-management glaucoma identified by multivariate analysis included melanoma-related increased IOP at presentation (OR:1.1, [1.08-1.22] per 1-mmHg increase, p 0.001), increased mean tumour basal diameter (OR:1.17, [1.02-1.33] per 1-millimetre increments, p = 0.03), advanced AJCC clinical T subcategory (OR:1.23, [1.04-1.46] per 1-subcategory increments, p = 0.02) and plaque radiotherapy treatment (OR:2.32, [1.13-4.75], p = 0.02).Features of iris melanoma that predicted post-management glaucoma included melanoma-related increased IOP on presentation, advanced AJCC clinical T subcategory, increased mean tumour basal diameter, and plaque radiotherapy treatment.

Purpose T' trunk, along with hemi-hypertrophy of the left side of the face and left upper limb, presented for evaluation of an intraocular mass of the left eye (OS). Anterior examination OS showed diffuse episcleral and iris melanocytosis. Fundus examination OS showed diffuse choroidal melanocytosis as well as an elevated choroidal lesion. B-scan ultrasonography demonstrated a hollow lesion, and the patient was diagnosed with choroidal melanoma OS in the setting of phakomatosis pigmentovascularis with overlap of SWS and KTS. Fine-needle aspiration biopsy confirmed the diagnosis, and Iodine-125 plaque radiotherapy was performed. Conclusion Individuals with clinical features suggestive of phakomatosis pigmentovascularis, SWS or KTS should undergo a complete ophthalmological evaluation for the presence of ocular melanocytosis and uveal melanoma.

We report the case of a 9-month-old girl with bilateral retinoblastoma who had incomplete tumor resolution after selective ophthalmic artery infusion chemotherapy (SOAIC). Systemic chemotherapy, rarely used as salvage therapy after SOAIC, with systemic carboplatin, etoposide, and vincristine achieved complete and sustained regression in both eyes.

Neoplasms of the retinal pigment epithelium (RPE) are rare tumors that can simulate choroidal melanoma, but clinical and imaging characteristics often differentiate these lesions. We report a 70-year-old male with an abruptly elevated pigmented lesion that arose at the site of congenital hypertrophy of the RPE and demonstrated associated exudation, as well as feeding and draining vessels, suggestive of RPE adenoma. Optical coherence tomography showed retinal elevation with serous retinal detachment adjacent to the mass, and ultrasonography revealed an abruptly elevated, moderately echodense mass of 6.4-mm thickness. Fluorescein ­angiography showed early tumor hypofluorescence, late ­tumor hyperfluorescence with staining and leakage, and ­retinal vessels buried under the mass, suggestive of a retinal tumor. The patient was monitored with the presumed diagnosis of RPE adenoma, but 3 months later, the growth was documented and fine-needle aspiration biopsy revealed choroidal melanoma. Management with I-125 plaque radiotherapy was performed leading to tumor regression and a thickness of 4.6 mm.

[ Ophthalmic Surg Lasers Imaging Retina. 2021;52:682–683.]

AimTo characterise combinations of multimodal imaging risk factors and predictive value for choroidal nevus transformation into melanoma.MethodsThis is a retrospective review of multimodal imaging features for 3806 choroidal nevi from 1 January 2007 through 1 January 2017. Kaplan-Meier estimates and Cox regression analyses were used to calculate 5-year percentages of growth to melanoma and HR.ResultsUsing multimodal imaging, six risk factors predictive of choroidal nevus transformation into melanoma were identified, namely tumour thickness >2 mm, subretinal fluid, symptoms of visual acuity loss to 20/50 or worse, orange pigment, hollow acoustic density and tumour largest basal diameter >5 mm. Kaplan-Meier 5-year estimated tumour growth was found in 1% of nevi with no risk factors, 11% (range 9%–37%) with one factor, 22% (12%–68%) with two factors, 34% (21%–100%) with three factors, 51% (0%–100%) with four factors and 55% (0%–100%) with five factors. HR for growth was 0.1 with no factor, 2.1–7.8 with one factor, 1.8–12.1 with two factors, 4.0–24.4 with three factors, 4.6–170.0 with four factors and 12.0–595.0 with five factors. The highest HR with each combination of two, three, four or five risk factors always included symptoms of visual acuity loss and orange pigment.ConclusionSix risk factors for choroidal nevus transformation into melanoma by multimodal imaging have been identified. Risk for transformation into melanoma is 1% when no factors are present, and approaches 100% with specific combinations of three or more risk factors. Understanding how combinations of factors influence risk of transformation into melanoma can guide counselling and treatment decisions.

Purpose To describe the relationship of choroidal melanoma with phakomatosis pigmentovascularis in patients with Klippel-Trenaunay syndrome. Methods Retrospective review of 5 patients. Results In all 5 cases, the patient was white and the cutaneous port-wine stain was congenital. The port-wine stain involved the chin (n = 1), jawline (n = 2), lower cheek (n = 1), thorax (n = 5), abdomen (n = 4), upper (n = 4), and lower (n = 3) limb(s). The ocular melanocytosis involved the sclera (n = 5), iris (n = 2) and choroid (n = 4). At diagnosis of choroidal melanoma, mean patient age was 57 years (median 61, range 17-83 years). The melanoma demonstrated mean basal diameter of 11.6 mm (median 12, range 5-16 mm) and mean thickness of 5.7 mm (median 6.1, range 2-9), revealing intrinsic tumor pigment and subretinal fluid in all cases. Melanoma management included plaque radiotherapy (n = 3), thermotherapy (n = 1), or enucleation (n = 1). At mean follow-up of 4 years, one patient demonstrated melanoma-related metastasis with death. Conclusion Phakomatosis pigmentovascularis represents coexistence of Klippel-Trenaunay syndrome (or Sturge-Weber syndrome) and oculo(dermal) melanocytosis, promoting risk for life-threatening uveal melanoma. The authors suggest that all patients with Klippel-Trenaunay syndrome be evaluated for phakomatosis pigmentovascularis and affected patients have dilated fundus examination once or twice a year.

Background and purpose: To report the factors predictive of radio-induced complications (i.e. radiation retinopathy [RR], radiation papillopathy [RP] and neovascular glaucoma [NVG]) in uveal melanoma (UM) patients undergoing gamma knife radiosurgery (GKR). Materials and methods: Longitudinal cohort study on patients with UM treated at the Ocular Oncology Service, San Raffaele Scientific Institute, Milan, between June 1994 and November 2018. Data were retrospectively reviewed. Rates of GKR-related complications were reported. Variables associated with each complication were investigated using multivariable Cox models and confirmed by logistic regression analysis. Hazard ratio (HR) and 95% confidence intervals (CI) were reported for significant associations. Results: One hundred ninety-four patients (99 males, 51%) were included, and 184 tumours were primarily located in the choroid (95%). Median follow-up was 57months (range 6–286). Local control was achieved in 182 eyes (94%), and 152 eyes (78%) experienced at least one radiation-induced complication. Radiation retinopathy was documented in 67 eyes (35%) after a median of 23months. Older age (HR=0.97, 95% CI=0.95–0.99, p=0.02) had a protective effect for RR. Radiation papillopathy was diagnosed in 35 eyes (18%) after a median of 14months after GKR. Macular or peripapillary location (HR=3.06, 95% CI=1.52–6.16, p=0.002) was associated with increased risk of RP, while older age was protective (HR=0.95, 95% CI=0.93–0.98, p=0.001). New-onset NVG was found in 53 eyes (27%), and median onset was 28months. Tumour thickness (HR=4.41, 95% CI=2.23–8.72, p&nbsp

A 34-year-old Caucasian woman with a past medical history of hypertension presented with acute floaters in the right eye (OD) for 1 week. Best-corrected visual acuity (VA) was 20/20 in both eyes. Fundus examination OD (Figure 1 ) revealed a retinal hemangioblastoma (RH) in the temporal midperiphery with a prominent feeding artery and draining vein. A retinal arterial microaneurysm (RAM) was noted within the feeding artery, and subretinal hemorrhage with a cuff of fluid was present. Fundus autofluorescence (Figure 2 ) showed hypoautofluorescence in the area of the RH, RAM, and subretinal hemorrhage. A hyperautofluorescent ring surrounding the hemorrhage suggested shallow subretinal fluid. Early phase fluorescein angiography (Figure 3 ) demonstrated appropriate arterial and venous filling and hyperfluorescence of the RH. The RAM appeared hyperfluorescent, whereas the subretinal hemorrhage blocked. There was no evidence of leakage on late images. Optical coherence tomography (OCT) (Figure 4 ) through the RAM revealed an inner retinal structure with a central lumen and hyperreflective border. Mild outer retinal exudation was also noted. Fovea was normal on OCT. Fundus examination of the left eye was normal. Genetic work-up was negative for von Hippel-Lindau disease. Treatment of the hemangioblastoma was offered, but the patient preferred observation. VA remained stable, and better systemic hypertension control was recommended. [ Ophthalmic Surg Lasers Imaging Retina . 2020;51:365–366. ]

To determine factors predictive of visual outcome in patients with circumscribed choroidal hemangioma treated with photodynamic therapy (PDT).Retrospective case series.Seventy-nine patients with circumscribed choroidal hemangioma treated with PDT.Patients with circumscribed choroidal hemangioma treated with PDT were identified, and factors predictive of final visual acuity were assessed.Factors predictive of final visual acuity of 20/40 or better versus 20/50 or worse.Seventy-nine eyes of 79 patients with circumscribed choroidal hemangioma were treated with PDT. All tumors were unilateral and posterior to the equator. Mean largest basal diameter was 5.7 mm (range, 2.0-10.0 mm); mean thickness was 3.0 mm (range, 1.4-4.5 mm). A total of 116 PDT sessions were performed (mean, 1.5 sessions; range, 1.0-7.0 sessions). Standard duration PDT was used in most cases (83 seconds; n = 110/116 [95%]). Mean follow-up was 43 months. Of 79 patients, 49 (62%) demonstrated good visual acuity (≥20/40) and 30 (38%) showed intermediate to poor visual acuity (≤20/50) after PDT. A comparison (final visual acuity, good vs. intermediate to poor) revealed a statistically significant difference in baseline features of photopsia (100% vs. 0%; P = 0.04), initial visual acuity of 20/40 or better (77% vs. 23%; P0.001), mean tumor basal diameter (5.4 mm vs. 6.2 mm; P = 0.03), mean tumor thickness (2.9 mm vs. 3.2 mm; P = 0.01), cystoid macular edema (CME) involving the foveola (30% vs. 70%; P = 0.001), retinal edema overlying the lesion (39% vs. 61%; P = 0.003), retinoschisis involving the foveola (0% vs. 100%; P = 0.002), lack of CME regardless of foveola involvement at presentation (79% vs. 21%; P0.001), previous treatment (33% vs. 67%; P = 0.04), and CME progression after treatment (0% vs. 100%; P = 0.006). Partial or complete resolution of subretinal fluid was achieved in 93% of patients.In this comparative analysis, PDT was an effective treatment method for circumscribed choroidal hemangioma. Good final visual outcome (≥20/40) was correlated with good baseline visual acuity, smaller tumor size, lack of CME, and lack of treatment before PDT.

Neurofibromatosis type 1 is a genetically determined multisystemic disease that affects up to 1:2500–3500 people throughout the world. Several organs and systems can be affected by the disorder, including the eye and orbit. Some ocular manifestations, such as Lisch nodules in the iris, choroidal nodules, or retinal vascular abnormalities are completely innocuous and require no treatment. Other manifestations such as optic nerve gliomas and plexiform neurofibromas can carry significant morbidity, including vision loss and facial disfigurement. Therefore, the role of the ophthalmologist in managing patients with ophthalmic manifestations of NF1 is significant and varied.

The American Joint Committee on Cancer (AJCC) classification was updated to the eighth edition in January 2017, providing staging for iris melanoma. This study evaluated outcomes of iris melanoma per the AJCC classification, eighth edition.Retrospective case series.Four hundred thirty-two patients with iris melanoma.Management including tumor resection, plaque radiotherapy, or enucleation.Local tumor recurrence, melanoma-related systemic metastasis, and melanoma-related death.Of 432 patients with iris melanoma, AJCC classification was category T1 (n = 324 [75%]), T2 (n = 83 [19%]), T3 (n = 2 [1%]), and T4 (n = 23 [5%]). There was no difference in age, race, gender, eye, or iris color among T categories. Overall, Kaplan-Meier analysis of outcomes (at 5 and 10 years) revealed visual acuity reduction by 3 lines or more (42% and 54%, respectively), secondary glaucoma (29% and 33%, respectively), local recurrence (8% and 17%, respectively), secondary enucleation (12% and 19%, respectively), lymph node metastasis (1% and 1%, respectively), melanoma-related systemic metastasis (5% and 10%, respectively), and melanoma-related death (3% and 4%, respectively). Compared with T1 category, the hazard ratio (HR) for local recurrence in nonenucleated eyes was 1.31 for T2, not evaluable (NE) for T3 (because of small cohort), and 6.61 for T4; the HR for metastasis was 3.41 for T2, NE for T3 (because of small cohort), and 25.6 for T4; the HR for death was 7.51 for T2, NE for T3 (because of small cohort), and 26.5 for T4; and the odds ratio for enucleation was 1.23 for T2, 3.63 for T3, and 4.72 for T4. Features predictive of melanoma-related metastasis (multivariate analysis) included secondary glaucoma (P0.001; HR, 4.51), T2 category (vs. T1; P = 0.01; HR, 4.09), and T4 category (vs. T1; P0.001; HR, 30.8). Features predictive of melanoma-related death (multivariate analysis) included older age (P = 0.008; HR, 2.16 per 10-year increase), T2 category (vs. T1; P = 0.005; HR, 8.07), and T4 category (vs. T1; P0.001; HR, 20.3).The AJCC eighth edition classification provides prognostic stratification of iris melanoma. By multivariate analysis, the ratio for melanoma-related metastasis was 4 times greater in category T2 and 31 times greater in T4 compared with T1. The ratio for melanoma-related death was 8 times greater in category T2 and 20 times greater in T4 compared with T1. The cohort size for T3 was too small to provide useful information.

Purpose: Intraocular tuberculosis (IOTB) can be complicated by choroidal neovascularization (CNV). However, when the CNV development is not accompanied by clear signs of inflammation, the etiology can be missed, especially in countries nonendemic for tuberculosis. We describe the clinical and imaging features of CNVs presenting as the first sign of IOTB initially misdiagnosed as exudative age-related macular degeneration (AMD). Methods: A retrospective review of clinical and imaging data of patients initially misdiagnosed with neovascular AMD later diagnosed with inflammatory CNV secondary to IOTB at tertiary referral centers was conducted. Features of fundus photography, fluorescein angiography, indocyanine green angiography, and enhanced depth imaging optical coherence tomography were analyzed. Distinguishing features between neovascular AMD and IOTB-associated CNV were evaluated. Results: Five patients over 55 years of age, erroneously diagnosed with exudative AMD, were included in the study. Multimodal imaging analysis allowed identification of peculiar choroidal alterations such as choroidal granulomas or choroiditis suggestive for posterior uveitis. Systemic workup for granulomatous uveitis including immunologic investigations such as tuberculin skin test or QuantiFERON TB Gold® and radiologic investigations revealed tubercular etiology in all the cases, allowing correct diagnosis and management of the uveitis and related CNV. Conclusions: Choroidal neovascularization represents a rare and unusual presenting sign of IOTB that can be misleading especially when it occurs in the elderly living in countries with low prevalence of the disease. Multimodal imaging can be helpful and should be employed, especially in atypical cases of CNV, in order to avoid misdiagnosis and/or diagnostic delays.

We studied the scientific literature and disease guidelines in order to summarize the clinical utility of the genetic test for pattern dystrophies. Pattern dystrophies are mostly inherited in an autosomal dominant manner (autosomal recessive transmission is rare). The overall prevalence is currently unknown. Pattern dystrophies are caused by variations in the BEST1, IMPG1, IMPG2, OTX2, PRPH2 and CTNNA1 genes. Clinical diagnosis is based on clinical findings, ophthalmological examination, optical coherence tomography, electrooculography and electroretinography. The genetic test is useful for confirming diagnosis and for differential diagnosis, couple risk assessment and access to clinical trials.

Purpose To describe the clinical and imaging findings and treatment options in a series of patients with iris microhemangiomatosis. Design Retrospective observational case series. Methods Setting : Single institution. Study Population : Twenty-two eyes of 14 consecutive patients with iris microhemangiomatosis were reviewed. Observation Procedures : Clinical examination and slit-lamp photography were performed on every patient. Anterior segment optical coherence tomography (AS-OCT), anterior segment optical coherence tomography angiography (AS-OCTA), anterior segment intravenous fluorescein angiography (AS-IVFA), and ultrasound biomicroscopy were performed when possible. Main Outcome Measures : Clinical and imaging features and treatment strategies. Results Twenty-two eyes of 14 patients with iris microhemangiomatosis were included in this series. Mean patient age was 70 years (range, 58–82 years), and all patients were white. The iris hemangiomatosis appeared as subtle, multifocal, ectatic vascular channels arranged circumferentially at the pupillary margin. Iris color was blue (n = 19 eyes) or green (n = 3 eyes), and the features were unilateral (n = 6 patients) or bilateral (n = 8 patients). Additional features included reduced visual acuity (n = 13 eyes), hyphema (n = 12 eyes), and glaucoma (n = 7 eyes). There was no history of ocular trauma or systemic vasculopathic conditions. AS-OCT documented the fine tuft at the pupillary margin, AS-OCTA showed vascular flow, and AS-IVFA demonstrated pupillary margin vascular lesions with mild staining. Observation was advised in 20 cases (91%), and argon laser photocoagulation was required for repetitive hyphema in 2 cases (9%). Conclusion Iris microhemangiomatosis is a rare ocular condition, often manifesting with spontaneous unilateral hyphema and/or secondary glaucoma. AS-IVFA confirms the diagnosis, and intervention with laser photocoagulation is necessary in only 9% of eyes.

Purpose: The purpose of the study was to investigate the corticosteroids for uveal effusion syndrome (UES). Methods: Retrospective series of 104 eyes with UES treated with oral corticosteroids (OCS), periocular corticosteroids (PCS), topical corticosteroids (TCS), or observation (OBS). Main outcome measure was UES resolution. Results: Of 104 eyes, treatment included OCS (n = 27), PCS (n = 12), TCS (n = 11), and OBS (n = 54). A comparison of the four groups (OCS vs. PCS vs. TCS vs. OBS) revealed differences in those managed with OCS versus OBS as younger (66 vs. 72 years, P = 0.049), PCS versus OBS as male (100% vs. 54%, P = 0.002), PCS versus OBS with decreased visual acuity (VA)/visual field (91% vs. 51%, P = 0.018), and OBS versus OCS as asymptomatic (28% vs. 0%, P = 0.001). Of the 59 with follow-up information, management included OCS (n = 21), PCS (n = 12), TCS (n = 6), and OBS (n = 20). There were differences in initial VA

Purpose Our aim was to prospectively analyze the use of contrast-enhanced ultrasound (CEUS) in the quantitative assessment of the response of uveal melanoma (UM) to gamma-knife radiosurgery (GKR), investigating whether changes in tumor vascularization precede thickness reduction, which on average occurs at 12 months after GKR. Methods Ten patients with UM treated with GKR underwent sonography (US) and CEUS at baseline and at 3, 6, and 12 months after GKR. The transverse diameter, thickness, and quantitative parameters of the UM (ie, area under the curve in the wash-in phase, wash-in perfusion index, peak enhancement, and wash-in rate) were calculated by using dedicated software and compared by using Wilcoxon's signed-rank test. Results The mean tumor thickness on US was significantly less at both 6 (6.6 mm) and 12 months after GKR (5.8 mm) than it was at baseline (8.3 mm; p

Red blood cell, white blood cell, and platelet disorders often lead to a large variety of hematologic effects in ocular tissues, and especially in the retina. Although retinal findings may not be pathognomonic of a specific blood disorder, ophthalmologists can directly visualize characteristic retinal changes and be the first to diagnose a blood dyscrasia. Typical findings on indirect ophthalmoscopy are retinal hemorrhages, microaneurysms, hard exudates, cotton wool spots, macular edema, retinal vascular edema, pallor of the disc, edema of the disc, retinal and optic disc neovascularization, vitreous hemorrhage and inflammation, retinal and retinal pigment epithelium detachment, and retinal and choroidal infiltrates. Other retinal findings may be more indicative of a specific blood disorder, such as thalassemia or sickle cell disease. High-resolution imaging can help ophthalmologists in the detection of early retinal changes or more peculiar findings and can guide physicians in an early diagnosis of a specific blood dyscrasia.

AimsThe aim of the present work is to assess the main predictors of the most clinically relevant radio-induced effects after Gamma Knife stereotactic radiosurgery (GKRS) for uveal melanoma (UM).Materials and methodsMedical records and three-dimensional dosimetry data of critical structures of 66 patients were retrospectively reviewed. Cox’s proportional hazard model was used to identify clinical and dosimetric variables as independent risk factor for GKRS-related complications.ResultsThe fraction of the posterior segment receiving more than 20Gy (V20), Bruch’s membrane rupture and tumour thickness were significant prognostic factors for neovascular glaucoma. A clear relationship with the dose received by 1% of the optic nerve (D1%) was found for radiation retinopathy and papillopathy. Multivariables models resulted for visual acuity (VA) reduction >20% of the basal value and for complete VA loss, both including largest tumour diameter and D1% to the optic nerve. The predictive model for complete VA loss includes also Bruch’s membrane rupture. An alternative model for complete visual acuity loss, including the optic nerve-prescription isodose minimum distance, was also suggested.ConclusionsWe found clinical and dosimetric variables to clearly predict the risk of the main side effects after GKRS for UM. These results may provide dose constraints to critical structures, potentially able to reduce side effects. Constraining D1% to the optic nerve below 12-13Gy may result in a dramatic reduction of blindness risk, while reducing V20 of the posterior segment of the bulb could limit the neovascular glaucoma onset.

Purpose: To evaluate demographic characteristics, clinical features, systemic disease associations, visual outcomes, and treatment modalities of patients with episcleritis and scleritis in an Italian tertiary care referral center. Methods: Data from 25 patients with episcleritis and from 85 patients with scleritis followed from 2003 to 2012 were retrospectively evaluated. The main outcome measures were demographics, ocular disease characteristics, presence of systemic associated disease, treatment regimen, and follow-up period. Results: Episcleritis and scleritis were found bilaterally in 24% and 31% of patients, respectively (p

Early detection of choroidal melanoma at a small tumor size is emphasized in the literature. However, there is little published information on the specific risks of plaque-irradiated small choroidal melanoma on visual acuity and metastasis.To analyze outcomes of plaque radiotherapy for small choroidal melanoma 3 mm in thickness or less.This retrospective noncomparative series at a tertiary referral center included 1780 consecutive patients who had received plaque radiotherapy treatment for small choroidal melanoma.Visual acuity outcomes and melanoma-associated metastasis, assessed by Kaplan-Meier analyses.The mean (SD) patient age at melanoma diagnosis was 58 (14) years. Of 1780 patients, 908 were female (51.0%), and 1752 were white (98.4%). Visual acuity was 20/40 OU or better in 1276 of the patients (71.7%), and the mean (SD) visual acuity was 20/40 (20/50) OU (median, 20/30; range, 20/20 to counting fingers). The mean (SD) tumor basal dimension was 8.8 (2.9) mm (median, 8.0 mm; range, 2.0-20.0 mm) and mean (SD) tumor thickness was 2.6 (0.5) mm (median, 2.7; range, 0.2-3.4 mm). Mean (SD) distance to the foveola was 3.4 (3.9) mm and to the optic disc was 3.7 (3.7) mm. The Kaplan-Meier rate of visual acuity loss (≥3 Snellen lines) was 9.5% (95% CI, 8.2%-11.0%) at 1 year, 39.2% (95% CI, 36.5%-42.0%) at 5 years, and 48.9% (95% CI, 45.6%-52.3%) at 10 years, whereas poor visual acuity (≤20/200) was 7.1% (95% CI, 5.9%-8.4%) at 1 year, 38.2% (95% CI, 35.5%-41.1%) at 5 years, and 53.5% (95% CI, 50.1%-57.1%) at 10 years. Regarding melanoma-associated metastasis, the rate was 0.2% (95% CI, 0.09%-0.6%) at 1 year, 4.5% (95% CI, 3.4%-5.9%) at 5 years, and 8.8% (95% CI, 6.9%-11.1%) at 10 years. Using 1.0-mm thickness increments, the 10-year risk for metastasis was 25.0% (95% CI, 3.9%-87.2%) at 0-mm to 1.0-mm thickness, 5.9% (95% CI, 2.5%-13.5%) at 1.1-mm to 2.0-mm thickness, 8.1% (95% CI, 5.9%-11.0%) at 2.1-mm to 3.0-mm thickness, and 13.4% (95% CI, 8.7%-20.4%) at thicknesses greater than 3.0 mm. The greater relative risk (RR) for metastasis in thinnest tumors was 1.83 (95% CI, 1.09-3.07), which likely represented more aggressive diffuse (flat) melanoma. By multivariable analysis, clinical features predictive of melanoma-associated metastasis included increasing patient age (RR, 1.32 [95% CI, 1.07-1.63] per decade; P = .01), tumor diameter (RR, 1.15 [95% CI, 1.06-1.24] per mm; P .001), tumor thickness (RR, 2.22 [95% CI, 1.22-4.05] per mm; P = .01), photopsia symptoms (RR, 2.45 [95% CI, 1.35-4.43]; P = .003), and prior treatment before plaque radiotherapy (RR, 3.31 [95% CI, 1.31-8.33]; P = .01).This retrospective study suggests that small choroidal melanoma treated with plaque radiotherapy has a 10-year risk for visual acuity loss of 48.9% (95% CI, 45.6%-52.3%) and a 10-risk of systemic metastasis of 8.8% (95% CI, 6.9%-11.1%). In this analysis, each millimeter of increasing thickness and diameter contributed risk for metastatic disease.

Deferoxamine mesylate (DFO) is the most commonly used iron-chelating agent to treat transfusion-related hemosiderosis. Despite the clear advantages for the use of DFO, numerous DFO-related systemic toxicities have been reported in the literature, as well as sight-threatening ocular toxicity involving the retinal pigment epithelium (RPE). The damage to the RPE can lead to visual field defects, color-vision defects, abnormal electrophysiological tests, and permanent visual deterioration. The purpose of this review is to provide an updated summary of the ocular findings, including both functional and structural abnormalities, in DFO-treated patients. In particular, we pay particular attention to analyzing results of multimodal technologies for retinal imaging, which help ophthalmologists in the early diagnosis and correct management of DFO retinopathy. Fundus autofluorescence, for example, is not only useful for screening patients at high-risk of DFO retinopathy, but is also a prerequisite for identify specific high-risk patterns of RPE changes that are relevant for the prognosis of the disease. In addition, optical coherence tomography may have a clinical usefulness in detecting extent and location of different retinal changes in DFO retinopathy. Finally, this review wants to underline the need for universally approved guidelines for screening and followup of this particular disease.

Our aim was to prospectively analyze the use of contrast-enhanced ultrasound (CEUS) in the quantitative assessment of the response of uveal melanoma (UM) to gamma-knife radiosurgery (GKR), investigating whether changes in tumor vascularization precede thickness reduction, which on average occurs at 12 months after GKR.Ten patients with UM treated with GKR underwent sonography (US) and CEUS at baseline and at 3, 6, and 12 months after GKR. The transverse diameter, thickness, and quantitative parameters of the UM (ie, area under the curve in the wash-in phase, wash-in perfusion index, peak enhancement, and wash-in rate) were calculated by using dedicated software and compared by using Wilcoxon's signed-rank test.The mean tumor thickness on US was significantly less at both 6 (6.6 mm) and 12 months after GKR (5.8 mm) than it was at baseline (8.3 mm; p 0.05, both comparisons). Compared with baseline data, the median flow quantitative parameters on CEUS were significantly changed as follows: the peak enhancement (in arbitrary units [au]) at baseline was 5 × 10(6) ; 6 months after GKR, it was 2 × 10(1) (p 0.05), and 12 months after GKR, it was 4 × 10(1) (p0.05). The wash-in rate (in au) at baseline was 1 × 10(6) ; 6 months after GKR, it was 2.1 (p 0.05), and 12 months after GKR, it was 9.3 (p 0.05). The wash-in perfusion index (in au) at baseline was 2 × 10(7) ; 6 months after GKR, it was 7 × 10(1) (p 0.05), and 12 months after GKR, it was 1 × 10(2) (p 0.05). The area under the curve during the wash-in phase (in au) at baseline was 1 × 10(8) ; 12 months after GKR, it was reduced to 6 × 10(2) (p 0.05).At 6 months after GKR, a reduction of tumor thickness, as detected on US, occurred in 6 of the 10 patients, whereas a reduction in all the quantitative parameters measured on CEUS occurred in all 10 patients. However, a larger population is needed to investigate whether CEUS could become the first-choice technique for monitoring the response of UM to GKR.