Ultra-low-dose (boom-boom) radiotherapy for choroidal lymphoma in three consecutive cases.

PURPOSE: To describe the outcome of ultra-low-dose (boom-boom) radiotherapy for choroidal lymphoma. METHODS: Retrospective series of three consecutive patients with biopsy-proven choroidal lymphoma treated with ultra-low-dose radiotherapy. RESULTS: The three patients (two male, one female) of mean age 70 years (range, 64-74 years) demonstrated presenting visual acuity in the affected eye between 20/40 and 20/50. The choroidal lymphoma was unilateral in all cases and presented with multifocal yellow patchy choroidal infiltration, located in all four quadrants and measuring mean 2.9 mm (range, 1.9-4.0 mm) in thickness by ultrasonography. Anterior epibulbar extension of 5 mm diameter was noted in one case. By enhanced depth imaging optical coherence tomography, the choroidal infiltration demonstrated classic undulating appearance (n = 3), with subretinal fluid (n = 2) and intraretinal edema (n = 1). There was no systemic lymphoma in any case. Biopsy was performed in all three cases and was diagnostic (n = 1) or suggestive (n = 2) of B-cell lymphoma. Management involved ultra-low-dose radiotherapy (4 Gy delivered in two fractions, "boom-boom"). On follow-up (mean = 14 months, range = 6-24 months), complete tumor regression on ophthalmoscopy was documented in all three cases, with enhanced depth imaging optical coherence tomography and ultrasonography demonstrating evidence of lymphoma resolution and visual acuity improvement to 20/25-20/40. There were no radiation complications. CONCLUSION: In this small case series, ultra-low-dose (boom-boom) radiotherapy was effective for choroidal lymphoma with favorable response and minimal side effects.

Visual outcomes of proton beam therapy for choroidal melanoma at a single institute in the Republic of Korea.

We evaluate the ocular effects of proton beam therapy (PBT) in a single institution, in Korea, and identify factors contributing to decreasing visual acuity (VA) after PBT. A total of 40 patients who received PBT for choroidal melanoma (2009‒2016) were reviewed. Dose fractionation was 60‒70 cobalt gray equivalents (CGEs) over five fractions. Complete ophthalmic examinations including funduscopy and ultrasonography were performed at baseline and at 3, 6, and 12 months after PBT, then annually thereafter. Only patients with at least 12 months follow-up were included. During the follow-up, consecutive best-corrected visual acuity (BCVA) changes were determined, and univariate and multivariate logistic regression analyses were performed to identify predictors for VA loss. The median follow-up duration was 32 months (range: 12‒82 months). The final BCVA of nine patients was > 20/40. The main cause of vision loss was intraocular bleeding, such as neovascular glaucoma or retinal hemorrhage. Vision loss was correlated with the tumor size, tumor distance to the optic disc or fovea, maculae receiving 30 CGEs, optic discs receiving 30 CGEs, and retinas receiving 30 CGEs. Approximately one-third of PBT-treated choroidal melanoma patients with good pretreatment BCVA maintained their VA. The patients who finally lost vision (VA < count fingers) usually experienced rapid declines in VA from 6‒12 months after PBT. Tumor size, tumor distance to the optic disc or fovea, volume of the macula, and optic discs or retinas receiving 30 CGEs affected the final VA.

Efficient Coding by Midget and Parasol Ganglion Cells in the Human Retina.

In humans, midget and parasol ganglion cells account for most of the input from the eyes to the brain. Yet, how they encode visual information is unknown. Here, we perform large-scale multi-electrode array recordings from retinas of treatment-naive patients who underwent enucleation surgery for choroidal malignant melanomas. We identify robust differences in the function of midget and parasol ganglion cells, consistent asymmetries between their ON and OFF types (that signal light increments and decrements, respectively) and divergence in the function of human versus non-human primate retinas. Our computational analyses reveal that the receptive fields of human midget and parasol ganglion cells divide naturalistic movies into adjacent spatiotemporal frequency domains with equal stimulus power, while the asymmetric response functions of their ON and OFF types simultaneously maximize stimulus coverage and information transmission and minimize metabolic cost. Thus, midget and parasol ganglion cells in the human retina efficiently encode our visual environment.

RETINAL OXIMETRY IS ALTERED IN EYES WITH CHOROIDAL MELANOMA BUT NOT IN EYES WITH CHOROIDAL NEVI.

PURPOSE: To compare retinal vessel oxygenation in eyes with an untreated choroidal nevus or choroidal melanoma. METHODS: The affected and fellow eye of patients with an untreated choroidal nevus (n = 42) or choroidal melanoma (n = 45) were investigated using noninvasive retinal oximetry (Oxymap T1). Oxygen saturation of arterioles (ArtSat) and venules (VenSat) was determined, together with the arteriovenous difference (AV-difference). RESULTS: In choroidal nevus patients, retinal oximetry did not differ between the affected and fellow eye: the mean ArtSat was 94.5% and 94.2% (P = 0.56), the VenSat was 60.5% and 61.3% (P = 0.35), and the AV-difference was 34.0% and 32.9% (P = 0.18), respectively. In choroidal melanoma patients, alterations were detected: the mean ArtSat was 94.8% and 93.2% (P = 0.006), the VenSat was 58.0% and 60.0% (P = 0.014), and the AV-difference was 36.8% and 33.2% (P < 0.001), respectively. The largest increase in AV-difference was observed between the retinal halves without the lesion in melanoma eyes compared with the corresponding half in the fellow eye (37.5% vs. 32.1%, P < 0.001). CONCLUSION: Although retinal oximetry was not significantly altered in eyes with a choroidal nevus, eyes with choroidal melanoma showed an increased ArtSat and decreased VenSat, leading to an increased AV-difference. These changes may be caused by inflammation and a higher metabolism, with larger oxygen consumption, leading to altered blood flow and intraocular oxygen relocation.

Optical coherence tomography angiography-based analysis of intrinsic vasculature in juxtapapillary melanoma after ruthenium-106 plaque brachytherapy.

In this case report, we demonstrate the use of optical coherence tomography angiography (OCTA) as a tool to evaluate intrinsic vasculature in a case of juxtapapillary melanoma which underwent ruthinium.106 plaque brachytherapy. In this case, OCTA could demonstrate a decrease in caliber and density of the intrinsic vasculature of the tumor post brachytherapy.

The Effects of Ocular Perfusion Pressure Variations on a Case of Circumscribed Choroidal Hemangioma Affected by Primary Open-Angle Glaucoma.

The authors describe the clinical and anatomical correlation between mean ocular perfusion pressure (MOPP) changes and macular exudation in a 62-year-old man affected by circumscribed choroidal hemangioma (CCH) and primary open-angle glaucoma. During the 5-year follow-up, we noted an increase of the subretinal fluid (SRF) and cystoid macular edema (CME) when the intraocular pressure (IOP) was in the low teens. Optical coherence tomography scans performed during an isometric exercise revealed a transient increase of the retinal (choroidal) thickness. In the presented case, MOPP appears to play a role in SRF and CME associated with CCH. Management of the macular exudation could improve if IOP and blood pressure are considered and treated accordingly. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e163-e165.].

Combined Parafoveal Telangiectasia, Multifocal Central Serous Chorioretinopathy, and Choroidal Hemangioma.

The exact pathogenesis of parafoveal telangiectasia (PFT) is unknown, but various associations have been reported in literature such as diabetes and hypertension. Coexisting pathologies like diabetic retinopathy with PFT are not uncommon. We encountered a unique combination of PFT with multifocal central serous chorioretinopathy and choroidal hemangioma in a patient with diabetes mellitus. Multimodal imaging confirmed the findings and focal treatment to central serous chorioretinopathy leaks led to resolution of the subretinal fluid. This unique combination has been rarely reported so far. The long-term follow-up of such cases can help us to understand if there exists common pathogenesis of these varied diseases.

TREATMENT OF EXUDATIVE CIRCUMSCRIBED CHOROIDAL HEMANGIOMA: Efficacy of Fractionated Proton Therapy (20 Gray Relative Biological Effectiveness in 8 Fractions).

PURPOSE: To evaluate treatment of circumscribed choroidal hemangioma by hyperfractionated proton beam therapy protocol (20 gray relative biological effectiveness in 8 fractions) on tumor control, attachment of retina and visual function. METHODS: Retrospective review of patients treated between January 2010 and April 2015 with at least 6 months of follow-up. RESULTS: Forty-three patients with exudative and symptomatic circumscribed choroidal hemangioma were included. Before treatment, 41 (95%) presented an exudative retinal detachment, median visual acuity was 20/63 and median tumor thickness was 3.3 mm. Mean follow-up was 26 months (7-62). At last follow-up, all patients presented regression of ultrasound tumor thickness and 23/43 (53.5%) a totally flat scar. The mean time to achieve a flat scar was 20 months. Retina was reattached in all patients except one with 9 months of follow-up. Visual acuity was improved or stabilized in 37 patients (86%) and final median visual acuity was 20/25. No patient presented radiation maculopathy or papillopathy. CONCLUSION: Proton beam therapy with a dose of 20 gray relative biological effectiveness delivered in 8 fractions provides excellent anatomical and functional results and are comparable with those obtained with the same dose delivered in 4 fractions. Longer follow-up is required to determine the long-term radiation sequelae.

Rhegmatogenous retinal detachment in a patient with choroidal melanoma simulating choroidal detachment: a case report.

BACKGROUND: Ophthalmologists and retina specialists may consider choroidal detachment if patients with rhegmatogenous retinal detachment present with choroidal elevation. That misdiagnosis may lead to inappropriate treatments, development of tumor cell dissemination, and eventual promotion of patient death. We report a case of a patient with rhegmatogenous retinal detachment associated with choroidal melanoma simulating choroidal detachment according to fundus findings. CASE PRESENTATION: A 78-year-old Japanese woman with blurred vision in her right eye was referred to our hospital because of rhegmatogenous retinal detachment with complicated atypical choroidal detachment. Her intraocular pressure was normal with clear anterior chamber. Retinal detachment involving the inferior and nasal retina was observed, and a retinal hole was noted in the same quadrant. A small yellowish choroidal elevation was located in the inferonasal site. Gadolinium-enhanced magnetic resonance imaging revealed enhancement corresponding to the elevation, leading to the identification of a choroidal tumor. Enucleation of the patient's right eye was eventually performed. The enucleated eye histologically demonstrated malignant melanoma. CONCLUSIONS: If hypotony or an inflammatory sign is absent, ophthalmologists should pay attention to the differential diagnosis of choroidal elevations observed in such patients.

Visual Outcome and Millimeter Incremental Risk of Metastasis in 1780 Patients With Small Choroidal Melanoma Managed by Plaque Radiotherapy.

Importance: 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. Objective: To analyze outcomes of plaque radiotherapy for small choroidal melanoma 3 mm in thickness or less. Design, Setting, and Participants: This retrospective noncomparative series at a tertiary referral center included 1780 consecutive patients who had received plaque radiotherapy treatment for small choroidal melanoma. Main Outcomes and Measures: Visual acuity outcomes and melanoma-associated metastasis, assessed by Kaplan-Meier analyses. Results: 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). Conclusions and Relevance: 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.

Outcome Analysis of Visual Acuity and Side Effect after Ruthenium-106 Plaque Brachytherapy for Medium-sized Choroidal Melanoma.

PURPOSE: The purpose of this study is to report on treatment outcomes for medium-sized choroidal melanoma treated with Ruthenium-106 (Ru-106) plaque brachytherapy. METHODS: A retrospective case series of 28 patients received Ru-106 brachytherapy treatment for choroidal melanoma. The prescribed tumor dose was 85 Gy to a depth of 5 mm. RESULTS: Median follow-up was 31.2 months. At 12 and 24-month postirradiation, the best corrected visual acuity ≥20/70 (LogMar ≥-0.54) was 53.8% and 64.2%, respectively. Median time to tumor regression was estimated to be 10 months (95% CI = 9-18 months), with 100% of response rate by 32 months. Radiation-induced side effects were limited, and there were no postradiation enucleations. CONCLUSIONS: The majority of patients maintained good visual acuity, with no enucleations and minimal side effects. In this cohort, the Ru-106 plaque brachytherapy proved to be an efficacious and safe treatment option for patients with medium-sized choroidal melanomas with a maximal tumor height of 5 mm.

Atypical posterior scleritis mimicking choroidal melanoma.

We report a case of atypical posterior scleritis mimicking amelanotic choroidal melanoma. A 30-year-old healthy Filipino man, with a history of painless subacute loss of vision in his left eye over 5 months, was referred to our institute for further workup and management. On examination, visual acuity of the left eye was 20/200. Anterior segment examination yielded unremarkable results, with injected conjunctiva and quiet episcleral blood vessels, while fundus examination revealed non-pigmented nasal choroidal mass, with significant subretinal fluid resembling amelanotic choroidal melanoma. Right eye examination yielded unremarkable results. The patient was diagnosed with atypical posterior scleritis, and treated with oral steroids for 2 weeks, with no improvement. A periocular steroid was then injected to the left eye, causing dramatic reduction in choroidal mass size, and complete resolution of subretinal fluid. The visual acuity improved to 20/28.5 one month after the injection. Timely treatment was crucial for minimizing vision-threatening complications.

Dose-response in choroidal melanoma.

PURPOSE: In choroidal melanoma the radiation threshold dose for local control remains largely unknown. The present study examined a group of patients that received a wide range of minimum tumor dose in order to investigate a dose-response relationship. A literature review is performed to compare our results with available evidence in brachytherapy and charged particle external beam radiotherapy. MATERIALS AND METHODS: A retrospective study was conducted on all choroidal melanomas treated with Strontium-90 (Sr-90) at the University Hospital of Leuven between 1983 and 2012. Local failure was defined as primary endpoint and was estimated according to the competing risk method. RESULTS: In 135 patients, the minimum tumor dose (Dmin) ranged from 0 Gy to 287 Gy (median: 27.6 Gy). Multivariable analysis revealed Dmin ≥ 65 Gy (p = 0.04; HR = 0.09) and tumor distant from the optic disc (p < 0.001, HR = 0.09) to be independent variables favoring local control. The scleral dose, the tumor diameter and tumor height did not significantly affect local failure in multivariate analysis. CONCLUSION: This is the first study to examine a group of patients treated with a Dmin ranging from 0 Gy to >250 Gy. Treatment with a Dmin of 65 Gy is necessary to achieve durable tumor response. The dose-response data provided by our study could be used for the design of future trials examining the ideal dose for the treatment of choroidal melanoma with brachytherapy or charged particle external beam radiotherapy.

Optical coherence tomography angiography features of choroidal hemangioma.

This is an observational case series of four cases of choroidal hemangioma. All cases underwent OCT angiography with swept source OCTA (Topcon DRI OCT Triton plus) 6 mm * 6 mm protocol. OCTA at the level of large choroidal vessels demonstrated peculiar vascular pattern in all four eyes. Patterns observed were bag of worms, spaghetti like and vessels with terminal bulbs. In eye with diffuse choroidal hemangioma comparison of choroidal vascular pattern with the contralateral eyes showed significant difference in the vascular architecture. OCTA is an excellent non invasive tool in assessing the choroidal vascular pattern in eyes with choroidal hemangioma.

Photodynamic therapy with double duration for circumscribed choroidal haemangioma: functional and anatomical results based on initial parameters.

IMPORTANCE: Pre-treatment symptoms longer than 12 months and foveal cystoid changes are indicators for poor anatomical and functional outcome after photodynamic therapy (PDT). BACKGROUND: To evaluate the prognostic factors on the effectiveness of PDT with double duration for treatment of exudative circumscribed choroidal haemangioma. DESIGN: Retrospective study. PARTICIPANTS: Twenty-seven patients with symptomatic exudative circumscribed choroidal haemangioma treated with PDT. METHODS: Clinical charts of patients with exudative circumscribed choroidal haemangioma treated with PDT were analysed with regard to visual acuity, duration of symptoms, subfoveal fluid, foveal cystoid changes and foveal thickness in optical coherence tomography. MAIN OUTCOME MEASURES: Change of best-corrected visual acuity, sub- and intrafoveal fluid and foveal thickness measured with optical coherence tomography from baseline to last follow-up. RESULTS: Mean visual acuity improved by 0.05 from 0.42 logMAR (standard deviation [SD] 0.34) to 0.37 logMAR (SD 0.47). In 70% of the patients, PDT stopped exudation and revealed a dry fovea. The recurrence or persistence of sub- or intrafoveal fluid was significantly associated with pre-therapeutic symptoms existing for more than 12 months (P = 0.046). Mean foveal thickness in optical coherence tomography decreased from 324 µm (SD 223 µm) to 209 µm (SD 109 µm). CONCLUSIONS AND RELEVANCE: PDT proved to be a safe and effective treatment procedure for exudative circumscribed choroidal haemangioma in our series, with few side effects. We observed a more successful treatment with regard to anatomical and functional results in cases with pre-existing symptoms less than 12 months and in cases without pre-therapeutic foveal cystoid changes.

Blood flow pattern in a choroidal hemangioma imaged on swept-source-optical coherence tomography angiography.

This report demonstrates the blood flow pattern in a case of choroidal hemangioma (CH) using swept-source-optical coherence tomography angiography (SS-OCTA). Fluorescein angiography, SS-OCT, and SS-OCTA images of a patient with CH were obtained using a standard protocol. The internal vascular pattern of the tumor was identified on both OCT and OCTA. Dark areas were identified in the CH. These were interspersed between areas of visible blood flow, as imaged on SS-OCTA. Peripheral vascular arcades were also identified within the tumor. SS-OCTA should be evaluated as an imaging tool to study the blood flow within choroidal tumors.

Multimodal imaging of suspicious choroidal neoplasms in a primary eye-care clinic.

Small suspicious choroidal neoplasms commonly present a diagnostic challenge in primary eye-care clinics, where they are most likely to present. Differentiating benign from malignant choroidal neoplasms is essential for facilitating early diagnosis and treatment, potentially decreasing the risk of metastasis and vision loss in some cases. The purpose of this review is to describe the clinical, spectral-domain optical coherence tomography and fundus autofluorescence features of suspicious choroidal neoplasms in a case series of patients presenting to a primary eye-care clinic at the Veterans Health Administration, Cleveland, Ohio, USA.

BRACHYTHERAPY ALONE OR WITH NEOADJUVANT PHOTODYNAMIC THERAPY FOR AMELANOTIC CHOROIDAL MELANOMA: Functional Outcomes and Local Tumor Control.

PURPOSE: To compare visual outcomes and local tumor control between two groups of patients with amelanotic choroidal melanoma treated with brachytherapy alone, or neoadjuvant photodynamic therapy before brachytherapy. METHODS: Patients diagnosed with amelanotic choroidal melanoma were recruited for the study and divided into two groups: brachytherapy alone (Group A) and photodynamic therapy preceding brachytherapy (Group B). Patients of both groups were selected to be comparable. RESULTS: Twenty-six patients with amelanotic choroidal melanoma were enrolled in the study. Within Group B, 1 month after photodynamic therapy, ultrasonography showed reduction of tumor height in 11 patients (73.4%). The mean doses of irradiation to macula and optic nerve, at baseline were 74.37 and 52.07 Gy, whereas after photodynamic therapy there was a decrease of 17.26% (P = 0.008) and 21.22% (P = 0.025), respectively. In terms of visual acuity, a mean decrease of 14 ETDRS letters and 5 ETDRS letters was observed at 24 months follow-up, in Groups A and B, respectively (P = 0.001). CONCLUSION: Photodynamic therapy as neoadjuvant therapy before brachytherapy reduces tumor thickness in 73.4% of cases. As a result, a decrease of radiation toxic effects on visual function could be obtained, without compromising disease control.