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Purpose: To report on the venous abnormalities of a patient with Sturge-Weber syndrome., Method: Case report., Patient: A 29-year-old woman with a history of Sturge-Weber syndrome since infancy was referred for evaluation of possible diffuse choroidal hemangioma. Multimodal imaging, including ultra-widefield fluorescein, indocyanine green, and optical coherence tomography angiography, was performed., Results: Dilated fundus examination was remarkable for increased cupping of the optic disk in the right eye, venous tortuosity, and marked dilation of the choroidal vessels. Ultra-widefield fluorescein angiography confirmed marked venous tortuosity and dilation, as well as anastomoses of the retinal veins ipsilateral to the port wine stain. Indocyanine green angiography revealed marked engorgement of the vortex veins and choroidal vasculature. Optical coherence tomography angiography revealed dilated vascular channels in the deep capillary plexus that were directly anastomosing to the superficial capillary plexus, but not the intermediate capillary plexus. Engorgement of the ampullae of the deep capillary plexus vortex system was also observed. The normal contralateral eye was used as comparison for all imaging studies., Conclusion: These findings support the notion of generalized venous hypertension state in adult eyes with Sturge-Weber syndrome and corroborate previous evidence that the deep capillary plexus acts as a venous outflow system., Competing Interests: B. Fortune: Heidelberg Engineering, GmbH (equipment support). The remaining authors have no conflicts of interest.

Purpose: To review long-term outcomes of circumscribed choroidal hemangioma (CCH)., Methods: Hospital charts of all CCH cases diagnosed from 2008 to 2019 were retrospectively reviewed., Results: All 172 patients were managed with either observation, transpupillary thermotherapy, argon laser photocoagulation, photodynamic therapy, plaque brachytherapy or stereotactic radiosurgery. The most common 3 modes of management were clinical observation (30.2%), transpupillary thermotherapy (52.9%) and argon laser photocoagulation (8.7%). Median follow-up time was 10 months (range: 3, 160). Anatomical outcomes were stable in 87.1% of observation group and improved in 60.5% of thermotherapy group. Quantified optical coherence tomography angiography findings showed statistical differences in vascular and perfusion densities in fellow eyes of hemangioma patients., Conclusion: Circumscribed choroidal hemangioma can be treated in various ways. Transpupillary thermotherapy is an anatomically effective treatment in selected cases. The diagnosis of CCH may have vascular implications in fellow eyes of the patients., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Background: Langerhan cell histiocytosis (LCH), although not a common cause, should be kept in the differential diagnosis for a patient that presents with a choroidal mass., Case Presentation: A 28-year-old female presented with a 4-day history of vision loss and associated pain in her right eye., Examination and Investigations: A dilated fundus examination revealed a deep subretinal, orange, mottled lesion with associated serous retinal detachment. Ultrasonography demonstrated a solid mass at the posterior pole. Fluorescein angiography revealed corresponding, small, punctate, hyperfluorescent areas with leakage and pooling in the late phase outlining the subretinal fluid. Optical coherence tomography confirmed the choroidal elevation and subretinal fluid. In addition to starting oral steroids for addressing the patient's acute symptoms, a metastatic workup was ordered due to the lesion's appearance. Diagnosis: Computed tomography (CT) of the chest showed nodular lesions and subsequent lung biopsy was S-100 and CD1a positive, diagnostic of Langerhan's cell histiocytosis (LCH)., Treatment and Outcome: The patient was treated with six cycles of vinblastine and prednisolone therapy followed with a subsequent taper of steroids. Complete resolution of signs and symptoms was noted., Discussion: A review of all reported cases of ophthalmic LCH with or without choroidal involvement was conducted. Diagnostic and therapeutic approaches described in these reportshave been summarized in the current manuscript. This case highlights the importance of pursuing a systemic workup in patients with uveal mass lesions. LCH should be considered in the differential of every choroidal mass.

Purpose: To report a very rare and atypical case of an elderly Caucasian female patient who developed perilesional multiple polypoidal choroidal vasculopathy (PCV) as a probable complication of choroidal osteoma (CO), associated to preretinal neovascular membrane overlying the lesion., Methods: Observational case report., Case Observation: A 60-year-old Caucasian woman presented with blurred vision in her right eye (RE). Fundus examination revealed a round white-yellowish calcified deep lesion in the juxta-papillary superior area, measuring 4 disc-diameters, with well-defined scalloped margins and an irregular surface. B-scan ultrasonography and orbital tomography confirmed the diagnosis of choroidal osteoma (CO). Further investigation with multimodal imaging including infracyanine green angiography, fluorescein angiography, swept source optical coherence tomography and angiography highlighted the presence of multiple aneurysmal choroidal dilations around the CO, corresponding to PCV. We also noted the presence of a preretinal neovascular membrane overlying the CO. The patient was monitored with regular follow-up since no signs of activity were detected on multimodal imaging., Conclusion: Our case report represents an exceptional and atypical association between pre-retinal neovascularization, PCV and choroidal osteoma. While the mechanisms underlying the development of PCV and pre-retinal neovascularization in the setting of CO are not well understood, it is imperative for ophthalmologists to recognize this association as a potential cause of sudden vision loss in patients with CO, and to consider appropriate diagnostic and management strategies., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

This article describes a clinical case of a female patient with choroidal nevus, who was previously diagnosed in another clinic with "subretinal neovascular membrane as a result of central serous chorioretinopathy" and subsequently underwent multiple intravitreal anti-VEGF injections. Based on the analysis of OCT angiography images, the macular changes in this case were interpreted as a polypoidal form of neovascularization in a patient with subfoveolar choroidal nevus.

Purpose: To evaluate the use of swept-source optical coherence tomography angiography to detect distinct vascular features in small choroidal melanomas and choroidal nevi., Methods: Patients with a choroidal nevus or a treatment-naïve choroidal melanoma were imaged with color fundus photography, ultrasound, and swept-source optical coherence tomography angiography (12 × 12 mm). High-risk features including overlying fluid, orange pigment, shaggy photoreceptors, acoustic hollowness, depth >2 mm, and basal diameter >5 mm were assessed. Optical coherence tomography angiography vascular markers included: choroidal vessel visualization, choroidal vessel depth, and choriocapillaris flow signal, assessed qualitatively by comparison with surrounding, unaffected choriocapillaris., Results: Twenty-nine lesions were included in this study, seven flat choroidal nevi, 17 elevated choroidal nevi, and 5 choroidal melanomas. Distinct vascular patterns were noted between flat nevi, elevated nevi, and small choroidal melanomas. Choroidal melanomas displayed two types of vasculature: "nevus-like" vasculature with straight parallel vessels and complex vasculature with vascular loops and crosslinking. Visualized choroidal vessels were significantly deeper in melanomas (110 µm) than elevated (84 µm) or flat nevi (70 µm). In a size-matched subanalysis of 5 elevated choroidal nevi and 5 choroidal melanomas, choroidal melanomas had increased mean choroidal vessel depth (P = 0.015), deepest choroidal vessel visualized (P = 0.034), and presence of a deep choroidal vessel >155 µm (P = 0.048)., Conclusion: Swept-source optical coherence tomography angiography may detect distinct vascular features in choroidal nevi and small choroidal melanomas., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.)

Purpose: Radiation retinopathy is a common side effect of ocular radiotherapy with no long-term effective therapy. Optical coherence tomography angiography (OCTA) and wide-field fluorescein angiography (FA) are widely used for the study of radiation maculopathy and peripheral nonperfusion, respectively. We investigated the role of extended field imaging (EFI-OCTA) for the study of retinal and choroidal alterations after radiotherapy for choroidal melanoma., Methods: Cross-sectional observational study of 20 eyes of 20 patients diagnosed with radiation retinopathy. All patients underwent a complete imaging evaluation including FA and indocyanine green angiography (ICGA) with 55° and 102° lens (Spectralis Heidelberg Engineering, Heidelberg, Germany). Optical coherence tomography angiography imaging was performed with the Zeiss PlexElite 9000 Swept Source OCTA (Carl Zeiss Meditec, Dublin, CA) using a 12 × 12-mm volume scan pattern centered on the fovea and a +20.00-diopter lens specifically designed to obtain EFI examination. The imaging methods were then compared in terms of visible field of view, extension of nonperfused areas, and vessel density., Results: The mean extension ratio of EFI-OCTA compared to OCTA without EFI, FA/ICGA 55° and FA/ICGA 102° was, respectively, 1.98 ± 0.02, 1.21 ± 0.01 and 0.36 ± 0.003. The mean extension of retinal and choroidal nonperfused areas evaluated by EFI-OCTA (63.03 ± 48.21 and 38.63 ± 30.83 mm2) were significantly higher than with OCTA without EFI (40.40 ± 34.87 and 24.26 ± 21.82 mm2, P < 0.001) but lower than with FA/ICGA 102° (140.7 ± 69.23 and 108.3 ± 69.51 mm2, P < 0.001). No significant differences were found between mean extension of retinal and choroidal ischemic areas measured with EFI-OCTA and FA/ICGA 55° (69.64 ± 51.92 and 47.23 ± 33.59 mm2). The mean vessel density of EFI-OCTA (retina and choroid segmentation) was significantly different compared to OCTA without EFI (P < 0.05). Retinal vessel density was negatively correlated to retinal extension of nonperfused areas (r = -0.5, P = 0.02), and choroidal vessel density was negatively correlated to choroidal nonperfused areas (r = -0.6, P = 0.003) measured with EFI-OCTA., Conclusion: In our series, EFI-OCTA captured larger areas than OCTA without EFI and FA/ICGA with 55° lens. EFI-OCTA images showed a good definition of retinal and choroidal vascular changes after radiotherapy, suggesting a possible role of this safe and noninvasive imaging technique in the follow-up of patients with radiation retinopathy.

Background: Hematologic malignancies occasionally cause serous retinal detachment (SRD); however, its pathogenesis remains unclear. Here we present the imaging characteristics of metastatic choroidal lymphoma masquerading as Vogt-Koyanagi-Harada (VKH) disease., Case Presentation: A 45-year-old Japanese woman was referred to our clinic because of bilateral SRD with blurred vision. Fluorescein angiography revealed multiple pinpoint leakage followed by pooling OU. Enhanced depth imaging optical coherence tomography showed marked choroidal thickening OU. Laser speckle flowgraphy detected choroidal circulation impairment OU. Although these results totally agreed with the inflammatory manifestations of acute VKH disease, indocyanine green angiography demonstrated various sizes of sharply marginated hypofluorescent lesions that seemed atypical for the finding of VKH disease, i.e., vaguely marginated hypofluorescent small dots. Cerebrospinal fluid pleocytosis was not detected. Blood tests revealed leukocytosis together with elevation of lactate dehydrogenase and soluble interleukin-2 receptor levels. Corticosteroid pulse therapy did not improve any ocular findings. Bone marrow biopsy was then performed, leading to a definite diagnosis of diffuse large B-cell lymphoma. After starting systemic chemotherapy, both SRD and choroidal thickening resolved rapidly with visual recovery. However, choroidal hypoperfusion persisted, which contrasted distinctly with the inflammatory pattern of VKH disease, i.e., the restoration of choroidal blood flow in parallel with normalization of choroidal thickness., Conclusions: Our detailed multimodal observations highlighted the differential imaging features of choroidal lymphoma despite close resemblance to VKH disease especially at the initial stage. Impaired circulation in the thickened choroid marked the pseudo-inflammatory pathogenesis of SRD due to choroidal involvement with neoplastic, but not inflammatory cells.

Purpose: To describe changes in blue-light fundus autofluorescence (FAF) and corresponding alterations in optical coherence tomography (OCT) within the irradiation field after ruthenium-106 brachytherapy (RBT) for choroidal melanoma., Methods: Consecutive patients with choroidal melanoma were included in a retrospective case series. Patients were treated with RBT at a single institution. As part of their routine examination patients underwent multimodal imaging including ultrasonography, fundus photography, OCT, and FAF imaging (excitation = 488 nm). FAF images were analysed for changes within the irradiation field., Results: 31 patients (mean age 65.7 years) were treated with RBT for unilateral choroidal melanoma. Mean tumour height before therapy was 2.7 mm (SD 1.0). Mean follow-up time was 23.3 months (SD 13.3). Main FAF characteristics attributable to RBT emerged as increased FAF with speckled decreased FAF (FAF mottling) within the irradiation field and a rim of increased FAF at its border. OCT scans demonstrated loss of the ellipsoid zone and the external limiting membrane, thinning of the neurosensory retina, and alterations of the retinal pigment epithelium like clumping, migration, and atrophy., Conclusions: FAF changes in the irradiation field after RBT of choroidal melanomas follow a characteristic pattern that correlates with distinct OCT alterations. FAF and OCT imaging give additional information to monitor effects of RBT and, therefore, complement multimodal imaging techniques after plaque therapy., (© 2020 S. Karger AG, Basel.)

Purpose: To study the choroidal vascular pattern in patients with Sturge-Weber syndrome (SWS) using swept-source OCT (SS-OCT)., Design: Prospective comparative observational study., Participants: All patients with SWS with no history of prior treatment for posterior segment pathology were included., Methods: Both eyes of all patients were studied using fundus imaging, SS-OCT, fundus fluorescein angiography (FFA), and indocyanine green angiography (ICG) by 2 independent observers., Main Outcome Measures: The FFA and ICG were screened for any vascular abnormalities. The SS-OCT was evaluated for choroidal changes., Results: A total of 34 eyes of 17 patients with diagnosed SWS in the age group 9 to 26 years were studied. The FFA and ICG in 7 and 11 patients, respectively, showed some vascular abnormalities. SS-OCT was performed in all patients. The diffuse choroidal hemangioma (DCH) was characterized by loss of the choroidal vascular pattern, increase in the choroidal thickness and loss of visualization of the sclerochoroidal interface. Based on the FFA, ICG, and SS-OCT imaging, there were 3 patients with no DCH, 5 with bilateral DCH, and the remaining 9 patients had unilateral DCH. The detection rate was 50% clinically: 52.94% with FFA, 82.35% with ICG, and 86.36% with SS-OCT. There was substantial agreement between the 2 observers for all 3 investigations., Conclusion: SS-OCT is a reliable noninvasive imaging modality for early diagnosis and follow-up of DCH over time., (Copyright © 2019 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To study optical coherence tomography (OCT) and optical coherence tomography angiography (OCT-A) features of circumscribed choroidal hemangioma (CCH) following treatment with photodynamic therapy (PDT) and transpupillary thermotherapy (TTT)., Methods: A retrospective chart review of consecutive patients treated for CCH over 2 years (May 2016-April 2018). The investigations, in addition to comprehensive eye examination, included color fundus photography, B-scan ultrasonography, OCT, and OCT-A., Results: The study included 16 eyes of 16 patients (9 males and 7 females). The mean age at presentation was 43.5 ± 9 years (range 33-62 years). Macula (n = 6) and superior arcade (n = 5) were the common tumor locations. Twelve eyes received multiple treatment sessions: TTT (seven eyes; mean 2.4 sessions) and PDT (five eyes; mean 2 sessions). Four eyes were observed because vision was not threatened. Pretreatment OCT features were Bruch's membrane atrophy (15 eyes), retinal pigment epithelial atrophy (13 eyes), outer retinal abnormalities (12 eyes), and macular subretinal fluid (12 eyes). Pretreatment OCT-A features were complete loss of choriocapillaris (16 eyes), irregularly arranged fine arborizing vessels (11 eyes), and more than 50% signal void hyporeflective areas (12 eyes). Posttreatment OCT-A showed persistence of choriocapillaris loss, flat scar with fibrosis and thinning of choroid in all eyes treated with TTT, and persistence of deeper choroidal vessels and no loss of choriocapillaris in eyes treated with PDT., Conclusion: OCT and OCT-A help understand the structural outcome following PDT and TTT in circumscribed choroidal hemangioma., Competing Interests: None

Competing Interests: None

Purpose: To describe co-occurrence of choroidal osteoma in a patient with pattern dystrophy of retinal pigment epithelium., Methods: Clinical case report RESULTS: A young female presented with decreased vision in the right eye due to decalcification of choroidal osteoma. Multimodal imaging including fundus autofluorescence, fluorescein angiography and optical coherence tomography showed features of pattern dystrophy of retinal pigment epithelium that simulates the Stargardt disease., Conclusions: This co-occurrence of choroidal osteoma and pattern dystrophy is likely to be incidental. Multimodal imaging may help in differentiating pattern dystrophy of retinal pigment epithelium that simulates Stargardt disease from Stargardt disease.

Purpose: To evaluate the efficacy of photodynamic therapy using a double dose of verteporfin for patients with circumscribed choroidal hemangioma., Methods: This retrospective comparative case series evaluated data from 10 patients who were treated using double dose photodynamic therapy (12 mg/m) and seven patients who were treated using the standard dose (6 mg/m). A laser was applied with a radiant exposure of 50 J/cm. The ophthalmologic examinations were performed at baseline and 1 year after the treatment and included best-corrected visual acuity, slit-lamp biomicroscopy, fundus examination, spectral domain optical coherence tomography, and B-scan ultrasonography., Results: The mean age in the double dose group was 51.60 years, compared with 50.57 years in the standard-dose group. The only significant difference between the two groups' baseline characteristics was observed in their initial tumor heights. Foveal center thickness, subretinal fluid, and subfoveal choroidal thickness decreased significantly at 1 year after treatment in both groups. Tumor height and the greatest linear dimension of the tumor's base only decreased significantly in the double dose group (P = 0.031). Both groups did not experience significant visual improvements., Conclusion: Double dose photodynamic therapy was effective and safe for treating circumscribed choroidal hemangioma and provided better tumor regression with similar resorption of subretinal fluid, compared with standard-dose photodynamic therapy.

Purpose: To investigate spectral domain-optical coherence tomography (SD-OCT) features of untreated circumscribed choroidal hemangioma (CCH), and the optical density (OD) of the associated subretinal fluid (SRF)., Procedures: This international retrospective multicenter study conducted at 5 tertiary retina referral centers included 34 patients with symptomatic CCH imaged on SD-OCT. CCH surface, size, and overlying retinal changes and OD ratio (ODR) were evaluated., Results: The mean ODR of the SRF was 0.808 ± 0.287. Tumor base measured on ultrasound correlated significantly with the ODR (p = 0.012). ODR values in cases of CCH were similar to those of choroidal metastasis, but significantly lower than those of choroidal melanoma (p = 0.006). Thirty-two out of 34 cases (94%) presented with a regular smooth choroidal surface. OCT demonstrated SRF overlying the CCH in 29 eyes (85%). The presence of SRF correlated significantly with a larger basal tumor diameter (p = 0.035). OCT displayed sub- and intraretinal hyperreflective foci, correlating with both larger maximal retinal thickness overlying the tumor (p = 0.031) and the amount of SRF (p = 0.016) in 29 eyes (85%)., Conclusion: CCH displays typical findings on SD-OCT. ODR is a new important tool in the differential diagnosis of choroidal tumors and should be considered for enhancing the accuracy of diagnosing CCH. This benefit is especially relevant for cases in which the presentation of CCH is atypical., (© 2018 S. Karger AG, Basel.)

Purpose: To describe the vascular changes of circumscribed choroidal hemangioma (CCH) after ruthenium-106 brachytherapy using optical coherence tomography angiography (angio-OCT)., Methods: Seven eyes of 7 patients diagnosed with symptomatic CCH who underwent ruthenium-106 plaque radiotherapy were included in the study. Patients underwent complete ophthalmic examination, bulbar echography and angio-OCT. The same protocol was applied 1 year after ruthenium-106 brachytherapy., Results: At baseline, the mean best-corrected visual acuity (BCVA) in affected eyes was 0.67 ± 0.14 logMAR, and the mean tumor thickness was 4.46 ± 0.91 mm measured by standardized A-scan echography. After ruthenium-106 plaque treatment, the mean BCVA increased to 0.17 ± 0.10 logMAR (p = 0.001) and the mean tumor thickness to 2.29 ± 0.71 mm (p = 0.003). Moreover, there was a significant reduction of the tumor basal diameter (p= 0.009). Intra-tumor angio-OCT measurements revealed a statistically significant reduction of the vessel and flow areas (p = 0.006 and p = 0.002, respectively) 1 year after brachytherapy., Conclusions: Angio-OCT is a non-invasive reliable method with which to evaluate the vascular features of CCH and consequently to monitor changes after conservative treatment.

Purpose: To describe the imaging features of choroidal neovascularization (CNV) associated with choroidal nevus using optical coherence tomography angiography (OCT-A) imaging., Methods: Retrospective observational case series. Patients with CNV secondary to choroidal nevus underwent full imaging examination including fundus photography, fluorescein angiography, indocyanine green angiography, spectral domain OCT, and OCT-A. The OCT-A features were analyzed and correlated with conventional angiography findings and spectral domain OCT., Results: There were 11 eyes from 11 patients (6 men and 5 women, mean age of 65 ± 20.4 years) included in the analysis. Fluorescein angiography and indocyanine green angiography disclosed CNV in 90% and 83%, respectively. Optical coherence tomography angiography displayed CNV network in 11 eyes (100%) and the pattern was classified as "sea-fan" in 8 (73%) and "long filamentous linear vessels" in 3 (27%) eyes. Distinct from CNV, intrinsic vasculature within the nevus was observed in six eyes (55%), corresponding to those with chronic retinal pigment epithelium changes., Conclusion: Optical coherence tomography angiography is a useful imaging technique to disclose CNV associated with choroidal nevus. Despite the presence of intraretinal or subretinal fluid and hemorrhage, OCT-A revealed the CNV in all cases, results noninferior to indocyanine green angiography. This imaging modality can be useful for analysis of long-standing nevi with related exudation.

Purpose: The purpose of our study was to describe features of choroidal naevi as seen on swept source optical coherence tomography angiography (OCTA) and also on en face images derived from structural data from OCTA., Methods: A prospective observational cohort study was carried out. Patients attending a specialised choroidal naevomelanocytic with known naevi were imaged with swept source OCTA., Results: Seventy-one eyes of 70 patients were imaged. Forty-three patients and 44 eyes were included. Mean age was 57.7 years (SD 14.9), range 29-81 years. Male to female ratio was 20:23. On OCTA after manual segmentation, naevi could be seen in 47.6% of cases, whereas in the en face images, naevi could be clearly visualised in 79.5% of cases. In OCTA, the superficial and deep capillary plexuses appeared undisturbed as did the outer retinal layer appeared in all cases of flat naevi. In choroidal naevi with mild elevation, the outer retinal layer appeared more susceptible to projection artefacts from overlying retinal vasculature. The choriocapillaris layer showed a fading of the normal homogenous vascular mosaic corresponding to the area of the naevus. In the en face images, even the faintest and thinnest naevi could be visualised in striking detail, and naevi greater than 120 μm thickness appeared darker (p = 0.0034)., Conclusions: OCTA presents characteristic changes in the choriocapillaris layers in cases of choroidal naevi. The association of naevus substance appearing darker with increasing thickness may offer a novel prognostic clue. En face structural OCT may allow accurate, detailed measurement of lateral dimensions which could be of value in the monitoring of suspicious naevi.

Purpose: To evaluate the morphological findings of outer retinal tubulations (ORTs) shown on multimodal imaging modalities in patients with choroidal osteoma., Methods: Nineteen eyes of 17 patients with choroidal osteoma underwent full clinical and imaging assessments. Color fundus photography, spectral-domain optical coherence tomography (OCT), and en face OCT were used to identify and detect the characteristics of ORT structures, including the shape, configuration, location, and distribution in the fundus. Optical coherence tomography angiography was implemented as an assist to differentiate tumor's feeder vessels from choroidal neovascularization. The correlations between age, gender, tumor features, best-corrected visual acuity at baseline, OCT characteristics, and the presence of ORTs were analyzed., Results: Outer retinal tubulations were identified in five eyes (26.3%). All were located at the decalcified region of the tumor where the choroidal vessels, retinal pigment epithelium, and overlying outer retinal structures were considerably disrupted to varying degrees. With spectral-domain OCT, the ORTs appeared as one or multiple, round or ovoid, hyporeflective lumens with hyperreflective borders confined to the outer nuclear layer, sometimes with hyperreflective luminal content (four eyes, 80%). In one eye, ORTs were found at the focal choroidal excavation. On en face OCT, these tubulations exhibited different shapes, including a dendritic pattern in two eyes, a tube-like pattern in one eye, a circular pattern in one eye, and a hairpin pattern in one eye. Simultaneous OCT angiography imaging demonstrated that the area of choroidal neovascularization was underneath ORT in one eye and very close to ORT in two eyes. The ORTs of three eyes were above or adjacent to tumor's rich feeder vessels. Statistically, age (P = 0.007), greatest tumor linear dimension (P = 0.003), total tumor area (P = 0.002), decalcification area (P = 0.000), and the presence of intraretinal fluid (P = 0.01) and retinal pigment epithelium alterations (P = 0.038) within the foveola and central 1-mm region of patients with ORT were significantly different from those of patients without ORTs., Conclusion: The results of this study suggest that age, the greatest tumor linear dimension, total tumor area, decalcification area, and the presence of intraretinal fluid and retinal pigment epithelium alterations within the foveola and central 1-mm region might be risk factors for ORT formation. Spectral-domain OCT combined with en face OCT provides comprehensive imaging information for ORTs in choroidal osteoma.

We describe features of a circumscribed choroidal hemangioma (CCH) treated with photodynamic therapy (PDT) by means of optical coherence tomography angiography (OCT-A). A 40-year-old man had a complaint of decreasing visual acuity in his left eye for 10 days. A diagnosis of CCH was confirmed by fundus examination, fluorescein angiography, and indocyanine green angiography. An OCT-A study of choroidal lesion before and after PDT was carried out. Segmented en face OCT-A of CCH showed multiple irregular connected vascular channels with hyperflow and a capillary-like lesion. Absent and/or reduced intrachannel flow in most of the intratumoral vessels was shown by OCT-A soon after treatment (2 days), with normalization of vascular flow 7 days and 1 month after PDT. OCT-A was able to noninvasively visualize intrinsic vasculature of CCH showing vascular remodeling after PDT.

Background and Objective: To illustrate the features of a circumscribed choroidal hemangioma (CCH) using Annidis RHA multi-spectral imaging (MSI) technology (Annidis Corporation, Ottawa, Canada). This novel imaging technique uses non-overlapping, narrow-band LED light sources ranging from 520 nm to 940 nm to create a monochromatic series of en face fundus images and oxy/deoxyhemoglobin contrasted maps of the retina and choroid., Patients and Methods: Annidis RHA MSI images were taken of a 67-year-old Chinese male with CCH. These en face fundus images are presented alongside the traditional fundus photograph, optical coherence tomography, and fluorescein and indocyanine green angiograms., Results: Multi-spectral en face images of the outer retina and choroid demonstrated a clearly delineated lesion with a disorganized choroidal vascular network and increased reflectance/ oxyhemoglobin levels within., Conclusion: MSI technology is a noninvasive imaging technique that can depict the vascular features and choroidal oxygenation of CCH lesions and has a potential role in monitoring its growth complications. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:572-575.]., (Copyright 2017, SLACK Incorporated.)

Purpose: To describe the features of choroidal tumors on infrared (IR) imaging and to determine the diagnostic sensitivity and specificity of intratumoral choroidal vascular loops on IR imaging for circumscribed choroidal hemangioma (CCH)., Methods: Infrared and indocyanine green images of CCH, choroidal metastases, and choroidal melanomas were reviewed. The main outcome measure was the presence of intratumoral choroidal vascular loops and tufts on IR images. The secondary outcome measure was the presence of peritumoral vascular expansion on indocyanine green images., Results: Intratumoral CCH vessels appear as dark beaded spaces on IR imaging; 95.5% of CCH had vascular loops compared with the controls of 65% in choroidal melanomas and 64% in choroidal metastases. The sensitivity of intratumoral vessels on IR for CCH was 95.4%. Subanalysis of six patients with CCH showed the presence of peritumoral vascular expansion on indocyanine green images., Conclusion: Infrared imaging delineates intratumoral vessels in choroidal tumors appearing as dark beaded loops and tufts. Vascular loops on IR imaging showed a high diagnostic sensitivity for CCH. The absence of these loops on IR can help rule out the diagnosis of CCH. Peritumoral vascular expansion on indocyanine green is an additional diagnostic tool that is helpful in the diagnosis of CCH.

AimTo describe the vascular features of choroidal tumors using enhanced depth imaging (EDI), optical coherence tomography (OCT), and OCT-angiography.MethodsIn this prospective study, we evaluated 116 Caucasian patients with choroidal tumors (60 eyes with choroidal nevi, 40 with choroidal melanoma, 6 with choroidal hemangioma, 2 with optic disc melanocytoma, 6 with choroidal osteoma, and 2 with retinal metastases). Patients underwent a complete ophthalmic examination including bulbar echography, EDI-OCT, OCT-angiography, and multicolor imaging. Sixteen patients also underwent fluorescein and indocyanine angiography.ResultsThe left eye was more involved than the right eye. The mean tumor thickness was 1.23±0.17 mm in the 60 eyes with choroidal nevi; 2.75±0.83 mm in the 40 eyes with choroidal melanoma; 3.28±0.78 mm in the 6 eyes with retinal angioma; 2.02±0.001 mm in the 2 eyes with optic disc melanocytoma; 2.40±0.31 mm in the 6 eyes with choroidal osteoma; and last, 3.49±0.001 mm in the 2 eyes with retinal metastases. OCT-angiography showed: (i) a lack of blood flow in the outer retinal layer (ORL) and a normal choroid capillary layer in choroidal nevi and optic disc melanocytomas; (ii) a lack of blood flow in the ORL of choroidal metastases; and (iii) a dense irregular vascular network in the ORL and choroid capillary layers of choroidal melanomas, choroidal hemangiomas, and choroidal osteomas.ConclusionsOCT-angiography is a noninvasive reliable method with which to evaluate the vascularization of small choroidal tumors and may improve the diagnosis of these tumors.

Background and Objective: To compare the maximum basal diameter of choroidal tumors measured by clinical estimation and different imaging methods in intraocular tumors for therapeutic planning., Patients and Methods: Retrospective, observational study reviewing the measurements of the longest basal diameter of choroidal tumors using the clinical estimation based on the indirect ophthalmoscopy, ophthalmic ultrasonography, 50° fundus imaging, ultra-wide-field imaging color composite, red laser, and green laser., Results: The study included measurements of 74 tumors in 74 eyes. Correlation analysis between imaging measurements and clinical estimates led to following results: ultra-wide-field imaging measurements were 2.7% to 7.1% smaller than estimated clinical measurements by indirect ophthalmoscopy; followed by ultrasound, with 10.9% smaller measurements; and 50° fundus imaging, measuring 25.8% smaller than clinical estimates. When evaluating the precision of tumor measurements, ultrasound resulted in the highest precision, with a standard error of 1.6 mm compared to clinical estimates, followed by other imaging techniques with standard errors ranging from 2.4 mm to 3.0 mm. Determination of tumor margins with the ultrasound technique was better defined when subretinal fluid was present, or when the height of the tumor was greater than 3 mm., Conclusion: The authors' results suggest that measurements of the longest basal diameter of choroidal tumors can be accurately performed with ultrasound and ultra-wide-field color composite or red laser images. In the presence of subretinal fluid or tumor height above 3 mm, ultrasound can better delineate tumor margins. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:459-464.]., (Copyright 2017, SLACK Incorporated.)

Purpose: To explore the utility of fundus autofluorescence (FAF) and red reflectance (RR) imaging using ultra-wide-field scanning laser ophthalmoscope in choroidal nevi., Methods: Retrospective observational case study reviewing clinical data, color, FAF, and RR images of patients with choroidal nevi and comparing the findings. The ultra-wide-field scanning laser ophthalmoscope uses green laser 532 nm and red laser 633 nm that enabled FAF and RR imaging, respectively in separate channels. Superimposition of both images yielded a composite color image., Results: The study included 46 eyes of 45 patients. Nevi were unilateral in 44 patients (98%). Forty-one nevi (89.1%) were located temporally between the macula and the equator. All nevi (100%) were deeply pigmented. The most frequent surface changes were lipofuscin pigments, zones of retinal pigment epithelium atrophy, and retinal pigment epithelium pigment clumps in 31 (67.3%), 18 (39.1%), and 8 eyes (17.3%), respectively. Color photographs were superior to FAF in detecting nevus boundaries and surface changes. Red reflectance correlated strongly with color images, although the nevus boundaries and surface changes were better delineated in RR mode. Red reflectance was superior to FAF in delineating the boundaries and surface changes of the nevus; clear visibility (3+) for RR versus no or poor visibility (0/1+) for FAF. Nevertheless, the areas of retinal pigment epithelium atrophy were better delineated in FAF mode; clear visibility (3+) for FAF versus poor visibility (1+) for FAF., Conclusion: Red reflectance imaging is more sensitive than conventional photography for follow-up of choroidal nevi. Fundus autofluorescence should be considered only as a complementary tool to RR imaging.

Importance: Accurate diagnosis of choroidal melanoma is challenging and has important implications for both physicians and patients. We assessed the utility of quantification of fundus autofluorescence in the evaluation and follow-up of choroidal nevomelanocytic tumors., Objective: To assess the utility of autofluorescence quantification in distinguishing clinically diagnosed choroidal nevi, melanoma, and indeterminate nevomelanocytic lesions., Design, Setting, and Participants: A retrospective observational study from 2006 to 2012 of patients with choroidal nevomelanocytic lesions who had digital autofluorescence and color fundus imaging performed at the University of Michigan Kellogg Eye Center., Intervention: ImageJ software was used to output autofluorescence gray-scale values for each pixel of a 500 × 50-pixel region within each lesion and a corresponding adjacent control region., Main Outcome and Measure: A single value was generated, termed the Index of Retinal Autofluorescence (IRA), to represent the total difference in gray-scale values between the 2 regions in each affected eye., Results: Thirteen of the 14 clinically diagnosed nevi exhibited an IRA less than 150 gray-scale intensity squared (gsi2). Eight of 9 clinically diagnosed melanomas exhibited an IRA more than 150 gsi2. An IRA of 150 gsi2 distinguished nevi from melanomas with a sensitivity of 0.89 and specificity of 0.93. Fifteen of 19 patients with indeterminate nevomelanocytic lesions underwent clinical assessment and initial imaging with clinical follow-up at a median of 10 months. All 3 patients with an IRA less than 150 gsi2 showed no evidence of clinical progression and 6 of 12 lesions with an IRA more than 150 gsi2 showed clinical progression to melanoma. An IRA of 150 gsi2 identifies indeterminate lesions that progressed to melanoma with a sensitivity of 1.00 and specificity of 0.33., Conclusions and Relevance: Quantification of digital autofluorescence images can differentiate between clinically benign and malignant choroidal nevomelanocytic lesions and may be predictive for clinical progression of indeterminate lesions.

Background and Objective: Ultra wide field technology was originally designed for use in children. The purpose of this study was to evaluate ultra wide field fluorescein angiography (UWFFA) in children., Patients and Methods: Retrospective case series of patients younger than 13 years who underwent UWFFA. Angiographic findings and their impact on diagnosis and management were assessed. UWFFA quality was evaluated, including phases of the angiogram available in each study, field of view, and artifacts., Results: Forty images of 16 patients with a mean age of 9.3 years were included. Peripheral angiographic findings such as vasculitis, telangiectasia, and neovascularization were present in 12 of 16 patients (75%). Seven of 16 patients (43.8%) had images during the arterial phase, and 16 of 16 patients (100%) had venous and recirculation phases captured., Conclusion: UWFFA was successfully performed in this group of children. The macula and periphery were adequately imaged, obtaining important information for documentation, diagnosis, and management., (Copyright 2013, SLACK Incorporated.)

Purpose: To describe fundus autofluorescence (FAF) characteristics associated with choroidal osteomas and their secondary complications., Methods: Retrospective descriptive case series of six eyes of five patients with choroidal osteomas. Findings of FAF correlated with visual acuity, clinical features, lesion characteristics, and findings from other imaging modalities., Results: All 6 choroidal osteomas (100%) had totally or partially calcified, orange portions that were isoautofluorescent. Partial decalcification also produced areas of hyperautofluorescence and granular hypoautofluorescence corresponding to overlying retinal pigment epithelium mottling in 3 eyes (50%). Total decalcification with retinal pigment epithelial atrophy produced decreased FAF in 2 eyes (33%). Serous retinal detachment was present in 3 eyes (50%). When the overlying retinal pigment epithelium was viable, hyperautofluorescence as a result of elongation of the outer segments of photoreceptor was observed. In one eye where geographic atrophy of the retinal pigment epithelium was present, FAF was decreased even in the presence of serous retinal detachment. Portions of three partially or totally decalcified osteomas within the treatment field of photodynamic therapy for choroidal neovascularization were hypoautofluorescent. Four eyes (67%) had reduced foveal FAF and visual acuity <20/20, while both eyes with foveal isoautofluorescence had normal (20/20) visual acuity., Conclusion: Calcified portions of choroidal osteomas not previously treated with photodynamic therapy were isoautofluorescent. Decalcification and secondary complications of serous retinal detachment, choroidal neovascularization, and geographic atrophy altered foveal autofluorescence and were associated with reduced visual acuity.

Background and Objective: To correlate fundus autofluorescence (FAF) characteristics of metastatic choroidal tumors with optical coherence tomography/scanning laser ophthalmoscope (OCT/SLO)., Patients and Methods: A retrospective review of 10 choroidal metastases in 9 patients., Results: All tumors were amelanotic, although 8 exhibited surface pigmentation. FAF imaging revealed hyperautofluorescence in areas of focal pigmentation and subretinal fluid with hypoautofluorescent margins (n = 5) corresponding to OCT evidence of retinal pigment epithelial (RPE) thickening and subretinal fluid. Loss of RPE was FAF imaging hypoautofluorescent. FAF images changed with tumor growth. OCT best revealed elevation of the RPE and retina, RPE thickening and folds, and retinal detachment., Conclusion: FAF imaging best defined surface characteristics and tumor margins. FAF imaging hyperautofluorescence correlated to focal hyperpigmentation, subretinal fluid, and advancing tumor edges. OCT better demonstrated intraretinal findings (atrophy, subretinal fluid, and increased and lost RPE). This study shows that FAF imaging and OCT reveal unique tumor characteristics of choroidal metastasis.

Purpose: The purpose of this study was to describe the autofluorescence (AF) features of choroidal hemangioma in 34 consecutive patients., Methods: Standard fundus photography and AF photography (580-nm excitation, 695-nm barrier filter) were performed on all patients. The clinical features were correlated with AF features. The main outcome measures were autofluorescence features of choroidal hemangioma (intrinsic AF) and overlying retinal pigment epithelium (extrinsic AF)., Results: There were 27 eyes with circumscribed choroidal hemangioma (CCH) and 7 eyes with diffuse choroidal hemangioma (DCH). The mean patient age was 53 years for CCH and 15 years for DCH. Intrinsic AF of untreated CCH was generally iso-AF (n = 7, 58%) and for treated CCH was hypo-AF (n = 15, 100%). Extrinsic AF of CCH disclosed hyper-AF (orange pigment and fresh subretinal fluid) and hypo-AF (retinal pigment epithelium hyperplasia, fibrous metaplasia, and atrophy). Intrinsic AF of untreated DCH was generally hypo-AF (n = 2, 67%) and for treated DCH was hypo-AF (n = 3, 75%). Extrinsic AF of DCH was similar to CCH., Conclusion: Choroidal hemangioma shows little intrinsic AF. Overlying orange pigment and fresh subretinal fluid show hyper-AF and retinal pigment epithelium hyperplasia and atrophy show hypo-AF.

Purpose: To demonstrate the ability of ultra wide-field fundus imaging to document the response to treatment of a choroidal metastatic lesion from primary breast carcinoma., Design: Observational case report., Methods: A 45 year old female with a history of metastatic breast cancer and progressive vision loss in the right eye was noted to have an elevated, minimally pigmented choroidal lesion in the superior fundus periphery associated with a serous retinal detachment involving the macula. Ultra wide-field imaging allowed for comparison of the lesion size and secondary serous retinal detachment in response to systemic chemotherapy., Results: Over a 3 month follow up period of observation while the patient received systemic chemotherapy the lesion demonstrated increased growth and increased subretinal fluid., Conclusion: Ultra wide-field fundus photography allowed for accurate documentation of growth of a peripheral choroidal metastatic lesion and associated serous retinal detachment.

Purpose: To correlate fundus autofluorescence (FAF) patterns with fluorescein/indocyanine green angiographic (FA/ICGA) features in choroidal melanocytic lesions., Methods: Retrospective chart review of 30 consecutive patients with choroidal nevi and melanoma who underwent FAF photography and FA/ICGA. The FAF pattern was classified as patchy or diffuse. The FA images were evaluated at the arterial, early venous, late venous, midphase, and late phases. The ICGA images were evaluated at the early and midlate phase. The fluorescence within the tumor was classified as hyperfluorescent, pinpoint hyperfluorescent, isofluorescent, or hypofluorescent with respect to the surrounding retina or choroid. Statistical analysis was performed using two sample t test for continuous data. For categorical or ordinal data, Pearson chi-square or Fisher's exact test was used depending on the sample size being studied., Results: Nineteen of 30 tumors (63.3%) were choroidal melanoma and 11 (36.7%) were choroidal nevus. Thirteen choroidal melanomas had a diffuse FAF pattern. Six choroidal melanomas and 11 choroidal nevi had a patchy FAF pattern. The diffuse FAF pattern was significantly associated with the clinical diagnosis of choroidal melanoma versus choroidal nevus (P = 0.00001), increased tumor thickness (P = 0.00001), and increased tumor base diameter (P = 0.001), partially pigmented or amelanotic versus pigmented lesion color (P = 0.006), early venous hyperfluorescence on FA (P = 0.015), and late hyperfluorescence on FA (P = 0.018)., Conclusion: Diffuse FAF is more often associated with larger choroidal melanomas as well as early venous and late hyperfluorescence on FA angiography.

Purpose: To evaluate the clinical usefulness of fundus fluorescein angiography (FFA) in characterizing diffuse choroidal hemangiomas in Sturge-Weber syndrome., Methods: A retrospective case review of children attending a university hospital clinic with a diagnosis of Sturge-Weber syndrome was undertaken. The FFA findings of seven patients who had undergone angiography were reviewed., Results: FFA confirmed the presence of choroidal hemangioma in each of the seven cases and delineated the extent of the choroidal lesions. No adverse effects of fluorescein injection were encountered., Conclusions: FFA is effective in diagnosing and characterizing diffuse choroidal hemangiomas, and may be performed safely in children.

Background: To describe autofluorescence patterns of choroidal melanocytic lesions using the Heidelberg Retinal Angiograph 2 system (HRA2)., Methods: 20 patients with choroidal melanocytic lesions in the ocular fundus underwent ophthalmologic examination, fundus photography, autofluorescence and optical coherence tomography (OCT). Pathologic examination was performed on one enucleated eye with a large choroidal melanoma., Results: 15 patients had choroidal nevi and 5 had malignant choroidal melanoma (1 small, 1 medium and 3 large tumours). Choroidal nevi did not show any characteristic autofluorescence pattern, although secondary retinal pigment epithelium (RPE) changes, such as drusen and pigment epithelium detachment, appeared faintly hyperautofluorescent in 2 patients. Only the small malignant choroidal melanomas had prominent orange pigmentation, although all melanomas had an intense confluent hyperautofluorescent signal over the lesions. Pathology of one large malignant melanoma revealed lipofuscin underlying RPE., Conclusion: Most nevi did not have characteristic hyperautofluorescent features, but choroidal melanomas seemed to have a pattern of confluent hyperautofluorescence. Therefore, autofluorescence may be a useful non-invasive tool to assess lipofuscin in pigmented choroidal lesions, which may contribute to the diagnosis of malignancy. This hypothesis, however, remains to be confirmed in large prospective studies.