SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FEATURES OF VITREORETINAL LYMPHOMA IN 55 EYES.

PURPOSE: To evaluate spectral domain optical coherence tomography (SD-OCT) features of vitreoretinal lymphoma (VRL). METHODS: Review of records and SD-OCT images of vitreoretinal lymphoma evaluated at Ocular Oncology Service, Wills Eye Hospital between July 1, 2000, and April 1, 2019. RESULTS: There were 55 eyes of 32 patients included. At presentation, SD-OCT features included vitreous opacities (n = 36, 65%), preretinal deposits (n = 7, 13%), intraretinal deposits (n = 8, 15%), subretinal deposits (n = 20, 36%), retinal pigment epithelium abnormalities (n = 35, 64%), and subretinal pigment epithelium deposits (n = 35, 64%). Of 36 eyes with observed tumor progression, comparison (initial visit vs. time of progression) revealed more intraretinal deposits (17% vs. 50%, P = 0.005) at progression. Of 15 eyes with tumor recurrence, comparison (initial visit vs. time of recurrence) revealed more intraretinal deposits (7% vs. 47%, P = 0.04) at recurrence. At last visit, 39 eyes demonstrated tumor regression. By comparison (initial presentation vs. regression), there were less frequent vitreous opacities (67% vs. 0%, P < 0.001), intraretinal deposits (15% vs. 0%, P = 0.03), subretinal deposits (36% vs. 0%, P < 0.001), and subretinal pigment epithelium deposits (69% vs. 21%, P < 0.001) at regression. CONCLUSION: Using SD-OCT in patients with vitreoretinal lymphoma, local tumor regression correlated with a reduction in vitreous opacities, intraretinal deposits, subretinal deposits, and subretinal pigment epithelium deposits. SD-OCT is useful in judging vitreoretinal lymphoma response to therapy.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY DETECTS SUBCLINICAL RADIAL PERIPAPILLARY CAPILLARY DENSITY REDUCTION AFTER PLAQUE RADIOTHERAPY FOR CHOROIDAL MELANOMA.

PURPOSE: To evaluate radial peripapillary capillary density (RPCD) in irradiated eyes without radiation papillopathy clinically. METHODS: Patients treated with plaque radiotherapy for unilateral choroidal melanoma without radiation papillopathy clinically received optical coherence tomography and optical coherence tomography angiography imaging at ∼12- to 24-month follow-up. Comparison of RPCD globally and meridian closest to plaque and meridian farthest to plaque of irradiated versus nonirradiated eyes was performed. RESULTS: Mean age was 55 years (n = 10). Mean largest basal diameter and thickness were 10.1 and 4.4 mm, respectively. Mean radiation dose to the optic nerve head and foveola was 41.7 and 66.2 Gy, respectively. No radiation papillopathy was detected by ophthalmoscopy throughout follow-up (mean:14 months). Radial peripapillary capillary density was significantly reduced globally (all P < 0.02). Meridian closest to plaque RPCD was significantly reduced (P < 0.01), but not meridian farthest to plaque RPCD (P = 0.07). Circumpapillary retinal nerve fiber layer thickness was not significantly reduced (P > 0.26). Radiation dose to the optic nerve head was correlated with meridian closest to plaque RPCD reduction (r = 0.76; P < 0.01). Mean radiation dose to the optic nerve head for <5% and ≥5% RPCD reductions was 35.9 ± 12.2 and 55.2 ± 6.4 Gy, respectively. CONCLUSION: Radial peripapillary capillary density reduction was found in irradiated eyes before clinical evidence of radiation papillopathy and circumpapillary retinal nerve fiber layer thickness reduction. Radial peripapillary capillary density reduction is correlated to plaque location and radiation dose to the optic nerve head.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS OF DEEP CAPILLARY PLEXUS MICROISCHEMIA AFTER INTRAVENOUS CHEMOTHERAPY FOR RETINOBLASTOMA.

PURPOSE: To study changes in the foveal microvascular anatomy using optical coherence tomography angiography (OCTA) after intravenous chemotherapy (IVC) for retinoblastoma (RB). METHODS: A retrospective comparative case-control series included 10 age-matched normal eyes with no documented ocular pathology (control), 10 fellow eyes of patients with unilateral RB treated with IVC (RB fellow), and 10 eyes with extramacular RB in patients with bilateral RB treated with IVC (RB tumor). All eyes were scanned using enhanced depth imaging optical coherence tomography and OCTA. Enhanced depth imaging optical coherence tomography measurements of central macular thickness and subfoveolar choroidal thickness as well as OCTA measurements of foveal avascular zone (FAZ) area in superficial (sFAZ) and deep (dFAZ) plexus and capillary density (CD) in the superficial (sCD) and deep (dCD) plexus were performed. Comparison among the three groups was conducted. RESULTS: Among the three cohorts (control, RB fellow, and RB tumor), there was no difference in mean age at measurement (12, 10, and 12 years) and mean interval between last IVC and OCTA (RB fellow and RB tumor) (9, 10 years). Optical coherence tomography and OCTA revealed no significant difference in central macular thickness (all P ≥ 0.161), choroidal thickness (all P ≥ 0.066), sFAZ (all P ≥ 0.618), dFAZ (all P ≥ 0.610), and sCD (all P ≥ 0.638) comparing controls versus RB fellow, controls versus RB tumor, and RB fellow versus RB tumor. By contrast, mean dCD was significantly greater in controls (52%), compared with both RB fellow (49%, P = 0.026) and RB tumor (48%, P = 0.028) groups, but no difference was found between RB fellow and RB tumor (49% vs. 48%, P = 0.515). LogMAR visual acuity showed no difference among the three groups (all P ≥ 0.150). CONCLUSION: At mean 10-year follow-up, slight reduction in dCD seems to occur after IVC for RB without alterations in central macular thickness, choroidal thickness, FAZ, or sCD and without visual compromise.

PARAFOVEAL MICROVASCULAR FEATURES ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN EYES WITH CIRCUMSCRIBED CHOROIDAL HEMANGIOMA.

PURPOSE: To evaluate parafoveal microvasculature in eyes with circumscribed choroidal hemangioma using optical coherence tomography angiography. METHODS: Fourteen eyes with unilateral circumscribed choroidal hemangioma were imaged using OCT for central macular thickness and optical coherence tomography angiography for superficial and deep foveal avascular zone area, and superficial and deep capillary density (CD), comparing affected and paired fellow eyes. RESULTS: Mean patient age was 53 years, and mean visual acuity was 20/60 in the involved eye and 20/25 in the fellow eye. In the affected eye, clinical findings included cystoid macular edema (CME) in 1, parafoveal/subfoveal subretinal fluid (SRF) in three, and both CME and SRF in two eyes. OCT revealed current CME/SRF in six eyes, and normal foveal contour in eight eyes, among which five never demonstrated OCT evidence of CME or SRF. By OCT (affected vs. fellow eye), mean central macular thickness was greater in affected eyes (312 vs. 264 µm, P = 0.042). By optical coherence tomography angiography, there was no difference in superficial foveal avascular zone (P = 0.327), deep foveal avascular zone (P = 0.563), and superficial CD (P = 0.159), but mean deep plexus CD was reduced in affected eyes (50% vs. 54%, P = 0.010). Subgroup analysis showed that eyes with previous or current CME and/or SRF had reduced deep plexus CD (53% vs. 57%, P = 0.005), while eyes without previous/current CME/SRF had similar deep plexus CD (P = 0.399) compared with fellow eyes. CONCLUSION: Circumscribed choroidal hemangioma generally does not affect parafoveal inner retinal microvasculature. However, a reduction in deep plexus CD can be expected in eyes with previous or current CME/SRF.

ALTERED PARAFOVEAL MICROVASCULATURE IN TREATMENT-NAIVE CHOROIDAL MELANOMA EYES DETECTED BY OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To determine whether parafoveal microvascular changes have occurred in choroidal melanoma eyes before radiotherapy. METHODS: A retrospective cohort study included 30 consecutive patients with unilateral choroidal melanoma. The microvascular structure was analyzed by optical coherence tomography angiography. Fellow eyes served as control for affected eyes. RESULTS: Optical coherence tomography angiography demonstrated no difference in superficial foveal avascular zone (P = 0.316), but showed significant enlargement in deep foveal avascular zone (P < 0.0001) in affected eyes as compared with fellow eyes. It also showed significant decrease in superficial (P = 0.004) and deep (P = 0.0003) capillary vascular density (CVD). In subgroup analyses, the presence of subretinal fluid contributed to significant enlargement of deep foveal avascular zone (P < 0.0001) as well as reduction in superficial (P = 0.0001) and deep (P = 0.001) CVD. By linear regression, greater tumor diameter correlated with reduction in deep CVD (y = -0.42x + 1.01, Pslope = 0.021); greater tumor thickness correlated with reduction in both superficial (y = -0.67x + 0.24, Pslope = 0.039) and deep (y = -0.82x + 0.56, Pslope = 0.002) CVD. CONCLUSION: Optical coherence tomography angiography documented significant enlargement of deep foveal avascular zone as well as reduction in superficial and deep CVD in affected eyes. These findings correlated with the presence of subretinal fluid and increasing tumor size, suggesting that tumor-related factors contribute to parafoveal microvascular ischemia.

DIFFERENTIAL MACULAR FEATURES ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN EYES WITH CHOROIDAL NEVUS AND MELANOMA.

PURPOSE: To evaluate optical coherence tomography angiography (OCTA) characteristics of macular vascular details in eyes with choroidal nevus and melanoma, compared with unaffected contralateral eye. METHODS: Retrospective comparative analysis comprising 70 patients with choroidal nevus and 36 patients with choroidal melanoma examined with optical coherence tomography angiography. Measurements included central macular thickness (CMT), superficial (sFAZ) and deep (dFAZ) foveal avascular zone (FAZ) area, and superficial (sCVD) and deep (dCVD) capillary vascular density (CVD). RESULTS: In patients with nevus, CMT (affected vs. unaffected eye) was similar (267 vs. 265 µm, P = 0.67), whereas in patients with melanoma, CMT demonstrated greater thickness in affected eye (322 vs. 259 µm, P < 0.0001). In eyes with nevus, the FAZ (affected vs. unaffected eye) showed similar values in sFAZ (0.238 vs. 0.232 mm, P = 0.72) and dFAZ (0.284 vs. 0.271 mm, P = 0.48). In eyes with melanoma, similar values for sFAZ (0.249 vs. 0.260 mm, P = 0.73) were noted, whereas dFAZ was larger in the affected eye with macular melanoma (0.530 vs. 0.252 mm, P = 0.012) and similar in eyes with extramacular melanoma (0.300 vs. 0.285, P = 0.75). Regarding CVD (affected vs. unaffected eye), eyes with nevus showed similar sCVD (54 vs. 53.9%, P = 0.81) and dCVD (60 vs. 59.4%, P = 0.19). Reduced dCVD was found in four eyes with nevus, all with three or more risk factors for growth (P < 0.01). In eyes with melanoma, significant reduction was found in both sCVD (50.5 vs. 53.1%, P = 0.006) and dCVD (56.6% vs. 59.8%, P = 0.0003). Reduction in sCVD was related to presence of subretinal fluid (P = 0.03). Reduction in dCVD was found with subretinal fluid presence (P = 0.003) or absence (P = 0.03), with both macular (P < 0.001) and extramacular (P < 0.001) melanoma, and with increasing tumor thickness (P = 0.005). Reduced dCVD was found in 11% of eyes with nevus versus 58% of eyes with melanoma (Chi-square test: 26.31; P < 0.0001). CONCLUSION: Compared with contralateral eye, eyes with choroidal nevus demonstrate similar CMT, FAZ, and CVD. In contrast, eyes with melanoma show increased CMT, enlarged FAZ, and reduced CVD, particularly related to increasing tumor thickness. Optical coherence tomography angiography could be a useful tool in differentiating choroidal nevus from melanoma.

VARIABILITY IN FOVEAL AVASCULAR ZONE AND CAPILLARY DENSITY USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY MACHINES IN HEALTHY EYES.

PURPOSE: To evaluate the variability in foveal avascular zone (FAZ) and capillary density measurements on optical coherence tomography angiography using Optovue RTVue XR Avanti (OA) (Optovue) and Zeiss Cirrus HD-OCT 5000 (ZC) (Carl Zeiss Meditec). METHODS: In this prospective, comparative case series, parafoveal (3 × 3 mm) optical coherence tomography angiography scans were obtained on healthy volunteers using both the Avanti and Cirrus. The FAZ area and capillary density at the level of both the superficial and deep capillary plexus were measured automatically using the built-in ReVue software (Optovue) with the Avanti as well as manually using ImageJ (National Institutes of Health) with both machines. RESULTS: There were 50 eyes in 25 healthy volunteers included in the analysis. Mean subject age was 33 years and there were 14 women (56%). On optical coherence tomography, mean central macular thickness was significantly greater on OA (259.1 µm) than ZC (257.6 µm, P = 0.0228). On optical coherence tomography angiography, mean superficial and deep plexus FAZ measured 0.2855 mm and 0.3465 mm on Avanti automated (A-A), 0.2739 mm and 0.3637 mm on Avanti manual (A-M), and 0.2657 mm and 0.3993 mm on Cirrus manual (C-M), respectively. There were no statistically significant differences in superficial plexus FAZ measurements between the A-A and A-M (P = 0.4019) or A-A and C-M (P = 0.1336). The A-M measured significantly larger than C-M (P = 0.0396). Deep plexus FAZ measurements were similar on A-A and A-M (P = 0.6299), but both were significantly less compared with C-M (P < 0.0001 for A-A vs. C-M, P = 0.0184 for A-M vs. C-M). Mean superficial and deep plexus capillary densities were 53.6% and 59.3% on A-A, 48.1% and 47.7% on A-M, and 52.5% and 48.1% on C-M, respectively. Superficial plexus capillary density measurements were statistically similar on A-A and C-M (P = 0.0623), but both were significantly higher than A-M (P < 0.0001 for A-A vs. A-M, P < 0.0001 for A-M vs. C-M). However, deep plexus capillary density measurements on A-A were significantly higher than A-M (P < 0.0001) and C-M (P < 0.0001), but A-M and C-M measurements were similar (P = 0.5986). There was no significant difference in all parameters measured in both eyes of one subject using any of the three measuring techniques. CONCLUSION: While measurements taken with the same machine and technique are consistent and reliable between fellow eyes, significant variability exists in FAZ and capillary density measurements among different machines and techniques. Comparison of measurements across machines and techniques should be considered with caution.

IMAGE QUALITY AND ARTIFACTS ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY: Comparison of Pathologic and Paired Fellow Eyes in 65 Patients With Unilateral Choroidal Melanoma Treated With Plaque Radiotherapy.

PURPOSE: To study image quality and artifacts seen on optical coherence tomography angiography (OCTA). METHODS: Sixty-five consecutive patients with unilateral posterior uveal melanoma treated with plaque radiotherapy had OCTA during follow-up. Optical coherence tomography angiography was performed on both the affected and fellow eye. Signal strength and frequency of image artifacts on en face images were compared between affected and fellow eyes. RESULTS: A total of 130 eyes in 65 patients were analyzed, the mean age at time of OCTA was 55 years (median: 56, range: 12-81 years), and 39 (39/65, 60%) were female. Majority of tumors were located in the choroid (62/65, 95%) and extramacular (55/65, 85%). The mean distance to the foveola was 4 mm (median: 3, range: 0-18 mm) and optic nerve was 4 mm (median: 4, range: 0-16 mm). Optical coherence tomography angiography was performed at a mean 46 months after plaque radiotherapy. Most patients had a history of radiation maculopathy or papillopathy in the treated eye at the time of OCTA (46/65, 71%). Overall, 95 eyes (95/130, 73%) had at least one significant artifact on OCTA. The most common major artifacts were loss of focus (71/130, 55%), broad (>5 pixels width and >4 lines) blink lines (48/130, 37%), motion artifact (34/130, 26%), specular dot (33/130, 25%), and edge duplication (10/130, 8%). Statistically, eyes treated with plaque radiotherapy (affected vs. fellow eye) were more likely to have at least one major OCTA artifact (92 vs. 54%, P < 0.001) and, specifically, loss of focus was more frequent (78 vs. 31%, P < 0.001). Multivariate analysis found decreased visual acuity significantly associated with higher incidence of broad blink lines (P = 0.0166) and loss of signal (P < 0.0001), whereas male sex was associated with increased loss of signal (P = 0.0015), and distance to the foveola was related to edge duplication (P = 0.0119). CONCLUSION: Image artifacts on OCTA are commonly encountered and appear to be more frequent in eyes with pathology and poor visual acuity. Recognition of these artifacts might help improve image interpretation and decision making.

PARAFOVEAL CAPILLARY DENSITY AFTER PLAQUE RADIOTHERAPY FOR CHOROIDAL MELANOMA: Analysis of Eyes Without Radiation Maculopathy.

PURPOSE: To study the parafoveal capillary density using optical coherence tomography angiography (OCTA) in eyes treated with plaque radiotherapy. RESEARCH DESIGN: Retrospective comparative case series. PARTICIPANTS: Ten patients treated with plaque radiotherapy for choroidal melanoma without evident radiation maculopathy on ophthalmoscopy or optical coherence tomography were imaged with OCTA. MAIN OUTCOME MEASURE: Comparison of the parafoveal capillary density in the superficial and deep capillary plexuses in the irradiated versus fellow nonirradiated eye. RESULTS: Overall, mean patient age was 55 years (median: 57, range: 45-65 years), and majority were white (9/10, 90%) and female (9/10, 90%). No patient had diabetes mellitus, and 2 (2/10, 20%) had controlled systemic hypertension. The melanoma was located in the choroid in all patients (10/10, 100%), with 2 (2/10, 20%) in the macular region. The mean largest basal diameter was 11 mm (12, 6-16 mm), and mean thickness was 5 mm (5, 2-10 mm). Mean radiation dose to the tumor apex was 72 Gy (median: 70 Gy, range: 70-85 Gy). Mean foveolar radiation dose was 53 Gy (median: 35 Gy, range: 14-110 Gy). Mean follow-up duration after plaque radiotherapy was 17 months (median: 16 months, range: 5-39 months). There was no ophthalmoscopic evidence of radiation maculopathy throughout the follow-up in every case. Optical coherence tomography demonstrated normal foveal contour without edema or subretinal fluid in every case. On OCTA, there was no significant difference in signal strength (P = 0.1151), central macular thickness (P = 0.9316), and foveal avascular zone area in the superficial (P = 0.1595), and deep (P = 0.1534) capillary plexuses between the irradiated versus fellow eyes. However, there was a statistically significant decrease in capillary density in the parafoveal superficial (P = 0.0005) and deep (P = 0.0067) plexuses in the irradiated eye. In addition, mean logMAR visual acuity was 0.2 (0.1, 0.0-0.5) (Snellen equivalent 20/32) in the irradiated eye and 0.0 (0.0, range: 0.0-0.1) (Snellen equivalent 20/20) in the fellow eye (P = 0.0252). CONCLUSION: Optical coherence tomography angiography allows qualitative and quantitative analysis of parafoveal capillary density. After plaque radiotherapy for choroidal melanoma, in eyes with normal macular features on ophthalmoscopy and optical coherence tomography, there is a statistically significant decrease in parafoveal capillary density and logMAR visual acuity in irradiated eyes compared with fellow eyes. These subclinical ischemic findings represent the commencement of radiation maculopathy.

Choroidal osteoma shows bone lamella and vascular channels on enhanced depth imaging optical coherence tomography in 15 eyes.

PURPOSE: To describe enhanced depth imaging optical coherence tomography findings of choroidal osteoma. METHODS: Retrospective, observational case series of 15 eyes with choroidal osteoma imaged with EDI-OCT. RESULTS: The mean age at presentation was 27 years. There were 10 women and 3 men. The visual acuity ranged from 20/20 to hand motion, with reduced visual acuity secondary to photoreceptor loss in the foveola (n = 5) or subfoveal fluid (n = 1), and additional choroidal neovascular membrane (n = 3). The mean basal tumor diameter was 8.0 mm, and ultrasonographic thickness was 1.5 mm. Using enhanced depth imaging optical coherence tomography, the mean tumor thickness was 589 µm when compared with a matched choroidal region in the unaffected eye of 247 µm (138% increased thickness) (P = 0.009). The tumor surface topography was classified (ultrasonography vs. enhanced depth imaging optical coherence tomography) as flat (87 vs. 13%), dome (13 vs. 40%), or undulating (0 vs. 47%). On enhanced depth imaging optical coherence tomography, unique features included horizontal lamellar lines (presumed bone lamella) (n = 15, 100%) and hyperreflective horizontal lines (presumed cement lines) (n = 8, 53%). Other features included horizontal tubular lamella with optically empty center (presumed Haversian canals or vascular channels) (n = 9, 60%), vertical tubular lamella (presumed Volkmann canals or vascular channels) (n = 2, 13%), and speckled regions (presumed compact or small trabecular bone) (n = 6, 40%). Of the nine eyes with subfoveolar osteoma, the tumor was completely ossified (n = 4), partially deossified (n = 2), or completely deossified (n = 3). Photoreceptor thinning/atrophy was found in all five eyes with deossified osteoma, whereas intact photoreceptor appearance was noted in the four eyes with ossified subfoveal osteoma. CONCLUSION: Enhanced depth imaging optical coherence tomography reveals characteristic surface topography of choroidal osteoma as dome or undulating (87%) with unique intrinsic features of horizontal lamellar lines (100%), horizontal (60%) or vertical (13%) tubules, and speckled regions (40%). Photoreceptor loss was evident in every case of tumor deossification.

CORRELATION OF FOVEAL AVASCULAR ZONE SIZE WITH FOVEAL MORPHOLOGY IN NORMAL EYES USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To analyze the foveal avascular zone (FAZ) in normal eyes using optical coherence tomography angiography. METHODS: Prospective noncomparative case series. The parafoveal region of 70 eyes from 67 healthy subjects was imaged using optical coherence tomography angiography to visualize the superficial and deep capillary plexuses and correlated with standard macular volume scans using spectral domain optical coherence tomography to determine foveal morphology. RESULTS: In all 70 eyes imaged, 2 vascular plexuses could be detected within the retina: a superficial plexus within the ganglion cell layer and a deep plexus within the inner nuclear layer. A measurable FAZ was visualized in both plexuses in all imaged eyes. The FAZ area was variable in the study population with a mean of 0.266 mm² ± 0.097 mm² in the superficial plexus (range: 0.071 mm²-0.527 mm²) and a mean of 0.495 mm² ± 0.227 mm² in the deep plexus (range: 0.160 mm²-0.795 mm²). The FAZ area was significantly larger in the deep plexus (P < 0.0001) compared with superficial plexus. The FAZ area in both plexuses correlated inversely with central macular thickness and central macular volume (P < 0.0001). No significant correlation was found between superficial plexus FAZ area and age (P = 0.55) or sex (P = 0.34). In the same manner, no significant correlation was found between deep plexus FAZ area and age (P = 0.13) or sex (P = 0.13). CONCLUSION: Optical coherence tomography angiography provides a noninvasive method to visualize and measure the superficial and deep plexus FAZ in a normal population. The FAZ can vary in size and shape, with the FAZ area significantly larger in the deep compared with the superficial plexus. Both superficial and deep FAZ area correlate inversely with foveal thickness and volume.

Enhanced depth imaging optical coherence tomography of intraocular tumors: from placid to seasick to rock and rolling topography--the 2013 Francesco Orzalesi Lecture.

PURPOSE: To review enhanced depth imaging optical coherence tomography of intraocular tumors. METHODS: Review of tumor surface topography and internal characteristics based on published reports and personal experience. RESULTS: Using enhanced depth imaging optical coherence tomography, choroidal nevus showed smooth moderate dome-shape and with overlying retinal pigment epithelial alterations, subretinal cleft, and photoreceptor loss. Choroidal melanoma was smooth, moderately dome-shaped, and with overlying "shaggy" photoreceptors. Choroidal metastasis showed "lumpy, bumpy" irregular surface topography, subretinal fluid, and shaggy photoreceptors. Choroidal hemangioma was smooth, acutely dome-shaped and with subretinal fluid and/or cystoid retinal edema. Choroidal lymphoma showed "placid, rippled, or seasick" surface, correlating with increasing tumor thickness. Choroidal osteoma displayed smooth undulating surface with intralesional lamellar lines and tubules, representing bone lamellae or vessels. Choroidal melanocytosis produced flat but uniformly thickened choroid with increased stromal density. Choroidal hemorrhage displayed slightly "scalloped" surface in the outer choroid. All choroidal tumors showed inward compression of the choroidal vasculature, except for hemangioma in which the vessels were expanded. Sclerochoroidal calcification arose within the sclera as a "rocky" or "rolling" topography and solitary idiopathic choroiditis appeared as a domed or "volcanic" focal scleral thickening, each causing intense choroidal compression. Retinal tumors such as small retinoblastoma, astrocytic hamartoma, and hemangioblastoma arose abruptly adjacent to normal retina. Exophytic retinoblastoma and retinal hemangioblastoma depicted a full-thickness disorganized retinal mass with normal retina draped over the margins. Flat astrocytic hamartoma arose within the nerve fiber layer, and thicker tumors involved full-thickness retina with "moth-eaten" or cavitary appearance. Retinal pigment epithelial lesions such as congenital hypertrophy of retinal pigment epithelial showed flat topography with transmission of light through lacunae, occasional subretinal cleft and uniform photoreceptor loss, whereas combined hamartoma of retina/retinal pigment epithelial showed "sawtooth" pattern of vitreoretinal traction leading to mini-peak or maxi-peak retinal folds. CONCLUSIONS: Enhanced depth imaging optical coherence tomography shows characteristic topographical and intralesional patterns that appear to be suggestive for selected intraocular tumors.

Combined hamartoma of the retina and retinal pigment epithelium: findings on enhanced depth imaging optical coherence tomography in eight eyes.

PURPOSE: To assess combined hamartoma of the retina and retinal pigment epithelium with enhanced depth imaging optical coherence tomography. METHODS: Retrospective, observational cases series in eight eyes of eight patients, with comparison between affected and unaffected eyes regarding enhanced depth imaging optical coherence tomography features of tumor, fovea, and choroid. RESULTS: The mean age at presentation was 7 years. The tumor was macular (n = 5) or extramacular (n = 3). Enhanced depth imaging optical coherence tomography revealed irregularities in inner retina (n = 8) and/or all retinal layers (n = 3), with epiretinal membrane (n = 8), causing an inner retinal sawtooth (mini-peak) pattern (n = 2), full thickness retinal folds (maxi-peak) (n = 3), or both (n = 3). In the 5 macular tumors, foveal retinal thickness measured mean 608 µm compared with 244 µm in the unaffected eye (P = 0.0004). Mean tumor epicenter retinal thickness in 8 tumors measured 650 µm compared with 327 µm in a corresponding area in the unaffected eye (P = 0.01). In all cases, choroidal thickness beneath the tumor epicenter was decreased at mean 210 µm compared with 328 µm in the corresponding area of unaffected eye (P = 0.009). CONCLUSION: Enhanced depth imaging optical coherence tomography of combined hamartoma revealed epiretinal membrane with vitreoretinal traction in a sawtooth (mini-peak) or folded (maxi-peak) pattern. Combined hamartoma seems to be a thickened retinal mass secondary to focal vitreoretinal traction.

Enhanced depth imaging optical coherence tomography of choroidal nevus in 104 cases.

PURPOSE: To describe the characteristics of choroidal nevus using the enhanced depth imaging (EDI) feature of spectral-domain optical coherence tomography (OCT). DESIGN: Retrospective, observational case series. PARTICIPANTS: One hundred four eyes with choroidal nevus. METHODS: Spectral-domain EDI OCT was performed with a Heidelberg Spectralis HRA+OCT (Heidelberg Engineering, Heidelberg, Germany) using a custom scan acquisition protocol of up to 13 raster lines of 9-mm scan length with automatic real-time image averaging set at 100 images. The thickness of choroidal nevus was measured by combining Heidelberg's autosegmentation with manual segmentation. MAIN OUTCOME MEASURES: Imaging features and thickness correlation of choroidal nevus by EDI OCT versus standard ultrasonography. RESULTS: Of 104 eyes with choroidal nevus imaged with EDI OCT, 51 (49%) displayed image detail suitable for study. The remaining 53 cases were suboptimal because of statistically identified factors of age older than 60 years (P = 0.027), female gender (P = 0.008), extramacular location of nevus (P<0.001), mean distance from foveola more than 3 mm (P = 0.002), mean distance from optic disc more than 4 mm (P<0.001), and mean maximal basal diameter more than 5 mm (P = 0.006). Of the 51 suitable cases, mean nevus thickness was 685 µm (median, 628 µm; range, 184-1643 µm) by EDI OCT compared with 1500 µm (median, 1500 µm; range, 1000-2700 µm) by ultrasonography. The most common EDI OCT imaging features included partial (59%) or complete (35%) choroidal shadowing deep to the nevus, choriocapillaris thinning overlying the nevus (94%), retinal pigment epithelial (RPE) atrophy (43%), RPE loss (14%), RPE nodularity (8%), photoreceptor loss (43%), inner segment-outer segment junction (IS-OS) irregularity (37%), IS-OS loss (6%), external limiting membrane irregularity (18%), outer nuclear and outer plexiform layer irregularity (8%), and inner nuclear layer irregularity (6%). Overlying subretinal fluid was identified by EDI OCT (16%), ophthalmoscopic examination (8%), and ultrasonographic evaluation (0%). A comparison of pigmented versus nonpigmented nevus showed only 1 significant difference of more intense choroidal shadowing with pigmented nevus (P = 0.046). CONCLUSIONS: Imaging of choroidal nevus with EDI OCT enables precise measurement of tumor thickness with comparatively reduced thickness relative to ultrasonography. Using EDI OCT, 94% of choroidal nevi were found to have overlying choriocapillaris thinning.

White Paper on Ophthalmic Imaging for Choroidal Nevus Identification and Transformation into Melanoma.

Purpose: To discuss the evolution of noninvasive diagnostic methods in the identification of choroidal nevus and determination of risk factors for malignant transformation as well as introduce the novel role that artificial intelligence (AI) can play in the diagnostic process. Methods: White paper. Results: Longstanding diagnostic methods to stratify benign choroidal nevus from choroidal melanoma and to further determine the risk for nevus transformation into melanoma have been dependent on recognition of key clinical features by ophthalmic examination. These risk factors have been derived from multiple large cohort research studies over the past several decades and have garnered widespread use throughout the world. More recent publications have applied ocular diagnostic testing (fundus photography, ultrasound examination, autofluorescence, and optical coherence tomography) to identify risk factors for the malignant transformation of choroidal nevus based on multimodal imaging features. The widespread usage of ophthalmic imaging systems to identify and follow choroidal nevus, in conjunction with the characterization of malignant transformation risk factors via diagnostic imaging, presents a novel path to apply AI. Conclusions: AI applied to existing ophthalmic imaging systems could be used for both identification of choroidal nevus and as a tool to aid in earlier detection of transformation to malignant melanoma. Translational Relevance: Advances in AI models applied to ophthalmic imaging systems have the potential to improve patient care, because earlier detection and treatment of melanoma has been proven to improve long-term clinical outcomes.

Photoreceptor morphology and correlation with subretinal fluid chronicity associated with choroidal nevus.

BACKGROUND: Subretinal fluid (SRF) can be associated with choroidal nevus and can cause progressive change in the morphology of overlying photoreceptors. METHODS: A retrospective observational study was performed using optical coherence tomography to assess nevus and SRF features, as well as photoreceptor morphology over time. RESULTS: There were 232 choroidal nevi that presented with or developed SRF. Photoreceptor morphology at presentation was classified as normal (n=60, 26%), shaggy (elongated) (n=73, 31%), retracted (stalactite appearance) (n=76, 33%), or absent (n=23, 10%). There was a progression in photoreceptor morphology with increasing SRF chronicity (p=0.003). For nevus presenting with normal photoreceptors and later developed SRF (n=60), photoreceptors became shaggy in 29 (48%), retracted in 24 (40%), and absent in 7 (12%) after 15, 19 and 22 months, respectively. For nevus presenting with SRF and shaggy photoreceptors (n=73), progression to retracted photoreceptors occurred in 31 (42%) after a mean of 22 months; for nevus with SRF and retracted photoreceptors (n=76), progression to absent photoreceptors occurred in 19 (25%) after a mean of 34 months; and for nevus with absent photoreceptors (n=23), photoreceptor morphology showed no change after mean follow-up of 33 months. Risk of nevus growth to melanoma was not associated with photoreceptor morphology at presentation (p=0.19). CONCLUSION: In eyes with choroidal nevus and SRF, there is a longitudinal evolution in photoreceptor morphology from normal to shaggy to retracted then absent with increasing SRF chronicity. SRF chronicity, as indicated by photoreceptor morphology on presentation, did not correlate with nevus growth to melanoma.

Wide-Field (15â€Š× 9 mm) Swept-Source Optical Coherence Tomography Angiography Following Plaque Radiotherapy of Choroidal Melanoma: An Analysis of 105 eyes.

PURPOSE: The aim of this study was to evaluate retinal microvascular abnormalities following plaque radiotherapy of choroidal melanoma (CM) using wide-field swept-source optical coherence tomography angiography (OCTA). DESIGN: Single-centre retrospective review. METHODS: Retrospective case series of 105 CM patients treated with I-125 plaque radiotherapy and imaged with wide-field (15 × 9 mm) SS-OCTA from March 2018 to August 2018 at the Ocular Oncology Service, Wills Eye Hospital (Philadelphia, PA). RESULTS: At mean follow-up of 49 months (range 4-297) after plaque radiotherapy, there were 52 eyes (50%) with clinically evident radiation retinopathy (CERR) and 53 eyes (50%) without CERR. Comparison (CERR vs controls) revealed foveal avascular zone enlargement (1.7 vs 0.23 mm, P = 0.03) and reduction of capillary vascular density (CVD) in the superficial and deep plexus in the total wide-field (43% vs 47%, P < 0.001, and 46% vs 48%, P = 0.001, respectively), peripapillary region (66% vs 77%, P < 0.001, and 66% vs 72%, P = 0.001, respectively), and papillomacular bundle (60% vs 68%, P < 0.001, and 61% vs 64%, P = 0.03, respectively). Comparison (no CERR vs controls) revealed nonsignificant foveal avascular zone enlargement (1.20 vs 0.23 mm, P = 0.16) and reduction of CVD in the superficial plexus (46% vs 47%, P = 0.008), and not the deep plexus (48% vs 48%, P = 0.42) of the total wide-field. Comparison of irradiated eyes (CERR vs no CERR) showed reduction of CVD in the superficial and deep plexus of the total wide-field (43% vs 46%, P < 0.006, and 46% vs 48% P < 0.02, respectively), peripapillary region (66% vs 74%, P < 0.001, and 66% vs 72% P < 0.01, respectively), and superficial plexus in the papillomacular bundle (60% vs 65%, P = 0.03). CONCLUSIONS: Following plaque radiotherapy for choroidal melanoma, wide-field swept-source optical coherence tomography angiography demonstrates retinal microvascular abnormalities in the CVD in eyes with and without CERR. These findings are important in early detection and monitoring of radiation retinopathy.