Search

Purpose: To describe the optical coherence tomography angiography features of congenital simple hamartoma of the retinal pigment epithelium., Methods: Case report., Results: A 14-year-old boy was referred for an asymptomatic fundus tumor in the left eye. Visual acuity was 20/20 in the right eye and 20/40 in the left eye. The right fundus was normal. The left fundus disclosed a gray-white nodular retinal mass of 2-mm diameter in the juxtafoveal region, protruding into the vitreous cavity and causing radial retinal folds. The mass demonstrated echodensity without calcification on ultrasonography, hypoautofluorescence on short-wavelength autofluorescence, and mixed hyporeflective and hyperreflective areas on near-infrared reflectance imaging. By fluorescein angiography, the mass showed absolute hypofluorescence through the arteriovenous phase and diffuse late hyperfluorescence and staining. Optical coherence tomography revealed an optically dense lesion with abrupt posterior shadowing, whereas optical coherence tomography angiography showed an extensive haphazard intratumoral vascular network with fairly large-caliber (100-200 microns) vessels occupying full-thickness tumor, demonstrating more vascular details than visualized on fluorescein angiography. Serial segmental evaluation of the optical coherence tomography angiography en face images confirmed an intratumoral vascular network for full tumor depth down to retinal pigment epithelium, not related to projection artifact. Final diagnosis was congenital simple hamartoma of the retinal pigment epithelium. Considering the benign tumor, observation was recommended., Conclusion: Optical coherence tomography angiography of congenital simple hamartoma of the retinal pigment epithelium revealed an extensive intratumoral, haphazard, large-caliber vascular network, more evident than seen on fluorescein angiography. Optical coherence tomography angiography has added new insight into this rare tumor.

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.

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: To report subclinical retinal hemangioblastoma detected by enhanced depth imaging optical coherence tomography and fluorescein angiography in at-risk twins., Methods: Case report., Results: A set of twins, age 7 years, (Twin A and Twin B) with known family history of von Hippel-Lindau disease (gene test positive) and no systemic manifestations were evaluated. Visual acuity was 20/20 in both eyes of both twins. Anterior segment examination and intraocular pressures were unremarkable in both eyes. Twin A showed no clinically visible tumor in the right eye, and a clinically evident 4-mm hemangioblastoma in the superior retina of the left eye. The enhanced depth imaging optical coherence tomography demonstrated normal fovea in both eyes. However, imaging at the inferonasal juxtapapillary region in the right eye documented an intraretinal mass from nerve fiber layer to outer plexiform layer on enhanced depth imaging optical coherence tomography and with hyperfluorescence on fluorescein angiography, consistent with retinal hemangioblastoma. Twin B demonstrated no clinically visible tumors in both eyes, but the left eye showed a small hyperreflective lesion in the parafoveal region on spectral domain optical coherence tomography from inner to outer nuclear layers, with no cystoid changes or subretinal fluid. The lesion was slightly hyperfluorescent on fluorescein angiography, consistent with hemangioblastoma. The optical coherence tomography angiography showed no vascularity within the lesion. Twin A was treated with laser photocoagulation to the larger hemangioblastoma in the left eye, and the asymptomatic juxtapapillary tumor was observed. Twin B was managed with cautious observation as treatment to the left eye could lead to vision loss., Conclusion: Patients at risk for retinal hemangioblastoma should have routine imaging with fundus photography, fluorescein angiography, and enhanced depth imaging optical coherence tomography for subclinical detection of asymptomatic tumors.

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.

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.