Recent advances in understanding and managing retinal vein occlusions.

Retinal vein occlusions are the second most common form of retinal vascular disease. Previously, laser treatment for branch retinal vein occlusion and intravitreal triamcinolone acetonide for central retinal vein occlusion were the standard of care. Recent studies have demonstrated that anti-vascular endothelial growth factor (anti-VEGF) agents have a superior safety and efficacy profile for the treatment of both branch and central retinal vein occlusions. The use of wide-field fluorescein angiography has also allowed better visualization of the retinal periphery. Despite the better documentation of retinal non-perfusion, laser photocoagulation to the areas of non-perfusion does not seem to result in a reduction of macular edema or reduction in treatment burden and has been relegated to patients who develop rubeosis or neovascularization of the retina. More recently, several studies have demonstrated the use of a long-acting dexamethasone implant administered intravitreally or triamcinolone administered in the suprachoroidal space as a viable approach to treat retinal vein occlusion.

IATROGENIC OCCLUSION OF THE OPHTHALMIC ARTERY AFTER SODIUM TETRADECYL SULFATE INJECTION IN THE FOREHEAD.

PURPOSE: To describe a patient with ophthalmic artery occlusion after sodium tetradecyl sulfate foam injection in the forehead. METHODS: In this case report, a description of the clinical examination and imaging findings, including fundus photography, spectral-domain optical coherence tomography, and fluorescein angiography are reported. RESULTS: A 33-year-old white woman presented with severe painless vision loss in the left eye after cosmetic treatment of a forehead vein with sodium tetradecyl sulfate foam. Her visual acuity in the left eye was hand motions, and her fundus revealed diffuse retinal whitening in the macula with the absence of a cherry red spot and disruption of the arteriolar blood supply. Spectral-domain optical coherence tomography revealed intense hyperreflectivity of the inner and outer retina with loss of the foveal contour, and fluorescein angiography revealed delayed filling of the choroidal vasculature and cilioretinal artery with incomplete filling of the retinal arterioles in the late frames consistent with ophthalmic artery occlusion. CONCLUSION: This case represents a devastating ocular complication after the inadvertent injection of sodium tetradecyl sulfate into a forehead artery, resulting in occlusion of the ophthalmic artery and severe vision loss.

New associations of classic acute macular neuroretinopathy.

PURPOSE: To describe novel underlying associations of classic acute macular neuroretinopathy (AMN). METHODS: Multimodal imaging case series evaluating patients with classic AMN lesions and previously unreported underlying aetiologies. RESULTS: Six patients were included (five women, one man, mean age 30±7 years). Mean distance best corrected visual acuity at initial presentation was 0.21±0.3 logMAR (mean Snellen acuity: 20/30, range 20/15-20/100) and at last follow-up visit 0.09±0.17 logMAR (Snellen acuity: 20/20, range 20/15-20/60). All cases but one had bilateral lesions and showed typical parafoveal hyporeflective lesions on infrared imaging, which corresponded to the hyper-reflectivity in the Henle's layer with attenuation of the external limiting membrane, the ellipsoid zone and interdigitation zone. Underlying diseases included thrombocytopenia and anaemia associated with dengue fever, acute lymphoblastic leukaemia, chronic kidney disease and ulcerative colitis, while Valsalva-like manoeuvre was found to be a potential trigger. Other novel associations included the use of lisdexamphetamine. CONCLUSIONS: Classic AMN may be associated with leukaemia, dengue fever, ulcerative colitis and chronic kidney disease, probably as a result of chorioretinal hypoxia in the setting of thrombocytopenia and anaemia. Adrenergic agonists such as lisdexamphetamine may also contribute to the manifestation of AMN.

CHORIORETINAL ATROPHY IN ALAGILLE SYNDROME.

PURPOSE: To describe multimodal imaging findings in a patient with Alagille syndrome and associated chorioretinopathy. METHODS: In this case report, clinical examination and multimodal imaging, including fundus photography, spectral domain optical coherence tomography, and fundus autofluorescence (AF) and wide-field AF, are presented. RESULTS: An 18-year-old Iranian-American girl with known history of Alagille syndrome reported diminished vision in both eyes. Color photographs depicted diffuse hypopigmentation of the fundus and atrophy of the macular retinal pigment epithelium with foveal sparing. Spectral domain optical coherence tomography revealed attenuation of the retinal pigment epithelium and overlying ellipsoid band. Fundus AF imaging of the macula revealed a geographic area of decreased AF that extended to the peripapillary region and inferotemporal arcade and contained discernable ovoid areas of deep AF loss in the nasal macula. Wide-field AF revealed a continuous and circumferential area of well-delineated decreased AF in the retinal periphery of both eyes. CONCLUSION: This case represents a severe manifestation of posterior and peripheral chorioretinopathy in a patient with Alagille syndrome. This is the first documentation of significant peripheral chorioretinal atrophy as assessed by wide-field AF imaging in this condition.

Spectral-domain optical coherence tomography as an indicator of fluorescein angiography leakage from choroidal neovascularization.

PURPOSE: To evaluate spectral-domain optical coherence tomography (SD-OCT) findings that predict angiographic leakage in choroidal neovascularization (CNV). METHODS: SD-OCT and fluorescein angiography (FA) images of 93 eyes of 93 patients were retrospectively analyzed. All patients were previously treated with anti-vascular endothelial growth factor agents for CNV from age-related macular degeneration. FA images were analyzed to assess the presence of leakage. SD-OCT images were analyzed to identify the overall presence of fluid, as well as specific patterns of fluid presentation, including intraretinal cystic spaces (ICS), retinal pigment epithelium detachment (PED), and neurosensory detachment (NSD). The presence of ultrastructural features such as intraretinal hyperreflective flecks and the inherent reflectivity and boundary definition of the subretinal material were evaluated. Both the association and the sensitivity, specificity, and both positive and negative predictive values of SD-OCT findings compared with FA leakage were calculated. RESULTS: A statistically significant association between SD-OCT findings and FA leakage was found for eyes that displayed fluid, NSD, intraretinal flecks, and low reflectivity or undefined boundaries from subretinal material, and not for PED or ICS. Sensitivity and specificity for SD-OCT findings were, respectively: 94% and 27% for fluid; 68% and 88% for NSD; 81% and 83% for intraretinal flecks; 63% and 92% for undefined boundaries of subretinal material; and 94% and 87% for low reflectivity from subretinal material. CONCLUSIONS: The evidence of fluid on SD-OCT is sensitive but nonspecific in identifying FA leaky CNV. The assessment of neurosensory detachment as well as other ultrastructural elements may increase the specificity of analysis.

Displayed reflectivity of choroidal neovascular membranes by optical coherence tomography correlates with presence of leakage by fluorescein angiography.

PURPOSE: To evaluate and correlate the displayed optical reflectivity of choroidal neovascularization (CNV) subretinal material on spectral-domain optical coherence tomography with the presence of dye leakage on fluorescein angiography (FA). METHODS: Thirty-nine eyes of 39 patients with a diagnosis of predominantly classic CNV from age-related macular degeneration underwent simultaneous spectral-domain optical coherence tomography and FA imaging. Eight patients had a newly diagnosed untreated CNV. Thirty-one patients had already been treated with anti-vascular endothelial growth factor agents. In 18 of these eyes, CNV lesions showed persistent leakage on FA. In 13 eyes, CNV lesions did not show leakage by FA. Subretinal CNV material boundaries visualized on spectral-domain optical coherence tomography B-scans were manually traced, and optical reflectivity was calculated using the mean grayscale value. To account for variable image brightness, the retinal pigment epithelial reflectivity was measured. The absolute difference between CNV material and retinal pigment epithelial reflectivity (ΔREF) from the three groups (newly diagnosed CNV, previously treated CNV showing FA leakage, and previously treated CNV not showing FA leakage) was compared. RESULTS: In untreated lesions, ΔREF was significantly higher compared with previously treated, but still leaky CNV (P < 0.0001). Lesions showing FA leakage had significantly higher ΔREF compared with those that did not display leakage (P < 0.0001). CONCLUSION: The displayed reflectivity of subretinal CNV material in spectral-domain optical coherence tomography appears to be an important parameter that can provide information regarding the FA leakage status.

Rhegmatogenous retinal detachment due to paravascular linear retinal breaks over patchy chorioretinal atrophy in pathologic myopia.

OBJECTIVE: To characterize posterior paravascular linear retinal breaks over areas of patchy chorioretinal atrophy as a cause of retinal detachment among patients with pathologic myopia. METHODS: In this retrospective case series, we evaluated 10 pathologically myopic eyes having rhegmatogenous retinal detachment associated with posterior paravascular linear retinal breaks. RESULTS: Ten eyes with posterior paravascular linear retinal breaks and retinal detachment were identified from January 1, 2008, to July 31, 2009. The retinal breaks were most frequently found along the inferotemporal vascular arcade, followed by the superotemporal arcade. The length of the breaks ranged from 0.25 to 1 disc diameter, and their distance from the optic disc ranged from 1 to 5 disc diameters. These paravascular linear retinal breaks have distinct clinical characteristics, including a strong propensity to occur over areas of patchy chorioretinal atrophy, a linear shape that is oriented parallel to the adjacent retinal vessels, and a tendency to result in progressive retinal detachment. Vitrectomy with gas tamponade was performed in all cases, and retinal reattachment was achieved in 9 cases by a single operation. CONCLUSIONS: Paravascular linear retinal breaks over areas of patchy chorioretinal atrophy represent a distinct clinical entity that can result in a special category of retinal detachment among patients with pathologic myopia. These breaks are apt to elude detection before surgery, and a careful search along the posterior vascular arcades during vitrectomy may help to detect these abnormalities. Pars plana vitrectomy with photocoagulation and intraocular tamponade may lead to a resolution of such rhegmatogenous retinal detachments.

Artifacts in automatic retinal segmentation using different optical coherence tomography instruments.

PURPOSE: The purpose of this study was to compare and evaluate artifact errors in automatic inner and outer retinal boundary detection produced by different time-domain and spectral-domain optical coherence tomography (OCT) instruments. METHODS: Normal and pathologic eyes were imaged by six different OCT devices. For each instrument, standard analysis protocols were used for macular thickness evaluation. Error frequencies, defined as the percentage of examinations affected by at least one error in retinal segmentation (EF-exam) and the percentage of total errors per total B-scans, were assessed for each instrument. In addition, inner versus outer retinal boundary delimitation and central (1,000 microm) versus noncentral location of errors were studied. RESULTS: The study population of the EF-exam for all instruments was 25.8%. The EF-exam of normal eyes was 6.9%, whereas in all pathologic eyes, it was 32.7% (P < 0.0001). The EF-exam was highest in eyes with macular holes, 83.3%, followed by epiretinal membrane with cystoid macular edema, 66.6%, and neovascular age-related macular degeneration, 50.3%. The different OCT instruments produced different EF-exam values (P < 0.0001). The Zeiss Stratus produced the highest percentage of total errors per total B-scans compared with the other OCT systems, and this was statistically significant for all devices (P < or = 0.005) except the Optovue RTvue-100 (P = 0.165). CONCLUSION: Spectral-domain OCT instruments reduce, but do not eliminate, errors in retinal segmentation. Moreover, accurate segmentation is lower in pathologic eyes compared with normal eyes for all instruments. The important differences in EF among the instruments studied are probably attributable to analysis algorithms used to set retinal inner and outer boundaries. Manual adjustments of retinal segmentations could reduce errors, but it will be important to evaluate interoperator variability.

Simultaneous presentation of Kimura disease and angiolymphoid hyperplasia with eosinophilia.

Kimura disease and angiolymphoid hyperplasia with eosinophilia are rare disease entities that can manifest as subcutaneous nodules, plaques, or papules of the head and neck, and less commonly the orbit and ocular adnexa. The merits of each entity have been debated with regard to whether they are truly individual or are separate points on a single spectrum. Current opinion favors the former. This clinicopathologic report of a patient who presented with a right lower eyelid mass with pathologic features consistent with Kimura disease and a left conjunctival mass with features consistent with angiolymphoid hyperplasia with eosinophilia poses a challenge to the notion that these are distinct conditions.

In vivo three-dimensional imaging of neovascular age-related macular degeneration using optical frequency domain imaging at 1050 nm.

PURPOSE: To assess the application of optical frequency domain imaging (OFDI) at 1050 nm for the detection of choroidal neovascularization (CNV) in age-related macular degeneration (AMD) and its response to treatment. Three patients presenting with blurred vision and exudative AMD were imaged before and after anti-VEGF treatment with ranibizumab. METHODS: The patients were imaged with OFDI, a swept-source-based, high-speed optical coherence tomography (OCT) system developed at the Wellman Center for Photomedicine. A center wavelength of 1050 nm was used that has been demonstrated to provide better imaging of the deeper structures of the retina below the RPE, such as the choroidal vasculature. Three-dimensional data sets were acquired in 2 to 4 seconds. RESULTS: En face images were compiled from cross-sectional OFDI data and correlated with color fundus photography (CF) and fluorescein angiograms (FAs). Cross-sectional images were coregistered with CF and FA to obtain depth-resolved information about CNV, CNV volume, retinal thickness, subretinal fluid volume and height of neurosensory detachment before and after treatment with ranibizumab. A band of reduced reflectivity below the RPE was identified in all three subjects that corresponded to areas of confirmed and suspected occult CNV on FA. After treatment, this band was reduced in volume in all patients. CONCLUSIONS: High-speed 3-D OFDI at 1050 nm is a promising technology for imaging the retina and choroid in neovascular AMD. The developed system at 1050 nm provides good contrast for occult (type 1) CNV and may have advantages compared with time domain and current state of the art spectral domain OCT systems (SD-OCT) at 850 nm.