Faculty Bibliography

PURPOSE/OBJECTIVE:To describe atypical fundus autofluorescence (FAF) patterns in geographic atrophy (GA) secondary to age-related macular degeneration (AMD) with associated age-related choroidal atrophy (ARCA). METHODS:Multimodal imaging of two cases using (pseudo-)color fundus photography, optical coherence tomography (OCT), fluorescein and indocyanine green angiography, and FAF employed with blue- and green excitation wavelengths on several devices (Spectralis, Heidelberg and (ultra-)widefield [UWF] FAF [California, Optos and EIDON, iCare]). RESULTS:Two female patients, with foveal-involving GA secondary to AMD, were assessed. All eyes demonstrated concurrent features indicative of ARCA on multimodal imaging including a paucity of choroidal vasculature, reduced choroidal pigmentation, macular pigmentary changes, peripapillary atrophy, and subretinal drusenoid deposits. Clinically, progression of GA with coalescence of lobular lesions was observed. Notably, UWF FAF with green-(California) and blue excitation wavelengths (California and EIDON) revealed atypical patterns characterized by isofluorescent FAF signals (indistinguishable from surrounding tissue) or hyperautofluorescent GA lesions. In these cases, blue excitation wavelengths were more effective than green light for delineating GA, owing to increased contrast from hypoautofluorescence related to macular pigment surrounding the lesion. CONCLUSION/CONCLUSIONS:In patients with GA and concomitant ARCA, atypical FAF patterns on UWF imaging complicate the accurate delineation and monitoring of GA. Atypical FAF patterns appear due to the properties of the confocal apertures and postprocessing features of UWF imaging that allow for the detection of scleral autofluorescence in patients with reduced choroidal vasculature, pigment and thickness. In patients with concomitant ARCA, multimodal imaging plays a crucial role in precisely identifying and tracking GA progression.

PURPOSE: To describe the multimodal imaging findings of transient subretinal deposits occurring in multiple evanescent white dot syndrome (MEWDS). METHODS: The multimodal imaging characteristics of transient subretinal deposits occurring in MEWDS were investigated with ultra-widefield color and fundus autofluorescence, cross-sectional and en-face optical coherence tomography (OCT), en face OCT-angiography, and quantitative autofluorescence. RESULTS: A 28-year-old woman presented with photopsia and temporal visual field loss in her right eye. Her best-corrected visual acuity was 20/20 in her right eye and 20/25 in her left eye. Funduscopic examination showed characteristic peripapillary hyperautofluorescent white dots of MEWDS corresponding to ellipsoid zone disruption on OCT. These lesions became confluent throughout the posterior fundus over the next 4 weeks. As the patient's symptoms were resolving, a second type of transient hyperautofluorescent lesion was noted which corresponded to hyperreflective subretinal deposits on cross-sectional and en face structural OCT. These subretinal deposits were most evident at 10-week follow-up and had nearly resolved at 14-week follow-up. Quantitative autofluorescence showed that, unlike the acute MEWDS lesions, the hyperautoflurescence of the subretinal deposits persisted after photobleaching. At multiple time points over 14 weeks of follow-up, OCT angiography showed no evidence of retinal or choroidal flow abnormalities. CONCLUSION: Transient subretinal deposits may develop during MEWDS in areas of previous diffuse outer retinal disruption. As these deposits remain hyperautoflurescent on quantitative autofluorescence after photobleaching, they may represent accumulations of debris originating from damaged photoreceptor outer segments.

PURPOSE: To evaluate the spectrum of macular chorioretinal lesions occurring in idiopathic multifocal choroiditis using optical coherence tomography angiography (OCTA) to evaluate those showing neovascular flow. METHODS: This was a descriptive, retrospective study of 18 eyes of 14 patients with multifocal choroiditis. Macular lesions were characterized as subretinal pigment epithelium, subretinal, or mixed and evaluated during active and presumed inactive states of multifocal choroiditis. Correlations between structural optical coherence tomography and OCTA were performed. In select cases, correlations between OCTA, fluorescein angiography, and fundus autofluorescence were evaluated. In 5 eyes, quantitative measurements of neovascular lesions were compared at baseline and following intravitreal anti-vascular endothelial growth factor therapy. RESULTS: Mean patient age was 48 years (SD: 13.8; 86% women). Optical coherence tomography angiography flow signatures consistent with neovascularization were identified in 83% of eyes, including in 0% of subretinal pigment epithelium, 91% of subretinal, and 100% of mixed lesions. Lesions that did not demonstrate definitive signs of fluorescein angiography leakage were frequently found to have neovascularization using OCTA. There was no change in quantitative measurements of neovascular lesions after anti-vascular endothelial growth factor therapy (all tested variables P > 0.05). CONCLUSION: Optical coherence tomography angiography may be a useful imaging modality for understanding the pathophysiology of multifocal choroiditis and monitoring its clinical course.

PURPOSE:: To compare the effect of 30-gauge versus 32-gauge needle size on postinjection reflux and immediate postinjection intraocular pressure (IOPimmed_post) spikes in eyes injected with anti-vascular endothelial growth factor agents. METHODS:: This was a prospective interventional case series of 65 eyes of 54 consecutive patients in a clinical practice setting who received intravitreal anti-vascular endothelial growth factor therapy. All eyes had preinjection IOP, IOPimmed_post, postinjection reflux, and axial lengths recorded. RESULTS:: There was a higher incidence of postinjection reflux in eyes injected with 30-gauge (53%) compared with those injected with 32-gauge (13%, P = 0.0007). Among 34 eyes injected with 30-gauge, 16 eyes without appreciable postinjection reflux had mean IOPimmed_post of 44.3 +/- 7.48 mmHg and mean IOPimmed_post elevation of 29.6 +/- 2.10 mmHg, which was significantly higher than the 18 eyes with reflux (mean IOPimmed_post of 18.8 +/- 7.15 mmHg and mean IOPimmed_post elevation of 4.5 +/- 1.74 mmHg, P < 0.0001). Among 31 eyes injected with 32-gauge, 27 eyes without appreciable postinjection reflux had mean IOPimmed_post of 44.4 +/- 10.82 mmHg and mean IOPimmed_post elevation of 29.5 +/- 1.99 mmHg, which was significantly higher than the 4 eyes with reflux (mean IOPimmed_post of 21.3 +/- 8.54 mmHg and mean IOPimmed_post elevation of 9.5 +/- 4.05 mmHg, P < 0.001). The differences in reflux and IOP between the two groups were unrelated to axial lengths (P = 0.451). CONCLUSION:: Eyes receiving injections with 32-gauge needles had a lower incidence of postinjection reflux and higher mean IOP immediately after injection.

BACKGROUND AND OBJECTIVE: To assess the cone photoreceptor mosaic in acute macular neuroretinopathy (AMN) using adaptive optics (AO) imaging. PATIENTS AND METHODS: Four patients with AMN were evaluated retrospectively by near-infrared reflectance (IR) confocal scanning laser ophthalmoscopy (SLO), spectral-domain optical coherence tomography (SD-OCT), and a flood-illuminated retinal AO camera. Microperimetry was performed in one patient. RESULTS: The cone photoreceptor density was decreased at the level of the AMN lesions. The cone mosaic disruption appeared heterogeneous and more widespread than the lesion detected in the IR-SLO and SD-OCT images. The areas of cone loss correlated with SD-OCT and microperimetry. After resolution of the AMN lesion on IR-SLO, there was incomplete recovery of the cone photoreceptor mosaic. CONCLUSION: Cone photoreceptor damage and reconstitution were documented in vivo at the cellular level in AMN using AO imaging. AO imaging appeared more sensitive than combined IR-SLO and SD-OCT to detect and follow photoreceptor damage in patients with AMN. [Ophthalmic Surg Lasers Imaging Retina. 2014;45:562-569.].

Purpose To assess the cone photoreceptor mosaic in acute macular neuroretinopathy(AMN) using adaptive optics(AO) imaging. Methods Four consecutive patients with AMN were evaluated by dilated funduscopic examination, near-infrared reffectance(IR), confocal scanning laser ophthalmoscopy(SLO), eye-tracked spectral-domain optical coherence tomography(SDOCT) and a flood-illuminated retinal AO camera. Correlations were made between IRSLO, SDOCT and AO images at baseline and follow-up in 3 patients. Microperimetry was performed and correlated with SDOCT and AO images in 1 patient. Results At presentation, the cone photoreceptor density was decreased at the level of the AMN lesions in AO images in all patients. The cone photoreceptor mosaic disruption was more widespread than the lesion detected by IRSLO and SDOCT. At complete resolution of the AMN lesion in IRSLO, there was incomplete recovery of cone photoreceptor mosaic in AO. In 2 cases, there was persistent cone damage in AO despite restoration of the ellipsoid zone integrity on SDOCT at last follow-up. The area of disruption in cone mosaic revealed characteristic appearance of RPE cells in AO and correlated well with both SDOCT and microperimetry findings. Conclusions Cone photoreceptor damage and reconstitution were documented in vivo at the cellular level in AMN using AO imaging. AO appeared more sensitive than combined IRSLO and SDOCT to detect and follow photoreceptor damage in AMN patients. There was some irreversible cone photoreceptor damage in the 4 patients evaluated

The authors present three cases of multiple evanescent white dot syndrome (MEWDS) with characteristic fundus autofluorescence (FAF) findings, including one patient without any visible white dots on funduscopic examination and another with many more hyperautofluorescent lesions than seen ophthalmoscopically. Additionally, the findings support an alternative mechanism for the hyperautofluorescent lesions in MEWDS, whereby photoreceptor loss causes unmasking of normal underlying retinal pigment epithelium autofluorescence. This hypothesis is demonstrated in two cases by optical coherence tomography showing clear ellipsoid zone attenuation with registration to hyperautofluorescent lesions. It is further supported in two cases by photoreceptor bleaching in successive FAF images captured in the same session leading to diminished autofluorescence intensity of the characteristic dots.

IMPORTANCE: With the advent of more sophisticated imaging systems, such as spectral domain optical coherence tomography (SD-OCT), disruption of the inner segment/outer segment (IS/OS) band, and thinning of the outer nuclear layer (ONL) have been identified in association with acute macular neuroretinopathy (AMN). OBJECTIVES: To characterize a new SD-OCT presentation of AMN as a paracentral acute middle maculopathy and to describe multimodal imaging findings that implicate an underlying pathogenesis related to retinal capillary ischemia. DESIGN, SETTING, AND PARTICIPANTS: Retrospective observational case series (January 1, 2012, to January 1, 2013) reviewing clinical and imaging data from 9 patients (11 eyes) with AMN at 6 tertiary referral centers. Lesions were classified as type 1 or 2 in relation to the SD-OCT location of the lesion above (type 1) or below (type 2) the outer plexiform layer (OPL) at 6 tertiary referral centers. RESULTS: Of the 9 patients, 5 were female and 4 were male (mean age, 47.6 years; range, 21-65 years). All patients presented with an acute paracentral scotoma and demonstrated a classic dark gray paracentral lesion with near-infrared imaging. Visual acuity ranged from 20/15 to 20/30. Six eyes (5 patients) had type 1 SD-OCT lesions, also referred to as paracentral acute middle maculopathy, and 5 eyes (4 patients) had type 2 SD-OCT lesions. Although type 1 lesions lead to inner nuclear layer (INL) thinning, type 2 lesions resulted in ONL thinning. Type 2 lesions were always associated with significant outer macular defects, including disruption of the inner segment/outer segment and outer segment/retinal pigment epithelium bands, whereas type 1 lesions spared the outer macula. CONCLUSIONS AND RELEVANCE: Paracentral acute middle maculopathy may represent a novel variant of AMN that affects the middle layers of the macula above the OPL as diagnosed with SD-OCT imaging. Two types of AMN lesions may be seen with SD-OCT occurring above and below the OPL. Type 1 refers to hyperreflective bands in the OPL/INL region with subsequent INL thinning. Type 2 is hyperreflective bands in the OPL/ONL region with subsequent ONL thinning. Type 2 lesions may be associated with concomitant defects of the inner segment/outer segment layer. We propose that each of these lesions may be explained by occlusion of either the superficial capillary plexus (type 1) or deep capillary plexus (type 2) located in the innermost and outermost portion of the INL, respectively, immediately adjacent to each corresponding lesion type.