APMPPE and Placoid Variant Diseases Masquerading as Age-Related Macular Degeneration in the Elderly: A Case Series.

PURPOSE: To report eight cases of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) or persistent placoid maculopathy (PPM) initially masquerading as age-related macular degeneration (AMD) in elderly individuals. METHODS: APMPPE or PPM eyes in patients above age 55 years with macular RPE disruption including drusenoid lesions on macular examination and/or with multimodal imaging were included. At least one method of multimodal imaging including fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT) and OCT angiography (OCTA) was performed in all eyes for diagnosis and to monitor for macular neovascularization (MNV). RESULTS: Eight elderly male patients presented with vision loss and were all initially diagnosed with non-neovascular or neovascular AMD. With the aid of multimodal retinal imaging, a final diagnosis of either APMPPE or PPM was rendered. With FA and ICGA, choroidal hypoperfusion was detected in all but one eye. With OCT, the angular sign of Henle fiber layer hyperreflectivity (ASHH) was identified in >50% of eyes. With OCTA, inner choroidal flow deficits were detected in all eyes. MNV requiring anti-VEGF injection therapy complicated 3 of 8 cases. CONCLUSIONS: Both APMPPE and PPM may develop in elderly individuals and may masquerade as AMD on presentation. Multimodal imaging including FA, ICGA, and OCTA are important diagnostic modalities to assess for inner choroidal hypoperfusion to arrive at an accurate diagnosis, and to detect MNV which frequently complicates APMPPE and PPM. In these patients, serial anti-VEGF intravitreal injections are essential in treating MNV and in preventing significant vision loss.

Pathogenesis of Central Serous Chorioretinopathy and the Link Between Choroidal Hyperpermeability and Retinal Pigment Epithelium Pump Reversal.

PURPOSE: To describe 6 cases of acute central serous chorioretinopathy (CSCR) and the response to laser treatment, focusing on the underlying pathogenic mechanism. METHODS: Multimodal imaging from 6 eyes of 6 patients with acute and recurrent CSCR were reviewed, including fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT) at baseline and after laser therapy. RESULTS: In 3 of the 6 cases with acute CSCR, the hyporeflective lucency sign was identified with cross-sectional and en face OCT and co-localized with an intense active inkblot retinal pigment epithelium (RPE) leak on FA. The development of this sign was suggestive of active leakage into the subretinal space displacing overlying subretinal hyperreflective material (SHRM) and suggestive of a reversal of RPE pump function. All 6 cases were treated with focal laser to directly target the intense leakage points with remarkable resolution of the fluid due to destruction of the RPE cells mediating reversal of pump function. CONCLUSIONS: Unlike chronic CSCR in which degenerative changes of the RPE lead to oozing of fluid into the subretinal space, in acute forms of CSCR including bullous CSCR, there are focal leaks of the RPE that actively drive fluid into the subretinal space suggestive of RPE pump reversal. We propose that pachychoroid disease causes increased hydrostatic pressure and increased resistance to the RPE pump, thereby triggering a reversal in pump function. Understanding this concept can have therapeutic implications.

Retinal Ischemic Perivascular Lesions Are Associated With Myocardial Infarction in Patients With Coronary Artery Disease.

PURPOSE: To evaluate the association of retinal ischemic perivascular lesions (RIPLs) with myocardial infarction (MI) among patients diagnosed with coronary artery diseases (CAD). DESIGN: Retrospective cross-sectional study. METHODS: Consecutive patients (317 patients) with CAD who underwent macular spectral domain optical coherence tomography (SD-OCT) were captured. Patients with CAD who developed MI were compared to those without MI. SD-OCT were reviewed by 2 independent and masked graders for the presence of RIPLs. Medical records were reviewed. Multivariate logistic regression analysis was used to evaluate the relationship between RIPLs and MI including the following covariates age, gender, smoking status, hypertension, diabetes, dyslipidemia and body mass index. RESULTS: Of 317 patients with CAD for whom OCT scans were available to study, there were 54 (17%) with a history of MI. A higher prevalence of RIPLs was observed in the MI group compared to the non-MI group (59.3% vs 35.7%; P < .001). The presence of RIPLs was significantly associated with MI with an odds ratio of 3 (1.91-4.74; P < .001), after adjusting for age, gender, smoking status, hypertension, diabetes, dyslipidemia, and body mass index. CONCLUSIONS: The presence of RIPLs, detected with SD-OCT, is significantly associated with MI in patients with CAD. These findings underscore the potential clinical utility of incorporating RIPL evaluation in the medical management of CAD.

Relative yield of retinal imaging versus clinical exam in following neovascular exudative age related macular degeneration.

PURPOSE: To compare therapeutic decisions between 3 diagnostic protocols and to assess the need for in-person physical doctor-patient encounter in follow up and treatment of neovascular exudative age-related macular degeneration (AMD). METHODS: Analysis of 88 eyes of 88 unique patients with neovascular AMD who were routinely followed at our medical retina clinic. A retinal specialist reviewed all images in advance and wrote his decisions. He later attended an in-person encounters with all patients and documented his decisions. Masking was done by not exposing any identifying information to the specialist and by randomizing patient's images order before the in-person encounter. Therapeutic decisions regarding intravitreal injections intervals and agent selection were made based on three protocols: (1) optic coherence tomography (OCT); (2) OCT/Ultra-widefield (UWF) color image; (3) OCT/UWF/full clinical exam. Visual acuity (VA) was incorporated into all protocols. RESULTS: We found an agreement of 93% between those protocols regarding the intervals of injections, and of 100% regarding injection agent selection. When comparing OCT, OCT/UWF and OCT/UWF/clinical exam guided decision making, there were no discrepancies between OCT and OCT/UWF. There were 6 out of 88 discrepancies (7%) between OCT/UWF and OCT/UWF/clinical exam. Of those 6 discrepancies, all were regarding intervals (Bland-Altman bias = - 0.2386). All discrepancies between OCT/UWF and OCT/UWF/Clinical exam were due to patients' preferences, socioeconomic issues and fellow eye considerations, addressed during the face-to-face encounter with patients. Physical examination itself did not affect decision making. CONCLUSIONS: Neovascular exudative AMD follow up and treatment decisions can be guided by VA and OCT, with UWF adding important information regarding macula and peripheral retina, but rarely affecting decision making. However, decision making may also be driven by patients' preferences and other considerations that are being made only during the face-to-face visit and discussion. Thus, every approach supporting imaging only decision making, must take these factors into account.

En face OCT analysis of Bietti's crystalline dystrophy.

Objective: To describe the multimodal imaging features, including en face optical coherence tomography (OCT), of Bietti's crystalline dystrophy (BCD). Methods: Wide field fundus photography, autofluorescence (FAF) imaging, and cross sectional and en face OCT were performed in a case of BCD. The level of the crystals in the retina were analyzed. Results: A 42-year-old patient was referred for retinal evaluation with nyctalopia, photophobia and metamorphopsia. Retinal examination and wide field color fundus photography were remarkable for bilateral crystalline deposits in the posterior pole and midperipheral retina. Wide field FAF showed extensive nummular atrophy of the retinal pigment epithelium (RPE) in the macula and periphery. Spectral-domain (SD) OCT illustrated bilateral chorioretinal atrophy in the macula. En face SD OCT captured the hyperreflective crystals in various retinal layers, depending on the selected segmentation. The patient was diagnosed with BCD and genetic testing confirmed the diagnosis (CYP4V positive for two variants). Conclusion: In this case report, we describe the multimodal imaging features of Bietti's Crystalline Dystrophy. Wide field FAF illustrated diffuse nummular RPE atrophy in the posterior pole and periphery and en face OCT captured the hyperreflective crystals in different layers of the retina.

En Face Optical Coherence Tomography Illustrates the Trizonal Distribution of Drusen and Subretinal Drusenoid Deposits in the Macula.

PURPOSE: To analyze the topographic distribution of macular drusen and subretinal drusenoid deposits (SDDs) using single-capture en face spectral domain optical coherence tomography (SD-OCT) imaging. DESIGN: Retrospective case series. METHODS: Analysis of 33 eyes of 20 patients with evidence of SDDs. Structural en face OCT images were reconstructed using a 40-µm-thick slab positioned from 48 to 88 µm above the Bruch membrane. The Early Treatment of Diabetic Retinopathy Study (ETDRS) grid and a rod/cone density map were overlaid on the en face OCT images, and the distribution of different subtypes of SDDs and macular drusen were assessed. RESULTS: A total of 31 eyes (94%) showed a trizonal distribution pattern of drusen and SDDs. Whereas small to large drusen tended to aggregate in the central circle, dot SDDs predominated in the inner ring and the inner portion of the outer ring of the ETDRS grid and ribbon SDDs localized to the outer ring and outside the ETDRS grid. Of note, drusen colocalized to the region of greatest cone density, whereas ribbon SDDs colocalized to the area of greatest rod density. The dot SDDs mapped to the intermediate region with mixed rod and cone representation. CONCLUSION: Dot and ribbon subtypes of SDDs and macular drusen show a characteristic trizonal distribution. The locations of these lesions colocalize according to the different densities of the cones and rods in the retina and may reflect varying pathophysiological activities of these photoreceptor subtypes.

FROM DRUSEN TO TYPE 3 MACULAR NEOVASCULARIZATION.

PURPOSE: To investigate the imaging features preceding the occurrence of type 3 (T3) macular neovascularization (MNV) using tracked spectral-domain optical coherence tomography. METHOD: From a cohort of eyes with T3 MNV and ≥ 12 months of previously tracked spectral-domain optical coherence tomography, T3 lesions that developed above soft drusen were selected for optical coherence tomography analysis. Retinal imaging findings at the location where type T3 MNV occurred were analyzed at each follow-up until the onset of T3 MNV. The following optical coherence tomography parameters were assessed: drusen size (height and width), outer nuclear layer/Henle fiber layer thickness at the drusen apex, and the presence of intraretinal hyperreflective foci, retinal pigment epithelium disruption, incomplete retinal pigment epithelium and outer retina atrophy, and complete retinal pigment epithelium and outer retina atrophy. RESULTS: From a cohort of 31 eyes with T3 MNV, T3 lesions developed above soft drusen in 20 eyes (64.5%). Drusen showed progressive growth ( P < 0.001) associated with outer nuclear layer/Henle fiber ( P < 0.001) thinning before T3 MNV. The following optical coherence tomography features were identified preceding the occurrence of T3 MNV, typically at the apex of the drusenoid lesion: disruption of the external limiting membrane/ellipsoid zone and/or the retinal pigment epithelium, hyperreflective foci, and incomplete retinal pigment epithelium and outer retina atrophy/complete retinal pigment epithelium and outer retina atrophy. CONCLUSION: The results demonstrate specific anatomic alterations preceding the occurrence of T3 MNV that most commonly originates above soft drusen. Drusen growth, reduced outer nuclear layer/Henle fiber thickness, and retinal pigment epithelium atrophy at the drusen apex precede the development of T3 MNV. Identifying these optical coherence tomography features should warrant close monitoring for identification of T3 MNV, which can benefit from prompt intravitreal anti-vascular endothelial growth factor therapy.

Foveal Contour: Presence versus Complete Absence as a Prognostic Factor in Epiretinal Membrane Surgery.

INTRODUCTION: The study explains the presence versus complete absence of the foveal contour on optical coherence tomography (OCT) image as predictor of improvement in visual acuity (VA) following epiretinal membrane removal surgery. METHODS: We conducted a retrospective observational study in which 100 eyes that underwent vitrectomy for epiretinal membrane, with preoperative and postoperative VA and OCT, were analyzed. The study population was categorized into four groups based on the preoperational presence of a foveal contour and an intraocular lens implantation. RESULTS: The most significant improvement in VA was found among eyes lacking a foveal contour. Pseudo-phakic eyes demonstrated greater improvement than phakic. The smallest improvement was documented in pseudo-phakic eyes with a foveal contour. Phakic eyes that had a foveal contour showed deterioration in VA. Among eyes that lacked foveal contour, the fraction of eyes with improved VA was only slightly larger than among pseudo-phakic eyes during midterm follow-up and no difference was observed at long-term follow-up. Among eyes with foveal contour, the fraction with improved VA was significantly larger among pseudo-phakic eyes. This difference became more prominent over long-term follow-up. Regardless of the presence of foveal contour, the fraction of patients whose VA worsened was greater among those with phakic versus pseudo-phakic eyes, and this difference increased during long-term follow-up. No correlation was found between the central macular thickness and the VA. CONCLUSION: Complete lack of foveal contour is positively correlated with greater improvement in postoperative VA. The presence of an intraocular lens contributes to improvement in VA, especially among patients with foveal contour.

SLE presenting as demyelinative autoimmune visual loss.

A healthy 38-year-old woman developed sudden unilateral vision loss due to retrobulbar optic neuritis in the wake of varicella-zoster virus infection. She had no further central nervous system (CNS) lesions. Antinuclear antibodies (ANA) and anti-aquaporin 4 antibodies were found, consistent with neuromyelitis optica (NMO). Later, serial MRIs showed dynamic short-segment and long-segment myelitis lesions, ANA titre increased and additional autoantibodies were found including anti-dsDNA, anti-chromatin/nucleosome and antiphospholipid antibodies. In that setting, NMO can be regarded a rare presenting manifestation of systemic lupus erythematosus (SLE). The relevant literature is reviewed and the implications of NMO spectrum disorder demyelinating syndromes as the first manifestation of SLE (with or without antiphospholipid syndrome) (APS) or their later development (in a patient diagnosed with SLE) as part of the spectrum of neuropsychiatric SLE are analysed in view of recent research developments in the field.

Orthostatic hypotension is associated with nocturnal change in systolic blood pressure.

BACKGROUND: The circadian pattern of blood pressure (BP) has yet to be defined among individuals with orthostatic hypotension (OH). The objective of this study was to evaluate whether OH is associated with nocturnal change in systolic BP. METHODS: In a prospective study, we evaluated patients who were referred for 24-h ambulatory blood pressure monitoring (ABPM). All subjects underwent orthostatic BP testing before recording their respective 24-h ABPM. RESULTS: The study includes 185 subjects, 114 males, mean age 58 ± 18 years (range 19-89). Participants were classified, based on pattern of systolic BP changes at night, as dippers (greater than 10% decrease; n = 74), nondippers (0-10% decrease; n = 77), and reverse-dippers (increase; n = 34). Nineteen patients (10.3%) had OH. Almost all participants with OH (95%) had an abnormal diurnal BP pattern, and most of them (58%) were reverse-dippers, whereas only 56% of the participants without OH had an abnormal diurnal BP variation, and only 14% were reverse-dippers (P < 0.001). Systolic BP decreased with upright posture by 12 and 2 mm Hg in the reverse-dippers and the nondippers, respectively, and increased by 2 mm Hg in the dippers (P < 0.001). Postural changes in systolic BP were inversely related to the changes between day and night BP readings(r = -0.43; P < 0.01). In a multivariate linear regression analysis, orthostatic BP change, use of ≥2 antihypertensive drugs and female sex were related to nocturnal BP changes. CONCLUSIONS: The decrease in BP during upright posture may be a marker of nondipping or reverse-dipping pattern of diurnal BP.