Faculty Bibliography

PURPOSE/OBJECTIVE:To investigate a possible microvascular component of poppers maculopathy (PMP) using optical coherence tomography angiography (OCTA). METHODS:Twelve patients suffering from poppers maculopathy were included. Health records, optical coherence tomography (OCT), and OCTA data was gathered and compared to a healthy control group (HC). PMP lesion type was determined by manifestation in OCT. OCTA-based evaluation of retinal vascular plexus and choriocapillaris (CC) was executed. Vessel density (VD) and vessel length density (VLD) in superficial and deep capillary plexus (SCP, DCP), as well as flow deficits (FD), within the foveal avascular zone (FAZ) in CC were assessed. RESULTS:Median age of PMP patients was 40 (min 24; max 64) years, all male. Eleven patients presented with ellipsoid zone-type lesions; one patient showed a vitelliform-type lesion. No qualitative microvascular changes between PMP patients and HC were identified. Quantitative values for VD and VLD of SCP and DCP did not differ in between the two groups. The analysis of FDs in CC showed no deviation from PMP patients to HC. CONCLUSIONS:No vascular anomalies in qualitative and quantitative analysis in OCTA were detected in PMP patients. The constitution of the CC within FAZ of PMP patients does not differ from HC when assessed as FD.

AIMS/OBJECTIVE:(1) To assess the morphology and 3-dimensional (3D) displacements of the eye globe and optic nerve (ON) in adduction/abduction using MRI. (2) To assess differences between healthy emmetropic and highly myopic (HM) subjects. METHODS:MRI volumes of both eyes from 18 controls and 20 HM subjects in primary gaze, abduction and adduction (15°) were postprocessed. All ONs were manually segmented and fitted to a 3D curve to assess ON tortuosity. ON displacements were evaluated in four quasicoronal planes which were perpendicular to the ON in primary gaze and were 3 mm apart. RESULTS:Axial length was higher in the HM group (28.62±2.60 vs 22.84±0.89 mm; p<0.0001). Adjusted ON tortuosities (ie, ON tortuosities estimated before myopia onset) were lower in HM eyes (0.9063±0.0591) versus controls (1.0152±0.02981) in primary gaze, adduction (0.9023±0.05538 vs 1.0137±0.0299) and abduction (0.9100±0.0594 vs 1.0182±0.0316); p<0.0001 for all cases. In all eyes, ON displacements in adduction were significantly different from those in abduction in the naso-temporal direction (p<0.0001 in all planes) but not in the supero-inferior direction. ON displacements in the posterior segments of the ON were smaller in the HM group in both gaze directions and were larger in the anterior-most ON segment in adduction only. CONCLUSION/CONCLUSIONS:The adjusted tortuosity of the ON was significantly lower in HM eyes, suggesting that eyes destined towards HM exhibited higher ON traction forces during eye movements before the onset of myopia. Our ON metrics may be valuable to explore a potential link between eye movements and axial elongation.

PURPOSE/OBJECTIVE:To describe the multimodal imaging findings of extensive choroidal caverns within a choroidal nevus in an eye with pachychoroid spectrum disease. METHODS:A 69 year-old woman was referred with a known history of branch retinal vein occlusion (BRVO) in the right eye and choroidal nevus in the left eye. Fundus examination of both eyes (OU) revealed subretinal yellow deposits, suggestive of pachydrusen. Retinal venous collaterals were noted in the temporal macular of the right eye. A lightly pigmented choroidal lesion with nearly confluent overlying drusen and retinal pigment epithelial (RPE) alterations, consistent with chronic choroidal nevus, was noted in the macula of the left eye. RESULTS:Optical coherence tomography B-scans revealed thickened choroid (pachychoroid) with subfoveal choroidal thickness of 504 and 580 µm with large hyporeflective spaces suggestive of pachyvessels OU. In the region of the choroidal nevus, the choroidal vascular spaces appeared comparatively large and were classified as "caverns", measuring up to 480 µm in diameter. OCT angiography (OCTA) and indocyanine green angiography (ICGA) demonstrated absence of flow within the caverns. ICGA further illustrated choroidal vascular hyperpermeability with patchy hyperfluorescent areas OU. Widefield swept source OCT showed mild posterior scleral bowing, a feature occasionally documented with choroidal nevus, and highlighted greater hyporeflectivity and hypertransmission on OCT within the caverns compared to the non-cavernous choroidal vessels. CONCLUSION/CONCLUSIONS:Choroidal caverns can occur within choroidal nevus in the setting of pachychoroid disease.

BACKGROUND/AIMS/OBJECTIVE:To analyse the long-term anatomic and visual outcomes of patients with peripapillary pachychoroid syndrome (PPS), a recently described entity in the pachychoroid disease spectrum. METHODS:This study retrospectively included patients from several retina centres worldwide. Visual acuity (VA), retinal thickness and choroidal thickness at baseline, 6 months and final follow-up were assessed. Temporal trends in VA and anatomic characteristics were evaluated. Visual and anatomic outcomes in eyes that were observed versus those that were treated were analysed. RESULTS:Fifty-six eyes of 35 patients were included with mean follow-up of 27±17 months. Median VA was 20/36 at baseline and remained stable through follow-up (p=0.77). Retinal thickness significantly decreased subfoveally (p=0.012), 1.5 mm nasal to the fovea (p=0.002) and 3.0 mm nasal to the fovea (p=0.0035) corresponding to areas of increased thickening at baseline. Choroidal thickness significantly decreased subfoveally (p=0.0030) and 1.5 mm nasal to the fovea (p=0.0030). Forty-three eyes were treated with modalities including antivascular endothelial growth factor injection, photodynamic therapy, and others. VA remained stable in treated eyes over follow-up (p=0.67). An isolated peripapillary fluid pocket in the outer nuclear layer was characteristic of PPS. CONCLUSION/CONCLUSIONS:Patients with PPS experienced decreased retinal oedema and decreased choroidal thickening throughout the course of disease. While some patients experienced visual decline, the overall visual outcome was relatively favourable and independent of trends in retinal or choroidal thickening.

PURPOSE/OBJECTIVE:To report a case of acute exudative polymorphous vitelliform maculopathy (AEPVM) associated with primary Epstein-Barr virus (EBV) infection. METHODS:Multimodal imaging including color fundus photography, spectral domain optical coherence tomography (SD-OCT), blue light fundus autofluorescence (FAF), fluorescein angiography (FA) and indocyanine green angiography (ICGA). RESULTS:A 24-year-old otherwise healthy woman presented with an acute bilateral visual disturbance associated with cervical lymphadenopathy. SD-OCT showed bilateral foveal serous retinal detachment (SRD) with thickening of the ellipsoid zone (EZ) throughout the posterior pole corresponding to hyperautofluorescence on FAF, faint diffuse hyperfluorescence on FA without leakage, and mild late hyperfluorescence on ICGA. Systemic workup revealed an acute EBV infection. Within several weeks, multifocal SRDs developed in the macula and paramacula. The SRDs then became increasingly hyperautofluorescent with SD-OCT showing subretinal hyperreflective material. This vitelliform material then slowly resolved while the thickness of the surrounding EZ normalized. The FA and ICGA appeared normal at month 8. Visual acuity was 20/20 in both eyes at all times. No treatment was initiated. CONCLUSION/CONCLUSIONS:AEPVM may be associated with an acute EBV-infection. Acutely, multimodal imaging revealed findings consistent with RPE dysfunction and reduced photopigment density. Subsequent accumulation of vitelliform material gradually resolved over an 8 month follow up.

PURPOSE/OBJECTIVE:To describe the recovery timeline of spots and dots in multiple evanescent white dot syndrome. METHODS:Sequential multimodal retinal imaging including fundus autofluorescence and cross-sectional and en face optical coherence tomography was performed to track the development and resolution of spots and dots in a case of multiple evanescent white dot syndrome. RESULTS:En face optical coherence tomography showed that the spots are the result of ellipsoid zone loss and are hyperautofluorescent due to unmasking of the underlying retinal pigment epithelium autofluorescence. Conversely, the dots are hyperreflective with cross-sectional and en face optical coherence tomography and hyperautofluorescent, which we propose may be due to accumulation of degenerated photoreceptor material including fluorophores with autofluorescent capability such as precursors of A2E. The earlier resolution of the hyperautofluorescent spots allowed for later detection of the hyperautofluorescent dots. CONCLUSION/CONCLUSIONS:This case report illustrates the different recovery timelines of spots and dots in multiple evanescent white dot syndrome. Although both lesion types are hyperautofluorescent, the mechanism of autofluorescence is distinctive and may be explained by their contrasting pathoanatomy.

PURPOSE/OBJECTIVE:To report new multimodal imaging features that enhance our understanding of the inflammatory and ischemic nature of acute idiopathic maculopathy (AIM) and to correlate structural and functional changes due to a reversible initial choroidal ischemia over a clinical course of 3.5 months. METHODS:A 31-year-old man presented with acute central vision loss in his right eye due to coxsackievirus-associated AIM. Serial multimodal retinal imaging including confocal true color fundus photography, blue-light fundus autofluorescence (BAF), near-infrared reflectance (NIR), spectral domain optical coherence tomography and swept-source optical coherence tomography (SD-OCT + SS-OCT), and en face SS-OCT angiography (SS-OCTA) were performed over a 3.5-month follow-up. Eidon true color confocal scanner camera (Centervue, Padova, Italy) was used for color and BAF imaging. Near-infrared reflectance and SD-OCT images were obtained with the Heidelberg Spectralis OCT (HRA2 + OCT; Heidelberg Engineering, Heidelberg, Germany). For SS-OCT and en face SS-OCT and SS-OCTA images, the PLEX Elite 9,000 (Carl Zeiss Meditec, Inc, Dublin, CA) was used. Central alterations in choriocapillaris flow were analyzed with SS-OCTA using the University of Washington choriocapillaris (CC) flow deficit quantification algorithm available through the ARI Network. Flow deficit area and density values were analyzed and compared between the first and last examinations. Corresponding en face OCT imaging was used to distinguish true flow defects from artifacts secondary to shadowing. RESULTS:In the acute stage of AIM, a bacillary layer detachment appearing as a yellow-grayish foveal elevation surrounded by a hypopigmented parafoveal ring was evident in a Bull's eye configuration, corresponding to a hyperreflective ring upon NIR and a hyperautofluorescent ring with BAF. SD + SS-OCT showed mostly intraretinal fluid consistent with a bacillary layer detachment in conjunction with a thickened inner choroid. At presentation, SS-OCTA demonstrated a marked reduction of choriocapillaris flow signal. At 1 week, early resolution of retinal fluid was followed by restoration of the ellipsoid zone at 5 weeks, while restoration of the interdigitation zone and reduction in retinal pigment epithelium/Bruch membrane complex thickening occurred more slowly. Swept-source OCT angiography showed a gradual, but incomplete, recovery of inner choroidal flow signal at 3.5-month follow-up. CONCLUSION/CONCLUSIONS:Acutely, AIM may present with a photoreceptor splitting foveal bacillary layer detachment associated with a marked reduction in inner choroidal flow signal on SS-OCTA. Thereafter, restoration of the outer retinal layers and gradual normalization of choroidal flow signal appear to support the often-benign nature of the disease.

PURPOSE/OBJECTIVE:To describe clinical features including multimodal imaging in a rare case of presumed embolic cilioretinal artery occlusion with long-term follow-up examination. METHODS:A 61-year-old man presented after noticing an acute-onset central scotoma in the left eye during coronary arteriography. Fundus color photography, red-free imaging, near-infrared reflectance, spectral domain optical coherence tomography (OCT), fluorescein angiography, and swept-source OCT angiography were performed. RESULTS:Color and red-free fundus imaging both showed retinal whitening in the territory corresponding to a cilioretinal artery in the left eye. Consistent with an ischemic event, the near-infrared reflectance showed a hyporeflective area in the same location. Spectral domain OCT showed hyperreflectivity and generalized thickening of the inner nuclear layer, ganglion cell layer, and retinal nerve fiber layer. Seven years later, color, red-free, and near-infrared reflectance imaging appeared normal, but the spectral domain OCT showed thinning of all retinal layers in the affected area. Furthermore, swept-source OCT angiography demonstrated markedly reduced capillary flow signal in the superficial and deep capillary plexuses, corresponding to a persistent scotoma described by the patient. CONCLUSION/CONCLUSIONS:This case report illustrates hallmark features of a rare presumed embolic cilioretinal artery occlusion and its long-term manifestations with thorough historical and multimodal imaging data. When standard fundus imaging with color, red-free, and near-infrared reflectance appears normal years after a retinal vascular insult, OCT and OCT angiography can corroborate a patient's persistent visual deficit and provide clarity to the diagnosis.