Faculty Bibliography

PURPOSE/OBJECTIVE:To describe pre-exudative stage of exudative Perifoveal Vascular Anomalous Complex (ePVAC) referred to as non-exudative PVAC (nePVAC). DESIGN/METHODS:Retrospective non-comparative case series. METHODS:Patients diagnosed with nePVAC were identified at 4 retina referral centers worldwide. Multimodal retinal imaging including structural optical coherence tomography (OCT) and OCT-angiography (OCT-A) were performed at baseline and follow-up visits. RESULTS:Six eyes (6 patients, mean age 75±10years) were included. Unrelated chorioretinal diseases were diagnosed in the affected eyes in 5 of 6 cases. At the baseline, nePVAC is characterized by microvascular abnormalities featuring isolated, perifoveal, large intra-retinal aneurysm, surrounded by capillary rarefaction at OCT-A examination, without any sign of exudation with structural OCT, and without visual impairment. Four patients were followed for a mean of 21±14months. During the follow-up, 3 out of 4 eyes(75%) developed signs of exudation after a mean of 15±9months, associated with metamorphopsia and visual decline at the time of exudation. Best-corrected visual acuity(BCVA) decreased from 20/25 to 20/40 Snellen equivalent (p=0.035) and central macular thickness increased from 268±27 to 339±65μm (p=0.145). Three patients were treated with 2.3±0.6 intravitreal injections of anti-vascular endothelial growth factor without significant improvement of BCVA or macular edema. CONCLUSIONS:nePVAC may represent the sub-clinical pre-exudative stage of ePVAC, notable for an absence of exudation and/or visual impairment. nePVAC and ePVAC should be considered as part of the same spectrum, namely PVAC. Typically, nePVAC develops signs of exudation over time, causing metamorphopsia and visual decline and therefore these lesions warrant continued close monitoring with multimodal retinal imaging.

Retinal vascular tortuosity may occur in a wide range of ocular disorders. When retinal vascular tortuosity involves both arteries and veins, and presents unilaterally and without hemorrhage, a diagnosis of Wyburn Mason syndrome (WMS) should be considered due to the potential morbidity and mortality associated with cerebral involvement. Magnetic resonance imaging (MRI) and MRI angiography (MRA) are important tools for identifying cerebral arteriovenous malformations (AVMs), but these imaging modalities have limited spatial resolution to detect very small vascular lesions. Annular array contact ocular ultrasound is a new imaging modality capable of detecting small intraorbital AVMs. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:239-243.].

PURPOSE/OBJECTIVE:To describe novel spectral-domain (SD) and swept-source (SS) optical coherence tomography (OCT) linear and planar reflection artifacts produced by hyperreflective crystalline deposits (HCD). METHODS:Imaging from 10 eyes with HCD producing linear and planar artifacts on OCT was retrospectively analyzed. All eyes had SD-OCT (Spectralis HRA + OCT, Heidelberg Engineering, Germany) and SS-OCT angiography (PLEX Elite 9000, Carl Zeiss Meditec, Inc., Dublin, CA) acquired on the same day. The horizontal extent of planar artifacts and the corresponding HCD on B-scans was measured using a digital caliper. Artifact features from HCD in eyes with non-neovascular age-related macular degeneration (AMD) were analyzed and compared to those seen in two eyes with the "onion sign," an OCT signature previously shown to represent cholesterol crystals (CC) in the sub-retinal pigment epithelium-basal laminar space of eyes with neovascular AMD. A third eye with the "onion sign" was imaged with dense B-scan (DB)-OCTA. RESULTS:Ten eyes of ten patients (77.4 ± 8.7 years) with HCD were analyzed. On SS-OCTA, HCD produced linear artifacts of high signal intensity passing through the HCD and spanning the entire scan depth. On SD-OCT, HCD produced planar artifacts located anterior to both the retina and a hyporeflective space representing normal vitreous signal. The horizontal extent of the artifact did not differ significantly from the corresponding HCD on OCT B-scans (P = 0.62). The OCT artifacts produced by the "onion sign" appeared similar to those of HCD. The additional eye with neovascular AMD imaged with DB-OCTA was characterized by a single, vertical, linear false-flow signal crossing retinal layers. CONCLUSIONS:To the authors' knowledge, this is the first description of SD- and SS-OCT/OCTA artifacts corresponding to both HCD and the "onion sign." These artifacts are likely due to highly reflective CC previously shown on histology to correspond to both of these OCT signatures.

PURPOSE/OBJECTIVE:Cuticular drusen (CD) have been associated with manifestations of age-related macular degeneration such as atrophy and neovascularization in the macula. In this study, eyes with CD were followed and investigated for the estimated 5-year risk of progression to sequelae of age-related macular degeneration such as geographic atrophy (GA) and macular neovascularization (MNV). METHODS:A consecutive series of patients with CD were followed for the development of GA and MNV. Whenever possible, they were also studied retrospectively. The patients with CD were categorized into three phenotypic groups. Phenotype 1: eyes had concentrated, densely populated CD in the macular and paramacular area, Phenotype 2: eyes showed scattered CD in the posterior fundus, and Phenotype 3: involved eyes with CD mixed with large drusen (>200 µm). The 5-year incidence of progression was then estimated using a Kaplan-Meier estimator. RESULTS:A total of 63 eyes from 38 patients (35 women with a mean age at presentation of 58.9 ± 14.2 years) were studied and followed for a mean of 40 ± 18 months. Thirteen patients had single eyes with GA (84.5%; 11/13) or MNV (15.5%; 2/13) in one eye at presentation and were subsequently excluded. Geographic atrophy developed in 19.0% (12/63) of eyes and MNV in 4.8% (3/63) of eyes. The cumulative estimated 5-year risk of GA and MNV was 28.4% and 8.7%, respectively. The estimated 5-year incidence of MNV or GA was 12.6%, 50.0%, and 51.6% in Phenotype 1, Phenotype 2, and Phenotype 3, respectively (P = 0.0015, log-rank test). No difference in risk was found in the development of GA or MNV (P = 0.11) between the subgroup of patients presenting with GA or MNV in their fellow eye and those with both eyes included. CONCLUSION/CONCLUSIONS:When patients with CD are followed longitudinally, there was a significant risk of progression to GA or MNV for Phenotype 2 and Phenotype 3. Patients with CD are commonly first diagnosed in the fifth decade of life, and there is a female predominance. Clinicians should use multimodal imaging to detect and be aware of the risk of progression to manifestations of GA and MNV. These risks of GA and MNV suggest that patients with CD may be part of the overall spectrum of age-related macular degeneration.

PURPOSE/OBJECTIVE:To characterize structural and angiographic findings in macular telangiectasia Type 2 (MacTel 2) and examine associations with visual acuity. METHODS:MacTel 2 patients with complete ophthalmologic examination, including fundus photography, autofluorescence, spectral-domain optical coherence tomography, and projection-resolved optical coherence tomography angiography, were retrospectively evaluated. RESULTS:There were 43 eyes of 22 patients with a mean age 63.9 (±10.3) years. Six patients had diabetes. Twenty-one eyes (48.8%) had retinal-choroidal anastomoses (RCAs) without any evidence of neovascularization extending laterally in a plane above or below the retinal pigment epithelium. None of the eyes had hemorrhage, lipid, or signs of subretinal exudation. When present, an average of 55 (±33.7) individual RCAs were clustered primarily in temporal juxtafoveal region of involved eyes. Right-angle veins were seen in all 21 eyes with RCAs, and hyperpigmentation was present in 18 (P < 0.001 for both). A conical collection of hyperreflective material spanning from Bruch membrane past external limiting membrane of ≥200-μm basal diameter was found in 21 eyes and labeled outer retinal hyperreflective lesion. Retinal-choroidal anastomoses occurred in clusters, often within the outer retinal hyperreflective lesion. This lesion colocalized with focal thinning of the outer nuclear layer and was surrounded by a larger defect in the ellipsoid zone. The presence of diabetes (P = 0.015), outer retinal hyperreflective lesion (P = 0.006), RCA (P = 0.005), and ellipsoid zone defect extent (P < 0.001) were associated with decreased visual acuity. CONCLUSION/CONCLUSIONS:Retinal-choroidal anastomoses occur in eyes with MacTel 2 without signs of exudation. Retinal-choroidal anastomoses occur in numerous clusters particularly in the temporal juxtafoveal macula. Diabetes, ellipsoid zone defect extent, RCAs, and the outer retinal hyperreflective lesion predict poorer vision in MacTel 2.

PURPOSE/OBJECTIVE:To describe two cases of retinal detachment with hydration folds and discuss the possible cause of these outer retinal abnormalities. METHODS:The medical and imaging records of two patients with retinal detachment and hydration folds were examined. PATIENTS/METHODS:A 43-year-old myopic woman who developed a retinal detachment secondary to a macular hole and a 35-year-old man referred with a rhegmatogenous retinal detachment masquerading as an exudative detachment were each found to have retinal hydration folds. RESULTS:On near-infrared reflectance imaging, the hydration folds appeared similar to eddy currents, and these corresponded to curvilinear outer retinal plications on optical coherence tomography. The photoreceptor outer segments appeared thickened and elongated, and there was apparent lateral expansion of the outer retinal layers. CONCLUSION/CONCLUSIONS:Hydration folds are found in rhegmatogenous retinal detachment and demonstrate reproducible imaging characteristics on near-infrared imaging and optical coherence tomography. The cause for such outer retinal plications is currently unknown. We suspect that they form as a result of hydration of the glycosaminoglycans in the interphotoreceptor matrix, which lies between the photoreceptors. Additional studies are warranted to explore this pathophysiology.

PURPOSE/OBJECTIVE:We describe a healthy 37-year-old man with Bartonella henselae (B. henselae) neuroretinitis with concurrent central retinal vein occlusion and ischemic optic neuropathy resulting in optic atrophy and choroidal ischemia. METHODS:Case report. RESULTS:A 37-year-old man presented with unilateral decreased vision and a fundus examination consistent with neuroretinitis. Further imaging review supported a concurrent diagnosis of central retinal vein occlusion. Although initially negative, repeat serological testing for B. henselae infection was positive. Multimodal imaging displayed severe outer retinal disruption, ischemic optic neuropathy, and choroidal ischemia. The patient demonstrated near complete resolution of fundus findings and restoration of outer retinal architecture. Residual findings included optic disk pallor and ischemic choroidopathy. DISCUSSION/CONCLUSIONS:B. henselae neuroretinitis may be associated with concurrent retinal vascular occlusive disease and ischemic optic neuropathy. Central retinal vein occlusion and choroidal ischemia leading to optic nerve atrophy are additional sequelae further expanding the clinical spectrum of this entity.

BACKGROUND AND OBJECTIVE/OBJECTIVE:To describe the prevalence and anatomic correlates for hyperautofluorescence related to outer retinal disruption in eyes with multifocal choroiditis (MFC). PATIENTS AND METHODS/METHODS:Retrospective review of MFC patients. RESULTS:Fifty-nine eyes from 37 patients were analyzed. Multimodal imaging was utilized to identify nine eyes (15.2%) of six patients with either transient (Group 1) or persistent (Group 2) regions of hyperautofluorescence associated with ellipsoid zone (EZ) disruption over intact retinal pigment epithelium (RPE). Group 1 included four eyes (6.8%) of three patients in which the hyperautofluorescence and EZ loss resolved within a few months (range: 28 days to 125 days) and had intact overlying outer nuclear (ONL) and outer plexiform layers (OPL) (mean follow-up: 1.3 years). Group 2 included five eyes (8.5%) of three patients with regions of permanent EZ disruption associated with absent or reduced ONL and OPL (mean follow-up: 4.6 years). CONCLUSIONS:Hyperautofluorescence correlating with EZ disruption over intact RPE is a rare occurrence in MFC. Evaluating outer retinal integrity by optical coherence tomography may help identify eyes with potential for EZ restoration, which may have implications regarding treatment strategies. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:675-683.].

PURPOSE/OBJECTIVE:To evaluate the vascular structure within combined hamartoma of retina and retinal pigment epithelium (CHRRPE) lesions using OCT angiography (OCTA). DESIGN/METHODS:Multicenter, retrospective, observational analysis, PARTICIPANTS: Twenty eyes of patients diagnosed with CHRRPE. METHODS:Retrospective analysis of color fundus photographs, OCT, and OCTA of 20 eyes with CHRRPE. Morphologic characteristics of CHRRPE and the OCT features were correlated with the density of the filigree vascular pattern and with the published histopathologic findings of CHRRPE lesions. MAIN OUTCOME MEASURE/METHODS:Density of flow signals, that is, the filigree vascular pattern seen on OCTA in the deep capillary plexus, graded as high (>20), intermediate (10-20), or low (<10). RESULTS:Of 20 lesions, 11 were peripapillary, 8 were macular, and 1 was equatorial in location. A high density of filigree vascular pattern was observed in most peripapillary CHRRPE lesions, which also showed full-thickness retinal involvement (8/10). A low density of filigree pattern was seen in macular lesions, which showed partial-thickness retinal involvement and preretinal fibrosis (5/6). CONCLUSIONS:A filigree vascular pattern on OCTA is seen in CHRRPE lesions. High density of this pattern is noted in CHRRPE lesions with a peripapillary location, full-thickness retinal disorganization, and minimal preretinal fibrosis. These findings correlate well with published histopathologic findings of CHRRPE lesions both in terms of topographic and morphologic features. OCT angiography provides a promising method for further study of these lesions.