Faculty Bibliography

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.

Purpose/UNASSIGNED:imaging findings at the level of ellipsoid zone (EZ) in two cases of occult macular dystrophy (OMD) with retinitis pigmentosa 1-like 1 (RP1L1) p.Arg45Trp mutation. Observations/UNASSIGNED:imaging at the EZ plane. Conclusions and importance/UNASSIGNED:imaging modality can also be used to monitor OMD progression.

PURPOSE/OBJECTIVE:To present multimodal imaging of multifocal chorioretinitis secondary to endogenous candida infection in a young adult. METHODS:A 49-year-old woman who presented for evaluation of bilateral endogenous candida chorioretinitis underwent complete ophthalmic examination, in addition to fundus photography (FP), enhanced depth imaging optical coherence tomography, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA) and optical coherence tomography angiography (OCTA). RESULTS:Multimodal imaging of both eyes of the patient affected by endogenous candida chorioretinitis was performed. FP showed multiple white chorioretinal lesions at the posterior pole, FAF showed dark dot at the posterior pole surrounded by hyperautofluorescence area, FA showed early hyperfluorescence round perifoveal lesion at the posterior pole and small hyperfluorescence dots under the inferior retinal vessels. Early ICGA showed hypofluorescence dots at the posterior pole. Late ICGA showed dark hypofluorescence dots at the posterior pole surrounded by faint hyperautofluorescent ring. OCTA showed dark areas corresponded to hypoperfusion areas seen with early ICGA. CONCLUSION/CONCLUSIONS:We reported multimodal imaging of an unusual occurrence of multifocal chorioretinitis due to immunosuppression. These findings suggested that the infection resulted from choroidal infiltration via the short posterior ciliary arteries with resultant breakthrough into the retina, rather than via the central retinal artery. By comparing findings on OCTA with data obtained from traditional systems, we are gaining essential information on the pathogenesis of endogenous candida chorioretinitis.

Background: Leber congenital amaurosis (LCA) due to RDH12 mutations typically manifests with severe vision loss and panretinal dystrophy. We sought to describe a case of LCA with choroidal neovascularization (CNV) in a 17-year-old patient. Materials and Methods: Case report of a 17-year old with LCA who presented with acute central vision loss of the right eye in the context of a chronic retinal dystrophy. Multimodal retinal imaging including spectral-domain optical coherence tomography and indocyanine green angiography revealed CNV. Results: A 17-year-old boy with previously diagnosed LCA/early-onset retinal dystrophy (EOSRD), with subsequently identified biallelic mutations in RDH12 was found to have type 2 CNV. Patient was treated with intravitreal ranibizumab and exhibited improvement on follow-up exam. Conclusions: Choroidal neovascularization may be a unique occurrence in RDH12-associated retinal dystrophy. Successful treatment of the neovascularization could be accomplished with intravitreal antivasogenic therapy.

Central serous chorioretinopathy (CSC) is a common cause of central vision loss, primarily affecting men 20-60 years of age. To date, no consensus has been reached regarding the classification of CSC, and a wide variety of interventions have been proposed, reflecting the controversy associated with treating this disease. The recent publication of appropriately powered randomised controlled trials such as the PLACE trial, as well as large retrospective, non-randomised treatment studies regarding the treatment of CSC suggest the feasibility of a more evidence-based approach when considering treatment options. The aim of this review is to provide a comprehensive overview of the current rationale and evidence with respect to the variety of interventions available for treating CSC, including pharmacology, laser treatment, and photodynamic therapy. In addition, we describe the complexity of CSC, the challenges associated with treating CSC, and currently ongoing studies. Many treatment strategies such as photodynamic therapy using verteporfin, oral mineralocorticoid antagonists, and micropulse laser treatment have been reported as being effective. Currently, however, the available evidence suggests that half-dose (or half-fluence) photodynamic therapy should be the treatment of choice in chronic CSC, whereas observation may be the preferred approach in acute CSC. Nevertheless, exceptions can be considered based upon patient-specific characteristics.

Purpose/UNASSIGNED:To explore patterns of disease progression in nonneovascular age-related macular degeneration (AMD) associated with hyperreflective crystalline deposits (HCDs) in the sub-retinal pigment epithelium-basal laminar space. Methods/UNASSIGNED:Retrospective review of medical records, multimodal imaging, and longitudinal eye-tracked near-infrared reflectance (NIR) and optical coherence tomography (OCT) spanning ≥2 years. NIR/OCT images were analyzed with ImageJ software to identify HCD morphology and location. Associated macular complications were reviewed from the time of HCD detection to the most recent follow-up, using NIR/OCT. Results/UNASSIGNED:Thirty-three eyes with HCDs from 33 patients (mean age: 72 ± 7.5 years) had 46.7 months (95% confidence limits: 33.7, 59.6) of serial eye-tracked NIR/OCT follow-up. Baseline best-corrected visual acuity (BCVA) was 0.44 logMAR (Snellen equivalent 20/55). At a mean of 11.3 months (3.1, 19.6) after HCD detection, 31/33 (93.9%) eyes had developed macular complications including de novo areas of complete retinal pigment epithelium and outer retinal atrophy (cRORA) in 21/33 (64%) eyes, enlargement of preexisting cRORA in 4/33 (12%) eyes, and incident macular neovascularization in 3/33 (9%) eyes. Movement and clearance of HCDs in 9/33 (27%) eyes was associated with enlargement of preexisting cRORA (r = 0.44, P = 0.02). BCVA at the last follow-up visit had decreased to 0.72 logMAR (20/105). Conclusions/UNASSIGNED:Eyes with nonneovascular AMD demonstrating HCDs are at risk for vision loss due to macular complications, particularly when movement and clearance of these structures appear on multimodal imaging. HCD reflectivity and dynamism may be amenable to automated recognition and analysis to assess cellular activity related to drusen end-stages.

OBJECTIVE:To explore the structural differences between X-linked retinoschisis (XLR) and stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) using swept-source optical coherence tomography angiography (SS-OCTA). METHODS:A case series of two patients, a 9-year-old male with XLR and a 58-year-old woman with SNIFR were imaged with swept-source optical coherence tomography angiography (SS-OCTA; PLEX Elite 900, Carl Zeiss Meditec, Inc, Dublin, CA). Automated segmentation was manually adjusted to include the areas of retinoschisis within en face flow and structural slabs. The flow data were binarized using ImageJ 1.51s (Wayne Rasband, National Institutes of Health, USA, http://imagej.nih.gov.ij ) and superimposed onto the structural slab. RESULTS:In the eye with XLR, OCTA flow data superimposed on the structural slab demonstrated flow signal within numerous bridging structures connecting the inner and outer plexiform layers containing the intermediate (ICP) and deep (DCP) capillary plexuses. In contrast, the same technique applied to the eye with SNIFR demonstrated an absence of flow signal in the cystic retinal spaces within Henle's fiber layer. CONCLUSIONS:The vascular pattern of bridging vessels between the ICP and DCP is closely related to the structural "retinoschisis" pattern of XLR and appears to be structurally different from that seen in SNIFR. Moreover, the connecting vessels appear to be highly represented and regularly distributed, thereby supporting a serial arrangement of the retinal capillary plexuses within the perifoveal macula.

PURPOSE/OBJECTIVE:To compare clinical, optical coherence tomography (OCT), and fundus autofluorescence (FAF) characteristics of peripapillary versus (vs.) macular variants of combined hamartoma of the retina and retinal pigment epithelium (combined hamartoma). DESIGN/METHODS:Retrospective observational, comparative case series METHODS:SETTING: Multicentre collaborative study STUDY POPULATION: 50 eyes with a clinical diagnosis of combined hamartoma OBSERVATIONAL ANALYSIS: A comparative analysis of color fundus photographs (CFPs), OCT and FAF was performed for peripapillary and macular variants of combined hamartoma. MAIN OUTCOME MEASURES/METHODS:Pigmentation and OCT features of macular and peripapillary combined hamartoma RESULTS: The review of imaging from 50 eyes of 49 patients diagnosed with combined hamartoma identified 18 (36%) peripapillary lesions, 27 (54%) macular lesions and 5 (10%) peripheral lesions. A comparative analysis of peripapillary vs. macular combined hamartoma identified differences in the following features: lesion pigmentation on CFPs corresponding to hypoautofluorescent FAF (88% vs. 0%, p<0.001) and OCT features of full thickness involvement (88% vs. 3%, p<0.001), preretinal fibrosis (27% vs. 81%, p<0.001), maxi peaks (5% vs. 88%, p<0.001), intraretinal cystoid spaces (72% vs. 40%, p<0.038), outer plexiform layer involvement (5% vs. 96%, p<0.001), ellipsoid zone disruption (83% vs. 3%, p<0.001), RPE disruption (77% vs. 3%, p<0.001) and choroidal neovascularization (16% vs. 0%, p=0.028). CONCLUSIONS:This comparative analysis identified a higher frequency of pigmentation with hypoautofluorescence, full thickness retinal involvement, intraretinal cystoid spaces, ellipsoid zone disruption, RPE disruption and choroidal neovascularization in peripapillary combined hamartoma. These findings suggest that lesions occurring near or at the optic nerve are associated with a more severe degree of pigmentary changes and retinal disruption than those located in the macula.