MYOPIC PRESENTATION OF CENTRAL SEROUS CHORIORETINOPATHY.

PURPOSE: To increase insight into the myopic presentation of central serous chorioretinopathy (CSC) by comparing a large group of myopic patients with CSC with reference groups with only one of the diagnoses. METHODS: Myopic patients with CSC (spherical equivalent ≤-3D, n = 46), emmetropic patients with CSC (spherical equivalent -0.5 to 0.5 D, n = 83), and myopic, non-CSC patients (n = 50) were included in this multicenter cross-sectional study. Disease characteristics and imaging parameters, such as subfoveal choroidal thickness and indocyanine green angiography patterns, were compared between cases and reference groups. RESULTS: In myopic patients with CSC, median subfoveal choroidal thickness (286 µm [IQR 226-372 µm]) was significantly thicker than subfoveal choroidal thickness in myopic, non-CSC patients (200 µm [IQR 152-228 µm], P < 0.001) but thinner than emmetropic patients with CSC (452 µm [IQR 342-538 µm], P < 0.001). They also had pachyvessels in 70% of the eyes comparable with emmetropic CSC (76%, P = 0.70). Choroidal hyperpermeability was frequently present on indocyanine green angiography in both myopic and emmetropic CSC eyes. Need for treatment, treatment success, and recurrence rate were not significantly different between CSC groups. CONCLUSION: Myopic CSC presents with similar imaging and clinical characteristics as emmetropic CSC, apart from their thinner choroids. Keeping in mind the structural changes of myopia, other imaging characteristics could aid the diagnostic process.

Long-term Visual Outcomes and Causes of Vision Loss in Chronic Central Serous Chorioretinopathy.

PURPOSE: To evaluate the long-term visual outcomes and causes of vision loss in chronic central serous chorioretinopathy (CSC). DESIGN: Retrospective, longitudinal study. PARTICIPANTS: A total of 133 participants (217 eyes) with chronic CSC. METHODS: A retrospective review of clinical and multimodal imaging data of patients with chronic CSC managed by 3 of the authors between May 1977 and March 2018. Multimodal imaging comprised color photography, fluorescein angiography, indocyanine green angiography, fundus autofluorescence (FAF), and OCT. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA) at the final visit; change in BCVA between first visit and 1-, 5-, and 10-year follow-up visits; and causes of vision loss at final visit. RESULTS: Data from 6228 individual clinic visits were analyzed. Mean age of patients at the first visit was 60.7 years, and mean period of follow-up from first to last visit was 11.3 years. The cohort included 101 male patients (75.9%). At the final visit, 106 patients (79.7%) maintained driving-standard vision with BCVA of 20/40 or better in at least 1 eye, and 17 patients (12.8%) were legally blind with BCVA of 20/200 or worse in both eyes. Mean BCVA at first visit was not significantly different from mean BCVA at 1- or 5-year follow-up visits (both P ≥ 0.65) but was significantly better than the mean BCVA at the 10-year follow-up visit (P = 0.04). Seventy-nine percent of eyes with 20/40 or better vision at the first visit maintained the same level of vision at the 10-year follow-up visit. Ninety-two percent of eyes with 20/200 or worse vision at the first visit maintained the same level of vision at the 10-year follow-up visit. Cystoid macular degeneration, choroidal neovascularization (CNV), outer retinal disruption on OCT, and FAF changes were associated with poorer vision at the final visit (all P ≤ 0.001). Multivariable analysis revealed that greater age at first visit was associated with greater BCVA change at the 10-year follow-up visit (P = 0.001). CONCLUSIONS: Chronic CSC can be a sight-threatening disease leading to legal blindness. Age at presentation and outer retinal changes on multimodal imaging were associated with long-term BCVA changes and may be predictors of long-term visual outcomes.

Repair of a Tractional Retinal Detachment in the Setting of an Idiopathic Vasoproliferative Tumor.

Vasoproliferative tumors (VPT) are benign retinal lesions that may cause epiretinal membrane proliferation and tractional retinal detachments (TRD). We describe a case of a 71-year-old woman who presented with a macula involving TRD in the setting of a VPT. Given the limited number of publications on the management of these cases, we aim to articulate some principles we believe may be helpful in planning a surgical approach that maximizes postoperative anatomic and functional outcomes. We hope that our video provides useful guidance in preparing the vitreoretinal surgeon for managing this uncommon entity. [Ophthalmic Surg Lasers Imaging Retina 2023;54:485-488.].

Hyperpigmented epiretinal membrane in macular telangiectasia type 2: imaging characteristics and correlation with transretinal pigment migration.

OBJECTIVES: To explore the features of black hyperpigmentation in macular telangiectasia (MacTel) type 2 and correlate these findings with the characteristics of hyperpigmented epiretinal membranes (ERMs) using multimodal imaging. METHODS: A case series of three patients with MacTel type 2 and hyperpigmented ERMs imaged with colour fundus photography, fluorescein angiography (FA), spectral-domain optical coherence tomography (OCT) and swept-source OCT angiography. Registration of different types of imaging was done using ImageJ v1.53f51 (National Institutes of Health, USA). RESULTS: Three female patients with late-stage MacTel type 2 presented with unilateral hyperpigmented ERMs in the absence of peripheral retinal breaks. In one patient, an extensive ERM occurred along with a full-thickness macular hole (FTMH); in 2 patients, smaller ERMs were seen adjacent to superficial retinal veins. Serial imaging demonstrated that transretinal pigment migration preceded epiretinal proliferation of the hyperpigmented ERM towards superficial retinal veins. CONCLUSION: Hyperpigmented ERMs may occur in the late phases of MacTel type 2 following a FTMH or transretinal migration of pigmented cells to the retinal surface. Once on the retinal surface, black pigment cells seem to proliferate centripetally toward superficial retinal veins.

OCT En Face Analysis of the Posterior Vitreous Reveals Topographic Relationships among Premacular Bursa, Prevascular Fissures, and Cisterns.

PURPOSE: To characterize the topographic relationships among vitreous structures, including the premacular bursa, prevascular vitreous fissures, cisterns, and lacunae, in healthy participants using en face and cross-sectional swept-source (SS) OCT. DESIGN: Prospective, comparative study. PARTICIPANTS: Sixty eyes of 60 healthy participants (age range, 4-35 years). Eyes of individuals younger than 20 years (n = 29) were compared with eyes of individuals 20 years of age or older (n = 31). METHODS: From each study eye, 12 × 12-mm SS OCT volume scans comprising 1024 × 1024 A-scans centered at the fovea were acquired. MAIN OUTCOME MEASURES: En face and cross-sectional data were analyzed to characterize topographic relationships between hyperreflective spaces anterior to the vitreoretinal interface. RESULTS: Prevascular vitreous fissures are an almost universal feature of human eyes. Cisterns became more prevalent over the course of the first 20 years (r = 0.49; P = 0.002). In 97% of eyes in individuals older than 20 years, en face and cross-sectional SS OCT showed the premacular bursa and prepapillary gap merge at a distance superior to the optic nerve and then follow a superonasal course anteriorly. However, only 69% of individuals younger than 20 years demonstrated such a connection (P = 0.01). A close topographic relationship of vitreous fissures and cisterns to the underlying vasculature of the posterior pole was visible on en face projections. En face imaging readily distinguished these spaces. Degenerative, eyewall-parallel fissure planes and their course were described for the first time in a 3-dimensional manner. The fissure planes were rare in younger eyes (12%) and significantly more common in older eyes (42%; P < 0.001). CONCLUSIONS: En face SS OCT demonstrated that (1) premacular bursa and Cloquet's canal are not connected in younger patients, but are connected in older patients; (2) prevascular vitreous fissures overly the retinal vessels; and (3) cisterns are continuous with prevascular fissures.

A Two Forceps Technique For Approaching Tractional Diabetic Membranes.

BACKGROUND AND OBJECTIVE: To describe a two-forceps bimanual approach for complex diabetic detachments. PATIENTS AND METHODS: Curved scissors were used to create a zone of decreased resistance within a thick fibrovascular membrane in a previously vitrectomized patient. Using two forceps, the linear defect was propagated into the periphery in a direction tangential to the retina. The tissue was repeatedly regrasped at the leading edge of the propagating tear, allowing for progressive separation of the hyaloid from the retinal surface. RESULTS: Separation of the diabetic membrane and hyaloid from the retinal surface in a previously vitrectomized patient. CONCLUSION: The authors describe a two-forceps approach for dense fibrovascular proliferation in the setting of persistently attached cortical vitreous that may be employed in cases of severe diabetic retinopathy with traction and fibrovascular membranes, even after previous vitrectomy. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e176-e178.].

The Nature and Frequency of Outer Retinal Disruption in Idiopathic Multifocal Choroiditis Associated With Persistent Fundus Hyperautofluorescence.

BACKGROUND AND OBJECTIVE: To describe the prevalence and anatomic correlates for hyperautofluorescence related to outer retinal disruption in eyes with multifocal choroiditis (MFC). PATIENTS AND 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.].

A proposed mechanism influencing structural patterns in X-linked retinoschisis and stellate nonhereditary idiopathic foveomacular retinoschisis.

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.

Long-Term Multimodal Imaging of Solar Retinopathy.

This is a rare, multimodal imaging report spanning a decade of monitoring in a patient with chronic solar retinopathy showing the natural course of the disease. Spectral-domain optical coherence tomography (SD-OCT) showed mild widening of subfoveal loss of ellipsoid and interdigitation zones bilaterally, progressive retinal pigment epithelial thinning in the right eye, and hyperplasia in the left eye. Structural en face OCT showed subfoveal tissue loss bilaterally. There was no leakage on fluorescein angiography and OCT angiography (OCTA), and dense B-scan OCTA images were unremarkable. Microperimetry revealed bilateral decreased central sensitivity and eccentric fixation in the left eye. Vision remained stable throughout. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:388-392.].

Early hydroxychloroquine retinopathy: optical coherence tomography abnormalities preceding Humphrey visual field defects.

BACKGROUND/AIMS: Hydroxychloroquine (HCQ) retinopathy may result in severe and irreversible vision loss, emphasising the importance of screening and early detection. The purpose of this study is to report the novel finding of early optical coherence tomography (OCT) abnormalities due to HCQ toxicity that may develop in the setting of normal Humphrey visual field (HVF) testing. METHODS: Data from patients with chronic HCQ exposure was obtained from seven tertiary care retina centres. Ten patients with HCQ-associated OCT abnormalities and normal HVF testing were identified. Detailed analysis of the OCT findings and ancillary tests including colour fundus photography, fundus autofluorescence, multifocal electroretinography and microperimetry was performed in these patients. RESULTS: Seventeen eyes from 10 patients illustrated abnormalities with OCT and normal HVF testing. These OCT alterations included (1) attenuation of the parafoveal ellipsoid zone and (2) loss of a clear continuous interdigitation zone. Several eyes progressed to advanced parafoveal outer retinal disruption and/or paracentral visual field defects. CONCLUSION: Patients with high risk HCQ exposure and normal HVF testing may develop subtle but characteristic OCT abnormalities. This novel finding indicates that, in some cases of early HCQ toxicity, structural alterations may precede functional impairment. It is therefore important to employ a screening approach that includes OCT to assess for these early findings. Ancillary testing should be considered in cases with suspicious OCT changes and normal HVFs.

Cuticular drusen associated with aneurysmal type 1 neovascularization (polypoidal choroidal vasculopathy).

BACKGROUND: Aneurysmal type 1 neovascularization (AT1) is a term recently introduced to better describe the aneurysmal dilatation that may arise from neovascular lesions, more commonly known as polypoidal choroidal vasculopathy. The proposed term, AT1, includes an expanded clinical spectrum of aneurysmal (polypoidal) lesions observed in both different ethnicities and associated with varied clinical phenotypes. CASE PRESENTATION: A 61-year-old woman of European descent was referred for a new, asymptomatic retinal hemorrhage found on routine examination. Ophthalmoscopy revealed cuticular drusen in both eyes best appreciated on fundus autofluorescence, and a hemorrhagic retinal pigment epithelium detachment above the superior arcade in the right eye. In the fellow eye, a reddish appearing pigment epithelial detachment was noted nasal to the optic nerve. Indocyanine green angiography showed findings of AT1 in both eyes. Optical coherence tomography angiography showed intrinsic flow signal within the aneurysmal lesions. CONCLUSIONS: Eyes with cuticular drusen may develop AT1 which, to our knowledge, has not been described. This is an important observation because the documented coexistence of AT1 in the setting of a variant of age-related macular degeneration lends supports to this new understanding of AT1 as a growth pattern of neovascular tissue proliferating between the RPE and Bruch membrane, rather than as a distinct disease entity.

UNILATERAL BEST DISEASE: A CASE REPORT.

PURPOSE: To describe the multimodal imaging findings observed unilaterally in a patient with Best disease due to a p.G15D mutation in the BEST1 gene. METHODS: The clinical history of a 62-year-old female patient with unilateral Best disease was reviewed. Retinal findings were documented by clinical examination and multimodal imaging. RESULTS: Posterior segment examination of the patient's right eye demonstrated retinal pigment epithelium hypopigmentation and clumping in the central macula beneath a chronic shallow serous retinal detachment (SRD), confirmed by optical coherence tomography. Fluorescein angiography showed central staining with no evidence of focal leakage or choroidal neovascularization, and correlated with the hypoautofluorescence seen on fundus autofluorescence. There was no evidence of choroidal hyperpermeability on indocyanine green angiography, nor was there any neovascularization detected on optical coherence tomography-angiography. The left eye appeared normal with all imaging modalities. CONCLUSION: Best disease is an autosomal dominant disease that is generally bilateral. We present a case of a unilateral Best disease with serous retinal detachment in a patient with a p.G15D mutation in BEST1. Best disease should be considered in the differential diagnosis of serous retinal detachment and may masquerade as central serous chorioretinopathy.

Quantification of Oxygen Consumption in Retina Ex Vivo Demonstrates Limited Reserve Capacity of Photoreceptor Mitochondria.

PURPOSE: Cell death in neurodegeneration occurs at the convergence of diverse metabolic pathways. In the retina, a common underlying mechanism involves mitochondrial dysfunction since photoreceptor homeostasis and survival are highly susceptible to altered aerobic energy metabolism. We sought to develop an assay to directly measure oxygen consumption in intact retina with the goal of identifying alterations in respiration during photoreceptor dysfunction and degeneration. METHODS: Circular punches of freshly isolated mouse retina, adjacent to the optic nerve head, were used in the microplate-based Seahorse Extracellular Flux Analyzer to measure oxygen consumption. Tissue integrity was evaluated by propidium iodide staining and live imaging. Different substrates were tested for mitochondrial respiration. Basal and maximal respiration were expressed as oxygen consumption rate (OCR) and respectively measured in Ames' medium before and after the addition of mitochondrial uncoupler, BAM15. RESULTS: We show that glucose is an essential substrate for retinal mitochondria. At baseline, mitochondria respiration in the intact wild-type retina was close to maximal, with limited reserve capacity. Similar OCR and limited mitochondrial reserve capacity was also observed in cone-only Nrl-/- retina. However, the retina of Pde6brd1/rd1, Cep290rd16/rd16 and Rpgrip1-/- mice, all with dysfunctional or no photoreceptors, had reduced OCR and higher mitochondrial reserve capacity. CONCLUSIONS: We have optimized a method to directly measure oxygen consumption in acutely isolated, ex vivo mouse retina and demonstrate that photoreceptors have low mitochondrial reserve capacity. Our data provide a plausible explanation for the high vulnerability of photoreceptors to altered energy homeostasis caused by mutations or metabolic challenges.