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Retinitis pigmentosa (RP) is a monogenic disease characterized by irreversible degeneration of the retina. PRPF31, the second most common causative gene of autosomal dominant RP, frequently harbors copy number variations (CNVs), but the underlying mechanism is unclear. In this study, we summarized the phenotypic and genotypic characteristics of 18 RP families (F01-F18) with variants in PRPF31. The prevalence of PRPF31 variants in our cohort of Chinese RP families was 1.7% (18/1024). Seventeen different variants in PRPF31 were detected, including eight novel variants. Notably, four novel CNVs encompassing PRPF31, with a proportion of 22.2% (4/18), were validated to harbor gross deletions involving Alu/Alu-mediated rearrangements (AAMRs) in the same orientation. Among a total of 12 CNVs of PRPF31 with breakpoints mapped on nucleotide-resolution, 10 variants (83.3%) were presumably mediated by Alu elements. Furthermore, we described the correlation between the genotypes and phenotypes in PRPF31-related RP. Our findings expand the mutational spectrum of the PRPF31 gene and provide strong evidence that Alu elements of PRPF31 probably contribute to the susceptibility to genomic rearrangement in this locus.

To compare a treat-and-extend (TAE) strategy with a fixed dosing regimen of intravitreal conbercept (IVC) for the management of treatment-naïve polypoidal choroidal vasculopathy (PCV) patients.249 patients with treatment-naïve PCV were randomized 1:1 to fixed dosing regimen with injections every 12 weeks (3 + Q12W) group or treat-and-extend regimen(3 + TAE) group. Patients received 3 monthly intravitreal injections of 0.5 mg conbercept as loading dose in both groups. The 3 + Q12W patients were monitored monthly and received mandated injections every 12 weeks; the 3 + TAE patients were monitored and treated monthly until the resolution of exudative disease activity; the interval between visits was then individualized according to study protocol. Visual and anatomical outcomes were compared between the two groups.At 48 weeks, there was no significant difference between the 3 + Q12W group and 3 + TAE group in mean BCVA improvement (p = 0.421), mean changes in central retinal thickness (CRT) (p = 0.818), maximum retinal thickness (MRT) (p = 0.448), pigment epithelial detachment (PED) height (p = 0.221), PED volume (p = 0.076), branching vascular network (BVN) area (p = 0.615), polypoidal lesion number (p = 0.701), polypoidal lesion area (p = 0.424), rates of patients who avoided vision loss of ≥15 ETDRS letters (p = 0.397) or complete polypoidal lesion regression rate (43.8% vs. 41.8%, p = 0.814). The 3 + Q12W group had more decreased retinal haemorrhage area (p = 0.014) and fewer mean numbers of injections comparing with 3 + TAE group (6.6 vs. 9.4, p 0.001). Mean maximum extension interval between injections after loading injections was 9.6 ± 2.0 weeks for 3 + TAE group, with 27.8% of patients achieving an extension interval of 12 weeks and 61.1% patients 8 weeks or more.Both 3 + Q12W and 3 + TAE regimens of IVC could result in improvement in visual and anatomical outcomes in PCV patients.

AIM: To quantitatively analyze the retinal intermediate and deep capillary plexus (ICP and DCP) in patients with retinal deep vascular complex ischemia (RDVCI), using 3D projection artifacts removal (3D PAR) optical coherence tomography angiography (OCTA). METHODS: RDVCI patients and gender- and age-matched healthy controls were assessed and underwent OCTA examinations. The parafoveal vessel density (PFVD) of retinal deep vascular complex (DVC), ICP, and DCP were analyzed, and the percentage of reduction (PR) of PFVD was calculated. RESULTS: Twenty-four eyes in 22 RDVCI patients (20 in acute phase and 4 in chronic phase) and 24 eyes of 22 healthy subjects were enrolled as the control group. Significant reduction of PFVD in DVC, ICP, and DCP was observed in comparison with the controls (DVC: acute: 43.59%±6.58% vs 49.92%±5.49%, PR=12.69%; chronic: 43.50%±3.33% vs 51.20%±3.80%, PR=15.04%. ICP: acute: 40.28%±7.91% vs 46.97%±7.14%, PR=14.23%; chronic: 41.48%±2.87% vs 46.43%±3.29%, PR=10.66%. DCP: acute: 45.44%±8.27% vs 51.51%±9.97%, PR=11.79%; chronic: 37.78%±3.48% vs 51.73%±5.17%, PR=26.97%; all P

PurposeTo compare the efficacy of initial vs. delayed photodynamic therapy (PDT) in combination with intravitreal injection of conbercept (IVC) for polypoidal choroidal vasculopathy (PCV).DesignMulticenter, randomized, non-inferiority clinical trial.SubjectsNaïve PCV patients.MethodsPatients were randomized 1:1 into two groups: initial PDT with IVC and delayed PDT with IVC. At baseline, patients in the initial combination group were treated with PDT and IVC within 1 week, while patients in the delayed combination group were treated with IVC alone. PDT and IVC was given PRN during the follow-up in each group.Main Outcome MeasuresNon-inferiority of delayed PDT with IVC to initial PDT with IVC for mean change in best-corrected visual acuity from baseline to month 12 (95% CI of the difference entirely above −5 letters).ResultsEighty-six patients were enrolled, with 43 in each group. At month 12, the change of BCVA in initial combination group was equivalent to that in the delayed combination group, with gains of 6.42 ± 1.89 and 7.49 ± 2.14 (mean ± standard error) letters, respectively [delayed group minus initial group: 1.07 letters; 95% confidence interval (CI): −4.62 to 6.76; Pnon−inferiority = 0.0198]. The rates of complete polyp regression were 66.67 and 45.83% in the initial and delayed combination groups, respectively. The difference was not statistically significant (P = 0.386). The mean reductions of CRT were 204.77 ± 28.79 and 84.14 ± 30.62 μm in each group respectively. The difference was statistically significant (P = 0.005). In addition, the mean injection numbers were 3.47 ± 2.39 and 4.91 ± 2.65 in each group respectively. The differences were statistically significant (P = 0.010).ConclusionsThere was effective in both groups in patients with PCV. The initial combination group showed a more efficient decrease in CRT and polyp regression, along with fewer injections. However, the delayed combination group was non-inferior compared with the initial combination group in terms of the improvement of BCVA.Trial Registrationhttps://ClinicalTrials.gov, Identifier: NCT02821520.

PURPOSE To investigate the prevalence and associated factors of fundus tessellation in highly myopic children and adolescents. METHODS A total of 513 high myopes (spherical equivalent [SE] ≤ -5.0 D, 4-19 years of age) without any advanced pathological myopic lesions were enrolled. Fundus photographs and choroidal thickness (ChT) data were collected by SS-OCT. A novel grading approach was adopted to classify fundus tessellation into four categories on colour fundus photography, referring to the location of tessellation divided by an Early Treatment Diabetic Retinopathy Study grid centred on the fovea, through which closer to the fovea represents higher grades of fundus tessellation. Peripapillary atrophy (PPA) area and ovality index were also measured. RESULTS Among the participants, with a mean age of 13.47 ± 3.13 years and mean SE of - 8.34 ± 1.91 D, there were 29 (5.7%), 95 (18.5%), 233 (45.4%) and 156 (30.4%) participants with grade 0 to grade 3 fundus tessellation, respectively. The ChT in both the macular and peripapillary area was negatively correlated with the fundus tessellation grade (R = -0.763 and -0.537, respectively, all p

Background To determine the clinical characteristics and molecular genetic background responsible for USH2A mutations associated with nonsyndromic retinitis pigmentosa (RP) in five Chinese families, a retrospective cross‐sectional study was performed. Methods Data on detailed history and comprehensive ophthalmological examinations were extracted from medical charts. Genomic DNA was sequenced by whole‐exome sequencing. The pathogenicity predictions were evaluated by in silico analysis. The structural modeling of the wide‐type and mutant USH2A proteins was displayed based on the I‐Tasser software. Results The ultra‐wide‐field fundus imaging showed a distinctive pattern of hyperautofluorescence in the parafoveal ring with macular sparing. Ten USH2A variants were detected, including seven missense mutations, two splicing mutations, and one insertion mutation. Six of these variants have already been reported, and the remaining four were novel. Of the de novo mutations, the p.C931Y and p.G4489S mutations were predicted to be deleterious or probably damaging; the p.M4853V mutation was predicted to be neutral or benign; and the IVS22+3A>G mutation was a splicing mutation that could influence mRNA splicing and affect the formation of the hairpin structure of the USH2A protein. Conclusions Our data further confirm that USH2A protein plays a pivotal role in the maintenance of photoreceptors and expand the spectrum of USH2A mutations that are associated with nonsyndromic RP in Chinese patients., This study identified novel mutations of the USH2A gene using a whole‐exome sequencing approach in nonsyndromic RP patients. RP patients with USH2A mutations have a distinctive pattern in ultra‐wide‐field fundus autofluorescence.

Purpose The present study analyzed the appearances of optical coherence tomography angiography (OCTA) in patients with central serous chorioretinopathy (CSC) based on fluorescein angiography (FA) and indocyanine green angiography (ICGA). Method In the current case series, 54 eyes of 50 patients diagnosed as CSC were evaluated retrospectively. OCTA, FA, and ICGA were performed on each patient. Two trained observers examined the OCTA images independently to confirm and compare the choriocapillary appearance with that on FA/ICGA. Also, the leakage of vessels on FA, perfusion of choroidal blood flow on ICGA, blood flow density, and vascular morphology on OCTA, as well as, the effect of serous retinal detachment (SRD) on imaging were observed. Furthermore, the image findings of contralateral eyes were included. Results 47/54 eyes (corresponding to 43 patients in 50 patients) were finally diagnosed with CSC that presented a leakage on FA and dilated vessels on ICGA, and the corresponding areas could be recognized on OCTA. However, in some of the cases (15 eyes, 31.9%), a portion of the leakage lesion on FA did not overlap completely with that on OCTA. On the OCTA B-scan, six eyes did not show a choriocapillary flow signal under subretinal fluid (SRF) with a median SRD height of 485 µm, despite the dilated vessels on ICGA. Approximately, 21 contralateral eyes without SRD and leakage presented dilated vessels on ICGA; however, only 13 eyes could be recognized on OCTA. In addition, seven eyes presented CSC on FA/ICGA but manifested explicit abnormal vascularization beneath the retinal pigment epithelium (RPE) on OCTA. Conclusion FA/ICGA remains the gold standard for the diagnosis of CSC and cannot be completely replaced by OCTA. However, in some cases displaying hot-spots CNV, OCTA can contribute toward a definite diagnosis. The SRD height may exert a shielding effect on the choriocapillary flow signals on OCTA. Lasers Surg. Med. 50:987-993, 2018. © 2018 Wiley Periodicals, Inc.

Objective The present study aimed to investigate the characteristics of macular telangiectasia type 1 (Mac tel type 1) using spectral-domain optical coherence tomography angiography (OCTA) and compare them with the characteristics of mild diabetic macular edema (DME), to provide a new objective method for quick clinical diagnosis and treatment. Methods A retrospective comparative analysis of 9 Mac tel type 1, 15 DME, and 15 normal eyes was performed using fluorescein angiography, spectral-domain optical coherence tomography, and OCTA. The morphological changes, retinal vessel density, and nonperfused areas were evaluated using split-spectrum amplitude-decorrelation angiography algorithm. Results OCTA revealed obvious saccular capillary telangiectasia and loss of parafoveal vascular density of Mac tel type 1. However, a number of line segment hyperreflective signals around the macula and more distinct nonperfusion in DME were observed. The quantitative foveal avascular zone mean area in Mac tel type 1 was larger than that in the normal eyes (0.40 ± 0.06 mm2 vs. 0.88 ± 0.19 mm2, P 0.05). Conclusion Morphological differences between Mac tel 1 and DME can be observed on OCTA. Superficial vascular density and non-perfusion area may serve as a quantitative method to identify them. Lasers Surg. Med. 49:225–232, 2017. © 2017 Wiley Periodicals, Inc.

Letter to the Editor

AIM To estimate the efficacy of oral spironolactone in patients with chronic central serous chorioretinopathy (CSC). METHODS This was a pilot study consisting of patients who were diagnosed with chronic central serous chorioretinopathy for at least a 6-month duration. This study included 75 eyes of 74 patients who were treated with spironolactone (20 mg orally, twice daily) for 8 weeks. The best corrected visual acuity (BCVA), subretinal fluid (SRF), central macular thickness (CMT) and subfoveal choroidal thickness (SFCT) were each measured at baseline, 4 weeks and 8 weeks. RESULTS In our study, 75 eyes of 74 patients (49 men and 25 women) were included. The mean BCVA was 0.39 ± 0.25 at baseline, 0.29 ± 0.19 at 4 weeks and 0.23 ± 0.19 at 8 weeks. The BCVA at 4 weeks and 8 weeks was significantly improved compared to the baseline BCVA (p = 0.000). The mean SRF and the mean CMT decreased significantly at 4 weeks and 8 weeks (p = 0.000). The mean SFCT decreased slightly at 4 weeks and 8 weeks but without significance compared to the baseline (p = 0.654). CONCLUSION Spironolactone can competitively bind to mineralocorticoid receptors (MRs), reduce glucocorticoid binding or reduce endogenous activation of MR. It is possible to improve the visual acuity and safety of patients with chronic CSC by reducing the high permeability of the choroid membrane and by promoting the absorption of the subretinal fluid.

To assess the treatment response to mineralocorticoid antagonists in a pilot study of patients diagnosed with central serous chorioretinopathy using multimodal imaging.This retrospective observational case series included 23 eyes of 14 patients with central serous chorioretinopathy treated by a single physician (L.A.Y.) with either spironolactone, eplerenone, or both consecutively over a 12-month period. Choroidal thickness, central macular thickness, and best-corrected visual acuity were measured and compared with baseline values. Twelve eyes of 11 patients demonstrated subretinal fluid before or during the initiated treatment course. Subretinal fluid was measured and compared with baseline values in this subgroup.In all eyes (n = 23), best-corrected visual acuity improved at 12 months of treatment; however, central macular thickness and choroidal thickness showed no improvement. In the subgroup with subretinal fluid (n = 12), subretinal fluid was significantly decreased at 6 months and 12 months of treatment; however, central macular thickness, choroidal thickness, and best-corrected visual acuity showed no significant change.Mineralocorticoid antagonists may improve best-corrected visual acuity and decrease subretinal fluid in patients with central serous chorioretinopathy, but do not affect the choroidal or macular thickness. This pilot study demonstrates that mineralocorticoid receptor antagonists may be effective in treating central serous chorioretinopathy but warrants consideration for future research within a randomized clinical trial.

Purpose: The purpose of this study was to use optical coherence tomography angiography (OCTA) to compare the morphology characteristics of type 1 and 2 choroidal neovascularization (CNV) in neovascular age-related macular degeneration (nAMD) patients. Materials and Methods: This is a retrospective, observational study of 51 eyes with nAMD using OCTA from July 2016 through March 2017. Results: In total, 51 eyes of 50 patients were included in the analysis. According to the anatomical classification based on OCT, 27 eyes (53%) were diagnosed with type 1 CNV, and 24 eyes (47%) were type 2 CNV. Type 2 CNV was characterized by smaller flow area, smaller greatest vascular caliber (GVC), smaller greatest linear dimension (GLD), and shorter duration of disease. The duration of disease only correlated with GVC (p = 0.026) in multivariate linear regression results. Meanwhile, GLD correlated with GVC and flow area whereas GVC was not associated with flow area. Conclusion: This study demonstrated that type 2 CNV was characterized by shorter duration of disease, smaller GVC, smaller GLD, and smaller flow area compared with type 1 CNV. Additionally, we showed that the duration of the disease correlated with GVC of the CNV.

The present study analyzed the appearances of optical coherence tomography angiography (OCTA) in patients with central serous chorioretinopathy (CSC) based on fluorescein angiography (FA) and indocyanine green angiography (ICGA).In the current case series, 54 eyes of 50 patients diagnosed as CSC were evaluated retrospectively. OCTA, FA, and ICGA were performed on each patient. Two trained observers examined the OCTA images independently to confirm and compare the choriocapillary appearance with that on FA/ICGA. Also, the leakage of vessels on FA, perfusion of choroidal blood flow on ICGA, blood flow density, and vascular morphology on OCTA, as well as, the effect of serous retinal detachment (SRD) on imaging were observed. Furthermore, the image findings of contralateral eyes were included.47/54 eyes (corresponding to 43 patients in 50 patients) were finally diagnosed with CSC that presented a leakage on FA and dilated vessels on ICGA, and the corresponding areas could be recognized on OCTA. However, in some of the cases (15 eyes, 31.9%), a portion of the leakage lesion on FA did not overlap completely with that on OCTA. On the OCTA B-scan, six eyes did not show a choriocapillary flow signal under subretinal fluid (SRF) with a median SRD height of 485 µm, despite the dilated vessels on ICGA. Approximately, 21 contralateral eyes without SRD and leakage presented dilated vessels on ICGA; however, only 13 eyes could be recognized on OCTA. In addition, seven eyes presented CSC on FA/ICGA but manifested explicit abnormal vascularization beneath the retinal pigment epithelium (RPE) on OCTA.FA/ICGA remains the gold standard for the diagnosis of CSC and cannot be completely replaced by OCTA. However, in some cases displaying hot-spots CNV, OCTA can contribute toward a definite diagnosis. The SRD height may exert a shielding effect on the choriocapillary flow signals on OCTA. Lasers Surg. Med. 50:987-993, 2018. © 2018 Wiley Periodicals, Inc.

Worldwide, polypoidal choroidal vasculopathy (PCV) is a common vision-threatening exudative maculopathy, and pigment epithelium detachment (PED) is an important clinical characteristic. Thus, precise and efficient PED segmentation is necessary for PCV clinical diagnosis and treatment. We propose a dual-stage learning framework via deep neural networks (DNN) for automated PED segmentation in PCV patients to avoid issues associated with manual PED segmentation (subjectivity, manual segmentation errors, and high time consumption).The optical coherence tomography scans of fifty patients were quantitatively evaluated with different algorithms and clinicians. Dual-stage DNN outperformed existing PED segmentation methods for all segmentation accuracy parameters, including true positive volume fraction (85.74 ± 8.69%), dice similarity coefficient (85.69 ± 8.08%), positive predictive value (86.02 ± 8.99%) and false positive volume fraction (0.38 ± 0.18%). Dual-stage DNN achieves accurate PED quantitative information, works with multiple types of PEDs and agrees well with manual delineation, suggesting that it is a potential automated assistant for PCV management.

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.

Purposes:To evaluate the multimodal imaging findings in retinal deep capillary ischemia (DCI).Methods:This was a retrospective review of 5 eyes of 4 patients with sudden onset of paracentral scotomas caused by DCI. Multimodal imaging techniques, including color and red-free photographs, near-infrare

Purpose To describe an outbreak of intraocular inflammation caused by endotoxin-contaminated counterfeit bevacizumab in China. Design Retrospective, observational case series. Participants Patients undergoing intravitreal injection at a public hospital in September 2010. Methods The medical records and microbiology results of patients who presented with intraocular inflammation after injection with intravitreal counterfeit bevacizumab were reviewed. Main Outcome Measures The incidence of intraocular inflammation, results of pathogen cultures, and clinical features of inflammation. Results A total of 116 patients (70 men and 46 women) were injected from 3 vials of counterfeit bevacizumab. Intraocular inflammation developed in 80 patients. The estimated median incubation period was 12 hours (range, 2–24 hours), and the median duration of symptoms was 6 days (range, 3–22 days). All patients were treated initially with topical corticosteroid and antibiotics. Vitreous tap and intravitreal injection were performed on 43 patients. Twenty-one patients with hypopyon and significant vitreous inflammation underwent vitrectomy. Microscopic evaluations and microbiologic cultures of all ocular specimens were negative for bacterial and fungal contamination. The presence of endotoxin in specimens was confirmed by laboratory testing. We refer to this new clinical syndrome as "endotoxin-induced ocular toxic syndrome" (EOTS). The inflammation regressed rapidly after treatment, and 63 patients (78.8%) recovered their pre-injection vision. Conclusions This study implicates endotoxin as the cause of intraocular inflammation after the intravitreal injection of counterfeit bevacizumab. The EOTS appeared clinically distinct from typical infectious endophthalmitis. Financial Disclosure(s) Proprietary or commercial disclosure may be found after the references.

Purpose: To demonstrate the detection of choroidal neovascularization (CNV) in clinically diagnosed central serous chorioretinopathy (CSC) by optical coherence tomographic angiography (OCTA). Methods: Seventy-five eyes of 70 patients were included. OCTA was performed on each patient. Two trained readers evaluated the images independently to confirm the diagnosis and identify CNV at the level of the outer retina. Results: All studied eyes presented characteristics consistent with CSC based on eye examination and traditional imaging technologies, including fundus fluorescein angiography, which showed no evidence of CNV. However, OCTA revealed definite abnormal vascularization at the level of the outer retina in 8 eyes (10.7%) of 8 patients (11.4%). Conclusion: This study suggests that in some cases of CSC, OCTA could be an alternative option to detect CNV that is undetectable by other conventional imaging techniques.

Purpose.To describe the morphological characteristics and efficacy of OCTA in detecting CNV in nAMD. We retrospectively reviewed 53 patients (86 eyes) with suspected CNV secondary to wet AMD. All the patients underwent a multimodal assessment for CNV. Two independent readers calculated the sensitivity and specificity of OCTA in detecting CNV compared with FA. A qualitative analysis of OCTA was also performed to describe the morphological appearance of CNV. Among 86 eyes of 53 patients, 52 eyes were diagnosed as having CNV based on the FA imaging analysis. According to FA, CNV was classified as classic in 28 eyes, predominantly classic in 6 eyes, minimally classic in 9 eyes, and occult in 9 eyes. In 56 eyes, CNV was visualized on OCTA and corresponding OCT B-scans. In total, 46.4% (26/56) had well-circumscribed vessels, and 53.6% (30/56) showed poorly circumscribed vessels. There were 11 false positives and 7 false negatives using OCTA. The specificity of OCTA for the detection of CNV was 67.6%, with sensitivity of 86.5%. OCTA may help in the noninvasive diagnosis of CNV and may provide a method for monitoring the evolution of CNV.

Purpose To describe optical coherence tomography angiography (OCTA) findings in multifocal choroiditis (MFC) with active lesions and to characterize the concordance between the OCTA and other traditional imaging modalities. Design Reliability and validity analysis. Methods Patients with suspected choroidal neovascularization (CNV) or acute inflammatory lesions associated with MFC were assessed in this study. All participants underwent preliminary traditional multimodal imaging including color fundus photography, fundus autofluorescence, near-infrared reflectance imaging, spectral-domain optical coherence tomography, and fluorescence angiography (FA). The participants were prospectively recruited to perform OCTA. OCTA findings of active lesions were compared with other traditional imaging results. Vascular flow signal representing CNV was identified, and a quantitative analysis of CNV size was performed on OCTA. Results Fifty-two eyes of 26 MFC patients (14 were bilaterally affected and 12 were unilaterally affected) were included. Among the 23 active CNV cases, 20 were confirmed on OCTA while the other 3 were invalid owing to severe motion artifacts. OCTA of CNV showed a well-circumscribed vascular network (mean flow area, 0.271 mm 2 [± 0.144 mm 2 ]). Among the 34 inflammatory lesions in 13 eyes, 32 showed no blood flow in the outer retina on OCTA while the other 2 showed blood flow signal. Conclusions OCTA has a predominant advantage in differentiating CNV from inflammatory lesions. It also allows the visualization of detailed vascular structure of CNV and the function of flow area measurement realizes the quantitative analysis of CNV. Hence, it could be an alternative option for CNV identification and may better guide treatment.

Objective. To observe the fellow eye in patients undergoing surgery on one eye for treating myopic traction maculopathy.Methods. 99 fellow eyes of consecutive patients who underwent unilateral surgery to treat MTM were retrospectively evaluated. All patients underwent thorough ophthalmologic examinations, including age, gender, duration of follow-up, refraction, axial length, intraocular pressure, lens status, presence/absence of a staphyloma, and best-corrected visual acuity (BCVA). Fundus photographs and SD-OCT images were obtained. When feasible, MP-1 microperimetry was performed to evaluate macular sensitivity and fixation stability.Results. At an average follow-up time of 24.7 months, 7% fellow eyes exhibited partial or complete MTM resolution, 68% stabilized, and 25% exhibited progression of MTM. Of the 38 eyes with “normal” macular structure on initial examination, 11% exhibited disease progression. The difference in progression rates in Groups 2, 3, and 4 was statistically significant. Refraction, axial length, the frequency of a posterior staphyloma, chorioretinal atrophy, initial BCVA, final BCVA, and retinal sensitivity all differed significantly among Groups 1–4.Conclusions. Long axial length, chorioretinal atrophy, a posterior staphyloma, and anterior traction contribute to MTM development. Patients with high myopia and unilateral MTM require regular OCT monitoring of the fellow eye to assess progression to myopic pre-MTM. For cases exhibiting one or more potential risk factors, early surgical intervention may maximize the visual outcomes.

To evaluate the morphology and functional recovery of the retina after treatment of idiopathic choroidal neovascularization using intravitreal injections of bevacizumab in young adults.For this interventional case series, 20 eyes of 19 patients with idiopathic choroidal neovascularization were treated with multiple intravitreal injections of bevacizumab. Changes in best-corrected visual acuity before the treatment and at follow-up visits were recorded. Structural changes were evaluated using optical coherence tomography and functional changes were assessed using microperimetry.Twenty eyes were followed for 12 months after their first injection. The eyes underwent an average of 3.95 injections. All eyes had a stable or an improved vision. The mean logarithm of the minimum angle of resolution best-corrected visual acuity improved from 0.43 to 0.06 (Wilcoxon signed-ranks test, P0.005). Improvement in macular function was detected as early as 1 month after the treatment and lasted for at least 6 months. Microperimetry demonstrated that mean retinal sensitivities within the central 10° field (10.29 ± 5.12 dB) at baseline improved to 13.98 ± 3.96 dB at the last visit. Dense scotomas were found in 13 of the 20 eyes at baseline and 5 of the 20 eyes at the last visit. Stable fixation (6 of 20 at baseline) was found in 17 of the 20 eyes at the last visit. Twelve of the 14 eyes with either a predominantly eccentric or poor central fixation at baseline established central fixation at 12 months. No serious local or systemic complications were encountered.In young adults with idiopathic choroidal neovascularization, an improvement in visual acuity and macular function was detected after intravitreal injections of bevacizumab.

PURPOSE To represent and evaluate the findings of bilateral perifoveal macular ischemia in a patient with biopsy-proven, multiorgan involved sarcoidosis. METHODS Case report. A 55-year-old man, with a medical history of pulmonary disease, experienced reduced vision bilaterally. General ocular examination, fundus color photographs, fluorescence angiography, and high-resolution spectral domain optical coherence tomography were performed in both eyes. RESULTS The patient's visual acuity was 20/200 in the right eye and 20/30 in the left eye with normal intraocular pressure and anterior segment. Fundus examination showed symmetric changes with the loss of transparency in macular and small intraretinal hemorrhages in both eyes. Fluorescence angiography demonstrated markedly enlarged avascular zone while optical coherence tomography revealed marked cystic change in the macula bilaterally. A series of blood test was conducted without a specific diagnosis. A lung biopsy confirmed the diagnosis of pulmonary sarcoidosis, and a magnetic resonance imaging of his brain revealed neurosarcoidosis. CONCLUSION Bilateral perifoveal ischemia, formerly one of the three idiopathic macular telangiectasia diseases, is rare and usually suggests a systemic etiology. It is not unexpected that sarcoidosis, a multisystem, chronic inflammatory disorder, may affect retinal vessels and be associated with this peculiar form of ischemia and edema.

Purpose To describe multimodal imaging findings in a patient with foveal red spot syndrome. Methods We report a case of a 57-year-old man with foveal red spot syndrome. Multimodal imaging techniques, including fundus color and red-free photographs, fluorescence angiography (Topcon 50DX; Topcon, Tokyo, Japan), MultiColor scanning laser imaging, spectral domain optical coherence tomography (Spectralis; Heidelberg Engineering, Heidelberg, Germany), swept source optical coherence tomography (DRI OCT-1 Atlantis; Topcon, Tokyo, Japan), adaptive optics (RTX-1; Imagine Eyes, Orsay, France), and microperimetry (MP1 Microperimeter; Nidek Technologies, Padua, Italy), were performed to confirm the diagnosis and determine the anatomical abnormalities related to the disease. Results Conventional clinical examination and imaging studies failed to explain the patient's visual dysfunction and this specific macular abnormality. Using advanced ophthalmic technologies, including MultiColor imaging, spectral domain optical coherence tomography with high-density acquisition, swept source optical coherence tomography, adaptive optics, and microperimetry, we identified the nature of the macular manifestation accounting for pathology of the foveal red spot syndrome and his visual dysfunction. Conclusion When conventional clinical examination and imaging techniques fail to identify the presence of and visual symptoms in foveal red spot syndrome, advanced technologies may be used to confirm the diagnosis and explain the etiology of the abnormality.

Purpose To describe the spectrum of retinal capillary ischemia, including superficial and deep capillary ischemia, as identified with spectral-domain optical coherence tomography (SD OCT), that occurs in retinal arterial occlusive disease. Design Retrospective observational case series. Methods Clinical charts, color fundus photography, red-free fundus photography, fluorescein angiography, near-infrared reflectance, and SD OCT imaging in 40 eyes of 35 patients with retinal arterial occlusive disease were studied in both the acute and chronic phases in multicenter clinical practices. SD OCT imaging analysis was employed to characterize the presence of superficial and deep capillary ischemia in each eye. Results Of the 40 eyes, 15 eyes had central retinal artery occlusion (CRAO), 22 eyes had branch retinal artery occlusion (BRAO), and 3 eyes had cilioretinal artery occlusion. During the acute phase, SD OCT showed the following 3 distinct patterns, related to retinal ischemia occurring at varying levels within the retina: (1) thickening and hyperreflectivity of the inner retinal layers, including the nerve fiber and ganglion cell layers owing to ischemia of the superficial capillary plexus; (2) a hyperreflective band at the level of the inner nuclear layer, termed "paracentral acute middle maculopathy," representing ischemia of the intermediate and deep retinal capillary plexuses (deep capillary ischemia); and (3) diffuse thickening and hyperreflectivity of both the inner and middle retinal layers, which represented both superficial and deep capillary ischemia. Of all eyes, 31 (78%) had both superficial and deep lesions. The remaining 9 eyes (22%) had isolated deep capillary ischemia producing paracentral acute middle maculopathy with sparing of the superficial capillary plexus and a normal fluorescein angiographic appearance. As the lesions evolved into the chronic phase over the ensuing 3 months, the resultant thinning and atrophy reflected the retinal layers affected during the acute phase. Conclusion SD OCT imaging reveals the spectrum of capillary ischemia in retinal artery occlusive disease showing variable involvement of the superficial and intermediate/deep capillary plexuses. Isolated deep capillary ischemia manifested as paracentral acute middle maculopathy on SD OCT and may be seen in some eyes with retinal arterial circulation compromise despite complete absence of perfusion abnormalities on fluorescein angiography.

To measure the retinal thickness of the posterior pole in healthy Chinese subjects using a retinal thickness analyzer and to compare the retinal thicknesses between different age, gender, and refractive error groups.Between February 2002 and September 2004, healthy Chinese subjects (age range, 21-50 years), underwent measurements of visual acuity, refractive error, intraocular pressure, and posterior pole retinal thickness with the RTA, as well as ophthalmoscopy and slit lamp examinations. Eleven retinal thickness parameters were directly derived from the reports of the RTA measurement. The average value of each parameter was compared between the male and female groups, the emmetropia and low-myopia groups, and the three age groups (21-30, 31-40, and 41-50 years). Linear regression analysis was also applied to determine the effect of age on 11 parameters. Some participants were also enrolled in the reproducibility assessment procedure.The 272 eyes of 272 subjects (mean age, 34.4 years) were included. The intraclass correlation coefficients of intravisit and intervisit reproducibility were 0.95 and 0.88, respectively. The average retinal thicknesses at the foveola and fovea are 147.6 and 160.0 microm, respectively. No significant difference was found in any of the parameters between the different gender groups, emmetropia and low-myopia groups, or age groups. Regression analysis revealed no significant negative linear correlation between any of the parameter values and age.The retinal thickness of macular region in the Chinese is a little lower than in Westerners, but not statistically significant. The Chinese retinal thickness was not gender or refractive error related.