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An 84-year-old man presented with decreased right-eye visual acuity. Upon initial examination, the rightand left-eye visual acuities were 0.03 and 1.2, respectively; moreover, the right- and left-eye intraocular pressure was 12 mmHg and 13 mmHg, respectively. Examination revealed a shallow anterior chamber of the right eye, anterior chamber inflammation, vitreous opacity, and marked retinochoroidal detachment. Optical coherence tomography (OCT) revealed retinal detachment (RD) and choroidal folds; moreover, B-scan ultrasonography (B-scan) showed RD as well as thickened sclera with fluid in Tenon's space. Fluorescent fundus angiography revealed hyperfluorescence in the optic disc and vascular hyperpermeability in the right eye. The left eye lacked extra-ocular symptoms or abnormalities. The right ocular axis measured 23.4 mm with no apparent subretinal fluid migration due to positional changes. Accordingly, the patient was diagnosed with panuveitis associated with posterior scleritis and immediately started on 40 mg prednisolone, which improved his symptoms. However, at 3 post-treatment months, choroidal folds were observed and was restarted on 20 mg prednisolone. The choroidal folds subsequently disappeared, with a current visual acuity of 0.3 in the right eye and no recurrence. Our findings indicated the utility of accurate diagnosis of posterior scleritis by B-scan and prompt systemic steroid administration.

Background/purpose: To present a case of sclerochoroidal calcification (SCC) associated with dural calcification along the optic nerves and severe visual loss., Methods: Case report., Results: A 74-year-old white female patient with a 25-year history of primary hyperparathyroidism and surgical removal of a single parathyroid gland presented with blurred vision. On presentation, she had a calcium level of 12.6 mg/dL (reference range: 8.7-10.3 mg/dL). Her best-corrected visual acuity (BCVA) was 20/40 in both eyes, and she was diagnosed with bilateral SCC. After 2 years, the patient returned with a complaint of progressive vision loss, and the BCVA was 20/150 in the right eye and hand motion in the left eye. She had stable focal SCC on fundus examination with no significant changes from the previous examination. The fluorescein angiogram was unremarkable with no leakage. Optical coherence tomography (OCT) of the macula showed no edema or subretinal fluid and was not significantly changed from the first OCT. B-scan showed areas of calcification in the sclera consistent with the SCC. Computerized tomography (CT) scans showed dural calcifications along both optic nerves. She had no enlargement of SCC lesions and no other ocular or neurologic pathology associated with her vision loss., Conclusion: We present a patient with bilateral SCC and associated calcification in both globes. Unlike previous reports of SCC, our case demonstrated progressive severe vision loss because of dural calcification along the optic nerves. Patients with SCC and decreased vision should receive a CT scan to look for this rare associated finding.

Purpose: To describe two cases of severe peripapillary pachychoroid syndrome successfully managed with monthly intravitreal aflibercept therapy., Methods: Medical and imaging records were retrospectively reviewed. Patients were imaged with ultra-widefield fluorescein, indocyanine green angiography, and fundus autofluorescence. Spectral-domain optical coherence tomography was performed to evaluate macular edema and choroidal thickness. Optical coherence tomography angiography excluded macular neovascularization., Results: This report summarizes two cases of peripapillary pachychoroid syndrome complicated by very severe bilateral macular edema. In all four eyes, the diffuse intraretinal and subretinal fluid remarkably improved or completely resolved after monthly intravitreal aflibercept injections with commensurate improvement of visual acuity. Multimodal imaging documented the significant improvement of fluid and the reduction in choroidal thickening in response to anti-vascular endothelial growth factor therapy in each case., Conclusion: Severe cases of peripapillary pachychoroid syndrome associated with vision loss can be successfully treated with intravitreal aflibercept therapy.

Purpose: To evaluate presenting features and visual outcomes in eyes with acute syphilitic posterior placoid chorioretinopathy (ASPPC)., Design: Retrospective cohort study., Subjects: A total of 24 eyes of 17 adult patients with ASPPC., Methods: Chart review of patients with ASPPC who presented to the University of Michigan W. K. Kellogg Eye Center between January 1, 2012, and November 4, 2022. Demographic and clinical information, fundus photographs, fundus autofluorescence, and spectral-domain-OCT (SD-OCT) findings were reviewed., Main Outcome Measures: Clinical characteristics and visual acuity (VA) on presentation and follow-up examination., Results: The median age was 46 (interquartile range [IQR], 38-51) years. At presentation, 20 (83.3%) eyes had subjectively decreased vision, with a median initial VA of 0.54 (IQR, 0.35-1.00) logarithm of the minimum angle of resolution (logMAR); at 45 days, median logMAR VA was 0.096 (IQR, 0.02-0.17). Initial VA was positively associated with posterior pole-sparing lesions (coefficient estimate [CE], -0.75; 95% confidence interval [CI], -1.38 to -0.12); P = 0.03), and negatively associated with ellipsoid zone (EZ) disruption (CE, 0.72; 95% CI, 0.03-1.42; P = 0.04), subfoveal EZ disruption (CE, 0.62; 95% CI, 0.02-1.23; P = 0.046), and initial hyperreflective foci on SD-OCT (CE, 0.66; 95% CI, 0.09-1.23; P = 0.03). Female eyes were more likely (hazard ratio [HR], 3.36; 95% CI, 1.07-10.6; P = 0.04), and eyes with optic nerve abnormality were less likely (HR, 0.34; 95% CI, 0.12-0.96; P = 0.04), to achieve a VA ≥ 20/40 (logMAR, 0.30)., Conclusions: This study of patients with ASPPC showed that symptomatic eyes had an improvement from a median VA of 20/69 on presentation to a median VA of 20/25 at 45 days. Female sex and absence of optic nerve involvement were associated with higher probability of achieving ≥ 20/40. These findings provide refined guidance for counseling patients who present with decreased vision due to ASPPC., Financial Disclosure(s): The authors have no proprietary or commercial interest in any materials discussed in this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Central serous chorioretinopathy (CSC) is the fourth most common non-surgical retinopathy associated with fluid leakage. The pathogenesis is not yet completely understood, but changes in the choroid, sclera and RPE have been described associated with venous congestion of choroidal outflow. CSC can be categorised into acute, chronic, and recurrent subtypes with recent classifications of simple and complex based on the area of RPE change seen on fundus autofluorescence. A multimodal imaging approach is helpful in the diagnosis and management of CSC and secondary complications such as type 1 neovascularisation. Although spontaneous resolution with relatively good visual outcomes is common, treatment should be considered in patients with persistent or recurrent SRF. Treatment options include laser, systemic medications, intravitreal therapy, and surgery. Of these, argon laser for focal extramacular fluid leaks and photodynamic therapy of leakage identified by indocyanine-green angiography currently have the greatest supportive evidence., (© 2023 The Authors. Clinical & Experimental Ophthalmology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Ophthalmologists.)

Aims: To characterise and classify the morphological, clinical and tomographic characteristics of focal choroidal excavation (FCE) lesions to determine their prognostic implications., Methods: 36 eyes with FCE (32 patients) underwent multimodal imaging, including spectral domain optical coherence tomography and fundus autofluorescence. FCE lesions were classified into three subtypes: (1) type 1: myopic (central choroidal thickness: <100 µm), (2) type 2: suspected congenital (central choroidal thickness: 100-200 µm, without associated chorioretinal pathology) and (3) type 3: secondary or acquired (central choroidal thickness: >200 µm, with associated chorioretinal pathology)., Results: 80.6% of eyes were followed longitudinally (26.8±18.8 months). There were 9 type 1 FCEs (myopic), 8 type 2 FCEs (U-shaped, congenital) and 19 type 3 FCEs (V-shaped, secondary). Type 2 FCEs trended towards larger maximum widths (p=0.0563). Type 3 FCEs were associated with central serous chorioretinopathy or pachyvessels (47.4%), but were also seen in pattern dystrophy, geographic atrophy, inactive choroiditis, torpedo maculopathy and adult-onset vitelliform dystrophy. Choroidal neovascular membranes (CNVMs) were more prevalent in type 3 FCE (41.2% compared with 11.1% for type 1 FCE, p=0.251, and 0% for type 2 FCE, p=0.043)., Conclusions: The FCE types, stratified by central choroidal thickness, demonstrated distinct morphological characteristics and associated findings. The classification scheme held prognostic implications as type 3 FCE with V shapes were associated with other chorioretinal conditions and were more likely to develop CNVM., Competing Interests: Competing interests: KK: consultant for Regenxbio. DD’A: consultant—Alcon, IVERIC bio and Aufbau Holdings; equity—IVERIC bio and Aufbau Holdings; intellectual property—Aufbau Holdings. Szilárd Kiss: consultant—Adverum, Alcon, Novartis, Optos and Genentech/Roche; research funding—Allergan, Novartis, Optos, Genentech/Roche and Regeneron; equity—Adverum, Regenxbio and Fortress Bio; intellectual property—gene therapy for age-related macular degeneration and T cells for CMV retinitis, assigned to Cornell University., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: Report a case of hypertensive chorioretinopathy with massive bilateral serous retinal detachment in a young patient with chronic kidney disease., Methods: Observational case report., Results: An 18-year-old man with Grade 5 chronic kidney disease, systemic high blood pressure, secondary acute pulmonary edema, and acute uremic syndrome was referred to our service complaining of bilateral decreased vision starting one week ago. The patient was treated at that moment with hemodialysis and losartan. At initial examination, the patient's blood pressure was 170/120 mmHg; dilated fundus examination evidenced optic disk edema, hypertensive chorioretinopathy, and massive serous retinal detachment with best-corrected visual acuity of hand motion in both eyes. The case was diagnosed as undertreated hypertension and was referred to the nephrologist for treatment adjustments. At 1-month follow-up, blood pressure was 160/90 mmHg; there was clinical improvement in both eyes but with ischemic sequelae. At the final follow-up 6 months later, blood pressure was 100/60 mmHg, best-corrected visual acuity was 20/80 in the right eye and count fingers at 2 min the left eye, and there was a complete resolution of the retinal serous detachment in both eyes., Conclusion: Patients with massive serous detachments due to systemic hypertension are atypical so it is of immense importance for the ophthalmologists to recognize the ocular manifestations of systemic diseases that put the patient's life at risk as in this case.

Introduction: Pregnancy may be associated to unique retinal disorders and is associated to worsening of retinal disease that also occurs in non-pregnant females. We report a case of chorioretinopathy caused by pre-eclampsia associated to HELLP (Hemolysis-Elevated-Liver enzymes and Low Platelet count) syndrome., Methods: Multimodal Imaging features of HELLP syndrome-related hypertensive chorioretinopathy including retinography, Spectral Domain-Optical Coherence Tomography (SD-OCT), Fluorescein angiography (FA), Indocyanine-green angiography (ICG) along with OCT-angiography (OCTA) are presented and discussed., Results: Multimodal imaging and OCTA show both retinal and choroidal involvement by HELLP syndrome, resolved after hypertension treatment., Conclusions: Multimodal imaging is useful to study HELLP syndrome-related hypertensive chorioretinopathy. Moreover, OCTA is a new technology able to study and follow the circulatory status of the choriocapillaris during the disease.

Competing Interests: None

Blood vessel dysfunction causes several retinal diseases, including diabetic retinopathy, familial exudative vitreoretinopathy, macular degeneration and choroidal neovascularization in pathological myopia. Vascular endothelial growth factor (VEGF)-neutralizing proteins provide benefits in most of those diseases, yet unsolved haemorrhage and frequent intraocular injections still bothered patients. Here, we identified endothelial CD146 as a new target for retinal diseases. CD146 expression was activated in two ocular pathological angiogenesis models, a laser-induced choroid neovascularization model and an oxygen-induced retinopathy model. The absence of CD146 impaired hypoxia-induced cell migration and angiogenesis both in cell lines and animal model. Preventive or therapeutic treatment with anti-CD146 antibody AA98 significantly inhibited hypoxia-induced aberrant retinal angiogenesis in two retinal disease models. Mechanistically, under hypoxia condition, CD146 was involved in the activation of NFκB, Erk and Akt signalling pathways, which are partially independent of VEGF. Consistently, anti-CD146 therapy combined with anti-VEGF therapy showed enhanced impairment effect of hypoxia-induced angiogenesis in vitro and in vivo. Given the critical role of abnormal angiogenesis in retinal and choroidal diseases, our results provide novel insights into combinatorial therapy for neovascular fundus diseases., (© 2021. Science China Press and Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To illustrate with multimodal imaging a case of HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelets) complicated by bilateral multifocal serous retinal detachments, subretinal exudation, and papilledema., Methods: Case report. Fundus photography, spectral domain optical coherence tomography (SD-OCT), fluorescein angiography, and indocyanine green angiography were performed at presentation and the day after. We also present the SD-OCT follow-up at 8 days, 1 year, and 4 years., Results: A 25-year-old 5-month-pregnant Guinean woman complained about decreased visual acuity in the right eye. Eye fundus and multimodal imaging were abnormal in both eyes. Spectral domain optical coherence tomography showed the presence of multifocal serous retinal detachments, subretinal deposits, and intraretinal cysts. Indocyanin green angiography revealed an irregular choroidal perfusion and localized choroidal ischemia. Spectral domain optical coherence tomography also provided assessment of retinal changes during the long-term follow-up, showing tissue damage in the outer retina., Conclusion: Serous retinal detachments during pregnancy can be the leading sign of HELLP syndrome-a potentially life-threatening condition. Spectral domain optical coherence tomography is a noninvasive and useful tool for its diagnosis and follow-up. ICG is important to confirm the choroidal ischemia and choroidal vascular abnormalities, underlying conditions leading to main sign of HELLP syndrome in the eye.

Research on retinopathy of prematurity (ROP) focuses mainly on the abnormal vascularization patterns that are directly visible for ophthalmologists. However, recent findings indicate that children born prematurely also exhibit changes in the retinal cellular architecture and along the dorsal visual stream, such as structural changes between and within cortical areas. Moreover, perinatal sustained systemic inflammation (SSI) is associated with an increased risk for ROP and the visual deficits that follow. In this paper, we propose that ROP might just be the tip of an iceberg we call visuopathy of prematurity (VOP). The VOP paradigm comprises abnormal vascularization of the retina, alterations in retinal cellular architecture, choroidal degeneration, and abnormalities in the visual pathway, including cortical areas. Furthermore, VOP itself might influence the developmental trajectories of cerebral structures and functions deemed responsible for visual processing, thereby explaining visual deficits among children born preterm., (© 2021. The Author(s).)

Purpose: To evaluate the flow signals in subretinal hyperreflective material (SHRM) that represents classic choroidal neovascularization (CNV) on fluorescein angiography in eyes with polypoidal choroidal vasculopathy., Methods: We retrospectively reviewed 20 eyes with polypoidal choroidal vasculopathy that appeared to have classic CNV on fluorescein angiography, accompanied by SHRM on optical coherence tomography (OCT) at the same location. Using OCT angiography (OCTA), we analyzed intrinsic flow signals in the SHRM (cross-sectional B-scans and en face). The possible association between pretreatment OCT angiography findings and fibrotic scar formation after antivascular endothelial growth factor (VEGF) treatment was evaluated., Results: Six of 20 eyes (30%) showed vascular SHRM; the remaining 14 eyes (70%) showed avascular SHRM at the classic CNV site at baseline. The SHRM corresponded with polypoidal lesions seen on indocyanine green angiography in 5 of 6 eyes with vascular SHRM and in all 14 eyes with avascular SHRM. After anti-VEGF treatment, all 6 eyes with vascular SHRM left a fibrotic scar, whereas all 14 eyes with avascular SHRM showed no scar formation (P < 0.001)., Conclusion: Using OCT angiography, we evaluated the flow signals in SHRM that represented classic CNV in eyes with polypoidal choroidal vasculopathy and successfully differentiated true Type 2 macular neovascularization from pseudo classic CNV.

Background: Uveal effusion syndrome is a rare disease characterized by exudative detachments of the choroid, ciliary body, and retina. Various surgical procedures and nonsurgical strategies have been described to treat uveal effusion syndrome with limited success. The treatment for uveal effusion syndrome remains a serious challenge for clinicians. To the best of our knowledge, no previous report has described a severe uveal effusion syndrome patient with nanophthalmos treated by using an anti-vascular endothelial growth factor agent alone. We report here one such case with unexpected positive results., Case Presentation: A 30-year-old Chinese male patient presented with painless vision loss in both eyes that had persisted for 2 months. Examination of the right eye revealed a best corrected visual acuity of 0.03; the best corrected visual acuity of the left eye was finger count/20 cm. The intraocular pressure was normal on both eyes. A-scan revealed an right eye axial length of 15.88 mm and a left eye axial length of 16.21 mm. In the right eye, half of the peripheral choroid and nearly three-fourths of the retina were detached. The left fundus was not visible because of the total retinal detachment located just behind the lens, which could be clearly observed directly with a slit lamp. Considering all the possibilities and available treatments as well as the patient's intentions after discussion, we first administered an intravitreal injection of ranibizumab 0.5 ml into both eyes. The patient's visual perception improved 3 days after the injection. One month later, most of the effusion under the choroid and retina was absorbed. Visual acuity improved from finger count to 0.05 in both eyes, and vision quality was remarkably improved. Encouraged by this good result, the patient opted to undergo a second injection 1 month later. Choroidal and retinal detachment completely vanished 30 days after the second injection., Conclusions: Using an anti-vascular endothelial growth factor agent alone may be a potentially effective and safe method for managing some types of uveal effusion syndrome, such as in nanophthalmos. The injection may be administered before considering more aggressive procedures in some uveal effusion syndrome patients., (© 2021. The Author(s).)

Abstract: Concomitant chorioretinal folds with disc edema can be seen in cases of thyroid eye disease presenting with compressive optic neuropathy and may portend optic nerve ischemia. We describe an unusual case of a 64-year-old man who developed partial vision loss after orbital decompression., Competing Interests: V. I. Elmalem: Thyroid Eye Disease Advisory Board 8/24/2019, Horizon Therapeutics, Plc; I. Belinsky: Thyroid Eye Disease Advisory Board 7/31/2020, Horizon Therapeutics, Plc. The remaining authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Purpose: To investigate the incidence rate of massive submacular haemorrhage (SMH) and risk factors in polypoidal choroidal vasculopathy (PCV) and typical neovascular age-related macular degeneration (tnAMD)., Methods: A total of 465 patients who were diagnosed with either PCV (n = 245) or tnAMD (n = 220) from 2003 to 2014 were enrolled. Cumulative incidence of massive SMH in PCV and that in tnAMD were compared. Risk factors of massive SMH were also analysed., Results: Massive SMH occurred in 32 patients (13.1%) with PCV and 9 patients (4.1%) with tnAMD. Incidence rates of massive SMH 5 and 10 years after the first visit were 11.1% and 29.9% in PCV and 4.3% and 9.9% in tnAMD, respectively. Incidence rates of massive SMH in PCV were significantly higher than those in tnAMD (hazard ratio [HR], 2.66; p = 0.007). Cox regression analysis revealed that mean number of photodynamic therapies (PDTs) per year (HR, 4.24; p < 0.001), cluster type of polypoidal lesion (HR, 3.42; p = 0.003) in PCV, and mean number of anti-VEGF injections per year (HR, 1.58; p < 0.001) in tnAMD were significantly associated with risk of massive SMH. For patients with severe vision loss, proportion of incident massive SMH was significantly higher in PCV (29.5%) than in tnAMD (6.9%, p < 0.001)., Conclusion: The incidence rate of massive SMH in eyes with PCV was about three times higher than that in eyes with tnAMD. Treatment methods that can reduce the incidence of massive SMH should be considered, especially for eyes with PCV., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To describe breakthrough vitreous hemorrhage secondary to polypoidal choroidal vasculopathy (PCV)., Methods: Patients with the diagnosis of PCV from January 2005 to March 2020 at Peking Union Medical College Hospital were retrospectively reviewed, cases with breakthrough vitreous hemorrhage were analyzed. Subgroup analysis was conducted regarding pachychoroid PCV and nonpachychoroid PCV., Results: Among 722 PCV patients (834 eyes), 103 eyes with breakthrough vitreous hemorrhage (12.4%) were included. Pars plana vitrectomy and proper further interventions could significantly improve the best-corrected visual acuity from logMAR 2.15 ± 0.48 (Snellen 20/2825) to 1.65 ± 0.67 (20/893). Hemorrhagic retinal detachment, baseline central macular thickness, and best-corrected visual acuity were factors associated with final best-corrected visual acuity (P < 0.05). In the pachychoroid PCV group, patients were younger, all had hemorrhagic pigment epithelial detachment, with a higher prevalence of choroidal vascular hyperpermeability and hemorrhagic retinal detachment, thicker subfoveal choroidal thickness, and thinner central macular thickness; besides, the initial pars plana vitrectomy were more complicated, more additional surgeries had to be performed. More eyes in the nonpachychoroid PCV group had received anti-vascular endothelial growth factor or photodynamic therapy, mostly fibrovascular pigment epithelial detachment, the best-corrected visual acuity and the status of the fellow eye were significantly worse. For the final ocular status, more eyes in nonpachychoroid PCV group were taking anti-vascular endothelial growth factor monotherapy, whereas more eyes in pachychoroid PCV group were stable. The choroidal parameters of these two groups were all significantly different., Conclusion: Breakthrough vitreous hemorrhage is a troublesome complication of PCV. Pars plana vitrectomy and additional interventions are required for better prognosis. Vitreous hemorrhage secondary to pachychoroid PCV or nonpachychoroid PCV have different characteristics and prognosis.

Purpose: To describe the vitreoretinal structure at the margin of the choroidal coloboma in infants and older patients using swept-source (SS) OCT., Design: Retrospective case series., Participants: Nineteen eyes of 16 patients with choroidal coloboma (7 males, 9 females; average age, 12.3 ± 7.1 years)., Methods: The patients were classified into 2 groups: infants 1 year of age or younger (3 eyes) and older patients (16 eyes). Each finding on SS OCT was documented according to previously defined histopathologic findings., Main Outcome Measures: Description of the SS OCT features of choroidal colobomas., Results: Swept-source OCT showed that the extracolobomatous retina centrally traversed the margin to continue as the marginal intercalary membrane (MICM), whereas the outer layers of the MICM were reversed at the point (point of reversal [POR]). The expected duplication was seen in all infant eyes, but in none of the older eyes whose outer layers of the MICM were ambiguous. However, at the boundary between the layered MICM and monolayered central intercalary membrane (CICM), the POR was detectable in all patients. Further SS OCT analysis showed that the MICM schisis and CICM schisis occurred simultaneously with vitreous traction. Retinal detachments (RDs) seen in 4 eyes were connected to the only MICM schisis, and a MICM break was identified in 1 eye. Swept-source OCT showed that retinal pigment epithelial hyperplasia adhered tightly to the retina and that the glial triangle was adhered tightly to the sclera, indicating barriers to the development of RD after MICM schisis., Conclusions: Swept-source OCT first visualized the POR in infant eyes and showed that the POR was identifiable despite the atrophic changes in the outer layer of the MICM in the older eyes. Based on the POR location, we confirmed that the intercalary membranes reported in previous OCT studies were clearly differentiated between the MICM and CICM. We also showed that the presence of MICM and CICM schisis resulted from vitreous traction at the coloboma margin and that MICM breaks induced RD only if the barrier that prevented the development of RD was broken., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To evaluate the efficacy and safety of monthly and pro re nata (PRN, guided by visual acuity stabilization and disease activity criteria) ranibizumab regimens in Chinese patients with neovascular age-related macular degeneration (nAMD) and polypoidal choroidal vasculopathy (PCV)., Methods: This double-masked study randomized nAMD patients (1:1) to ranibizumab monthly from baseline to Month (M) 11 to a PRN regimen from M12 to M23 (monthly group, n = 167) versus ranibizumab three monthly doses followed by a PRN regimen up to M23 (PRN group, n = 166). Subgroups were assessed based on the presence/absence of PCV (indicated by indocyanine green angiography)., Results: Of 334 randomized patients, 41.7% had PCV at baseline. Mean average best-corrected visual acuity (BCVA) change from M3 to M4 through M12 was 3.3 letters with monthly and 1.7 letters with PRN (mean difference: 1.6; 95% CI: -2.95, -0.20, primary end-point). Mean change in BCVA from baseline (monthly/PRN, 53.8/53.7) to M12 and M24 was 12.3 and 11.3 letters in monthly and 9.6 and 9.3 letters in PRN group. Corresponding values for patients with PCV/without PCV were 12.7/12.1 letters (M12) and 12.3/10.6 letters (M24) in monthly and 9.4/9.4 letters (M12) and 9.7/8.7 letters (M24) in PRN groups. The mean number of injections was 11.4 (monthly) and 8.2 (PRN) from Day 1 to M11 and 4.8 (monthly) and 5.0 (PRN) from M12 to M23. No new safety findings were reported., Conclusions: The study results support the use of either ranibizumab monthly or PRN regimens in Chinese patients with nAMD, regardless of presence of PCV., (© 2020 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

We evaluated changes in the complement system resulting from anti-vascular endothelial growth factor (VEGF) in eyes with age-related choroidal neovascularization (CNV) including neovascular age-related macular degeneration, pachychoroid neovasculopathy, and polypoidal choroidal neovasculopathy. We measured the concentrations of the complement activation products (C3a, C4a), VEGF, and monocyte chemotactic protein-1 in the aqueous humor during intravitreal anti-VEGF injections for CNV. The VEGF level decreased significantly (P < 0.001), while the C3a and C4a levels increased significantly (P < 0.001 for both comparisons) 1 month after two monthly anti-VEGF injections. The VEGF level was correlated with the C3a (R = 0.328, P = 0.007) and C4a (R = - 0.237, P = 0.055) levels at baseline, but the correlation between the VEGF and C3a levels (R = - 0.148, P = 0.242) changed significantly (P = 0.028 by analysis of covariance) after anti-VEGF treatment. The C3a increase after anti-VEGF therapy did not change the visual outcomes in eyes with CNV for 1 year. Dysregulation of the complement system can be induced after anti-VEGF therapy.

Purpose: To study the characteristics of focal choroidal excavation (FCE) in Indian eyes based on spectral-domain optical coherence tomography (SD-OCT) findings and their association with macular pathologies., Methods: Retrospective study of 26 patients diagnosed with FCE. All patients' clinical and imaging data were reviewed., Results: There were eight females and 18 males aged between 24 and 85 years. FCE was noted in 31 eyes of 26 patients - unilateral in 21 and bilateral in 5. The conforming type was noted in 13 and nonconforming in 19 eyes. The location was extrafoveal in 16 and subfoveal in 15 eyes. The morphology was bowl-shaped in 24, cone-shaped in six eyes, and mixed type in one eye. Associated pathologies were central serous chorioretinopathy in nine eyes, choroidal neovascular membrane in seven eyes, Stargardt's disease in three eyes, Best disease in four eyes, other retinal dystrophies in two eyes, polypoidal choroidal vasculopathy and moderate non-proliferative diabetic retinopathy, each in one eye. The mean FCE width was 1667.2 ± 817.7 μ, mean depth was 95.7 ± 46.4 μ, and the mean choroidal thickness under the FCE was 234.8 ± 85.9 μ. No abnormal choroidal tissue was found under any FCE., Conclusion: FCE is a relatively common entity and frequently associated with macular pathologies. The presence of an FCE did not alter the course or management of these conditions., Competing Interests: None

Pachychoroid spectrum diseases have attracted increasing attention, though their pathophysiology has yet to be fully elucidated. In this study, we assessed the vascular diameters of vortex veins in pachychoroid spectrum diseases such as central serous chorioretinopathy (CSC), pachychoroid neovasculopathy without polypoidal lesions (PNV), and pachychoroid neovasculopathy with polypoidal lesions (polypoidal choroidal vasculopathy: PCV). In a retrospective case series of 94 eyes with CSC, 60 eyes with PNV and 57 with PCV, we binarized en face optical coherence tomography (OCT) images of choroidal vortex veins and analyzed the mean diameter of vortex veins. The presence of anastomosis between the superior and inferior vortex veins and central choroidal thickness (CCT) were also evaluated using OCT images. CSC showed significantly larger mean diameter of vortex veins than PCV (P < 0.05). Anastomosis between superior and inferior vortex veins was observed in over 90% of eyes with each pachychoroid spectrum disease. The patients with CSC were the youngest, followed by PNV patients, and then patients with PCV. The largest CCT values were observed in CSC eyes, followed by PNV eyes, and then PCV eyes. CCT correlated with the mean diameter of vortex veins (rs = 0.51, P < 0.01). These findings suggest that congestion of vortex veins might show gradual amelioration corresponding to the development of anastomosis between the superior and inferior vortex veins during the course of progression of pachychoroid spectrum diseases. Moreover, the mean diameter of vortex veins can be used as a parameter indicating choroidal congestion.

Purpose: To report the morphological and clinical features of a case of retinitis pigmentosa with large choroidal excavation., Methods: The patient underwent a complete ophthalmologic examination including best-corrected visual acuity assessment, anterior segment and dilated fundus examination and spectral-domain optical coherence tomography., Results: A 41-year-old woman affected by retinitis pigmentosa with genetic confirmation with mutation in RDH12 gene had a best-corrected visual acuity of 20/50 in both eyes. Dilated fundus examination revealed waxy pallor of the optic disc, diffuse narrowing of the retinal arterioles and a generalized retinal pigment epithelium mottling with bony spicule associated with diffuse retinal atrophy. At the posterior pole, an extended bilateral chorioretinal atrophy was evident with a partial sparing of the macular area. On spectral-domain optical coherence tomography, a bilateral large choroidal excavation could be clearly detected and it was associated with a diffuse retinal thinning at the posterior pole and a partial sparing of the fovea., Conclusion: Large choroidal excavation has been rarely reported. Although the pathogenetic mechanisms leading to the formation of large choroidal excavation are still a matter of debate, a combination of primary degenerative-inflammatory factors could be retained responsible for the large choroidal excavation development.

Background: Pachychoroid, or the structural and functional abnormalities of the choroid, is one of the most important causes of exudative maculopathies. The purpose of this article is to review the current definitions of pachychoroid and their potential consequences. Most publications are from Asian countries. Although no consensus diagnosis has been reached, pachychoroid is defined by thickened choroid and choroidal vascular hyperpermeability, pachyvessels with inner choroidal attenuation; it is closely linked to pachydrusen. Although some studies suggest choroidal congestion may play a role in its pathogenesis, the exact causes of this condition are still unknown. Pachychoroid is associated with exudative maculopathies including central serous chorioretinopathy, pachychoroid neovasculopathy and polypoidal choroidal vasculopathy (PCV). It is widely accepted that macular neovascular membranes may develop secondary to pachychoroid. Recent clinical observations illustrate the importance of pachychoroid in the etiology of macular neovascularization including neovascular age-related macular degeneration (nAMD)., Conclusion: Pachychoroid is an important cause of exudative maculopathies. Both drusen and pachychoroid are increasingly recognized as important causes of macular neovascularization, and eyes formally categorized as typical nAMD or PCV can be further sub-categorized based on the presence or absence of pachychoroid and drusen. There is a need to develop a consensus definition, which will greatly enhance our understanding of pachychoroid and facilitate the development of individual interventions in pachychoroid diseases.

Purpose: Peripheral exudative haemorrhagic chorioretinopathy (PEHCR) is a rare disorder that is often misdiagnosed. The aim of this study was to better characterise PEHCR and to assess treatment options., Material and Methods: Retrospective multicentric chart review., Results: Of 84 eyes (69 patients) with PEHCR referred between 2005 and 2017, the most common referral diagnosis was choroidal melanoma (41.3%). Bilateral involvement was found in 21.7% of cases. Haemorrhagic retinal pigment epithelium detachment was the most common peripheral lesion (53.6%). Maculopathy was associated with peripheral lesions in 65.8% of cases. PEHCR lesions were mostly heterogeneous (58.8%) on B-scan ultrasonography. Choroidal neovascularisation was found in 10 eyes (26.3%) out of 38 eyes that underwent fluorescein angiography. Polyps were observed in 14 eyes (58.3%) out of 24 eyes that underwent indocyanine green angiography. Fifty-one eyes were treated (62.2%). Intravitreal injections (IVTI) of antivascular endothelial growth factor (VEGF) were the most used treatment (36.6%) before laser photocoagulation, photodynamic therapy, vitrectomy and cryotherapy. Only vitrectomy improved visual acuity. Most lesions (65.6%) regressed at the last follow-up visit., Conclusion: In case of PEHCR, multimodal imaging is useful to avoid misdiagnosis, to characterise PEHCR lesions and to guide treatment strategies. Regression of PEHCR lesions was observed in two-thirds of the patients. Vitrectomy improved visual acuity. More than a third of patients underwent anti-VEGF IVTI. Further studies are needed to assess IVTI's efficacy., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: To compare the choriocapillary flow density (CFD) among the fellow eyes of polypoidal choroidal vasculopathy (PCV), neovascular age-related macular degeneration (nAMD), and healthy controls using spectral-domain optical coherence angiography tomography (SD-OCTA)., Methods: This is a cross-sectional study that includes the fellow eyes of 38 patients with unilateral PCV, 36 patients with unilateral nAMD, and 36 eyes from 36 healthy volunteers. The PCV group was further classified into polypoidal CNV (P-CNV) and typical PCV (T-PCV) for subgroup analysis. The age, subfoveal choroidal thickness (SFCT), Age-Related Eye Disease Study (AREDS) classification, and fellow eye diagnosis were acquired. All subjects underwent SD-OCTA with a 6.0-mm scan pattern. Circles with radius of 1.00, 1.50, and 3.00 mm were manually selected in the choriocapillaris (CC) slab, and the CFD was calculated as the percentage of the flow area to the whole selected area as CFD-1.00, 1.50, and 3.00, respectively. Univariate and multivariate analysis were performed to study the correlation between the aforementioned factors with CFD., Results: The mean CFD-1.00, 1.50, and 3.00 of the nAMD group were 61.51, 63.18, and 66.20, respectively; these were significantly lower than those of the PCV group (65.90, 66.89, and 67.94; P < 0.001, P < 0.001, and P = 0.010; respectively) and control group (66.28, 66.96, and 68.42; P < 0.001, P < 0.001, and P = 0.001, respectively), and no difference was detected between the PCV and control group or between PCV subtypes. The AREDS classification and fellow eye diagnosis were correlated with CFD in univariate analysis; however, only the fellow eye diagnosis showed a significant correlation after multiple linear regression., Conclusions: The CFD of nAMD fellow eyes was significantly lower than that of PCV and control eyes, and no difference was detected between PCV and control group, indicating that CC loss plays a different role in the early pathogenesis of nAMD and PCV.

Background: Macular hole (MH) is a retinal break in the fovea involving partial or complete dehiscence of the neural retinal layers affecting the visual quality by decreasing visual acuity (VA) and visual deformation. We describe a case of secondary MH associated with submacular hemorrhage (SMH) due to polypoidal choroidal vasculopathy (PCV), which showed spontaneous closure., Case Presentation: A 67-year-old man developed decreased VA in his right eye due to an SMH. The VA was 20/50, and monthly intravitreal injection of aflibercept was administered three times. The SMH gradually decreased, and 10 months later the external limiting membrane was found to be perforated, resulting in MH. The old clot disappeared, and the MH remained for 10 months. Twenty-three months later, serous retinal detachment (SRD) involving the macula appeared and the MH had disappeared. SRD gradually disappeared, and macular configuration recovered. VA gradually improved and became 20/20 38 months later., Conclusion: Dynamic change of the ultrastructure in an unusual case of secondary-developed and spontaneously closed MH was clearly observed. Although the mechanism was unknown, the small diameter size and exudative PCV are thought to have contributed to the closure.

Purpose: To study the association between the risk of massive submacular hemorrhage (SMH) and polyp regression after initial treatment of polypoidal choroidal vasculopathy using long-term follow-up data., Methods: Retrospective study of 223 patients who were diagnosed with polypoidal choroidal vasculopathy and were followed up for up to 11 years. Subjects were categorized into "regression" and "no regression" groups, according to their polyp status after the initial treatment. Kaplan-Meier survival analyses were performed on development of massive SMH. The association between treatment methods and the occurrence of massive SMH was also analyzed., Results: The incidence rates of massive SMH at 3, 6, and 9 years in the "no regression" group were 6.50, 22.59, and 38.03%, respectively, and in the "regression" group were 1.14, 6.47, and 10.92%, respectively (P = 0.005, log-rank test). The hazard ratio of massive SMH was 3.677 for cluster-type polyps and 0.271 for polyp regression after initial treatment. A higher rate of polyp regression was associated with photodynamic therapy (PDT) than anti-VEGF monotherapy (64.4 vs. 33.3%, P < 0.001). Additional anti-VEGF treatments after initial PDT showed lower risk of massive SMH than PDT only. (9.5 vs 38.5%, P = 0.005)., Conclusion: The long-term risk of massive SMH after initial treatment on polypoidal choroidal vasculopathy is significantly higher in eyes with persistent polyps than those with regressed polyps. Ophthalmologists should pay attention to the risk of massive SMH and the polyp status when treating polypoidal choroidal vasculopathy.

Purpose: To investigate retinal pigment epithelium (RPE) tears in patients with polypoidal choroidal vasculopathy., Method: A retrospective review of polypoidal choroidal vasculopathy cases with confirmed RPE tears was conducted. Patients' comprehensive clinical data were collected and analyzed. The treatment strategy was a loading dose of one intravitreal antivascular endothelial growth factor injection, combined with additional injections if exudative activities or visual deterioration were detected., Results: Among 397 polypoidal choroidal vasculopathy patients, 33 patients with RPE tears (8.3%) were included. 42.4% of them happened spontaneously. Pigment epithelial detachment (PED) occurred more frequently in RPE tear patients and most of them had serous vascularized or hemorrhagic PED. The height and greatest linear diameter of PED, and the subfoveal choroidal thickness of these cases were significantly larger, whereas the central foveal thickness was significantly smaller. Most of the RPE tears occurred at the edge of the PED. After our treatment strategy, patients' best-corrected visual acuity improved significantly from 2.13 ± 1.24 (median 20/52) to 1.32 ± 1.31 (median 20/166). Large subretinal hemorrhage may increase the risk of the formation of subretinal fibrosis (P < 0.05)., Conclusion: Retinal pigment epithelium tears in polypoidal choroidal vasculopathy are associated with high subRPE hydrostatic pressure, produced by a large PED or hemorrhage. After our intervention strategy, this condition may not necessarily result in poor prognosis.

To investigate the long-term clinical course after vitrectomy for breakthrough vitreous hemorrhage secondary to neovascular age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV). This retrospective study included 45 eyes that underwent vitrectomy due to breakthrough vitreous hemorrhage secondary to neovascular AMD. The patients were divided into 2 groups: neovascular AMD group and PCV group. Within each group, the status of the eye within 6 months after the surgery and that at the final follow-up was identified. The visual acuity at the final visit was additionally compared between the 2 groups. The patients were followed up for a mean period of 39.9 ± 19.4 months after the surgery. In the neovascular AMD group (n = 17), re-bleeding requiring vitrectomy was noted in 4 eyes and extensive scar formation was noted in 6 eyes within 6 months after the surgery. At the final visit, treatment was discontinued due to poor visual outcome in 10 eyes. In the PCV group (n = 28), re-bleeding requiring vitrectomy was noted in 1 eye, and extensive scar formation was noted in 4 eyes within 6 months after the surgery. At the final visit, treatment was discontinued in 8 eyes. The visual acuity at the final visit was significantly better in the PCV group (P = 0.003). The long-term clinical course after vitrectomy for breakthrough vitreous hemorrhage was markedly different between neovascular AMD and PCV, showing significantly better long-term visual outcomes in PCV.

Purpose: To investigate the long-term incidence and growth rate of chorioretinal atrophy (CRA) in patients with polypoidal choroidal vasculopathy (PCV) and determine the associated risk factors., Methods: The medical records of 88 patients with unilateral symptomatic PCV who received anti-vascular endothelial growth factor (anti-VEGF) injections with or without photodynamic therapy (PDT) were analyzed retrospectively. Near-infrared fundus imaging and spectral domain optical coherence tomography were used to measure the CRA area and growth rate. Kaplan-Meier survival analysis was performed to estimate the CRA incidence. Logistic and linear regression analyses were used to investigate risk factors (e.g., age, frequency of abnormal OCT findings, PDT history, total injection number, and choroidal thickness) associated with the CRA incidence and growth rate, respectively., Results: The overall CRA incidence was 40.8% at 5 years. The absence of subretinal fluid, the presence of intraretinal fluid, and a thin choroid were significant risk factors for CRA occurrence with a history of PDT. Overall 5-year CRA growth rate was 0.69 mm2/year. Faster CRA growth was significantly related to the presence of subretinal hyperreflective material and thin choroid. PDT history was not significantly related to CRA growth., Conclusions: Thin choroid may be a significant risk factor for long-term development and growth of CRA in eyes with PCV. Intraretinal fluid seems to promote the development of CRA, while subretinal fluid seems to be associated with CRA prevention. The history of PDT was significantly related to the occurrence of CRA, but not to the growth rate of CRA., (© 2019 S. Karger AG, Basel.)

Purpose: To report the outcomes of pars-plana approach for the management of brunescent cataract in eyes with severe microcornea and associated chorio-retinal coloboma., Methods: This was a retrospective, single center, interventional case series performed in a tertiary eyecare center in central Medical records of consecutive cases of microcornea with coloboma who underwent pars-plana vitrectomy with phacofragmentation (PF) between January 2015 and December 2017 were reviewed., Results: The study group comprised of 30 eyes of 30 patients, of which 18 (60%) were males and 12 (40%) were females. The mean age of the patients was 41.9 years (range of 17-70 years). The mean corneal diameter was 6.7 mm with a range of 4-8 mm and all the eyes had dense cataract with nuclear sclerosis of grade 4 or more. The mean preoperative visual acuity was 1.97 (+/-0.067) Log MAR and the mean postoperative vision at 1 month was 1.6 (+/-0.39) Log MAR. Postoperatively, 21 patients (70%) gained ambulatory vision. The visual gain in all the patients was maintained over a mean follow-up period of 15.5 months., Conclusion: Pars-plana vitrectomy with PF can be considered in eyes with severe microcornea and brunescent cataracts, where cataract surgery through the limbal (anterior) approach is not only difficult but at times impossible due to anatomical restraints., Competing Interests: None

Purpose: To create a diagnostic algorithm for the management of chorioretinal folds., Methods: We reviewed the existing literature about chorioretinal folds focusing our attention on three specific conditions and created a diagnostic algorithm in order to otpimize the choice and the number of investigations., Results: Chorioretinal folds are visible striations of the fundus usually arranged in parallel lines and disposed horizontally. They may be either unilateral or bilateral, symptomatic or asymptomatic and are often associated with different possible ocular and extra ocular pathologies, including systemic diseases like autoimmune disorders and intracranial hypertension. They are named idiopathic when no apparent cause for their development is detectable. However, with improved diagnostic testing, the patients with idiopathic choroidal folds are likely to represent only a smaller portion of the total., Conclusions: Since choroidal folds be the sole sign of an underlying disease possibly requiring a multidisciplinary approach, an appropriate work-up varying according to the specific clinical features of each case is needed to define the etiology and the treatment. A diagnosting algorithm may be useful in order to optimize the diagnostic approach and management.