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Purpose: To describe the role of optical coherence tomography angiography (OCTA) to help in differential diagnosis between papilledema and a rare case of choroidal neovascularization (CNV) associated with Morning Glory Syndrome (MGS)., Methods: Observational case report., Results: A 22-year old man was referred to Eye clinic from Neurological unit with a diagnosis of papilledema in right eye. OCTA scans on the optic disc revealed a dense radial peripapillary vascular network while two polypoidal choroidal vasculopathy (PCV) were present in peripapillary region at the edge of staphyloma in corrispondence of choriocapillary layer. Therefore, the patient underwent three-monthly intravitreal injections of anti-vascular endothelial growth factor. One and six months after loading phase, the PCVs showed no clinical activity at structural spectral domain-OCT and OCTA., Conclusion: This case revealed the crucial role of OCTA as an efficace diagnostic technique in discriminating papilledema from MGS complicated by CNV, allowing to make differential diagnosis, useful in ophthalmological and neurological clinical practice., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To evaluate retinal and choriocapillary vessel density (VD) changes in patients with chronic central serous chorioretinopathy (CSC) treated with half-fluence verteporfin photodynamic therapy (vPDT) or eplerenone, using optical coherence tomography angiography (OCTA)., Methods: Patients affected by CSC and treated with vPDT and eplerenone were retrospectively studied. At baseline and 3 months after each treatment, all patients underwent a complete ophthalmological examination, including an evaluation of best-corrected visual acuity (BCVA), slit-lamp biomicroscopy, fundus examination, fluorescein angiography (FA), indocyanine green angiography (ICGA), spectral-domain optical coherence tomography (SD-OCT) and OCTA., Results: Forty-eight eyes of patients with CSC were analysed. Twenty-four eyes were placed in the vPDT group, and 24 eyes formed the eplerenone group. In both groups, OCTA showed a significant improvement in the VD of deep capillary plexus (DCP) and choriocapillaris (CC) after treatments with respect to baseline (p < 0.001), whereas the VD of superficial capillary plexus (SCP) did not show significant differences (p > 0.05). The PDT group demonstrated a statistically significant increase in the VD of DCP and CC with respect to the eplerenone group (DCP p = 0.012; CC p = 0.004). A statistically significant reduction with respect to baseline in subfoveal choroidal thickness (SFCT) (p = 0.001 for vPDT group; p = 0.001 for eplerenone group) and in central foveal thickness (CFT) (p = 0.001 for vPDT group; p = 0.001 for eplerenone group) was also found. The SFCT was significantly thinnest in the PDT group with respect to the eplerenone group (p = 0.021)., Conclusion: OCTA allowed us to study retinal and choriocapillary vascular changes in patients with CSC treated with vPDT and eplerenone.

Purpose: To evaluate the radial peripapillary vascular plexus of a cavitary congenital optic disc anomaly in a young patient with recessive autosomal metaphyseal acroscyphodysplasia using optical coherence tomography angiography (OCTA)., Methods: Observational case report., Results: A 17-year-old man, with diagnosis of metaphyseal acroscyphodysplasia was referred to Eye Clinic for fundus examination and multimodal imaging for retinal epithelium hypertrophy in the right eye. Clinical examination showed cup-shaped metaphyses, short stature, hyperthelorism, and telecanthus. An optic disc coloboma was detected in the right eye on fundus examination. Wide field en-face Optical Coherence Tomography (OCT) showed a hyporeflective area corresponding to the right optic disc coloboma. At OCTA examination, the whole papillary region revealed a rarefaction of the vascular network, while the ganglion cell complex's and retinal fiber layers' parameters were normal in both eyes., Conclusion: The presence of coloboma disc congenital defect linked to embryological abnormalities during the development process could pave the way for a wider understanding of the pathogenesis of metaphyseal acroscyphodysplasia by increasingly framing it as a systemic disease.

Purpose: To evaluate a case of macular retinoschisis associated with a TJP2 mutation in a young woman affected by a Progressive Familiar Intrahepatic Cholestasis (PFIC) using multimodal imaging., Methods: Observational case report., Results: A 35-year-old woman, undergone a liver transplant for PFIC, was referred to Eye Clinic for complete ophthalmological examination and multimodal imaging. Fundus examination showed no significant alterations in both eyes while multicolor image revealed the presence of several cystes in macular region. The autofluorescence image highlighted small areas of iperautofluorescence in macular region. En-face image showed a "coral shape pattern" and structural optical coherence tomography (OCT) revealed foveal hyporeflective cystic spaces in outer and inner nuclear layers, areas of splitting within the nerve fiber layer (schisis areas) and an initial preretinal fibrosis. Lastly, OCT-angiography (OCT-A) demonstrated small perifoveal teleangectasias and slight reduction of the foveal avascular zone area., Conclusion: Multimodal imaging could help to highlight the presence of a rare retinal disorder associated with a gene related systemic disease.

This study describe the optical coherence tomography angiography (OCTA) features of a young patient with Tuberous sclerosis complicated with CNV unilateral macular choroidal neovascularization during the ranibizumab therapy. OCTA scans of macular region of right eye, revealed a dense microvascular network confirming the diagnosis of CNV. After four monthly intravitreal injections, OCTA revealed a decrease of size and activity of CNV. OCTA is a valid, non-invasive, dyeless, and reliable method that could improve the diagnosis and management of CNV in child with Tuberous sclerosis.

Purpose: To compare efficacy of treat and extend (T&E) versus fixed regimen treatment protocols in neovascular age-related macular degeneration (nAMD)., Methods: Randomized clinical trials (RCTs) comparing T&E versus fixed regimen protocols for nAMD were systematically searched. Primary outcome was to compare the mean best corrected visual acuity (BCVA) change in T&E regimen versus fixed regimen. Secondary outcomes were change in the mean optical coherence tomography (OCT) central retinal thickness (CRT) and mean number of injections. Standardized mean difference (SMD) along with 95% confidence intervals (CIs) were calculated. Random-effect models were used for meta-analyses., Results: Four RCTs were included, with a total of 649 and 621 eyes in the T&E and fixed regimen cohort at 12 months, and 267 and 249 eyes at 24 months. Pooled analysis of mean BCVA change included all four RCTs at 12 months and two RCTs at 24 months, showing no difference between the two groups (12-month: SMD = 0.08, 95% CI: -0.20 to 0.35, p  = 0.55; 24-month: SMD = 0.04, 95% CI: -0.13 to 0.21, p  = 0.64). Pooled analysis of OCT CRT change at 12 months included three studies, showing no difference between the two groups (SMD = 0.03, 95% CI: -0.46 to 0.51, p  = 0.91). Pooled analysis of mean injection number included all four RCTs at 12 months and two RCTs at 24 months, showing significant difference between the two groups (12-month: SMD = -1.11, 95% CI: -1.67 to -0.56, p  < 0.001; 24-month: SMD = -1.34, 95% CI: -1.54 to -1.15, p  < 0.001)., Conclusion: A T&E regimen proved as effective as a fixed dosage regimen throughout a 24-month follow-up and with a lower number of injections.

Purpose: To report a case of quiescent choroidal neovascularization (CNV) associated with choroidal nevus using optical coherence tomography angiography (OCTA) during 5 years follow-up., Methods: Observational case report., Results: A 53-year old woman was referred our department with diagnosis of choroidal nevus. At 1 year follow-up, fundus autofluorescence revealed hyper/hypoautofluorescent area, infrared image showed hyperreflective area and enhanced depth imaging OCT presented a shallow pigment epithelium detachment without exudation or hemorrhage. Fluorescein angiography and indocyanine green angiography suspected the presence of CNV. Finally, OCTA confirmed a quiescent CNV with an increased flow area of the vascular lesion without any changes in best-corrected visual acuity. At 20 months and 5 years follow-up, the CNV remained quiescent showing no clinical activity on structural SD-OCT and no changes in CNV morphology on OCTA., Conclusion: This case shows the features of quiescent CNV secondary to choroidal nevus after long-term follow-up. OCTA represents a valid imaging technique that allows to identify the CNV and to monitor its progression over time.

Purpose: To detect the vessel density (VD) of the radial peripapillary capillary (RPC) in eyes affected by pathological myopia with or without a peripapillary intrachoroidal cavitation (PICC) and in eyes with PICC complicated by choroidal neovascularization (CNV), using optical coherence tomography angiography (OCTA)., Methods: We prospectively enrolled highly myopic patients from January 2016 to December 2019 at the Eye Clinic of the University of Naples "Federico II." We divided included patients into three groups: group 1 including patients with PICC complicated by CNV; group 2 including patients with PICC without complications; group 3 including patients with high myopia without PICC and CNV. One-way analysis of variance (ANOVA) followed by Bonferroni post hoc analysis was used to evaluate differences in VD of radial peripapillary capillary (RPC) in papillary whole, peripapillary regions and its sectors among the three groups., Results: We enrolled 12 highly myopic eyes with PICC complicated by CNV, 21 highly myopic eyes with PICC without CNV and 23 highly myopic eyes without PICC. The myopic eyes with PICC revealed a statistically significant reduction in VD of the RPC comparing to the other groups ( p  < 0.001), especially in eyes affected by myopic PICC complicated by CNV ( p  < 0.001). These results were similar analyzing the VD in different sectors of the peripapillary region among the three groups ( p  < 0.001)., Conclusion: OCTA detects the changes in peripapillary vascular density of highly myopic eyes. We demonstrated that the RPC vasculature is significantly influenced by the presence of PICC, especially in myopic eyes developing a CNV.

Purpose: Usher syndrome (USH) is an autosomal recessive disorder characterized by congenital sensorineural hearing impairment and retinitis pigmentosa. Classification distinguishes three clinical types of which type I (USH1) is the most severe, with vestibular dysfunction as an added feature. To date, 15 genes and 3 loci have been identified with the USH1G gene being an uncommon cause of USH. We describe an atypical USH1G -related phenotype caused by a novel homozygous missense variation in a patient with profound hearing impairment and relatively mild retinitis pigmentosa, but no vestibular dysfunction., Methods: A 26-year-old female patient with profound congenital sensorineural hearing loss, nyctalopia and retinitis pigmentosa was studied. Audiometric, vestibular and ophthalmologic examination was performed. A panel of 13 genes was tested by next-generation sequencing (NGS)., Results: While the hearing loss was confirmed to be profound, the vestibular function resulted normal. Although typical retinitis pigmentosa was present, the age at onset was unusually late for USH1 syndrome. A novel homozygous missense variation (c.1187T>A, p.Leu396Gln) in the USH1G gene has been identified as causing the disease in our patient., Conclusions: Genetic and phenotypic heterogeneity are very common in both isolated and syndromic retinal dystrophies and sensorineural hearing loss. Our findings widen the spectrum of USH allelic disorders and strength the concept that variants in genes that are classically known as underlying one specific clinical USH subtype might result in unexpected phenotypes.

This study describes the optical coherence tomography and optical coherence tomography angiography features of three eyes of three patients affected by contractile morning glory syndrome. Optical coherence tomography angiography scans of the peripapillary retina revealed a dense microvascular network without any vascular difference between the superficial vascular plexus and the deep vascular plexus around the optic nerve. These optical coherence tomography angiography findings confirm that the contractile movement could be due to the presence of an autonomic cholinergic muscular mechanism in the posterior part of the globe. In fact in our cases, the contractile movement seemed to be induced by massage of the eyeball. Optical coherence tomography angiography is a valid, non-invasive, dyeless, and reliable method that could shed light on the pathogenesis of this rare disease of the optic disk.

Purpose: To evaluate choriocapillary vascular density changes in choroidal neovascularization (CNV) type 2 associated with choroidal nevus using optical coherence tomography angiography (OCT-A) before and after anti-VEGF treatment., Methods: Observational case report., Results: A 25-year-old woman, was referred to Eye Clinic and presented a decreased visual acuity in right eye. Fundus examination showed a pigmented lesion located in macular region associated with subretinal hemorrhages adjacent to the mass in right eye. The diagnosis of choroidal nevus-associated with CNV was made and confirmed by spectral domain (SD)-OCT, OCT-A, and ultrasound examination. Two monthly ranibizumab intravitreal injections (IVT) were performed and a regression of the activity of neovascular lesion was noted. OCT-A showed a reduction of choriocapillaris vessel density (VD) before the treatment and an increase of VD after IVT injections., Conclusion: OCT-A could be a non-invasive and diagnostic tool to evaluate choriocapillaris vascular changes after IVT injections in CNV associated with choroidal nevus.

Purpose:: To describe the optical coherence tomography angiography characteristics of myopic patients with choroidal neovascularization secondary to pathologic myopia during ranibizumab therapy., Methods:: Nineteen patients were enrolled in this prospective study (13 females, 6 males, mean age 55.25 ± 9.63 years) for a total of 20 eyes examined (14 right eyes, 6 left eyes). Images were analyzed independently by two examiners., Results:: Mean follow-up was 5.75 ± 1.88 months, with a mean intravitreal injections of 1.90 ± 0.44. Mean best-corrected visual acuity at baseline was 0.39 ± 0.18 logMAR versus 0.26 ± 0.16 logMAR 6 months after treatment. The neovascular area (Z = -2.091, p = 0.037) was significantly reduced after treatment, whereas vessel density was not (Z = -1.848, p = 0.065). Moreover, the best-corrected visual acuity was increased (Z = -3.055, p = 0.002). Neovascular area was significantly correlated with best-corrected visual acuity, at both baseline and follow-up (p < 0.05)., Conclusion:: Our data suggest that optical coherence tomography angiography is a reproducible non-invasive examination with which to monitor changes in the neovascular area in patients with pathologic myopia treated with ranibizumab.

Purpose: To evaluate improvement in best-corrected visual acuity (BCVA) and the reduction of central retinal thickness (CRT) in patients with macular edema secondary to retinal vein occlusion (RVO) after intravitreal bevacizumab (IVB) injection combined with macular grid photocoagulation., Methods: A retrospective study of 54 consecutive eyes with macular edema associated with RVO. The BCVA and CRT, recorded with spectral-domain optical coherence tomography, were evaluated at baseline and 1, 3, 6, and 12 months after treatment onset. Intravitreal bevacizumab injection was administered at baseline, and macular grid photocoagulation 15 days later. During follow-up, additional IVB, at 1-month intervals, was administered if persistent or recurrent macular edema was observed on optical coherence tomography., Results: Best-corrected visual acuity was improved and CRT was reduced at 1, 3, 6, and 12 months of follow-up. Compared with initial values, the final CRT was significantly reduced in patients with branch RVO (35 patients) and in patients with central RVO (19 patients) (p<0.05). Also, BCVA was significantly improved in both groups of patients (p<0.05)., Conclusions: Intravitreal bevacizumab injection combined with macular grid photocoagulation reduces recurrent macular edema associated with branch RVO and central RVO.

Purpose: To report the functional and anatomic features of a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE)., Methods: Case report., Results: A 30-year-old man presented with a decrease of visual acuity that started a few days earlier. Fundus examination showed multiple, subretinal, white-yellowish lesions at the posterior pole in both eyes, suggesting APMPPE. Symptoms and clinical signs resolved within a few weeks, leaving well-defined areas of atrophy of the retinal pigment epithelium (RPE)., Conclusions: This case report emphasizes the role of the RPE in the pathogenesis of APMPPE.