The role of choriocapillaris vessel density in the pathogenesis of macular neovascularization associated with choroidal osteoma.

PURPOSE: To detect the vessel density of choriocapillaris (CC) vascular network in eyes affected by choroidal osteoma and in eyes complicated by macular neovascularization (MNV), using optical coherence tomography angiography (OCTA). METHODS: In this retrospective study, twenty-eight eyes of 28 patients were divided into three groups: group 1 including patients with calcified choroidal osteoma, group 2 including patients with decalcified choroidal osteoma, and group 3 including patients with decalcified choroidal osteoma complicated by MNV. OCTA analyzed the vessel density of CC in these lesions localized in the peripapillary region. RESULTS: We enrolled 12 eyes with calcified choroidal osteoma, 11 eyes with decalcified choroidal osteoma, and 5 eyes with decalcified choroidal osteoma complicated by MNV. The eyes with decalcified choroidal osteoma and MNV revealed a statistically significant reduction in vessel density of the CC respect to the other groups (p < 0.001). Moreover, the vessel density of CC in decalcified choroidal osteoma was significantly reduced compared to calcified choroidal osteoma (p < 0.001). CONCLUSIONS: OCTA allowed a quantitative evaluation of choriocapillaris vessel density in choroidal osteoma, in order to detect the changes of this vascular network, which could lead to the development of MNV. Therefore, OCTA could be a new diagnostic tool in the clinical management of the choroidal osteoma. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT05342324.

The role of quantitative deep capillary plexus in the pathogenesis of type 3 macular neovascularization: an optical coherence tomography angiography study.

PURPOSE: To quantitatively investigate the role of deep capillary plexus (DCP) in patients affected by type 3 macular neovascularization (MNV), compared to patients with reticular pseudodrusen (RPD) eyes and healthy controls, using optical coherence tomography angiography (OCTA). METHODS: In this prospective observational study, a total of seventy-eight eyes of 78 patients were included. Group 1 consisted of 40 eyes of 40 patients with stage 1 of type 3 MNV (22 males, 18 females, mean age 73.7, SD ± 6.60) and group 2 included 38 eyes of 38 patients with RPD (17 males, 21 females, mean age 73.2, SD ± 4.55). The control group included 40 eyes of 40 healthy subjects (20 males, 20 females, mean age 71.4, SD ± 6.36 years). We evaluated the retinal vessel density (VD) of superficial capillary plexus (SCP) and deep capillary plexus (DCP) using OCTA. RESULTS: Patients with diagnosis of type 3 MNV showed statistically lower values of VD in DCP with respect to controls and to RPD group (p < 0.001), while there were no statistical differences between RPD and control group in macular region. No significant differences in VD of SCP were detected among the three study groups. CONCLUSION: OCTA provides a reproducible, non-invasive detailed quantitative analysis of retinal vascular features and changing in early-stage type 3 MNV patients, which allowed to shed the light on the main role of DCP ischemia in the development of type 3 MNV.

Dark halo, a new biomarker in macular neovascularization: comparison between OCT angiography and ICGA-a pilot prospective study.

PURPOSE: To compare optical coherence tomography angiography (OCTA) and indocyanine green angiography (ICGA) in terms of reliability in detecting dark halo in patients affected by age-related macular degeneration (AMD) complicated with type 1 macular neovascularization (MNV). METHODS: Eighty-nine eyes of 89 patients were analyzed at the University of Naples Federico II between January 2018 and October 2021. Each patient underwent a complete ophthalmological evaluation including fluorescein angiography, ICGA, spectral domain optical coherence tomography (SD-OCT), and OCTA. OCTA and ICGA images of dark halo were compared. The paired Student's test and intraclass correlation coefficients were used to evaluate the differences in dark halo measurements between OCTA and ICGA images. RESULTS: Thirty-six eyes of 36 patients were included in this prospective study. Dark halo area was significantly larger in OCTA than in ICGA (1.49 ± 1.8 mm2 vs. 0.54 ± 0.5 mm2; p = 0.001). Moreover, the agreement between the two types of devices for measuring dark halo areas was poor, with a low intraclass coefficient correlation (0.397). CONCLUSION: OCTA could be a useful and necessary tool to investigate dark halo in neovascular AMD due to its ability to visualize the areas of reduced vessel density around MNV in greater detail compared to ICGA. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT05108285.

Correlation between the optic nerve pial diameter and radial peripapillary vascular changes in primary open-angle glaucoma.

PURPOSE: To assess the optic nerve pial diameter (ONPD) in patients with primary open-angle glaucoma (POAG) using standardized A-scan ultrasound and to evaluate the correlation between the ONPD and structural, vascular optic nerve head features and visual field parameters in glaucomatous eyes. METHODS: In this prospective study, we enrolled 126 eyes of 63 POAG patients and 124 eyes of 62 healthy controls. In all subjects, the ONPD was evaluated by means of A-scan ultrasound. Spectral domain (SD)-OCT was used to assess ganglion cell complex (GCC), retinal nerve fiber layer (RNFL), thicknesses, and the optic nerve head (ONH) morphology. OCTA measured the vessel density (VD) of radial peripapillary capillary (RPC) plexus. RESULTS: The ONPD showed a statistically significant reduction in POAG group with respect to controls (p < 0.001). SD-OCT and OCTA parameters showed a significant impairment in patient group with respect to controls (p < 0.001). The ONH analysis revealed significantly lower values in rim area (p = 0.009) and an increased cup-to-disc area ratio (p = 0.013) and cup volume (p < 0.001) in patients with respect to controls. Significant correlations were shown in POAG group between ONPD and RPC plexus (p = 0.006). Moreover, significant correlation was also found between ONPD and structural SD-OCT parameters (p = 0.001) and between ONPD and visual field parameters (p = 0.001). CONCLUSIONS: The standardized A-scan ultrasound measurements of the ONPD showed a significant correlation with structural and vascular glaucomatous changes measured by means of SD-OCT and OCTA. These results confirm the diagnostic reliability of the ultrasound evaluation in glaucoma optic neuropathy.

Radial peripapillary vessel density as early biomarker in preperimetric glaucoma and amnestic mild cognitive impairment.

PURPOSE: To investigate the vessel density (VD) of the radial peripapillary capillary (RPC) plexus in patients affected by preperimetric glaucoma (PPG), amnestic mild cognitive impairment (aMCI) and in a healthy control group using optical coherence tomography angiography (OCTA) in order to clarify the pathogenetic mechanisms of these neurodegenerative diseases. METHODS: In this prospective study, we studied 54 eyes of 54 patients with PPG, 54 eyes of 54 patients with aMCI and 54 healthy controls. All subjects underwent structural spectral domain optical coherence tomography (SD)-OCT to assess the ganglion cell complex (GCC) and the retinal nerve fibre layer (RNFL). OCTA was used to evaluate the VD of the RPC in different regions (whole image, inside disc and peripapillary). RESULTS: The PPG and aMCI groups showed a statistically significant reduction in SD-OCT and parameters with respect to controls (p < 0.001). No statistically significant difference was found in GCC and RNFL parameters between the two study groups (p > 0.05). At OCTA examination, PPG and aMCI patients exhibited a statistically significant reduction in the VD of the RPC in whole image, inside and peripapillary regions compared to healthy controls (p < 0.001). When comparing the two study groups, the OCTA parameters were significantly impaired in PPG with respect to aMCI patients. Significant correlations were found between structural OCT and OCTA parameters in PPG and aMCI groups (p < 0.05). CONCLUSIONS: RPC vessel density could represent a helpful and sensible biomarker to identify early retinal microvascular changes in PPG and MCI in order to better understand the vascular pathophysiological mechanisms involved in these neurodegenerative diseases.

Structural and Vascular Changes of the Choroid in Polypoidal Choroidal Vasculopathy after Intravitreal Anti-VEGF Therapy.

INTRODUCTION: This study aimed to identify the changes in vessel density (VD) of the choriocapillaris (CC) and in subfoveal choroidal thickness (SFCT) and to evaluate their correlation with functional response after 3 monthly intravitreal injections of ranibizumab (loading phase [LP]) in patients affected by polypoidal choroidal vasculopathy (PCV). METHODS: A total of 30 eyes of 30 PCV patients and 30 eyes of 30 healthy subjects as the control group were enrolled in this prospective study. The best-corrected visual acuity (BCVA) was measured at baseline and after 1 month from the third intravitreal injection in each patient. The VD of CC was evaluated in the macular area by means of optical coherence tomography angiography (OCTA). Central macular thickness (CMT) and SFCT were analyzed by enhanced depth imaging (EDI)-OCT. RESULTS: The VD of CC showed statistically lower values in PCV patients at baseline with respect to after LP and normal eyes (p < 0.001). CMT and SFCT revealed a statistically significant reduction after LP (p < 0.001). Multiple regression analysis revealed a significant negative correlation between the reduced SFCT and CMT at baseline and the improvement of BCVA after LP (p < 0.05). CONCLUSION: The close relationship between the thinner SFCT and better visual outcome after LP reveals the role of the EDI-OCT assessment of the choroid as a predictive biomarker of functional response to anti-VEGF therapy. This tool could provide a quantitative evaluation of structural features of the choroid avoiding mistakes of evaluation at OCTA.

Peripapillary vascular density in resolved non-arteritic anterior ischemic optic neuropathy: colocalization between structural and vascular parameters.

INTRODUCTION/AIMS: Non-arteritic anterior ischemic optic neuropathy (NAION) is an acute infarction of the prelaminar anterior optic disc, resulting from the occlusion of posterior ciliary arteries. Here, we evaluated the correlation between structural and vascular features in a case of resolved NAION. METHODS: Observational case report. RESULTS: A 50-year-old male patient was referred at an Eye clinic due to an altitudinal visual field defect in the left eye, occurred 9 months before. Fundus examination was unremarkable, while structural SD-OCT reveals retinal nerve fiber layer (RNFL), ganglion cell complex (GCC) thinning associated with reduction of peripapillary vascular density at OCT Angiography. DISCUSSION: We found a precise spatial correlation among RNFL and GCC thinning, peripapillary vascular reduction and visual field defects. This case demonstrates that OCTA represents a new, valid and non-invasive imaging technique in the diagnosis and follow-up of NAION, even after the resolution of the acute phase, in the absence of clinical signs at fundus examination.

Optical coherence tomography angiography findings in Huntington's disease.

OBJECTIVES: To evaluate the retinal and choriocapillaris vascular networks in macular region and the central choroidal thickness (CCT) in patients affected by Huntington disease (HD), using optical coherence tomography angiography (OCTA) and enhanced depth imaging spectral-domain OCT (EDI SD-OCT). METHODS: We assessed the vessel density (VD) in superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillaris (CC) using OCTA, while CCT was measured by EDI SD-OCT. RESULTS: Sixteen HD patients (32 eyes) and thirteen healthy controls (26 eyes) were enrolled in this prospective study. No significant difference in retinal and choriocapillaris VD was found between HD patients and controls while CCT turned to be thinner in patients respect to controls. There were no significant relationships between OCTA findings and neurological parameters. CONCLUSION: The changes in choroidal structure provide useful information regarding the possible neurovascular involvement in the physiopathology of HD. Choroidal vascular network could be a useful parameter to evaluate the vascular impairment that occurs in this neurodegenerative disease.

Multimodal Imaging in Choroidal Metastasis.

BACKGROUND: Choroidal metastasis represents the most common malignant intraocular tumours. OBJECTIVES: The objective of this study is to detect the structural and vascular features of choroidal metastasis by multimodal imaging. METHODS: Sixteen eyes of 16 patients with choroidal metastasis were enrolled in this prospective study. The multimodal imaging was performed in all patients: fluorescein angiography, indocyanine green angiography, enhanced depth imaging optical coherence tomography (EDI-OCT), OCT angiography (OCTA), and ultrasonography. RESULTS: The choroidal metastasis was located in the macula region in 9 eyes (57%) and in the extramacular region in 7 eyes (43%). EDI-OCT showed a mean thickness of 950 ± 246 µm, a smooth anterior tumour surface in 5 eyes (31%), and a lumpy bumpy appearance in 11 eyes (69%). The most frequent EDI-OCT features were represented by choriocapillaris thinning (100%), shaggy photoreceptors (82%), subretinal fluid with speckles (69%), subretinal lipofuscin pigment (6%), absence of drusen (100%), optical shadowing (94%), low-internal optical reflectivity (75%), and retinal pigment epithelium alterations (43%). OCTA revealed an absence of intratumoral vascular network in all cases. CONCLUSIONS: The multimodal imaging contributed to greater insights into the anatomical and vascular features of choroidal metastasis. It allows for the collection of useful information to establish an appropriate diagnosis and follow-up.

Optical coherence tomography angiography in nonarteritic anterior ischemic optic neuropathy due to optic nerve head drusen.

Optic nerve head drusen (ONHD) are typically benign hyaline and calcified concretions located within the optic nerve. Initially asymptomatic, they can increase slowly in size and number over time, and they can cause mechanical stress at the axons of the optic nerve and progressively lead to a loss of the visual field and papillary vascular changes. Here, we reported a rare case of a young patient with bilateral ONHD complicated by a nonarteritic anterior ischemic optic neuropathy (NAION) in the right eye. The aim of this case was to evaluate, using optical coherence tomography angiography (OCTA), the vascular network of the papillary region in both eyes in the presence of this ischemic event associated to ONHD. At OCTA examination, the whole papillary region revealed a general rarefaction of the vascular network, but it turned out to be greater in the right eye affected by nonarteritic anterior ischemic optic neuropathy than in fellow eye. Therefore, these findings demonstrated that ONHD may determine significant ischemic events of the optic nerve and OCTA represents a valid and noninvasive tool in the diagnosis and in the pathogenesis of these rare cases of NAION associated to ONHD in young patients.

Expanding the Clinical and Genetic Spectrum of RAB28-Related Cone-Rod Dystrophy: Pathogenicity of Novel Variants in Italian Families.

The small Ras-related GTPase Rab-28 is highly expressed in photoreceptor cells, where it possibly participates in membrane trafficking. To date, six alterations in the RAB28 gene have been associated with autosomal recessive cone-rod dystrophies. Confirmed variants include splicing variants, missense and nonsense mutations. Here, we present a thorough phenotypical and genotypical characterization of five individuals belonging to four Italian families, constituting the largest cohort of RAB28 patients reported in literature to date. All probands displayed similar clinical phenotype consisting of photophobia, decreased visual acuity, central outer retinal thinning, and impaired color vision. By sequencing the four probands, we identified: a novel homozygous splicing variant; two novel nonsense variants in homozygosis; a novel missense variant in compound heterozygous state with a previously reported nonsense variant. Exhaustive molecular dynamics simulations of the missense variant p.(Thr26Asn) in both its active and inactive states revealed an allosteric structural mechanism that impairs the binding of Mg2+, thus decreasing the affinity for GTP. The impaired GTP-GDP exchange ultimately locks Rab-28 in a GDP-bound inactive state. The loss-of-function mutation p.(Thr26Asn) was present in a compound heterozygosis with the nonsense variant p.(Arg137*), which does not cause mRNA-mediated decay, but is rather likely degraded due to its incomplete folding. The frameshift p.(Thr26Valfs4*) and nonsense p.(Leu13*) and p.(Trp107*) variants, if translated, would lack several key structural components necessary for the correct functioning of the encoded protein.

Vitreous and intraretinal macular changes in diabetic macular edema with and without tractional components.

Diabetic macular edema (DME) is still one of the main causes of visual impairment. Repeated intravitreal injections of ranibizumab are considered the gold standard treatment, but the efficacy in patients with prominent cystic characteristics remains uncertain. In diabetic retinas, the identification of both antero-posterior and, particularly, tangential tractions is crucial to prevent misdiagnosis of tractional and refractory DME, and therefore to prevent poor treatment outcomes. The treatment of tractional DME with anti-VEGF injections could be poorly effective due to the influence of a tractional force. Pars plana vitrectomy (PPV) is a surgical procedure that has been widely used in the treatment of diffuse and refractory DME. Anatomical improvement, although stable and immediate, did not result in visual improvement. PPV with internal limiting membrane (ILM) peeling for the treatment of non-tractional DME in patients with prominent cysts (> 390 µm) causes subfoveal atrophy, defined as "floor effect". Epiretinal tangential forces and intraretinal change evaluation by SD-OCT of non-tractional DME are essential for determining appropriate management.

LATE-ONSET OCULAR HYPERTENSION AFTER VITRECTOMY: A Multicenter Study of 6,048 Eyes.

PURPOSE: To determine the incidence and risk factors for late-onset ocular hypertension (LOH) after vitrectomy. METHODS: From the electronic medical records of consecutive patients who underwent primary vitrectomy, from January 2010 to December 2015, at 5 tertiary vitreoretinal centers in Italy, patient demographics, systemic, ophthalmic, operative, and postoperative data were drawn. Main outcome measure was the presence of LOH, defined as intraocular pressure >21 mmHg detected more than 2 months after vitrectomy on at least 2 consecutive visits. RESULTS: Among 6,048 patients, LOH was found in 294 (4.9%) vitrectomized eyes and in 87 (1.4%) fellow eyes, (chi square; P < 0.001). Multivariable logistic regression showed that significant risk factors for developing LOH included intraoperative triamcinolone use (odds ratio [OR], 7.62; P < 0.001), longer axial length (OR, 1.55; P = 3.023), preoperative higher intraocular pressure (OR, 1.81; P = 0.003), and postvitrectomy pseudophakic/aphakic status (OR, 2.04; P < 0.001). Decision-tree analysis showed that the stronger predictor of LOH was intraoperative triamcinolone use (P < 0.001). Secondary predictors were a preoperative intraocular pressure more than 15 mmHg (P < 0.001) in eyes that use triamcinolone, and postvitrectomy pseudophakic/aphakic status (P = 0.007) in eyes that did not use triamcinolone. CONCLUSION: Late-onset ocular hypertension occurred in 4.9% of vitrectomized eyes. The main risk factors were intraoperative use of triamcinolone and postvitrectomy pseudophakic/aphakic status.

The Relationship between Macular Pigment and Vessel Density in Patients with Type 1 Diabetes Mellitus.

AIM: Macular pigment density and microvascular density on optical coherence tomography angiography (OCTA) were measured in a cohort of type 1 diabetes mellitus (T1DM) patients with retinopathy in the attempt to shed light on the pathophysiology of this condition. METHODS: Eighty-two consecutive eyes of 59 patients with diabetic retinopathy examined at the Eye Clinic of the University of Naples Federico II from November 2016 to April 2017 were enrolled in this prospective study. Eighty normal eyes of 40 age-matched subjects without diabetes mellitus, without a history of glaucoma or evidence of intraocular surgery, and without retinal pathologic features constituted the control group. All patients and controls underwent a complete ophthalmic examination, best corrected visual acuity evaluation according to the ETDRS visual logMAR scale, measurement of intraocular pressure, OCTA, and evaluation of macular pigment. RESULTS: There were no significant age differences between patients and controls. Both macular pigment measurements and vessel density measured by OCTA were significantly lower in patients than in controls. A moderate correlation was found between vessel density in all ETDRS sectors and macular pigment parameters. CONCLUSIONS: There was a reduction in macular pigment and in OCTA vessel density in T1DM patients with retinopathy, which may have prognostic value in determining disease progression.

Characterization of Human Tear Fluid by Means of Surface-Enhanced Raman Spectroscopy.

Tears are exceptionally rich sources of information on the health status of the eyes, as well as of whole body functionality, due to the presence of a large variety of salts and organic components whose concentration can be altered by pathologies, eye diseases and/or inflammatory processes. Surface enhanced Raman spectroscopy (SERS) provides a unique method for analyzing low concentrations of organic fluids such as tears. In this work, a home-made colloid of gold nanoparticles has been used for preparing glass substrates able to efficiently induce an SERS effect in fluid samples excited by a He⁻Ne laser ( λ = 633 nm). The method has been preliminary tested on Rhodamine 6G aqueous solutions at different concentrations, proving the possibility to sense substance concentrations as low as few µ M, i.e., of the order of the main tear organic components. A clear SERS response has been obtained for human tear samples, allowing an interesting insight into tear composition. In particular, aspartic acid and glutamic acid have been shown to be possible markers for two important human tear components, i.e., lactoferrin and lysozyme.

Optical coherence tomography angiography retinal vascular network assessment in multiple sclerosis.

BACKGROUND: Optical coherence tomography (OCT) angiography is a new method to assess the density of the vascular networks. Vascular abnormalities are considered involved in multiple sclerosis (MS) pathology. OBJECTIVE: To assess the presence of vascular abnormalities in MS and to evaluate their correlation to disease features. METHODS: A total of 50 MS patients with and without history of optic neuritis (ON) and 46 healthy subjects were included. All underwent spectral domain (SD)-OCT and OCT angiography. Clinical history, Expanded Disability Status Scale (EDSS), Multiple Sclerosis Severity Score (MSSS) and disease duration were collected. RESULTS: Angio-OCT showed a vessel density reduction in eyes of MS patients when compared to controls. A statistically significant reduction in all SD-OCT and OCT angiography parameters was noticed both in eyes with and without ON when compared with control eyes. We found an inverse correlation between SD-OCT parameters and MSSS ( p = 0.003) and between vessel density parameters and EDSS ( p = 0.007). CONCLUSION: We report a vessel density reduction in retina of MS patients. We highlight the clinical correlation between vessel density and EDSS, suggesting that angio-OCT could be a good marker of disease and of disability in MS.

Macular peeling-induced retinal damage: clinical and histopathological evaluation after using different dyes.

PURPOSE: To describe functional and histopathological findings after macular peeling using different dyes. METHODS: Prospective, randomized, comparative, interventional, and immunohistochemical study. Forty-five eyes from 45 patients with idiopathic epiretinal membrane (ERM) underwent pars plana chromovitrectomy with ERM and inner limiting membrane (ILM) using trypan blue 0.15% + brilliant blue 0.05% + lutein 2% in group 1 (15 eyes), trypan blue 0.15% + brilliant blue 0.025% + polyethylene glycol 3350 4% in group 2 (15 eyes), and indocyanine green 0.05% in group 3 (15 eyes). We evaluated visual acuity (VA) and macular sensitivity (MS) preoperatively, 1, 3, and 6 months after surgery. The expression of glial fibrillary acidic protein (GFAP) and neurofilament protein (NF) was assessed immunohistochemically on the ILMs peeled as markers of glial and neuronal cells. RESULTS: In group 1, both mean VA and MS were significantly better at 1 and 3 months after surgery (P < 0.05), whereas no significant difference was found after 6 months. GFAP and NF expression was significantly lower in group 1 (P < 0.05). CONCLUSIONS: The ERM/ILM peeling is thought to rip off the intraretinal tissue, based on the amounts of GFAP and NF in the specimens. The use of lutein dyes reduces iatrogenic stress to the retinal tissue and allows a faster functional recovery in the first 3 months after surgery, suggesting a less iatrogenic adhesion to the retinal tissue.

Optical Coherence Tomography Angiography in Optic Nerve Drusen.

AIM: To determine the efficacy of optical coherence tomography angiography (OCT-A) in diagnosing optic nerve head flow impairment in patients with optic nerve drusen. METHODS: Patients affected by optic-nerve head drusen (ONHD) attending the Eye Clinic of the Federico II University of Naples were enrolled in this prospective case series between October 2015 and October 2016. Each patient underwent evaluation of best corrected visual acuity (BCVA), Goldman applanation tonometry, slit-lamp biomicroscopy, fundus examination, standard visual-field testing (perimetry), spectral domain (SD)-OCT and OCT-A. RESULTS: Thirteen patients (6 females and 7 males with a mean age of 22.05 ± 7.54 years) with ONHD (19 eyes) were enrolled. Mean BCVA was 0.16 ± 0.21 LogMar and mean intraocular pressure was 15.68 ± 1.66 mm Hg. The control group constituted 16 individuals (24 eyes). Both ganglion cell complex (GCC) and retinal nerve fiber layer (RNFL) parameters were lower in patients than in controls. Similarly, the flow index (U = 134, p = 0.021) and vessel density (U = 90, p = 0.001) were significantly lower in eyes affected by ONHD than in normal eyes. Visual-field parameters did not differ between the 2 groups. GCC parameters were significantly correlated with OCT-A parameters (p < 0.05). No correlation was found between RNFL and OCT-A parameters. CONCLUSIONS: Our data suggest that OCT-A could be an objective method, helpful in the analysis of flow changes in patients with ONHD.

Evaluation of Vascular Changes with Optical Coherence Tomography Angiography after Plaque Radiotherapy of Choroidal Melanoma.

AIM: The purpose of this paper was to evaluate whether optical coherence tomography angiography (OCT-A) can be used to quantify the vascular changes in radiation maculopathy, and changes in the tumor vasculature in eyes treated with plaque radiotherapy for choroidal melanoma. METHODS: In this prospective study, we evaluated 39 Caucasian patients with choroidal melanoma (39 eyes) treated with ruthenium-106 plaque radiotherapy. The patients underwent complete ophthalmic examination, bulbar echography, and OCT-A before and 1 year after treatment. RESULTS: At baseline, the mean best-corrected visual acuity (BCVA) in the affected eyes was 0.35 ± 0.40 logMAR, and the mean tumor thickness was 2.68 ± 0.25 mm at A-scan echography. After treatment, the mean BCVA increased to 0.41 logMAR, the mean tumor thickness decreased to 1.66 ± 0.23 mm, and the tumor basal diameter was significantly reduced (U = 108, p = 0.001). Moreover, the capillary vessel density was significantly lower in all Early Treatment of Diabetic Retinopathy Study sectors, and both the vessel and flow areas were significantly reduced (p = 0.030 and p = 0.001, respectively). CONCLUSIONS: OCT-A is a noninvasive, reliable method with which to quantify the vessel changes in radiation maculopathy and, given the association between vascularization and malignancy, this procedure may be an aid in treatment decision-making and in monitoring the efficacy of treatment.

Multimodal Imaging in Autosomal Dominant Cone-Rod Dystrophy Caused by Novel CRX Variant.

AIM: To characterize by multimodal approach the phenotype of patients from a 3 generations pedigree, affected by autosomal dominant cone-rod dystrophy (CRD), found to carry a novel pathogenic variant in the cone-rod homeobox-containing (CRX) gene. METHODS: Examination of the adult patients included the following tests: visual acuity, multicolour imaging, spectral domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF) and OCT angiography (OCT-A) recordings. In a 2.5-year-old child, cycloplegic refraction, fundoscopy, ocular motility evaluation and electrophysiological exams were performed. Next Generation Sequencing of patients' DNA has been carried out. RESULTS: A novel CRX pathogenic variant has been identified in our patients. The 2.5-year-old child in the third generation was found to have inherited the variant, with no clinical signs of the condition, but electroretinographic abnormalities in the scotopic component. In the adult patients, diffuse atrophy of the retinal pigment epithelium/photoreceptor complex in the macular region was evident at the OCT and FAF, while OCT-A showed choriocapillaris density reduction. CONCLUSIONS: Multimodal study allowed the characterization of a peculiar form of CRD. The novel pathogenic variant seems to have a different effect on the phenotype if compared with a previously described similar one, giving an insight into the pathogenic mechanism of CRX-related retinal dystrophies and offering valuable information that could lead to the development of possible future therapies.