Adult-onset Foveomacular Vitelliform Dystrophy Research Articles

Lack of Response to Intravitreal Ranibizumab Treatment in Adult Onset Foveomacular Vitelliform Dystrophy Complicated with Choroidal Neovascularization: A Case Report

Adult-onset foveomacular vitelliform dystrophy (AOFVD) is a rare disease characterized by accumulation of yellowish deposits in the macula. Rarely, it may be complicated by choroidal neovascularization (CNV). Cases with CNV may be confused with occult CNV in age-related macular degeneration. In our case, we will present the visual and anatomical results of a patient with AOVF-related CNV, in which we administered 3 doses of intravitreal ranibizumab (IVR). A 59-year-old female patient, who attended our clinic with the complaint of decreased vision in both eyes, was diagnosed with AOVF-related CNV in both eyes and was treated with 3 doses of IVR for 3 months. Despite the improvement in visual and anatomical functions 1 month after the first dose, vision decreased, and anatomical functions regressed to the pre-injection state in continued injections. IVR therapy is not an appropriate treatment option in the treatment of AOVF-associated CNV.

Risk Factors for Worsening Morphology and Visual Acuity in Eyes with Adult-Onset Foveomacular Vitelliform Dystrophy

PurposeTo explore clinical risk factors and optical coherence tomography (OCT) features associated with worse visual acuity (VA), progression of disease, choroidal neovascularization (CNV), and atrophy in eyes with adult-onset foveomacular vitelliform dystrophy (AOFVD). DesignSingle-center, retrospective, observational cohort study ParticipantsPatients seen at Duke Eye Center between January 2012 and May 2023 with a diagnosis of AOFVD confirmed via OCT and fundus autofluorescence. MethodsBaseline and final visit images from eyes with AOFVD were examined. Disease stage was assigned, and presence of atrophy or CNV was determined. Clinical and OCT features associated with progression to atrophy and CNV were determined using t-tests and chi-square analysis. Correlation with lower VA was determined using linear regression. Outcome MeasuresAssociation of clinical characteristics and OCT features with worse VA, progression of disease, CNV, and atrophy as determined by independent t-tests, chi-square analysis, and linear regression (p<0.05). Results101 eyes (63 patients) met inclusion criteria for this study, with mean follow-up duration of 48 months (SD 31 months). 51% of eyes progressed beyond baseline staging during follow-up; among baseline stage 1 eyes, incidence of atrophy was 0.068/person-year; incidence of CNV was 0.022/person-year. Risk factors for worse final VA were baseline presence of vitreomacular traction (VMT) (p=0.006), ellipsoid zone attenuation (p=0.024), increased lesion height and width (p<0.001). Predictors of progression include diabetes mellitus (p=0.012), statin use (p=0.031), presence of hyperreflective foci (p=0.012), and increased lesion width and volume (p=0.034, p=0.040). Predictors of atrophy include the baseline presence of VMT (p=0.018), decreased choroidal thickness (p=0.027), and greater maximal height, width, and volume of the lesion (p=0.031, p=0.020, p=0.009, respectively). Lower baseline VA (p=0.031) and increased lesion volume (p=0.042) were associated with CNV. ConclusionsClinical and OCT imaging features at baseline may prove useful in stratifying patient risk for progression, atrophy, CNV, and worse VA. Features such as statin use, diabetes, baseline VA, and laterality should be accounted for. OCT features such as lesion size, VMT, ellipsoid zone attenuation, choroidal thickness and hyperreflective foci may impart greater risk of poor outcomes. Future prospective analysis accounting for the time to development of atrophy and CNV is needed.

Retinal sensitivity and fundus autofluorescence in adult-onset foveomacular vitelliform dystrophy

The present study aimed to compare retinal sensitivity (RS) at each stage and to evaluate the relationship between RS and fundus autofluorescence (FAF) pattern in adult-onset foveomacular vitelliform dystrophy (AOFVD). We retrospectively reviewed 17 eyes of 13 patients with AOFVD. In addition to best-corrected visual acuity (VA), RS within the affected lesion and optical coherence tomography (OCT) measurements were carried out in each participant. All the examined eyes were classified into 4 stages and 3 FAF patterns. RS was superimposed on OCT fundus image and RS within the affected lesion was calculated in each eye. The relationships between visual functions (VA and RS within the affected lesion) and stages and also FAF patterns were analyzed using the linear mixed model. As a result, RS within the affected lesion was significantly associated with FAF pattern, but not with stage. In contrast, VA was correlated with neither stages nor FAF patterns. Our current result suggested that RS within the affected lesion was related to FAF patterns but this was not the case with VA in eyes with AOFVD, demonstrating the usefulness of measuring RS, not only VA, to comprehend the disease status in AOFVD.

The Retinal Phenotype Associated with the p.Pro101Thr BEST1 Variant

To describe the retinal phenotype associated with the p.Pro101Thr BEST1 variant. Retrospective, observational case series. Patients diagnosed with bestrophinopathies in which molecular genetic testing identified the p.Pro101Thr BEST1 as well as healthy carriers among their first-degree relatives. Medical records were reviewed to obtain data on family history and ophthalmic examinations, including retinal imaging. The imaging protocol included OCT and fundus autofluorescence using Spectralis HRA+ OCT (Heidelberg Engineering). Genetic analysis was performed by next-generation sequencing. Results of ophthalmic examinations and multimodal imaging features of retinal phenotypes. The c.301C>A, p.Pro101Thr BEST1 missense variant was identified as the causative variant in 8 individuals (all men) from 5 families, accounting for 13% of cases (8/61) and 10% of pathogenic alleles (9/93) in our cohort of patients affected by bestrophinopathies. Seven individuals (14 eyes) had the variant in heterozygous status: all eyes had a hyperopic refractive error (median spherical equivalent of+ 3.75 diopters [D]) and 4 individuals had a macular dystrophy with mildly reduced visual acuity (median of 20/25 Snellen), whereas the other 3 were asymptomatic carriers. On multimodal retinal imaging, 5 (36%) out of 14 eyes had subclinical bestrophinopathy, 4 (29%) had typical findings of adult-onset foveomacular vitelliform dystrophy (AOFVD), and the remaining 5 (36%) displayed a pattern dystrophy-like phenotype. Follow-up data were available for 6 subjects, demonstrating clinical stability up to 11 years, in both subclinical and clinical forms. An additional patient with autosomal recessive bestrophinopathy was found to harbor the p.Pro101Thr variant in homozygosity. The p.Pro101Thr BEST1 variant is likely a frequent cause of bestrophinopathy in the Italian population and can result in autosomal dominant macular dystrophies with incomplete penetrance and mild clinical manifestations as well as autosomal recessive bestrophinopathy. The spectrum of autosomal dominant maculopathy includes the typical AOFVD and a pattern dystrophy-like phenotype. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

The pseudohypopyon stage in adult-onset foveomacular vitelliform dystrophy.

To gain insight into the pathogenesis of adult-onset foveomacular vitelliform dystrophy (AFVD) via assessment of its pseudohypopyon stage (PHS). Retrospectively, data were collected in a tertiary center from established cohorts of a genetically evaluated AFVD and best vitelliform macular dystrophy (BVMD) eyes in the pseudohypopyon stage. Best-corrected visual acuity (BCVA, LogMAR), lesion characterization, including lesion dimensions, liquefaction areas and patterns (altitudinal or lateral), and ellipsoid zone integrity were analyzed from spectral-domain optical coherence tomography images. Out of 167 eyes of 90 AFVD patients and 56 eyes of 28 BVMD patients, 8 eyes of six AFVD patients and five eyes of four BVMD patients were at the PHS were included. The mean LogMAR BCVA ± SD was 0.21 ± 0.20 and 0.41 ± 0.10 in AFVD and BVMD diseases, respectively (p = 0.13). Seven AFVD eyes (87.5%) demonstrated lateral liquefaction, while all BVMD eyes demonstrated an altitudinal pattern (p = 0.005). Maximal horizontal lesion diameters were 1.41 ± 0.46mm and 2.64 ± 0.77mm in AFVD and BVMD, respectively (p = 0.02). AFVD patients were older (69 ± 14) than BVMD patients (22 ± 13; p = 0.009). The pseudohypopyon stage in AFVD is often characterized by a lateral liquefaction pattern, unlike the altitudinal pattern characterizing BVMD. Age, lesion size, or pathogenesis pathways may underline the different pseudohypopyon stage patterns in AFVD and BVMD.

Optical Coherence Tomography Findings in a Patient with Adult-Onset Foveomacular Vitelliform Dystrophy

We present a case of adult-onset foveomacular vitelliform dystrophy (AFMVD) in a 73-year-old man. Ophthalmoscopy revealed discoloration in both eyes, but typical subretinal depositions of yellowish material were not observed. OCT revealed epiretinal membrane in the right eye. Hyper-reflective subretinal deposits at the level of the retinal pigment epithelium in the left eye. Moreover, hyper-reflective deposits were also detected at the sub-ellipsoid zone and intraretinal layers. Based on these findings, we diagnosed our patient with AFMVD in vitelliruptive phase. OCT was useful in visualizing the location of the vitelliform material in a patient with AFMVD.

Choroidal Vascularity Index in Adult-Onset Foveomacular Vitelliform Dystrophy: A Pilot Study

This pilot study aims to investigate choroidal vascular status in eyes with adult-onset foveomacular vitelliform dystrophy (AOFVD), early age-related macular degeneration (AMD), and age-matched controls. In this retrospective study, choroidal thickness (CT) was measured manually using spectral domain optical coherence tomography images of the fovea, and 500 and 1500 µm from the nasal and temporal regions in the fovea. The horizontal B-scan was imported into Fiji software. Choroidal vascularity index (CVI) and luminal and stromal areas were calculated. A total of 36 eyes from 36 patients, including 18 eyes with AOFVD and 18 eyes with CD, and 16 eyes of healthy subjects were included. CVI was significantly different among subgroups (ANOVA, p = 0.004). Eyes with AOFVD presented a higher CVI (+0.03 ± 0.01, p = 0.001) than eyes with CD and controls (p = 0.03). No differences in CVI were detected between controls and eyes with CD (p = 0.25). AOFVD eyes accounted for the greatest luminal area, particularly significant in comparison with healthy controls (+0.27 ± 0.11, p = 0.02). AOFVD eyes present a greater CVI than eyes with CD and controls. The major choroidal involvement is on the luminal component, further corroborating a possible role of the choroidal vasculature in the pathological manifestations of AOFVD disease.

Vitelliform Distrofiye Sekonder Koroidal Neovaskülarizasyon: Optik Koherens Tomografi Anjiyografi ve Fluoresein Anjiyografi Bulguları

This case series aims to interpret the findings of optical coherence tomography angiography (OCTA) and fluorescein angiography (FA) in detecting choroidal neovascularization (CNV) in juvenile Best vitelliform macular dystrophy (BVMD) and adult-onset foveomacular vitelliform dystrophy (AOFVD). Two patients with BVMD and one patient with AOFVD were evaluated, and color fundus photography, spectral-domain optical coherence tomography, FA, and OCTA were performed. In 4 of 6 eyes, OCTA revealed neovascular formations in the outer retina and choriocapillaris. It is an effective, non-invasive method for showing CNV formation using OCTA in BVMD and AOFVD, and it has aspects that are superior to FA.

Multimodal imaging of hereditary retinal dystrophies (a series of clinical cases)

Multimodal visualization data of inherited retinal degeneration (IRD) on a Mirante platform (Nidek, Japan), used in a number of clinical cases, is compared with the data obtained by electrophysiological diagnostic methods. 4 patients with varying IRD were examined: adult-onset foveomacular vitelliform dystrophy, Stargardt disease, including those with fundus flavimaculatus, and retinitis pigmentosa. Multimodal imaging includes: colour fundus imaging, fundus autofluorescence, retro mode, and optical coherence tomography. Electroretinography was performed using an MBN electroretinograph (Russia), and electrooculography was performed using a RETIscan Science system (Roland Consult, Germany). Using non-invasive retinal imaging methods, specific patterns of inherited dystrophies were shown, which correlated well with the data of electrophysiological research methods. The combination of multimodal imaging on the Mirante platform (Nidek, Japan) in combination with electrophysiological diagnostic methods can be successfully used in complex diagnostics, monitoring of the progression, and evaluation of the results of IRD treatment.

Assessment of retinal vessel density in adult-onset foveomacular vitelliform dystrophy by optical coherence tomography angiography

To evaluate retinal thickness, area of foveal avascular zone (FAZ), flow area and flow density of choriocapillaris, vessel density of both superficial capillary plexus (SCP) and deep capillary plexus (DCP) of eyes with adult-onset foveomacular vitelliform dystrophy (AOFVD) using optical coherence tomography angiography (OCT-A) and compare the results with healthy controls. 17 eyes of 14 patients diagnosed with AOFVD and 17 eyes of 17 healthy subjects were enrolled in this study. All patients underwent a complete ophthalmological examination and a 6 x 6 mm macular OCT-A scanning. Quantiative results of retinal thickness, retinal vessel density of SCP and DCP, FAZ area, flow area and flow density of choriocapillaris were analyzed. No statistically significant differences were noted in the vessel density of the SCP, except for the parafoveal nasal sector (P = 0.048). Similarly, no statistically significant differences were observed in the vessel density of the DCP, except for the parafoveal (P = 0.037) and the parafoveal temporal (P = 0.048) sectors. The choriocapillaris flow area and the flow density were significantly lower in the patients with AOFVD than in the healthy controls (P = 0.001 for both). The mean FAZ area and the retinal thickness measurements were comparable in both groups. Patients with AOFVD show vascular abnormalities that can be detected with OCT-A. OCT-A, as a noninvasive imaging modality, could provide a new perspective for understanding the pathophysiology of AOFVD and could also be useful in the follow-up of these patients and in the management of the disease progression.

Study of vessel density in adult-onset foveomacular vitelliform dystrophy with optical coherence tomography angiography.

To evaluate retinal and choriocapillaris (CC) vessel density in macular region in patients affected by adult-onset foveomacular vitelliform dystrophy (AOFVD) using optical coherence tomography angiography (OCTA) METHODS: A total forty-four right eyes of 44 AOFVD patients (20 females, 24 males, mean age 69.17 ± 11.57 years) divided in 3 stages (vitelliform, pseudohypopyon and vitelliruptive) and 60 normal right eyes of 60 controls (20 females, 40 males, mean age 66.04 ± 6.40 years) were included in this prospective study. We evaluated the vessel density of superficial capillary plexus (SCP), deep capillary plexus (DCP) and CC in different macular areas (whole image, parafovea and fovea). We also analyzed the subfoveal choroidal thickness (SFCT) with Enhanced Depth Image (EDI)-OCT. The vessel density of SCP and of DCP did not differ between patients and controls in all macular sectors. The vessel density of CC was lower in patients compared to controls but the difference turned out to be statistically significant only in foveal region (p < 0.001). We found that the foveal vessel density of the CC was lower in vitelliform stage and significantly increased in vitelliruptive stage (p = 0.031). At EDI-OCT, the SFCT revealed a statistically significant increase in patients compared to controls (p = 0.002) whereas it was similar in the different stages of this dystrophy (p = 0.276). In vitelliform stage of AOFVD, OCTA and EDI-OCT can be useful to avoid mistakes of evaluation, due to the masking effect artifact. OCTA provides us a better understanding of the vascular role in the physiopathology of the macular diseases.

Optical Coherence Tomography of Adult-Onset Foveomacular Vitelliform Dystrophy Concomitant with Diabetic Cystoid Macular Edema

Optical Coherence Tomography of Adult-Onset Foveomacular Vitelliform Dystrophy Concomitant with Diabetic Cystoid Macular Edema

Current research status of optical coherence tomography angiography in hereditary retinochoroidal degeneration

OCT angiography (OCTA) is a fast, noninvasive and quantifiable new technique, which is especially suitable for long-term follow-up in patients with hereditary retinochoroidal degeneration, such as retinitis pigmentosa, Best vitelliform macular dystrophy, adult onset foveomacular vitelliform dystrophy, doyne honeycomb retinal dystrophy, choroideremia and Stargardt disease. During the follow-up, clinicians can find the subtle signs that explain disease development from the blood flow imaging, quantitatively describe the vascular density, timely detect and treat choroidal neovascularization. It is significant to explore the etiology and monitor the course of these diseases. With the development of more treatments for these diseases, OCTA parameters can also be used as indicators to evaluate and compare different therapeutic effects. In the future, more quantitative indicators of OCTA will be applied to evaluate the course of hereditary retinochoroidal degeneration, and provide valuable basis for early diagnosis and treatment. Key words: Tomography, optical coherence; Retinal diseases/diagnosis; Choroid diseases/ diagnosis; Eye Diseases, Hereditary; Review

ADULT-ONSET FOVEOMACULAR VITELLIFORM DYSTROPHY EVALUATED BY MEANS OF OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY: A Comparison With Dry Age-Related Macular Degeneration and Healthy Eyes.

To investigate alterations of superficial and deep retinal vascular densities, as well as of choroidal thickness, in patients affected by adult-onset foveomacular vitelliform dystrophy (AOFVD). A total of 22 eyes (15 patients) affected by AOFVD were recruited in the study. Furthermore, 20 eyes of 20 healthy subjects and 20 eyes of 18 patients affected by intermediate dry age-related macular degeneration (AMD) were enrolled. All patients underwent a complete ophthalmologic examination, including optical coherence tomography angiography. Outcome measures were superficial vessel density, deep vessel density, and choroidal thickness. Parafoveal superficial vessel density was increased in patients with AOFVD compared with the AMD group (50.6 ± 4.3% and 46.3 ± 4.3%, respectively, P = 0.016). Parafoveal deep vessel density was 57.9 ± 6.4% in patients with AOFVD, 52.2 ± 3.8% in patients with AMD, and 52.7 ± 6.0% in healthy controls (P = 0.006 and P = 0.035, respectively, after comparison with the AOFVD group). We demonstrated that both superficial and deep vessel densities were significantly increased in patients with AOFVD, after the comparison with intermediate patients with AMD. These findings suggest that the pathogenic mechanisms in AOFVD are different from those in AMD and that optical coherence tomography angiography could be useful in differentiate early stages of these two diseases.

Adult-onset vitelliform macular dystrophy: case report

Adult-onset foveomacular vitelliform dystrophy is a rare disease. It shares heritance features with Best disease. Its onset is in the 3rd and 5th decade, and it is characterized by subretinal deposition of yellowish material in the foveal area. Visual acuity ranges from 20/25 to 20/50, which can be seen in routine examination. Patient remains with good visual function throughout theirs lives. Typically the electro-oculogram may be normal or subnormal. We present a case of adult-onset vitelliform macular dystrophy, diagnosed in a patient with complaint of bilateral blurred vision.

Understanding Optical Coherence Tomography (OCT) is Crucial in the Diagnosis of Adult-Onset Foveomacular Vitelliform Dystrophy (AOFVD)!

Understanding Optical Coherence Tomography (OCT) is Crucial in the Diagnosis of Adult-Onset Foveomacular Vitelliform Dystrophy (AOFVD)!

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN ADULT-ONSET FOVEOMACULAR VITELLIFORM DYSTROPHY.

To determine the ability of optical coherence tomography angiography (OCTA) to detect choroidal neovascularization (CNV) in the pseudohypopyon stage of adult-onset foveomacular vitelliform dystrophy. Prospective case series of eight consecutive patients with adult-onset foveomacular vitelliform dystrophy with at least one eye in the pseudohypopyon stage (a total of 14 eyes). Patients were assessed with spectral domain OCT, flourescein angiography, and OCTA. Main outcome measures were the presence or absence of CNV and any unifying patterns that could be identified on OCTA for adult-onset foveomacular vitelliform dystrophy. One (12.5%) of eight eyes in the pseudohypopyon stage had CNV on OCTA, without definitive evidence of CNV on flourescein angiography. Twelve of 14 eyes (86%) had OCTA segmentation errors, giving the false appearance of deep capillary plexus drop out. All 14 eyes (100%) had blockage of flow signal under the vitelliform lesion on OCTA that presented as artifactual loss of flow in the choriocapillaris. Optical coherence tomography angiography may be superior to flourescein angiography in detecting CNV in adult-onset foveomacular vitelliform dystrophy, especially in the pseudohypopyon stage. There are common artifacts that must be considered when analyzing vitelliform lesions with OCTA, including segmentation errors and inability to visualize flow under the vitelliform lesion in the choriocapillaris.

QUANTITATIVE ANALYSIS OF OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN ADULT-ONSET FOVEOMACULAR VITELLIFORM DYSTROPHY

To quantify the foveal avascular zone area at superficial capillary plexus (SCP) and deep capillary plexus (DCP) and the global, parafoveal, and perifoveal vessel densities at SCP, DCP, and choriocapillaris using optical coherence tomography angiography in patients affected by adult-onset foveomacular vitelliform dystrophy (AOFVD). Twenty eyes of 20 consecutive patients (10 females, 50%) with AOFVD and 20 eyes of 20 healthy controls presenting at the Department of Ophthalmology of San Raffaele Hospital, Milan, Italy were enrolled. All patients underwent a complete ophthalmic examination, including 3-mm × 3-mm optical coherence tomography angiography centered on the macula. The vessel density was calculated in the three plexuses (i.e., SCP, DCP, and choriocapillaris) by mean of image binarization, and foveal avascular zone area was manually outlined at SCP and DCP with ImageJ software. In the SCP, patients with AOFVD disclosed a significant reduction of global (P < 0.001), parafoveal (P = 0.0017), and perifoveal (P = 0.0019) vessel density. In the DCP, parafoveal vessel density was higher in patients with AOFVD (P = 0.0026), whereas no significant difference was appreciated for both the global image (P = 0.5) and the perifoveal area (P = 0.5). Patients with AOFVD showed less dense choriocapillaris (P = 0.012) and perifoveal circle (P = 0.0152), whereas no difference was observed in the perifoveal zone (P = 0.07). Foveal avascular zone area was significantly enlarged at the DCP (P = 0.0184), but not at the SCP. Patients with AOFVD have quantitative vascular alterations in all vascular layers.

Clinical and imaging findings of pattern dystrophy subtypes; Diagnostic errors and unnecessary treatment in clinical practice

To evaluate the clinical and multimodal imaging findings of various pattern dystrophy (PD) subtypes and report the initial misdiagnosis rate of PD patients resulting in unnecessary treatment in actual clinical practice. Retrospective, observational study. Forty eyes of 24 patients with PD were included. The distribution of PD subtypes, optical coherence tomography (OCT) and fundus autofluorescence (FAF) findings, initial misdiagnoses, revised diagnoses, duration between misdiagnosis and revised diagnosis, and unnecessary treatments administered were evaluated over this time-period. Twenty-eight eyes (70%) showed adult-onset foveomacular vitelliform dystrophy, 6 eyes (15%) showed butterfly PD (BPD), 4 eyes (10%) showed reticular PD, and 2 eyes (5%) showed PD simulating fundus flavimaculatus and BPD mixed type PD. Most of the patients showed various types of hyperreflective material in the subretinal space on OCT, and hyperautofluorescence on FAF imaging. Eighteen eyes (45%) had a true PD diagnosis initially, whereas 22 (55%) of them were misdiagnosed as age-related macular degeneration, central serous chorioretinopathy, or non-specific RPE change. The mean duration between the initial and revised diagnosis was 18.7±16.8 months. In addition, 5 eyes in the misdiagnosed group underwent intravitreal anti-vascular endothelial growth factor treatment during this period. Pattern dystrophies are a heterogeneous group of macular disorders which may mimic several macular diseases. By knowing the multimodal imaging findings, especially the distinctive FAF findings of the PDs, we may easily diagnose the disease and save our patients from unnecessary treatments.

Quantitative changes in flow density in patients with adult-onset foveomacular vitelliform dystrophy: an OCT angiography study.

To quantitatively compare the flow density, the retinal thickness, and the area of the foveal avascular zone (FAZ) between patients with adult-onset foveomacular vitelliform dystrophy (AOFVD) and a healthy controls. Thirteen eyes (eight patients) with AOFVD and 13 matched eyes (13 patients) without any ocular pathology were included in this study. A 6×6mm optical coherence tomography angiography (OCTA) scan was performed for every included eye. The flow density (superficial retinal vascular layer, deep retinal vascular layer and choriocapillary layer), retinal thickness and FAZ (superficial retinal vascular layer and deep retinal vascular layer) were subsequently analyzed. The mean flow density was decreased in the AOFVD patients in all measured vascular layers. The difference from the control group was statistically significant in the parafoveal sector of the deep retinal vascular layer (P=0.02), and a clear trend was found in the superficial retinal vascular layer (P=0.05). Both groups had comparable FAZs in the superficial and deep retinal vascular layers. The retinal thickness values were higher in the fovea (P=0.840) and lower in the parafoveal sectors (P=0.125). The difference was significant in the superior parafoveal sector (P=0.034). Flow densities as measured by OCTA are decreased in the superficial retinal vascular layer and the deep retinal vascular layer in patients with AOFVD. These findings could be helpful for diagnosing and understanding the pathogenesis of this disease.