Photoreceptor Integrity in MEWDS: Longitudinal Structure-Function Correlations.

Purpose: The purpose of this study was to investigate structure-function correlations in multiple evanescent white dot syndrome (MEWDS) using microperimetry (MP) and spectral-domain optical coherence tomography (SD-OCT). Methods: Single-center prospective observational study including 14 eyes from 13 patients with MEWDS monitored over a median of 49.5 days (interquartile range = 29-92 days). Investigations focused on best-corrected visual acuity (BCVA), foveal granularity, and the Photoreceptor Reflectivity Ratio (PRR) as a measure of photoreceptor integrity. MP assessed average retinal threshold sensitivity (RTS) and bivariate contour ellipse area (BCEA) for fixation stability. A linear mixed model was used to test associations and interactions among RTS, time, and clinical variables. A hierarchical linear mixed model was used to analyze structure-function relationships, addressing both individual and location-specific variations. Results: Overall, 2340 MP locations were tested. PRR revealed a transient decrease within 30 days post-presentation, indicative of early photoreceptor disruption, followed by a progressive increase, signaling recovery. Significantly lower foveal sensitivity (RTS = 14.8 ± 7.4 vs. 22.5 ± 4.4 decibel [dB], P = 0.04) and increased fixation spread (63% BCEA = 1.26 ± 0.97 vs. 0.48 ± 0.35 deg2, P = 0.06) were noted in eyes with foveal granularity compared to those without. A significant increase in RTS was demonstrated over time (0.066 dB/day, P < 0.001), with a central-to-peripheral gradient of improvement. The interaction between follow-up time and baseline BCVA (P < 0.001) indicated more rapid improvement in eyes with worse initial vision. There was a robust, nonlinear association between PRR and RTS across all tested locations (P < 0.001), becoming asymptotic for sensitivity losses exceeding 20 dB. Conclusions: Photoreceptor reflectivity accurately aligned with visual function in MEWDS on longitudinal examinations. The central-to-peripheral gradient of improvement may suggest specific vulnerabilities underlying the area around the disc.

Ocular complications of SAVI: A unique case of bilateral uveitis and retinal vasculitis.

Purpose: To describe a case of bilateral panuveitis in a patient with Stimulator of Interferon Genes (STING)-Associated Vasculitis with Onset in Infancy (SAVI). Observations: A 45-year-old patient diagnosed with SAVI presented bilateral panuveitis and uncontrolled secondary intraocular hypertension due to structural complications from uveitis. Multimodal imaging revealed the presence of intraretinal fluid and bilateral vasculitis. The patient was started with systemic methotrexate. Conclusions and importance: This case is essential to characterize ocular involvement in patients with SAVI. Awareness of these ocular manifestations is crucial for timely management and improvement of visual prognosis.

Multimodal Imaging of Vitreo-Retinal Lymphoma: A Comprehensive Review.

PURPOSE: Vitreoretinal lymphoma (VRL) is a rare lymphoma affecting the vitreous and the retina. Clinical diagnosis is challenging and often delayed and may lead to aggravated prognosis. This study aims to review multimodal imaging findings in VRL. METHODS: We performed a comprehensive narrative review of the multimodal imaging findings that might be useful in the detection of VRL lesions. RESULTS: The most frequent ocular manifestations of VRL are vitritis, and retinal and sub-retinal Pigmented Epithelium (RPE) infiltrations. Color Fundus Photography (CFP) detects vitreous haze, optic nerve, retinal and sub-RPE infiltration. Ultra-wide field imaging allows visualization of different patterns of vitreous haze and monitoring of VRL evolution through the detection of chorio-retinal atrophy (CRA). Fundus Autofluorescence shows granular hypo- and hyper-autofluorescent pattern. Optical Coherence Tomography (OCT) reveals vitreous cells, vertical hyper-reflective lesions and sub-RPE infiltrates. Fluorescein Angiography (FA) shows hypo or hyperfluorescent round lesions at the late stages of the examination, while Indocyanine Green Angiography (ICGA) detects round areas of focal hypo-fluorescence in the early phases that gradually enlarge in the late phases. B-scan ultrasonography detects vitreous opacities and homogeneous hyperreflective corpuscular material in the vitreous, and is a strongly recommended tool in suspecting VRL and is particularly useful when vitreous haze is impeding retinal examination. CONCLUSION: Diagnostic vitrectomy with cytopathological analysis remains the gold standard for VRL diagnosis, however multimodal imaging allows the identification of suggestive retinal and vitreal lesions for early suspicion, diagnosis, and treatment and monitoring disease progression and response to treatment.

Anterior segment involvement in vitreoretinal lymphoma: clinical manifestations, molecular findings and in vivo confocal microscopy.

BACKGROUND: Intermediate and posterior manifestations of vitreoretinal lymphoma (VRL) are well characterised. However, there is limited information on anterior segment involvement in VRL. This study aimed to describe the anterior manifestations of VRL, and their association with molecular testing. METHODS: Retrospective analysis of patients with biopsy-proven VRL. Study variables included anterior segment manifestations, findings from slit-lamp photos and in vivo confocal microscopy (IVCM) when available. MYD88 L265P mutation and cytology in the aqueous humour, retinal and systemic findings were also analysed. RESULTS: The analysis included 108 eyes of 55 VRL patients. Anterior segment involvement was present in at least one visit in 55 eyes (51%) of 33 patients (60%); it included keratic precipitates (dendritiform with branching and irregular margins in 33 eyes, dust-like in 16 eyes and large granulomatous in 12 eyes), cells in the anterior chamber (51 eyes) and posterior synechiae (2 eyes). IVCM was available for 41 eyes and showed different morphologies of keratic precipitates, including floral, spikes and mulberry patterns (66%, 56% and 20%, respectively). MYD88 L265P mutation in the aqueous humour was detected in 10/21 (48%) eyes with no anterior segment involvement and 24/37 (65%) eyes with anterior segment involvement. CONCLUSIONS: Anterior segment manifestations are often present in VRL and include dendritiform and dust-like keratic precipitates. IVCM in VRL can identify different patterns associated with keratic precipitates. MYD88 L265P mutation in the aqueous humour of VRL patients can also be found in eyes without significant anterior segment involvement.

A comprehensive overview of diagnosis, imaging and treatment of vitreoretinal lymphoma.

Vitreoretinal lymphoma (VRL) is a rare B-cell intraocular neoplasia characterized by poor long-term prognosis and lack of effective therapies. It mainly involves the vitreous humor, the retina, and the retinal pigment epithelium (RPE), although anterior segment involvement can occur. VRL is classified as a lymphoma of immune privileged sites, along with testis lymphoma and primary central nervous system lymphoma (PCNSL). VRL and PCNSL are strictly connected indeed: 80% of VRL develop PCNSL, while 20% of patients with PCNSL present VRL during natural history of lymphoma. Due to the lack of worldwide consensus about diagnosis, therapy, and follow-up timing, VRL represents one of the most challenging ocular affections.VRL commonly masquerades as a posterior uveitis, and misdiagnosis often occurs because of partial response to topical steroids. Gold standard for diagnosis is cytological analysis of vitreous humor. However, this technique lacks sensitivity and supplemental molecular analyses can improve the diagnostic process. Multimodal imaging allows ophthalmologists to empower their clinical suspicion and a comprehensive examination can highlight typical features of VRL and justify further invasive procedures.There is no consensus about VRL therapy, and none of the therapeutical scheme has demonstrated to prevent cerebral involvement and improve patient's overall survival. Intravitreal injections of chemotherapeutics drugs, ocular radiation therapy and systemic chemotherapy can be considered in the treatment of VRL. Once cerebral involvement occurs, systemic chemotherapy must be included in the treatment as a life-saving therapy. Further multicentric studies are required to find out the best treatment of patients with VRL.

Presumed Müller Cell Activation in Multiple Evanescent White Dot Syndrome.

Purpose: The purpose of this study was to investigate the foveal changes occurring in multiple evanescent white dot syndrome (MEWDS) using multimodal imaging techniques with a specific focus on hyper-reflective dots (HRDs). Methods: This was a retro-prospective observational study including 35 eyes with active MEWDS. Structural and en face optical coherence tomography (OCT) was performed, with follow-up visits at 2 weeks, 6 weeks, and 2 months from baseline. HRD percentage area (HRD % area) was calculated in a 600 µm fovea centered circle on en face OCT, after background subtraction and image binarization. HRD % area was compared with 23 fellow control eyes. Longitudinal changes in the HRD % areas were assessed using repeated-measure statistics. Results: HRDs were observed as scattered hyper-reflective spots on the vitreoretinal interface on en face OCT images, colocalizing with HRDs or vertical hyper-reflective lines on structural OCT images. The baseline evaluation showed a significantly higher HRD % area in MEWDS eyes compared to fellow eyes (0.10 ± 0.03 vs. 0.08 ± 0.04, P = 0.01). The HRD % area correlated positively with LogMAR visual acuity and inversely with the duration of symptoms. Longitudinal analysis revealed a significant reduction in the HRD % area over time. There was no significant interaction between the rate of HRD disappearance and clinical or demographic factors at baseline. Conclusions: As HRD potentially represents the end-feet projections of activated Müller cells on the retinal surface, this study supports the involvement of Müller cells in the pathogenesis of the disease. The findings highlight the potential of en face OCT imaging for monitoring the progression of MEWDS.

DISAPPEARING CHOROIDAL SPOTS ON OCT ANGIOGRAPHY PRECEDING RECURRENCE OF MULTIFOCAL CHOROIDITIS WITH CHORIORETINAL ATROPHY.

PURPOSE: To describe idiopathic multifocal choroiditis (iMFC) with chorioretinal atrophy developing choroidal flow voids on optical coherence tomography angiography (OCTA) that preceded a recurrence of the disease. METHODS: Case report. RESULTS: A 24-year-old woman presented with visual field changes and occasional photopsias. Systemic work-up for syphilis, tuberculosis, and sarcoidosis, was negative. Clinical findings and multimodal imaging were consistent with iMFC with chorioretinal atrophy, complicated by inactive choroidal neovascularization in her right eye. She was treated with systemic corticosteroids with a taper over 3 months without change in her examination. She was then stable for two years. At that point, the patient experienced increased photopsias, but her examination was unchanged. OCTA showed multiple flow voids in the choroid that were not present 6 months prior. No lesions were seen on other imaging modalities. Structural OCT showed some subtle hyper-reflectivity throughout the choroid that was previously absent. Given the unknown significance of these flow voids, the patient was asked to return for follow-up in one month. Her photopsias improved and her vision remained normal. On repeat examination after one month, the patient had developed a few subtle yellow lesions in the supero-nasal quadrant of the left eye. There were no macular lesions. The repeat OCTA revealed that the flow voids were fading. CONCLUSION: Imaging findings using OCTA in our patient with iMFC showed choroidal flow voids that preceded clinical recurrence, not detected by other imaging modalities. Future studies should determine if OCTA can be used to detect subclinical lesions preceding clinical recurrences of iMFC.

Acute and Chronic Manifestations of Sympathetic Ophthalmia on Multimodal Imaging.

PURPOSE: To report the clinical and multimodal imaging features of sympathetic ophthalmia in the acute and chronic phases. METHODS: Retrospective cohort study of consecutive patients with sympathetic ophthalmia seen at a tertiary referral center. Charts, imaging studies, and histopathological specimens were reviewed. The clinical features and multimodal imaging in the sympathizing eye were analyzed by sorting features into those seen in the acute and chronic phase. RESULTS: Ten patients were included in the analysis and all of them had previous ocular trauma or complicated retinal detachment. In the acute phase, 70% had anterior uveitis, 70% had vitritis, and 100% had active posterior uveitis; posterior uveitis included multifocal choroiditis (80%), optic disc swelling (40%), multiple serous retinal detachments (20%), MEWDS-like findings (10%), and retinal vasculitis with chorioretinitis (10%). In the chronic phase, posterior manifestations included widespread patches of chorioretinal atrophy in the mid- and far-periphery (80%), peripapillary subretinal fibrosis (50%), and nummular perivascular atrophy (50%). CONCLUSIONS: Sympathetic ophthalmia shows different posterior segment manifestations in the acute and chronic phase. Active sympathetic ophthalmia should be ruled out in eyes with a MEWDS-like presentation or rapidly progressing chorioretinitis, and history of trauma in the fellow eye. Peripapillary subretinal fibrosis and perivascular nummular atrophy may be useful features to suspect SO once acute inflammation has resolved.

MULTIZONAL OUTER RETINOPATHY AND RETINAL PIGMENT EPITHELIOPATHY (MORR): A Newly Recognized Entity or an Unusual Variant of AZOOR?

PURPOSE: To describe specific clinical, multimodal imaging, and natural history features of an unusual variant of acute zonal occult outer retinopathy. METHODS: Retrospective, observational, longitudinal, multicenter case series. Patients exhibiting this unusual clinical condition among cases previously diagnosed with acute zonal occult outer retinopathy were included. Multimodal imaging, laboratory evaluations, and genetic testing for inherited retinal diseases were reviewed. RESULTS: Twenty eyes from 10 patients (8 females and 2 males) with a mean age of 54.1 ± 13.3 years (range, 38-71 years) were included. The mean follow-up duration was 13.1 ± 5.3 years (range, 8-23 years). Presenting symptoms were bilateral in 7 patients (85% of eyes) and included scotomata and photopsia. All patients had bilateral lesions at presentation involving the peripapillary and far peripheral retina. Baseline optical coherence tomography showed alteration of the retinal pigment epithelium and photoreceptor layers corresponding to zonal areas of fundus autofluorescence abnormalities. Centrifugal and centripetal progression of the peripapillary and far-peripheral lesions, respectively, occurred over the follow-up, resulting in areas of complete outer retinal and retinal pigment epithelium atrophy. CONCLUSION: Initial alteration of photoreceptors and retinal pigment epithelium and a stereotypical natural course that includes involvement of the far retinal periphery, characterize this unusual condition. It may represent a variant of acute zonal occult outer retinopathy or may be a new entity. We suggest to call it multizonal outer retinopathy and retinal pigment epitheliopathy .

Latest advances in white spot syndromes: New findings and interpretations.

White spot syndromes (WSS) pose challenges in the field of ophthalmology, particularly in terms of accurate diagnosis and effective management. However, recent advancements in multimodal imaging (MMI) have significantly contributed to our understanding of WSS, allowing for improved characterization of these inflammatory chorioretinopathies. By employing various imaging modalities, including fundus fluorescein angiography, indocyanine green angiography, fundus autofluorescence, optical coherence tomography (OCT), ultra-widefield imaging, and OCT angiography, researchers and clinicians have gained valuable insights into the underlying pathophysiological changes and clinical progression of WSS. Furthermore, MMI has unveiled novel and atypical variants within the spectrum of WSS, expanding our knowledge in this field. Notably, the identification of secondary forms of WSS occurring concurrently with unrelated chorioretinal disorders has suggested a potential autoimmune mechanism underlying these conditions. The introduction of MMI has also facilitated a more comprehensive evaluation of previously ill-defined entities, such as acute zonal occult outer retinopathy, leading to improved diagnostic criteria and enhanced recognition of distinct features. This review paper provides a comprehensive overview of the latest advances and interpretations in WSS. By integrating MMI into the diagnosis and management of these conditions, this review aims to enhance patient outcomes and provide valuable insights into the complexities surrounding WSS.

UNUSUAL CASE OF CLADOSPORIUM SPHAEROSPERMUM ENDOGENOUS ENDOPHTHALMITIS DURING GOLIMUMAB THERAPY: CASE REPORT AND LITERATURE REVIEW.

PURPOSE: To report a case of fungal endogenous endophthalmitis from Cladosporium sphaerospermum in a patient with juvenile idiopathic arthritis receiving chronic immunosuppressive therapy with golimumab (tumor necrosis factor-α blocker). METHODS: Case report and review of the literature. RESULTS: A 34-year-old woman receiving chronic immunosuppressive therapy with golimumab for juvenile idiopathic arthritis was referred for unilateral visual loss and ocular pain. Worsening conditions after corticosteroid therapy and raised serum beta-D-glucan levels pointed to an infectious fungal etiology. Panfungal polymerase chain reaction-based genetic sequencing on vitreous specimens obtained during vitrectomy detected C. sphaerospermum. The patient management combined surgical treatment and systemic and intravitreal voriconazole. CONCLUSION: Endogenous fungal endophthalmitis can be a rare complication in patients undergoing chronic immunosuppressive therapy (including golimumab) without other predisposing factors. Prompt diagnosis and appropriate treatment are the keys to preserve vision.

Swept-source optical coherence tomography angiography metrics of retinal ischaemic perivascular lesions in patients being evaluated for carotid artery stenosis and controls.

BACKGROUND/AIMS: Retinal microvascular ischaemia may produce localised middle retinal disruption with corresponding scotoma, a phenomenon termed paracentral acute middle maculopathy (PAMM). Small chronic middle retinal atrophic lesions termed retinal ischaemic perivascular lesions (RIPLs) appear qualitatively similar to PAMM lesions and have recently been hypothesised to result specifically from PAMM. However, no studies have quantitatively demonstrated an ischaemic origin of RIPLs. We quantitatively investigated the pathophysiology of RIPLs and their relationship with PAMM with swept-source optical coherence tomography angiography (SS-OCTA). METHODS: A total of 14 controls and 25 patients being evaluated for carotid artery stenosis (CAS) were enrolled. SS-OCTA imaging of each eye was taken. Projection-resolved en face 6 mm × 6 mm superficial capillary plexus (SCP) and deep capillary plexus (DCP) images were quantitatively analysed with two algorithms for changes in vessel linear density (VLD) and vessel tortuosity (VT) at RIPLs relative to both the immediately surrounding macula and the entire macula, as well as between eyes with RIPLs and eyes without RIPLs. RESULTS: All controls and 22 of 25 CAS patients were included in the analysis. RIPLs demonstrated a localised decrease in DCP VLD in CAS patients and controls. RIPLs tended to show a localised decrease in SCP VLD in CAS patients but a localised increase in controls. No changes in VT were found. Eyes with RIPLs had VLD and VT similar to their RIPL-free fellow eyes. CONCLUSION: RIPLs are associated with quantifiable local, but not global, ischaemia, supporting the idea of shared pathophysiology with classic PAMM lesions along a continuum of ischaemia severity.

Fixation Location and Stability in Best Vitelliform Macular Dystrophy.

Purpose: To analyze fixation location and stability in best vitelliform macular dystrophy (BVMD) and test their association with best-corrected visual acuity (BCVA). Design: Observational, cross-sectional study. Participants: Thirty patients (55 eyes) affected by genetically confirmed BVMD were followed up at the Retinal Heredodystrophies Unit of IRCCS San Raffaele Scientific Institute, Milan. Methods: Patients underwent testing with macular integrity assessment (MAIA) microperimeter. Fixation location was measured as distance in degrees (°) between preferred retinal locus (PRL) and estimated fovea location (EFL); fixation was defined as eccentric when the distance between PRL and EFL exceeded 2°. Fixation stability was graded as stable, relatively unstable, or unstable and expressed as bivariate contour ellipse area (BCEA, °2). Main Outcome Measures: Fixation location and stability. Results: The median distance of the PRL from the anatomic fovea was 0.7°, and fixation location was eccentric in 27% of eyes. Fixation was graded as stable in 64% of eyes, relatively unstable in 13%, and unstable in 24%, with a median 95% BCEA of 6.2°2. The atrophic/fibrotic stage was associated with worse fixation parameters (all P < 0.01). Both PRL eccentricity and fixation stability were linearly associated with BCVA: every 1° increase in PRL eccentricity was associated with a 0.07 logarithm of the minimum angle of resolution (logMAR) worse BCVA (P < 0.0001) while every 1°2 increase in 95% BCEA was associated with a 0.01 logMAR worse BCVA (P < 0.001). No significant intereye correlation was found for PRL eccentricity and fixation stability, as well as no association between the patient's age and fixation parameters. Conclusions: We demonstrated that most eyes affected by BVMD retain a central stable fixation and provided evidence that both fixation eccentricity and stability are strongly associated with visual acuity in BVMD. These parameters may serve as secondary end points for future clinical trials. Financial Disclosures: Proprietary or commercial disclosure may be found after the references.

Diagnostic and therapeutic results of aqueous real-time polymerase chain reaction in infectious uveitis.

OBJECTIVE: This study was aimed primarily at describing the results of aqueous real-time polymerase chain reaction (RT-PCR) and reporting the rate of therapeutic modifications directly attributable to this procedure (profitability). Our secondary outcome was to compare demographic and clinical characteristics between patients with RT-PCR positivity and those with RT-PCR negative results. DESIGN: Retrospective observational study conducted at the Uveitis Service of San Raffaele Hospital (Milan, Italy) between November 2016 and July 2022. PARTICIPANTS: Patients with infectious uveitis suspect (anterior, intermediate, posterior uveitis, or panuveitis). METHODS: Patients with suspected infectious uveitis underwent aqueous RT-PCR for detection of herpes simplex 1 (HSV-1), herpes simplex 2 (HSV-2), varicella zoster virus (VZV), cytomegalovirus (CMV), and Toxoplasma gondii. RESULTS: Sixty-five eyes of 61 patients (60 ±16 years of age; 54% males) were included. Aqueous RT-PCR tested positive in 58% and negative in 42% of patients. CMV and HSV-1 were the most frequently detected pathogens. RT-PCR confirmed clinical suspicion in 38% of patients and altered the presumed etiologic diagnosis and treatment in 20% of patients. Profitability was associated with CMV positivity. HSV-1 positivity was related to iris atrophy. CMV positivity was correlated with keratic precipitates. Vitritis and retinitis were related to VZV, CMV, and T. gondii detection. Synechiae, retinitis, and neuritis were related to positive tests regardless of the pathogen investigated. Early complications related to paracentesis were rarely reported. CONCLUSION: Aqueous RT-PCR was a safe semi-invasive tool to confirm a presumptive diagnosis and to change initial suspicion in ambiguous cases of herpetic uveitis. Thus aqueous RT-PCR may alter therapeutic management.

Posterior Herpetic Uveitis: A Comprehensive Review.

PURPOSE: To report and illustrate the main clinical presentations of posterior herpetic uveitis. METHODS: Narrative review. RESULTS: The ocular manifestations of posterior herpetic uveitis include different clinical presentations. Herpes simplex and varicella zoster can cause acute retinal necrosis, progressive outer retinal necrosis, and non-necrotizing herpetic retinopathies. Cytomegalovirus has been associated with fulminant retinitis with confluent areas of retinal necrosis and retinal hemorrhages, indolent/granular retinitis, and frosted branch angiitis. These diverse clinical presentations are often associated with specific risk factors and different immunological profiles of the host. CONCLUSIONS: Herpetic viruses can cause posterior uveitis, presenting various clinical findings. Specific ocular manifestations and the immunological status of the host can help to differentiate the various herpetic entities before laboratory tests confirm the diagnosis.

STELLATE MULTIFORM AMELANOTIC CHOROIDOPATHY: Clinical and Multimodal Imaging Features.

PURPOSE: To describe the clinical and multimodal imaging features of stellate multiform amelanotic choroidopathy (SMACH; also known as serous maculopathy due to aspecific choroidopathy). METHODS: Retrospective observational case series of eyes presenting with SMACH. Multimodal imaging including fundus photography, optical coherence tomography (OCT), OCT angiography (OCTA), and indocyanine green angiography (ICGA) was analyzed. RESULTS: Eighteen eyes from 18 patients (mean age: 28 ± 19 years) were included. The mean follow-up duration was 9 years. Ophthalmoscopy showed a yellowish orange, dendriform choroidal lesion. At presentation, subretinal fluid (SRF) was seen in 10 of 18 cases (56%). Eight patients (44%) showed no evidence of SRF during a mean follow-up of 6 years. Cross-sectional OCT showed hyperreflective fibrous-like changes within the inner choroid with choriocapillaris flow preservation on OCTA. En face OCT showed a hyperreflective choroidal lesion with finger-like projections oriented in a stellate configuration. On ICGA, SMACH showed early and late hypofluorescence. None of the cases showed lesion growth. CONCLUSION: SMACH seems to be a unilateral choroidopathy characterized by distinctive multimodal imaging features. As SRF was absent in some cases, while a dendriform pattern was a consistent finding in all eyes, the authors propose renaming this entity "stellate multiform amelanotic choroidopathy," a name that retains its previous abbreviation "SMACH."

CLINICAL CHARACTERISTICS AND MULTIMODAL IMAGING FINDINGS IN UNILATERAL FROSTED BRANCH ANGIITIS: A CASE REPORT.

PURPOSE: Our aim is to report a comprehensive multimodal imaging case of unilateral frosted branch angiitis in a 40-years-old Caucasian female . METHODS: Case report involving clinical examination, ultra- wide field fundus photograph, ultra-wide field fluorescein angiography (UWFA), optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA). RESULTS: A 40 years old patients presented with unilateral acute vision loss. On fundus examination, extensive retinal veins sheathing, macular edema and vascular congestion were observed while UWFA revealed an hyperfluorescent "hot" optic disc and blood retinal barrier disruption. OCTA displayed foveal avascular zone (FAZ) enlargement and excluded papillary neovascularization. Extensive laboratory work-up for infectious, autoimmune and inflammatory disorders were negative, thus, a diagnose of acute idiopathic unilateral frosted branch angiitis was made. Intravitreal injection of dexamethasone implant was administered with a good clinical response. CONCLUSIONS: Multimodal imaging is crucial to correctly diagnose and treat FBA. Up to our knowledge, the use of OCTA as a complementary tool to the diagnostic process in FBA has been described in literature just once as a photo essay of cytomegalovirus-related FBA and it might be of great value for better characterizing clinical features of this disorder and for following disease activity in a non-invasive fashion.