Alessandro Invernizzi

Description:Objective: To describe multi-modal imaging of intra-retinal sarcoidosis. Methods: Case series and literature review. Patients were included if they had presumed or definite ocular sarcoidosis and optical coherence tomography (OCT) evidence of intra-retinal lesions. Results: In total, 23 retinal lesions from six patients with were included. All patients were female and Caucasian, with a mean age of 81 years (range 73-88 years). The lesions were ovoid 20/23 (87%) and hyper-reflective 23/23 (100%) on OCT. Retinal pigment epithelial (RPE) atrophy was identified in 12/23 (52%) and RPE elevation in 8/23 (35%). Out of 13 retinal lesions that had a follow up OCT post-corticosteroid treatment, 7 (54%) completely resolved and 6 (46%) were smaller. The lesions were hyporeflective on near infrared imaging (12/17, 71%), hypo-autofluorescent (12/15, 80%), hyperfluorescent on fluorescein angiography (15/17, 88%) and hypofluorescent (12/17, 71%) on indocyanine green angiography. Literature review identified only five other studies describing 9 lesions of intra-retinal sarcoidosis confirmed on OCT. Conclusions: Ocular sarcoidosis can present with intra-retinal lesions that are ovoid and hyper-reflective on OCT, hyporeflective on near infrared, hypoautofluorescent, hyperfluorescent on FFA and hypo- or isofluorescent on ICGA. New findings of retinal sarcoidosis include multi-modal imaging characteristics, foveal involvement, associated photoreceptor or RPE atrophy and spontaneous resolution and recurrence. Identification of intra-retinal sarcoid lesions can assist in diagnosis and should be considered for inclusion in future diagnostic criteria. Prompt treatment of macular lesions with corticosteroids is recommended to avoid photoreceptor or RPE atrophy with permanent visual loss.

Description:Objective: To report the incidence, features, and clinical outcomes of macular neovascularization (MNV) in a large Italian cohort of patients with extensive macular atrophy with pseudodrusen-like appearance (EMAP). Methods: Retrospective, longitudinal study including 79 EMAP patients (158 eyes) with ≥6 months of follow-up at three retina clinics. Medical records and imaging were reviewed for demographic and clinical data, including age, best-corrected visual acuity (BCVA), MNV features, and retinal pigment epithelium (RPE) atrophy size, measured by short-wavelength autofluorescence and refined with near-infrared and OCT imaging. Main outcomes included cumulative MNV incidence, MNV risk factors, and BCVA and RPE atrophy changes in eyes with and without MNV. Results: Over a mean follow-up of 40.4 months, MNV developed in 14 eyes (10 patients), with a 4-year cumulative incidence of 15.2%. Most MNVs were type 2 (86%) and subfoveal (64%). Cox regression identified younger age, fellow eye involvement, smaller RPE atrophy size, and greater central subfield thickness (all p<0.01) as significant risk factors for MNV. While eyes with MNV had lower baseline BCVA (58.4 vs. 71.4 letters, approximately 20/63 vs. 20/40 Snellen; p=0.005), BCVA decline over time was similar between the two groups (-3.9 vs. -4.1 letters/year, p=0.69). However, RPE atrophy progressed faster in MNV eyes (3.4 vs. 2.8 mm 2 /year, p=0.02). Conclusions: In this EMAP cohort, MNV had a cumulative incidence of 15.2% at 4 years. Although BCVA outcomes were comparable, MNV was associated with faster atrophy progression, potentially due to a more aggressive disease phenotype or fibro-atrophic changes.

Description:Objective: This study aims to assess the reliability and agreement of fundus autofluorescence (FAF), near-infrared reflectance (NIR), and a combination of NIR and dense structural optical coherence tomography (OCT) scans (OCT+NIR) in delineating the areas of reticular pseudodrusen (RPD) in eyes affected by age-related macular degeneration (AMD). Methods: This was a single-center, cross-sectional study. Patients with non-advanced AMD exhibiting signs of RPD on multimodal imaging were enrolled. Two independent masked graders manually delineated the margins of the area occupied by RPD using the three distinct imaging techniques. Results: The study included 60 eyes from 51 patients, with a mean age of 81.5 (±7.1) years.The intraclass correlation coefficient between the two graders across all imaging modalities was 0.96 for FAF, 0.92 for NIR, and 0.98 for OCT+NIR. The narrowest limits of agreement were observed with OCT+NIR (-4.38 +5.17 mm 2 ). Foveal involvement and age were significantly correlated with larger RPD area ( p =0.036 and p=0.019 respectively). Pairwise comparisons of square root-transformed RPD areas indicated that FAF detected a significantly larger RPD area compared to other methods ( p <0.001). Conclusions: These findings validate OCT+NIR as a reliable approach for measuring RPD areas, potentially serving as a critical biomarker for AMD in future clinical trials.

Description:The survey aims to explore the use of existing nomenclature and current clinical and multimodal imaging (MMI) approach in diagnosing white dot syndromes (WDS) among uveitis and retina specialists. The members of the International Uveitis Study Group (IUSG) task force MUV (Multimodal imaging in UVeitis) developed a survey. The questionnaire, created using Qualtrics, consisted of 22 questions. The responses were compared against regions, workplace setting, sub-specialty, and experience of the participants. A total of 432 participants initiated the background section; 343 initiated the investigation section and 263/343 completed the survey (76.7%). The majority (43.7%) reported a specialty/practice focus mostly on uveitis, 32.2% on uveitis and retina, and 20.1% mostly on retina. Specifically, 55.7% were in practice > 10 years post-fellowship and 65.8% worked in academic settings. The term WDS was not universally used in clinical practice, with no significant differences by region, subspecialty, experience, workplace setting or number of WDS patients managed in the prior year (p > 0.01). Nearly 90% of participants reported using MMI to diagnose WDS. More than 70% advocated redefining the nomenclature and classification of WDS based on the primary anatomical location of disease using MMI without significant regional or professional differences (p > 0.01). These results underscore the widespread adoption of MMI among uveitis and retina specialists in the characterization of entities traditionally grouped under the term WDS. Respondents strongly agree that MMI provides a precise distinction between these posterior uveitis, advocating for the overcoming of the clinical term WDS in favor of a patho-anatomic redefinition.

Description:To report the clinical course of two cases of acute retinal necrosis (ARN) caused by varicella zoster virus (VZV) and cytomegalovirus (CMV) co-infection detected by polymerase chain reaction (PCR) on aqueous tap. Observational case reports. Two patients presented to our services with unilateral panuveitis suggestive of ARN complicated by hemorrhagic vasculitis and started empirical therapy. Aqueous PCR was performed on the same day and showed double positivity for VZV and CMV, which guided treatment. At follow-up, wide-field color fundus imaging and high-resolution optical coherence tomography showed resolution of active retinitis. Our cases suggest that ARN complicated by hemorrhagic vasculitis may be secondary to CMV and VZV co-infection, both in patients with an unremarkable clinical history and in those with immunodeficiency. In our cases, aqueous PCR testing was of paramount importance to determine the aetiology of ARN and to adjust the antiviral therapy accordingly.