IRVAN syndrome: A retrospective review of 9 cases from Tunisia.

PURPOSE: To describe clinical features, relevant imaging findings, disease course, and response to treatment in 9 patients (18 eyes) with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. METHODS: Retrospective review of the charts of nine patients (18 eyes) diagnosed with IRVAN syndrome at Fattouma Bourguiba University Hospital, Monastir, Tunisia, from January 1, 2011 to January 1, 2022. RESULTS: Nine patients were included with bilateral involvement in all cases. Mean initial best-corrected visual acuity (VA) was 20/32 (range, 20/1600-20/20). Clinical findings at presentation included vitreous cells (10 eyes, 55.6%), peripapillary exudates (12 eyes, 66.7%), partial or complete macular star (11 eyes, 61.1%), and vascular sheathing (11 eyes, 61.1%). Fluorescein angiography showed arteriolar aneurysms (18 eyes, 100%), areas of peripheral capillary non-perfusion (16 eyes, 88.9%), and retinal neovascularization (6 eyes, 33.3%). Optical coherence tomography showed macular edema in 5 eyes (27.8%). Optical coherence tomography angiography of the optic disc demonstrated papillary aneurysms in 4 eyes of 2 patients. Indocyanine green angiography showed retinal arteriolar aneurysmal dilatations in 4 eyes of 2 patients. Ten eyes (55.6%) had stage 2 disease, 6 eyes (33.3%) had stage 3, and 2 eyes (11.1%) had stage 1. Treatment modalities included peripheral photocoagulation (16 eyes, 88.9%), intravitreal bevacizumab (4 eyes, 22.2%), and intravitreal triamcinolone acetonide (1 eye, 5.6%). Mean final best-corrected VA was 20/32 (range, 20/600-20/20). Ocular complications included vitreous hemorrhage in 3 eyes (16.7%), branch retinal artery occlusion in 2 eyes (11.1%) and submacular fibrosis in 3 eyes (16.7%). CONCLUSION: IRVAN syndrome should be highly suspected in patients with peripapillary exudates associated with vascular sheathing and vitreous cells. Multimodal imaging confirms the diagnosis by showing retinal macroaneurysms. Early treatment of macular edema and/or peripheral retinal non-perfusion is mandatory to improve prognosis.

Comparison of Indocyanine Green Angiography and Fluorescein Angiography for Imaging of Central Serous Chorioretinopathy Patients as Candidates for Photodynamic Therapy.

PURPOSE: To compare fluorescein angiography (FA) and indocyanine green angiography (ICGA) for imaging of central serous chorioretinopathy (CSC) patients. METHODS: The observational cross-sectional clinical study was conducted on 131 eyes of 131 patients with CSC who were candidates for photodynamic therapy (PDT). An experienced ophthalmologist marked the leakage sites and choroidal hyperpermeability sites that needed PDT. For each eye, simultaneous FA and ICGA imaging with the maximum leakage area was selected for comparison regarding the site and size. RESULTS: The mean±standard deviation age of patients was 44.53±9.03 years. Of 226 leakage points, 177 (78.32%) points were in the same site, and 168 (74.34%) points were in the same size on FA. No statistical difference was found between age (P=0.45), sex (P=0.32), and chronicity (P=0.11) of the disease in comparing the ICGA images to the FA images regarding leakage at the same site. A statistically significant difference was also found regarding size of leakage and chronicity (P<0.001). CONCLUSION: The current results suggested that FA could be considered an alternative ocular imaging technology as a guide for PDT in CSC patients.

Multimodal imaging in multiple evanescent white dot syndrome and new insights in pathogenesis.

PURPOSE: To report the multimodal imaging in multiple evanescent white dot syndrome (MEWDS) during the acute and convalescent stages in order to better understand the focus of the inflammatory process. METHODS: Retrospective cohort study of 4 patients with MEWDS. Each patient underwent: enhanced depth imaging-optical coherence tomography (EDI-OCT), fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA) and en-face OCT and OCT angiography (OCT-A). Choroidal subfoveal thickness (CST) was measured manually. All patients were studied in the acute stage and convalescent stage after disappearance of OCT abnormalities and resolution of visual symptoms. RESULTS: Four MEWDS patients with a mean age of 23.5years were studied (range: 16-33years). Two patients were women. Initial mean visual acuity (VA) was 80.25 ETDRS. Final mean VA was 84.25 ETDRS. OCT imaging showed disruption of the ellipsoid zone and a slightly elevated RPE layer with overlying hyperreflective material, all of which corresponded to hyperautofluorescent FAF lesions. FA revealed multiple hyperautofluorescent lesions, correlated with hypocyanescent spots on the late ICGA. OCT-A showed normal superficial and deep retinal capillary plexus as well as choriocapillaris. The disease was self-limited in all the cases, with a mean time of 9weeks to resolution (range: 4-16). CONCLUSION: The pathophysiology of MEWDS is still debated. We believe that there is still not enough evidence to implicate the outer retina as the primary cause. For now, we suggest that this transient disease is the consequence of choriocapillaris hypoperfusion, but further studies are required to elucidate this hypothesis.

[Role of OCT-angiography in the management of sickle cell retinopathy]. / Apports de l'OCT angiographie dans la prise en charge des rétinopathies drépanocytaires.

INTRODUCTION: Sickle cell retinopathy is the main ophthalmologic complication of sickle cell syndrome. Optical coherence tomography (OCT) and optical coherence tomography-angiography (OCT-A) permit demonstration of central retinal involvement. The goal of this study is to determine whether central retinal involvement is predictive of peripheral retinal ischemia. MATERIALS AND METHODS: We carried out a retrospective study of 31 patients with sickle cell disease who underwent a complete ophthalmologic examination. We focused on capillary density of the superficial and deep plexuses and the central avascular surface by OCT-A, and retinal layer thickness by OCT. All of the findings obtained by OCT-A and OCT were classified according to the Goldberg stages on fluorescein angiography. RESULTS: A thinning of the mean and temporal deep plexus capillary layer as well as a loss of the temporal density of the superficial plexus capillaries are significantly higher in the case of proliferative sickle cell retinopathy (P=<0.05). A significant negative correlation is observed between the mean and temporal density of the superficial (R=-0.31; P=0.02 and R=-0.43; P=0.0009) and deep plexus capillaries (R=-0.39; P=0.003 et R=-0.43; P=0.0009) and the Goldberg stage in fluorescein angiography. CONCLUSION: The study of the temporal capillary densities of the superficial and deep plexuses on OCT angiography may prove to be a useful tool for the ophthalmologist in order to diagnose patients at risk for proliferative sickle cell retinopathy.

[Saturday night retinopathy: A French case of post-compressive CRAO]. / Saturday night retinopathy : un cas français d'OACR post-compressive.

We report herein the first French case of Saturday Night Retinopathy. A 39-year-old man presented to the emergency room with unilateral vision loss in the left eye with redness but no pain. Visual acuity OS was "light perception" and OD 20/20. The left eye was hyperemic with a fixed, dilated pupil; fundus examination revealed a macular cherry-red spot within a pale, ischemic retina. The patient was admitted to a stroke centre. The neurological work-up and head CT were normal. He also underwent evaluation for possible carotid or cardiac etiologies, all of which were negative. Current smoking was the only cardiovascular risk factor found. The patient reportedly fell asleep face down at his kitchen table after consuming a large amount of alcohol, with his left eye pressed into his arm throughout the night. Six similar cases have been reported in the literature since 1973. The prognosis for vision is dismal. Only public awareness and prevention might avoid this serious functional disability.

Multimodal imaging based biomarkers predictive of early and late response to anti-VEGFs during the first year of treatment for neovascular age-related macular degeneration.

PURPOSE: To evaluate baseline predictive markers of early and late anatomical response to anti-vascular endothelial growth factor (anti-VEGF) treatment in patients with neovascular age-related macular degeneration (nAMD). METHODS: The records of the nAMD patients who underwent intravitreal ranibizumab or aflibercept treatment, received the 3 monthly loading doses, and completed a follow-up period of 12 months were included retrospectively. The anatomical treatment response at month 3 (early) and between month 3 and 12 (late) was classified as good, intermediate or poor. Baseline demographic, fluorescein angiography, and optical coherence tomography findings were compared among the three groups. RESULTS: One hundred and ten eyes (74.3%) showed good, 18 (12.2%) showed intermediate and 20 (13.5%) showed poor anatomical response at month 3, and 114 eyes (77.0%) showed good, 27 (18.2%) showed intermediate and 7 (4.7%) showed poor anatomical response between month 3 and month 12. Of the evaluated parameters, drug type (better in aflibercept), showed a statistically significant difference in regards to anatomical outcomes at both the early and late periods (P=0.02 and P=0.03). The greatest linear dimension of choroidal neovascularization (CNV) and presence of peaked pigment epithelial detachment (PED) were important factors for early anatomical anti-VEGF treatment response. CONCLUSION: Larger CNV and the presence of a peaked PED appeared to be associated with a good early response, and the drug type seemed to be associated with both early and late poor anatomical response of anti-VEGF treatment in nAMD patients. Aflibercept appears to be more effective than ranibizumab in regards to the percentage of patients with better anatomical response in both the early and late treatment periods.

[Update from France Macula Federation: Diagnosis of wet AMD]. / Actualisations de la Fédération France Macula : diagnostic de la DMLA exsudative.

PURPOSE: To update the recommendations of the France Macula Federation for the diagnosis of wet age-related macular degeneration (AMD). METHODS: Analysis of literature and expert opinion. RESULTS: The FMF recommends diagnosing wet AMD by combining the results of fundus examination (or color or monochromatic fundus photographs), optical coherence tomography (OCT) showing exudative signs, and morphological visualization of the neovascular membrane, which may be obtained non-invasively (OCT-angiography) or invasively (fluorescein and/or indocyanine green angiography). Under optimal conditions in which all these tools are available, the FMF recommends using non-invasive methods as first-line tools and resorting to dye angiography if diagnostic doubt remains. CONCLUSION: As observed in other fields of medical imaging, non-invasive methods are preferred to invasive methods for the diagnosis of wet AMD, while the latter are reserved for more difficult cases.

[Contribution of multimodal imaging in the various stages of Stargardt disease]. / Intérêt de l'imagerie multimodale dans les différents stades de la maladie de Stargardt.

PURPOSE: To describe the contribution of multimodal imaging in the various stages of Stargardt disease (STGD). PATIENTS AND METHODS: We retrospectively reviewed 46 eyes of 23 STGD patients with identified ABCA4 mutations. All patients underwent a complete ophthalmic examination, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), fluorescein angiography (FA) and Indocyanine green angiography (ICGA). RESULTS: The mean age of patients was 25.5 years (range 8-56). Fundus examination was normal in 2 patients (subclinical stage), where SD-OCT showed localized retrofoveolar retinal pigment epithelium (RPE) thickening. FAF was normal in 1 eye and showed mild heterogeneous hyper-FAF in 3 eyes. Twelve eyes had mild salt and pepper changes in the macula (early stage) with diffuse retinal atrophy on SD-OCT and mixed hyper and hypoautofluorescence on FAF. Nine patients showed central atrophy with white-yellow flecks distributed in the posterior pole and mid-periphery. This phenotype showed total foveal atrophy on SD-OCT and normal peripapillary area on FAF. Twelve eyes had a large demarcated area of RPE atrophy, pigment clumping and migration extending to the peripheral retina associated with peripapillary atrophy. These eyes showed diffuse retinochoroidal atrophy on OCT with diffuse alterations reaching the peripapillary area on FAF. On FA, it was difficult to analyze the choroidal silence sign in patients with advanced stages of the disease. A hyperfluorescent window defect pattern was also found in patients with white-yellow flecks and did not correspond exactly to them, or to the areas of peripheral autofluorescent lesions. ICGA showed hypocyanescent areas seen at intermediate and late phases with multiple cyanescent points adjacent to them. On ICGA, hypocyanescent areas were more extensive than lesions observed on FAF. CONCLUSIONS: Multimodal imaging is helpful for the diagnosis of early stages of STGD disease and to better understand its pathophysiology. FAF and mostly SD-OCT have supplanted FA in the early, especially subclinical, stages. Over all, ICGA shows more extensive damage, making this tool useful for better understanding STGD and suggesting possible direct damage to the choriocapillaris associated with RPE lesions. In advanced stages, only DNA testing can confirm the diagnosis of STGD.

[Choroidal neovascularization complicating Best's vitelliform macular dystrophy in a child]. / Maladie de Best compliquée de néovascularisation choroïdienne chez un enfant.

INTRODUCTION: Best's disease is a progressive macular dystrophy, beginning either in childhood or adolescence. CASE STUDY: We report a rare case of choroidal neovascularization complicating vitelliform dystrophy in a child of 8 years with bilateral progressive loss of visual acuity. The ophthalmoscopic examination showed vitelliform lesions in both foveas. Fluorescein angiography confirmed a subretinal neovascular membrane in the left eye. Additional testing also confirmed the diagnosis of Best's disease associated with choroidal neovascularization. DISCUSSION: Best's vitelliform macular dystrophy is often asymptomatic because visual acuity tends to remain stable for a long time. A sudden loss of vision suggests the occurrence of complications, such as choroidal neovascularization.

[Acute Retinal Pigment Epitheliitis: spectral-domain optical coherence tomography findings]. / Épithélite rétinienne aiguë : apport de la tomographie par cohérence optique en Spectral Domain.

PURPOSE: Through a case presentation of Acute Retinal Pigment Epitheliitis (ARPE) we highlight the role of spectral-domain optical coherence tomography (SD-OCT) in the management of this rare entity. MATERIALS AND METHODS: A 29-year-old woman presented for reduced visual acuity in the right eye occurring one week after a viral episode. Fundus examination showed zones of macular hyperpigmentation surrounded by yellowish hypopigmented haloes. Fluorescein angiography noted early hyperfluorescence of the hypopigmented lesions. ICG angiography revealed central hyperfluorescence surrounded by a hypofluorescent halo. SD-OCT showed a linear disruption between the photoreceptor inner/outer segments (IS/OS) and an accumulation of material in the photoreceptor outer segments and retinal pigment epithelium (RPE). Spontaneous normalization of visual acuity was noted after 10 weeks. SD-OCT revealed restored and continuous inner segment and outer segment layers and some persistent deposits in the photoreceptor layer. RESULTS: SD-OCT findings suggest that the initial lesion in ARPE is located at the junction between the photoreceptor outer segments and the apical side of the RPE cells. It would correspond to an accumulation of photoreceptor outer segment debris secondary to RPE dysfunction, which can occur as a post-viral reaction. CONCLUSION: SD-OCT provides very specific information about the topography of retinal lesions during ARPE, allowing a better understanding of its pathogenesis.