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.

Cross-validation of the Tunisian version of the National Eye Institute Visual Function Questionnaire 25.

INTRODUCTION: The goal of our study was to create a cross-cultural adaptation of the questionnaire « National Eye Institute Visual Function Questionnaire ¼ (NEI-VFQ-25) for the Tunisian population (Tu-VFQ-25) and to test its psychometric properties in a group of patients with chronic uveitis. PATIENTS AND METHODS: The study consisted of two steps. The first was translation of the NEI-VFQ-25 and its cross-cultural adaptation to the Tunisian dialect (Tu-VFQ-25) according to the guidelines of Beaton et al. The second step consisted of studying the psychometric properties of the Tu-VFQ-25. Enrolled patients, diagnosed with chronic uveitis, were interviewed face-to-face by two different investigators, followed by a telephone interview by the first investigator 48hours later. Feasibility, internal consistency and reproducibility of the Tu-VFQ-25 were then tested using statistical methods. RESULTS: The mean time taken to administrate the questionnaire to each of the 45 patients was 11min 40 s (± 2min 24 s). Internal consistency was acceptable, with Cronbach's alpha coefficients above 0.7 except the "Ocular Pain" and "Driving" subscales (0.645 and 0.559 respectively). Inter-observer reliability was excellent, with intra-class correlation coefficients (ICC) ranging from 0.857 to 0.985 for all subscales. Test-retest reliability, assessed for 43 patients, was good to excellent, with ICC above 0.80 for all subscales except "General Vision," which had a value of 0.786. CONCLUSION: As with previous versions, the Tu-VFQ-25 is an easy and reliable tool for assessing vision-related quality of life of Tunisian patients with chronic uveitis.

[Specular microscopy findings in active uveitis]. / Résultats de la microscopie spéculaire au cours des uvéites actives.

PURPOSE: To study the effect of ocular inflammation in active uveitis on the corneal endothelium. MATERIALS AND METHODS: This is a prospective cross-sectional study involving 63 eyes of 38 patients with active newly-diagnosed uveitis. Eighty-four eyes of 42 non-uveitic subjects served as a control group. All patients underwent detailed ophthalmic examination, laser flare photometry, and non-contact specular microscopy. Eyes with uveitis had a follow-up visit at one month after initiation of treatment, including laser flare photometry and specular microscopy. RESULTS: The mean age of the active uveitis patients was 33 (range: 9-67 years) with 21 men (55%) and 17 women (45%). For the uveitis patients at baseline, the mean flare value was 32ph/ms (range: 15-100ph/ms) with flare photometry value ≥50ph/ms in 13 eyes (21%) and<50ph/ms in 50 eyes (79%). At baseline, cell density (CD) of the uveitis patients was similar to CD in control eyes (p꞊0.16). The percent of hexagonality was significantly lower (p<0.0001), and the coefficient of variance (CV) (p<0.0001) and central corneal thickness (CCT) (p<0.0001) were significantly higher than in the control group. After one month, there was a significant decrease in flare photometry values (p<0.0001), with a mean flare value of 14.5ph/ms (range: 4-60ph/ms). In the active uveitis group, the overall results of specular microscopy did not significantly vary between the initial examination and the 1-month examination. For eyes with flare photometry value ≥50ph/ms at baseline, CD and hexagonal cell count significantly increased on the 1-month examination (p꞊0.01 and p꞊0.02 respectively), while CV and CCT decreased significantly at the one-month follow-up visit (p꞊0.007/p꞊0.03 respectively). For eyes with flare photometry value at baseline <50ph/ms, there was no significant differences in specular microscopy results between the initial examination and the 1-month examination. CONCLUSIONS: In eyes with newly-diagnosed uveitis and active anterior chamber inflammation, specular microscopy shows transient qualitative abnormalities of the corneal endothelium that depend on the severity of the anterior chamber inflammation as measured by the laser flare meter.

[Eye and Behçet's disease]. / Œil et maladie de Behçet.

Behçet's disease is a chronic multisystem disorder characterized by relapsing inflammation for which the underlying histopathology is an occlusive vasculitis. The disease has a high prevalence in the Mediterranean region, Far and Middle Eastern countries. It is clinically characterized by oral and genital ulcerations, ocular inflammatory involvement, skin lesions, vascular involvement and numerous other systemic manifestations. Uveitis is by far the most common ocular manifestation of Behçet's disease. It typically presents in the form of relapsing-remitting panuveitis with retinal vasculitis that may result in severe visual loss. Management of Behçet's uveitis relies on corticosteroid therapy, conventional immunosuppressive drugs and biological agents and requires a close collaboration between ophthalmologist and internist. A better understanding of the auto-inflammatory process and of the role of cytokines implicated in the pathogenesis of Behçet's disease will enable researchers to develop more specific and effective therapy.

Eye and Behçet's disease.

Behçet's disease is a chronic multisystem disorder characterized by relapsing inflammation for which the underlying histopathology is an occlusive vasculitis. The disease has a high prevalence in the Mediterranean region, Far and Middle Eastern countries. It is clinically characterized by oral and genital ulcerations, ocular inflammatory involvement, skin lesions, vascular involvement and numerous other systemic manifestations. Uveitis is by far the most common ocular manifestation of Behçet's disease. It typically presents in the form of relapsing-remitting panuveitis with retinal vasculitis that may result in severe visual loss. Management of Behçet's uveitis relies on corticosteroid therapy, conventional immunosuppressive drugs and biological agents and requires a close collaboration between ophthalmologist and internist. A better understanding of auto-inflammatory process and of the role of cytokines implicated in the pathogenesis of Behçet's disease will enable researchers to develop more specific and effective therapy.