Familial Mediterranean fever associated frosted branch angiitis, retinal vasculitis and vascular occlusion.

OBJECTIVES: To analyse the entity of retinal vasculitis, including frosted branch angiitis (FBA), or retina vascular occlusion in patients with familial Mediterranean fever (FMF). METHODS: Retrospective collaborative case series using invitation by email to uveitis specialists around the Mediterranean basin. This series was combined with a literature review. Exclusion criteria included infectious diseases, Behçet's disease or other autoimmune diseases. RESULTS: A total of 16 patients (21 eyes) had FMF and retinal vasculitis (FBA 11 patients, mild retinal vasculitis 5 patients). The mean age at onset of vasculitis was 29.5 ± 13.4 (range 9-62) with a female to male ratio of 9 to 7. In 19 eyes treated with various forms of corticosteroid and/or immunosuppression, the mean initial spectacle-corrected visual acuity improved from 6/194 to 6/10.5 at the last mean follow-up of 29.0 ± 34.9 months (p < 0.001). The most common FEVR mutations were M680I and M694V. In addition, retinal vascular occlusions included one case of central retinal artery occlusion and one case of branch retinal artery occlusion. CONCLUSION: FBA and milder forms of retinal vasculitis are associated with FMF. Therapy involves an increase in colchicine dosage in early cases, a long period of oral corticosteroid, intravitreal dexamethasone implant or periocular corticosteroid in select cases, and combination therapy with systemic immunosuppression in severe cases. FMF needs to be included in the differential diagnosis of retinal vasculitis.

Large Choroidal Melanocytoma Simulating Choroidal Melanoma: A Difficult Differential Diagnosis and an Inevitable Enucleation.

PURPOSE: To describe a case of choroidal melanocytoma mimicking a melanoma. METHODS: Retrospective case report. Patient. A 48-year-old Moroccan woman presented with progressive, painless decreased vision in her left eye for 2 months. RESULTS: Her visual acuity was light perception in the left eye and 20/20 in the right one. Fundus examination and fluorescein angiography of the left eye showed a total retinal detachment with a large superior brownish mass. The clinical examination, B-scan ultrasonography, and magnetic resonance imaging all suggested a malignant melanoma. Consequently, the eye was enucleated. The histopathology later revealed a benign melanocytoma of the choroid. Discussion. Melanocytoma is a rare benign pigmented tumor. It is classically described as a tumor of the optic nerve head, but there are some exceptional case reports of uveal tract locations (iris, ciliary body, and choroid). In such cases, it can be difficult to clinically differentiate a melanocytoma from a malignant melanoma.

Œdème papillaire isolé révélant une sarcoïdose oculaire: Isolated papillary edema revealing ocular sarcoidosis.

Sarcoidosis is a multisystem granulomatous disorders of unknown cause, characterised by the presence of epithelioid granulomas and giant-cell granulomas without caseous necrosis. Ocular sarcoidosis mainly manifests as bilateral anterior granulomatous uveitis. The involvement of the posterior segment in patients with ocular sarcoidosis is rare and manifests as periphlebitis or choroidal invasion. Isolated papillary edema is uncommon; hence the peculiarity of our case study. Posterior involvement is a prognostic indicator of poor outcome, threatening visual acuity. Early management, in close cooperation with pneumologists and based on systemic corticosteroid therapy, improves visual outcome and reduces complications.