[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.

[Neuromuscular hamartoma of the orbit: case report and discussion of the role of corticosteroids in treatment]. / Hamartome neuromusculaire de l'orbite : rapport d'un cas et discussion de la place de corticothérapie dans le traitement.

INTRODUCTION: Neuromuscular hamartoma, also known as neuromuscular choristoma or benign triton tumor, is a rare benign tumor of well-differentiated striated muscle fibers mixed with peripheral nerve fibers. According to our research, this is the sixth case of benign triton tumor of the trigeminal nerve and the third case of isolated orbital location reported in the world literature. PURPOSE: To report a rare case of orbital neuromuscular hamartoma and discuss the role of corticosteroids in the treatment of these lesions for which surgical excision is often difficult. OBSERVATION: A 47-year-old woman, with a history of tuberculous lymphadenitis treated in 2006, presented with a clinical scenario of inflammatory orbitopathy without loss of visual acuity progressing over 20 days. MRI showed a lesion centered on the soft tissues of the infero-lateral right orbit. A biopsy was performed, showing neuromuscular hamartoma on histology. The patient was put on a tapering dose of corticosteroids with clear clinical and anatomical improvement. Orbital CT follow-up was obtained two months after discontinuation of treatment, confirming the disappearance of the tumor mass. CONCLUSION: Hamartoma of the orbit is a very rare entity and may clinically simulate malignant neoplasms; the diagnosis is histologic. Given the difficulties encountered in the resection of these tumors, we believe that corticosteroids might be proposed as an alternative treatment that could modulate inflammation and bring about regression or disappearance of the tumor.