Bests disease is an autosomal dominant hereditary macular dystrophy characterized by the presence of auto-fluorescent vitelline deposits. We report a case of a vitelliform stage of bests disease in a 23-year-old female patient. The diagnosis was made based on the fundus appearance: egg yolk macular lesion, hypofluorescence in the early stages, OCT appearance and alteration of the electro-oculogram and the electroretinogram. The exact frequency of this condition is difficult to determine and varies according to the various studies. It is thought to account for 4% of all retinal dystrophies. Its management is an annual surveillance.
We report an observation of an uncommon form of extra-papillary myelin fibers of fortuitous discovery in a patient, 55 years old, who consulted for a change of correction.
The clinical aspect of the fundus led to the diagnosis of retinal myelin fibers in front of a whitish placard with a finely striated scalloped edge, partially masking the vessels and located at the level of the inferior temporal arch.
These fibers, contiguous to the papilla or distant from it, have an incidence varying from 0.57% to 0.98% according to studies and their pathogenesis is still uncertain. This congenital anomaly of the retinal fibers of clinical diagnosis is often asymptomatic, but can however be associated with other ocular disorders.
Amnion, the innermost layer of the placental sac, can be harvested as graft material. It has several mechanisms of action. There are three types of this AM fresh, cryoconserved and dehydrated. The dehydrated Amniotic Membrane is the type used in our observations. We report two cases the first one a 70 years old patient with bullous keratopathy and the second one a 65 years old patient with a diffuse epithelial defect in whom the dehydrated amniotic membrane graft gave good results The effectiveness of dehydrated membrane grafting has been demonstrated in several studies but a larger study is needed to confirm its effectiveness.
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