[Optical coherence tomography and fundus autofluorescence in Best macular dystrophy]. / Aspects tomographiques et en autofluorescence du fond d'œil dans la maladie de Best.

Best vitelliform macular dystrophy is the second most frequent hereditary maculopathy, with bilateral involvement and juvenile onset. It is clinically characterized by bilateral deposits of lipofuscin-like autofluorescent material in the subretinal space, with a typical phenotypic manifestation taking the form of a vitelliform macular lesion evolving gradually into more advanced stages. The purpose of our study was to describe fundus autofluorescence patterns and OCT findings in three patients (6 eyes) with several stages of Best vitelliform macular dystrophy. Optical coherence tomography (OCT) has become the first imaging technique to order when confronted with a hereditary maculopathy suggesting Best disease. Fundus autofluorescence combined with OCT allow for better diagnosis and management, which are necessary for any genetic analysis.

[SD-OCT contribution in congenital achromatopsia diagnosis (6 patients)]. / Contribution du SD-OCT de la macula dans le diagnostic de l'achromatopsie congénitale : à propos de 6 patients.

PURPOSE: Achromatopsia (ACH) is a congenital autosomal recessive cone disorder. The puspose is to describe particular SD-OCT macular images in ACH. METHODS: The study included 6 patients from 3 consanguineous Tunisian families with congenital nystagmus and amblyopia with ACH. All patients had clinical examination with fundus photography, autofluorescence, 100-Hue Color vision and the appearance and thickness of all retinal layers were evaluated by spectral-domain optical coherence tomography (SD-OCT). RESULTS: All patients had ACH. The feature was loss of inner- and outer-segments (IS/OS) with disruption of the ciliary layer on OCT and an appearance of partial-thickness hole in the outer macular retina. CONCLUSION: This feature seems to be characteristic of ACH. SD-OCT correlated to clinic signs help the diagnosis.

[Macular choroidal thickness assessment with SD-OCT in high myopia with or without choroidal neovascularization]. / Épaisseur choroïdienne fovéolaire au SD-OCT dans la myopie forte avec ou sans néovaisseaux choroïdiens.

PURPOSE: To measure macular choroidal thickness (CT) using spectral-domain optical coherence tomography (SD-OCT) in eyes with myopic macular choroidal neovascularization (CNV), and to compare choroidal thickness in these eyes with highly myopic eyes without CNV. PATIENTS AND METHODS: Sixty-four eyes with myopic CNV matched with 64 highly myopic eyes without CNV by age and axial length (AL) were examined between January 2010 and November 2011. OCT scans were performed with spectral-domain OCT (TOPCON OCT 2000). The reference position was changed from the vitreous to the choroid. OCT scan patterns consisted of seven sections; the subfoveal CT was measured manually between Bruch's membrane and the internal portion of the sclera in eyes with CNV and from the pigment epithelium to the scleral interface in eyes without CNV. RESULTS: In the subgroup with CNV, the mean subfoveal CT was 51.71 µm ± 17.35. A statistically significant negative correlation was found between CT and AL (r=-0.615, P=0.0001). Regression analysis demonstrated a decrease of 8.4 µm per mm of AL. In the subgroup without CNV, matched with the CNV subgroup by age (P=0.597), and AL (P=0.813), the mean subfoveal CT was 93.35 µm ± 34.81 µm. The difference between the two subgroups was statistically significant (P<10(-4)). DISCUSSION: Macular choroidal thickness is reduced in high myopia, especially when complicated by CNV. It has not yet been shown that choroidal thinning may be a risk factor for choroidal neovascularization, but our results may suggest that macular choroidal thinning may lead to hypoxic retinal changes resulting in secretion of VEGF and thus CNV. CONCLUSION: Macular choroidal thinning observed in high myopia with CNV. These findings may suggest that choroidal changes may play a role in the pathogenesis of choroidal neovascularization.