Retinal structure in young patients aged 10 years or less with Best vitelliform macular dystrophy

Incidence and Natural History of Retinochoroidal Neovascularization in Enhanced S-Cone Syndrome

“…3 On the other hand, RCN presentation at young age is not unique to ESCS but may occur also in other retinal dystrophies, such as Best disease. 14 Our study provides further support to the idea that RCN is the cause of > _1 pattern of subretinal fibrosis encountered in ESCS: the unifocal fibrotic ''nodule.'' This pattern was encountered in 11 of our 14 patients (13 eyes and 22 lesions).…”

Clinical outcomes of treated macular neovascularisation secondary to inherited retinal diseases: a literature review

“…Good visual and anatomical outcomes at 1 month was also described using a combined approach with a single intravitreal injection of ranibizumab and PDT. 24 Recurrence at 8 months was treated with a single intravitreal injection of ranibizumab and vision with a fairly stable VA at 4 years (0.2 at 1 month and 0.3 at 4 years).…”

Multimodal Image Analysis in Acquired Vitelliform Lesions and Adult-Onset Foveomacular Vitelliform Dystrophy

“…It is well known that all VLs share a yellowish appearance in fundoscopic evaluation, a subretinal location and homogeneous hyperreflectivity in SD-OCT, hyperautofluorescence in FAF, and a similar behavior during FA [ 2 , 4 , 6 , 9 , 10 ]. Theoretically, we can distinguish these patients by the age of diagnosis, familial history, and the presence or absence of associated diseases.…”

“…When detected in younger patients, VLs usually occur in the setting of Best macular dystrophy (Best disease), an autosomal dominant disease associated with a mutation in bestrophin 1 (BEST1) [ 3 , 4 ].…”