Neovascularized Best Disease in Child: Contribution of Optical Coherence Tomography Angiography.

Choroidal neovascularization (CNV) is a rare but severe complication in Best disease and autosomal recessive bestrophinopathy. However, the visualization of the neovascular membrane is difficult on fluorescein angiography (FA) and indocyanine green angiography (ICGA) because of dye leakage due to the accumulation of material. The authors' study reports a case series of pediatric Best disease where optical coherence tomography angiography (OCTA) contributed to the diagnosis of CNV and prompt treatment. Five eyes of three patients were included (two Best disease and one autosomal recessive bestrophinopathy). The mean age at diagnosis was 6.8 years ± 1.8 years (range: 5 years to 10 years). OCTA showed the typical "sea fan-shaped" neovascular membrane in all five eyes, whereas, in most cases, conventional imaging by FA and ICGA did not show clearly the neovascularization due to masking effect of the vitelliform material. OCTA seems to be a good alternative to diagnosing CNV in Best disease, especially in children, as it is a noninvasive, rapid technique for imaging, and does not require the administration of dyes. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:597-601.].