Fig. 2

Multimodal images of CNV secondary to hereditary diseases. a-f A 6-year-old boy with bilateral BVMD and CNV membrane in the left eye. Fundus examination of the left eye revealed a subfoveal yellow-gray fibrotic CNV membrane with hemorrhage. In AF image, the vitelliform material surrounding the CNV was hyperautofluorescent (vitelliruptive stage). Early-phase FFA showed hyperfluorescence of CNV, and the simultaneous ICGA showed hypercyanescent neovascular network surrounded by hypocyanescence. OCT images demonstrated a type 2 CNV. Hyperreflective material could be seen in the subretinal space with a loss of outer retinal layers. g-j An 11-year-old boy was diagnosed with ARB due to the biallelic pathologic mutation of BEST1 gene. Fundus examination revealed a yellow-white juxtafoveal CNV and yellow dots around the fovea. AF image demonstrated multiple hyperautofluorescent extramacular punctate deposits. CNV had significant fluorescence leakage in FFA. OCTA showed a type 2 CNV with subretinal fluid (SRF). k-p A 17-year-old boy with BCD showed numerous glittering crystalline deposits in the posterior pole on fundus examination. The CNV appeared as a round light grey lesion temporal to the fovea. AF image showed diffuse hypoautofluorescence interspersed with hyperautofluorescence in the macular region. FFA showed hyperfluorescent window defects around the macula and the CNV showed mild fluorescence leakage. OCT images demonstrated a type 2 CNV located inferotemporal to the fovea with RPE irregular, interdigitation zone (IZ) and ellipsoid zone (EZ) interruption and loss