CTNNA1-associated retinal dystrophy also lacks ADVIRC-associated features such as microcornea, shallow anterior chamber, iris dysgenesis, macular oedema, optic nerve dysplasia, progressive generalised atrophy and severe ERG abnormalities distinguishing it from BEST1-related ADVIRC [17, 19–23]. The gene discussed is CTNNA1; the disease is autosomal dominant vitreoretinochoroidopathy.