In addition, three rare variants, p.Ser193Gly, p.Val1817Leu, and p.Asp3329Tyr, identified in our ICAS patients without RNF213 p.Arg4810Lys were absent from both the control subjects and dbSNP, and so were considered as candidate variants associated with ICAS. This evidence concerns the gene RNF213 and familial isolated congenital asplenia.