PKD1 and familial isolated congenital asplenia: A prevalence of 8% for asymptomatic ICAs has been reported in patients with ADPKD, and those with PKD1 and PKD2 mutations appear to be at an equal risk of developing ICAs, while patients with mutations to the 5′ half of PKD1 are more likely to develop ICAs12, 13, 14, 15.