Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common monogenic human diseases, with an incidence of 1 in 1000. Asymptomatic aneurysms can be detected in 6% of patients with ADPKD without a family history, but in up to 16% of patients with a family history.1 This compares with an estimated prevalence of 1-2% in the general population. Intracranial aneurysm rupture is a rare but devastating complication of AKPKD that occurs on average 10 years younger than sporadic intracranial aneurysms. The youngest reported case was a 13 week old infant, and in one study 10% of patients were younger than 21 years.2 Intracranial aneurysm rupture is associated with a death rate of up to 65%. Treatment of a ruptured intracranial aneurysm by either neurosurgical clipping or endovascular treatment also carries an unacceptably high mortality rate of 8-10% and morbidity (disability or dependency) rate of 16-21%.3

The risk of rupture of asymptomatic intracranial aneurysms occurring in the general population is primarily determined by size, location, and a history …