Background: Approximately 8% of autosomal-dominant polycystic kidney disease (ADPKD) patients have intracranial aneurysms. The risk of growth and rupture of those discovered by presymptomatic screening is key to the feasibility and success of a screening program. This study was initiated to ascertain this risk.
Methods: ADPKD patients were offered screening with magnetic resonance (MR) imaging that included three-dimensional time-of-flight MR angiographic and three-dimensional phase-contrast sequences. Patients with aneurysms were recommended periodic surveillance, initially at 6 months and yearly, and less frequently after demonstration of their stability.
Results: Twenty-two saccular and one fusiform aneurysms were detected at the initial screening in 21 patients from 19 families (seven men and 14 women, 47.9 +/- 10.6 years old). All the saccular aneurysms were small (mean diameter 3.5 mm, range 2.0 to 6.5 mm) and the majority (77%) in the anterior circulation. Two patients died from unrelated causes without further follow-up. One patient was lost to follow-up. A new 2 mm middle cerebral artery aneurysm developed in one patient. One aneurysm increased from a size of 4 mm to 5 mm after a follow-up of 105 months. No aneurysmal development or growth occurred in the remaining 16 patients. No aneurysmal rupture occurred during a mean imaging follow-up of 81 months and a mean clinical follow-up of 92 months. During the period of the study, two additional ADPKD patients, with three intracranial aneurysms detected elsewhere by presymptomatic MR angiographic screening, were referred for surgical treatment. The larger size of these aneurysms (10, 8, and 8 mm) probably reflects referral bias.
Conclusion: Most intracranial aneurysms detected by presymptomatic screening in ADPKD patients are small and in the anterior circulation. The follow-up results do not suggest an increased risk for growth and rupture, compared to those of intracranial aneurysms in the general population. These data do not support widespread screening for intracranial aneurysms in the ADPKD population.