Intracranial aneurysms in patients with autosomal dominant polycystic kidney disease: prevalence, risk of rupture, and management. A systematic review

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder associated with high incidences of intracranial aneurysms. We performed a systematic review with the purpose of clarifying the prevalence, risk of rupture, and appropriate management of intracranial aneurysms in the ADPKD population. METHOD: PRISMA guidelines were followed. We conducted a comprehensive literature search of three databases (PubMed, Ovid MEDLINE, and Ovid EMBASE) on all series reporting ADPKD patients with intracranial aneurysms. RESULTS: Our systematic review included 16 articles with a total of 563 patients with ADPKD and intracranial aneurysms. The prevalence of unruptured aneurysms was 11.5% (95% CI = 10.1-13%), whereas 1.9% (95% CI = 1.3-2.6%) of aneurysms were ruptured. Hypertension was present in 79.3% of patients with ADPKD and renal impairment in 65%. The mean size of ruptured aneurysms was slightly higher than unruptured (6 mm vs. 4.4 mm). The most common locations of unruptured and ruptured aneurysms were the ICA (40.5%) and MCA (45%), respectively. Asymptomatic patients studied with four-vessel angiography experienced 25% transient complications. Overall, 74% unruptured aneurysms were surgically treated with lower complication rates compared to endovascular treatment (11% vs. 27.7%). Among conservatively treated aneurysms, 2.9% ruptured at follow-up (rupture rate 0.4%/patient-year). Finally, the growth rate was 0.4% per patient-year, and the incidence of de novo aneurysm formation was 1.4% per patient-year. CONCLUSIONS: The prevalence of unruptured intracranial aneurysms in the ADPKD population is approximately 11%. Given the non-negligible rate of procedural complications, the management of these patients must be cautious and individualised. The rupture rate appears comparable to that of the general population. On the other hand, the 1.4% rate per patient-year of de novo aneurysms is non-negligible. These findings should be considered when counselling ADPKD patients regarding the appropriate management of intracranial aneurysms.