Index cases of intracranial aneurysms in autosomal dominant polycystic kidney disease: longitudinal experience from a single renal transplantation centre

BackgroundThe prevalence of intracranial aneurysms (ICAs) is higher in patients with autosomal dominant polycystic kidney disease (ADPKD) than in the general population. This extrarenal manifestation carries significant mortality and morbidity risks. Our study aims to estimate the prevalence of, and to characterize patients with ICA(s) among the ADPKD cohort of South Australia's statewide transplantation centre.MethodsA retrospective review of prospectively collected data was conducted for all patients who underwent nephrectomy/ies between 1 January 1995 and 31 December 2021. Incidences of neuroimaging and radiologically diagnosed ICAs, and the presence of risk factors including family history are compared between ADPKD patients and those with an alternate primary nephrological diagnosis (control). Descriptive statistics and a narrative review of cases are presented.ResultsSeven patients had ICAs (14.3% ADPKD patients, 23.3% those with neuroimaging). Six did not meet current criteria for ICA screening, and five had no known family history of ICA. Three patients suffered aneurysmal rupture (mean age of 36). Two of these cases involved smaller ICAs. Cases demonstrated detectable vascular changes on early neuroimaging, hypoplastic anatomical variants, aneurysmal growth, de novo ICA formation, and association with other vascular abnormalities.ConclusionEarly detection of ICAs and pre-aneurysmal changes should be prioritized in ADPKD. Although limited by the small study cohort, our findings are consistent with previous literature suggesting ICA ruptures occur earlier in ADPKD and in smaller aneurysms. Further investigation is required into how these ICAs behave. Nonetheless, it is vital that centres coordinate a multidisciplinary, patient-centred approach to ICA screening, surveillance, and management for ADPKD patients.