Cerebral Aneurysms in Autosomal Dominant Polycystic Kidney Disease: A Comparison of Management Approaches

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a risk factor for formation of intracranial aneurysms (IAs), though the ideal screening and treatment strategies in this population are unclear. OBJECTIVE: To report outcomes of observation, open surgical, or endovascular management of ruptured and unruptured aneurysms in patients with ADPKD. METHODS: We performed a retrospective analysis of all patients with ADPKD and IAs at a single center from 2000 to 2016. RESULTS: Forty-five patients with ADPKD harboring 71 aneurysms were identified, including 11 patients with subarachnoid hemorrhage (SAH). Of 22 aneurysms managed with observation, none ruptured in 136 yr of clinical follow-up. Thirty-five aneurysms were treated with open surgery and 14 with an endovascular approach. Among treated aneurysms, poor neurologic outcome (modified Rankin scale >2) was seen only in patients presenting with SAH (17% SAH vs 0% elective, P = .06). Acute kidney injury (AKI) was also significantly associated with SAH presentation (22% SAH vs 0% elective, P = .05). Neither procedural complications nor AKI were associated with treatment modality. Among 175 yr of radiographic follow-up in patients with known IAs, 8 de novo aneurysms were found, including 3 that were treated. Of 11 patients with SAH, 7 ruptured in the setting of previously known ADPKD, including 2 with prior angiographic screening and 5 without screening. CONCLUSION: Poor outcomes occurred only with ruptured presentation but were equivalent between treatment modalities. Screening is performed only selectively, and 64% (7 of 11) of patients presenting with SAH had previously known ADPKD.