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

Abstract

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. To report outcomes of observation, open surgical, or endovascular management of ruptured and unruptured aneurysms in patients with ADPKD. We performed a retrospective analysis of all patients with ADPKD and IAs at a single center from 2000 to 2016. 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. 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.