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

Neurosurgery. 2019 Jun 1;84(6):E352-E361. doi: 10.1093/neuros/nyy336.


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.

Keywords: ADPKD; Cerebral aneurysm; Intracranial aneurysm; Polycystic kidney disease; Screening; Subarachnoid hemorrhage.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aneurysm, Ruptured / complications
  • Aneurysm, Ruptured / therapy
  • Female
  • Humans
  • Intracranial Aneurysm / etiology
  • Intracranial Aneurysm / therapy*
  • Male
  • Mass Screening
  • Middle Aged
  • Polycystic Kidney, Autosomal Dominant / complications*
  • Retrospective Studies
  • Subarachnoid Hemorrhage / complications
  • Young Adult