Should patients with autosomal dominant polycystic kidney disease be screened for cerebral aneurysms?

Abstract

Autosomal dominant polycystic kidney disease is a genetic disorder affecting 1 in 1000 people worldwide and is associated with an increased risk of intracranial aneurysms. It remains unclear whether there is sufficient net benefit to screening this patient population for IA, considering recent developments in imaging and treatment and our evolving understanding of the natural history of unruptured aneurysms. There is currently no standardized screening protocol for IA in patients with ADPCKD. Our review of the literature focused on the above issues and presents our appraisal of the estimated value of screening for IA in the setting of ADPCKD.

Fig 1.

Specific to IA, the preclinical phase between formation and symptom development/rupture can be quite variable, and the clinical phase between symptom development and SAH/death can be quite short or nonexistent. Lead time is dependent on screening frequency. DPCP indicates detectable preclinical phase. Adapted from Morrison by permission of Oxford University Press, USA.