Screening for intracranial aneurysms in ADPKD

BMJ 2009; 339 doi: https://doi.org/10.1136/bmj.b3763 (Published 21 September 2009) Cite this as: BMJ 2009;339:b3763

This article has a correction. Please see:

  1. Albert C M Ong, professor of renal medicine
  1. 1Academic Nephrology Unit, Faculty of Medicine, Dentistry and Health, University of Sheffield, Beech Hill Road, Sheffield S10 2RX
  1. a.ong{at}sheffield.ac.uk

    A more accurate risk assignment model is needed

    Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common monogenic human diseases, with an incidence of 1 in 1000. Asymptomatic aneurysms can be detected in 6% of patients with ADPKD without a family history, but in up to 16% of patients with a family history.1 This compares with an estimated prevalence of 1-2% in the general population. Intracranial aneurysm rupture is a rare but devastating complication of AKPKD that occurs on average 10 years younger than sporadic intracranial aneurysms. The youngest reported case was a 13 week old infant, and in one study 10% of patients were younger than 21 years.2 Intracranial aneurysm rupture is associated with a death rate of up to 65%. Treatment of a ruptured intracranial aneurysm by either neurosurgical clipping or endovascular treatment also carries an unacceptably high mortality rate of 8-10% and morbidity (disability or dependency) rate of 16-21%.3

    The risk of rupture of asymptomatic intracranial aneurysms occurring in the general population is primarily determined by size, location, and a history …

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