Screening for intracranial aneurysms in ADPKD

doi:10.1136/bmj.b3763

Published 21 September 2009, doi:10.1136/bmj.b3763
Cite this as: BMJ 2009;339:b3763

Editorials

Screening for intracranial aneurysms in ADPKD

A more accurate risk assignment model is needed

The first 150 words of the full text of this article appear below.

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