open access
Natural history of intracranial aneurysms in autosomal dominant polycystic kidney disease
- Department of Immunology, Transplant Medicine, and Internal Diseases, Medical University of Warsaw, Poland
- 1 st Department of Radiology, Medical University of Warsaw, Chałubińskiego str 5, 02-004 Warsaw, Poland
open access
Abstract
Autosomal-dominant polycystic kidney disease (ADPKD) is a relatively frequent genetic disorder that is associated with increased prevalence of intracranial aneurysms (IAs). However, evidence on the natural history of IAs in ADPKD is suboptimal. That leads to difficulties in development of recommendations on surveillance on patients with IAs in their medical history, or the need for repeat imaging for IAs in those with a negative result of the initial screening. The aim of the article is to present our experience on the natural history of IAs in ADPKD patients.
Material and methodsThirty-four ADPKD patients, managed at our outpatient department, with imaging for intracranial aneurysms performed at least twice, were included into present retrospective analysis.
ResultsAmong 8 patients with an IA in their medical history, no new IA was observed during 93 patient-years of follow-up. In 6 patients with untreated, unruptured IAs, IA growth was observed in 2 cases during 32 patient-years of follow-up. Finally, among 20 patients with a negative result of initial screening, 2 new IAs were noticed during 115 patient-years of follow-up, including 1 patient with a positive family history for an IA, and 1 patient without a family history.
ConclusionsOur observations support repeat imaging for IAs in patients with ADPKD, positive family history of IA, and negative result of initial screening. Additionally, efforts should be made to develop clinical and/or laboratory risk factors for IAs development in ADPKD patients without family history of IA, which enable to identify patients who should undergo repeat imaging for IAs.
Abstract
Autosomal-dominant polycystic kidney disease (ADPKD) is a relatively frequent genetic disorder that is associated with increased prevalence of intracranial aneurysms (IAs). However, evidence on the natural history of IAs in ADPKD is suboptimal. That leads to difficulties in development of recommendations on surveillance on patients with IAs in their medical history, or the need for repeat imaging for IAs in those with a negative result of the initial screening. The aim of the article is to present our experience on the natural history of IAs in ADPKD patients.
Material and methodsThirty-four ADPKD patients, managed at our outpatient department, with imaging for intracranial aneurysms performed at least twice, were included into present retrospective analysis.
ResultsAmong 8 patients with an IA in their medical history, no new IA was observed during 93 patient-years of follow-up. In 6 patients with untreated, unruptured IAs, IA growth was observed in 2 cases during 32 patient-years of follow-up. Finally, among 20 patients with a negative result of initial screening, 2 new IAs were noticed during 115 patient-years of follow-up, including 1 patient with a positive family history for an IA, and 1 patient without a family history.
ConclusionsOur observations support repeat imaging for IAs in patients with ADPKD, positive family history of IA, and negative result of initial screening. Additionally, efforts should be made to develop clinical and/or laboratory risk factors for IAs development in ADPKD patients without family history of IA, which enable to identify patients who should undergo repeat imaging for IAs.
Keywords
Autosomal dominant polycystic kidney disease, Intracranial aneurysm, Magnetic resonance imaging
Title
Natural history of intracranial aneurysms in autosomal dominant polycystic kidney disease
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
476-480
Page views
267
Article views/downloads
332
DOI
10.1016/j.pjnns.2017.08.007
Bibliographic record
Neurol Neurochir Pol 2017;51(6):476-480.
Keywords
Autosomal dominant polycystic kidney disease
Intracranial aneurysm
Magnetic resonance imaging
Authors
Mariusz Niemczyk
Monika Gradzik
Magda Fliszkiewicz
Andrzej Kulesza
Marek Gołębiowski
Leszek Pączek