open access

Vol 51, No 6 (2017)
Original research articles
Submitted: 2017-07-19
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Natural history of intracranial aneurysms in autosomal dominant polycystic kidney disease

Mariusz Niemczyk, Monika Gradzik, Magda Fliszkiewicz, Andrzej Kulesza, Marek Gołębiowski, Leszek Pączek
DOI: 10.1016/j.pjnns.2017.08.007
·
Neurol Neurochir Pol 2017;51(6):476-480.

open access

Vol 51, No 6 (2017)
Original research articles
Submitted: 2017-07-19

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 methods

Thirty-four ADPKD patients, managed at our outpatient department, with imaging for intracranial aneurysms performed at least twice, were included into present retrospective analysis.

Results

Among 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.

Conclusions

Our 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 methods

Thirty-four ADPKD patients, managed at our outpatient department, with imaging for intracranial aneurysms performed at least twice, were included into present retrospective analysis.

Results

Among 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.

Conclusions

Our 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.

Get Citation

Keywords

Autosomal dominant polycystic kidney disease, Intracranial aneurysm, Magnetic resonance imaging

About this article
Title

Natural history of intracranial aneurysms in autosomal dominant polycystic kidney disease

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 51, No 6 (2017)

Pages

476-480

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

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