A follow-up study of autosomal dominant polycystic kidney disease with intracranial aneurysms using 3.0 T three-dimensional time-of-flight magnetic resonance angiography
Introduction
Autosomal dominant polycystic kidney disease (ADPKD) is a common multi-systemic and monogenic hereditary disease. The relationship between intracranial aneurysms (IAs) and ADPKD was first found through a large number of autopsies and confirmed by conventional and non-invasive angiography [1], [2], [3], [4]. Many ADPKD patients died of subarachnoid hemorrhage due to ruptured aneurysms rather than nephropathy. Unruptured intracranial aneurysms (UIAs) can also cause various focal neural symptoms such as parmlysis of cranial nerves, transient ischemic attack and epileptic seizure. Due to the high incidence and mortality of ADPKD with IAs, the importance of screening for UIAs in asymptomatic ADPKD patients has been gradually realized.
Three-dimensional time-of-flight magnetic resonance angiography (3D-TOF MRA) displays blood vessels by taking advantage of the natural contrast between the flow effect of continuous running blood and the surrounding stationary tissues in MR imaging. This technique is non-invasive, non-radioactive and contrast-free, and can perform 3D reconstruction and rotate at arbitrary angles to demonstrate the site, morphology and size of an aneurysm and its relationship with the involved artery, thus helping show the overall perspective of the intra-cranial arteries and compare the bilateral arteries simultaneously. Some authors had assessed the value of MRA in the follow-up of patients with ADPKD and saccular IAs [1], [5], [6], but rare cases were performed using clinical 3.0 T MR scanner with three-dimensional reconstruction technology. Therefore, the present study was intended to screen and follow up the UIAs detected by 3.0 T 3D-TOF MRA in patients with ADPKD in order to evaluate the growth of UIAs and the value of 3D-TOF MRA in the follow-up.
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Participants
This study was approved by the ethics committee of our department. Informed consent was obtained from each subject participating in this study.
From November 1, 2007 to November 20, 2008, 355 consecutive patients with ADPKD participated in the study [7]. The diagnosis of ADPKD was made on the basis of the family history, and abdominal ultrasound findings or CT examination showing more than 5 cysts in both kidneys. Each patient underwent a 3.0 T 3D-TOF MRA. The 355 patients (169 women and 186 men)
Clinical features of ADPKD patients with IAs
Of the 355 ADPKD patients, 54 UIAs were detected in 44 patients (12.4%). The mean duration of ADPKD in company with IAs was 12.2 ± 8.0 years. Except for 12 patients (27.3%), most patients (n = 32, 73.7%) did not have a definite or possible family history of stoke or IAs. Thirty-two (72.7%) ADPKD patients with IAs had a history of hypertension.
Forty patients (age 52.3 ± 9.54 years; 21 women and 19 men) who underwent MRA follow-up with an interval of at least 36 months came from 36 families, in whom 50
Statistical results
Inter- and intraobserver measurement agreements were very good for the calculation of UIAs. Intraobserver agreement among the three observers in 40 patients was 83.3% and the kappa value was 0.85 (P < 0.001). Logistic regressive analysis did not elicit statistically significant risk factors or interactive variables. Although the whole model had statistic significance (P < 0.05), none of the possible risk factors was statistically significant (P > 0.05).
Discussion
In this study, we used 3.0 T 3D-TOF MRA to follow up ADPKD patients with UIAs, and found that compared with 1.5 T MR [1], it had a higher signal-to-noise ratio and a better background suppression, allowing for a clearer delineation of vascular walls and providing an easier view to observe the relationship between the aneurysm and the adjacent small vessels. Although digital subtraction angiography (DSA) is considered as the gold standard for the detection of intracranial aneurysms, the use of DSA
Funding sources
This work was supported in part by the National Natural Science Foundation of China (No.81101044, 81230030), Shanghai Rising-Star Program (12QA1404700), Special program of military medicine of second military medical university (2011JS18), Shanghai Postdoctoral Science Funds (12R21418400), Changhai Hospital 1255 Scientific Innovation Funds (CH125541000).
Role of the funding source
We declare no conflicts of interest.
Conflict of interest statement
The authors have reported that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
Acknowledgements
We thank the National Natural Science Foundation of China (No. 81101044, 81230030), Shanghai Rising-Star Program (12QA1404700), Special program of military medicine of second military medical university (2011JS18), Shanghai Postdoctoral Science Funds (12R21418400), Changhai Hospital 1255 Scientific Innovation Funds (CH125541000) for the financial supports.
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These authors contributed equally to this work.