scholarly journals Value of magnetic resonance angiography for the detection of intracranial aneurysms in autosomal dominant polycystic kidney disease.

1993 ◽  
Vol 3 (12) ◽  
pp. 1871-1877 ◽  
Author(s):  
J Huston ◽  
V E Torres ◽  
P P Sulivan ◽  
K P Offord ◽  
D O Wiebers

The association of intracranial aneurysms with autosomal dominant polycystic kidney disease (ADPKD), the 30-day mortality rate exceeding 50% for aneurysmal rupture, the effectiveness of surgical repair of unruptured aneurysms with a low surgical risk, and the development of noninvasive imaging techniques for their detection have led physicians to consider the value of screening patients with ADPKD for unruptured intracranial aneurysms. The sensitivity and specificity of high-resolution computed tomography and magnetic resonance imaging for the diagnosis of small intracranial aneurysms have been disappointing. To determine the value of magnetic resonance angiography (MRA), 85 patients with ADPKD without symptoms related to an intracranial aneurysm and 2 patients with ADPKD presenting with a subarachnoid hemorrhage or a suspected aneurysmal leak were studied. MRA was performed with the Multisequence Vascular Package (GE Medical Systems) with use of three-dimensional time-of-flight and three-dimensional phase-contrast techniques, and postprocessing maximum intensity projection images were generated to eliminate the problem of overlapping vessels. Asymptomatic intracranial aneurysms were detected in 6 (22%) of 27 patients with and 3 (5%) of 56 patients without a family history of intracranial aneurysm or subarachnoid hemorrhage (P = 0.02, information missing in 2 patients) and in the 2 patients who presented with a symptomatic aneurysm. A stepwise logistic regression analysis indicated that a family history of intracranial aneurysm or subarachnoid hemorrhage was independently associated with the presence of intracranial aneurysms. All of the aneurysms were < or = 6.5 mm in diameter.(ABSTRACT TRUNCATED AT 250 WORDS)

2017 ◽  
Vol 44 (1-2) ◽  
pp. 75-82 ◽  
Author(s):  
Zien Zhou ◽  
Ying Xu ◽  
Candice Delcourt ◽  
Jiehui Shan ◽  
Qiang Li ◽  
...  

Background: The prevalence of intracranial aneurysm in patients with autosomal dominant polycystic kidney disease (ADPKD) is higher than that among the general population. We performed a systematic review and meta-analysis on the prevalence and natural history of intracranial aneurysm among patients with ADPKD. Methods: Medline, Embase, Web of Science and Scopus, from inception to July 2016, were searched for studies reporting the occurrence of intracranial aneurysms among participants with ADPKD. Two authors independently assessed the eligibility of all retrieved studies and extracted data. Information on the prevalence of intracranial aneurysms and their natural history in participants with ADPKD was collected from all included studies. Results: Fifteen studies with 1,490 participants with ADPKD were pooled to study about the prevalence of intracranial aneurysm in participants with ADPKD, and the prevalence rate was found to be 10% (95% CI 7-13%). Studies from China, Japan and Europe (Germany, Poland) reported a higher prevalence of intracranial aneurysm. Having a family history of haemorrhagic stroke or intracranial aneurysm was a risk factor for aneurysm occurrence. Twenty-three percent (95% CI 15-31%) of the participants had multiple aneurysms. Most of the aneurysms were small (<6 mm) and located in the anterior circulation. Five studies with 171 participants (83 with 106 aneurysms at baseline and 88 without) were analyzed to understand the natural history of aneurysms, with an incidence of aneurysm growth, new aneurysm and aneurysm rupture of 1.84% (followed up for 435 person-years), 0.57% (1,227 person-years) and 0.13% (792 person-years) respectively. Conclusions: Screening for intracranial aneurysm is recommended in patients with ADPKD when there is a family history of haemorrhagic stroke or intracranial aneurysm and when they are from China, Japan or Europe (Germany, Poland). Based on existing data, regular imaging follow-up is not supported. High-quality, prospective studies are needed in the future.


2019 ◽  
Vol 14 (8) ◽  
pp. 1151-1160 ◽  
Author(s):  
Irina M. Sanchis ◽  
Shehbaz Shukoor ◽  
Maria V. Irazabal ◽  
Charles D. Madsen ◽  
Fouad T. Chebib ◽  
...  

Background and objectivesIntracranial aneurysm rupture is the most devastating complication of autosomal dominant polycystic kidney disease. Whether selective or widespread intracranial aneurysm screening is indicated remains controversial.Design, setting, participants & measurementsRecords of 3010 patients with autosomal dominant polycystic kidney disease evaluated at the Mayo Clinic between 1989 and 2017 were reviewed. Those who had presymptomatic magnetic resonance angiography screening were included.ResultsNinety-four intracranial aneurysms were diagnosed in 75 of 812 (9%) patients who underwent magnetic resonance angiography screening. Sex, age, race, and genotype were similar in the groups with and without aneurysms; hypertension and history of smoking were more frequent in the aneurysm group. Twenty-nine percent of patients with aneurysms compared with 11% of those without aneurysms had a family history of subarachnoid hemorrhage (P<0.001). Most aneurysms were small (median diameter =4 mm; range, 2–12 mm); 85% were in the anterior circulation. During a total imaging follow-up of 469 patient-years, de novo intracranial aneurysms were detected in five patients; eight intracranial aneurysms grew (median =2 mm; range, 1–3 mm). During a total clinical follow-up of 668 patient-years, seven patients had preemptive clipping or coil embolization; no intracranial aneurysms ruptured. During a total clinical follow-up of 4783 patient-years in 737 patients with no intracranial aneurysm detected on the first magnetic resonance angiography screening, two patients had an intracranial aneurysm rupture (0.04 per 100 person-years; 95% confidence interval, 0 to 0.10). The rate of intracranial aneurysm rupture in large clinical trials of autosomal dominant polycystic kidney disease was 0.04 per 100 patient-years (95% confidence interval, 0.01 to 0.06).ConclusionsIntracranial aneurysms were detected by presymptomatic screening in 9% of patients with autosomal dominant polycystic kidney disease, more frequently in those with familial history of subarachnoid hemorrhage, hypertension, or smoking. None of the patients with and two of the patients without aneurysm detection on screening suffered aneurysmal ruptures. The overall rupture rate in our autosomal dominant polycystic kidney disease cohort was approximately five times higher than that in the general population.


2017 ◽  
Vol 51 (6) ◽  
pp. 476-480 ◽  
Author(s):  
Mariusz Niemczyk ◽  
Monika Gradzik ◽  
Magda Fliszkiewicz ◽  
Andrzej Kulesza ◽  
Marek Gołębiowski ◽  
...  

Author(s):  
Yves Pirson ◽  
Dominique Chauveau

An asymptomatic intracranial aneurysm (ICA) is found by screening in about 8% of patients with autosomal dominant polycystic kidney disease (ADPKD), with a trend to cluster in some families. Though most ICAs will remain asymptomatic, a minority of them may rupture, causing subarachnoid haemorrhage (SAH). Given the grave prognosis of ICA rupture, screening and prophylactic repair of unruptured ICAs have to be considered, with the aim to identify patients with a risk of ICA rupture that exceeds the risk of a prophylactic procedure, surgical or endovascular. Relying on a decision analysis model established in the general population, widespread screening in ADPKD patients is today not recommended. However, the chapter authors advise screening in ADPKD patients with a familial history of ICA or SAH. Additional acceptable indications are high-risk occupations and patient anxiety despite adequate information. Screening is preferably performed by high-resolution, three-dimensional, time-of-flight magnetic resonance imaging. When an asymptomatic ICA is found, a recommendation for whether to intervene depends on its size, site, morphology, patient life expectancy, and general health as well as the experience of the neuroradiologist–neurosurgeon team. Since the risk of new ICAs or enlargement of an existing one is very low in those with small (< 6 mm) ICAs, conservative management is usually recommended. Elimination of tobacco use and aggressive treatment of hypertension are strongly recommended.


1992 ◽  
Vol 3 (1) ◽  
pp. 88-95 ◽  
Author(s):  
W I Schievink ◽  
V E Torres ◽  
D G Piepgras ◽  
D O Wiebers

The literature on the association of intracranial aneurysms in autosomal dominant polycystic kidney disease (ADPKD) consists mainly of case reports and small series of patients. To provide a more-detailed description of this association and its frequency, the records of all ADPKD patients with saccular intracranial aneurysms, all ADPKD autopsy cases including brain examination, and sex- and age-matched autopsy cases without ADPKD seen at the Mayo Clinic between 1950 and 1989 and of all Rochester residents with a diagnosis of subarachnoid hemorrhage or ADPKD between 1945 and 1984 were reviewed. The presentation of the 41 patients (22 men and 19 women; mean age, 46.4 yr) with this association was subarachnoid hemorrhage in 33, transient ischemic attacks in 2, incidental angiographic or autopsy finding in 5, and discovery during angiographic screening in 1. Thirty-one, seven, and three patients harbored one, two, and three aneurysms, respectively, arising from the middle cerebral artery (N = 23), anterior communicating artery (N = 16), internal carotid artery (N = 11), and vertebral or basilar artery (N = 4). A family history of intracranial aneurysm, subarachnoid hemorrhage, or intracranial hemorrhage at an early age was present in 22% of the patients. Small aneurysms (less than 5 mm) were less likely to have ruptured or caused symptoms (P less than 0.04). There was a trend for hypertension to be associated with the severity of the subarachnoid hemorrhage. Aneurysmal rupture occurred before age 50 in 64% of patients. Of the 89 ADPKD autopsy cases with brain examination, 22.5% had intracranial aneurysms.(ABSTRACT TRUNCATED AT 250 WORDS)


1998 ◽  
Vol 89 (3) ◽  
pp. 479-482 ◽  
Author(s):  
Wouter I. Schievink ◽  
Virginia Prendergast ◽  
Joseph M. Zabramski

✓ Intracranial aneurysms are common extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD). Although their natural history is not completely understood, small asymptomatic intracranial aneurysms in patients with ADPKD often are not treated but are followed with serial magnetic resonance (MR) angiography. The authors report the unique case of a patient with ADPKD who bled from a previously documented asymptomatic 3-mm intracranial aneurysm. This 42-year-old man with ADPKD suffered a subarachnoid hemorrhage (SAH) from a 7-mm left pericallosal artery aneurysm. This aneurysm was clipped and the patient made an excellent recovery. An irregular asymptomatic 3-mm right middle cerebral artery (MCA) aneurysm had also been demonstrated on angiography. While the patient was considering elective surgery for the MCA aneurysm, he suffered a hemorrhage from this lesion 10 weeks after the initial SAH. The aneurysm was clipped and the patient made a satisfactory recovery (he was moderately disabled). In this report the authors indicate that small asymptomatic intracranial aneurysms are not always innocuous in patients with ADPKD, and they suggest that treatment should be strongly considered for these lesions in this group of patients when there is a history of SAH or the aneurysm is irregular in appearance. Because MR angiography studies may not adequately define the configuration of small aneurysms and irregularity may easily be missed, conventional angiography is recommended for patients with ADPKD who are found to have an intracranial aneurysm on screening with MR angiography.


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