Association of Intracranial Aneurysms With Aortic Aneurysms in 125 Patients With Fusiform and 4253 Patients With Saccular Intracranial Aneurysms and Their Family Members and Population Controls

Background-Varying degrees of co-occurrence of intracranial aneurysms (IA) and aortic aneurysms (AA) have been reported. We sought to compare the risk for AA in fusiform intracranial aneurysms (fIA) and saccular intracranial aneurysms (sIA) disease and evaluate possible genetic connection between the fIA disease and AAs. Additionally, the characteristics and aneurysms of the fIA and sIA patients were compared. Methods and Results-The Kuopio Intracranial Aneurysm Database includes all 4253 sIA and 125 fIA patients from its Eastern Finnish catchment population, and 13 009 matched population controls and 18 455 first-degree relatives to the IA patients were identified, and the Finnish national registers were used to identify the individuals with AA. A total of 33 fIA patients were studied using an exomic gene panel of 37 genes associated with AAs. Seventeen (14.4%) fIA patients and 48 (1.2%) sIA patients had a diagnosis of AA. Both fIA and sIA patients had AAs significantly more often than their controls (1.2% and 0.5%) or relatives (0.9% and 0.3%). In a competing risks Cox regression model, the presence of fIA was the strongest risk factor for AA (subdistribution hazard ratio 7.6, 95% CI 3.9-14.9, P Conclusions-The prevalence of AAs is increased slightly in sIA patients and significantly in fIA patients. fIA patients are older and have more comorbid diseases than sIA patients but this alone does not explain their clinically significant AA risk.Peer reviewe

Neurofibromatosis type 1 is not associated with subarachnoid haemorrhage.

The prevalence of intracranial aneurysms (IAs) has been proposed to be elevated in the patients with neurofibromatosis type 1 (NF1). Our aims were to determine the prevalence of NF1 in a large Finnish population based cohort of IA patients and, on the other hand, the occurrences of subarachnoid haemorrhage and unruptured intracranial aneurysms in a nationwide population-based cohort of NF1 patients and its matched ten-fold control cohort.The Kuopio IA Database (www.kuopioneurosurgery.fi) includes all ruptured and unruptured IA cases admitted to the Kuopio University Hospital (KUH) from its defined Eastern Finnish catchment population since 1980. In this registry-based study, we cross-linked the Kuopio IA database with the Finnish national registry covering all hospital diagnoses. The NF1 diagnoses of the 4543 patients with either saccular of fusiform IA were identified from 1969 to 2015 and verified from patient records. Our second approach was to analyze the occurrence of aneurysmal subarachnoid haemorrhage (aSAH) and unruptured IAs in a nationwide population-based database of 1410 NF1 patients and its ten-fold matched control cohort (n = 14030) using national registry of hospital diagnoses between 1987 and 2014.One NF1 patient was identified among the 4543 IA patients. Three verified IA cases (one unruptured IA and two aSAH cases) were identified in the cohort of 1410 NF1 patients, with similar occurrences in the control cohort.We found no evidence in our population-based cohorts to support the conception that NF1 is associated with IAs. Our results indicate that the incidence of aSAH is not elevated in patients with NF1. Further studies are required to confirm that there is no association between NF1 and unruptured IAs

NF1 patients with intracranial aneurysms.

<p>NF1 patients with intracranial aneurysms.</p

Manifestations of NF1 in different organ systems[8].

<p>Manifestations of NF1 in different organ systems[<a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0178711#pone.0178711.ref008" target="_blank">8</a>].</p

Diagnostic criteria for NF1: Two or more of the following findings[2].

<p>Diagnostic criteria for NF1: Two or more of the following findings[<a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0178711#pone.0178711.ref002" target="_blank">2</a>].</p

NF1 cohorts with intracranial aneurysms.

<p>NF1 cohorts with intracranial aneurysms.</p

Development of the SAFETEA Scores for Predicting Risks of Complications of Preventive Endovascular or Microneurosurgical Intracranial Aneurysm Occlusion

Background and ObjectivesPreventive unruptured intracranial aneurysm (UIA) occlusion can reduce the risk of subarachnoid hemorrhage, but both endovascular and microneurosurgical treatment carry a risk of serious complications. To improve individualized management decisions, we developed risk scores for complications of endovascular and microneurosurgical treatment based on easily retrievable patient, aneurysm, and treatment characteristics.MethodsFor this multicenter cohort study, we combined individual patient data from patients with UIA aged 18 years or older undergoing preventive endovascular treatment (standard, balloon-assisted or stent-assisted coiling, Woven EndoBridge-device, or flow-diverting stent) or microneurosurgical clipping at one of the 10 participating centers from 3 continents between 2000 and 2018. The primary outcome was death from any cause or clinical deterioration from neurologic complications ≤30 days. We selected predictors based on previous knowledge about relevant risk factors and predictor performance and studied the association between predictors and complications with logistic regression. We assessed model performance with calibration plots and concordance (c) statistics.ResultsOf the 1,282 included patients, 94 (7.3%) had neurologic symptoms that resolved <30 days, 140 (10.9%) had persisting neurologic symptoms, and 6 died (0.5%). At 30 days, 52 patients (4.1%) were dead or dependent. Predictors of procedural complications were size of aneurysm, aneurysm location, familial subarachnoid hemorrhage, earlier atherosclerotic disease, treatment volume, endovascular modality (for endovascular treatment) or extra aneurysm configuration factors (for microneurosurgical treatment, branching artery from aneurysm neck or unfavorable dome-to-neck ratio), and age (acronym: SAFETEA). For endovascular treatment (n = 752), the c-statistic was 0.72 (95% CI 0.67-0.77) and the absolute complication risk ranged from 3.2% (95% CI 1.6%-14.9%; ≤1 point) to 33.1% (95% CI 25.4%-41.5%; ≥6 points). For microneurosurgical treatment (n = 530), the c-statistic was 0.72 (95% CI 0.67-0.77) and the complication risk ranged from 4.9% (95% CI 1.5%-14.9%; ≤1 point) to 49.9% (95% CI 39.4%-60.6%; ≥6 points).DiscussionThe SAFETEA risk scores for endovascular and microneurosurgical treatment are based on 7 easily retrievable risk factors to predict the absolute risk of procedural complications in patients with UIAs. The scores need external validation before the predicted risks can be properly used to support decision-making in clinical practice.Classification of EvidenceThis study provides Class III evidence that SAFETEA scores predict the risk of procedural complications after endovascular and microneurosurgical treatment of unruptured intracranial aneurysms

Development of the SAFETEA Scores for Predicting Risks of Complications of Preventive Endovascular or Microneurosurgical Intracranial Aneurysm Occlusion

Background and ObjectivesPreventive unruptured intracranial aneurysm (UIA) occlusion can reduce the risk of subarachnoid hemorrhage, but both endovascular and microneurosurgical treatment carry a risk of serious complications. To improve individualized management decisions, we developed risk scores for complications of endovascular and microneurosurgical treatment based on easily retrievable patient, aneurysm, and treatment characteristics.MethodsFor this multicenter cohort study, we combined individual patient data from patients with UIA aged 18 years or older undergoing preventive endovascular treatment (standard, balloon-assisted or stent-assisted coiling, Woven EndoBridge-device, or flow-diverting stent) or microneurosurgical clipping at one of the 10 participating centers from 3 continents between 2000 and 2018. The primary outcome was death from any cause or clinical deterioration from neurologic complications ≤30 days. We selected predictors based on previous knowledge about relevant risk factors and predictor performance and studied the association between predictors and complications with logistic regression. We assessed model performance with calibration plots and concordance (c) statistics.ResultsOf the 1,282 included patients, 94 (7.3%) had neurologic symptoms that resolved <30 days, 140 (10.9%) had persisting neurologic symptoms, and 6 died (0.5%). At 30 days, 52 patients (4.1%) were dead or dependent. Predictors of procedural complications were size of aneurysm, aneurysm location, familial subarachnoid hemorrhage, earlier atherosclerotic disease, treatment volume, endovascular modality (for endovascular treatment) or extra aneurysm configuration factors (for microneurosurgical treatment, branching artery from aneurysm neck or unfavorable dome-to-neck ratio), and age (acronym: SAFETEA). For endovascular treatment (n = 752), the c-statistic was 0.72 (95% CI 0.67-0.77) and the absolute complication risk ranged from 3.2% (95% CI 1.6%-14.9%; ≤1 point) to 33.1% (95% CI 25.4%-41.5%; ≥6 points). For microneurosurgical treatment (n = 530), the c-statistic was 0.72 (95% CI 0.67-0.77) and the complication risk ranged from 4.9% (95% CI 1.5%-14.9%; ≤1 point) to 49.9% (95% CI 39.4%-60.6%; ≥6 points).DiscussionThe SAFETEA risk scores for endovascular and microneurosurgical treatment are based on 7 easily retrievable risk factors to predict the absolute risk of procedural complications in patients with UIAs. The scores need external validation before the predicted risks can be properly used to support decision-making in clinical practice.Classification of EvidenceThis study provides Class III evidence that SAFETEA scores predict the risk of procedural complications after endovascular and microneurosurgical treatment of unruptured intracranial aneurysms