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Key Points IAs location distribution in patients with ADPKD differ from the ones in non-ADPKD patients IAs in patients with ADPKD are more commonly located in the anterior circulation and in large caliber arteries Because of IA multiplicity and singular IA distribution, patients with ADPKD represent a special population who need to be closely followed Background Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic condition associated with intracranial aneurysms (IAs). The associated pathophysiology remains unknown, but an association with wall shear stress is suspected. Cerebral arterial location is the principal factor influencing IA natural history. This study aims to compare IA location-specific distribution between ADPKD and non-ADPKD patients. Methods The ADPKD group comprised data from a systematic review of the literature (2016–2020, N =7) and three cohorts: integrated biomedical informatics for the management of cerebral aneurysms, Novosibirsk, and Unruptured Cerebral Aneurysms Study. The non-ADPKD group was formed from the integrated biomedical informatics for the management of cerebral aneurysms, Unruptured Cerebral Aneurysms Study, International Stroke Genetics Consortium, and the Finnish cohort from the literature. Patients and IAs characteristics were compared between ADPKD and non-ADPKD groups, and a meta-analysis for IA locations was performed. Results A total of 1184 IAs from patients with ADPKD were compared with 21,040 IAs from non-ADPKD patients. In total, 78.6% of patients with ADPKD had hypertension versus 39.2% of non-ADPKD patients. A total of 32.4% of patients with ADPKD were smokers versus 31.5% of non-ADPKD patients. In total, 30.1% of patients with ADPKD had a positive family history for IA versus 15.8% of the non-ADPKD patients. Patients with ADPKD showed a higher rate of IA multiplicity (33.2% versus 23.1%). IAs from patients with ADPKD showed a significant predominance across the internal carotid and middle cerebral arteries. Posterior communicating IAs were more frequently found in the non-ADPKD group. The meta-analysis confirmed a predominance of IAs in the patients with ADPKD across large caliber arteries (odds ratio [95% confidence interval]: internal carotid artery: 1.90 [1.10 to 3.29]; middle cerebral artery: 1.18 [1.02–1.36]). Small diameter arteries, such as the posterior communicating, were observed more in non-ADPKD patients (0.21 [0.11–0.88]). Conclusion This analysis shows that IAs diagnosed in patients with ADPKD are more often localized in large caliber arteries from the anterior circulation in comparison with IAs in non-ADPKD patients. It shows that primary cilia driven wall shear stress vessel remodeling to be more critical in cerebral anterior circulation large caliber arteries.

Objective: We previously derived the Unruptured Intracranial Aneurysm Treatment Score (UIATS), designed using a multidisciplinary consensus approach among neurovascular specialists from diverse geographic and practice backgrounds. Here, we report on the development and validation of the final version of UIATS. Method: An international, multidisciplinary (neurosurgery, neuroradiology, neurology, clinical epidemiology) group of 69 (39 panel members and 30 blinded external reviewers) specialists in the research and treatment of UIAs was convened. A web survey-based Delphi consensus process consisting of 7 rounds was utilized to rate numerous features of potential relevance in the assessment and treatment of UIAs and to develop the UIATS. Mean ratings were repeatedly used to determine statistical weight for each factor and then transformed into corresponding scores for every item to create the UIATS. For internal and blinded external validation, 30 representative cases of patients with UIAs were used to test the level of agreement (5 point Likert Scale) with treatment recommendations based on the UIATS. Results: The final UIATS system was designed in three domains (patient-, aneurysm - and treatment-related), comprising 13 different categories and 29 different features (Figure 1). Mean agreement based on Likert scores (5 indicating strong agreement and 1 indicating strong disagreement) was 4·2 for both reviewer cohorts, whereas mean agreement per case was 4·2 (panel members) and 4·5 (external reviewers) (p=0·017, Mann-Whitney-U Test). Conclusion: The final version of UIATS system was internally and externally validated by a large multidisciplinary group of neurovascular specialists, which suggests that the UIATS reflects contemporary decision-making regarding management of a patient with an UIA. The UIATS may aid clinicians in deciding on the appropriate management for an UIA.

Abstract BACKGROUND Giant fusiform and dolichoectatic aneurysms of the basilar trunk and vertebrobasilar junction (BTVBJ-GFDA) are extremely difficult to treat. OBJECTIVE To evaluate factors influencing survival and outcome of BTVBJ-GFDA by performing a retrospective multicenter cohort study. METHODS A total of 32 patients with BTVBJ-GFDA were included in this study. Clinicopathological characteristics, treatment measures, and outcomes were collected from medical records and imaging studies. Autopsy and histological findings of the aneurysm and adjacent brain tissue were also obtained in 9 cases. RESULTS A total of 11 patients did not undergo surgery, of whom 10 died; 3 from progressive brainstem compression, 4 from subarachnoid hemorrhage, 2 from brainstem infarction, and 1 from associated atherosclerotic disease. The remaining 21 patients underwent a surgical treatment, consisting of immediately proximal parent artery occlusion, remotely proximal parent artery occlusion, clip reconstruction, and distal bypass and achieved significantly longer overall survival compared with those who received conservative therapy (adjusted hazard ratio 1.508, 95% CI 1.058-2.148, P = .02). Histological examination of the aneurysms demonstrated staged clots, open lumen, and intrathrombotic channels with endothelial lining. The patients younger than 45 yr of age showed statistically longer survival than those equal and older than 45 yr ( P = .03). CONCLUSION Surgical intervention achieved greater survival than conservative management in BTVBJ-GFDA. Narrow ideal treatment window of the blood flow within the aneurysm to maintain sufficient but not excess supply should be targeted based on the hemodynamics of both the posterior communicating arteries and perforating vessel collaterals.

Background and Purpose— To address the increasing need to counsel patients about treatment indications for unruptured intracranial aneurysms (UIA), we endeavored to develop a consensus on assessment of UIAs among a group of specialists from diverse fields involved in research and treatment of UIAs. Methods— After composition of the research group, a Delphi consensus was initiated to identify and rate all features, which may be relevant to assess UIAs and their treatment by using ranking scales and analysis of inter-rater agreement (IRA) for each factor. IRA was categorized as very high, high, moderate, or low. Results— Ultimately, 39 specialists from 4 specialties agreed (high or very high IRAs) on the following key factors for or against UIA treatment decisions: (1) patient age, life expectancy, and comorbid diseases; (2) previous subarachnoid hemorrhage from a different aneurysm, family history for UIA or subarachnoid hemorrhage, nicotine use; (3) UIA size, location, and lobulation; (4) UIA growth or de novo formation on serial imaging; (5) clinical symptoms (cranial nerve deficit, mass effect, and thromboembolic events from UIAs); and (6) risk factors for UIA treatment (patient age and life expectancy, UIA size, and estimated risk of treatment). However, IRAs for features rated with low relevance were also generally low, which underlined the existing controversy about the natural history of UIAs. Conclusions— Our results highlight that neurovascular specialists currently consider many features as important when evaluating UIAs but also highlight that the appreciation of natural history of UIAs remains uncertain, even within a group of highly informed individuals.

ABSTRACT OBJECTIVE Remote cerebellar hemorrhage (RCH) is an infrequent and poorly understood complication of supratentorial neurosurgical procedures. We retrospectively compared 42 patients who experienced RCH with a case-matched control cohort, to delineate risk factors associated with the occurrence of this complication. METHODS Between 1988 and 2000, 42 patients experienced RCH after supratentorial neurosurgical procedures at our institution. Diagnoses were made on the basis of postoperative computed tomographic or magnetic resonance imaging findings in all cases. The medical records for these patients were reviewed and compared with those for a control cohort of 43 patients, matched for age, sex, surgical lesion, and type of craniotomy, who were treated during the same period. RESULTS RCH most commonly occurred after frontotemporal craniotomies for unruptured aneurysm repair or temporal lobectomy and was frequently an incidental finding on postoperative computed tomographic scans. However, some cases of RCH were associated with significant morbidity, and two patients died. Preoperative aspirin use and elevated intraoperative systolic blood pressure were significantly associated with RCH (P = 0.026 and P = 0.036, respectively). Pathological findings for two cases demonstrated hemorrhagic infarctions in both. CONCLUSION RCH most commonly follows supratentorial neurosurgical procedures, performed with the patient in the supine position, that involve opening of cerebrospinal fluid cisterns or the ventricular system (such as unruptured aneurysm repair or temporal lobectomy). Preoperative aspirin use and moderately elevated intraoperative systolic blood pressure are potentially modifiable risk factors associated with the development of RCH. Although RCH can cause death or major morbidity, most cases are asymptomatic or exhibit a benign course. Cerebellar “sag” as a result of cerebrospinal fluid hypovolemia, causing transient occlusion of superior bridging veins within the posterior fossa and consequent hemorrhagic venous infarction, is the most likely pathophysiological cause of RCH.