Abstract WP226: Perioperative Stroke and Cardiac Anomalies Associated With Posterior Cerebral Artery Stenosis in Children With Moyamoya

Abstract

Introduction: Moyamoya is a progressive steno-occlusive arteriopathy of the intracranial circulation affecting the anterior circulation and less often the posterior circulation. Risk factors and severity of disease progression in children with moyamoya and posterior cerebral artery (PCA) stenosis have not been well described in the literature. Previous research has shown an association between PCA stenosis and increased risk of post-operative stroke. We wanted to further explore this relationship in children with moyamoya and delineate the associated risk factors. Methods: Children with moyamoya who underwent surgical revascularization at our institution between December 2015 and December 2022 were reviewed for presence of PCA stenosis. Clinical and radiographic features were compared between children with moyamoya with and without PCA involvement. Categorical variables were compared using Fisher's exact test and continuous variables using Wilcoxon rank-sum. Associations between perioperative stroke and PCA stenosis were analyzes using a multivariate logistic regression. Results: In our cohort of 160 children with moyamoya, 45 (28%) had PCA stenosis; 26 of these 45 (57%) had PCA stenosis at time of diagnosis. Of the 19 children who did not have PCA stenosis at time of diagnosis but developed it during follow up, the median time to development of PCA stenosis was 1.0 years. Children with moyamoya and PCA stenosis had significant more cardiac anomalies than those without (p=0.02). Other associated disorders were found in 13 (28%) including trisomy 21 (6, 13%), cranial radiation (2,4%) and NF1 (3,7%). Interestingly, we found no significant difference in age, sex or prevalence of bilateral disease between the groups. Children with moyamoya and PCA stenosis had a 3.5 higher odds of having a stroke within 2 weeks of surgery (p=0.049). Conclusion: Children with moyamoya and PCA stenosis had a 3 fold higher odds of perioperative stroke compared to those without PCA stenosis. Genetic evaluations may be warranted given association of PCA stenosis and cardiac anomalies.