Nonischemic Presentations of Pediatric Moyamoya Arteriopathy: A Natural History Study.

Abstract

HomeStrokeVol. 53, No. 6Nonischemic Presentations of Pediatric Moyamoya Arteriopathy: A Natural History Study Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessResearch ArticlePDF/EPUBNonischemic Presentations of Pediatric Moyamoya Arteriopathy: A Natural History Study John R. Gatti, BS and Lisa R. Sun, MD John R. GattiJohn R. Gatti https://orcid.org/0000-0001-9759-2855 Johns Hopkins School of Medicine, Baltimore, MD (J.R.G.). Search for more papers by this author and Lisa R. SunLisa R. Sun Correspondence to: Lisa R. Sun, MD, 200 N Wolfe St Baltimore, MD 21287. Email E-mail Address: [email protected] https://orcid.org/0000-0003-4948-0415 Department of Neurology, Johns Hopkins School of Medicine, Baltimore, MD (L.R.S.). Search for more papers by this author Originally published3 May 2022https://doi.org/10.1161/STROKEAHA.122.038771Stroke. 2022;53:e219–e220Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: May 3, 2022: Ahead of Print Most children with moyamoya arteriopathy (MMA) present with ischemic stroke or transient ischemic attack, but many present with nonischemic symptoms or increasingly via incidental radiographic finding, especially with advancing imaging capabilities.1,2 We describe the natural history of children with nonischemic MMA presentations to characterize long-term stroke risk and optimize management strategies in this unique group.MethodsClinical and neuroimaging data, including mode of presentation (ischemic versus nonischemic) were extracted from the records of pediatric patients with MMA seen at our center.1 Pediatric stroke outcome measure scores were stratified as previously described.3 Differences in stroke-free survival are represented using Kaplan-Meier survival curves and analyzed using Cox Proportional hazard models. See supplement for additional methods.ResultsOf 83 children with MMA, 36 (43%) had nonischemic presentations (Table S1). The most common nonischemic presentation was incidental radiographic finding (53%). Among patients with nonischemic presentations, 47% had evidence of remote ischemic injury on baseline magnetic resonance imaging. Over a median clinical follow-up time of 5.6 years, 5 patients with nonischemic presentations (14%) experienced clinical stroke. Radiographic progression was observed in 13 patients with nonischemic presentations (36%) over a median radiological follow-up time of 4.2 years. Fifteen patients with nonischemic presentations (42%) underwent surgical revascularization of 26 hemispheres at a median of 6 months (interquartile range, 2.5–11 months) after diagnosis. Only 2 surgical patients experienced stroke: one before surgery, one perioperatively. Most children in this cohort had a normal or mild Pediatric Stroke Outcome Measure score, but 2 children (6%) had severe deficits at follow-up.Children with ischemic presentations had higher risk of stroke after diagnosis compared to children with nonischemic presentations (Figure [A], Table S2; hazard ratio, 3.95; P=0.005). This relationship persisted after adjusting for syndromic association and age at presentation (Figure [B]; hazard ratio, 2.94; P=0.050). In surgical patients, there was a trend toward less favorable stroke-free survival in those with ischemic presentations, both after diagnosis (Figure [C]; hazard ratio, 3.01; P=0.075) and after surgery (Figure [D]; hazard ratio, 3.92; P=0.195), despite no difference in time from diagnosis to surgery between groups (P=0.386).Download figureDownload PowerPointFigure. Kaplan-Meier survival curves stratified by ischemic vs nonischemic presentation. Stroke-free survival, (A) unadjusted, (B) adjusted for age and syndromic association, (C) among only surgically managed patients, unadjusted, and (D) postoperatively, unadjusted. HR indicates hazard ratio.DiscussionThe stroke rate among patients with nonischemic presentations was 14% over 5.6 years, which is lower than rates of recurrent stroke when children present with ischemic symptoms in our cohort and in previous literature.1,2,4 The reason for this discrepancy is unclear but likely due to a combination of factors, including inherently milder arteriopathy and implementation of therapies earlier in the disease course. Limitations of our study include its retrospective nature and small sample size. Strengths include robust follow-up time and a diverse cohort with good representation of children with syndromic associations.ConclusionsWe describe a relatively low rate of stroke among children with MMA who present without ischemic symptoms, though unfavorable outcome is still possible. This informs prognosis in this unique group. Further studies concerning selection of patients presenting without ischemic symptoms for surgical management and ideal timing of surgery are necessary.Article InformationSources of FundingThis work is supported by the Laney Jaymes Foundation for Pediatric Stroke, the American Heart Association (Career Development Award [850044]), and the D.C. Women’s Board.Supplemental MaterialSTROBE checklistSupplemental MethodsSupplemental ResultsTables S1–S3Figures S1–S2Disclosures None.FootnotesSupplemental Material is available at https://www.ahajournals.org/doi/suppl/10.1161/STROKEAHA.122.038771.For Sources of Funding and Disclosures, see page e220.Correspondence to: Lisa R. Sun, MD, 200 N Wolfe St Baltimore, MD 21287. Email [email protected]edu