{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T14:23:20Z","timestamp":1793024600682,"version":"4.5.0"},"reference-count":30,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2024,4,10]],"date-time":"2024-04-10T00:00:00Z","timestamp":1712707200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>To describe the rates of stroke and craniocervical vasculopathy progression in children with posterior fossa malformations, hemangioma, arterial anomalies, coarctation of the aorta\/cardiac defects, and eye abnormalities (PHACE) syndrome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>A single\u2010center, retrospective natural history study of children with PHACE syndrome. Clinical and sequential neuroimaging data were reviewed to study the characteristics and progression of vasculopathy and calculate the rates of arterial ischemic stroke (AIS) and transient ischemic stroke (TIA). Vasculopathy progression was defined as worsening or new vascular findings on follow\u2010up magnetic resonance angiography.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010four children with cerebrovascular abnormalities at the PHACE syndrome diagnosis were studied (age range\u2009=\u20092 to 18\u2009years, 85% females). Median age at the initial diagnosis was 5.5\u2009months (interquartile range\u2009=\u20091\u201352\u2009months); median age at the last follow\u2010up was 8\u2009years 6\u2009months (range\u2009=\u20092\u201318\u2009years). Overall, 10 (29%) patients had radiological progression of their vasculopathy, with a cumulative progression\u2010free rate of 73% (95% confidence interval [CI]\u2009=\u20090.57\u20130.89), and a cumulative TIA\u2010free and AIS\u2010free rate of 87% (95% CI\u2009=\u20090.745\u20130.99). Vasculopathy was continuously progressive in six patients (18%) at the last follow\u2010up. Three patients (9%) had TIA and all had progressive vasculopathy. One patient had presumed perinatal AIS at the initial PHACE diagnosis, while no other patient experienced an AIS during the follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>In children with PHACE syndrome, craniocervical vasculopathy is non\u2010progressive and asymptomatic in the majority of cases. The risk of ischemic stroke in these children is very low. Larger and prospective studies are necessary to confirm these findings.<\/jats:p><\/jats:sec><jats:sec><jats:title>What this paper adds<\/jats:title><jats:p>\n<jats:list list-type=\"bullet\">\n<jats:list-item><jats:p>Cerebrovascular vasculopathy in children with PHACE syndrome is predominantly non\u2010progressive and asymptomatic.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>Cerebrovascular vasculopathy in children with PHACE syndrome is associated with a low risk of stroke during childhood.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>In children with progressive vasculopathy, neuroimaging progression was generally slow over the first years of life.<\/jats:p><\/jats:list-item>\n<\/jats:list><\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.15916","type":"journal-article","created":{"date-parts":[[2024,4,10]],"date-time":"2024-04-10T13:54:20Z","timestamp":1712757260000},"page":"1348-1360","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Long\u2010term clinical and radiological trajectories of craniocervical vasculopathy in children with <scp>PHACE<\/scp> syndrome"],"prefix":"10.1111","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5315-3738","authenticated-orcid":false,"given":"Moran","family":"Hausman\u2010Kedem","sequence":"first","affiliation":[{"name":"Tel Aviv Sourasky Medical Center Pediatric Neurology Institute, Dana\u2010Dwek Children's Hospital  Tel Aviv Israel"},{"name":"Faculty of Medicine Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0986-5476","authenticated-orcid":false,"given":"Elysa","family":"Widjaja","sequence":"additional","affiliation":[{"name":"Division of Neuroradiology, Department of Diagnostic Imaging The Hospital for Sick Children  Toronto ON Canada"},{"name":"Department of Medical Imaging Ann &amp; Robert H. Lurie Children's Hospital  Chicago USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronan J.","family":"Vieira\u00a0Neto","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology Hospital S\u00edrio\u2010Liban\u00eas  S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Pope","sequence":"additional","affiliation":[{"name":"Division of Dermatology, Department of Pediatrics Hospital for Sick Children  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene","family":"Lara\u2010Corrales","sequence":"additional","affiliation":[{"name":"Division of Dermatology, Department of Pediatrics Hospital for Sick Children  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5927-3132","authenticated-orcid":false,"given":"Nomazulu","family":"Dlamini","sequence":"additional","affiliation":[{"name":"Children's 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Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1440-0641","authenticated-orcid":false,"given":"Mahendranath","family":"Moharir","sequence":"additional","affiliation":[{"name":"Children's Stroke Program, Division of Neurology, Department of Pediatrics The Hospital for Sick Children  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,4,10]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1996.03890270083012"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2006.06.021"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.31189"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A0937"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320520203"},{"issue":"24","key":"e_1_2_10_7_1","article-title":"PHACE 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