{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T04:33:23Z","timestamp":1790483603681,"version":"4.1.0"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,10,15]],"date-time":"2018-10-15T00:00:00Z","timestamp":1539561600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2019,3]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>Neurodevelopmental outcomes in children with congenital cerebellar malformations (<jats:styled-content style=\"fixed-case\">CCM<\/jats:styled-content>s) remain poorly defined. We aimed to assess whether specific neuroimaging features in <jats:styled-content style=\"fixed-case\">CCM<\/jats:styled-content> patients correlate with neurodevelopmental outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>Hospital records and neuroimaging of 67 children with <jats:styled-content style=\"fixed-case\">CCM<\/jats:styled-content>s were systematically reviewed. Logistic regression analyses were used to assess associations between specific imaging features and neurodevelopmental outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">CCM<\/jats:styled-content> categories were distributed as follows: 28 percent isolated vermis hypoplasia (<jats:italic>n<\/jats:italic>=19), 28 percent global cerebellar hypoplasia (<jats:italic>n<\/jats:italic>=19), 15 percent Dandy\u2013Walker malformation (<jats:italic>n<\/jats:italic>=10), 13 percent pontocerebellar hypoplasia (<jats:styled-content style=\"fixed-case\">PCH<\/jats:styled-content>,<jats:italic> n<\/jats:italic>=9), 9 percent molar tooth malformation (<jats:italic>n<\/jats:italic>=6), 3 percent rhombencephalosynapsis (<jats:italic>n<\/jats:italic>=2), and 3 percent unilateral cerebellar malformation (<jats:italic>n<\/jats:italic>=2). Overall, 85 percent (55\/65) of the cohort had global developmental delay (<jats:styled-content style=\"fixed-case\">GDD<\/jats:styled-content>). Intellectual disability was present in 61 percent (27\/43) and autism spectrum disorder (<jats:styled-content style=\"fixed-case\">ASD<\/jats:styled-content>) in 12 percent (6\/52). Adjusting for supratentorial malformations and presence of genetic findings, severe <jats:styled-content style=\"fixed-case\">GDD<\/jats:styled-content> was associated with cerebellar hypoplasia (<jats:italic>p<\/jats:italic>=0.049) and <jats:styled-content style=\"fixed-case\">PCH<\/jats:styled-content> (<jats:italic>p<\/jats:italic>=0.030), whereas children with vermis hypoplasia were less likely to have severe <jats:styled-content style=\"fixed-case\">GDD<\/jats:styled-content> (<jats:italic>p<\/jats:italic>=0.003). Presence of supratentorial abnormalities was not significantly associated with worse neurodevelopmental outcome but was associated with epilepsy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Children with <jats:styled-content style=\"fixed-case\">CCM<\/jats:styled-content>s have high prevalence of neurodevelopmental deficits. Specific features on imaging can aid prognostication and establish early intervention strategies.<\/jats:p><\/jats:sec><jats:sec><jats:title>What this paper adds<\/jats:title><jats:p>\n<jats:list list-type=\"bullet\">\n\n<jats:list-item><jats:p>Atypical long\u2010term neurodevelopmental outcome is very common in patients with congenital cerebellar malformations (CCMs).<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>Involvement of the brainstem and cerebellar hemispheres predicts more severe neurodevelopmental disability.<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>Most patients with vermis hypoplasia have language delay but are verbal.<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>Supratentorial abnormalities are not significantly associated with worse neurodevelopmental outcome but are associated with epilepsy.<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>Comorbidities are common in CCMs, especially ophthalmological issues in cerebellar hypoplasia and sensorineural hearing loss in pontocerebellar hypoplasia.<\/jats:p><\/jats:list-item>\n<\/jats:list>\n<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.14059","type":"journal-article","created":{"date-parts":[[2018,10,15]],"date-time":"2018-10-15T10:14:18Z","timestamp":1539598458000},"page":"350-358","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["Developmental outcomes in children with congenital cerebellar malformations"],"prefix":"10.1111","volume":"61","author":[{"given":"Elana F","family":"Pinchefsky","sequence":"first","affiliation":[{"name":"Division of Pediatric Neurology Department of Pediatrics Neurology\/Neurosurgery Montreal Children's Hospital McGill University Health Center Montreal 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