title: Intellectual, behavioral, and emotional functioning in children with syndromic craniosynostosis.
abstract: OBJECTIVE
  To examine intellectual, behavioral, and emotional functioning of children who have syndromic craniosynostosis and to explore differences between diagnostic subgroups.
  
  
  METHODS
  A national sample of children who have syndromic craniosynostosis participated in this study. Intellectual, behavioral, and emotional outcomes were assessed by using standardized measures: Wechsler Intelligence Scale for Children, Third Edition, Child Behavior Checklist (CBCL)/6-18, Disruptive Behavior Disorder rating scale (DBD), and the National Institute of Mental Health Diagnostic Interview Schedule for Children.
  
  
  RESULTS
  We included 82 children (39 boys) aged 6 to 13 years who have syndromic craniosynostosis. Mean Full-Scale IQ (FSIQ) was in the normal range (M = 96.6; SD = 21.6). However, children who have syndromic craniosynostosis had a 1.9 times higher risk for developing intellectual disability (FSIQ < 85) compared with the normative population (P < .001) and had more behavioral and emotional problems compared with the normative population, including higher scores on the CBCL/6-18, DBD Total Problems (P < .001), Internalizing (P < .01), social problems (P < .001), attention problems (P < .001), and the DBD Inattention (P < .001). Children who have Apert syndrome had lower FSIQs (M = 76.7; SD = 13.3) and children who have Muenke syndrome had more social problems (P < .01), attention problems (P < .05), and inattention problems (P < .01) than normative population and with other diagnostic subgroups.
  
  
  CONCLUSIONS
  Although children who have syndromic craniosynostosis have FSIQs similar to the normative population, they are at increased risk for developing intellectual disability, internalizing, social, and attention problems. Higher levels of behavioral and emotional problems were related to lower levels of intellectual functioning.
authors.forename: Marianne
authors.initials: M
authors.lastname: Maliepaard
authors.forename: Irene M J
authors.initials: IM
authors.lastname: Mathijssen
authors.forename: Jaap
authors.initials: J
authors.lastname: Oosterlaan
authors.forename: Jolanda M E
authors.initials: JM
authors.lastname: Okkerse
mesh_terms: D000168:Acrocephalosyndactylia / Q000175:diagnosis / Q000453:epidemiology / Q000523:psychology; D000293:Adolescent; D000342:Affective Symptoms / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D019958:Attention Deficit and Disruptive Behavior Disorders / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D002648:Child; D002653:Child Behavior Disorders / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D003072:Cognition Disorders / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D003398:Craniosynostoses / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D007389:Internal-External Control; D007406:Interview, Psychological; D008297:Male; D010552:Personality Assessment; D012307:Risk Factors; D012920:Social Behavior Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000523:psychology; D013577:Syndrome; D014888:Wechsler Scales