title: Cognitive and behavioral functioning in 82 patients with trigonocephaly.
abstract: BACKGROUND
  The main objective of the present study was to assess the prevalence rates of attention deficit hyperactivity disorder, oppositional defiant disorder, conduct disorder, and features of autism spectrum disorders in trigonocephalic patients, using validated instruments and by ruling out the confounding influence of IQ. The second aim was to assess the association between extracranial anomalies and cognitive and/or behavioral problems in patients with trigonocephaly.
  
  
  METHODS
  Objectives were studied in 82 trigonocephalic patients aged 4 to 18 years at the Erasmus Medical Center in Rotterdam, The Netherlands. Features of autism spectrum disorders were assessed using the Social Communication Questionnaire. Attention deficit hyperactivity disorder, oppositional defiant disorder, and conduct disorder were assessed with the Diagnostic Interview Schedule for Children-Parent Version. The presence and nature of extracranial anomalies were ascertained by a clinician.
  
  
  RESULTS
  Mental retardation (IQ <70) was present in 9 percent of patients with trigonocephaly. Findings indicated a 70 percent versus 24 percent prevalence of psychopathology (attention deficit hyperactivity disorder, oppositional defiant disorder, conduct disorder, or features of autism spectrum disorder) in patients with IQ levels of, respectively, <85 and ≥85. In the latter group, psychopathology was not significantly more common than expected based on prevalence rates reported in community samples. Extracranial anomalies were significantly correlated with lower IQ levels. However, when adjusted for IQ, the presence of extracranial malformations was not associated with an increased risk of behavioral problems.
  
  
  CONCLUSIONS
  The relatively high prevalence of behavioral problems in patients with trigonocephaly seems to be mainly attributable to the co-occurrence of trigonocephaly and low intelligence.
authors.affiliation: Rotterdam and Amsterdam, The Netherlands From the Departments of Plastic and Reconstructive Surgery and Child and Adolescent Psychiatry, Sophia Children&s Hospital/Erasmus Medical University Center, and the Faculty of Behavioral and Social Sciences, University of Amsterdam.
authors.forename: Joris J B
authors.initials: JJB
authors.lastname: van der Vlugt
authors.forename: Jacques J N M
authors.initials: JJNM
authors.lastname: van der Meulen
authors.forename: Hanneke E
authors.initials: HE
authors.lastname: Creemers
authors.forename: Frank C
authors.initials: FC
authors.lastname: Verhulst
authors.forename: Steven E R
authors.initials: SER
authors.lastname: Hovius
authors.forename: Jolanda M E
authors.initials: JME
authors.lastname: Okkerse
mesh_terms: D000293:Adolescent; D001289:Attention Deficit Disorder with Hyperactivity / Q000175:diagnosis / Q000453:epidemiology*; D019958:Attention Deficit and Disruptive Behavior Disorders / Q000175:diagnosis / Q000453:epidemiology*; D001321:Autistic Disorder / Q000175:diagnosis / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D019955:Conduct Disorder / Q000175:diagnosis / Q000453:epidemiology*; D003398:Craniosynostoses / Q000175:diagnosis* / Q000453:epidemiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008607:Intellectual Disability / Q000175:diagnosis / Q000453:epidemiology; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D011569:Psychiatric Status Rating Scales; D012189:Retrospective Studies; D012720:Severity of Illness Index; D017678:Sex Distribution