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Cognitive and Behavioral Functioning in 82 Patients with Trigonocephaly

van der Vlugt, Joris J. B. M.D.; van der Meulen, Jacques J. N. M. M.D., Ph.D.; Creemers, Hanneke E. Ph.D.; Verhulst, Frank C. M.D., Ph.D.; Hovius, Steven E. R. M.D., Ph.D.; Okkerse, Jolanda M. E. Ph.D.

Plastic and Reconstructive Surgery: October 2012 - Volume 130 - Issue 4 - p 885–893
doi: 10.1097/PRS.0b013e318262f21f
Pediatric/Craniofacial: Original Articles

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 disorders) 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.

Conclusion: 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.


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.

Received for publication September 23, 2011; accepted April 27, 2012.

Supported by the Department of Plastic Surgery and the Department Child and Adolescent Psychiatry, Erasmus Medical Center, Rotterdam, The Netherlands, and Nuts Ohra Foundation grant 080181.

Disclosure:None of the authors has any financial, personal, political, intellectual, or religious conflicts of interest.

Joris J. B. van der Vlugt, M.D., Department of Child and Adolescent Psychiatry, Erasmus Medical Center, Sophia Children&s Hospital, P.O. Box 2060, 3000 CB, Rotterdam, The Netherlands,

©2012American Society of Plastic Surgeons