Onderzoek naar gedrag en hersenfuncties bij kinderen met een craniofaciale aandoening.

“The neurocognitive functioning and behaviour of children aged six to twelve years with a syndromic or complex craniosynostosis“.

Craniosynostosis is a congenital malformation characterized by premature closure of cranial sutures. The premature closure of the cranial sutures hinders the growth of the skull, brains and face. Craniosynostosis is 1 in 2500 newborns and is for approximately 40% of patients a part of a syndrome such as Apert syndrome, Crouzon / Pfeiffer, Saethre-Chotzen and Muenke. The treatment of syndromic or complex craniosynostosis craniofacial comprises a correction within the first year of life. Depending on the syndrome, multiple corrections of the skull, face hands and feet occur. Besides the appearance, the skull abnormality, hand and foot abnormalities, and brain abnormalities may occur. These brain abnormalities can be congenital, such as abnormalities of the corpus callosum or acquired, such as hydrocephalus.



The neurocognitive functioning and behaviour of patients with syndromic or complex craniosynostosis remains still unclear. The number of studies of the neurocognitive functioning and behaviour of children with syndromic or complex craniosynostosis is small. Most of these studies exhibit methodological limitations, such as small research using non-validated and standardized instruments are not. Despite methodological limitations, all studies seem to indicate that children with syndromic or complex craniosynostosis an increased risk of learning disabilities and behavioural problems.



The objective of this research project focuses on the cognitive functioning of children with syndromic or complex craniosynostosis, we will investigate the frequency of behavioural problems in these children. Clinical findings presume that the executive functioning, attention and memory are less developed in comparison with the general population, with standardised questionnaires and instruments we will figure out if this assumption is true.

Furthermore we are interested in the medical and child factors, namely brain anomalies, obstructive sleep apnea, genotype, diagnosis and their prediction on the neurocognitive outcome.



The psychological assessment of children takes place in the outpatient department of Child and Adolescent Psychiatry at the Erasmus MC-Sophia Rotterdam the Netherlands. The study is divided into two sessions conducted. All tests are conducted according to a standard. The tests are possible in the same order will be used
The questionnaires are sent to the parents beforehand. The questionnaires are completed and then returned home for research. The TRF is the teacher handed to the parents. This completes the questionnaire and send it in an enclosed return envelope.



Statistical analysis: Data is entered into a database (windows office excel 2007) and using a statistical program worked (SPSS 15.0 for Windows 2000 and GraphPad Prism 4).

The average IQ of the group will be compared with that of the standard group by an independent t-test.

Internalizing, externalizing and total behavior will be compared with the control group using an independent t-test.

The (neuro) cognitive functioning in children with syndromic or complex craniosynostosis will be compared with the control group using an independent t-test.

The correlation between the dependent variables (intelligence, behavior and neurocognitive function) and independent (hypoplasia of the corpus callosum, septum pellucidum, hippocampus, cerebral cortex, white matter lesions and brain volume, ventriculomegaly, chronic tonsillar herniation, increased intracranial pressure, impressiones digitatae , hydrocephalus, OSAS, genotype, diagnosis, age at first craniofacial correction and type of correction) will be analyzed with a Spearman correlation procedure as a dichotomous variable is concerned with a Pearson correlation for the variables that a linear relationship assumption and a ratio or interval measurement scale.

Clinically, children with syndromic craniosynostosis seem to have an increased risk of learning disabilities and behavioral problems in comparison with the general population.

Children undergo the diagnostic procedure only once.

1. WISC-III-NL;

2. ANT 2.1;

3. FLANKER;

4. n-BACK;

5. TIME-TEST;

6. SSRT;

7. CBCL;

8. DBD;

9. i-DISC-IV;

10. CCC-2.