Intellectual, Behavioral, and Emotional Functioning in Children With Syndromic Craniosynostosis

Abstract

WHAT'S KNOWN ON THIS SUBJECT: Children who have syndromic craniosynostosis are at risk for developing intellectual disability, behavioral and emotional problems. Study results were often based on small samples and wide age-based variation, using nonvalidated instruments and describing no clear inclusion and exclusion criteria.WHAT THIS STUDY ADDS: Intellectual, behavioral, and emotional functioning is described in a national sample (N = 82) of schoolaged children with syndromic craniosynostosis. Using standardized instruments, this study indicates higher risks for intellectual disability and behavioral problems mainly in children having Apert and Muenke syndromes. abstract OBJECTIVES: 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 craniosynostosishave 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. Pediatrics 2014;133:e1608-e1615 AUTHORS:

Diffusion Tensor Imaging and Fiber Tractography in Children with Craniosynostosis Syndromes

“…As in patients with sagittal craniosynostosis in whom altered DTI parameters may underlie their neuropsychological deficits, 37 WM abnormalities of the cingulate gyrus and corpus callosum in syndromic patients with craniosynostosis syndromes may be responsible for existing attention and memory problems. 14 However, neurologic assessment of our patients with craniosynostosis syndromes cannot explain diffusivity abnormalities in the corticospinal tracts, and motor deficits might rather be caused by impair-ment of the frontal WM. 38 Remarkably, the fornix seems to be spared by the mechanical pressure or stretching caused by ventriculomegaly and altered brain shape.…”

“…4,[9][10][11][12][13] Patients with craniosynostosis syndromes have a 2-fold higher risk for de-veloping intellectual disability than the normative population, while they also have more behavioral and emotional functioning problems. 14 In this study, we investigated whether diffusion tensor imaging and fiber tractography (FT) can be used for studying WM organization in patients with craniosynostosis syndromes. This technique should give more objective and anatomically complete information about WM tracts than the subjective single ROI approach that has been used before, 7 because parameters will be defined over the total length of a particular WM tract rather than at 1 certain location in the tract.…”

An Exploration of the Cognitive, Physical and Psychosocial Development of Children with Apert Syndrome

“…It was found that there was no significant difference between GCA and NVC scores and all the children achieved a NVC score within the normal range. It is important to note the fact that this study used the british Ability Scales which are less demanding in terms of attention and fine motor skills than other assessments such as the Wechsler Intelligence Scale (maliepaard, mathijssen, Oosterlaan, & Okkerse, 2014). This might help explain the findings of Lefebvre et al (1986) who reported that children with Apert syndrome had lower verbal intelligence scores than their overall performance scores (using the Wechsler Intelligence Scale for Children (WISC)).…”

“…Lajeunie et al, 1999;maliepaard et al, 2014;Patton et al, 1988). In a more recent study of 18 people with Apert syndrome (yacubian-Fernandes et al, 2004) aged between one year and 26 years, with an average of eight years, assessments on developmental or cognitive functions were correlated with the age of the patient, age at which cranial surgery was performed, brain development and quality of family environment.…”

Apert syndrome: the Paris and Rotterdam philosophy

“…Agenesis of the corpus callosum (12-50%), anomalies of the septum pellucidum (24% PMID) and hydrocephalus (33.3%) may be present [37,40].…”