Behavioral Adjustment of Toddler and Preschool-Aged Children with Single-Suture Craniosynostosis

Kapp-Simon, Kathleen A.; Collett, Brent R.; Barr-Schinzel, Michael A.; Cradock, Mary Michaeleen; Buono, Lauren; Pietila, Kristen E.; Speltz, Matthew L.

doi:10.1097/prs.0b013e31825dc18b

Cited by 38 publications

(21 citation statements)

References 26 publications

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“…Group differences on these tasks were negligible, though fewer children with SSC than controls were able to complete the most challenging tasks, possibly indicating problems with EF under more demanding conditions. We also found slightly greater attention problems in children with SSC versus controls at age 36 months based on parent, but not teacher reports (Kapp-Simon et al, 2012). …”

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confidence: 66%

Attention and executive function in children with and without single-suture craniosynostosis

Collett

Kapp-Simon

Wallace

et al. 2015

Child Neuropsychology

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Children with single suture craniosynostosis have higher rates of learning disabilities and related neurocognitive problems than unaffected peers. Executive function and attention are thought to be areas of particular vulnerability, though studies to date have been limited by small sample sizes and a lack of control groups. We evaluated 179 school-aged children with single suture craniosynostosis (cases) and 183 unaffected controls at an average age of 7 years using clinician-administered and parent and teacher report measures of executive function and attention. Among children with craniosynostosis, we examined differences as a function of suture location (sagittal, metopic, unicoronal, or lambdoid) and age of corrective surgery. We used linear regression analyses, adjusted for potential confounders, to compare the two groups on all outcome measures. Cases scored lower than controls on most measures, though the magnitude of these differences was small and most were statistically insignificant. The largest relative deficit was on a measure of inhibitory control. Results changed little in sensitivity analyses adjusting for potential attrition bias and for the effects of developmental and academic interventions. Among cases, there were few differences in relation to the location of suture fusion or timing of surgery. Overall, we found limited evidence of broad deficits in executive function or attention in children with single suture craniosynostosis relative to unaffected controls. Neurocognitive development for children with SSC appears to be variable, across affected children and outcomes assessed. Further research is needed to understand the potential sources of this variability.

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confidence: 66%

Attention and executive function in children with and without single-suture craniosynostosis

Collett

Kapp-Simon

Wallace

et al. 2015

Child Neuropsychology

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“…Muenke syndrome is a molecular diagnosis based on a P250R mutation in FGFR3 and does not require craniosynostosis as a diagnostic criterion. Although previous research has examined similar constructs in craniosynostoses 11,14 , and case studies have collected data on behavior and development of individual patients with FGFR3 -related craniosynostosis 10,12–13,15–16,20 , these studies have had small numbers of participants. Evaluating 13 children with Muenke syndrome, Maliepaard et al found that parents of individuals with Muenke syndrome reported higher levels of behavioral and emotional problems compared with parents of children of other craniosynostosis syndromes.…”

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confidence: 99%

Executive Function and Adaptive Behavior in Muenke Syndrome

Yarnell¹,

Addissie²,

Hadley³

et al. 2015

The Journal of Pediatrics

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Objectives To investigate executive function and adaptive behavior in persons with Muenke syndrome using validated instruments with a normative population and unaffected siblings as controls. Study design Participants in a cross sectional study included individuals with Muenke syndrome (P250R mutation in FGFR3) and their mutation negative siblings. Participants completed validated assessments of executive functioning (Behavior Rating Inventory of Executive Function; BRIEF) and adaptive behavior skills (Adaptive Behavior Assessment System; ABAS-II). Results Forty-four FGFR3 mutation positive individuals, median age 9 years, range 7 months to 52 years were enrolled. Additionally, 10 unaffected siblings were used as controls (5 males, 5 females, median age of 13 years, range 3 to 18 years). For the General Executive Composite scale of the BRIEF, 32.1% of the cohort had scores greater than +1.5 SD, signifying ―Potential Clinical Significance. For the General Adaptive Composite of the ABAS-II, 28.2% of affected individuals scored in the 3rd – 8th percentile of the normative population and 56.4% were below the ―Average category (less than the 25th percentile). Multiple regression analysis did not show that craniosynostosis was a predictor of BRIEF (P = 0.7) and ABAS-II scores (P = 0.7). In the sibling pair analysis, affected siblings performed significantly poorer in the BRIEF General Executive Composite and the ABAS-II General Adaptive Composite. Conclusion Individuals with Muenke syndrome are at an increased risk for developing adaptive and executive function behavioral changes when compared with a normative population and unaffected siblings.

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“…However, cranial vault volume (CVV) in patients with sagittal synostosis has been reported to be the same or higher than normal controls (Lee SS, et al, 2010, Heller JB, et al, 2008, Anderson PJ, et al, 2007, Netherway DJ, et al, 2005, Posnick JC, et al, 1995). Currently, it is unclear whether cognitive and functional deficits are due to primary brain abnormalities or altered cranial vault morphology (Kapp-Simon KA, et al, 2007; Kapp-Simon KA, et al 2012; Sgouros S, 2005, Sgouros S, et al, 1999). …”

Section: Introductionmentioning

confidence: 99%

Effects of Open and Endoscopic Surgery on Skull Growth and Calvarial Vault Volumes in Sagittal Synostosis

Ghenbot¹,

Patel²,

Skolnick³

et al. 2015

Journal of Craniofacial Surgery

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Background There have been conflicting reports on how sagittal synostosis affects cranial vault volume (CVV) and which surgical approach best normalizes skull volume. In this study, we compare CVV and cranial index (CI) of children with sagittal synostosis (before and after surgery) to those of controls. We also compare the effect of repair type on surgical outcome. Methods CT scans of 32 children with sagittal synostosis and 61 age- and gender-matched controls were evaluated using previously validated segmentation software for CVV and CI. 16 cases underwent open surgery and 16 underwent endoscopic surgery. 27 cases had both preoperative and postoperative scans. Results Age of subjects at CT scan ranged from 1-9 months preoperatively and 15-25 months postoperatively. Mean age difference between cases and matched controls was 5 days. The mean CVV of cases preoperatively was non-significantly (17cc) smaller than controls (p = 0.51). The mean CVV of postoperative children was non-significantly (24cc) larger than controls (p = 0.51). Adjusting for age and gender, there was no significant difference in CVV between open and endoscopic cases postoperatively (β = 48cc, p = 0.31). The mean CI increased 12% in both groups. There was no significant difference in mean postoperative CI (p = 0.18) between the two groups. Conclusions Preoperatively, children with sagittal synostosis have no significant difference in CVV compared to controls. Type of surgery does not seem to affect CI and CVV one year postoperatively. Both open and endoscopic procedures result in CVVs similar to controls.

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Behavioral Adjustment of Toddler and Preschool-Aged Children with Single-Suture Craniosynostosis

Cited by 38 publications

References 26 publications

Attention and executive function in children with and without single-suture craniosynostosis

Attention and executive function in children with and without single-suture craniosynostosis

Executive Function and Adaptive Behavior in Muenke Syndrome

Effects of Open and Endoscopic Surgery on Skull Growth and Calvarial Vault Volumes in Sagittal Synostosis

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