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Predicting developmental delay in a longitudinal cohort of preschool children with single-suture craniosynostosis: is neurobehavioral assessment important?

ABSTRACT: The aim of this study was to determine whether neurobehavioral assessment before and after cranial vault surgery can improve prediction of developmental delay in children with single-suture craniosynostosis (SSC), after accounting for 'baseline' demographic and clinical variables (SSC diagnosis and surgery age).Children with SSC were referred by the treating surgeon or pediatrician before surgery. Neurobehavioral assessments were performed at ages of approximately 6, 18, and 36 months. Iterative models were developed to predict delay, as determined by one or more tests of cognitive, motor, and language skills at 36 months. We selected from groups of variables entered in order of timing (before or after corrective surgery), and source of information (parent questionnaire or psychometric testing).Good predictive accuracy as determined by area under the receiver operating characteristic curve (AUC), was obtained with the baseline model (AUC=0.66), which incorporated age at surgery, sex, and socio-economic status. However, predictive accuracy was improved by including pre- and post-surgery neurobehavioral assessments. Models incorporating post-surgery neurobehavioral testing (AUC=0.79), pre-surgery testing (AUC=0.74), or both pre- and post-surgery testing (AUC=0.79) performed similarly. However, the specifity of all models was considered to be moderate (?0.62).Prediction of delay was enhanced by assessment of neurobehavioral status. Findings provide tentative support for guidelines of care that call for routine testing of children with SSC.

SUBMITTER: Gray KE

PROVIDER: S-EPMC4397127 | biostudies-other | 2015 May

REPOSITORIES: biostudies-other

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Predicting developmental delay in a longitudinal cohort of preschool children with single-suture craniosynostosis: is neurobehavioral assessment important?

Gray Kristen E KE Kapp-Simon Kathleen A KA Starr Jacqueline R JR Collett Brent R BR Wallace Erin R ER Speltz Matthew L ML

Developmental medicine and child neurology 20141123 5

<h4>Aim</h4>The aim of this study was to determine whether neurobehavioral assessment before and after cranial vault surgery can improve prediction of developmental delay in children with single-suture craniosynostosis (SSC), after accounting for 'baseline' demographic and clinical variables (SSC diagnosis and surgery age).<h4>Method</h4>Children with SSC were referred by the treating surgeon or pediatrician before surgery. Neurobehavioral assessments were performed at ages of approximately 6, 1 ...[more]

PMID: 25418927

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Project description:BACKGROUND: Craniosynostosis, the premature fusion of calvarial sutures, is a common craniofacial abnormality. Causative mutations in more than 10 genes have been identified, involving fibroblast growth factor, transforming growth factor beta, and Eph/ephrin signalling pathways. Mutations affect each human calvarial suture (coronal, sagittal, metopic, and lambdoid) differently, suggesting different gene expression patterns exist in each human suture. To better understand the molecular control of human suture morphogenesis we used microarray analysis to identify genes differentially expressed during suture fusion in children with craniosynostosis. Expression differences were also analysed between each unfused suture type, between sutures from syndromic and non-syndromic craniosynostosis patients, and between unfused sutures from individuals with and without craniosynostosis. RESULTS: We identified genes with increased expression in unfused sutures compared to fusing/fused sutures that may be pivotal to the maintenance of suture patency or in controlling early osteoblast differentiation (i.e. RBP4, GPC3, C1QTNF3, IL11RA, PTN, POSTN). In addition, we have identified genes with increased expression in fusing/fused suture tissue that we suggest could have a role in premature suture fusion (i.e. WIF1, ANXA3, CYFIP2). Proteins of two of these genes, glypican 3 and retinol binding protein 4, were investigated by immunohistochemistry and localised to the suture mesenchyme and osteogenic fronts of developing human calvaria, respectively, suggesting novel roles for these proteins in the maintenance of suture patency or in controlling early osteoblast differentiation. We show that there is limited difference in whole genome expression between sutures isolated from patients with syndromic and non-syndromic craniosynostosis and confirmed this by quantitative RT-PCR. Furthermore, distinct expression profiles for each unfused suture type were noted, with the metopic suture being most disparate. Finally, although calvarial bones are generally thought to grow without a cartilage precursor, we show histologically and by identification of cartilage-specific gene expression that cartilage may be involved in the morphogenesis of lambdoid and posterior sagittal sutures. CONCLUSION: This study has provided further insight into the complex signalling network which controls human calvarial suture morphogenesis and craniosynostosis. Identified genes are candidates for targeted therapeutic development and to screen for craniosynostosis-causing mutations.

Dataset Information

Predicting developmental delay in a longitudinal cohort of preschool children with single-suture craniosynostosis: is neurobehavioral assessment important?

Publications

Predicting developmental delay in a longitudinal cohort of preschool children with single-suture craniosynostosis: is neurobehavioral assessment important?

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