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This is one of the first studies of reading in children with orofacial clefts to include a control sample. The findings suggest that children with clefts are less adept readers than demographically matched peers without clefts, supporting the need to monitor academic achievement in this population.

Background-The hypothesized association between single-suture craniosynostosis and neurodevelopment remains unclear, given the methodologic limitations of previous studies, most notably the absence of control groups.

To evaluate the hypothesis that 3-year-old children with single-suture craniosynostosis would receive lower neurodevelopmental scores than a comparable group of children born with patent sutures.

OBJECTIVE We assessed the neurodevelopment of infants with and without deformational plagiocephaly (DP), at an average age of 6 months. METHODS The Bayley Scales of Infant Development III (BSID-III) were administered to 235 case subjects and 237 demographically similar, control participants. Three-dimensional head photographs were randomized and rated for severity of deformation by 2 craniofacial dysmorphologists who were blinded to case status. RESULTS We excluded 2 case subjects with no photographic evidence of DP and 70 control subjects who were judged to have some degree of DP. With control for age, gender, and socioeconomic status, case subjects performed worse than control subjects on all BSID-III scales and subscales. Case subjects’ average scores on the motor composite scale were ~10 points lower than control subjects’ average scores (P < .001). Differences for the cognitive and language composite scales were ~5 points, on average (P < .001 for both scales). In subscale analyses, case subjects’ gross-motor deficits were greater than their fine-motor deficits. Among case subjects, there was no association between BSID-III performance and the presence of torticollis or infant age at diagnosis. CONCLUSIONS DP seems to be associated with early neurodevelopmental disadvantage, which is most evident in motor functions. After follow-up evaluations of this cohort at 18 and 36 months, we will assess the stability of this finding. These data do not necessarily imply that DP causes neurodevelopmental delay; they indicate only that DP is a marker of elevated risk for delays. Pediatricians should monitor closely the development of infants with this condition.

Object-Although most infants with single-suture craniosynostosis (SSC) appear to have neurodevelopmental test scores in the average range, SSC has been associated with cognitive and motor delays during infancy. Whether and when surgery improves such deficits are not yet known. The authors aimed to compare the pre-and postsurgical neurodevelopmental status of patients with SSC with those of control infants without craniosynostosis.Methods-The authors conducted a large, multicenter, longitudinal study of 168 infants with craniosynostosis and 115 controls without synostosis who were of similar age, race, sex, and socioeconomic status. The authors assessed participants by using the Bayley Scales of Infant Development, Second Edition (BSID-II) and the Preschool Language Scale, Third Edition (PLS-3) at enrollment, before patients' intracranial surgery, and when participants were 18 months of age (after surgery for patients).Results-After adjusting for potential confounding factors in linear regression analyses, the authors found a tendency for patients to perform similarly to or slightly worse than controls on neurodevelopmental examinations at both visits. After surgery, the patients' mean scores were 0.6 to three points lower than those of controls on the five BSID-II and PLS-3 scales (p = 0.02-0.07). Compared with controls, patients had 2.3 and 1.9 times the adjusted odds of scoring in the delayed range on either BSID-II scale (Mental Development Index and Psychomotor Development Index) for the first and second visits, respectively (p = 0.001 and p = 0.015, respectively). The patients' mean adjusted test scores were nearly unrelated to the timing of their surgery.Address reprint requests to: Jacqueline R. Starr, Ph.D., Children's Hospital and Regional Medical Center, 4800 Sand Point Way NE, Mailstop W-7847, Seattle, Washington 98105. jrstarr@u.washington.edu. NIH Public AccessAuthor Manuscript J Neurosurg. Author manuscript; available in PMC 2012 August 13. NIH-PA Author ManuscriptNIH-PA Author Manuscript NIH-PA Author ManuscriptConclusions-These findings support recommendations for neurodevelopmental screening in infants with SSC. Longer follow-up, as is being conducted with the patients in the present study, will be critical for identifying the potential longer-term correlates of SSC and its surgical correction. Keywordscranioplasty; neurodevelopment; pediatric neurosurgery; single-suture craniosynostosisSingle-suture craniosynostosis is the premature fusion of one suture in an infant's skull and occurs in approximately one in 2000 live births. Infants in whom a diagnosis of SSC is made typically undergo surgery as soon as it is deemed safe, both for cosmesis and to reduce the risk of sequelae such as raised ICP. A further justification for surgery is to reduce the possibility of neurodevelopmental deficits that may be caused either directly by the brain malformations that appear to accompany the skull malformation, 1,2 or indirectly through ICP. Single-suture craniosynostosis has been associated with co...

We compared the developmental status of school-age children with single-suture craniosynostosis (case group) and unaffected children (control group). Within the case group we compared the performance of children distinguished by location of suture fusion (sagittal, metopic, unicoronal, lambdoid). METHODS:We administered standardized tests of intelligence, reading, spelling, and math to 182 case participants and 183 control participants. This sample represented 70% of those tested during infancy before case participants had corrective surgery. RESULTS:After adjustment for demographics, case participants' average scores were lower than those of control participants on all measures. The largest observed differences were in Full-Scale IQ and math computation, where case participants' adjusted mean scores were 2.5 to 4 points lower than those of control participants (Ps ranged from .002 to .09). Adjusted mean case-control differences on other measures of achievement were modest, although case deficits became more pronounced after adjustment for participation in developmental interventions. Among case participants, 58% had no discernible learning problem (score ,25th percentile on a standardized achievement test). Children with metopic, unicoronal, and lambdoid synostosis tended to score lower on most measures than did children with sagittal fusions (Ps ranged from ,.001 to .82). CONCLUSIONS:The developmental delays observed among infants with single-suture craniosynostosis are partially evident at school age, as manifested by lower average scores than those of control participants on measures of IQ and math. However, case participants' average scores were only slightly lower than those of control participants on reading and spelling measures, and the frequency of specific learning problems was comparable. Among case participants, those with unicoronal and lambdoid fusions appear to be the most neurodevelopmentally vulnerable.