Developmental delays evident in school-age children with craniosynostosis

By Will Boggs MD

NEW YORK (Reuters Health) - Developmental delays are common among school-age children with single-suture craniosynostosis, according to results from a case-control study.

"There is great individual variation among the neurodevelopment of children with single-suture craniosynostosis," Dr. Matthew L. Speltz, from the University of Washington, Seattle, told Reuters Health by email. "Although on average they differ only slightly from matched control group children, cases are less likely to show above average intellectual development, and a higher proportion of cases than controls show low average intellectual development-so without help, a significant minority of children with single-suture craniosynostosis will struggle in school."

An earlier study from this group found infants and preschoolers with single-suture craniosynostosis to have standardized test scores 0.25 to 0.50 standard deviation lower than matched controls.

In this study, Dr. Speltz and colleagues followed to early elementary school 182 children with single-suture craniosynostosis and 183 matched controls.

In tests of intellectual and academic functioning, children with single-suture craniosynostosis had adjusted mean scores consistently lower than those of control participants, according to the February 23 Pediatrics online report.

The largest deficits were in the Full-Scale IQ, math computation, and phonological awareness, where cases scored 2.5 to 4 points lower than controls. Differences on other measures of achievement were modest.

More cases (73/182, 40%) than controls (54/183, 30%) were classified as having learning problems, though this difference fell short of statistical significance after adjustment for baseline demographics.

Children with metopic, unicoronal, or lambdoid synostosis scored lower on most measures of achievement and IQ and were more likely to have a learning problem, compared with children with sagittal synostosis.

"The probability of learning problems at school age seems predictable on the basis of sex (males at higher risk), location of suture fusion (metopic, unicoronal, and lambdoid fusions are associated with higher risk of learning problems than sagittal, especially the latter two), and family social economic status," Dr. Speltz said. "So, for example, girls with sagittal synostosis from low social risk families will probably do well, but males with other suture fusions from higher social risk families are more likely to have difficulties, and they should be the targets of early screening and intervention. This could be relatively easily done through craniofacial centers and other specialty programs."

For now, Dr. Speltz recommended "early (i.e., late infancy/toddler) developmental screening, identification, and referral to early intervention/educational services. We know that birth to three and preschool developmental programs are effective in other populations, and it is therefore quite likely that these programs would also be effective among young children with single-suture craniosynostosis (although this is yet to be proven)."

"The next most important step in research on the neurodevelopment of children with single-suture craniosynostosis is to better understand the effects of cranioplastic surgery," Dr. Speltz concluded. "Does it help cognition, hurt cognition (e.g., through exposure to lengthy surgeries, anesthesia, blood loss), or have no discernible effect either way?"

"We are planning a study to compare open cranial vault surgeries to briefer, minimally invasive, endoscopic procedures to see if either procedure is associated with better or worse neurodevelopmental outcomes," he said. "It is pretty clear that these surgeries can dramatically improve morphological outcomes, but there are little or no data on neurodevelopmental outcomes."

Dr. Sarah Knight from Murdoch Childrens Research Institute, Parkville, Victoria, Australia, recently reviewed neurodevelopmental outcomes of children with single-suture craniosynostosis. She told Reuters Health by email, "The results are generally consistent with those of previous research but add further weight to the notion that children treated for craniosynostosis are at risk of experiencing ongoing mild, cognitive difficulties when compared to their same-aged peers."

"To give children with single-suture craniosynostosis the best opportunity to reach their full potential, doctors and families should be aware of the long-term cognitive risks associated with single suture craniosynostosis," Dr. Knight said. "This would allow for early identification of children who may experience difficulties, together with close monitoring and individualized intervention as required."

"It will be important for future research to investigate other aspects of cognitive and behavioral development (e.g., memory, attention, language, executive function) in order to comprehensively understand the potential difficulties that these children may experience," Dr. Knight said. "Further, we do not know which infants with single suture craniosynostosis are at greatest risk of experiencing difficulties. Ideally, it would assist clinical care if we could identify children at greatest risk early in development so that they can receive the care and intervention they require."

Dr. Stephen Warren, from New York University School of Medicine, who has published widely on craniosynostosis, told Reuters Health by email, "This study was not designed to determine the etiology of the developmental delay. A variety of factors could be responsible for the small learning difference. For example, the etiopathogenic process that causes single suture fusion could contribute to the developmental delay. Alternatively, exposure to surgery and/or anesthesia could also be contributing factors."

"One finding in particular supports both of these hypotheses," Dr. Warren said. "Patients with sagittal craniosynostosis were less affected than patients with either coronal or lambdoidal synostosis; this observation is particularly interesting because sagittal suture fusion could either have a lesser impact on neurodevelopment or since patients with sagittal craniosynostosis are often treated with strip craniectomy (a shorter and less invasive procedure) compared to patients with coronal or lambdoidal, they may be less impacted by exposure to anesthesia, blood loss or length of surgery."

Dr. Timothy A. Turvey, from the University of North Carolina, Chapel Hill, told Reuters Health by email, "I am elated to see a number of our syndromically involved children who had complex forms of craniosynostosis (Apert and Crouzon Syndrome) complete comprehensive care through the UNC Craniofacial Center and are now college graduates. Early detection and progressive treatment over the first decade of life is critical, as is nurturing educational and social programs."

The National Institutes of Health funded this research. The authors report no disclosures.

SOURCE: http://bit.ly/1wldK6x

Pediatrics 2015.

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