After Olivia Bush was diagnosed with single-suture craniosynostosis, her parents did not know how her brain would develop over time. A new study led by Seattle Children’s is addressing these concerns.
Seattle Children’s researchers have published the results of a 10-year, multi-site study tracking the cognitive development of children with single-suture craniosynostosis from infancy to school age. The results could help families and clinicians better predict which children with this condition are at greater risk of having learning deficits so that they might intervene early in the child’s life.
The study, published today by the American Academy of Pediatrics and titled “Intellectual and Academic Functioning of School-Age Children with Single-Suture Craniosynostosis,” reported children with single-suture craniosynostosis, on average, were more likely than children without the disorder to have learning deficits once they started school. However, despite this trend, a little over half of the children with single-suture craniosynostosis showed no discernible learning problems.
Living with the unknown
The research is significant for parents like Cindy and Todd Bush. Twelve years ago, Cindy and Todd learned their 3-month-old daughter, Olivia, had craniosynostosis, a condition in which one or more of the special joints in a baby’s skull (sutures) grow together (fuse) earlier than normal. When these joints come together too early, a baby’s skull cannot grow properly. Craniosynostosis occurs in approximately one in 1700-2500 live births. Corrective surgery to restore the suture is preferentially performed in the first year of life.
“Our whole world changed at that point,” Cindy said. “We had expected our daughter to live a normal, healthy life, and all of the sudden we were faced with a diagnosis we knew nothing about.”
The family was referred to Seattle Children’s Craniofacial Center, an international leader in treating craniosynostosis that has the largest multidisciplinary team in the country, with more than 50 providers from 19 medical specialties. While the Bush family was comforted by the Center’s highly specialized team, they were unsure of how Olivia’s condition might affect her brain development.
“Our minds started wandering down some frightening roads,” Todd said. “So much was unknown at that time that we were left wondering what her future would hold.”
Investigating the issue
The Bush family was not alone. Neurodevelopmental impairment has long been suspected among children with single-suture craniosynostosis and is a focus of treatment and research at Seattle Children’s Craniofacial Center. The prematurely fused suture is believed to constrain or alter brain structures and increase intracranial pressure.
Dr. Matthew Speltz, a clinical psychologist in Seattle Children’s Craniofacial Center and a principal investigator at Seattle Children’s Research Institute’s Center for Child Health, Behavior and Development, had studied infants and preschoolers with single-suture craniosynostosis and discovered they consistently scored lower, on average, than same-aged children without this condition. He wanted to determine if these delays persisted into the school-age years and manifested as learning disabilities.
“There have been clinical impressions and hypotheses about the cognitive status of children who’ve had single-suture craniosynostosis, but kids with this disorder had never been studied longitudinally in order to see how they develop over time and how they compare to unaffected children over the same time period,” Speltz said. “Understanding the cognitive development of these children is important so we know which children are at greater risk of developing learning disabilities and how we can intervene early in their lives to make a difference.”
In 2002, Speltz initiated the first large-cohort study to track the development of children with single-suture craniosynostosis and unaffected controls from infancy to school age. The Bush family was eager to sign up for the study.
“We wanted to learn as much as we could about Olivia’s condition and how it might affect her growing up,” Cindy said. “And we wanted future families of children with craniosynostosis to have more information available to them than we had when Olivia was diagnosed.”
Finding answers
Over 10 years, Speltz compared the developmental status of 182 school age children with single-suture craniosynostosis and 182 unaffected children using standardized tests of intelligence, reading, spelling and math.
The children with craniosynostosis averaged lower scores than the control group on all measures. The largest observed differences were in full-scale IQ and math computation. The smallest differences were observed in reading and spelling. Still, most children with craniosynostosis performed well academically and 58% had no discernible learning problem.
“Although developmental delays observed among infants with single-suture craniosynostosis were partially evident in elementary school, our results provide more good news than bad for parents,” Speltz said. “On average, the children with single-suture craniosynostosis experienced mild cognitive and academic deficits, and most of them were doing pretty well in early elementary school.”
Olivia is currently 12 years old and one of the best performing students in her sixth grade class.
Researchers also discovered developmental delays varied significantly based on the subtype of craniosynostosis. Children with metopic, unicoronal or lambdoid synostosis were much more likely to have a learning problem than children with the most common form of single-suture craniosynostosis – sagittal synostosis. In related research, Speltz and colleagues also found that boys with single-suture craniosynostosis tended to score lower on academic and IQ tests than girls with this condition.
“This information helps us determine which children are at greatest risk of falling behind,” Speltz said. “This will enable us to better target children with single-suture craniosynostosis for early screening and intervention programs that prevent or minimize cognitive and academic difficulties later in life.”
Over the years, Olivia’s cognitive function almost always measured above average. Today, she is one of the best performing students in her sixth grade class.
“It’s amazing to see her now,” Todd said. “She loves school and we are so proud of her. We didn’t know what to expect 12 years ago, but today she is a happy, healthy child. We hope other families will find this research comforting.”
Other Seattle Children’s collaborators on this study include Dr. Michael Cunningham, medical director of Seattle Children’s Craniofacial Center and investigator in Seattle Children’s Research Institute’s Center for Developmental Biology and Regenerative Medicine, and Dr. Brent Collett, clinical psychologist in Seattle Children’s Craniofacial Center and investigator in Seattle Children’s Research Institute’s Center for Child Health, Behavior and Development.
Parents of children with craniosynostosis may contact the Seattle Children’s Craniofacial Center by calling 206-987-2208.
I’m unclear if the group with craniosynostosis, was operated on or not. If someone can please clarify that I would appreciate it. Thanks.
I had the same question. Were the cognitive delays seen in kids who had their cranio surgically corrected or no. Was Olivia operated on?
I have the same question as the others. I need to know if the children they studied had operations on their craniosynostosis or not.
Thank you for your comment. Please contact the Craniofacial Center directly with your question at 206-987-2528. You can also visit their website at http://www.seattlechildrens.org/clinics-programs/craniofacial/ for more information.
Hi, I’ve been researching what help I can get as an adult with a craniofacial issue. It’s gotten worse over time and no medical help was offered as a child or teen.
Its effected my growth and appearance hugely.
If there are any suggestions I’d be willing to hear them.
I would like to know when they were operated on as well. This could make a huge difference when it comes to results
Hi Melissa – thank you for your question. According to Dr. Speltz, almost all of the patients in this study had surgery to correct synostosis (179/182). The mean age at the time of surgery was 9.1 months (median=8.6; range 2-34 months).
Hello. I have 12 year old quintuplets. My son wS diagnosed with cranialsynostosis and had endoscopic surgery to correct. Following that, he wore 2 DOC bands. I am curious what Dr. Speltz would recommend when it comes to these kids playing full contact sports like lacrosse and football? Also, would they need a specific helmet in regards to playing football?
Indeed people have asked a very relevant question.
This report, I think, came out right after, asking this very question, and saying we can’t know for sure if this was a consequence to surgery procedure or not.
http://pediatrics.aappublications.org/content/pediatrics/early/2015/02/17/peds.2014-1634.full.pdf
Mind you, many have already dev delays before surgery, but studies also show no correlation with degree of cranio and dev delay extent…
So, it seems to be related to the original cause of the fusion, which in many cases is – yes i will say it – malnutrition; unless there’s an obvious genetic reason, or some complication during pregnancy.
The study in the article on this webpage mentions how Saggittal had the least chances of developing learning delays etc. Well, saggittal is also the least invasive as it is right up there where the incision is done, and fixing it is less complicated generally metopic and/or coronal and lamboid.
So, while there are delays for many prior to surgery, one has to keep in mind three things: 1. that malnutrition during preg. will cause both. It can too cause dev delays.
2. surgery may or may not have an impact, but when most study population are operated on infants; it is not conclusive.
3. Chances are those parents with that nutritional practice in pregnancy, never lit up on it and didn’t help compensate that soon enough and delays persisted…
Sorry. I quoted you the same study.
Dr Speltz’s study does specify that it cannot be concluded surgical procedure had a negative impact or not on dev delays.
If the mean age at the time of surgery was 9.1 months perhaps the reason children experienced developmental delays is because the surgery should have been done earlier (prior to 3 months). It would be interesting to Pareto the time of surgery and the extent odf dev delays for the different types of craniosynostosis. Not sure if the sample size is large enough to be able to see a trend.
My now-32 yo son has sagittal craniosynostosis, which has never been diagnosed, but likely should have been. Our biggest problem over his childhood was the lack of any safety helmets to fit; bike, kayak, skateboard, motorcycle, none were long enough front to back. He is, however, extremely smart, graduated with honors, and is far more “with it” than I’ll ever be. He was a big baby, 9#11oz, from a long line of big babies. Great pregnancy nutrition (I’m an IBCLC lactation consultant), exclusively BF, overall with solids for 2.5 yrs., has NEVER had a sick visit. He played select soccer for years, and is beginning to have some joint problems. I now have a patient (for breastfeeding help) who has coronal cranio, which put me on the internet. My question is if there are health risks my son should be aware of, perhaps affecting bones and joints, as an adult? Thank you!
Kay, I am so glad you found our blog. Thank you for reaching out with your question. Since it’s specific to your son’s medical needs, I believe your question would be best directed to a primary care provider or someone else that has been involved in his ongoing medical care.
Is there any study that includes children with untreated craniosynostosis? That would help for parents like we are. We have a 3 year old child with an untreated sagittal synostosis that has been discovered recently. What could be the predicted future for him? Is there any study abailable for such cases?
Hi Norbert – Thank you for your question. According to Dr. Speltz, almost all patients in this particular study had surgery to correct synostosis (179/182) I’ve reached out to him for his input about any ongoing studies for children without surgically-corrected synostosis and will respond here as soon as I have more information to share.
Hi Nobert – Please see below for Dr. Speltz’s response to your question.
We had a small handful of such children in our research sample, but we had too few to analyze separately–for this reason we really cannot say whether having surgery (or not) is related to the probability of a learning disorder.
My son has had his second cranial surgery, first one at 6months and second one at 5yrs. My question is how is the child studied to know the learning difficulty?
Hi Jamie – Thank you for your question. I’ve reached out to Dr. Speltz for his input and will respond here as soon as I have more information to share.
Hi Jamie – Please see below for Dr. Speltz’s response to your question.
In our research on cognitive development in children with single-suture craniosynostosis (SSC), we used standardized, individually administered developmental tests at all age points (infancy through age 7). These tests provide scores that reflect an individual child’s standing in comparison with a same-aged normative group. When the children in our research were infants and preschoolers, we used the Bayley Scales of Infant Development-II and the Preschool Language Scale.
At age 7 we gave several different tests. Intelligence was measured by the Full-Scale IQ score from the Wechsler Intelligence Scale for Children (4th Edition). The Wide Range Achievement Test (4th Edition) measured reading, spelling and math. The Test of Word Reading Efficiency measured the child’s ability to quickly and accurately decode increasingly difficult words. We used the Comprehensive Test of Phonological Processing to assess skills that support reading decoding, speed and fluency.
When any child is referred to a psychologist for clinical evaluation of a possible learning problem, tests like the ones we used in our research are typically given.
It is important to keep in mind that our research has shown that the majority of children with SSC (about 60%) do not have significant learning problems. The other 40% of children in our sample with SSC had mild to moderate learning problems–of the type that are very treatable, using the same educational interventions used with any child who has learning difficulties (i.e., children with SSC do not require specialized interventions because they have SSC). The earlier that children are identified as having learning problems, the higher the probability of a good outcome. If parents are concerned about the development of their preschool or school-aged child with SSC, they should ask their pediatrician for a referral to a psychologist who can evaluate cognitive ability and academic skills.
As the mother of son who had this surgery in 2001 at 4 months old, I can honestly say my son had suffered no learning disabilities or delays. In fact, he’s always excelled in school and sports. He is currently a senior in high school and has a 4.3 GPA. (His applied and planning on going to university in 2019 away from us to.) He’s also a baseball catcher and has played in varsity since his freshman year. Maybe my son is an exception, but however one sees it, I thank God for his continued life success.
I wish this study had been around when my son was born in ’92, he had sagital cranio, nd had the surgery done at 5 months, his head shape was already odd, and we have no regrets, other than the pain it may have caused him, I say may, because he didn’t seem to be uncomfortable, in fact te afterboon of the surgery he was happily bouncing away in the bouny seat in the nicu playroom, he has always been a very smart kis, months ahead of his classmates, I would not change a thing about him