To describe early development in children with nonsyndromic craniosynostosis (CS) compared to norms, longitudinally, and by surgery age.
Design
Prospective longitudinal cohort study.
Participants
Patients with CS who had open approach single-stage surgery with the same craniofacial surgeon and neurosurgeon 2009-2019.
Main Outcome Measure
Bayley Scales of Infant and Toddler Development—Third Edition (Bayley-III) at Time 1 (preoperative), Time 2 (6-months postoperative), Time 3 (18-months postoperative), and Time 4 (age 36 months).
Results
Participants (N = 268) had sagittal (50%), unicoronal (26%), or metopic (24%) CS and were mostly Latino (49%) males (67%) with public insurance (57%). The mean surgical age was 7.9 ± 4.3 months with 91% of surgeries completed by 12 months. Higher scores were associated with female sex, higher socioeconomic status, private insurance, and US-born fathers. All mean Bayley-III scores were in the average range clinically and most scores were similar to test norms. Average rates of participants with a delay (<10th percentile) across time points included: Metopic (27%), sagittal (20%), and unicoronal (15%). Development was generally stable over time. There was no relationship between surgery age and development within the context of only 9% of surgeries completed past 12 months.
Conclusions
Sociodemographic influences on development can help identify possible protective and risk factors. While mean performance was consistently in the average clinical range, delays were seen in nearly a third of metopic CS, a fifth of sagittal CS, and 15% of unicoronal CS. Ongoing screening can facilitate appropriate developmental supports, most often for language and gross motor skills.
ShlobinNABaticulonREOrtegaCA, et al.Global epidemiology of craniosynostosis: A systematic review and meta-analysis. World Neurosurg. 2022;164:413–423.e3. doi:https://doi.org/10.1016/j.wneu.2022.05.093
2.
OlssonKEngmanJNowinskiDRamklintMFrickMA. Cognitive development in single-suture craniosynostosis - a systematic review. Dev Neuropsychol. 2023;48(5):215–247. doi:https://doi.org/10.1080/87565641.2023.2225662
3.
KalmarCLLangSSHeuerGG, et al.Neurocognitive outcomes of children with non-syndromic single-suture craniosynostosis. Childs Nerv Syst. 2022;38(5):893–901.
4.
SpeltzMLKapp-SimonKACunninghamMMarshJDawsonG. Single-suture craniosynostosis: A review of neurobehavioral research and theory. J Pediatr Psychol. 2004;29(8):651–668. doi:https://doi.org/10.1093/jpepsy/jsh068
5.
JunnADinisJLongATimberlakeATPersingJAAlperovichM. Quantifying the impact of genetics on neurocognition in nonsyndromic sagittal craniosynostosis. Plast Reconstr Surg. 2023;152(2):300e–306e. doi:https://doi.org/10.1097/PRS.0000000000010400
6.
MandelaRBellewMChumasPNashH. Impact of surgery timing for craniosynostosis on neurodevelopmental outcomes: A systematic review. J Neurosurg Pediatr. 2019;23(4):442–454. doi:https://doi.org/10.3171/2018.10.PEDS18536
7.
OsbornAJRobertsRMDorstynDSGraveBGDavidDJ. Sagittal synostosis and its association with cognitive, behavioral, and psychological functioning: A meta-analysis. JAMA Netw Open. 2021;4(9):e2121937. doi:https://doi.org/10.1001/jamanetworkopen.2021.21937
8.
FotouhiARChiangSNPetersonAM, et al.Neurodevelopment in unilateral coronal craniosynostosis: A systematic review and meta-analysis. J Neurosurg Pediatr. 2022;31(1):16–23. doi:https://doi.org/10.3171/2022.9.PEDS22283
9.
OsbornAJRobertsRMMathiasJLAndersonPJFlapperWJ. Cognitive, behavioral and psychological functioning in children with metopic synostosis: A meta-analysis examining the impact of surgical status. Child Neuropsychol. 2019;25(2):263–277. doi:https://doi.org/10.1080/09297049.2018.1441821
10.
TønneEDue-TønnessenBJWiigU, et al.Epidemiology of craniosynostosis in Norway. J Neurosurg Pediatr. 2020;26(1):68–75. doi:https://doi.org/10.3171/2020.1.PEDS2051
11.
GreenwoodJFlodmanPOsannKBoyadjievSAKimonisV. Familial incidence and associated symptoms in a population of individuals with nonsyndromic craniosynostosis. Genet Med.2014;16:302–310. doi:https://doi.org/10.1038/gim.2013.134
12.
ProctorMRMearaJG. A review of the management of single-suture craniosynostosis, past, present, and future. J Neurosurg Pediatr. 2019;24(6):622–631. doi:https://doi.org/10.3171/2019.7.PEDS18585
13.
WuRTTimberlakeATAbrahamPF, et al.SMAD6 Genotype predicts neurodevelopment in nonsyndromic craniosynostosis. Plast Reconstr Surg. 2020;145(1):117e–125e. doi:https://doi.org/10.1097/PRS.0000000000006319
14.
TimberlakeATJunnAFloresRStaffenbergDALiftonRPPersingJA. Genetic influence on neurodevelopment in nonsyndromic craniosynostosis. Plast Reconstr Surg. 2022;149(5):1157–1165. doi:https://doi.org/10.1097/PRS.0000000000008976
15.
LapehnSGustafsonJATimmsAECunninghamMLPaquetteAG. Transcriptomic signatures of single-suture craniosynostosis phenotypes. Int J Mol Sci. 2023;24(6):5353. doi:https://doi.org/10.3390/ijms24065353
16.
BilleAFoss-SkiftesvikJJuhlerM. The current understanding of germline predisposition in non-syndromic sagittal craniosynostosis: A systematic review. Childs Nerv Syst. 2023;39(3):689–700. doi:https://doi.org/10.1007/s00381-022-05736-9
AldridgeKCollettBRWallaceER, et al.Structural brain differences in school-age children with and without single-suture craniosynostosis. J Neurosurg Pediatr. 2017;19(4):479–489. doi:https://doi.org/10.3171/2016.9.PEDS16107
19.
GaillardLTjaberingaMCDremmenMHGMathijssenIMJVroomanHA. Brain volume in infants with metopic synostosis: Less white matter volume with an accelerated growth pattern in early life. J Anat. 2024;245(6):894–902. doi:https://doi.org/10.1111/joa.14028
20.
US Census Bureau. https://www.census.gov/library/stories/2022/12/languages-we-speak-in-united-states.html https://data.census.gov/table/ACSDP5Y2022.DP02
21.
BlumJDNgJJCraigJ, et al.Sociodemographic disparities in craniosynostosis: A systematic review. Cleft Palate Craniofac J. 2025;62(1):87–96. doi:https://doi.org/10.1177/10556656231199832
22.
LeijserLMSiddiqiAMillerSP. Imaging evidence of the effect of socio-economic status on brain structure and development. Semin Pediatr Neurol. 2018;27:26–34. doi:https://doi.org/10.1016/j.spen.2018.03.004
23.
TooleyUABassettDSMackeyAP. Environmental influences on the pace of brain development. Nat Rev Neurosci. 2021;22(6):372–384. doi:https://doi.org/10.1038/s41583-021-00457-5
24.
LynnJVBuchmanLKBreulerCJBuchmanSR. Surgical timing and neurocognitive development among patients with craniosynostosis: Analysis of confounders. Plast Reconstr Surg. 2023;151(4):821–829. doi:https://doi.org/10.1097/PRS.0000000000010004
25.
StarrJRKapp-SimonKACloonanYK, et al.Presurgical and postsurgical assessment of the neurodevelopment of infants with single-suture craniosynostosis: Comparison with controls. J Neurosurg. 2007;107(2 Suppl):103–110. doi:https://doi.org/10.3171/PED-07/08/103
26.
StarrJRCollettBRGaitherR, et al.Multicenter study of neurodevelopment in 3-year-old children with and without single-suture craniosynostosis. Arch Pediatr Adolesc Med. 2012;166(6):536–542. doi:https://doi.org/10.1001/archpediatrics.2011.1800
27.
CohenSRChoDCNicholsSLSimmsCCrossKPBursteinFD. American Society of maxillofacial surgeons outcome study: Preoperative and postoperative neurodevelopmental findings in single-suture craniosynostosis. Plast Reconstr Surg. 2004;114(4):841–847. doi:https://doi.org/10.1097/01.prs.0000132854.14237.a8
28.
ByunIHHongJWHusseinMAKimYO. Demographic characteristics of craniosynostosis patients in Asia. J Craniomaxillofac Surg. 2018;46(4):674–678. doi:https://doi.org/10.1016/j.jcms.2018.02.008
29.
BayleyN. Bayley Scales of Infant Development. 2nd ed.Psychological Corporation; 1993.
30.
BayleyN. Bayley Scales of Infant and Toddler Development. 3rd ed.Psychological Corporation; 2006.
31.
JohnsonSMooreTMarlowN. Using the Bayley-III to assess neurodevelopmental delay: Which cut-off should be used?Pediatr Res. 2014;75(5):670–674. doi:https://doi.org/10.1038/pr.2014.10
32.
ÇelikPAyranci SucakliIYakutHI. Which Bayley-III cut-off values should be used in different developmental levels?Turk J Med Sci. 2020;50(4):764–770. doi:https://doi.org/10.3906/sag-1910-69
33.
YiYGSungIYYukJS. Comparison of second and third editions of the Bayley Scales in children with suspected developmental delay. Ann Rehabil Med. 2018;42(2):313–320. doi:https://doi.org/10.5535/arm.2018.42.2.313
34.
ActonBVBiggsWSCreightonDE, et al.Overestimating neurodevelopment using the Bayley-III after early complex cardiac surgery. Pediatrics. 2011;128(4):e794–e800. doi:https://doi.org/10.1542/peds.2011-0331
35.
FontanaSCBelingerSDanielsDTuttleMCamarataPJAndrewsBT. Longitudinal assessment of developmental outcomes in infants undergoing late craniosynostosis repair. J Craniofac Surg. 2018;29(1):25–28. doi:https://doi.org/10.1097/SCS.0000000000004024
36.
HollingsheadAA. Four-Factor Index of Social Status. Yale University; 1975.
37.
LoweJBannCMDempseyAGFullerJ, et al.Do Bayley-III composite scores at 18-22 months corrected age predict full-scale IQ at 6-7 years in children born extremely preterm?J Pediatr. 2023;263:113700. doi:https://doi.org/10.1016/j.jpeds.2023
38.
NishijimaMYoshidaTMatsumuraK, et al.Correlation between the Bayley-III at 3 years and the Wechsler Intelligence Scale for children, fourth edition, at 6 years. Pediatr Int. 2022;64(1):e14872. doi:https://doi.org/10.1111/ped.14872
39.
Abo HamzaETindleRPawlakSBedewyDMoustafaAA. The impact of poverty and socioeconomic status on brain, behaviour, and development: A unified framework. Rev Neurosci. 2024;35(6):597–617. doi:https://doi.org/10.1515/revneuro-2023-0163
CastroMFernándezVMartínezAAlcántaraMCampilloALópez-SolerC. Relationship between neurodevelopmental areas and difficulties in emotional-behavioural variables in children with typical development under 2 years of age: Sex differences. Psychol Belg. 2024;64(1):129–144. doi:https://doi.org/10.5334/pb.1203
42.
Da CostaACAndersonVAHolmesAD, et al.Longitudinal study of the neurodevelopmental characteristics of treated and untreated nonsyndromic craniosynostosis in infancy. Childs Nerv Syst. 2013;29(6):985–995. doi:https://doi.org/10.1007/s00381-012-2017-0
PatelAYangJFHashimPW, et al.The impact of age at surgery on long-term neuropsychological outcomes in sagittal craniosynostosis. Plast Reconstr Surg. 2014;134(4):608e–617e. doi:https://doi.org/10.1097/PRS.0000000000000511
45.
MaggeSNFotouhiARAllhusenV, et al.Cognitive outcomes of children with sagittal craniosynostosis treated with either endoscopic or open calvarial vault surgery. JAMA Netw Open. 2024;7(4):e248762. doi:https://doi.org/10.1001/jamanetworkopen.2024.8762
Supplementary Material
Please find the following supplemental material available below.
For Open Access articles published under a Creative Commons License, all supplemental material carries the same license as the article it is associated with.
For non-Open Access articles published, all supplemental material carries a non-exclusive license, and permission requests for re-use of supplemental material or any part of supplemental material shall be sent directly to the copyright owner as specified in the copyright notice associated with the article.
