Beyond aesthetic implications, craniosynostosis can profoundly impact neurocognitive development. There is a notable gap in standardized methodologies for identifying which of these patients are at-risk for developing adverse neuropsychosocial outcomes through screening techniques. This research aims to identify and recommend a standardized, routine approach inclusive of free or low-cost screening instruments for patients with craniosynostosis that are easily administered in clinic with or without a trained examiner.
Design
A systematic review was conducted. Data related to various developmental assessments were extracted and subsequently reviewed by two trained neuropsychologists for analysis, categorization, and recommendation.
Main Outcomes Measures
Neurocognitive screening assessments for patients with craniosynostosis.
Results
In total, 114 different tests regarding neurocognitive examination of patients with craniosynostosis were cited. We identified six areas of neuropsychosocial development that are relevant for routine screening: Development, Social-Emotional/Behavioral, Adaptive Functioning, Academic Achievement, Autism, and ADHD/Attention. Within each category, screening recommendations are made, including instruments that are free or low-cost and can be easily administered in clinic with or without a trained examiner.
Conclusions
Literature supports the impact of craniosynostosis on neuropsychosocial development, so we urge teams to screen patients from an early age using these suggestions as they are free or relatively low-cost, can be administered to a wide range of patient ages, and can be administered in clinic with or without a trained examiner. This study serves as a starting point towards a more standardized approach to effectively evaluate and address the neurocognitive implications of craniosynostosis, ultimately enhancing patient care and treatment outcomes.
HerringSW. Mechanical influences on suture development and patency. Craniofacial Sutures. 2008;12(1):41-56.
2.
RenierDLajeunieEArnaudEMarchacD. Management of craniosynostoses. Child's Nerv Syst.2000;16(10-11):645-658.
3.
MaliepaardMMathijssenIMOosterlaanJOkkerseJM. Intellectual, behavioral, and emotional functioning in children with syndromic craniosynostosis. Pediatrics. 2014;133(6):e1608-e1615. doi:https://doi.org/10.1542/peds.2013-3077
4.
SpeltzMLCollettBRWallaceER, Starr JR, Cradock MM, Buono L, Cunningham M, Kapp-Simon K.Intellectual and academic functioning of school-age children with single-suture craniosynostosis. Pediatrics. 2015;135(3):e615-e623.
5.
CollettBRWallaceERKartinDSpeltzML. Infant/toddler motor skills as predictors of cognition and language in children with and without positional skull deformation. Child's Nerv Syst.2019;35(1):157-163.
6.
Kapp-SimonKAWallaceECollettBRCradockMMCrerandCESpeltzML. Language, learning, and memory in children with and without single-suture craniosynostosis. J Neurosurg Pediatr. May 2016;17(5):578-588. doi:https://doi.org/10.3171/2015.9.Peds15238
7.
CloonanYKCollettBSpeltzMLAnderkaMWerlerMM. Psychosocial outcomes in children with and without non-syndromic craniosynostosis: Findings from two studies. Cleft Palate Craniofac J.2013;50(4):406-413.
8.
ChieffoDTamburriniGMassimiLDi GiovanniSGiansantiCCaldarelliMDi RoccoC.Long-term neuropsychological development in single-suture craniosynostosis treated early. J Neurosurg Pediatr. 2010;5(3):232-237. doi:https://doi.org/10.3171/2009.10.Peds09231
9.
AndrewsBTFontanaSC. Correlative vs. Causative relationship between neonatal cranial head shape anomalies and early developmental delays. Front Neurosci. 2017;11(1):708. doi:https://doi.org/10.3389/fnins.2017.00708
10.
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
11.
Thiele-NygaardAEFoss-SkiftesvikJJuhlerM. Intracranial pressure, brain morphology and cognitive outcome in children with sagittal craniosynostosis. Childs Nerv Syst. 2020;36(4):689-695. doi:https://doi.org/10.1007/s00381-020-04502-z
12.
ChiangSNFotouhiARDoeringMMSkolnickGBNaidooSDStrahleJMMcEvoySDPatelKB.Cognitive development in lambdoid craniosynostosis: A systematic review and meta-analysis. Cleft Palate Craniofac J. 2022;61(3):450-457. doi:https://doi.org/10.1177/10556656221129978
13.
FotouhiARChiangSNPetersonAMDoeringMM.SkolnickGB.NaidooSD.StrahleJM.McEvoySD.PatelKB.Neurodevelopment in unilateral coronal craniosynostosis: A systematic review and meta-analysis. J Neurosurg Pediatr. 2023;31(1):16-23. doi:https://doi.org/10.3171/2022.9.Peds22283
Kapp-SimonKASpeltzMLCunninghamMLPatelPKTomitaT. Neurodevelopment of children with single suture craniosynostosis: A review. Childs Nerv Syst. 2007;23(3):269-281. doi:https://doi.org/10.1007/s00381-006-0251-z
16.
HaywardRBrittoJDunawayDJeelaniO. Connecting raised intracranial pressure and cognitive delay in craniosynostosis: Many assumptions, little evidence. J Neurosurg Pediatr. Aug 2016;18(2):242-250. doi:https://doi.org/10.3171/2015.6.Peds15144
17.
FernbachSKFeinsteinKA. Radiologic evaluation of the child with craniosynostosis. Neurosurg Clin N Am. Jul 1991;2(3):569-585.
18.
SpeltzMLKapp-SimonKACunninghamMMarshJDawsonG. Single-suture craniosynostosis: A review of neurobehavioral research and theory. J Pediatr Psychol. Dec 2004;29(8):651-668. doi:https://doi.org/10.1093/jpepsy/jsh068
19.
BotteroLLajeunieEArnaudEMarchacDRenierD. Functional outcome after surgery for trigonocephaly. Plast Reconstr Surg. 1998;102(4):952-958. discussion 959-60.
20.
ShipsterCHearstDSomervilleAStackhouseJHaywardRWadeA. Speech, language, and cognitive development in children with isolated sagittal synostosis. Dev Med Child Neurol. Jan 2003;45(1):34-43.
21.
SpeltzMLKapp-SimonKCollettBKeichYGaitherRCradockMMBuonoLCunninghamML..Neurodevelopment of infants with single-suture craniosynostosis: Presurgery comparisons with case-matched controls. Plast Reconstr Surg. May 2007;119(6):1874-1881. doi:https://doi.org/10.1097/01.prs.0000259184.88265.3f
22.
Da CostaACAndersonVASavarirayanRWrennallJAChongDKHolmesADGreensmithALMearaJG, Neurodevelopmental functioning of infants with untreated single-suture craniosynostosis during early infancy. Childs Nerv Syst. 2012;28(6):869-877. doi:https://doi.org/10.1007/s00381-011-1660-1
23.
SidotiEJMarshJLMarty-GramesLNoetzelMJ. Long-term studies of metopic synostosis: Frequency of cognitive impairment and behavioral disturbances. Plast Reconstr Surg. 1996;97(2):276-281. doi:https://doi.org/10.1097/00006534-199602000-00002
24.
StarrJRCollettBRGaitherRKapp-SimonKACradockMMCunninghamMLSpeltzML.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
25.
SpeltzMLCollettBRWallaceERStarrJRCradockMMBuonoLCunninghamMKapp-SimonK.Intellectual and academic functioning of school-age children with single-suture craniosynostosis. Pediatrics. 2015;135(3):e615-e623. doi:https://doi.org/10.1542/peds.2014-1634
26.
Da CostaACWaltersISavarirayanRAndersonVAWrennallJAMearaJG. Intellectual outcomes in children and adolescents with syndromic and nonsyndromic craniosynostosis. Plast Reconstr Surg. 2006;118(1):175-181. doi:https://doi.org/10.1097/01.prs.0000221009.93022.50
27.
FlapperWJAndersonPJRobertsRMDavidDJ. Intellectual outcomes following protocol management in crouzon, pfeiffer, and muenke syndromes. J Craniofac Surg. Jul 2009;20(4):1252-1255. doi:https://doi.org/10.1097/SCS.0b013e3181acdf9a
28.
PattonMAGoodshipJHaywardRLansdownR. Intellectual development in apert's syndrome: A long term follow up of 29 patients. J Med Genet. Mar 1988;25(3):164-167. doi:https://doi.org/10.1136/jmg.25.3.164
29.
LajeunieECameronREl GhouzziVde ParsevalNJourneauPGonzalesMDelezoideA-LBonaventureJLe MerrerMRenierD.Clinical variability in patients with apert's syndrome. J Neurosurg. Mar 1999;90(3):443-447. doi:https://doi.org/10.3171/jns.1999.90.3.0443
30.
Standards for Approval of Cleft Palate and Craniofacial Teams.
SquiresJBrickerD. 2009. Ages & Stages Questionnaires®, Third Edition (ASQ®-3): A Parent-Completed Child Monitoring System.
33.
GlascoeFP. Screening for developmental and behavioral problems. Ment Retard Dev Disabil Res Rev. 2005;11(3):173-179. doi:https://doi.org/10.1002/mrdd.20068
34.
ThorpeJKamphausRWReynoldsCR. The behavior assessment system for children. In: Handbook of psychological and educational assessment of children: personality, behavior, and context, 2nd ed. New York, NY: The Guilford Press; 2003:387-405.
ThabrewHMcDowellHGivenKMurrellK. Systematic review of screening instruments for psychosocial problems in children and adolescents with long-term physical conditions. Glob Pediatr Health. 2017;4(1):2333794X-17690314. doi:https://doi.org/10.1177/2333794X17690314
38.
CamerotaMBagleyJMcGowanECCarterBS.CheckJDansereauLM.DellaGrottaSA.HeldermanJBHofheimerJALoncarCM, et al.Agreement between two behavioral rating scales in preschoolers born very preterm. J Dev Behav Pediatr. Jan 1 2024;45(1):e72-e78. doi:https://doi.org/10.1097/DBP.0000000000001238
39.
The Revised Child Anxiety and Depression Scale: A psychometric investigation with Australian youth. doi:10.1375/bech.19.2.90. Australian Academic Press; 2002.
40.
ChorpitaBFYimLMoffittCUmemotoLAFrancisSE. Assessment of symptoms of DSM-IV anxiety and depression in children: A revised child anxiety and depression scale. Behav Res Ther. Aug 2000;38(8):835-855. doi:https://doi.org/10.1016/s0005-7967(99)00130-8
SparrowSSCicchettiDV. Major psychological assessment instruments, Vol 2.
43.
CradockMMGrayKEKapp-SimonKACollettBRBuonoLASpeltzML. Sex differences in the neurodevelopment of school-age children with and without single-suture craniosynostosis. Childs Nerv Syst. Jul 2015;31(7):1103-1111. doi:https://doi.org/10.1007/s00381-015-2671-0
44.
WillcuttEGBoadaRRiddleMWChhabildasNDeFriesJCPenningtonBF. Colorado Learning difficulties questionnaire: Validation of a parent-report screening measure. Psychol Assess.2011;23(3):778-791. doi:https://doi.org/10.1037/a0023290
45.
ConradALBellucciCHeppnerCelia E.AlbertMCrerandCEWoodardSSheikhFKapp-SimonKA.Screening for academic risk among students with cleft lip and/or palate: Patterns of risk and qualities of effective tools. Cleft Palate Craniofac J. Jan 2024;61(1):68-78. doi:https://doi.org/10.1177/10556656221116008
46.
ThabtahFPeeblesD. Early autism screening: A comprehensive review. Int J Environ Res Public Health. Sep 19 2019;16(18):3502. doi:https://doi.org/10.3390/ijerph16183502
47.
WieckowskiATWilliamsLNRandoJLyallKRobinsDL. Sensitivity and specificity of the modified checklist for autism in toddlers (original and revised): A systematic review and meta-analysis. JAMA Pediatr.2023;177(4):373-383. doi:https://doi.org/10.1001/jamapediatrics.2022.5975
48.
RobinsDLCasagrandeKBartonMChenC-MADumont-MathieuTFeinD. Validation of the modified checklist for autism in toddlers, revised with follow-up (M-CHAT-R/F). Pediatrics. 2014;133(1):37-45. doi:https://doi.org/10.1542/peds.2013-1813
49.
GoldsteinS. Autism Spectrum rating scale. In: VolkmarFR, ed. Encyclopedia of autism Spectrum disorders. New York, NY: Springer New York; 2020:1-5.
50.
KljajićMMalteseGTarnowPSandPKölbyL. [Formula: See text] sustained attention and vigilance of children treated for sagittal and metopic craniosynostosis. Child Neuropsychol. May 2020;26(4):475-488. doi:https://doi.org/10.1080/09297049.2019.1682130
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