Skip to main content
SpringerLink
Log in

Neuropsychological Evaluation of Single Suture Craniosynostosis (SSC)

  • Living reference work entry
  • First Online:
Textbook of Pediatric Neurosurgery

  • 151 Accesses

Abstract

Single suture craniosynostosis (SSC) are characterized by the premature fusion of one or more sutures separating the bony plates of the skull during the early development. They can occur as isolated disorder or be part of a syndrome. Isolated craniosynostosis usually involves one suture (mono-sutural synostosis) and shows a significantly better esthetic, surgical, and developmental outcome if compared with multi-sutural/syndromic synostosis. However, also, children affected by mono-sutural synostosis can show an adverse neurodevelopmental outcome, especially concerning visual, cognitive, language, and motor domains.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References

  • Akinbami LJ, Liu X, Pastor PN, Reuben CA (2011) Attention deficit hyperactivity disorder among children aged 5–17 years in the United States, 1998–2009. NCHS Data Brief 70:1–8

    Google Scholar 

  • Aldridge K, Marsh JL, Govier D, Richtsmeier JT (2002) Central nervous system phenotypes in craniosynostosis. J Anat 201:31–39

    Article  PubMed  PubMed Central  Google Scholar 

  • Aldridge K, Kane AA, Marsh JL, Panchal J, Boyadjiev SA, Yan P, Govier D, Ahmad W, Richtsmeier JT (2005a) Brain morphology in nonsyndromic unicoronal craniosynostosis. Anat Rec A Discov Mol Cell Evol Biol 285:690–698

    Article  PubMed  Google Scholar 

  • Aldridge K, Kane AA, Marsh JL, Yan P, Govier D, Richtsmeier JT (2005b) Relationship of brain and skull in pre- and postoperative sagittal synostosis. J Anat 206:373–385

    Article  PubMed  PubMed Central  Google Scholar 

  • Anderson P (2002) Assessment and development of executive function (EF) during childhood. Child Neuropsychol 8:71–82

    Article  PubMed  Google Scholar 

  • Arnaud E, Renier D, Marchac D (1995) Prognosis for mental function in scaphocephaly. J Neurosurg 83:476–479

    Article  CAS  PubMed  Google Scholar 

  • Bagolini B, Campos EC, Chiesi C (1982) Plagiocephaly causing superior oblique deficiency and ocular torticollis. A new clinical entity. Arch Ophthalmol 100:1093–1096

    Article  CAS  PubMed  Google Scholar 

  • Becker DB, Petersen JD, Kane AA, Cradock MM, Pilgram TK, Marsh JL (2005) Speech, cognitive, and behavioural outcomes in nonsyndromic craniosynostosis. Plast Reconstr Surg 116:400–407

    Article  CAS  PubMed  Google Scholar 

  • Beckett JS, Brooks ED, Lacadie C, Wyk BV, Jou RJ, Steinbacher DM, Constable RT, Pelphrey KA, Persing JA (2014) Altered brain connectivity in sagittal craniosynostosis. J Neurosurg Pediatr 13:690–698. [Epub ahead of print]

    Article  PubMed  Google Scholar 

  • Bellew M, Chumas PD, Mueller R, Liddington M, Russell J (2005) Pre- and postoperative developmental attainment in sagittal synostosis. Arch Dis Child 90:346–350

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Bellew M, Liddington M, Chumas P, Russell J (2011) Preoperative and postoperative developmental attainment in patients with sagittal synostosis: 5-year follow-up. J Neurosurg Pediatr 7:121–126

    Article  PubMed  Google Scholar 

  • Boltshauser E, Ludwig S, Dietrich F, Landolt MA (2003) Sagittal craniosynostosis: cognitive, development, behavior, and quality of life in unoperated children. Neuropediatrics 34:293–300

    Article  CAS  PubMed  Google Scholar 

  • Bottero L, Lajeunie E, Arnaud E, Marchac D, Renier D (1998) Functional outcome after surgery for trigonocephaly. Plast Reconstr Surg 102:952–958

    Article  CAS  PubMed  Google Scholar 

  • Brent RC, Kapp-Simon KA, Wallace E, Michaeleen M, Cradock LB, Speltz ML (2015) Attention and executive function in children with and without single-suture craniosynostosi. Child Neuropsychol 23(1):83–98

    Google Scholar 

  • Bristol RE, Lekovic GP, Rekate HL (2004) The effects of craniosynostosis on the brain with respect to intracranial pressure. Semin Pediatr Neurol 11:262–267

    Article  PubMed  Google Scholar 

  • Chieffo D, Tamburrini G, Massimi L, Di Giovanni S, Giansanti C, Caldarelli M, Di Rocco C (2010) Long-term neuropsychological development in single-suture craniosynostosis treated early. J Neurosurg Pediatr 5:232–237

    Article  PubMed  Google Scholar 

  • Cohen SR, Persing JA (1998) Intracranial pressure in single-suture craniosynostosis. Cleft Palate Craniofac J 35(3):194–196

    Article  CAS  PubMed  Google Scholar 

  • Collett BR, Kapp-Simon KA, Wallace E, Cradock MM, Buono L, Speltz ML (2017) Attention and executive function in children with and without single-suture craniosynostosis. Child Neuropsychol 23(1):83–98

    Article  PubMed  Google Scholar 

  • Cohen SR, Cho DC, Nichols SL, Simms C, Cross KP, Burstein FD (2004) American Society of Maxillofacial Surgeons Outcome Study: preoperative and postoperative neurodevelopmental findings in single-suture craniosynostosis. Plast Reconstr Surg 114:841–847

    Article  PubMed  Google Scholar 

  • Da Costa AC, Walters I, Savarirayan R, Anderson VA, Wrennall J, Meara JG (2006) Intellectual outcomes in children and adolescents with syndromic and nonsyndraomic craniosynostosis. Plast Reconstr Surg 118:175–181

    Article  PubMed  CAS  Google Scholar 

  • Da Costa AC, Anderson VA, Savarirayan R, Wrennal JA, Chong DK, Holmes AD, Greensmith AL, Meara JG (2012) Neurodevelopmental functioning of infants with untreated single-suture craniosynostosis during early infancy. Childs Nerv Syst 28:869–877

    Article  PubMed  Google Scholar 

  • Demer JL (2006) Current concepts of mechanical and neural factors in ocular motility. Curr Opin Neurol 9:4–13

    Article  Google Scholar 

  • Denis D, Genitori L, Conrath J, Lena G, Choux M (1996) Ocular findings in children operated on for plagiocephaly and trigonocephaly. Childs Nerv Syst 12:683–689

    Article  CAS  PubMed  Google Scholar 

  • Dewey C, Fleming P, Golding J (1998) Does the supine sleeping position have any adverse effects on the child? II. Development in the first 18 months. ALSPAC Study Team. Pediatrics 101:E5

    Article  CAS  PubMed  Google Scholar 

  • Dubowitz LM, Mushin J, Morante A, Placzek M (1983) The maturation of visual acuity in neurologically normal and abnormal newborn infants. Behav Brain Res 10:39–45

    Article  CAS  PubMed  Google Scholar 

  • Espy KA, McDiarmid MM, Cwik MF, Stalets MM, Hamby A, Senn TE (2004) The contribution of executive functions to emergent mathematic skills in preschool children. Dev Neuropsychol 26:465–486

    Article  PubMed  Google Scholar 

  • Fernbach SK, Feinstein KA (1991) Radiologic evaluation of children with craniosynostosis. Neurosurg Clin N Am 2:569–586

    Article  CAS  PubMed  Google Scholar 

  • Fletcher JM, Francis DJ, Morris RD, Lyon GR (2005) Evidence-based assessment of learning disabilities in children and adolescents. J Clin Child Adolesc Psychol 34(3):506–522

    Article  PubMed  Google Scholar 

  • Froehlich TE, Lanphear BP, Epstein JN, Barbaresi WJ, Katusic SK, Kahn RS (2007) Prevalence, recognition, and treatment of attention-deficit/hyperactivity disorder in a national sample of US children. Arch Pediatr Adolesc Med 161(9):857–864

    Article  PubMed  Google Scholar 

  • Gewalli F, Guimaraes-ferreira JP, Sahlin P, Emanuelsson I, Horneman G, Stephensen H, Lauritzen CG (2001) Mental development after modified pi procedure: dynamic cranioplasty for sagittal synostosis. Ann Plast Surg 46:415–420

    Article  CAS  PubMed  Google Scholar 

  • Greenberg BM, Schneider SJ (2006) Trigonocephaly: surgical considerations and long term evaluation. J Craniofac Surg 17:528–535

    Article  PubMed  Google Scholar 

  • Gupta PC, Foster J, Crowe S, Papay FA, Luciano M, Traboulsi EI (2003) Ophthalmologic findings in patients with nonsyndromic plagiocephaly. J Craniofac Surg 14:529–532

    Article  PubMed  Google Scholar 

  • Hashim PW, Patel A, Yang JF, Travieso R, Terner J, Losee JE, Persing JA (2014) The effects of whole-vault cranioplasty versus strip craniectomy on long-term neuropsychological outcomes in sagittal craniosynostosis. Plast Reconstr Surg 134(3):491–501

    Article  CAS  PubMed  Google Scholar 

  • Hunter AG, Rudd NL (1977) Coronal synostosis: its familial characteristics and associated clinical findings in 109 patients lacking bilateral polysyndactyly or syndactyly. Teratology 15:301–310

    Article  CAS  PubMed  Google Scholar 

  • Kapp-Simon KA (1998) Mental development and learning disorders in children with single suture craniosynostosis. Cleft Palate Craniofac J 35(3):197–203

    Article  CAS  PubMed  Google Scholar 

  • Kapp-Simon KA, Leroux B, Cunningham ML, Speltz ML (2005) Multi-site study of infants with single-suture craniosynostosis: preliminary report of pre-surgery development. Cleft Palate Craniofac J 42:377–384

    Article  PubMed  Google Scholar 

  • Kapp-Simon KA, Speltz ML, Cunningham ML, Patel PK, Tomita T (2007) Neurodevelopment of children with single-suture craniosynostosis: a review. Childs Nerv Syst 23:269–228

    Article  PubMed  Google Scholar 

  • Kapp-Simon KA, Collett BR, Barr-Schinzel MA, Cradock MM, Buono LA, Pietila KE, Speltz ML (2012) Behavioral adjustment of toddler and preschool-aged children with single-suture craniosynostosis. Plast Reconstr Surg 130:635–647

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Khan SH, Nischal KK, Dean F, Hayward RD, Walker J (2003) Visual outcomes and amblyogenic risk factors in craniosynostotic syndromes: a review of 141 cases. Br J Ophthalmol 87: 999–1003

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Knight SJ, Anderson VA, Spencer-Smith MM, Da Costa AC (2014) Neurodevelopmental outcomes in infants and children with single-suture craniosynostosis: a systematic review. Dev Neuropsychol 39:159–186

    Article  PubMed  Google Scholar 

  • Lahey BB, Pelham WE, Stein MA, Loney J, Trapani C, Nugent K, Kipp H, Schmidt E, Lee S, Cale M, Gold E, Hartung CM, Willcutt E, Baumann B (1998) Validity of DSM-IV attention-deficit/hyperactivity disorder for younger children. J Am Acad Child Adolesc Psychiatry 37(7):695–702. Erratum in: J Am Acad Child Adolesc Psychiatry 38(2):222

    Article  CAS  PubMed  Google Scholar 

  • Lajeunie E, Le Merrer M, Marchac D, Renier D (1998) Syndromal and nonsyndromal primary trigonocephaly: analysis of a series of 237 patients. Am J Med Genet 13;75(2):211–215

    Article  CAS  PubMed  Google Scholar 

  • Lattanzi W, Bukvic N, Barba M, Tamburrini G, Bernardini C, Michetti F, Di Rocco C (2012) Genetic basis of single-suture synostoses: genes, chromoromes and clinical implications. Child Nerv Syst 28:1301–1310

    Article  Google Scholar 

  • Leonard CM, Eckert MA, Lombardino LJ, Oakland T, Kranzler J, Mohr CM, King WM, Freeman A (2001) Anatomical risk factors for phonological dyslexia. Cereb Cortex 11:148–157

    Article  CAS  PubMed  Google Scholar 

  • Limon de Brown E, Ortiz Monasterio F, Stark Feldman M (1988) Strabismus in plagiocephaly. J Pediatr Ophthalmol Strabismus 25:180–190

    Article  CAS  PubMed  Google Scholar 

  • Limperopoulos C, Majnemer A, Shevell MI, Rohlicek C, Rosenblatt B, Tchervenkov C, Darwish HZ (2002) Predictors of developmental disabilities after open heart surgery in young children with congenital heart defects. J Pediatr 141(1):51–58

    Article  PubMed  Google Scholar 

  • Loddenkemper T, Holland KD, Stanford LD, Kotagal P, Bingaman W, Wyllie E (2007) Developmental outcome after epilepsy surgery in infancy. Pediatrics 119(5):930–935

    Article  PubMed  Google Scholar 

  • Lomax-Bream LE, Barnes M, Copeland K, Taylor HB, Landry SH (2007) The impact of spina bifida on development across the first 3 years. Dev Neuropsychol 31(1):1–20

    Article  PubMed  Google Scholar 

  • Magge SN, Westerveld M, Pruzinsky T, Persing JA (2002) Long-term neuropsychological effects of sagittal craniosynostosis on child development. J Craniofac Surg 13:99–104

    Article  PubMed  Google Scholar 

  • Mash EJ, Wolfe DA (2005) Abnormal child psychiatry, 3rd edn. Thompson Wadsworth, Belmont

    Google Scholar 

  • McGee R, Williams S (1991) Social competence in adolescence: preliminary findings from a longitudinal study of New Zealand 15-year-olds. Psychiatry 54(3):281–291

    Article  CAS  PubMed  Google Scholar 

  • Mendonca DA, White N, West E, Dover S, Solanki G, Nishikawa H (2009) Is there a relationship between the severity of metopic synostosis and speech and language impairments. J Craniofac Surg 20:85–88

    Article  PubMed  Google Scholar 

  • Mercuri E, Atkinson J, Braddick O, Anker S, Nokes L, Cowan F, Rutherford M, Pennock J, Dubowitz L (1996) Visual function and perinatal focal cerebral infarction. Arch Dis Child 75:F76–F81

    Article  CAS  Google Scholar 

  • Panchal J, Marsh JL, Park TS, Kaufman B, Pilgram T, Huang SH (1999) Sagittal craniosynostosis outcome assessment for two methods and timings of intervention. Plast Reconstr Surg 103(6): 1574–1584

    Article  CAS  PubMed  Google Scholar 

  • Paniagua B, Emodi O, Hill J, Fishbaugh J, Pimenta LA, Aylward SR, Andinet E, Gerig G, Gilmore J, van Aalst JA, Styner M (2013) 3D of brain shape and volume after cranial vault remodeling surgery for craniosynostosis correction in infants. Proc Soc Photo Opt Instrum Eng 29(8672):86720V

    Google Scholar 

  • Patel A, Yang JF, Hashim PW, Travieso R, Terner J, Mayes LC, … Persing JA (2014) The impact of age at surgery on long-term neuropsychological outcomes in sagittal craniosynostosis. Plast Reconstr Surg 134(4):608–617

    Article  CAS  PubMed  Google Scholar 

  • Pelo S, Tamburrini G, Marianetti TM, Saponaro G, Moro A, Gasparini G, Di Rocco C (2011) Correlations between the abnormal development of the skull base and facial skeleton growth in anterior synostotic plagiocephaly: the predictive value of a classification based on CT scan classification. Childs Nerv Syst 27:1431–1443

    Article  PubMed  Google Scholar 

  • Renier D, Sainte-Rose C, Marchac D, Hirsch JF (1982) Intracranial pressure in craniostenosis. J Neurosurg 57(3):370–377

    Article  CAS  PubMed  Google Scholar 

  • Renier D, Lajeunie E, Arnaud E, Marchac D (2000) Management of craniosynostosis. Childs Nerv Syst 16:645–658

    Article  CAS  PubMed  Google Scholar 

  • Ricci D, Vasco G, Baranello G, Salerni A, Amante R, Tamburrini G, Dickmann A, Di Rocco C, Velardi F, Mercuri E (2007) Visual function in infants with non-syndromic craniosynostosis. Dev Med Child Neurol 49:574–576

    Article  CAS  PubMed  Google Scholar 

  • Shimabukuro S, Shimoji T, Sugama S (2001) Cranioplasty for isolated trigonocephaly with developmental disorder. No To Hattatsu 33(6):487–493

    CAS  PubMed  Google Scholar 

  • Shimoji T, Shimabukuro S, Sugama S, Ochiai Y (2002) Mild trigonocephaly with clinical symptoms: analysis of surgical results in 65 patients. Childs Nerv Syst. 18(5):215–224

    Article  PubMed  Google Scholar 

  • Shipster C, Hearst D, Somerville A, Stackhouse J, Hayward R, Wade A (2003) Speech, language, and cognitive development in children with isolated sagittal synostosis. Dev Med Child Neurol 45:34–43

    Article  PubMed  Google Scholar 

  • Sidoti EJ Jr, Marsh JL, Marty-Grames L, Noetzel MJ (1996) Long-term studies of metopic synostosis: frequency of cognitive impairment and behavioral disturbances. Plast Reconstr Surg 97(2):276–281

    Article  PubMed  Google Scholar 

  • Speltz ML, Kapp-Simon KA, Cunningham M, Marsh J, Dawson G (2004) Single-suture craniosynostosis: a review of neurobehavioral research and theory. J Pediatr Psychol 29:651–668

    Article  PubMed  Google Scholar 

  • Speltz ML, Kapp-Simon KA, Collett B, Keich Y, Gaither R, Cradock MM, Buono L, Cunningham M (2007) Neurodevelopment of infants with single-suture craniosynostosis: pre-surgery comparisons with case-matched controls. Plast Reconstr Surg 119:1874–1881

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Speltz ML, Kapp-Simon KA, Collett BR, Wallace E, Starr JR, Cradock MM, … Cunningham ML (2015) Intellectual and academic functioning of school-age children with single-suture craniosynostosis. Pediatrics 135:615–623

    Article  PubMed  PubMed Central  Google Scholar 

  • Starr JR, Collett BR, Gaither R, Kapp-Simon KA, Cradock MM, Cunningham ML, Speltz ML (2012) Multicenter study of neurodevelopment in 3-year-old children with and without single-suture craniosynostosis. Arch Pediatr Adolesc Med 166:536–542

    Article  PubMed  PubMed Central  Google Scholar 

  • Tamburrini G, Caldarelli M, Massimi L, Santini P, Di Rocco C (2005) Intracranial pressure monitoring in children with single suture and complex craniosynostosis: a review. Childs Nerv Syst 21:913–921

    Article  CAS  PubMed  Google Scholar 

  • Tay T, Martin F, Rowe N, Johnson K, Poole M, Tan K, Kennedy I, Gianoutsos M (2006) Prevalence and causes of visual impairment in craniosynostotic syndromes. Clin Exp Ophthalmol 34: 434–440

    Article  PubMed  Google Scholar 

  • Treyvaud K, Anderson VA, Howard K, Bear M, Hunt RW, Doyle LW, Inder TE, Woodward L, Anderson PJ (2009) Parenting behavior is associated with the early neurobehavioral development of very preterm children. Pediatrics. Feb 123(2):555–561

    Google Scholar 

  • Vasco G, Baranello G, Ricci D, Salerni A, Tamburrini G, Amante R, Dickmann A, Di Rocco C, Velardi F, Mercuri E (2008) Longitudinal assessment of visual development in non-syndromic craniosynostosis: a 1-year pre- and post-surgical study. Arch Dis Child 93:932–935

    Article  CAS  PubMed  Google Scholar 

  • Warschausky S, Angobaldo J, Kewman D, Buchman S, Muraszko KM, Azengart A (2005) Early development of infants with untreated metopic craniosynostosis. Plast Reconstr Surg 115: 1518–1523

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Institute of Infantile Neuropsychiatry, Fondazione Policlinico Gemelli IRCSS, Rome, Italy

    Daniela Chieffo

  2. Università Cattolica del Sacro Cuore, Rome, Italy

    Daniela Chieffo

Authors
  1. Daniela Chieffo
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Daniela Chieffo .

Editor information

Editors and Affiliations

  1. International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Oakland Medical Center, Oakland, CA, USA

    Dachling Pang

  3. University of Toronto Hospital for Sick Children, Toronto, ON, Canada

    James T. Rutka

Section Editor information

  1. Department of Neurosurgery, International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Neurochirurgie Pediatrique, Hopital Femme Mere Enfant, University Lyon 1, Lyon, France

    Rocco, F Di

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Chieffo, D. (2019). Neuropsychological Evaluation of Single Suture Craniosynostosis (SSC). In: Di Rocco, C., Pang, D., Rutka, J. (eds) Textbook of Pediatric Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-319-31512-6_66-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-31512-6_66-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-31512-6

  • Online ISBN: 978-3-319-31512-6

  • eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences

Publish with us

Policies and ethics

Search

Navigation