Download PDF
Neuropediatrics 2003; 34(6): 293-300
DOI: 10.1055/s-2003-44667
Original Article

Georg Thieme Verlag Stuttgart · New York

Sagittal Craniosynostosis: Cognitive Development, Behaviour, and Quality of Life in Unoperated Children[*]

E. Boltshauser 1 , S. Ludwig 1 , F. Dietrich 1 , M. A. Landolt 2
  • 1Department of Paediatric Neurology, University Children's Hospital, Zuerich, Switzerland
  • 2Department of Psychosomatics and Psychiatry, University Children's Hospital, Zuerich, Switzerland
Further Information

Publication History

Received: May 28, 2003

Accepted after Revision: September 22, 2003

Publication Date:
18 December 2003 (online)

    Permissions and Reprints

Abstract

30 patients (23 m/7 f) with untreated, isolated sagittal craniosynostosis (ISC) were re-evaluated at an average age of 9.25 years (2.5 - 25.5). Assessed were neurological status, head shape, school performance, behaviour (with Child Behavior Checklist CBCL), quality of life (TACQOL questionnaire), and a detailed neuropsychological testing (30 patients, 17 siblings as controls). Almost all patients/parents were pleased with the current situation. The aesthetic appearance seemed less obvious compared to photos in infancy. Psychological adjustment as measured by the CBCL was well within normal range. There was a slight tendency for increased internalizing symptoms. TACQOL revealed a lower score for positive emotions. Total score and subscores of test batteries assessing general intellectual performance were in the average range. 6 patients had below average scores in subtests for learning and memory. 12 children had below average scores in one or more subtests for attention. These “deficits” were obviously not of functional importance as all children attended regular school classes (1 exception, an immigrant child of low socio-economic status). These reassuring results regarding school performances, behaviour, and quality of life as well as aesthetic outcome may be helpful when discussing the indication for surgical correction of ISC.

Key words

Sagittal craniosynostosis - scaphocephaly - behaviour - cognition - development - quality of life - natural history

1 Results were presented in part at the Annual Meeting, Gesellschaft für Neuropädiatrie, Recklinghausen, on March 29, 2001.

References

  • 1 Achenbach T M. Manual for the Child Behavior Checklist 4 - 18 and 1991 profile. Burlington, VT; University of Vermont 1991
    Google Scholar
  • 2 Alvarez-Garijo J A, Cavadas P C, Vila M M, Alvarez-Llanas A. Sagittal synostosis: results of surgical treatment in 210 patients.  Child's Nerv Syst. 2001;  17 64-68
    PubMedGoogle Scholar
  • 3 Arnaud E, Renier D, Marchac D. Prognosis for mental function in scaphocephaly.  J Neurosurg. 1995;  83 476-479
    CrossrefPubMedGoogle Scholar
  • 4 Barritt J, Brooksbank M, Simpson D. Scaphocephaly: aesthetic and psychosocial considerations.  Dev Med Child Neurol. 1981;  23 183-191
    PubMedGoogle Scholar
  • 5 Camfield P R, Camfield C S, Cohen Jr M M. Neurologic aspects of craniosynostosis. Cohen MM Jr, Mac Lean RE Craniosynostosis. Diagnosis, Evaluation, and Management. 2nd ed. New York, Oxford; Oxford University Press 2000: 177-183
    Google Scholar
  • 6 Cohen Jr M M. Epidemiology of craniosynostosis. Cohen MM Jr, Mac Lean RE Craniosynostosis. Diagnosis, Evaluation, and Management. 2nd ed. New York, Oxford; Oxford University Press 2000: 112-117
    Google Scholar
  • 7 De Castro P, Pascual-Castroviejo I. Evolucion mental de 108 casos de craneosinostosis.  An Esp Pediatr. 1981;  15 443-448
    PubMedGoogle Scholar
  • 8 De Spiegelaere M, Dramaix M, Hennart P. Sociooeconomic status and changes in body mass from 3 to 5 years.  Arch Dis Child. 1998;  78 477-478
    PubMedGoogle Scholar
  • 9 Doepfner M, Melchers P, Fegert J. et al . Deutschsprachige Konsensus-Versionen der Child Behavior Checklist (CBCL 4 - 18), der Teacher Report Form (TRF) und der Youth Self Report Form (YSR).  Kindheit und Entwicklung. 1994;  3 54-59
    PubMedGoogle Scholar
  • 10 Földényi M, Tagwerker-Neuenschwander F, Giovanoli A, Schallberger U, Steinhausen H Ch. Die Aufmerksamkeitsleistungen von 6 - 10-jährigen Kindern in der TAP.  Z Neuropsychol. 1999;  2 87-102
    PubMedGoogle Scholar
  • 11 Gewalli F, Guimaraes-Ferreira J P, Sahlin P, Emanuelsson I, Horneman G, Stephensen H, Lauritzen C G. Mental development after modified pi procedure: dynamic cranioplasty for sagittal synostosis.  Ann Plast Surg. 2001;  46 415-420
    PubMedGoogle Scholar
  • 12 Grissemann H. Zürcher Lesetest ZLT. 6. Auflage, Bern; Huber 2000: 15-16
    Google Scholar
  • 13 Hammill D D, Pearson N A, Voress J K. Developmental Test of Visual Perception. 2nd ed. DTVP-2. Austin; pro-ed 1993
    Google Scholar
  • 14 Hooper H E. Hooper Visual Organization Test (VOT). Los Angeles; Western Psychological Services 1983
    Google Scholar
  • 15 Kapp-Simon K A. Mental development and learning disorders in children with single suture craniosynostosis.  Cleft Palate J. 1998;  35 197-203
    PubMedGoogle Scholar
  • 16 Kaufman A S, Kaufman N L. Kaufman Assessment Battery for Children. K-ABC. Deutschsprachige Fassung von P. Melchers und U. Preuss. 4. korrigierte und ergänzte Auflage, Frankfurt am Main; Swets & Zeitlinger 1994
    Google Scholar
  • 17 Krasnicanova H, Zemkova D, Skodova I. Longitudinal follow-up of children after surgical treatment of scaphocephaly.  Acta Chir Plast. 1996;  38 50-53
    PubMedGoogle Scholar
  • 18 Kunert H J, Derichs G, Irle E. Entwicklung von Aufmerksamkeitsfunktionen im Kindesalter: Ergebnisse einer vorläufigen Normierung der computergestützten Testbatterie zur Aufmerksamkeitsprüfung (TAP) an 9- bis 12-jährigen Kindern.  Z Neuropsychol. 1996;  2 92-113
    PubMedGoogle Scholar
  • 19 Landolt M A, Steinmann B, Superti-Furga A. Quality of life and psychologic adjustment in children and adolescents with early treated phenylketonuria can be normal.  J Pediatr. 2002;  140 516-521
    CrossrefPubMedGoogle Scholar
  • 20 Landolt M A, Grubenmann S, Meuli M. Family impact greatest: Predictors of quality of life and psychological adjustment in pediatric burn survivors.  J Trauma. 2002;  53 1146-1151
    PubMedGoogle Scholar
  • 21 Lavigne J V, Faier-Routman J. Psychological adjustment to pediatric physical disorders: a meta-analytic review.  J Pediatr Psychol. 1992;  17 133-157
    CrossrefPubMedGoogle Scholar
  • 22 Matsumoto K, Nakanishi H, Seike T, Shinno K, Hirabayashi S. Application of the distraction technique to scaphocephaly.  J Craniofac Surgery. 2000;  11 172-176
    PubMedGoogle Scholar
  • 23 Panchal J, Marsh J L, Park T S, Kaufman B, Pilgram T, Huang S H. Sagittal craniosynostosis outcome assessment for two methods and timings of intervention.  Plast Reconstr Surg. 1999;  103 1574-1584
    CrossrefPubMedGoogle Scholar
  • 24 Regard M, Strauss E. Children's production on verbal and nonverbal fluency tasks.  Percept Mot Skills. 1982;  55 839-844
    CrossrefPubMedGoogle Scholar
  • 25 Schellig D, Hättig H A. Die Bestimmung der visuellen Merkspanne mit dem Block-Board.  Z Neuropsychol. 1993;  2 104-122
    PubMedGoogle Scholar
  • 26 Schweisthal B. Die Leistungen von 7- bis 15-jährigen Kindern im verbalen Lern- und Merkfähigkeits-Test (VLMT).  Z Neuropsychol. 1997;  2 129-136
    PubMedGoogle Scholar
  • 27 Shipster C, Hearst D, Somerville A, Stackhouse J, Hayward R, Wade A. Speech, language, and cognitive development in children with isolated sagittal synostosis.  Dev Med Child Neurol. 2003;  45 34-43
    PubMedGoogle Scholar
  • 28 Speltz M L, Endriga M C, Mouradian W E. Presurgical and postsurgical mental and psychomotor development of infants with sagittal synostosis.  Cleft Palate J. 1997;  34 374-379
    PubMedGoogle Scholar
  • 29 Spreen O, Strauss E. A compendium of neuropsychological tests. New York; Oxford University Press 1991: 168-176
    Google Scholar
  • 30 Tewes U. Hamburg-Wechsler-Intelligenztest für Kinder III. HAWIK-III. Bern; Verlag Hans Huber 1999
    Google Scholar
  • 31 Tewes U. Hamburg-Wechsler-Intelligenztest für Erwachsene Revision 1991. HAWIE-R. 2. korrigierte Auflage, Bern; Verlag Hans Huber 1994
    Google Scholar
  • 32 Turtas S, Tondi M, Tola G, Sehrbundt-Viale E, Martinez P L. Neuropsychological evaluation in a series of 17 cases operated on for cranosynostosis.  Acta Neurochir. 1993;  120 123-125
    PubMedGoogle Scholar
  • 33 Vannier M W. Radiologic evaluation of craniosynostosis. Cohen MM Jr, Mac Lean RE Craniosynostosis. Diagnosis, Evaluation, and Management. 2nd ed. New York, Oxford; Oxford University Press 2000: 147-157
    Google Scholar
  • 34 Verrips E GH, Vogels T GC, Koopman H M. et al . Measuring health-related quality of life in a child population.  Eur J Pub Health. 1999;  9 114-119
    PubMedGoogle Scholar
  • 35 Virtanen R, Korhonen T, Fagerholm J, Viljanto J. Neurocognitive sequelae of scaphocephaly.  Pediatrics. 1999;  103 791-795
    CrossrefPubMedGoogle Scholar
  • 36 Vogels T, Verrips G HW, Koopman H M, Theunissen N CM, Fekkes M, Kamphuis R P. TACQOL manual: parent form and child form. Leiden; Center for child health and pediatrics LUMC-TNO 2000
    Google Scholar
  • 37 Wehrli A. Neuropsychologische Untersuchung im Kindesalter. Universität Zürich; Dissertation 1980
    Google Scholar
  • 38 Zimmermann P, Fimm B. Testbatterie zur Aufmerksamkeitsprüfung (TAP). Herzogenrath: Psytest. 1993
    Google Scholar

1 Results were presented in part at the Annual Meeting, Gesellschaft für Neuropädiatrie, Recklinghausen, on March 29, 2001.

Prof. Eugen Boltshauser

University Children's Hospital

Steinwiesstrasse 75

8032 Zuerich

Switzerland

Email: eugen.boltshauser@kispi.unizh.ch

      >