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

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.

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

References

• 1 Achenbach T M. Manual for the Child Behavior Checklist 4 - 18 and 1991 profile. Burlington, VT; University of Vermont 1991
• 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
• 3 Arnaud E, Renier D, Marchac D. Prognosis for mental function in scaphocephaly.  J Neurosurg. 1995;  83 476-479
• 4 Barritt J, Brooksbank M, Simpson D. Scaphocephaly: aesthetic and psychosocial considerations.  Dev Med Child Neurol. 1981;  23 183-191
• 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
• 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
• 7 De Castro P, Pascual-Castroviejo I. Evolucion mental de 108 casos de craneosinostosis.  An Esp Pediatr. 1981;  15 443-448
• 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
• 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
• 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
• 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
• 12 Grissemann H. Zürcher Lesetest ZLT. 6. Auflage, Bern; Huber 2000: 15-16
• 13 Hammill D D, Pearson N A, Voress J K. Developmental Test of Visual Perception. 2nd ed. DTVP-2. Austin; pro-ed 1993
• 14 Hooper H E. Hooper Visual Organization Test (VOT). Los Angeles; Western Psychological Services 1983
• 15 Kapp-Simon K A. Mental development and learning disorders in children with single suture craniosynostosis.  Cleft Palate J. 1998;  35 197-203
• 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
• 17 Krasnicanova H, Zemkova D, Skodova I. Longitudinal follow-up of children after surgical treatment of scaphocephaly.  Acta Chir Plast. 1996;  38 50-53
• 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
• 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
• 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
• 21 Lavigne J V, Faier-Routman J. Psychological adjustment to pediatric physical disorders: a meta-analytic review.  J Pediatr Psychol. 1992;  17 133-157
• 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
• 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
• 24 Regard M, Strauss E. Children's production on verbal and nonverbal fluency tasks.  Percept Mot Skills. 1982;  55 839-844
• 25 Schellig D, Hättig H A. Die Bestimmung der visuellen Merkspanne mit dem Block-Board.  Z Neuropsychol. 1993;  2 104-122
• 26 Schweisthal B. Die Leistungen von 7- bis 15-jährigen Kindern im verbalen Lern- und Merkfähigkeits-Test (VLMT).  Z Neuropsychol. 1997;  2 129-136
• 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
• 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
• 29 Spreen O, Strauss E. A compendium of neuropsychological tests. New York; Oxford University Press 1991: 168-176
• 30 Tewes U. Hamburg-Wechsler-Intelligenztest für Kinder III. HAWIK-III. Bern; Verlag Hans Huber 1999
• 31 Tewes U. Hamburg-Wechsler-Intelligenztest für Erwachsene Revision 1991. HAWIE-R. 2. korrigierte Auflage, Bern; Verlag Hans Huber 1994
• 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
• 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
• 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
• 35 Virtanen R, Korhonen T, Fagerholm J, Viljanto J. Neurocognitive sequelae of scaphocephaly.  Pediatrics. 1999;  103 791-795
• 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
• 37 Wehrli A. Neuropsychologische Untersuchung im Kindesalter. Universität Zürich; Dissertation 1980
• 38 Zimmermann P, Fimm B. Testbatterie zur Aufmerksamkeitsprüfung (TAP). Herzogenrath: Psytest. 1993

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