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Published in: Child's Nervous System 6/2012

01-06-2012 | Original Paper

Neurodevelopmental functioning of infants with untreated single-suture craniosynostosis during early infancy

Authors: Annette C. Da Costa, Vicki A. Anderson, Ravi Savarirayan, Jacquie A. Wrennall, David K. Chong, Anthony D. Holmes, Andrew L. Greensmith, John G. Meara

Published in: Child's Nervous System | Issue 6/2012

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Abstract

Purpose

Single-suture craniosynostosis (SSC) is a congenital craniofacial disorder, in which premature fusion of one of the skull sutures restricts and distorts growth of the cranium and underlying brain. This disorder of prenatal onset occurs during a critical phase of rapid growth and development of the immature brain. Craniosynostosis carries a known risk of developmental impairment. The neurodevelopmental sequelae of SSC prior to treatment remains however incompletely understood. This study sought to determine the neurodevelopmental sequelae of untreated single-suture craniosynostosis during early infancy.

Methods

Fifty-six consecutive patients with unoperated SSC (sagittal, metopic and unicoronal) comprised the sample cohort. Patients were aged between 4 and 16 months (M = 8.9 months, SD = 2.9 months). Neurodevelopmental functioning was assessed with the mental (Mental Development Index) and motor (Psychomotor Development Index) scales of the Bayley Scales of Infant Development, second edition.

Results

Children with SSC displayed significantly lower mean mental (M = 97.7, SD = 6.7, p < 0.05) and motor (M = 87.7, SD = 13.0, p < 0.001) scores than normative population averages. The distribution of these scores also differed significantly from the normative distribution; an increased rate of significant motor developmental delay was found, and none of the children displayed accelerated development. Subgroup comparisons between the primary diagnostic subtypes in this sample revealed no significant differences in mental or motor skill functioning.

Conclusions

Untreated SSC is associated with an increased incidence of developmental delay during early infancy, with motor skills appearing the most vulnerable to impairment during this developmental phase.
Literature
1.
go back to reference Cohen MJ, MacLean R (2000) Craniosynostosis: diagnosis, evaluation, and management. Oxford Uni Press, New York Cohen MJ, MacLean R (2000) Craniosynostosis: diagnosis, evaluation, and management. Oxford Uni Press, New York
2.
go back to reference Singer S, Bower C, Southall P, Goldblatt J (1999) Craniosynostosis in Western Australia, 1990–1994: a population-based study. Am J Med Genet 83:382–387PubMedCrossRef Singer S, Bower C, Southall P, Goldblatt J (1999) Craniosynostosis in Western Australia, 1990–1994: a population-based study. Am J Med Genet 83:382–387PubMedCrossRef
3.
go back to reference Selber J, Reid R, Chike-Obi C, Sutton L, Zackai E, McDonald-McGinn D, Sonnad S, Whitaker L, Bartlett S (2008) The changing epidemiologic spectrum of single-suture synostoses. Plast Reconstr Surg 122:527–533PubMedCrossRef Selber J, Reid R, Chike-Obi C, Sutton L, Zackai E, McDonald-McGinn D, Sonnad S, Whitaker L, Bartlett S (2008) The changing epidemiologic spectrum of single-suture synostoses. Plast Reconstr Surg 122:527–533PubMedCrossRef
5.
go back to reference Shaw E, Steinbach H (1968) Aminopterin-induced fetal malformation. Survival of an infant after attempted abortion. Am J Dis Child 115:477–482PubMed Shaw E, Steinbach H (1968) Aminopterin-induced fetal malformation. Survival of an infant after attempted abortion. Am J Dis Child 115:477–482PubMed
6.
go back to reference Graham J, deSaxe M, Smith D (1979) Sagittal craniostenosis: fetal head constraint as one possible cause. J Pediatr 95:747–750PubMedCrossRef Graham J, deSaxe M, Smith D (1979) Sagittal craniostenosis: fetal head constraint as one possible cause. J Pediatr 95:747–750PubMedCrossRef
7.
go back to reference Greensmith A, Holmes A, Lo P, Maxiner W, Heggie A, Meara J (2008) Complete correction of severe scaphocephaly: the Melbourne method of total vault remodeling. Plast Reconstr Surg 121:1300–1310PubMedCrossRef Greensmith A, Holmes A, Lo P, Maxiner W, Heggie A, Meara J (2008) Complete correction of severe scaphocephaly: the Melbourne method of total vault remodeling. Plast Reconstr Surg 121:1300–1310PubMedCrossRef
8.
go back to reference Davis C, Wickremesekera A, MacFarlane M (2009) Correction of nonsynostotic scaphocephaly without cranial osteotomy: spring expansion of the sagittal suture. Childs Nervous System 25:225–230CrossRef Davis C, Wickremesekera A, MacFarlane M (2009) Correction of nonsynostotic scaphocephaly without cranial osteotomy: spring expansion of the sagittal suture. Childs Nervous System 25:225–230CrossRef
9.
go back to reference Gault D, Renier D, Marchac D, Jones B (1992) Intracranial pressure and intracranial volume in children with craniosynostosis. Plast Reconstr Surg 90:377–381PubMedCrossRef Gault D, Renier D, Marchac D, Jones B (1992) Intracranial pressure and intracranial volume in children with craniosynostosis. Plast Reconstr Surg 90:377–381PubMedCrossRef
10.
go back to reference Siddiqi S, Posnick J, Buncic R, Humphreys R, Hoffman H, Drake J, Rutka J (1995) The detection and management of intracranial hypertension after initial suture release and decompression for craniofacial dysostosis syndromes. Neurosurgery 36:703–708, discussion 708–709PubMedCrossRef Siddiqi S, Posnick J, Buncic R, Humphreys R, Hoffman H, Drake J, Rutka J (1995) The detection and management of intracranial hypertension after initial suture release and decompression for craniofacial dysostosis syndromes. Neurosurgery 36:703–708, discussion 708–709PubMedCrossRef
11.
go back to reference Thompson D, Harkness W, Jones B, Gonsalez S, Andar U, Hayward R (1995) Subdural intracranial pressure monitoring in craniosynostosis: its role in surgical management. Childs Nervous System 11:269–275CrossRef Thompson D, Harkness W, Jones B, Gonsalez S, Andar U, Hayward R (1995) Subdural intracranial pressure monitoring in craniosynostosis: its role in surgical management. Childs Nervous System 11:269–275CrossRef
12.
go back to reference Renier D, Lajeunie E, Arnaud E, Marchac D (2000) Management of craniosynostosis. Child's Nervous System 16:645–658PubMedCrossRef Renier D, Lajeunie E, Arnaud E, Marchac D (2000) Management of craniosynostosis. Child's Nervous System 16:645–658PubMedCrossRef
13.
go back to reference Bottero L, Lajeunie E, Arnaud E, Marchac D, Renier D (1998) Functional outcome after surgery for trigonocephaly. Plast Reconstr Surg 102:952–958, discussion 959–960PubMedCrossRef Bottero L, Lajeunie E, Arnaud E, Marchac D, Renier D (1998) Functional outcome after surgery for trigonocephaly. Plast Reconstr Surg 102:952–958, discussion 959–960PubMedCrossRef
14.
go back to reference Fernbach S, Feinstein K (1991) Radiologic evaluation of children with craniosynostosis. Neurosurg Clin North America 2:569–586 Fernbach S, Feinstein K (1991) Radiologic evaluation of children with craniosynostosis. Neurosurg Clin North America 2:569–586
15.
go back to reference Aldridge K, Marsh J, Govier D, Richtsmeier J (2002) Central nervous system phenotypes in craniosynostosis. J Anat 201:31–39PubMedCrossRef Aldridge K, Marsh J, Govier D, Richtsmeier J (2002) Central nervous system phenotypes in craniosynostosis. J Anat 201:31–39PubMedCrossRef
16.
go back to reference Aldridge K, Kane A, Marsh J, Panchal J, Boyadjiev S, Yan P, Govier D, Ahmad W, Richtsmeier J (2005) Brain morphology in nonsyndromic unicoronal craniosynostosis. The Anatomical Record, Part A: Discoveries in Molecular and Cellular Evolutionary Biology 285:690–698CrossRef Aldridge K, Kane A, Marsh J, Panchal J, Boyadjiev S, Yan P, Govier D, Ahmad W, Richtsmeier J (2005) Brain morphology in nonsyndromic unicoronal craniosynostosis. The Anatomical Record, Part A: Discoveries in Molecular and Cellular Evolutionary Biology 285:690–698CrossRef
17.
go back to reference Aldridge K, Kane A, Marsh J, Yan P, Govier D, Richtsmeier J (2005) Relationship of brain and skull in pre- and postoperative sagittal synostosis. J Anat 206:373–385PubMedCrossRef Aldridge K, Kane A, Marsh J, Yan P, Govier D, Richtsmeier J (2005) Relationship of brain and skull in pre- and postoperative sagittal synostosis. J Anat 206:373–385PubMedCrossRef
18.
go back to reference Reardon W, Wilkes D, Rutland P, Pulleyn L, Malcolm S, Dean J, Evans R, Jones B, Hayward R, Hall C, Nevin N, Baraister M, Winter R (1997) Craniosynostosis associated with FGFR3 pro250arg mutation results in a range of clinical presentations including unisutural sporadic craniosynostosis. J Med Genet 34:632–636PubMedCrossRef Reardon W, Wilkes D, Rutland P, Pulleyn L, Malcolm S, Dean J, Evans R, Jones B, Hayward R, Hall C, Nevin N, Baraister M, Winter R (1997) Craniosynostosis associated with FGFR3 pro250arg mutation results in a range of clinical presentations including unisutural sporadic craniosynostosis. J Med Genet 34:632–636PubMedCrossRef
19.
go back to reference Arnaud E, Meneses P, Lajeunie E, Thorne J, Marchac D, Renier D (2002) Postoperative mental and morphological outcome for nonsyndromic brachycephaly. Plast Reconstr Surg 110:6–12PubMedCrossRef Arnaud E, Meneses P, Lajeunie E, Thorne J, Marchac D, Renier D (2002) Postoperative mental and morphological outcome for nonsyndromic brachycephaly. Plast Reconstr Surg 110:6–12PubMedCrossRef
20.
go back to reference 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 75:211–215PubMedCrossRef 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 75:211–215PubMedCrossRef
21.
go back to reference Bayley N (1993) Bayley scales of infant development (2nd edition)(Bayley-II). Psychological Corporation, San Antonio Bayley N (1993) Bayley scales of infant development (2nd edition)(Bayley-II). Psychological Corporation, San Antonio
22.
go back to reference Arnaud E, Renier D, Marchac D (1995) Prognosis for mental function in scaphocephaly. Neurosurgery 83:476–479CrossRef Arnaud E, Renier D, Marchac D (1995) Prognosis for mental function in scaphocephaly. Neurosurgery 83:476–479CrossRef
23.
go back to reference Kapp-Simon K, Figueroa A, Jocher C, Schafer M (1993) Longitudinal assessment of mental development in infants with nonsyndromic craniosynostosis with and without cranial release and reconstruction. Plast Reconstr Surg 92:831–839, discussion 840-831PubMedCrossRef Kapp-Simon K, Figueroa A, Jocher C, Schafer M (1993) Longitudinal assessment of mental development in infants with nonsyndromic craniosynostosis with and without cranial release and reconstruction. Plast Reconstr Surg 92:831–839, discussion 840-831PubMedCrossRef
24.
go back to reference Speltz M, Endriga M, Mouradian W (1997) Presurgical and postsurgical mental and psychomotor development of infants with sagittal synostosis. Cleft Palate-Craniofacial J 34:374–379CrossRef Speltz M, Endriga M, Mouradian W (1997) Presurgical and postsurgical mental and psychomotor development of infants with sagittal synostosis. Cleft Palate-Craniofacial J 34:374–379CrossRef
25.
go back to reference Warschausky S, Angobaldo J, Kewman D, Buchman S, Muraszko K, Azengart A (2005) Early development of infants with untreated metopic craniosynostosis. Plast Reconstr Surg 115:1518–1523PubMedCrossRef Warschausky S, Angobaldo J, Kewman D, Buchman S, Muraszko K, Azengart A (2005) Early development of infants with untreated metopic craniosynostosis. Plast Reconstr Surg 115:1518–1523PubMedCrossRef
26.
go back to reference Cohen S, Cho D, Nichols S, Simms C, Cross K, Burstein F (2004) American society of maxillofacial surgeons outcome study: preoperative and postoperative neurodevelopmental findings in single-suture craniosynostosis. Plast Reconstr Surg 15:841–847, discussion 848-849CrossRef Cohen S, Cho D, Nichols S, Simms C, Cross K, Burstein F (2004) American society of maxillofacial surgeons outcome study: preoperative and postoperative neurodevelopmental findings in single-suture craniosynostosis. Plast Reconstr Surg 15:841–847, discussion 848-849CrossRef
27.
go back to reference Kapp-Simon K, Leroux B, Speltz M (2005) Multi-site study of infants with single-suture craniosynostosis: preliminary report of pre-surgery development. Cleft Palate-Craniofacial J 42:377–384CrossRef Kapp-Simon K, Leroux B, Speltz M (2005) Multi-site study of infants with single-suture craniosynostosis: preliminary report of pre-surgery development. Cleft Palate-Craniofacial J 42:377–384CrossRef
28.
go back to reference Panchal J, Amirsheybani H, Gurwitch R, Cook V, Francei P, Neas B, Levine N (2001) Neurodevelopment in children with single-suture craniosynostosis and plagiocephaly without synostosis. Plast Reconstr Surg 108:1492–1498PubMedCrossRef Panchal J, Amirsheybani H, Gurwitch R, Cook V, Francei P, Neas B, Levine N (2001) Neurodevelopment in children with single-suture craniosynostosis and plagiocephaly without synostosis. Plast Reconstr Surg 108:1492–1498PubMedCrossRef
29.
go back to reference Bellew M, Chumas P, Mueller R, Liddington M, Russell J (2005) Pre- and postoperative developmental attainment in sagittal synostosis. Arch Dis Child Apr 90:346–350CrossRef Bellew M, Chumas P, Mueller R, Liddington M, Russell J (2005) Pre- and postoperative developmental attainment in sagittal synostosis. Arch Dis Child Apr 90:346–350CrossRef
30.
go back to reference Gewalli F, Guimarães-Ferreira J, Sahlin P, Emanuelsson I, Horneman G, Stephensen H, Lauritzen C (2001) Mental development after modified pi procedure: dynamic cranioplasty for sagittal synostosis. Ann Plast Surg 46:415–420PubMedCrossRef Gewalli F, Guimarães-Ferreira J, Sahlin P, Emanuelsson I, Horneman G, Stephensen H, Lauritzen C (2001) Mental development after modified pi procedure: dynamic cranioplasty for sagittal synostosis. Ann Plast Surg 46:415–420PubMedCrossRef
31.
go back to reference Starr J, Kapp-Simon K, Cloonan Y, Collett B, Cradock M, Buono L, Cunningham M, Speltz M (2007) Presurgical and postsurgical assessment of the neurodevelopment of infants with single-suture craniosynostosis: comparison with controls. J Neurosurg 107(2 Suppl):103–110PubMed Starr J, Kapp-Simon K, Cloonan Y, Collett B, Cradock M, Buono L, Cunningham M, Speltz M (2007) Presurgical and postsurgical assessment of the neurodevelopment of infants with single-suture craniosynostosis: comparison with controls. J Neurosurg 107(2 Suppl):103–110PubMed
32.
go back to reference De Moura DR, Costa JC, Santos IS, Barros AJD, Matijasevich A, Halpern R, Dumith S, Karam S, Barros FC (2010) Risk factors for suspected developmental delay at age 2 years in a Brazilian birth cohort. Paediatr Perinat Epidemiol 24:211–221PubMedCrossRef De Moura DR, Costa JC, Santos IS, Barros AJD, Matijasevich A, Halpern R, Dumith S, Karam S, Barros FC (2010) Risk factors for suspected developmental delay at age 2 years in a Brazilian birth cohort. Paediatr Perinat Epidemiol 24:211–221PubMedCrossRef
33.
go back to reference Mushkudiani NA, Engel DC, Steyerberg EW, Butcher I, Lu J, Marmarou A, Slieker F, McHugh GS, Murray GD, Maas AIR (2007) Prognostic value of demographic characteristics in traumatic brain injury: results from The IMPACT study. J Neurotraum 24:259–269CrossRef Mushkudiani NA, Engel DC, Steyerberg EW, Butcher I, Lu J, Marmarou A, Slieker F, McHugh GS, Murray GD, Maas AIR (2007) Prognostic value of demographic characteristics in traumatic brain injury: results from The IMPACT study. J Neurotraum 24:259–269CrossRef
34.
go back to reference Kolb B, Gibb R (1999) Neuroplasticity and recovery of function after brain injury. In: Stuss G, Winocur G, Robertson I (eds) Cognitive neurorehabilitation. Cambridge University Press, New York, pp 9–25 Kolb B, Gibb R (1999) Neuroplasticity and recovery of function after brain injury. In: Stuss G, Winocur G, Robertson I (eds) Cognitive neurorehabilitation. Cambridge University Press, New York, pp 9–25
35.
go back to reference Anderson V, Pentland L (1998) Residual attention deficits following childhood head injury. Neuropsychol Rehabil 8:283–300CrossRef Anderson V, Pentland L (1998) Residual attention deficits following childhood head injury. Neuropsychol Rehabil 8:283–300CrossRef
36.
go back to reference Duchowny M (1996) Identification of surgical candidates and timing of operation: an overview. In: Wyllie E (ed) The treatment of epilepsy: principles and practice, 2nd edn. Williams & Wilkins, Baltimore, pp 967–975 Duchowny M (1996) Identification of surgical candidates and timing of operation: an overview. In: Wyllie E (ed) The treatment of epilepsy: principles and practice, 2nd edn. Williams & Wilkins, Baltimore, pp 967–975
37.
go back to reference Leventer R, Phelan E, Coleman L, Kean M, Jackson G, Harvey A (1999) Clinical and imaging features of cortical manifestations in childhood. Neurology 53:715–722PubMedCrossRef Leventer R, Phelan E, Coleman L, Kean M, Jackson G, Harvey A (1999) Clinical and imaging features of cortical manifestations in childhood. Neurology 53:715–722PubMedCrossRef
38.
go back to reference Finger S, Stein D (1982) Brain damage and recovery. Academic, New York Finger S, Stein D (1982) Brain damage and recovery. Academic, New York
39.
go back to reference Anderson V, Bond L, Catroppa C, Grimwood K, Keir E, Nolan T (1997) Childhood bacterial meningitis: impact of age at illness and medical complications on long term outcome. J Int Neuropsychol Soc 3:147–158PubMed Anderson V, Bond L, Catroppa C, Grimwood K, Keir E, Nolan T (1997) Childhood bacterial meningitis: impact of age at illness and medical complications on long term outcome. J Int Neuropsychol Soc 3:147–158PubMed
40.
go back to reference Franco S, Cornelius V, Andrews B (1992) Long-term outcome of neonatal meningitis. Am J Dis Child 146:567–571PubMed Franco S, Cornelius V, Andrews B (1992) Long-term outcome of neonatal meningitis. Am J Dis Child 146:567–571PubMed
41.
go back to reference Grimwood K, Nolan T, Bond L, Anderson V, Catroppa C, Keir E (1996) Risk factors for adverse outcomes of bacterial meningitis. J Pediatr Child Health 32:457–462CrossRef Grimwood K, Nolan T, Bond L, Anderson V, Catroppa C, Keir E (1996) Risk factors for adverse outcomes of bacterial meningitis. J Pediatr Child Health 32:457–462CrossRef
42.
go back to reference Wright M, Nolan T (1994) Impact of cyanotic heart disease on school performance. Arch Dis Child 71:64–70PubMedCrossRef Wright M, Nolan T (1994) Impact of cyanotic heart disease on school performance. Arch Dis Child 71:64–70PubMedCrossRef
43.
go back to reference Wrightson P, McGinn V, Gronwall D (1995) Mild head injury in preschool children: evidence it can be associated with persisting cognitive defect. J Neurol Neurosurg Psychiatry 59:378–380CrossRef Wrightson P, McGinn V, Gronwall D (1995) Mild head injury in preschool children: evidence it can be associated with persisting cognitive defect. J Neurol Neurosurg Psychiatry 59:378–380CrossRef
44.
go back to reference Anderson V, Smibert E, Ekert H, Godber T (1994) Intellectual, educational, and behavioural sequelae after cranial irradiation and chemotherapy. Arch Dis Child 70:476–483PubMedCrossRef Anderson V, Smibert E, Ekert H, Godber T (1994) Intellectual, educational, and behavioural sequelae after cranial irradiation and chemotherapy. Arch Dis Child 70:476–483PubMedCrossRef
45.
go back to reference Rubinstein C, Varni J, Katz E (1990) Cognitive functioning in long-term survivors of childhood leukemia: a prospective analysis. Dev Behav Pediatr 11:301–305 Rubinstein C, Varni J, Katz E (1990) Cognitive functioning in long-term survivors of childhood leukemia: a prospective analysis. Dev Behav Pediatr 11:301–305
46.
go back to reference Anderson V (1988) Recovery of function: the myth of cerebral plasticity. In: Matheson M, Newman H (eds) 13th annual brain impairment conference. Academic, Sydney, pp 223–247 Anderson V (1988) Recovery of function: the myth of cerebral plasticity. In: Matheson M, Newman H (eds) 13th annual brain impairment conference. Academic, Sydney, pp 223–247
Metadata
Title
Neurodevelopmental functioning of infants with untreated single-suture craniosynostosis during early infancy
Authors
Annette C. Da Costa
Vicki A. Anderson
Ravi Savarirayan
Jacquie A. Wrennall
David K. Chong
Anthony D. Holmes
Andrew L. Greensmith
John G. Meara
Publication date
01-06-2012
Publisher
Springer-Verlag
Published in
Child's Nervous System / Issue 6/2012
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-011-1660-1

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