Skip to main content
Top
Published in: Child's Nervous System 6/2010

01-06-2010 | Original Paper

The incidence of Chiari malformation in nonsyndromic, single suture craniosynostosis

Authors: Junnu Leikola, Virve Koljonen, Leena Valanne, Jyri Hukki

Published in: Child's Nervous System | Issue 6/2010

Login to get access

Abstract

Introduction

This study was designed to determine the incidence of Chiari malformation (CM) in nonsyndromic single suture craniosynostosis (N-SSSC).

Materials and methods

A retrospective analysis of brain magnetic resonance imaging (MRI) studies of children undergoing craniofacial surgery during 1 January, 2004–31 March, 2009 in Cleft Palate and Craniofacial Centre, Department of Plastic Surgery, Helsinki University Hospital, Helsinki, Finland, was conducted.

Results and discussion

One hundred twenty-four N-SSSC patients were imaged using brain MRI. Of these 124 patients, seven patients were diagnosed with N-SSSC with an associated CM; the incidence CM in association with N-SSSC was thus 5.6%. The average age at the time of MRI was 37.7 months. All were males, except one. Only two types of synostosis were identified in this patient cohort: synostosis involving the sagittal suture in five cases and right coronal synostosis in two cases. The CM malformations were relatively large. The tonsillar herniation varied from 6 to 12 mm (median 9 mm). All these patients were asymptomatic of their CMs. None had operation designed directly to correct the CM.

Conclusion

As a conclusion, we state that the association of CM and N-SSSC is a relatively common finding, with an incidence of 5.6% in preoperative brain MRI. The significance of finding CM in preoperative brain MRI should be assessed individually in asymptomatic patients.
Literature
1.
go back to reference Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM (1986) Significance of cerebellar tonsillar position on MR. AJNR 7:795–799PubMed Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM (1986) Significance of cerebellar tonsillar position on MR. AJNR 7:795–799PubMed
2.
go back to reference Cinalli G, Spennato P, Sainte-Rose C, Arnaud E, Aliberti F, Brunelle F, Cianciulli E, Renier D (2005) Chiari malformation in craniosynostosis. Childs Nerv Syst 21:889–901CrossRefPubMed Cinalli G, Spennato P, Sainte-Rose C, Arnaud E, Aliberti F, Brunelle F, Cianciulli E, Renier D (2005) Chiari malformation in craniosynostosis. Childs Nerv Syst 21:889–901CrossRefPubMed
3.
go back to reference Currarino G (2007) Sagittal synostosis in X-linked hypophosphatemic rickets and related diseases. Pediatr Radiol 37:805–812CrossRefPubMed Currarino G (2007) Sagittal synostosis in X-linked hypophosphatemic rickets and related diseases. Pediatr Radiol 37:805–812CrossRefPubMed
4.
go back to reference Mikulis DJ, Diaz O, Egglin TK, Sanchez R (1992) Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology 183:725–728PubMed Mikulis DJ, Diaz O, Egglin TK, Sanchez R (1992) Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology 183:725–728PubMed
5.
go back to reference Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y (1997) Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg 86:40–47CrossRefPubMed Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y (1997) Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg 86:40–47CrossRefPubMed
6.
go back to reference Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, Tamburrini G, Di Rocco C (2008) The natural history of the Chiari type I anomaly. J Neurosurg Pediatr 2:179–187CrossRefPubMed Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, Tamburrini G, Di Rocco C (2008) The natural history of the Chiari type I anomaly. J Neurosurg Pediatr 2:179–187CrossRefPubMed
7.
go back to reference Pouratian N, Sansur CA, Newman SA, Jane JA Jr, Jane JA Sr (2007) Chiari malformations in patients with uncorrected sagittal synostosis. Surg Neurol 67:422–427 discussion 427–428CrossRefPubMed Pouratian N, Sansur CA, Newman SA, Jane JA Jr, Jane JA Sr (2007) Chiari malformations in patients with uncorrected sagittal synostosis. Surg Neurol 67:422–427 discussion 427–428CrossRefPubMed
8.
go back to reference Raybaud C, Di Rocco C (2007) Brain malformation in syndromic craniosynostoses, a primary disorder of white matter: a review. Childs Nerv Syst 23:1379–1388CrossRefPubMed Raybaud C, Di Rocco C (2007) Brain malformation in syndromic craniosynostoses, a primary disorder of white matter: a review. Childs Nerv Syst 23:1379–1388CrossRefPubMed
9.
go back to reference Renier D, Cinalli G, Lajeunie E, Arnaud E, Marchac D (1997) Oxycephaly, a severe craniosynostosis. Apropos of a series of 129 cases. Arch Pediatr 4:722–729CrossRefPubMed Renier D, Cinalli G, Lajeunie E, Arnaud E, Marchac D (1997) Oxycephaly, a severe craniosynostosis. Apropos of a series of 129 cases. Arch Pediatr 4:722–729CrossRefPubMed
10.
go back to reference Stovner LJ, Bergan U, Nilsen G, Sjaastad O (1993) Posterior cranial fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentation. Neuroradiology 35:113–118CrossRefPubMed Stovner LJ, Bergan U, Nilsen G, Sjaastad O (1993) Posterior cranial fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentation. Neuroradiology 35:113–118CrossRefPubMed
11.
go back to reference Tubbs RS, Elton S, Blount JP, Oakes WJ (2001) Preliminary observations on the association between simple metopic ridging in children without trigonocephaly and the Chiari I malformation. Pediatr Neurosurg 35:136–139CrossRefPubMed Tubbs RS, Elton S, Blount JP, Oakes WJ (2001) Preliminary observations on the association between simple metopic ridging in children without trigonocephaly and the Chiari I malformation. Pediatr Neurosurg 35:136–139CrossRefPubMed
12.
go back to reference Tubbs RS, Webb D, Abdullatif H, Conklin M, Doyle S, Oakes WJ (2004) Posterior cranial fossa volume in patients with rickets: insights into the increased occurrence of Chiari I malformation in metabolic bone disease. Neurosurgery 55:380–383 discussion 383–384CrossRefPubMed Tubbs RS, Webb D, Abdullatif H, Conklin M, Doyle S, Oakes WJ (2004) Posterior cranial fossa volume in patients with rickets: insights into the increased occurrence of Chiari I malformation in metabolic bone disease. Neurosurgery 55:380–383 discussion 383–384CrossRefPubMed
Metadata
Title
The incidence of Chiari malformation in nonsyndromic, single suture craniosynostosis
Authors
Junnu Leikola
Virve Koljonen
Leena Valanne
Jyri Hukki
Publication date
01-06-2010
Publisher
Springer-Verlag
Published in
Child's Nervous System / Issue 6/2010
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-009-1044-y

Other articles of this Issue 6/2010

Child's Nervous System 6/2010 Go to the issue