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Published in: Acta Neurochirurgica 10/2012

01-10-2012 | Clinical Article

Chiari malformation in nonsyndromal single craniosynostosis—Much Ado about Nothing?

Authors: M. Engel, G. Castrillón-Oberndorfer, J. Hoffmann, B. Orakcioglu, S. Rohde, R. Seeberger, C. Freudlsperger

Published in: Acta Neurochirurgica | Issue 10/2012

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Abstract

Background

The significance of Chiari malformation in nonsyndromal-isolated craniosynostosis is still not well documented. Hence, in the present study we investigated the incidence of Chiari malformation in a larger series of patients with nonsyndromic-isolated single-suture craniosynostosis over a 9-year period using preoperative magnetic resonance imaging (MRI).

Methods

Of 215 children who had undergone surgery for nonsyndromic-isolated craniosynostosis, 89 cases (41.4 %) had MRI prior to surgery. All MRIs were screened for Chiari malformation.

Results

Only one patient (1.1 %) with isolated lambdoid synostosis showed Chiari malformation preoperatively, which was defined as a cerebellar tonsillar descent greater than 5 mm below the foramen magnum. However, no clinical symptoms were associated with Chiari malformation in this patient.

Conclusions

As Chiari malformation is more likely to be associated with syndromic craniosynostosis, nonsyndromic bilateral coronal synostosis, or synostosis of the lambdoid suture, a general use of MRI as a screening tool for Chiari malformation should not be recommended for patients with nonsyndromic-isolated craniosynostosis who lack clinical symptoms.
Literature
1.
go back to reference Cinalli G, Renier D, Sebag G, Sainte-Rose C, Arnaud E, Pierre-Kahn A (1995) Chronic tonsillar herniation in Crouzon’s and Apert’s syndromes: the role of premature synostosis of the lambdoid suture. J Neurosurg 83:575–582PubMedCrossRef Cinalli G, Renier D, Sebag G, Sainte-Rose C, Arnaud E, Pierre-Kahn A (1995) Chronic tonsillar herniation in Crouzon’s and Apert’s syndromes: the role of premature synostosis of the lambdoid suture. J Neurosurg 83:575–582PubMedCrossRef
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 21:889–901PubMedCrossRef 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 21:889–901PubMedCrossRef
3.
go back to reference Fearon JA, Rhodes J (2009) Pfeiffer syndrome: a treatment evaluation. Plast Reconstr Surg 123:1560–1569PubMedCrossRef Fearon JA, Rhodes J (2009) Pfeiffer syndrome: a treatment evaluation. Plast Reconstr Surg 123:1560–1569PubMedCrossRef
4.
go back to reference Francis PM, Beals S, Rekate HL, Pittman HW, Manwaring K, Reiff J (1992) Chronic tonsillar herniation and Crouzon’s syndrome. Pediatr Neurosurg 18:202–206PubMedCrossRef Francis PM, Beals S, Rekate HL, Pittman HW, Manwaring K, Reiff J (1992) Chronic tonsillar herniation and Crouzon’s syndrome. Pediatr Neurosurg 18:202–206PubMedCrossRef
5.
go back to reference Frim DM, Jones D, Goumnerova L (1990) Development of symptomatic Chiari malformation in a child with craniofacial dysmorphism. Pediatr Neurosurg 16:228–231PubMedCrossRef Frim DM, Jones D, Goumnerova L (1990) Development of symptomatic Chiari malformation in a child with craniofacial dysmorphism. Pediatr Neurosurg 16:228–231PubMedCrossRef
6.
go back to reference Kotrikova B, Krempien R, Freier K, Mühling J (2007) Diagnostic imaging in the management of craniosynostoses. Eur Radiol 17:1968–1978PubMedCrossRef Kotrikova B, Krempien R, Freier K, Mühling J (2007) Diagnostic imaging in the management of craniosynostoses. Eur Radiol 17:1968–1978PubMedCrossRef
7.
go back to reference Leikola J, Koljonen V, Valanne L, Hukki J (2010) The incidence of Chiari malformation in nonsyndromic, single suture craniosynostosis. Childs Nerv Syst 26:771–774PubMedCrossRef Leikola J, Koljonen V, Valanne L, Hukki J (2010) The incidence of Chiari malformation in nonsyndromic, single suture craniosynostosis. Childs Nerv Syst 26:771–774PubMedCrossRef
8.
go back to reference Pouratian N, Sansur CA, Newman SA, Jane JA (2007) Chiari malformations in patients with uncorrected sagittal synostosis. Surg Neurol 67:422–427, discussion 427–428PubMedCrossRef Pouratian N, Sansur CA, Newman SA, Jane JA (2007) Chiari malformations in patients with uncorrected sagittal synostosis. Surg Neurol 67:422–427, discussion 427–428PubMedCrossRef
9.
go back to reference Saldino RM, Steinbach HL, Epstein CJ (1972) Familial acrocephalosyndactyly (Pfeiffer syndrome). Am J Roentgenol Radium Ther Nucl Med 116:609–622PubMed Saldino RM, Steinbach HL, Epstein CJ (1972) Familial acrocephalosyndactyly (Pfeiffer syndrome). Am J Roentgenol Radium Ther Nucl Med 116:609–622PubMed
10.
go back to reference Sgouros S, Goldin JH, Hockley AD, Wake MJ (1996) Posterior skull surgery in craniosynostosis. Childs Nerv Syst 12:727–733PubMedCrossRef Sgouros S, Goldin JH, Hockley AD, Wake MJ (1996) Posterior skull surgery in craniosynostosis. Childs Nerv Syst 12:727–733PubMedCrossRef
11.
go back to reference Strahle J, Muraszko KM, Buchman SR, Kapurch J, Garton HJ, Maher CO (2011) Chiari malformation associated with craniosynostosis. Neurosurg Focus 31:E2PubMedCrossRef Strahle J, Muraszko KM, Buchman SR, Kapurch J, Garton HJ, Maher CO (2011) Chiari malformation associated with craniosynostosis. Neurosurg Focus 31:E2PubMedCrossRef
12.
go back to reference Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO (2011) Natural history of Chiari malformation Type I following decision for conservative treatment. J Neurosurg Pediatr 8:214–221PubMedCrossRef Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO (2011) Natural history of Chiari malformation Type I following decision for conservative treatment. J Neurosurg Pediatr 8:214–221PubMedCrossRef
13.
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 35:136–139PubMedCrossRef 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 35:136–139PubMedCrossRef
14.
go back to reference Wall SA, Goldin JH, Hockley AD, Wake MJ, Poole MD, Briggs M (1994) Fronto-orbital re-operation in craniosynostosis. Br J Plast Surg 47:180–184PubMedCrossRef Wall SA, Goldin JH, Hockley AD, Wake MJ, Poole MD, Briggs M (1994) Fronto-orbital re-operation in craniosynostosis. Br J Plast Surg 47:180–184PubMedCrossRef
Metadata
Title
Chiari malformation in nonsyndromal single craniosynostosis—Much Ado about Nothing?
Authors
M. Engel
G. Castrillón-Oberndorfer
J. Hoffmann
B. Orakcioglu
S. Rohde
R. Seeberger
C. Freudlsperger
Publication date
01-10-2012
Publisher
Springer Vienna
Published in
Acta Neurochirurgica / Issue 10/2012
Print ISSN: 0001-6268
Electronic ISSN: 0942-0940
DOI
https://doi.org/10.1007/s00701-012-1439-5

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