The incidence of Chiari malformation in nonsyndromic, single suture craniosynostosis

Junnu Leikola, Virve Koljonen, Leena Valanne, Jyri Hukki
Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery 2010, 26 (6): 771-4

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.

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