Chiari malformation type I and craniosynostosis in adults: Retrospective analysis over a 10 year period in a tertiary care Centre.

Abstract

Background: Chiari malformation type 1 (CM1) pathophysiology remains uncertain. One theory involves small posterior fossa volumes leading to tonsillar herniation. Craniosynostosis causes suture fusion, which can limit skull growth, and has been investigated in paediatric CM1 cohorts. We aimed to identify the prevalence of concurrent craniosynostosis in adult CM1 patients.

Methods: Surgically managed CM1 patients were identified from a pre-existing Edinburgh neurosurgical centres database spanning 10 years. Patients aged 16 years of age or older had their case notes reviewed retrospectively. Cross sectional imaging identified and classified craniosynostosis, tonsillar herniation and the presence of hydrocephalus or syrinxes.

Results: 35 cases met inclusion criteria. Seven cases (20%) had craniosynostosis, all of which were sagittal synostosis. There was no significant association between the presence of craniosynostosis and the degree of tonsillar herniation, presence of hydrocephalus or syrinx formation.

Conclusion: This is the first study to evaluate the prevalence of craniosynostosis in a cohort of surgically managed adult CM1 patients. Future studies should potentially investigate whether conservatively managed adult CM1 patients have similar rates of craniosynostosis and also lack significant associations to known CM1-related complications.