Intracranial hypertension in syndromic craniosynostosis: Prevalence, detection, pathophysiology and treatment

This thesis highlights several aspects regarding the clinical course and treatment of syndromic craniosynostosis. First, we evaluate the prevalence of (syndromic) craniosynostosis: how many children are born with this rare condition in the Netherlands? Thereafter, we focus on the detection of (risk factors for) elevated intracranial pressure: can we use the head circumference as a reliable indicator of the intracranial volume? Can we improve the detection of intracranial hypertension by adding retina scans to the follow-up protocol? Also, the consequences of raised intracranial pressure are assessed: do we see changes in brain cortical thickness? Or in the retinal thickness and/or visual acuity? Finally, we evaluated the treatment protocol for Muenke and Saehtre-Chotzen syndrome: how do we prevent and treat raised intracranial pressure in these syndromes? Should we focus on intracranial hypertension only?