Level 2

For patients with Saethre-Chotzen syndrome, surgical treatment is indicated for both the cranial deformity and the risk of elevated ICP, given the prevalence of elevated ICP of 19–35% prior to cranial expansion around the age of 12 months. For patients with multisuture craniosynostosis, surgical treatment is indicated for both cranial deformity and the risk of increased ICP, given the prevalence of increased ICP of 58–67% prior to cranial expansion around the age of 12 months. For patients with Muenke syndrome, surgical treatment is mainly indicated for correction of the cranial deformity, given the low prevalence of elevated CPI of 0–4% prior to cranial expansion around the age of 12 months. For patients with Saethre-Chotzen and Muenke syndrome and multisuture craniosynostosis, it is unknown whether there is a clinically relevant difference in prevalence of elevated ICP during a 5-year follow-up between a routine cranial expansion in the first year of life and treatment once elevated ICP has been diagnosed. B Kress et al, 2008; De Jong et al, 2010; Thompson 1995, Renier 2000.