Practical Algorithm for the management of multisutural craniosynostosis with associated Chiari malformation and/or hydrocephalus.

The association between multisutural craniosynostosis with Chiari malformation (CM), venous hypertension and hydrocephalus is widely described in the literature, especially in children with pediatric craniofacial syndromes. Some efforts have been done in the last years to understand the complex pathogenetic mechanisms underlying this association and several theories have been proposed. However, whatever the pathophysiological mechanism, it is still unclear what is the best management and treatment of CM and hydrocephalus in multisutural craniosynostosis patients. The aim of this study is to report our twenty-five years'experience in treating pediatric patients affected by these rare pathologies, in order to propose a simple and effective therapeutic flow-chart for their management. We collected data of each patient that underwent a cranial vault remodelling for a complex craniosynostosis in our institution in the last 25 years. We recorded data concerning type of craniosynostosis and craniofacial syndromes, presence of ventriculomegaly and Chiari Malformation (CM) at presentation and clinical and radiological follow-up. Therefore, we evaluated the final outcomes (improved, stable, deteriorated) of these patients and created a practical flow-chart that could help physicians to choose the best surgical treatment when different pathological conditions, as CM I or hydrocephalus, affect complex craniosynostosis children. Despite the proposed algorithm comes from a clinical experience on 85% successfully treated patients with multiple craniosynostosis, more extensive and deep studies are needed to better understand CM and hydrocephalus development in such conditions.