Giuseppe Cinalli, Pietro Spennato, Christian Sainte-Rose, Eric Arnaud, Ferdinando Aliberti, Francis Brunelle, Emilio Cianciulli, Dominique Renier
INTRODUCTION: Chiari malformation (CM) is a frequent finding in multisutural and syndromic craniosynostosis, occurring in 70% of patients with Crouzon's syndrome, 75% with oxycephaly, 50% with Pfeiffer's syndrome and 100% with the Kleeblattschädel deformity. The pathogenesis of this condition and rationale for treatment are still controversial. DISCUSSION: Since its first description in 1972, several factors have been cited to play a role in inducing CM. In the light of recent publications, the roles of premature fusion of cranial vault and cranial base sutures, of congenital anomalies of the cerebellum and brain stem, of raised intracranial pressure, of venous hypertension and of hydrocephalus are reviewed...
October 2005: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery