title: Contemporary occurrence of hydrocephalus and Chiari I malformation in sagittal craniosynostosis. Case report and review of the literature.
abstract: Chiari malformation type I (CM-I) and hydrocephalus are often associated with complex craniosynostosis. On the contrary, their simultaneous occurrence in monosutural synostosis is extremely rare. The pathophysiological hypothesis is that they may alter posterior fossa growth and lead to cerebellar tonsil herniation also without skull base primary involvement. Hydrocephalus is multifactorial and may be secondary to fourth ventricle outlet obstruction. The management of these cases is quite complex and not well defined. Cranial vault remodeling should be the only treatment when CM-I is asymptomatic and not related to syringomyelia. Suboccipital decompression should be reserved only in complicated CM-I, usually as a second surgical step following the correction of the supratentorial deformity. In our opinion, the associated hydrocephalus should be treated first in order to normalize intracranial hypertension before opening the cranial sutures. We report the case of a 26-month-old child that presented with sagittal craniosynostosis, hydrocephalus, and CM-I. He was managed by performing endoscopic third ventriculostomy (ETV) first and cranial vault remodeling thereafter. Clinico-radiological outcome was very satisfying. Concerning literature is reviewed; physiopathology and surgical management are discussed.
authors.affiliation: Department of Neurosciences and Odontostomatological and Reproductive Sciences, Neurosurgical Clinic, Federico II University Naples, Naples, Italy. fgsgulo@live.com.
authors.forename: Francesco Giovanni
authors.identifier: http://orcid.org/0000-0003-2721-4052
authors.initials: FG
authors.lastname: Sgulò
authors.affiliation: Department of Pediatric Neurosurgery, Santobono-Pausilipon Pediatric Hospital, Naples, Italy.
authors.forename: Pietro
authors.initials: P
authors.lastname: Spennato
authors.affiliation: Department of Pediatric Neurosurgery, Santobono-Pausilipon Pediatric Hospital, Naples, Italy.
authors.forename: Ferdinando
authors.initials: F
authors.lastname: Aliberti
authors.affiliation: Department of Pediatric Neurosurgery, Santobono-Pausilipon Pediatric Hospital, Naples, Italy.
authors.forename: Giuliana
authors.initials: G
authors.lastname: Di Martino
authors.affiliation: Department of Pediatric Neuroradiology, Santobono-Pausilipon Pediatric Hospital, Naples, Italy.
authors.forename: Daniele
authors.initials: D
authors.lastname: Cascone
authors.affiliation: Department of Pediatric Neurosurgery, Santobono-Pausilipon Pediatric Hospital, Naples, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Cinalli
mesh_terms: D001139:Arnold-Chiari Malformation / Q000150:complications* / Q000601:surgery; D002675:Child, Preschool; D003398:Craniosynostoses / Q000150:complications* / Q000601:surgery; D006801:Humans; D006849:Hydrocephalus / Q000150:complications* / Q000601:surgery; D008297:Male; D014696:Ventriculostomy
keywords: Chiari malformation type I (CM-I); Craniosynostosis; Hydrocephalus