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Contemporary occurrence of hydrocephalus and Chiari I malformation in sagittal craniosynostosis. Case report and review of the literature

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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.

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Authors and Affiliations

  1. Department of Neurosciences and Odontostomatological and Reproductive Sciences, Neurosurgical Clinic, Federico II University Naples, Naples, Italy

    Francesco Giovanni Sgulò

  2. Department of Pediatric Neurosurgery, Santobono-Pausilipon Pediatric Hospital, Naples, Italy

    Pietro Spennato, Ferdinando Aliberti, Giuliana Di Martino & Giuseppe Cinalli

  3. Department of Pediatric Neuroradiology, Santobono-Pausilipon Pediatric Hospital, Naples, Italy

    Daniele Cascone

Authors
  1. Francesco Giovanni Sgulò
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  2. Pietro Spennato
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  3. Ferdinando Aliberti
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  4. Giuliana Di Martino
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  5. Daniele Cascone
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  6. Giuseppe Cinalli
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Correspondence to Francesco Giovanni Sgulò.

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Sgulò, F.G., Spennato, P., Aliberti, F. et al. Contemporary occurrence of hydrocephalus and Chiari I malformation in sagittal craniosynostosis. Case report and review of the literature. Childs Nerv Syst 33, 187–192 (2017). https://doi.org/10.1007/s00381-016-3189-9

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  • DOI: https://doi.org/10.1007/s00381-016-3189-9

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