Cited by 224Craniofacial Disorders and TreatmentsCerebrospinal fluid and hydrocephalusSpinal Dysraphism and Malformations

Hydrocephalus and craniosynostosis

Cinalli, Giuseppe, Sainte-Rose, Christian, Kollar, Eve Marie, Zerah, Michel, Brunelle, Francis, Chumas, Paul, Arnaud, Eric, Marchac, Daniel +2 more·1998·Journal of neurosurgery

Abstract

OBJECT: A retrospective study of 1727 cases of craniosynostosis was undertaken to determine the interrelationship between abnormal cerebrospinal fluid (CSF) hydrodynamics and craniosynostosis. METHODS: The patients were divided into two groups: nonsyndromic craniosynostosis and syndromic craniosynostosis. Cases of occipital plagiocephaly without suture synostosis and cases of shunt-induced craniosynostosis were excluded from the study. The majority of patients (1297) were treated surgically for their cranial deformity; 95% of these patients had a postoperative follow-up review period lasting 5 years. Clinical and radiographic charts covering the time from presentation through the follow-up period were reviewed. CONCLUSIONS: Abnormal intracranial CSF hydrodynamics was found in 8.1% of the patients (3.4% of whom had received shunts and 4.5% of whom had not). Three types of CSF hydrodynamic disturbance were observed: progressive hydrocephalus with ventricular dilation, nonprogressive ventriculomegaly, and dilation of the subarachnoid spaces. Hydrocephalus occurred much more frequently in patients with syndromic craniosynostosis (12.1%) than in those with isolated craniosynostosis (0.3%). In fact, patients with kleeblattschädel exhibited hydrocephalus as a constant feature and patients with Crouzon's syndrome were far more likely to have hydrocephalus than those with other syndromes. In Apert's syndrome, ventricular dilation occurred very frequently, but it was almost always nonprogressive in nature. In most cases of syndromic craniosynostosis, venous sinus obstruction and/or chronic tonsillar herniation were found. Their role in the pathophysiology of hydrocephalus in craniosynostosis is discussed.

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Crossref · 10.3171/jns.1998.88.2.0209

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Authors
Cinalli, G., Sainte-Rose, C., Kollar, E. M., Zerah, M., Brunelle, F., Chumas, P., Arnaud, E., Marchac, D., Pierre-Kahn, A., & Renier, D.
Affiliations
Hôpital Necker-Enfants Malades (FR)
Venue
Journal of neurosurgery
Published
1998-02
Volume
88
Issue
2
Pages
209-214
ISSN
0022-3085 (ISSN-L)
OpenAlex
W2155932531
Cited by
224
References
30
Citations by year
2026: 7 · 2025: 6 · 2024: 22 · 2023: 11 · 2022: 12 · 2021: 17 · 2020: 11 · 2019: 21 · 2018: 11 · 2017: 15 · 2016: 7 · 2015: 8 · 2014: 2 · 2013: 5 · 2012: 11
FWCI
1.33
Type
journal article
Language
en
Open access
closed
Locations
  • Journal of neurosurgery (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Craniofacial Disorders and Treatments
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Cerebrospinal fluid and hydrocephalus
  • Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Spinal Dysraphism and Malformations
Keywords
craniosynostosis, hydrocephalus, cerebrospinal fluid dynamics, syndromic craniosynostosis, nonsyndromic craniosynostosis, ventriculomegaly, Crouzon syndrome, Apert syndrome, cerebellar tonsil herniation, cranial deformity surgery
MeSH terms
Acrocephalosyndactylia, Brain, Child, Preschool, Craniofacial Dysostosis, Craniosynostoses, Humans, Hydrocephalus, Infant, Retrospective Studies, Syndrome, Tomography, X-Ray Computed, Disease Progression

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