Hydrocephalus and craniosynostosis

J Neurosurg. 1998 Feb;88(2):209-14. doi: 10.3171/jns.1998.88.2.0209.


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.

MeSH terms

  • Acrocephalosyndactylia / complications
  • Acrocephalosyndactylia / diagnostic imaging
  • Acrocephalosyndactylia / surgery
  • Brain / diagnostic imaging
  • Child, Preschool
  • Craniofacial Dysostosis / complications
  • Craniofacial Dysostosis / diagnostic imaging
  • Craniofacial Dysostosis / surgery
  • Craniosynostoses / complications*
  • Craniosynostoses / diagnostic imaging
  • Craniosynostoses / surgery
  • Disease Progression
  • Humans
  • Hydrocephalus / etiology*
  • Hydrocephalus / physiopathology
  • Hydrocephalus / surgery
  • Infant
  • Retrospective Studies
  • Syndrome
  • Tomography, X-Ray Computed