15 Introduction any ventricular dilatation, what could result in high CSF pressure with normal or even small ventricles. 14 If the skull then is opened by a vault expansion, a ‘pseudo-tumour’ /hydrocephalus of venous origin could occur postoperatively. Prevalence of Intracranial hypertension Isolated craniosynostosis Based on invasive ICP measurement or the presence of papilledema, the prevalence of ICH in isolated synostosis has been described.52-59 The prevalence of preoperatively increased ICP in sagittal suture synostosis probably ranges from 2.5 to 14% and increases with age. The prevalence of preoperatively increased ICP in metopic suture synostosis probably ranges from 2 to 8%, and for isolated coronal suture synostosis around 16%.8, 52-58, 60 The prevalence of increased ICP during follow-up after cranial correction in isolated craniosynostosis probably varies from 2 to 9% for sagittal suture synostosis, and around 1.5% for metopic suture synostosis.57, 58, 60-62 Corresponding figures for isolated coronal suture synostosis are not known. Syndromic craniosynostosis For syndromic craniosynostosis the following studies defined ICH by invasive preoperative ICP measurement, the presence of papilledema, an abnormal VEP, a deflecting head circumference curve or extensive endocortical erosion on CT images. 53, 63-68 One study uses a set of symptoms to determine increased ICP, namely papilledema, an abnormal VEP scan, a tense fontanel, progressive ventriculomegaly and invasive ICP measurement. 69 The prevalence of increased ICP in syndromic craniosynostosis before cranial surgery is likely to be 9 to 83% for Apert, 53 to 64% for Crouzon, 19 to 43% for Saethre-Chotzen and 0 to 4% for Muenke syndrome.64, 65, 67-69 The prevalence of increased ICP in syndromic craniosynostosis after cranial surgery is likely to be 35 to 45% for Apert, 20 to 47% for Crouzon, 17 to 42% for Saethre-Chotzen and 0 to 5% for Muenke syndrome, 58 to 67% for multisuture craniosynostosis and around 31% for bicoronal synostosis.53, 63-65, 68, 69 Signs and screening on Intracranial Hypertension Because ICH could impair cognitive development, cause behavioural issues, and visual loss by damaging the optic nerves, it is important to recognize signs of ICH as early as possible.1-3 4 Raised ICP is defined as increased pressure on invasive measurement, but to avoid invasive measurement a set of indicators is used to assess the risk of ICH, namely hydrocephalus, papilledema, and indirect signs on radiological images.70 At the Erasmus MC depending on suture involvement and syndromic/nonsyndromic synostosis, head circumference, fundoscopy or optical coherence tomography (OCT) are used to screen for ICH. 1
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