2016
DOI: 10.3171/2015.6.peds15177
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Raised intracranial pressure in Crouzon syndrome: incidence, causes, and management

Abstract: OBJECT Patients with Crouzon syndrome (CS) are at risk for developing raised intracranial pressure (ICP), which has the potential to impair both vision and neurocognitive development. For this reason, some experts recommend early prophylactic cranial vault expansion on the basis that if ICP is not currently raised, it is likely to become so. The aim of this study was to examine the justification for such a policy. This was done by analyzing the incidence, causes, and… Show more

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Cited by 47 publications
(38 citation statements)
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“…Craniosynostosis is defined as the premature fusion of skull sutures and can cause cranial deformities and functional problems including intracranial hypertension (IH), which in turn may cause ophthalmic and neurological deficits. Syndromic forms, such as Crouzon syndrome (CS) and Apert syndrome (AS), are associated with variable phenotypes but multisutural craniosynostosis and IH are much more common than in single-suture disease [1,2].…”
Section: Introductionmentioning
confidence: 99%
“…Craniosynostosis is defined as the premature fusion of skull sutures and can cause cranial deformities and functional problems including intracranial hypertension (IH), which in turn may cause ophthalmic and neurological deficits. Syndromic forms, such as Crouzon syndrome (CS) and Apert syndrome (AS), are associated with variable phenotypes but multisutural craniosynostosis and IH are much more common than in single-suture disease [1,2].…”
Section: Introductionmentioning
confidence: 99%
“…Multiple forms of craniosynostosis, a skull malformation caused by abnormal development or premature fusion of the cranial sutures, put patients at risk for elevated ICP (Abu-Sittah et al, 2016; Twigg and Wilkie, 2015). Elevated ICP secondary to craniosynostosis is often assumed to result from a small fused skull (Johnson and Wilkie, 2011), but patients can have chronic ICP after skull expansion, and evidence suggests their elevated ICP may result, at least in part, from venous malformations (Hayward, 2005; Liesegang, 2001; Stevens et al, 2007).…”
Section: Introductionmentioning
confidence: 99%
“…In a study performed by Abu-Sittah et al, patients nearly 50% of patients had 1-2 episodes of raised ICP and they recommended regular ophthalmologic and neurological management until age of eight years after which decision can be made about whether patients need cranial vault expansion or not depending on the degree of clinical improvement [11].…”
Section: Discussionmentioning
confidence: 99%