Raised intracranial pressure in Crouzon syndrome: incidence, causes, and management

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 subsequent risk of recurrence in a series of patients with CS, in whom raised ICP was treated only after it had been diagnosed. METHODS This study was a retrospective review of the medical records and imaging data of patients with a clinical diagnosis of CS. RESULTS There were 49 patients in the study, of whom 30 (61.2%) developed at least 1 episode of raised ICP. First episodes occurred at an average age of 1.42 years and were attributable to craniocerebral disproportion/venous hypertension (19 patients), hydrocephalus (8 patients), and airway obstruction (3 patients). They were managed, respectively, by vault expansion, ventriculoperitoneal shunt insertion, and airway improvement. Fourteen of the 30 patients developed a second episode of raised ICP an average of 1.42 years after treatment for their initial episode, and 3 patients developed a third episode an average of 3.15 years after that. Causes of subsequent episodes of raised ICP often differed from previous episodes and required different management. Patients who were < 1 year old when the first episode was diagnosed were at increased risk of recurrence. CONCLUSIONS Although the incidence of raised ICP in CS is high, it did not occur in nearly 40% of children during the course of this study. The several possible causes of CS require different management and may vary from episode to episode. The authors recommend an expectant policy toward these children with careful clinical, ophthalmological, respiratory, and radiological monitoring for raised ICP, reserving intervention for when it has been detected and the appropriate treatment can be initiated.