PhD defence S.D.G. (Stephanie) van de Beeten

Below is a brief summary of the dissertation: 

Treatment of patients with craniosynostosis is aimed at preventing and treating intracranial hypertension (ICH). Untreated ICH can lead to behavioral and developmental problems and blindness. However, there is no consensus on the optimal diagnostic method for detecting ICH. The most accurate method currently is intracranial pressure measurement, which is invasive and therefore not suitable for routine screening. This dissertation focuses on screening for ICH and evaluates the use of various risk factors (such as craniocerebral disproportion) and indirect indicators of ICH (such as headache and papilledema on funduscopy/OCT) in the clinic.
This dissertation shows, among other things, that occipital-frontal head circumference (OFC) measurements in scaphocephaly patients have a good diagnostic accuracy to detect ICH.  Additionally, headaches appear to be related to signs of ICH. Screening is further optimized by adding OCT in combination with funduscopy and OFC measurement. Based on the results, a flowchart has been developed for clinical application. OCT can also play a role in evaluating the effects of ICH on the retina. Irreversible vision loss is a feared consequence of prolonged ICH, which is presumably attributable to the loss of retinal axons due to prolonged papilledema. This can be captured by detecting a thinner retina at the papilla or macula using OCT. In syndromic and complex craniosynostosis, which underwent prophylactic surgery, retinal thinning at the papilla was not frequently found and was not related to vision loss