16 Chapter 1 On radiologic images intrinsic pressure from the brain could be seen by resorption of the bone, resulting in scalloping of the inner cortex and ultimately leading to full thickness defects. Also, herniation of the cerebellar tonsils through the foramen magnum and prominent venous collaterals could arise. At last, a decrease in central and peripheral CSF spaces could be seen as a sign of intrinsic pressure. Figure 3 shows an radiologic images of each of these examples. Figure 3. a.) scalloping skull b.) tonsillar herniation c.) venous collaterals. D) decrease of CSF central and peripheral Treatment To prevent/treat ICH is the main goal of surgical intervention in craniosynostosis.53, 71, 72 The timing of surgery is therefore diagnosis dependent. At the Erasmus MC, patients are surgically treated within their first year of life. Within our clinical protocol patients with Apert and Crouzon-Pfeiffer will be surgically treated by a vault expansion at the age of 6 months. An occipital expansion with springs is the primary choice because it allows to maximally increase the ICV and preserves the facial profile which facilitates a monobloc advancement, Le Fort III or facial bipartition in a later stage.73 In patients with severe exorbitism, a monobloc advancement with distraction is considered as first cranial vault expansion. In Saethre Chotzen and Muenke patients, retrusion of the orbital bar is a main clinical feature and therefore a fronto-orbital advancement will be performed in their first year of life: Saethre-Chotzen between 6-9 months of age, and Muenke between 9-12 months of age. Muenke patients have a low risk of developing ICH and therefore a better esthetical outcome of the facial appearance will be when operated at a later age.74-76 Occasionally Saethre-Chotzen patients need an additional vault expansion such as an occipital expansion or biparietal widening. In patients with complex craniosynostosis the choice of the procedure depends on the sutures that are involved. If coronal sutures have closed prematurely, a fronto-orbital advancement is the procedure of first choice, while in patients with involvement of
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