17 Introduction lambdoid sutures, an occipital vault expansion is typically performed. Nevertheless, in case of severe exorbitism, visual loss and/or severe OSA, a monobloc advancement with distraction should be performed as first procedure. If there is proof of elevated intracranial pressure, surgery will be performed at an earlier stage in all patients. Treatment focuses on the cause which appears to be clinically the major contributor to the increase in intracranial pressure. As described above, causes for syndromic patients could be a too small intracranial volume, moderate to severe obstructive sleep apnea, hydrocephalus and venous intracranial hypertension or a combination of these factors.4, 23, 77 Cognitive outcome sCs patients are at risk of developing intellectual disabilities and problems in behavioral and emotional function. Most patients with Crouzon-Pfeiffer and Saethre-Chotzen have a long-term intellectual outcome within the normal limits, while patients with Apert Syndrome have typically an IQ below the norm.68 In addition, a large group of patients within all 3 syndromes has an IQ that is 2SD or more below the normal limits. This means that a large proportion of patients cannot work and live independently. Trigonocephaly patients are at risk of developing mental deficiencies/disorders, behavioral problems, delays in speech and language.78, 79 However, preoperatively less than 2% of the trigonocephaly patients have papilledema as a sign of intracranial hypertension.59 Whether these derangements are caused by a primary disturbances in brain development or arise secondarily due to the synostosis is unknown.5 Is this neurocognitive disorder a consequence of mechanical distortion of the brain due to abnormal shape, ventriculomegaly and/or cerebellar tonsillar herniation, or does this finding reflect an intrinsic inborn brain problem? 80-85 While the most important aim of surgical treatment is to strive for best possible neurocognitive outcome by treating and reducing the risk of developing ICH, it remains unknown what the added value of surgery is in particularly trigonocephaly with respect to future brain development. To answer the questions in this thesis regarding to the intrinsic brain abnormalities and the potential effect of ICH, MRI brain imaging techniques have been used in both non-operated as operated craniosynostosis patients to investigate cerebral blood flow, microstructures, and cortical thickness. In the next chapter the used MRI brain imaging techniques are set out. MRI physics used in the upcoming studies Magnetic resonance imaging (MRI) is an imaging technique which can be used as a tool to image and investigate body regions or tissues, including the brain. MRI scanners 1
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