Neurosurgical Investigation of Craniofacial Malformations

Deformational plagiocephaly and craniosynostosis are two of the most common neonatal cranial head shape anomalies. Craniosynostosis is the most common entity involving neurosurgical diagnostics and therapy in the treatment of patients with craniofacial malformations. Craniosynostosis is characterized by the premature fusion of one up to all of the cranial sutures, resulting in skull deformation, impairment of brain development, increased intracranial pressure, amaurosis, and cognitive dysfunction. It usually occurs as an isolated condition but may also be associated with other malformations as part of complex syndromes. Whereas single-suture craniosynostosis is simple to treat, complex or syndromic craniosynostosis is challenging for the whole team. There is a significant onset of Chiari malformation including syringomyelia in complex cases. In this chapter, we point out the most important clinical and pathophysiological features and diagnostic tests in syndromic and nonsyndromic cases to ensure diagnosis and to prepare interdisciplinary surgical procedures.