Craniofaciosynostosis

Craniosynostosis is a condition in which there is a premature fusion of one or more sutures of the skull with a modification of cranial growth and an abnormal skull shape. The shape is specific to the suture involved so the diagnosis is essentially clinical. The genetic causes remain largely unknown. Molecular researches have identified several mutations. Complications can result to the deformation of the skull but also abnormal facial morphology. Cerebellar tonsillar prolapse, hydrocephalus, intracranial hypertension, insufficient eye protection, respiratory obstructions, and orthodontic problems are the most common. Several factors play a role in the surgical indications. The aim of the surgery is functional and cosmetic. Techniques have evolved from simple strip to total calvarial and supraorbital remodelling, osteogenic bone distraction, and endoscopic procedures. In all craniosynostosis, long-term follow-up is mandatory because there is a risk of secondary synostosis which exists independently from the originally affected suture.