Craniosynostosis

Abstract

Craniosynostosis is a group of conditions characterised by the premature fusion of one or more cranial vault sutures. This may lead to abnormal cranial development with severe skull and craniofacial deformities and if the condition is left untreated, other complications such as raised intracranial pressure and cranial growth restriction may be implicated. Craniosynostosis can arise as part of a genetic syndrome, or nonsyndromically where the pathophysiology remains less clear. Occurring in 1 in 2,000 to 2,500 live births, diagnosis is carried out shortly after birth and treatment of craniosynostosis mostly involves surgery varying from less invasive procedures in those patients diagnosed early to single or repeated open calvarial reconstruction in the more complex cases. This article reviews the different types of craniosynostosis with their variable presentations, underlying genetic mutations, associated complications and neuro-psychological outcomes before discussing its management with distinct emphasis on surgical treatment options within a multidisciplinary team.