ABSTRACT
This chapter describes the commonly encountered groups of craniofacial anomalies, outlines the principles of their management and reviews the role of the ENT surgeon in managing the patients. When considering the aetiology of craniofacial anomalies, it is helpful to consider their aetiology in terms of malformations, deformations and disruptions. Primarily, it is to prevent or manage the raised intracranial pressure that is synonymous with these conditions and to normalize the skull shape and morphology. If the posterior vault size and shape is satisfactory, a fronto-orbital advancement and remodelling is performed which both increases the volume of the skull and allows a conformational change or normalization of skull shape. Crouzon syndrome is characterized by craniosynostosis and maxillary hypoplasia with shallow orbits causing ocular proptosis. Distraction osteogenesis involves initial osteotomy, ensuring complete separation of bony segments, without mobilization or advancement, and application of an internal or external distraction device. Hydrocephalus can occur in up to 20% of patients with craniosynostosis.
