Craniofacial surgery is a subspecialty of maxillofacial surgery that deals with congenital and acquired deformities of the skull, face, jaw bone and associated structures. There are estimated to be over 500 syndromes with craniofacial anomalies. Most are morphologic, affecting the form and function of the head and face. These defects are typically treated by craniofacial surgeons which includes craniosynostosis (isolated and syndromic), rare craniofacial clefts, cleft lip and palate, micrognathia, hemifacial microsomia, Treacher Collins Syndrome, Apert’s Syndrome, Crouzon’s Syndrome, hemifacial microsomia and many others. The clinical manifestations of these symptoms extend beyond the craniofacial area. Therefore, the best treatment approach is one that is multidisciplinary and takes into consideration physical and mental development.
Craniosynostosis refers to the early closing of one or more of the sutures of an infant’s head. It is a congenital disorder marked by premature fusion of one or more cranial sutures, resulting in abnormal cranial growth. The skull is normally composed of bones which are separated by sutures. As an infant’s brain grows, open sutures allow the skull to expand and develop a relatively normal head shape. If one or more of the sutures has closed early, it causes the skull to expand in the direction of the open sutures. This can result in an abnormal head shape. In severe cases, this condition can also cause increased pressure on the growing brain.
Approximately one in 2500 infants is affected by this disorder. Most cases are nonsyndromic or isolated. Syndromic craniosynostoses are those conditions that have additional manifestations (craniofacial and/or extracraniofacial deformities). In addition to craniosynostosis, these children also have fusion of the sutures or bones in the cranial base and midface, and shallow eye sockets. This gives the appearance of a flat midface and eyes which protrude. Children with Apert Syndrome have syndactaly (webbing) of the hands and feet. Crouzon Syndrome may be transmitted as an autosomal dominant genetic condition or appear as a fresh mutation (no affected parents). The appearance of an infant with Crouzons can vary in severity from a mild presentation with subtle midface characteristics to severe forms with multiple cranial sutures fused and marked midface and eye problems.
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