Craniofacial surgery is a surgical subspecialty of maxillofacial surgery that deals with congenital and acquired deformities of the skull, face, and jaws bone, skin, muscle, teeth, etc. Craniofacial surgery does not, however, include surgery of the brain or eye.
typically treated by craniofacial surgeons include craniosynostosis (isolated and syndromic), rare craniofacial clefts, acute and chronic sequellae of facial fractures, cleft lip and palate, micrognathia, Treacher Collins Syndrome, Apert's Syndrome, Crouzon's Syndrome, hemifacial microsomia and many others.
Craniosynostosis refers to the early closing of one or more of the sutures of an infant's head. The skull is normally composed of bones which are separated by sutures. As in 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.
In scaphocephaly the saggital suture is prematurely fused. The saggital suture runs from the front to the back of the head. The shape of this deformity is a long narrow head, formed like a boat (greek skaphe, “light boat or skiff”).
In trigonocephaly the metopic suture is prematurely fused. The metopic suture is situated in the medial line of the forehead. Premature fusion of this suture caused the forehead to become pointed, giving the head a triangular shape when viewd from above (greek trigono, “triangle”).
In plagiocephaly one of the coronal sutures is prematurely fused. The coronal sutures run over the top of the head, just in front of the ears. The shape of this deformity is an asymmetrical distortion (flattening of one side of the head).
In brachycephaly both of the coronal sutures are prematurely fused. shape of this deformity is a wide and high head.
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. In 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.
The general features of a child with Apert syndrome are similar to those in Crouzon syndrome however there is not as much variability between cases and the degree of presentation is more severe.
Treatment in infancy is directed at correction of the suture fusion and resultant misshapen head. (See section on craniosynostosis).Surgical treatment of the midface deformity is usually done during the pre-school period (age 4-6 yrs). Conventional surgical advancement of the midface requires numerous cuts of the facial bones and advancing the midface region to a predetermined level. This usually requires bone grafts. Plates and screws are used to stabilize the new position.The past several years have seen a significant increase in treatment using a technique called Osteogenic Distraction. The same surgical boney cuts are performed and an expansion device is inserted, where by gradual advancement of the midface region can be obtained. Research has indicated this may provide a more stable correction.