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Premature ossified cranial sutures of infants (craniosynostis) may lead to skull deformities in the growth process. Surgical interventions are required to improve the patients' appearance and prevent skull deformation and Brain damage by increased ICP.
Crouzon and Apert syndromes are the most common of the craniosynostosis syndromes. 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. The coronal suture goes from ear to ear on the top of the head and fusion of both sides (bicoronal synostosis or brachycephaly) results in a very flat, recessed forehead. This is the suture fusion found most often in Crouzon and Apert Syndromes.

Shape of the Skull depend upon the suture or sutures fuse.Skull shape can short, wide elongate or depressed forehead.

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 addition, children with Apert Syndrome have syndactaly (webbing) of the hands and feet.Crouzon Syndrome occurs in approximately 1 in 25,000 births. It 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 incidence of Apert syndrome is approximately 1 in 100,000 births and most cases are fresh mutations. 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

Treatment in infancy is directed at correction of the suture fusion and resultant misshapen head.

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.