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. This diagram shows the different sutures which can be involved.

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.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.

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.

As with any craniofacial disorder, treatment by a multidisciplinary team working together with the family provides the best results. In the newborn period some potential problems that may need to be addressed include respiratory difficulties, feeding problems, neurologic complications such as hydrocephalus, and the potential risk of developmental delay.If a cleft palate is present this could cause some feeding difficulty. Techniques described in the section on feeding may be used.

The infants shallow midface and/or small or partially obstruced nasal passages can cause respiratory problems and also contribute to feeding difficulty. Evaluations by the ENT specialists are important and a tracheostomy may be necessary to relieve the airway problems in a newely born infant.In Crouzon and Apert syndromes synostosis of 2 or more cranial sutures may be involved, therefore there can be a risk for increased intracranial pressure. There is also a greater chance of hydrocephalus in these infants than those with single suture fusion and evaluation and close monitoring by the team neurosurgeon is important.The reported incidence of mental retardation in patients with Crouzon syndrome is between 0% and 20% while a higher incidence ( approx. 20-30%) has been reported with Apert syndrome. Referral to an infant stimulation program may be helpful.After the newborn period, the multidisciplinary team continues to evaluate the childs needs. Audiology (hearing test) and speech evaluations are important to insure good speech and language development. Assessment by the opthalmologist is important, especially if the eyelids are not protecting the eyes completely or if there are eye muscle problems