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What is CROUZON SYNDROME?

Crouzon Syndrome is a type of syndromic or genetic type of craniosynostosis that involves premature fusion of the cranial sutures, often both coronal sutures, with associated hypoplasia of the middle third of the face. Patients present with variable expression of the gene but appearance is characterized by protruding eyes (exorbitism), a result of the deficient surrounding skeleton. Exorbitism can result in visual disturbances due to corneal exposure. Patients with Crouzon Syndrome can have obstructive sleep apnea. Some may develop hydrocephalus (an accumulation of fluid within the brain).

It is distinguished from other forms of syndromic craniosynostosis in that hand or foot anomalies do not occur. Intelligence is typically normal. It is the most common type of syndromic craniosynostosis.

Symptoms SYMPTOMS

Crouzon Syndrome shares many of the same features as Apert syndrome, including abnormal development of the eye sockets, prominent and widely spaced eyes, and slow development of the midface. In patients with Crouzon Syndrome, the forehead generally appears more rounded with a more normally shaped nose, and the eyes tend to be more prominent. Another key difference is that patients with Crouzon Syndrome do not have associated hand or feet anomalies present in other syndromes.

Crouzon Syndrome presents many of the same associated issues as Apert syndrome, including airway compromise, sleep apnea, hydrocephalus and eye exposure issues. It is important for patients with Crouzon Syndrome to be treated by a multidisciplinary care team that specializes in caring for children with these complex disorders.

Treatment TREATMENT & MANAGEMENT

Treatment goals include protecting vision, allowing normal brain development, achieving normal facial proportions, and relieving any obstructive sleep apnea.

Evaluation should be performed early and must involve a multidisciplinary craniofacial team, including a craniofacial plastic surgeon and neurosurgeon. Typically skull surgery is performed sometime before one year of age. This could involve posterior skull expansion and/or frontal skull expansion. Midfacial advancement, in which the bony orbital cavities and upper teeth are moved forward, is usually undertaken after age 8. The procedure usually involves distraction osteogenesis, a technique in which the bones are slowly advanced by the turning of screws, either inserted under the skin or on the skull. Further jaw surgery at skeletal maturity, usually around age 18, may be necessary to correct an under bite deformity (the front teeth fall behind the lower teeth).

The treatment of Crouzon Syndrome is dependent upon both functional and appearance-related needs, and should be addressed immediately after your child is born. Because of the complex issues that can be associated with Crouzon Syndrome, your child should be treated at a medical center where she will have access to pediatric specialists across the many clinical areas she may need.

Coordinated care of patients with Crouzon Syndrome is typically managed through a Craniofacial Team. Because every patient with Crouzon Syndrome has unique problems, the timing and course of surgical treatment is highly individualized. It is important to see a surgeon with expertise in pediatric plastic and reconstructive surgery who specializes in treating these rare conditions.

As your child grows, she should also have access to psychosocial support services to address any mental, social or psychological issues that accompany these conditions.