Cleft & Craniofacial Team at St. Mary's

804-287-7396

Crouzon syndrome

Crouzon syndrome is a genetic disorder which affects the child’s skull and face. This condition occurs when there is abnormal fusion between some of the bones of the skull and the bones of the face, therefore preventing normal growth. Such growth affects the shape of the head, the appearance of the face and the relationship of the teeth. The eyes may be prominent, but the hands and feet usually appear normal. Crouzon syndrome is not expected to affect intellect.

Causes

Crouzon syndrome is a very rare genetic disorder. It can be passed on from the child’s parents through autosomal dominant inheritance. This means that only one parent needs to have the abnormal gene for the child to inherit the disease. The majority of cases are not inherited, which means that there was a new mutation in the child’s fibroblast growth factor receptor genes located on chromosome 10 or chromosome 4.

Treatment

Crouzon syndrome is a complex condition that requires multiple stages of treatment over the course of the patient’s lifetime. Craniosynostosis is treated by performing surgery to the skull. Oftentimes, the upper and middle face will require surgery as well. The first goal is to open the closed sutures to prevent problems with high pressure in the brain and allow the brain to grow normally. The second goal is to obtain a normal shape of the skull and face. The timing and type of surgery your child will have depends on your child’s condition and your surgeon. Our comprehensive and specialized team includes a geneticist, craniofacial plastic surgeon, pediatric neurosurgeon, and dental specialists who will care for your child every step of the way.

To learn more about Crouzon syndrome, please call 804-287-7396 to speak with our nurse coordinator, Linda Shait, RN.