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The normal skull consists of several plates of bone that are separated by sutures. The sutures (fibrous joints) are found between the bony plates in the head. As the infant grows and develops, the sutures close, forming a solid piece of bone, called the skull.
Craniosynostosis is a condition in which the sutures close too early, causing problems with normal brain and skull growth. Premature closure of the sutures may also cause the pressure inside of the head to increase and the skull or facial bones to change from a normal, symmetrical appearance.
Craniosynostosis occurs in one out of 2,200 live births and affects males slightly more often than females.
Craniosynostosis is most often sporadic (occurs by chance). In some families, craniosynostosis is inherited in one of two ways:
Craniosynostosis is a feature of many different genetic syndromes that have a variety of inheritance patterns and chances for reoccurrence, depending on the specific syndrome present. It is important for the child as well as family members to be examined carefully for signs of a syndromic cause (inherited genetic disorder) of craniosynostosis such as limb defects, ear abnormalities, or cardiovascular malformations.
Specific treatment for craniosynostosis will be determined by your child’s physician based on:
Surgery is typically the recommended treatment. The goal of treatment is to reduce the pressure in the head and correct the deformities of the face and skull bones.
The optimal time to perform surgery is before the child is 1 year of age since the bones are still very soft and easy to work with. Surgery may be necessary at a much earlier age depending upon the severity of the condition.
Before surgery, your child’s physician will explain the operation and may review “before and after” photographs of children who may have had a similar type of surgery.
Following the operation, it is common for the child to have a turban-like dressing around his/her head. The face and eyelids may be swollen after this type of surgery. The child is typically transferred to the intensive care unit (ICU) after the operation for close monitoring.
Problems after surgery may occur suddenly or over a period of time. The child may experience any or all of the following complications:
These complications require prompt evaluation by your child’s surgeon. The healthcare team educates the family after surgery on how to best care for their child at home, and outlines specific problems that require immediate medical attention.