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Craniosynostosis (Craniofacial Anomaly)

What is craniosynostosis?

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.

What causes craniosynostosis?

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:

  • autosomal recessive Autosomal recessive means that two copies of the gene are necessary to express the condition, one inherited from each parent, who are obligate carriers. Carrier parents have a one in four, or 25 percent, chance with each pregnancy, to have a child with craniosynostosis. Males and females are equally affected.
  • autosomal dominant Autosomal dominant means that one gene is necessary to express the condition, and the gene is passed from parent to child with a 50/50 risk for each pregnancy. Males and females are equally affected.

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.

Treatment of craniosynostosis:

Specific treatment for craniosynostosis will be determined by your child’s physician based on:

  • your child’s age, overall health, and medical history
  • extent of the craniosynostosis
  • type of craniosynostosis (which sutures are involved)
  • your child’s tolerance for specific medications, procedures, or therapies
  • expectations for the course of the craniosynostosis
  • your opinion or preference

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:

  • fever (greater than 101F)
  • vomiting
  • headache
  • irritability
  • redness and swelling along the incision areas
  • decreased alertness
  • fatigue

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.