What Is Craniosynostosis?

Craniosynostosis is a condition in which the growth seams in an infant’s skull close too early, causing problems with normal brain and skull growth. It may be non-syndromic or syndromic (genetic or inherited). There are close to 90 syndromes associated with syndromic craniosynostosis, including Apert, Crouzon, Pfeiffer, Muenke and Saethre-Chotzen.

The pediatric plastic surgery team at The Children's Hospital of Philadelphia performs more procedures each year in the treatment of syndromic and non-syndromic craniosynostosis than any other team in the country. Watch this educational video to learn more about the different types of craniosynostosis and how the condition is treated.

Transcript

What is craniosynostosis?

Scott Bartlett, MD: Craniosynostosis, or craniostenosis, is a condition which is premature closure of some of the seams on the skull. These are growth regions that allow normal expansion of the brain during growth. And when these seams close, you can constrict the brain in an area and cause abnormal growth in another area.

Craniosynostosis generally comes in two patterns. There’s the syndromic type in which there’s a genetic mutation, or an inheritable condition that causes a repeatable pattern. Or there can be the non-syndromic type where no one knows what causes it. There’s typically an isolated closure of one seam that gives you a distinct head shape abnormality.

There’s probably close to 90 syndromes associated with heritable forms of craniosynostosis. Apert, Crouzon, Pfeiffer, Muenke, Saethre-Chotzen Syndrome, the most common five that we see.

Some of these children are diagnosed by ultrasonography ahead of time, and there may be abnormal head shapes to alert them to the need for a special delivery. Many of those children will end up in our special delivery unit here at CHOP, which is focused on the care of fetal and congenital anomalies.

In syndromic-synostosis, these are typically genetic in origin, although they may be a new mutation to a new family. What they cause is not only closure of one of the seams of the skull, but they typically cause abnormalities in growth of the remainder of the face, and head and neck region. So these children frequently need a series of operations over their lifetime that may begin with a procedure on the cranial vault, or the skull, early in childhood, and progress to the need for secondary surgery on that. Or the need to move the entire mid-face into a different position. Or to adjust the jaws, and the upper and lower jaws, and correct a jaw deformity.

So beginning in childhood, and infancy, and all the way through adolescents, your child needs to be in a center that can know the needs and address the needs of a child growing. And can address them on multiple levels: the eye socket, the forehead, the orbits and the face sequentially.

So many of the non-syndromic patients have just a single seam closed prematurely. And the treatment of that usually involves removing that closed seam and reshaping the adjacent bone. If the child has a more severe, more established deformity, it involves an open approach where the seam is removed, the bones are cut and repositioned, and held in position with a series of sutures, or wires, or plates and screws that dissolve. This frequently involves the upper forehead, the orbits, and sometimes the remainder of the skull.

When it comes to the management of simple, non-syndromic synostosis, that is conditions in which there’s typically one suture closed, we have a variety of techniques available to us to treat that patient. They vary from simple strip craniotomies, placement of cranial springs, to formal open procedures that require cutting the bones and repositioning them.

What your child will need and what he will get will depend upon his age and the degree of deformity. And we have a team that has been utilizing these different techniques for years, and years, and years. And the team will tailor the treatment for your child.

We’ve had a Craniofacial Center here at The Children’s Hospital of Philadelphia since 1972. We perform more procedures each year in the treatment of syndromic and non-syndromic craniosynostosis than any center in the country.

Our team looks to coordinate the care and individualize the treatment of these complex deformities to fit your child.

Topics Covered: Craniosynostosis, Syndromic Craniosynostosis, Non-syndromic Craniosynostosis , Apert Syndrome, Crouzon Syndrome, Muenke Syndrome, Pfeiffer Syndrome, Saethre-Chotzen Syndrome

Related Centers and Programs: Craniofacial Program, Neonatal Craniofacial Program, Division of Plastic and Reconstructive Surgery