What is craniosynostosis?

Craniosynostosis (say “kray-nee-oh-sih-noss-TOH-sus”) is a problem with the skull. The soft areas between the plates of the baby’s skull are called sutures. The sutures usually start to fuse together after a child is 2 years of age. With craniosynostosis, one or more of the sutures fuses too soon. This can keep the skull from expanding as the baby grows. In severe cases, it can cause pressure on the brain.

This is a congenital condition. This means your baby was born with it.

If there’s a lot of pressure on your baby’s brain, surgery may be needed right away to relieve the pressure. Your child will be asleep during surgery.

Your baby may need special care, such as being in the neonatal intensive care unit (NICU). This may be scary for you. But the hospital staff understands this. They will explain what happens and will answer your questions.

The most common sign is a misshapen head at birth or by the time the child is a few months old. For example, the skull may become long and narrow. Or it may be very flat and broad in front or back or on the sides. The baby may have a misshapen nose or jaw.

A misshapen head may be the only sign of craniosynostosis.

In rare cases, the disease causes pressure to build up on the baby's brain. This can lead to brain damage and can cause the baby to develop more slowly than other children.

You or your doctor may notice that your baby has a misshapen head at birth, shortly after birth, or later at a well-child checkup.

Just because your baby has a misshapen head doesn't mean that your baby has craniosynostosis. Head shape may be affected by how your baby was positioned in your uterus, the birth process, or your baby's sleep position. Talk to your doctor if you are concerned about the shape of your baby's head.

Your doctor will:

• Look at each side of your baby's face and head.
• Measure your baby's head.
• Feel the sutures and soft spots (fontanelles) on the skull.
• Feel the top and sides of the head, where sutures are located, for unusual ridges or bumps.

Your baby's doctor may also order a skull X-ray or CT scan.

Surgery is the usual treatment to correct craniosynostosis. It's usually done in the first year of life. The earlier your child has surgery, the better the results.

The surgeon removes strips of bone in the skull to create artificial sutures. This surgery prevents or relieves pressure on the brain and allows the skull to expand normally. It also corrects the shape of your baby's head. Your child may wear a special helmet or other device after surgery.

If there is pressure on the brain, your child needs surgery right away. If your baby doesn't seem to have pressure on the brain, your doctor may advise you to wait and see if the head shape returns to normal without surgery. But your child may still need surgery later.

If your child needs surgery, talk with your doctor about what to expect. It may help to see some before-and-after pictures of other children who have had the same type of surgery so that you are prepared for how your child will look right after the surgery. There may be a lot of swelling and bruising at first.

Being involved in your baby's care while your baby is in the hospital may help you feel more comfortable when you take your baby home. You'll need to know how to care for your baby's incision and what problems to watch for. Problems after surgery aren't common.

It's normal to feel a wide range of emotions when your child has a problem like craniosynostosis. Counseling or a support group can help.

Regular exams and early treatment are key to helping avoid developmental problems that may be caused by this condition. Your baby will be seen by a team of specialists that can help give the best care.