What is craniosynostosis?

Craniosynostosis

Craniosynostosis

Craniosynostosis (craniostenosis) is a condition that affects the skull development of a fetus or newborn. One or more of the connections (sutures) between plates in a fetus's or baby's skull close too soon, and the bony plates of the skull fuse too early.

As a result of early fusion of the plates, the affected area of the skull does not expand normally with the growing brain, causing a misshapen head. In severe cases, usually when craniosynostosis affects more than one suture, pressure builds on the brain and may cause long-term complications. This is rare.

Some babies have craniosynostosis because they inherited certain genetic traits from one or both parents. Other causes are not fully understood.

An irregularly shaped head is the most noticeable feature of craniosynostosis, and it is usually noticed at birth or shortly after birth. Imaging tests (such as a skull X-ray or CT scan) may be done to confirm a diagnosis.

Craniosynostosis is treated with surgery to allow the skull to return to its normal shape, grow normally, and prevent further complications. In general, no lasting disfigurement occurs, especially when surgery is done early.

What are the symptoms of craniosynostosis?

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.

How is craniosynostosis treated?

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.

How is craniosynostosis diagnosed?

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.

How can you care for your newborn who has craniosynostosis?

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.

What causes craniosynostosis?

Experts aren't sure what causes this problem. In some cases, it runs in families. If you've had a baby with craniosynostosis and are planning another pregnancy, you may want to talk to your doctor about genetic counseling.

Learning About Craniosynostosis in Newborns—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.

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