Craniosynostosis

Topic Overview

What is craniosynostosis?

Craniosynostosis (say "kray-nee-oh-sih-noh-STOH-sus") is a problem with the skull that causes a baby’s head to be oddly shaped.

A baby's skull is not just one bowl-shaped piece of bone. It is made up of five thin, bony plates that are held together by fibrous material called sutures. The sutures let a baby's skull expand as the brain grows. Over time, the sutures harden and close the skull bones together.

When a baby has craniosynostosis, one or more of these sutures close too soon. The head doesn't form a normal shape.

How the disease affects your baby depends in part on how many of the skull sutures close too soon:

If only one suture closes, the baby's brain usually develops normally, but the head has an odd shape. This happens in most cases of craniosynostosis.
If more than one suture closes, the baby's brain may not be able to grow as fast as it should. If severe pressure builds up around the brain, it may cause brain damage, seizures, blindness, and developmental delays. But this severe pressure is rare.

Craniosynostosis occurs in about 1 out of 2,000 births.¹ It is also called craniostenosis.

What causes craniosynostosis?

Experts aren't sure what causes this problem. In some cases, it runs in families.

Craniosynostosis is more likely in babies:

Who lie in a breech position while in the uterus.
Whose mothers smoke or live at a high altitude during pregnancy.
Who are twins.

What are the symptoms?

The most common sign of craniosynostosis is an oddly shaped 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.

This oddly shaped 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 cause brain damage and can make the baby develop more slowly than other children. If you know your baby has this condition, call your doctor right away if your baby:

Begins vomiting.
Becomes sluggish and sleeps more and plays less.
Becomes crankier than usual.
Develops swollen eyes or has problems moving the eyes or following objects.
Has problems hearing.
Breathes noisily or doesn't breathe for short periods of time.

How is craniosynostosis diagnosed?

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

Your doctor will:

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

To be sure that the diagnosis of craniosynostosis is correct, your baby's doctor may order a skull X-ray, CT scan, or MRl.

Just because your baby has an oddly shaped head doesn't mean that he or she has craniosynostosis. A misshapen head may be caused 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.

How is it treated?

Surgery to form new suture(s) is usually needed in the first year of life. It helps prevent or relieve pressure on the brain. It also corrects your baby's head shape.

The earlier your child has surgery, the better the results. 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 your baby’s head shape returns to normal without surgery. Your child may wear a special helmet or other device to help reshape the skull. But your child may still need surgery later.

If your child needs surgery, talk with your doctor about the surgery and what you can expect afterward.

Frequently Asked Questions

Learning about craniosynostosis:	What is craniosynostosis? What are skull sutures?
Being diagnosed:	What are the symptoms? What else can cause my baby's head to be misshapen? How is craniosynostosis diagnosed? What does a doctor look for during a physical exam?
Getting treatment:	What is the treatment? How can I help my baby before and after surgery?

Symptoms

The most common sign of craniosynostosis is an irregularly shaped head. You may also be able to feel a ridge along the skull where the suture has closed. There may be no other symptoms when craniosynostosis affects only a single suture. Misshapen heads can also be caused by other factors or conditions that are unrelated to craniosynostosis, such as the birthing process or your baby's sleep position.

Although most cases of craniosynostosis are diagnosed based on an irregularly shaped head, illness may also be a sign. Craniosynostosis is usually more severe when more than one suture closes too early. This significantly restricts the skull's ability to expand as the brain grows. As pressure builds on the brain, your baby may:

Vomit.
Become sluggish, sleep more, and play less.
Become irritable because of head pain.
Develop swollen eyes or problems moving the eyes or following objects.
Have problems hearing.
Breathe noisily or have periods of not breathing (apnea).

When the pressure is very severe, it may cause brain damage and other problems, including seizures, blindness, and developmental delays. Untreated craniosynostosis may cause lasting disabilities.

Exams and Tests

The effects of craniosynostosis are often visible at birth or shortly afterward. In some cases a doctor may notice an irregularly shaped head during a routine well-child checkup in the first few months of life. Your baby's doctor may try to rule out other factors or conditions that can cause a misshapen head, such as those related to the birthing process or an infant's sleeping position.

Your baby's doctor will examine:

The appearance of each side of your baby's face and head.
Your baby's skull (sutures) and the soft spots (fontanelles).
The top and sides of the head where sutures are located to determine if there are unusual ridges or bumps.

Your baby's doctor will also ask you questions about your medical history to help determine whether your baby has inherited craniosynostosis. To confirm a diagnosis, your baby's doctor may order one or more of the following tests:

Skull X-ray. This type of X-ray is usually the first test done because it can often show whether the sutures on the baby's skull are open or closed.
CT scan. This test shows the skull shape and sutures in greater detail than an X-ray, so it often is done if the skull X-ray is unable to show clearly whether the sutures are open or closed.
MRI. An MRI scan of the head may be done to check whether the baby has developed any problems within the brain. Although rare, sutures that close prematurely can create these types of problems if the skull's growth is severely restricted.

If your baby has been diagnosed with craniosynostosis:

Ask your doctor about genetic counseling. Craniosynostosis is associated with genetic disorders in up to 20% of cases.¹ Having one baby with craniosynostosis increases your chance of having another baby with this condition.
Watch for signs of increased pressure on your baby's brain, such as vomiting, sluggishness, and swelling around the eyes. Call your doctor immediately if any of these signs develop.
Have your child's hearing checked because there's a chance of hearing loss. The United States Preventive Services Task Force recommends that all newborns be screened for hearing loss.²

Treatment Overview

Surgery is the usual treatment to correct craniosynostosis. It's usually done in the first year of life. The surgeon removes strips of bone in the skull to create artificial sutures. This surgery relieves pressure on the brain and allows the skull to expand normally. After surgery, your baby's skull should return to its normal shape and continue to grow without deformity.

The timing of surgery is very important. Usually, the earlier the diagnosis and surgery, the better the results. When done early, surgery not only corrects your baby's head shape but also may help prevent complications, such as developmental delays or lasting disabilities.

Several factors will help determine the timing of the surgery to correct craniosynostosis, including:

How severe the condition is (for example, whether or not pressure has built up on the brain).
Your child's overall health.
What your preferences are.
What your doctor recommends.

Most babies with craniosynostosis need only one surgery to correct the condition, and complications are not common. If complications develop, they are often related to excessive bleeding or injury to the baby's eyes or brain.

Newer surgical techniques for craniosynostosis are less invasive than traditional surgery. They generally result in less blood loss and fewer risks. One such technique, endoscopic-assisted strip craniectomy, sometimes requires the baby to wear a custom-made helmet after surgery until he or she is about 1 year of age.

In some cases, a child requires additional surgery or other treatments. Often this is because the craniosynostosis is severe, surgery was delayed, or complications have developed.

If your baby doesn't seem to have pressure on the brain, your doctor may recommend waiting to see whether your baby's head shape returns to normal without treatment. During this time, your baby may wear devices, such as a special helmet, to help the skull resume its normal shape. But your child may eventually need surgery.

Counseling or a support group can help you manage the stress related to having a child with craniosynostosis.

Home Treatment

The only treatment for craniosynostosis is surgery. There are things you can do at home before your child's surgery to help make your child more comfortable and to ensure proper healing after surgery.

Ask your doctor to show you before-and-after pictures of other children who have had the same type of surgery. This may help you prepare for how your child will look right after he or she has surgery. Photos can also show you the positive results that other children have experienced from surgery.

Talk with your baby's doctor about the details of surgery and what you can expect afterwards.

At the hospital:

Stay with your baby as much as possible before surgery.
Comfort your child, and provide gentle reassurance.
Have some of your child's familiar or treasured objects, such as blankets or stuffed animals, with you at the hospital.
Talk to your child and be involved in his or her care. This will help your child get well, and it will make you feel better. It will also help you be more comfortable caring for your child when he or she returns home.
Talk to the health professionals treating your baby about:
- Anything you do not understand.
- How to comfort your child when you are not present.

After surgery, continue to comfort and reassure your child during recovery in the hospital. Be prepared for how your child will appear right after surgery. There may be significant swelling, some bruising, and a large bandage covering his or her skull. Your baby may need to wear a helmet. If this is the case, you will receive instructions on how to put the helmet on and take it off.

When your baby returns home:

Take care of your baby's incision. Clean it regularly and check for signs of infection, such as redness, warmth, pain, or swelling. This incision will leave a scar, which most likely will be completely covered when your baby's hair grows over it.
Check for signs of complications from surgery, such as bleeding. Also look for signs of pressure on the baby's brain, such as vomiting or increased swelling around the eyes.
Follow instructions from your doctor about how to position your baby after surgery and about helmet care (if your baby needs to wear one).
Call your baby's doctor if you have any questions or concerns about your baby's condition.

Other Places To Get Help

Organizations

National Institute of Neurological Disorders and Stroke
P.O. Box 5801
Bethesda, MD 20824
Phone:	1-800-352-9424 (301) 496-5751
TDD:	(301) 468-5981
Web Address:	www.ninds.nih.gov

The National Institute of Neurological Disorders and Stroke (NINDS), a part of the National Institutes of Health, is the leading U.S. federal government agency supporting research on brain and nervous system disorders. It provides the public with educational materials and information about these disorders.

Children's Craniofacial Association (CCA)
13140 Coit Road
Suite 517
Dallas, TX 75240
Phone:	1-800-535-3643 (214) 570-9099
Fax:	(214) 570-8811
E-mail:	contactCCA@ccakids.com
Web Address:	www.ccakids.org

CCA is a nonprofit agency that helps address the medical, financial, psychosocial, emotional, and educational concerns relating to craniofacial conditions. CCA's services are for people who have facial differences and for their families. The Web site has information on resources, family networking, medical centers, and more.

KidsGrowth.com: Pediatric Health Care Alliance, P.A.
P.O. Box 1068
Oldsmar, FL 34677
Phone:	(813) 854-2003
Web Address:	www.kidsgrowth.com

The KidsGrowth Web site, created by pediatricians, has children's health resources for parents and teens. It offers a free newsletter and information about child development, behavioral issues, and illnesses. The TeenGrowth interactive Web site (www.teengrowth.com) offers a secure environment for teens to get valuable information on topics such as alcohol, drugs, emotions, health, family, friends, school, sex, and sports.

Related Information

References

Citations

Kinsman SL, Johnston MV (2007). Craniosynostosis section of Congenital anomalies of the central nervous system. In RM Kliegman et al., eds., Nelson Textbook of Pediatrics, 18th ed., pp. 2455–2456. Philadelphia: Saunders Elsevier.

U.S. Preventive Services Task Force (2008). Universal screening for hearing loss in newborns: U.S. Preventive Services Task Force Recommendation Statement. Pediatrics, 122(1): 143–148. Also available online: http://www.ahrq.gov/clinic/uspstf08/newbornhear/newbhearrs.pdf.

Other Works Consulted

Church MW, et al. (2007). Auditory brainstem response abnormalities and hearing loss in children with craniosynostosis. Pediatrics. Published online May 21, 2007 (doi:10.1542/peds.2006-3009).

Greer M (2005). Structural malformations. In LP Rowland, ed., Merritt's Neurology, 11th ed., pp. 592–593. Philadelphia: Lippincott Williams and Wilkins.

Persing JA (2008). MOC-PS CME article: Management considerations in the treatment of craniosynostosis. Plastic and Reconstructive Surgery, 121(1): 1–11.

Credits

Author	Debby Golonka, MPH
Editor	Susan Van Houten, RN, BSN, MBA
Associate Editor	Pat Truman, MATC
Primary Medical Reviewer	Adam Husney, MD - Family Medicine
Specialist Medical Reviewer	Michael J. Sexton, MD - Pediatrics
Last Updated	July 13, 2009

Topic Contents