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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. In rare cases it causes pressure on the baby's brain, which can cause damage. It is also called craniostenosis.

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

  • If only one suture closes too soon, the baby's brain usually develops normally, but the head has an odd shape. This is what happens in most cases.
  • If more than one suture closes too soon, 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.

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.

Craniosynostosis is more likely in:

  • Babies who lie in a breech position while in the uterus.
  • Babies whose mothers smoke or live at a high altitude during pregnancy.
  • Babies who are twins.

What are the symptoms?

The most common sign 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.

An 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 that your baby has this condition, call your doctor right away if your baby:

  • Starts vomiting.
  • Becomes sluggish and sleeps more and plays less.
  • Becomes crankier than usual.
  • Has 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.

Just because your baby has an oddly shaped head doesn't mean that he or she 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, CT scan, or MRl.

How is it treated?

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 prevents or relieves pressure on the brain and allows the skull to expand normally. It also corrects the shape of your baby's head.

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 the 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 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 he or she 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.

Other Places To Get Help

Organization

National Institute of Neurological Disorders and Stroke (U.S.)
Web Address: www.ninds.nih.gov

References

Other Works Consulted

  • AH Ropper, MA Samuels (2009). Craniostenoses section of Developmental diseases of the nervous system. In Adams and Victor's Principles of Neurology, 9th ed., p. 963. New York: McGraw-Hill.
  • 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).
  • Evans K, et al. (2012). Craniofacial malformations. In CA Gleason, SU Devaskar, eds., Avery's Diseases of the Newborn, 9th ed., pp. 1331–1350. Philadelphia: Elsevier Saunders.
  • Greer M (2010). Premature closure of cranial sutures section of Cerebral and spinal malformations. In LP Rowland, TA Pedley, eds., Merritt's Neurology, 12th ed., p. 589. Philadelphia: Lippincott Williams and Wilkins.
  • Kinsman SL, Johnston MV (2011). Congenital anomalies of the central nervous system. In RM Kliegman et al., eds., Nelson Textbook of Pediatrics, 19th ed., pp. 1998–2013. Philadelphia: Saunders.
  • Persing JA (2008). MOC-PS CME article: Management considerations in the treatment of craniosynostosis. Plastic and Reconstructive Surgery, 121(1): 1–11.

Credits

By Healthwise Staff
Primary Medical Reviewer John Pope, MD - Pediatrics
Specialist Medical Reviewer Chuck Norlin, MD - Pediatrics
Current as of June 4, 2014

Current as of: June 4, 2014

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