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"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:
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:
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
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:
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:
Your baby's doctor may also order a skull
CT scan, or
Surgery is the usual treatment to
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.
Learning about craniosynostosis:
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
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.
May 11, 2012
John Pope, MD - Pediatrics & Chuck Norlin, MD - Pediatrics
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