Pediatric Brain Injury: Craniosynostosis

Craniosynostosis - Topic Overview

What is craniosynostosis?

The skull of an infant is made up of free-floating bony plates separated by fibers called sutures. This arrangement allows the infant's head to pass through the birth canal and also enables the skull to grow with the brain in early infancy. Over time, the sutures harden and close (fuse) the skull bones together.

Premature fusing of the sutures is called craniosynostosis, which restricts skull growth. Simply put, synostosis is the union of two or more bones to form a single bone and causes the baby's head to become abnormally shaped. Children born with craniosynostosis may have increased pressure on the brain and vision problems. They also have low self-esteem and behavioral problems.

The effects of craniosynostosis on a fetus or baby depend in part on how many of the skull sutures are affected.

• Most often, craniosynostosis affects only one suture. More than half of all cases involve the sagittal suture. The sagittal suture runs across the top of a baby's head from front to back. The baby's brain usually develops normally in these cases, but the head becomes abnormally shaped. The skull may become long and narrow or very flat and broad in front or back or on the sides. This depends on which suture closes prematurely.
• Craniosynostosis can develop along more than one suture. When more than one suture closes prematurely, the baby's brain may not be able to grow at its expected rate. In addition, pressure may build up around the brain, causing brain damage, seizures, blindness, and developmental delays. Over time, this can lead to permanent disabilities if the condition is not treated. A baby with more than one suture affected by this condition may have facial defects, such as a misshapen nose or jaw.

What causes craniosynostosis?

Doctors do not fully understand what causes craniosynostosis. Up to 20% of babies with craniosynostosis have inherited a genetic trait from one or both parents that results in the condition.

Some cases of craniosynostosis may be related to fetal growth or position or other conditions during pregnancy. Fetuses that lie in a breech position while in the womb, are carried by mothers who smoke or who live at a high altitude during pregnancy, or are twins may have an increased risk of developing craniosynostosis.

Craniosynostosis is one of the most common physical deformities of a fetus or newborn, occurring in about 1 in every 2,000 to 2,500 births.

What are the 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, some babies become ill. 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 permanent disabilities.

How is craniosynostosis diagnosed?

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, prematurely closing sutures 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. 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.

How is it treated?

Craniosynostosis - Treatment Overview

Surgery is the only treatment to correct craniosynostosis. The surgeon removes strips of bone in the skull to create artificial sutures, which 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 permanent disabilities.

Several factors will help determine the timing of the surgery to correct craniosynostosis, including how severe the condition is, what your doctor recommends, and what your preferences are.

• If only one suture has closed prematurely and there is no evidence of pressure on your child's brain, your baby's 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 specially designed helmet, to help the skull resume its normal shape. However, your child may eventually need surgery.
• Surgery to correct only one suture that has closed too soon may be done when the baby is between the ages of 3 months and 1 year.
• Surgery may be needed before a baby is 6 months of age if craniosynostosis affects more than one suture. This is a more severe form of the condition and usually causes pressure on the child's brain. If the pressure building on the brain is severe enough to cause brain damage, your baby will need surgery as soon as possible.

Most babies with craniosynostosis need only one surgery to correct the condition, and complications are not common. However, 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, and may be especially useful for treating babies who are 3 months of age and younger. 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.

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

Counseling or support groups may help you manage the stress related to having a child with craniosynostosis. Ask your doctor about contacting support groups or other parents of children with craniosynostosis.

• Concussion
• Craniosynostosis
• Labyrinthitis
• Stuttering

General Brain Injury Topics

• General Brain Injury Verdicts
• Pediatric Brain Injury
• Types of Brain Injuries

General Brain Injury Videos

General Brain Injury Links

• General Brain Injury - Brain Injury Association of America
Official website for the Brain Injury Association of America - includes symptoms, treatments, contacts, etc.
• General Brain Injury - Medline Plus- Brain Injury
General information on head injuries including types, symptoms, treatment, and other resources.