What Is Craniosynostosis?
Sutures most often involved in craniosynostosis (drawn by Raymond Sze)
Craniosynostosis (pronounced crane-eo-sin-os-TOW-sis) is when one or more of the special joints in a baby's skull (sutures) grow together (fuse) earlier than normal. When these joints come together too early, a baby's skull cannot grow properly.
Sutures are located between the bone plates in a baby’s skull. They allow the baby’s head to come through the birth canal. After birth, the sutures let the skull get bigger to make room for brain growth.
After infancy, the sutures slowly begin to grow together to fully connect the skull bones. The skull bones begin to grow together when children are about 2 or 3 years old, but the process is not fully complete until adulthood.
The diagram below shows the several sutures in a baby’s skull. A baby can have one or more fused sutures.
When any of the sutures grow together early, the baby’s head develops a specific shape, depending on where the fusion occurs. You can usually see an unusual shape to a baby’s head at birth or shortly after. When several sutures close early, the baby's head can't expand to hold the growing brain. This can cause increased pressure in the skull that may hurt brain development.
Watch this video to learn more about craniosynostosis.
Craniosynostosis in Children
Craniosynostosis is usually present when babies are born (congenital). There are different types of craniosynostosis. Most children with craniosynostosis have only one fused suture. Otherwise, they are healthy. Craniosynostosis of the sagittal suture — where two bones on the top of the head come together — is the most common type. It happens most often in boys.
Doctors don't know exactly what causes craniosynostosis.
Symptoms of Craniosynostosis
The first sign of craniosynostosis is an unusually shaped head. Other symptoms of craniosynostosis include:
- Soft spot (fontanelle) on baby's head disappears early
- Raised ridge develops along the fused sutures in the skull
- Abnormal pressure develops inside the skull
Other problems happen depending on which of the joints grow together. For example, one type of fused suture may cause a baby's face to appear slightly twisted.
Craniosynostosis Diagnosis
Craniosynostosis usually is present when your baby is born (congenital). But in mild cases, you and your doctor may not notice it right away. Doctors usually identify craniosynostosis in the first few months of life.
When your baby is born, the doctor will feel their head during a physical exam. The shape of your child's head will help the doctor tell which sutures have grown together. The doctor will most likely measure around your baby's head. This may be all your baby needs for diagnosis.
If your child's craniofacial team thinks your child has craniosynostosis, they will recommend a CT (computerized tomography) scan
of your child's head. A CT scan is an X-ray procedure that takes a cross-sectional view of the body, which is enhanced by a computer. The timing of the CT scan will depend on your child's age, diagnosis and when surgery might be performed.
Surgery for Craniosynostosis
Surgery to correct craniosynostosis involves reshaping your child's skull.
Often, this means open surgery. Some babies may have a minimally invasive procedure instead, called endoscopic strip craniectomy.
Open surgery
The neurosurgeon makes a cut (incision) across the top of the head. (Usually, the scar is covered completely by your child's hair within months of the surgery.) Then the neurosurgeon and a plastic surgeon work together to reshape the skull.
The neurosurgeon on the team removes the affected suture. Then, the craniofacial plastic surgeon shapes the skull bones into a more normal shape. The new shape is held together by plates and screws while the bone heals. The plates and screws are made of a special material that breaks down and dissolves completely in one or two years.
If your baby has simple (single-suture) craniosynostosis, they may need only one surgery. Children with craniofacial syndromes that cause craniosynostosis often need more than one operation to fix the problem.
After the operation, your baby will probably stay in the intensive care unit for one to three nights before moving to the regular hospital room. Babies usually stay in the hospital three to five days.
If the sagittal suture is the only suture that needs repair, surgery takes place when your baby is less than 4 months old. If other sutures need repair, we operate when your baby is 6 to 12 months old.
Endoscopic strip craniectomy
Your baby may have another option if only their sagittal or lambdoid suture is fused. This option involves taking out a strip of bone along the top of the skull through small incisions. After the surgery, your baby wears a helmet for at least three months to mold their head to a more normal shape.
Some families prefer this choice instead of open surgery. It may mean:
- Less scarring
- Less bleeding, so less chance of a blood transfusion
- Less time in surgery
- Shorter hospital stay
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