Craniosynostosis

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.

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.



The normal skull of a newborn is shown above; it contains five sutures that slowly come together to create a normal newborns skull. When things go array, the sutures can close to fast and create an abnormal skull for the newborn.

Plagiocephaly occurs the most often and happens in approximately one out of every 2,500 births. It involves fusion of either the right or left side of the coronal suture that runs from ear to ear. This is called coronal synostosis and it causes the normal forehead and the brow to stop growing. Therefore, it produces a flattening of the forehead and the brow on the affected side, with the forehead tending to be excessively prominent on the opposite side. The eye on the affected side may also have a different shape. There may also be flattening of the back area (occipital). Unilateral lambdoidal suture synostosis may cause plagiocephaly, as well.

Trigonocephaly is a fusion of the metopic (forehead) suture. This suture runs from the top of the head down the middle of the forehead, toward the nose. Early closure of this suture may result in a prominent ridge running down the forehead. Sometimes, the forehead looks quite pointed, like a triangle, with closely placed eyes (hypotelorism).

Sagittal synostosis (Scaphocephaly) is the second most common form of synostosis accounting for about 50% of all cases with a prevalence of 1 in 2000 live births. Premature fusion of the sagittal suture restricts the transverse growth of the skull. This results in an increased anteroposterior skull length to accommodate the growing brain. Frontal bossing and an exaggerated occiput can be pronounced and results in the classic “boat skull” appearance.

Symptoms of Craniosynostosis include:


 * A full or bulging fontanelle (soft spot of head)
 * scalp veins
 * increased irritability
 * vomiting
 * crying
 * poor feeding
 * seizures
 * bulging eyes
 * developmental delays
 * increasing head circumference

Treatment for Craniosynostosis include:

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 * Surgery is typically the recommended treatment. The goal of treatment is to reduce the pressure in the head and correct the deformities of the face and skull bones.
 * Following the operation, it is common for the child to have a turban-like dressing around his/her head. The face and eyelids may be swollen after this type of surgery. The child is typically transferred to the intensive care unit (ICU) after the operation for close monitoring.

The key to treating craniosynostosis is early detection and treatment. Some forms of craniosynostosis can affect the brain and development of a child. The degree of the problem is dependent on the severity of the craniosynostosis, the number of sutures that are fused, and the presence of brain or other organ system problems that could affect the child. If detected early, there is enough time for reconstructive surgery on the skull and the chances of a healthy child increase.

Children’s Hospital of Wisconsin: Craniosynostosis "[]"

Mayo Clinic: Craniosynostosis "[]"

Seattle Children’s Hospital: Craniosynostosis "[]"