Craniosynostosis is a form of cranial (head) congenital (birth) defect that causes the bony plates in the baby’s skull to join prematurely. This can result in an under-developed brain as well as a misshapen head.
This condition can affect one or more joints in the baby’s skull. This condition is also related to a brain abnormality that results in retarded development of the brain.
This condition is mostly treatable using surgical methods to separate the prematurely joined plates of bones in the baby’s skull which allows for natural and normal growth of the baby’s brain.
The baby’s skull consists of seven boney plates that start to fuse after 2 years of age, which gives ample time for the natural growth and development of the baby’s brain. These joints are made from strong and fibrous tissue that holds these boney plates together and are called cranial sutures.
The signs of Craniosynostosis include:
These signs might not be noticeable right after birth but may become more apparent during the first few initial months.
There are several types of craniosynostosis. These are classified according to the cranial suture (joint) that they affect, these are:
Craniosynostosis is often classified as nonsyndromic or syndromic. Nonsyndromic craniosynostosis is the most common type of craniosynostosis, and its cause is unknown.
However, syndromic craniosynostosis is a complication caused by certain genetic syndromes, such as Apert syndrome, Pfeiffer syndrome and Crouzon syndrome, which can affect your baby’s skull development.
Babies with craniosynostosis, particularly those with an underlying syndrome, may develop increased pressure inside the skull (intracranial pressure). Their skulls don’t expand enough to make room for their growing brains.
If untreated, increased intracranial pressure can cause:
In addition, facial deformities that affect the middle of your child’s face may cause:
Craniosynostosis is usually diagnosed using:
Mild cases of craniosynostosis — those involving only a single suture with no other underlying condition — may require no treatment. Skull abnormalities may become less obvious as the baby grows and develops hair. Or the doctor may recommend a specially molded helmet to help reshape your baby’s head and allow room for your baby’s brain growth.
For most babies, however, surgery is the primary treatment for craniosynostosis. The type and timing of surgery depend on the type of craniosynostosis and whether there’s an underlying syndrome that needs treatment.
The purpose of surgery is to relieve pressure on the brain, create room for the brain to grow normally and improve your child’s appearance. A team that includes a specialist in surgery of the head and face (craniofacial surgeon) and a specialist in brain surgery (neurosurgeon) often performs the procedure.
The surgeon makes an incision in your baby’s scalp and cranial bones, and then reshapes the affected portion of the skull. Sometimes plates and screws, often made of material that is absorbed over time, are used to hold the bones in place. Surgery, which is performed during general anesthesia, usually takes hours.
After surgery, your baby remains in the hospital for at least three days. Some children may require a second surgery later because the craniosynostosis recurs. Also, children with facial deformities often require future surgeries to reshape their faces.
This less invasive form of surgery isn’t an option for everyone. But in certain cases, the surgeon may use a lighted tube (endoscope) inserted through one or two small scalp incisions over the affected suture. The surgeon then opens the suture to enable your baby’s brain to grow normally. Endoscopic surgery usually takes about an hour, causes less swelling and blood loss, and shortens the hospital stay, often to one day after surgery.
If your baby has an underlying syndrome, your doctor may recommend regular follow-up visits after surgery to monitor head growth and check for increased intracranial pressure. Head growth will be routinely monitored at the post-treatment check-ups.
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