What is craniosynostosis?

Before birth, a baby’s head is soft so that he or she can pass through the birth canal. Typically, a child’s skull continues to develop until they are 22 to 26 months old. Sometimes, their skull may flatten at the back from lying on their backs too much, called positional plagiocephaly. This condition is often remedied with frequent position changes, which alleviates the pressure from the flat spot, or by wearing a custom-made helmet. 

However, if your child’s head shape is changing because of premature fusing in their skull, he or she may be affected by craniosynostosis. 

Craniosynostosis (pronounced kray-nee-o-sin-os-TOE-sis) occurs when a child’s sutures, which separate the bone plates in a skull, fuse too soon — before your child’s head and brain are fully formed. One or multiple plates can fuse at the same time. This affects the skull’s growth and, in some cases, can affect brain growth. It causes increased head pressure and a non-symmetrical appearance. 

Simple craniosynostosis occurs when one suture fuses and complex craniosynostosis occurs when two or more sutures fuse. Craniosynostosis can be further categorized into four main types:

• Sagittal synostosis occurs when bones at the top of the head fuse together too early, forming a head that is longer and narrower than usual. This is the most common form of craniosynostosis.
• Coronal craniosynostosis occurs when one or both sutures connecting the top of the head to the ears fuse too early. This causes a child to have a flat forehead and high eye sockets on one or both eyes.
• Metopic synostosis causes a child’s head to have a triangular shape. It occurs when the sagittal suture, which runs along the middle of the head, and the nose fuse together too soon.
• Lambdoid synostosis is the rarest form of craniosynostosis. This form causes the back of the head to become flat. This happens when the suture that runs along the back of the head fuses prematurely with the plates that surround it. 
  
   

Whatever the cause may be, our dedicated Head-Shape multidisciplinary clinic can help. Here, our pediatric neurology and neurosurgery team partners with a professional physical therapist to care for your child.

What are the signs and symptoms of craniosynostosis?

If your child is experiencing craniosynostosis, you’ll notice a change in the shape of their face and head. If experiencing increased pressure in their skull, your child may also show rare but more severe symptoms of craniosynostosis including:

• Bulging eyes
• Cognitive impairment
• Developmental delays
• Eye movement disorders
• Feeding challenges
• Increased irritability
• Noticeable scalp veins
• Projectile vomiting
• Seizures
• Sleepiness

There are other reasons your child’s head might be shaped differently than what’s considered normal. For example, if the back of your baby’s head appears flat, it may be because he or she is spending too much time on their back. This can be treated with regular position changes or the use of a helmet. It’s important to see your doctor about any symptoms your child is be experiencing to receive the proper diagnosis.

Diagnosing craniosynostosis

To properly diagnose craniosynostosis, our team will perform a few tests on your child. Genetic testing may also be conducted if our team suspects an underlying genetic syndrome. In some cases, your child may also be diagnosed during a third-trimester ultrasound. Other tests may include: 

• Physical exams: Our team will conduct a physical exam to evaluate your child’s condition.
• Magnetic resonance imagining (MRI): MRIs use powerful radio waves and magnets to create detailed, 3D images of the brain.
• Computerized tomography (CT or CAT) scans: CT scans use X-rays to obtain cross-sectional images of the brain. 

These tests are necessary to help your child receive the best treatment possible, and we want to make sure you both fully understand your child’s treatment plan. We know these tests can be scary for you and your little one, and we’ll help you through every step of the process.

How is craniosynostosis treated?

Some studies show that certain medications taken during pregnancy may increase an unborn baby’s risk of developing craniosynostosis. If you’re pregnant, discuss your medications with your doctor to determine if any medications you’re taking may increase your child’s risks.  

Although not all forms of craniosynostosis require surgery, procedures can help correct the premature fusing of the skull plates in many forms. If craniosynostosis surgery is recommended, Geisinger’s pediatric neurosurgery team is trained in the latest techniques and equipment that will help your child’s head develop properly. 

If your child needs surgery, we will refer you to our craniofacial multidisciplinary clinic. Here, our pediatric neurosurgeon and pediatric plastic surgeon, who specializes in craniofacial surgery, will partner and use minimally invasive techniques whenever possible, tailoring the right treatment and surgery that is best for your child.