Isolated, Non-Syndromic Craniosynostosis

What Is Non-Syndromic Craniosynostosis?

Non-syndromic craniosynostosis is a condition that affects the shape and growth of a baby's skull. It's congenital, meaning a baby is born with it. Non-syndromic means the cause of the condition is unknown. Craniosynostosis is a term used to describe a condition where the sutures, or seams, in a child's skull close too early and don't allow the brain to grow. In most cases, only one suture is involved. This is why non-syndromic craniosynostosis is often called isolated craniosynostosis.


Non-Syndromic Craniosynostosis Causes

Doctors aren't sure why some children develop non-syndromic craniosynostosis. Some doctors and researchers think it may relate to a child's position in the womb, and others think it's related to an unknown genetic cause.

Symptoms & Diagnosis

Non-Syndromic Craniosynostosis Symptoms

While non-syndromic craniosynostosis is present at birth, it might not be noticeable until a child is a few months old. The most noticeable, and sometimes only, symptom of the condition is an abnormal skull shape. Your child may also have a hard ridge where the suture is closed or a smaller soft spot than is normal for their age.

There are four classes of non-syndromic craniosynostosis that are based on the suture that is affected. The names of the most commonly affected sutures in the skull are:

  • Unicoronal
  • Metopic
  • Sagittal
  • Lambdoid 

The shape of a child’s head is different depending on which suture is fused.

Non-Syndromic Craniosynostosis Diagnosis

To diagnose non-syndromic craniosynostosis, the doctor will perform a physical exam of your child's skull and may request a CT scan. A CT scan captures x-ray images of different angles of the skull and helps the doctor determine which suture is affected.

The doctor will also examine your child to see if they have any other symptoms that may indicate a different condition, such as syndromic craniosynostosis.


Non-Syndromic Craniosynostosis Treatment 

The treatment approach depends on your child's age and which suture is affected. In very mild cases, your doctors may decide to observe your child instead of performing surgery. However, most children with isolated, non-syndromic craniosynostosis will need surgery.

 Since your child's brain continues to grow at a normal pace, it's important to treat a craniosynostosis in the first few years of life. Left untreated, your child could experience increased pressure on the brain which can cause chronic headaches, developmental delay, seizures, and vision problems.

Treatments vary based on how the condition specifically affects your child, but it's likely that your doctors will recommend surgery. It's important to find a surgeon who specializes in craniofacial surgery. Your doctor at the UPMC Cleft and Craniofacial Center can recommend an treatment plan specific for the needs of your child.

At UPMC Children's Hospital of Pittsburgh, we offer many different techniques to meet your child’s individual needs. These include open cranial vault remodeling procedures such as fronto-orbital advancements, minimally invasive choices, such as endoscopic strip craniectomies and spring-assisted surgery, and craniofacial distraction osteogenesis.

Open Cranial Vault Remodeling

For many children with non-syndromic, or isolated, craniosynostosis, open procedures are required to keep the brain healthy. In these procedures, your surgeon will remove a section of the skull before reshaping it and replacing it in a new position. This surgery is typically performed around 1 year of age.

Minimally invasive Craniosynostosis surgery

For some children with non-syndromic craniosynostosis, minimally invasive procedures may be offered to reshape the skull and provide room to the growing brain. These procedures are performed earlier through smaller incisions. They usually have less bleeding and discomfort, and require shorter time in the operating room and shorter stays in the hospital.  These techniques require either the placement of a helmet or springs to help reshape the skull after the surgery is over. After 6 months of age, your child is no longer able to have a minimally invasive procedure, so it is important to get referred at an early age if you have concerns.

Contact Us

Meet our team at UPMC Children's Hospital of Pittsburgh's Cleft-Craniofacial Center and learn about our treatment options, or contact UPMC Children's Hospital of Pittsburgh at 412-692-8650.