Scaphocephaly (Sagittal Craniosynostosis)

Scaphocephaly (pronounced ska-fuh-SEH-fuh-lee), also known as sagittal craniosynostosis, is a long and narrow head shape caused by skull bones fusing together before birth. Your baby’s head, when looking at it from the top, may look like an egg or boat-shaped, with a broader front that tapers to a rounded point.

Your baby’s skull is made up of several bones. Growth plates or sutures separate each bone. Sutures allow the skull to expand. This makes room for your baby’s growing brain.

In scaphocephaly, the sagittal suture, which runs front to back along the top of your baby’s head, closes too early. This suture usually starts closing when you’re 22, and completely closes between ages 30 and 50.

A healthcare provider may recommend surgery in your child’s first year to make room for your baby’s brain to grow.

Is scaphocephaly dangerous?

In some cases, yes. When your baby’s skull fuses together too soon, it prevents their skull from expanding side to side. As your child’s brain grows, the shape of their skull doesn’t grow with it. This may put pressure on their brain. If not treated, scaphocephaly can affect your baby’s brain growth and development.

How common is scaphocephaly?

Scaphocephaly is a type of craniosynostosis (a condition caused by skull sutures closing too early). Craniosynostosis affects an estimated 1 in 2,500 live births in the United States. Scaphocephaly (sagittal craniosynostosis) is the most common type. It affects 53% to 60% of all craniosynostosis cases.

Symptoms of scaphocephaly

The main sign is an abnormally shaped head. It becomes more obvious and misshapen as your child grows. You may notice a ridge along the top of their head where the bones closed instead of the typical soft spot (fontanelle) that most babies have.

Your child’s head isn’t round, as you’d typically expect it to be. Instead, when looking at the top of your child’s head, it’s narrow and long. It may look similar to an egg. Your child’s face will have a rounded shape, but the side of their head is longer and narrower at the back.

Scaphocephaly causes

Scaphocephaly happens when the sagittal sutures in your child’s skull close too soon. Your child is still growing after this part of their skull closes. This causes their head to have an abnormal shape.

The reason why this happens isn’t well understood, but research suggests environmental and genetic factors may cause it.

Healthcare providers identified the following genetic changes (variants) associated with scaphocephaly:

• SMAD6
• TWIST1
• TCF12
• ERF
• MSX2

Not all cases of scaphocephaly are inherited from a child’s biological parents. Genetic changes can happen randomly and unpredictably. An estimated 10% of cases have a known genetic cause.

Scaphocephaly may happen as a symptom of an underlying genetic condition, as well.

Risk factors for scaphocephaly

Biological parents may be more at risk of having a child with scaphocephaly if:

• The mother needs valproic acid during pregnancy
• The mother uses tobacco products during pregnancy
• The biological paternal age is over 40 years

Males are more likely to develop scaphocephaly than females.

What are the long-term effects of scaphocephaly?

If scaphocephaly is severe or left untreated, the pressure inside your child’s skull may increase. This happens because your child’s brain is still growing within an enclosed space (their skull). The pressure can damage your child’s brain and cause:

• Developmental delays
• Seizures
• Vision problems

How a doctor diagnoses scaphocephaly

A provider will usually diagnose scaphocephaly shortly after your child is born or during a routine well-baby visit. During a physical exam, your child’s provider will:

• Look at your baby’s face and head
• Feel the sagittal suture for a ridge instead of a soft spot
• Measure your baby’s head to determine the severity of the condition

Imaging tests of your child’s head — such as an X-ray, MRI or CT scan — can also help your child’s provider confirm the diagnosis.

How do I know if my baby has scaphocephaly?

Many parents see that something isn’t right with their baby’s skull because their head isn’t round. This is usually the first sign of the condition. When you touch your baby’s head, you may notice that they don’t have a soft spot and instead have a bumpy ridge where their skull bones closed.

If you think your child’s skull isn’t the right shape, talk to their healthcare provider.

How is scaphocephaly treated?

The main treatment for scaphocephaly is surgery. Scaphocephaly surgery can remove the fused suture or, in some cases, reconstruct your child’s skull. It’s usually very effective at restoring the shape of your child’s head and making room for brain growth. A surgeon may recommend a procedure between 3 and 6 months of age.

Your healthcare provider will help you decide if surgery is necessary for your child. This decision may depend on the following:

• Any symptoms or complications your child experiences
• Your child’s head shape and concerns about your child’s appearance
• The severity of the condition, which can range from mild to severe

Scaphocephaly surgery

There are three types of surgery options for scaphocephaly:

• Strip craniectomy (endoscopic surgery). This is a minimally invasive surgery that uses two small incisions and an endoscope (a surgical tool). Your surgeon removes a strip of bone, including the sagittal suture, and sometimes several strips on the side of your baby’s head (barrel staves) to help their brain expand.
• Spring-assisted surgery. This uses the strip craniectomy technique, where a surgeon removes a strip of bone from your baby’s skull and adds surgical-metal springs in the open space. This allows the gap in the skull to widen with brain growth gradually.
• Cranial vault remodeling (open surgery). This is a complex surgery that involves creating cuts in your baby’s skull and removing skull sutures. A surgeon will reshape your baby’s skull bones and put them back together with plates, screws, sutures and, sometimes, bone grafts to improve the shape of their head.

Depending on the type of surgery, your child may need to wear a medical helmet for up to one year. This guides their skull to grow in the correct shape.

What are the scaphocephaly surgery risks?

As with any type of surgery, risks are possible and may include:

• Air bubbles entering your child’s bloodstream (air embolism)
• Asymmetry, bone defects or bone irregularities
• Bleeding (wound dehiscence), excessive blood loss (requiring blood transfusion)
• Cerebrospinal fluid leak
• Infection
• Embolism
• Hematoma
• Needing a second surgery
• Seizures

While rare, these complications may be life-threatening and could lead to early death.

There are more risks associated with open surgery than endoscopic procedures.

Your child’s provider will explain these risks to you before the procedure. Your child’s surgical team is highly trained and experienced. They’ll take extreme caution to prevent complications and make sure your baby is safe.

When should I see a healthcare provider?

Let your baby’s healthcare provider know if the shape of your baby’s head isn’t round. Schedule an appointment if you notice your baby is missing developmental milestones for their age, like saying their first words, crawling or standing.

If you plan on expanding your family, talk to your healthcare provider about genetic counseling. A genetic counselor can test for specific genes associated with this condition. This will help you understand your risk of having a child with scaphocephaly.

What questions should I ask my healthcare provider?

If your child has scaphocephaly, you may want to ask their provider:

• Does my child need surgery?
• What are the risks of surgery?
• How do I take care of my child at home?
• What symptoms should I look out for?

What’s the outlook for scaphocephaly?

Scaphocephaly doesn’t get better on its own. Most children who need surgery receive it within their first year of life. Surgery is usually successful in improving your child’s head shape. But even with surgery, some children may experience developmental delays.

Early detection and treatment can lead to better outcomes for your child. A healthcare provider should monitor your child as they get older to ensure their head continues to grow as expected.

Early intervention services can help improve your child’s learning and functional abilities. These programs are available in every state in the U.S. They offer a variety of support services, including speech therapy and physical therapy.