Scaphocephaly (Sagittal Craniosynostosis)

What is scaphocephaly?

Scaphocephaly, also known as sagittal craniosynostosis, is a birth defect that affects your baby’s skull. Craniosynostosis means skull bones fuse together before birth. This may create pressure as the brain grows and cause your baby’s head to become misshapen. If not treated, scaphocephaly can affect brain growth and development.

Your baby’s skull is made up of several bones. The bones of their skull are separated by growth plates, or sutures. Sutures allow their skull to expand to make room for your baby’s growing brain.

In scaphocephaly, the sagittal suture, which runs front to back along the top of their head, closes too early. Fusion of their sagittal suture prevents expansion of their skull from side to side. As their brain grows, their skull becomes long and narrow.

Are there other types of craniosynostosis?

Sagittal craniosynostosis (scaphocephaly) is the most common type of craniosynostosis. Other types can occur depending on which sutures fuse together:

• Coronal craniosynostosis: The coronal sutures run from each ear to the top of the head. Craniosynostosis can occur on one (unicoronal) or both sides (bicoronal). Babies with coronal craniosynostosis can develop a flattened forehead and raised eye socket on the closed side. Bicoronal craniosynostosis causes a broad, short head (brachycephaly).
• Lambdoid craniosynostosis: The lambdoid suture is on the back of the head. If this suture fuses, the back of your baby’s head can become flattened. This can occur on one side or bother sides. Bilateral lambdoid craniosynostosis is very rare and the least common form of craniosynostosis.
• Metopic craniosynostosis: The metopic suture travels from the nose to the sagittal suture. In metopic craniosynostosis, your baby’s head can take on a triangular shape — narrow in the front and flat in the back.

How common is scaphocephaly?

Estimates of how often craniosynostosis occurs range from 1 in 200 to 1 in 2,500 births. Sagittal craniosynostosis (scaphocephaly) accounts for over half of these cases.

One study found that 80% of babies with scaphocephaly were assigned male at birth (AMAB).

What are the symptoms of scaphocephaly?

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

If the condition is severe or left untreated, the pressure inside of your child’s skull may increase. Studies show this happens in about 15% of cases. An increase in pressure can damage their brain and cause:

• Developmental delays.
• Seizures.
• Vision problems.

What causes scaphocephaly?

Usually, the cause is unknown. Healthcare providers think it’s due to a combination of genetic changes (mutations) and environmental factors. Genetic changes can happen randomly or run in families. Other factors that may be associated with scaphocephaly include:

• Fertility medication, such as clomiphene citrate.
• Obesity of the mother.
• Older age of the father.
• Smoking during pregnancy.
• Thyroid disease treatment during pregnancy.

Sometimes, scaphocephaly is part of a genetic syndrome. This is an inherited group of signs and symptoms that usually occur together. Most genetic syndromes are rare.

How is scaphocephaly diagnosed?

Diagnosis usually occurs shortly after birth during a routine well-baby visit or if a parent raises concerns.

To check your baby for this condition, your healthcare 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, such as an X-ray or CT scan, of their head can also help your healthcare provider confirm the diagnosis.

How is scaphocephaly treated?

Scaphocephaly doesn’t get better on its own. The main treatment is surgery to remove the fused suture or in some cases, surgical remodeling of the skull. This reduces pressure on your child’s brain and allows their skull and brain to grow.

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

• Developmental delays your child is experiencing.
• Head shape and the desire for a better cosmetic appearance.
• Severity of the condition, which can range from mild to severe.

Most children who need surgery receive it within the first year of life. The two types of surgical treatment for scaphocephaly are:

• Strip craniectomy: This is a minimally invasive endoscopic surgery that uses two small incisions. Your surgeon removes a strip of bone, including the sagittal suture, and sometimes several strips on the side of your baby’s head to help their brain expand (barrel staves).
• Cranial vault remodeling: This is a complex surgery that involves creating cuts in your baby’s skull and removal of some of the bones in pieces. Their skull bones are reshaped and put back together with plates, screws, sutures, and sometimes additional bone grafts, to improve the shape of their head.

Depending on the type of surgery, your child may wear a medical helmet for up to one year to direct skull growth.

How can I prevent scaphocephaly?

As healthcare providers aren’t sure what causes this condition, you can’t prevent it.

What is the outlook for scaphocephaly?

Surgery is usually successful in improving your child’s head shape. But even with surgery, some children may experience developmental delays. Children should be monitored as they grow to ensure their heads continue to grow appropriately.

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

When should I talk to a healthcare provider?

Let your baby’s healthcare provider know if the shape of your baby’s head looks abnormal or your baby doesn’t seem to be developing correctly. Early detection and treatment of this condition can lead to better outcomes for your child.

If you have a child with scaphocephaly and are planning to have more children, 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 another child with scaphocephaly.

A note from Cleveland Clinic

Scaphocephaly occurs when the sagittal growth plate on the top of your child’s head closes too early. This interferes with brain and skull growth and causes your child to have an abnormal head shape. Some children also experience developmental delays. The main treatment is surgery, which is usually very effective at restoring the shape of their head and allowing room for brain growth. It’s preferable for children to have surgery between three and six months of age. After treatment, your child may continue to have learning disabilities. Programs such as early intervention can help your child overcome these challenges.