Scaphocephaly occurs when the sagittal growth plate of your child’s skull closes before birth. It causes abnormal growth of the brain and skull and developmental disabilities in some children. Surgery is the main treatment and is effective at restoring the shape of the head. Most children receive surgery before the age of one.
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.
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Sagittal craniosynostosis (scaphocephaly) is the most common type of craniosynostosis. Other types can occur depending on which sutures fuse together:
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).
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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:
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:
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.
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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:
Imaging tests, such as an X-ray or CT scan, of their head can also help your healthcare provider confirm the diagnosis.
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:
Most children who need surgery receive it within the first year of life. The two types of surgical treatment for scaphocephaly are:
Depending on the type of surgery, your child may wear a medical helmet for up to one year to direct skull growth.
As healthcare providers aren’t sure what causes this condition, you can’t prevent it.
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.
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.
Last reviewed on 05/14/2022.
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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy