What Is Metopic Craniosynostosis?

Published on August 09, 2021

Medically reviewed by Jonathan B. Jassey, DO

Metopic craniosynostosis is a rare form of the birth defect craniosynostosis. Craniosynostosis occurs when the flexible joints in a baby’s skull known as sutures fuse prematurely. These sutures usually stay flexible until your child’s second birthday and allow the brain to grow and develop.

Metopic craniosynostosis occurs when your child’s metopic suture fuses prematurely. The metopic suture runs from the nose up to the sagittal suture on the top of the head. This condition causes a misshapen skull and head, which may affect brain growth. A baby with metopic craniosynostosis usually has a triangular-shaped head, with the narrow part in the front and the broadest part in the back.

Metopic craniosynostosis can range from mild to serious, and may cause long-term complications. The cause is unclear, but the condition may be associated with a genetic disorder. Treatment for metopic craniosynostosis almost always requires surgery to correct the skull’s shape.

Mother holding baby in medical appointment — Fly View Productions / Getty Images

Types of Craniosynostosis

Metopic craniosynostosis is a rare type of craniosynostosis. There are other types of craniosynostosis, including:

Sagittal craniosynostosis: This is the most common type of craniosynostosis, and affects the sagittal suture that runs from the front to the back of the head. It results in a long, narrow head.
Coronal craniosynostosis: This type of craniosynostosis affects one or both of the coronal sutures that run from each ear to the top of the head. It causes the forehead to appear flattened and bulging on the affected side.
Lambdoid craniosynostosis: This is also a rare type of craniosynostosis, and affects the lambdoid suture. This suture runs along the back of the head, and premature fusing causes one side of the head to appear flat and one ear to be higher than the other.

Of all types of craniosynostosis, children with metopic craniosynostosis are at the highest risk of having neurodevelopmental problems. These delays may be noticeable right away or may not show up until a child starts school.

Metopic Craniosynostosis Symptoms

Metopic craniosynostosis causes the head to take a triangular shape, with the narrow point at the front and the broad side at the back. The front of the head will appear narrow, and the eyes will look close together. This triangular shape to the skull is known as trigonocephaly.

You may also notice a prominent ridge across your child’s forehead, while the back of their head appears flat.

In addition to physical symptoms, metopic synostosis is associated with an increased risk of neurodevelopmental delays.

Common symptoms of metopic craniosynostosis include:

A misshapen head and skull
A narrow brow with close-set eyes
A prominent forehead ridge
A flattened back of the head
A hard ridge along the metopic suture on the side of the head
Slowed head growth while the body continues to grow

Rare symptoms may include:

Sleepiness or fatigue
Irritability and crying
More prominent scalp veins
Poor feeding
Projectile vomiting

Causes

The cause of metopic craniosynostosis is often not known and thought to be random. Craniosynostosis is most likely caused by a combination of both genetic and environmental factors.

Risk factors associated with craniosynostosis include:

Multiple babies in one pregnancy, such as twins or triplets
Large head size in utero
Maternal thyroid disease
Maternal smoking
Fertility treatments such as Clomid (clomiphene citrate)
Maternal use of anticonvulsant drugs such as Valproate

Metopic craniosynostosis is sometimes a symptom of a genetic disorder. For example, C syndrome, also known as Opitz trigonocephaly syndrome, causes the fusion of the metopic suture, leading to metopic craniosynostosis.

Diagnosis

Metopic synostosis is often diagnosed at birth, but may not be detected until later on in your child’s first year. Your doctor can diagnose metopic craniosynostosis with a physical exam, and may order a computed tomography (CT) scan to confirm the diagnosis.

During the physical exam, your doctor will carefully inspect your child’s head and feel for a hard, bony ridge along the metopic suture. Your doctor will also feel your child’s soft spot, or fontanel, on the top of their head. Studies have found that the anterior fontanel closes in 55% of metopic craniosynostosis cases.

Your doctor will also ask detailed questions about your family history, pregnancy, and delivery. It may be helpful to keep a list of any family members who have been diagnosed with craniosynostosis, a skull malformation, or a genetic disorder.

A Misshapen Head Does Not Always Mean Craniosynostosis

A much more common cause of a broad, flat back of the head is positional plagiocephaly. This benign condition is caused by your baby lying in the same position for long periods of time. A baby’s flexible skull bones start to flatten on the affected side, usually the back of the head. This condition can be treated with a molding helmet.

Once your doctor has conducted a thorough physical and history, they may order a CT scan of your baby's head. A CT scan with 3D reconstruction is considered the most accurate way to diagnose metopic craniosynostosis. This test can show your child’s metopic suture, as well as any abnormalities in the brain.

If your doctor suspects that your child’s craniosynostosis is part of a genetic disorder, they will refer you for further testing.

Treatment

Metopic craniosynostosis causes the skull to form a triangular shape and needs to be treated with surgery. There are two surgical treatment options for craniosynostosis: endoscopic craniosynostosis surgery and calvarial vault remodeling.

During endoscopic craniosynostosis surgery, tiny incisions are made in your baby's scalp and a small tube called an endoscope is used to move the skull bones. Because metopic craniosynostosis causes such a severe malformation of the skull, this type of surgery is often not effective at correcting the skull’s shape.

Instead, metopic craniosynostosis usually requires calvarial vault remodeling. During this six-hour surgery, the surgeon makes an incision in your baby's scalp and moves the skull bones into a more circular shape. This surgery can result in blood loss, so your little one may need a blood transfusion during and after the surgery. Calvarial vault remodeling can be performed at between 6 months and 12 months of age because the skull bones are thick enough at this point to be moved and hold their new shape.

Timeline

Metopic craniosynostosis is usually diagnosed at birth or shortly afterward. Once diagnosed, your doctor will discuss a treatment timeline with you. Surgery will most likely occur shortly after your little one turns 6 months old.

Early diagnosis and treatment are critical for the best outcome for a baby with metopic craniosynostosis. This is because metopic craniosynostosis can lead to problems with vision, learning, and behavior, especially when left untreated.

After surgery, a typical schedule for follow-up appointments is as follows:

Three weeks post-surgery
Twice per year from ages 2 to 6 years
Every three years until age 18 years

Prognosis

Of all types of craniosynostosis, children with metopic craniosynostosis are at the highest risk of having developmental delays. They may be noticeable right away or may not show up until a child starts school.

Early diagnosis and treatment are important for treatment success. When metopic craniosynostosis is left untreated, babies can experience increased intracranial pressure and long-term complications, including:

Head or facial abnormalities
Breathing problems
Vision problems
Seizures
Low self-esteem

Coping

Metopic craniosynostosis is an overwhelming diagnosis for any parent to hear, and it is vital to find ways to cope with the stress and uncertainty of caring for an ill child. Studies have found that having a child with craniosynostosis is very stressful for parents, especially when the skull malformation is noticeable to others.

There are concrete steps parents can take to relieve some of the stress of caring for a child with craniosynostosis. Because it can be difficult to remember all the information shared at medical appointments, take notes each time you meet with your doctor. It may be helpful to write down your questions ahead of time.

Most of the parents surveyed in a 2020 study said that they were very interested in connecting with other families dealing with the same diagnosis in their child. Talk with your medical team about how to find a local support group or online community.

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Summary

Metopic craniosynostosis is a rare type of craniosynostosis, a condition in which sutures in a baby's skull fuse together prematurely. The defining feature of a child with this condition is a triangular-shaped head, with the narrow part at the front and the wide part at the back. The treatment for this condition is surgery, and the earlier it's treated, the better the outcome is for the child.

Frequently Asked Questions

What is metopic craniosynostosis?

Metopic craniosynostosis is a form of craniosynostosis, a birth defect that affects the flexible joints in a baby’s skull called sutures. Metopic craniosynostosis occurs when the metopic suture in the skull fuses prematurely, leading to a triangular shape of the head. The metopic suture runs from the nose to the top of the head. This condition can limit the room for your baby's brain to grow and cause developmental delays.

How is surgery done for metopic craniosynostosis?

The goal of craniosynostosis surgery is to correct the shape of the skull while creating enough room for your baby's brain to grow. Surgery for metopic craniosynostosis is done by making an incision (or several small ones) in your child’s scalp and then physically moving the skull bones into a more circular shape. This procedure is called calvarial vault remodeling.

When should you see the doctor for metopic craniosynostosis?

It’s best to see a doctor as soon as you suspect that there is something wrong with the shape of your child’s head. Check to see if your baby's head looks triangular and if they have a prominent ridge on their forehead, since these are signs of metopic craniosynostosis.

How common is metopic craniosynostosis surgery?

Most babies with metopic craniosynostosis need surgery to correct the condition. Because the triangular shape of the skull does not allow for brain growth, it is important to correct the skull’s shape with surgery. Surgery is typically recommended after a child turns 6 months old.

A Word From Verywell

Metopic craniosynostosis is a serious birth defect that causes the metopic suture in your baby’s skull to fuse prematurely. When this happens, the skull grows into a triangular shape and does not allow for brain growth. Metopic craniosynostosis is usually treated with surgery to correct the skull’s shape and relieve any increased pressure on the brain.

This is a scary diagnosis for parents to hear. It’s helpful to remember that metopic craniosynostosis is treatable with surgery. It will be helpful to stay in close contact with your medical team and to connect with a support system during this process.

13 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

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By Carrie Madormo, RN, MPH
Carrie Madormo, RN, MPH, is a health writer with over a decade of experience working as a registered nurse. She has practiced in a variety of settings including pediatrics, oncology, chronic pain, and public health.

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