Metopic Synostosis (Trigonocephaly)

What is metopic synostosis?

Did you know that your baby’s skull isn’t a single, solid entity — it’s actually made up of several bony plates? Between those plates are fibrous joints called sutures.

Normally, the sutures in a developing infant’s skull fuse in a gradual process over time. However, sometimes the fusing occurs too early. This is a medical problem known as craniosynostosis.

One type of craniosynostosis is called metopic synostosis (also referred to as trigonocephaly or metopic suture craniosynostosis). A prominent ridge along the forehead by itself is often a normal finding, but children with metopic synostosis from premature fusing of the metopic suture have a triangular shape to the forehead. To summarize with metopic synostosis:

They have a noticeable ridge along their foreheads.
They will also have a pointed, almost triangular shape to the front and top of their skulls and eyes that appear too close together.

As with any type of craniosynostosis, metopic synostosis can carry a risk of other complications, but it’s important to remember that every child is different, and the condition can vary widely in its severity.

Here are a few more important facts about metopic synostosis:

Metopic synostosis can be quite mild in some children and fairly serious in others.
There is no single proven cause for metopic synostosis.
Metopic synostosis is often noticeable at birth, but can also become apparent over time in older infants.
Metopic synostosis and other types of craniosynostosis should not be confused with plagiocephaly — a different condition that is associated with the baby’s position during sleep.
Children with more serious instances of metopic synostosis can experience problems with vision, learning, and behavior.
Some children with just a ridge or mild metopic synostosis don’t need any medical treatment. For those who do, surgery has proven to be a successful approach.

How Boston Children’s Hospital approaches metopic synostosis

Boston Children’s treats hundreds of patients with metopic synostosis and other types of craniosynostosis every year in our Craniofacial Program.

Drawing on our extensive experience treating these disorders in young patients, we will use a multidisciplinary approach to ensure the right treatment for your child's specific symptoms and circumstances.

If, after evaluation, your child is determined to need treatment for his metopic synostosis, members of his Craniofacial Program care team may include:

neurosurgeons, who perform surgical procedures on a child’s brain, spinal cord or nerves
plastic surgeons, who diagnose and surgically correct skeletal abnormalities of the skull, facial bones, and soft tissue
neurologists, who diagnose, treat, and manage conditions affecting the nervous system
oral and maxillofacial surgeons, whose surgical procedures to correct injuries or deformities of the mouth, jaws, face, and skull
audiologists, who diagnose, evaluate, and implement treatment approaches for a child’s hearing problems
neuroscience nursing professionals, who combine expertise in all aspects of pediatric nursing with a focus on the neurosciences, and act as liaisons between a child’s family and clinical treatment team
speech therapists, who evaluate a child’s communicative abilities and formulate treatment plans to enhance and improve speech and language
physical therapists, who help children recover, return to their previous level of function, or learn to work within their functional limitations
dentists, who maintain a child’s oral health
orthodontists, who evaluate the position and alignment of a child’s teeth and help design corrective treatment plans as needed
otolaryngologists, who are “ear-nose-throat specialists” who help manage a child’s ear infections, hearing loss, and other related issues that may be tied to a craniofacial abnormality
ophthalmologists, who evaluate vision problems and formulate medical or surgical treatment plans to improve or restore vision
psychiatrists, who assess a child’s psychosocial functions and behavioral development and can prescribe psychiatric medications if necessary
psychologists and social workers, who help children and families express and cope with feelings like worry, fear, frustration, anger and sadness

Working together, our team will develop a customized treatment plan that meets your child's physical, emotional, and social needs — and one that involves you and your family at every step of the way.

Metopic synostosis: Reviewed by Mark R. Proctor, MD

© Boston Children’s Hospital; posted in 2012

Metopic Synostosis (Trigonocephaly) | Symptoms & Causes

Before learning more about metopic synostosis, it’s helpful to understand the anatomy of a baby’s skull.

Normally:

An infant’s skull has several plates of bone that are separated by fibrous joints, called sutures.
The sutures gradually close as the child grows and develops.
When the sutures close, the skull is fully formed as a solid piece of bone.

When a child has metopic synostosis:

The metopic suture — the joint that runs from the baby’s fontanel (the “soft spot” at the top of the head) down the forehead to the top of her nose — closes too early.
The baby develops a noticeable ridge extending along the center of her forehead.
Her forehead will look overly narrow.
Her eyes may be spaced too closely together.
The front of her skull may appear pointed and rather “triangular.”

Some children have very mild cases of metopic synostosis that do not require specific treatment. In more serious cases, however, the condition can cause:

developmental delays
learning and behavioral problems
vision problems

Surgery has proven to be a beneficial treatment for children whose metopic synostosis necessitates medical intervention.

What causes metopic synostosis?

In most children, metopic synostosis happens without any identifiable reason.

Sometimes, however, metopic synostosis occurs as a component of a rare genetic syndrome. The following disorders have been linked to metopic synostosis:

Baller-Gerold syndrome, which also causes abnormalities in the bones of the arms and hands
Jacobsen syndrome, which results from missing material within a certain chromosome
Muenke syndrome, caused by a mutation in the gene that produces a protein responsible for brain and bone tissue health
Opitz syndrome, which causes several birth defects affecting the face, heart and larynx
Say-Meyer syndrome, which is characterized by developmental delays, problems with motor skills and a short stature

What are the symptoms of metopic synostosis?

Children with metopic synostosis have visible symptoms that include one or all of the following:

A noticeable ridge running down the middle of the forehead
An overly narrow, triangular shape to the forehead and top of the skull
Eyes that appear too close together

Frequently asked questions

Will my child be OK?

The severity of metopic synostosis can vary widely, from mild and barely noticeable to serious and with several complications.

If your child has mild metopic synostosis or just a metopic ridge, he may have no symptoms beyond a visible ridge in the middle of his forehead, and might not need any medical treatment.

However, more serious cases of metopic synostosis can cause complications with:

eyesight
learning and behavior

Your child’s treating physician will explain the extent of his condition and make specific recommendations for best next steps.

At what age does metopic synostosis tend to develop?

Metopic synostosis is almost always noticeable at birth, but some children—especially those with very mild symptoms—might not be diagnosed until later in infancy.

Is my baby going to need surgery?

That depends on his symptoms and the degree of problems they are causing.

For example, if he only has a noticeable ridge on his forehead but no other symptoms, he probably won’t need any medical treatment at all. But if he has more extensive difficulties, he may need surgery to prevent further problems with his brain and skull growth.

Here at Boston Children’s Hospital, our clinicians have extensive experience performing surgeries for metopic synostosis and all types of craniosynostosis. The vast majority of children who have these procedures go on to lead normal, active lives.

Questions to ask your doctor

You and your family play an essential role in your child’s treatment for metopic synostosis. It’s important that you share your observations and ideas with your child’s treating physician, and that you have all the information you need to fully understand the treatment team’s explanations and recommendations.

You’ve probably thought of many questions to ask about your child’s metopic synostosis. It’s often very helpful to jot down your thoughts and questions ahead of time and bring them with you, along with a notebook, to your child’s appointment. That way, you’ll have all of your questions in front of you when you meet with your child’s treating clinician and can make notes to take home with you.

Some questions to ask your doctor might include:

How did you arrive at this diagnosis?
Are there any other conditions my child might have in addition, or instead?
How advanced is my child’s metopic synostosis?
Is he going to need medical treatment?
Is surgery necessary?
What is the long-term outlook for my child?
Will he need support for any related medical problems?
Do I need to make any changes to my child’s daily routines?
How should I explain my child’s condition to others?
What other resources can you point me to for more information?

Metopic Synostosis (Trigonocephaly) | Testing & Diagnosis

Tests

At Boston Children’s Hospital, we know that the first step to treating your child’s metopic synostosis is to form a complete and accurate diagnosis.

How is metopic synostosis diagnosed?
Children with metopic synostosis usually display visible symptoms at birth, namely:

a ridge running down the forehead
a narrow, triangular shape to the forehead and top of the skull
sometimes, eyes that are spaced too closely together

Other times, a child’s metopic synostosis is diagnosed later in infancy during a routine physical examination.

Once an initial finding of metopic synostosis is made, your clinician may take the following steps to confirm the diagnosis:

obtaining a full family medical history
determining whether your child is experiencing any developmental delays or other problems in meeting age-appropriate milestones
measuring the circumference of your child’s head and plotting these measurements on a graph to detect unusual patterns
ordering a computed tomography (CT) scan, a non-invasive procedure using x-ray equipment and powerful computers to create detailed images of the skull

After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child’s condition. Then we will talk with you and your family to outline the best treatment options.

Metopic Synostosis (Trigonocephaly) | Treatments

Boston Children's Hospital has been a worldwide innovator in diagnosing and treating children with metopic synostosis and all types of craniosynostosis for decades. Clinicians in our Departments of Neurosurgery and Plastic and Oral Surgery have extensive experience in treating all forms and degrees of the condition.

We use minimally invasive techniques — medical and surgical procedures that use small incisions and miniaturized cameras and tools — whenever we can. In fact, Boston Children's is a global leader in creating and refining new surgical approaches.

Treatment: Not always necessary

A child with mild metopic synostosis may have no symptoms beyond a noticeable ridge down the middle of his forehead. In these cases, doctors may decide no medical treatment is needed.

Surgery

Many children with moderate to severe metopic synostosis will require surgical intervention.

Surgery for metopic synostosis:

is designed to correct deformities in the facial and skull bones
often works best in children who are less than 1 year old, since the bones are still soft and pliable, making them easier to manipulate
may be recommended in much younger infants, including newborns, depending on the severity of their symptoms
can also be performed in older children (although the older the child, the more complex the surgery)
is usually complete after a single surgery (but in some cases, may need to be repeated over time, depending on the seriousness of the condition)

Before the operation, your child's surgeon will:

explain the reasons to consider surgery
explain the steps involved in the operation
review aftercare instructions, including warning signs of possible complications

During surgery, the surgeon and treatment team will:

in the case of minimally invasive surgery, use endoscopy — a long, thin tube with an accompanying light and camera—to release the sutures that are abnormally fused
in the case of reconstructive surgery, reposition the skull bones to allow for normal growth and development

Monitoring and follow-up

After surgery, your child:

will stay in the hospital for one to five days
may have visible swelling for a few days to a few weeks
may need to wear a dressing covering her head, protecting the incision site from contamination and infection
will need to return for check-ups and evaluations to measure how well the skull, facial bones, and brain are developing

Coping and support

When your child has metopic synostosis, your family may have many concerns and questions. Not only are you focused on meeting all of your child's medical needs: You are also grappling with a significant emotional and psychological toll that can affect every member of the family.

In addition to the clinical information offered on this page, Boston Children's has several other resources designed to give your family comfort, support and guidance:

Patient and family resources at Boston Children's

Boston Children's Behavioral Medicine Clinic helps children who are being treated on an outpatient basis at the hospital — as well as their families — understand and cope with their feelings about:

being sick
facing uncomfortable procedures
handling pain
taking medication
preparing for surgery
changes in friendships and family relationships
managing school while dealing with an illness
grief and loss

The Experience Journal was designed by Boston Children's psychiatrist-in-chief David DeMaso, MD, and members of his team. This online collection features thoughts, reflections and advice from kids and caregivers about living with medical conditions and going through treatment.

Boston Children's Psychiatry Consultation Service is comprised of expert and compassionate pediatric psychologists, psychiatrists, social workers and other mental health professionals who understand the unique circumstances of hospitalized children and their families. The team provides several services, including:

short-term therapy for children admitted to one of our inpatient units
parent and sibling consultations
teaching healthy coping skills for the whole family
educating members of the medical treatment team about the relationship between physical illness and psychological distress
talking to your child about her condition
preparing for surgery and hospitalization
supporting siblings
taking care of yourself during your child's illness
adjusting to life after treatment

Boston Children's Hale Family Center for Families is dedicated to helping families locate the information and resources they need to better understand their child's particular condition and take part in their care. All patients, families, and health professionals are welcome to use the Center's services at no extra cost. The center is open Monday through Friday from 8 a.m. to 7 p.m. and on Saturdays from 9 a.m. to 1 p.m. Please call 617-355-6279 for more information.

The Boston Children's Hospital chaplaincy is a source of spiritual support for parents and family members. Our program includes nearly a dozen clergy members—representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian, and United Church of Christ traditions — who will listen to you, pray with you, and help you observe your own faith practices during your child's treatment.

Global Services is a dedicated resource for patients and families from countries outside the United States. The center can provide assistance with everything from reviewing medical records to setting up appointments and locating lodging. Contact the center by phone at +1-617-355-5209 or via e-mail at international.center@childrens.harvard.edu.

Helpful links

Please note that neither Boston Children's Hospital nor the Craniofacial Anomalies Program at Boston Children's unreservedly endorses all of the information found at the sites listed below. These links are provided as a resource.

General information for Children's patients and families
Read our comprehensive guide.

Metopic Synostosis (Trigonocephaly) | Research & Clinical Trials

At Boston Children’s Hospital, our care is informed by our research, and our discoveries in the laboratory strengthen the care we provide at each child's bedside. In fact, Boston Children’s scientific research program is one of the largest and most active of any pediatric hospital in the world.

Among our research efforts with promise for treating metopic synostosis and other types of craniosynostosis are:

Mark Proctor, MD, Boston Children’s neurosurgeon, has authored several key papers about endoscopic approaches to the treatment of craniosynostosis. In addition, he was the lead neurosurgeon involved in developing formal parameters of care for the condition.
John Meara, MD, DMD, MBA, Boston Children's plastic surgeon-in-chief, is analyzing the genetic, phenotypic, cognitive and behavioral aspects of craniosynostosis.
Bonnie Padwa, MD, PhD, Boston Children's oral surgeon-in-chief, is assessing facial growth patterns in children with several craniofacial anomaly types.

Clinical trials

Boston Children’s is known for pioneering some of the most effective diagnostic tools, therapies and preventive approaches in pediatric medicine. A significant part of our success comes from our commitment to research — and to advancing the frontiers of mental health care by conducting clinical trials.

Boston Children’s coordinates hundreds of clinical trials at any given time. Clinical trials are studies that may involve:

evaluating the effectiveness of a new drug therapy
testing a new diagnostic procedure or device
examining a new treatment method for a particular condition
taking a closer look at the causes and progression of specific diseases

While children must meet strict criteria in order to be eligible for a clinical trial, your child may be eligible to take part in a study. Before considering this option, you should be sure to:

consult with your child’s treating physician and treatment team
gather as much information as possible about the specific course of action outlined in the trial
do your own research about the latest breakthroughs relating to your child’s condition

If your physician recommends that your child participate in a clinical trial, you can feel confident that the plan detailed for that study represents the best and most innovative care available. Taking part in a clinical trial at Boston Children’s is entirely voluntary. Our team will be sure to fully address any questions you may have, and you may remove your child from the medical study at any time.