Crouzon Syndrome

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What is Crouzon syndrome?

Crouzon syndrome is a rare form of — the early closing of 1 or more of the soft, fibrous seams (sutures) between the skull bones. Crouzon is pronounced kroo-ZAHN. Crouzon syndrome affects about 1 out of every 60,000 newborns.

Because some of the bones of the skull and the face fuse early, the bones cannot grow in the typical way. Mainly, the skull, the middle of the face and the eye sockets are not typical.

This often leads to breathing problems as your child grows. It also affects the shape of their head, how their face looks and how teeth in the upper and lower jaws line up.

People with Crouzon syndrome usually have typical intelligence.

Children with Crouzon syndrome need coordinated care by providers from many areas of healthcare. At Seattle Children’s Craniofacial Center, our team of experts covers 19 different specialties.

What causes Crouzon syndrome?

Crouzon syndrome happens because of a change (variant) in a that is important in forming bone. Usually, the change affects the FGFR2 gene (fibroblast growth factor receptor 2). A form of Crouzon syndrome that affects the skin is caused by a mutation in the FGFR3 gene. That form is called Crouzon syndrome with .

Often, the condition is caused by a new genetic change that was not inherited from a parent. Other times, a parent passes the gene variant to a child.

Crouzon Syndrome Care at Seattle Children's

When it comes to diagnosing and treating craniofacial conditions, our team is among the most experienced in the world. No matter how rare the condition or syndrome, we have cared for someone like your child.

Symptoms of Crouzon Syndrome

Drawing of young child showing coronal sutures in the skull. Drawn by Raymond Sze

Crouzon syndrome often affects the coronal sutures that go over the top of the head from ear to ear. But other sutures also may close early.

Usually, these sutures let the skull expand as the brain grows. During childhood, the sutures slowly grow together to fully connect the skull bones. The different sutures close at different times. The process continues until adulthood.

In Crouzon syndrome, the early closing of a suture usually happens before birth. But it can happen later when a child is 2 or 3 years old.

If a suture closes early, it changes the shape of the head and reduces room for the growing brain. This can increase pressure inside your child’s skull.

Symptoms of the face and head
The facial features of babies with Crouzon syndrome vary widely.

You may see these differences in your child:
- A taller head shape that is short from front to back
- Wide-set eyes that seem to stick out because their eye sockets are shallow
- Eyes that do not line up
- Smaller middle of the face (midface) compared to the rest of the face
Changes to the head as your child grows

The differences in your child’s face and head will likely get easier to notice as they grow.

Over time, their upper jaw may look smaller and their lower jaw may stick out (underbite). This is because bones in the middle of their face grow slower than other parts of their head.

The roof of their mouth (palate) may be narrow. This can crowd their upper teeth.

If the sutures don’t close until your child is age 2 to 3 years, they may have a small upper jaw, headaches or vision changes.
Changes in other parts of the body

Many children with Crouzon syndrome have hearing loss. In some babies, the ear canals are narrow or missing.

Unlike other craniosynostosis syndromes, Crouzon syndrome rarely involves the hands and feet, other than some children having broad thumbs and big toes. Rarely, there may be problems in the neck (cervical spine), elbows, bones or internal organs.

About 5% of people with Crouzon syndrome develop a skin condition called acanthosis nigricans. They have patches of darker, thicker (velvety-feeling) skin. The patches may be on their neck and eyelids and around their mouth and other areas of thin skin. This form of Crouzon syndrome is caused by genetic changes in the FGFR3 gene.

How is Crouzon syndrome diagnosed?

To diagnose this condition, your doctor will examine your child’s skull carefully. The shape will help the doctor tell if any sutures have closed too soon, and the facial features will help tell if they have Crouzon syndrome or another condition.

A CT (computed tomography) scan of your child’s head can give the doctor more information and help us plan the best treatment for your child.

Genetic counseling and testing

Our Craniofacial Genetics Clinic helps identify conditions caused by changes in genes. Our geneticists and genetic counselors can help you understand the pros and cons of genetic testing. They can explain test results and your chance of having a child with Crouzon syndrome in a future pregnancy.

Our genetic counselor will give you information about your child’s condition. Counseling can help you make informed decisions about family planning and your child’s treatment.

Our genetic counselors also advise people with Crouzon syndrome about their chance of having a child with the condition. A person with Crouzon syndrome has a 50% chance in each pregnancy of passing down the gene variant that causes the condition.

How is Crouzon syndrome treated?

There is no single care plan for Crouzon syndrome. The treatments and timing we recommend depend on how the syndrome affects your child. Based on your child’s needs, our specialists provide a wide range of services to help with breathing, brain health, vision, teeth and bite alignment, hearing and speech.

Our goal is to minimize the number of steps in the treatment plan by improving the outcome of each step.

3-D imaging is a powerful tool that helps us predict future growth. Our surgery and orthodontic corrections take into account continued growth of your child’s bones. This way, when your child is finished growing, their bones are in the desired position.

Easing breathing

Making sure your child is breathing well is an immediate priority. Differences in the bones in the jaw or midface or an atypical windpipe can make it hard to breathe.

Children with severe breathing difficulties may need a tube placed in their windpipe (tracheostomy) soon after birth.

Later, your child will likely have surgery to change the position of their jaw and face bones to help with breathing and other problems.

Some children with Crouzon syndrome have trouble breathing while they sleep (obstructive sleep apnea). Over time, this may lead to problems with how the heart and lungs work. It also can play a part in problems with learning and behavior.

We will ask questions during clinic visits to check if this may be a problem. To better understand their breathing patterns, we may do an overnight sleep study (polysomnogram) in our sleep lab.
A healthy brain with room to grow
Most children with Crouzon syndrome will need surgery to expand and reshape their skull.

Most children with this condition have early closure of a skull suture at birth or in infancy. In other children, problems with fused skull bones happen later.

We check your child regularly and recommend surgery as needed. A craniofacial and work as a team in the operating room to protect your child’s brain and get the best results for your child.
- Most children who need surgery to expand their skull will have a procedure called fronto-orbital advancement.
- Less often, we recommend posterior cranial vault distraction.
- In severe cases, a child will need a procedure called monobloc frontofacial advancement to reposition the forehead and midface.
The procedure and timing of surgery will depend on which sutures have closed and how many are affected. If more than 1 suture is affected, your baby may need surgery in their first months of life. Some children who have early surgery will need a follow-up operation, but this is not common.

Some children with Crouzon syndrome need surgery because their brain is pressing on the base of their skull. This happens if a child has a Chiari malformation (problem with how the brain sits in the skull).

Checking fluid build-up and pressure in the skull

About 3 out of every 10 children with Crouzon syndrome have extra fluid in the skull (hydrocephalus). Symptoms in infants include:
- A bulging soft spot (anterior fontanelle)
- Rapidly increasing head size
- Acting cranky or sluggish
Older children may have:
- Headaches (worse in the morning)
- Vomiting
- Poor vision
- Declining school performance.
Your child’s team will check for signs of fluid build-up and increased pressure in the skull. These problems can happen during infancy or as your child grows.
- If fluid collects in your child’s brain, they may need a tube (shunt) inserted to drain away extra fluid.
- At regular checkups, we ask about warning signs of increased pressure, such as headaches or problems with eyesight. If there are concerns, your child may have a CT scan or a special eye exam to check for swelling at the back of the eye.
Midface surgery to improve breathing
In many children with Crouzon syndrome, their nose is small and the bones in the middle of the face (midface) do not grow right. Their eyes are not protected because their cheekbones and forehead are too small. They may have trouble breathing, especially while they sleep.

For some children, surgery to remove adenoids or lymphoid tissue in the back of the nose (adenoidectomy) will improve their breathing.

Most children need Le Fort III midface advancement surgery combined with distraction osteogenesis to bring forward the jaw and other bones of the middle face.

Le Fort III midface advancement with distraction

Le Fort III midface advancement with distraction enlarges your child’s airway and helps their jaws fit together better. It also creates larger cheekbones to protect their eyes.

Seattle Children’s pioneered midface distraction in the Northwest. We perform this surgery more often than most centers in the country. Our method results in less scarring and a shorter distraction time.

BEFORE (Above, left): The middle of this girl’s face did not grow well because of Crouzon syndrome. This caused severe breathing problems.

DISTRACTION (Above, center): During surgery to move her face bones, the surgeon attached a device called a distractor. While she recovered at home, the distractor was adjusted to move the bones slowly apart. New bone grew to fill in the gaps.

AFTER (Above, right): After the procedure, the child has a larger airway so she can breathe during sleep. Her cheekbones are larger, and her teeth fit together better.

Timing of surgery depends on your child’s needs

The timing of midface advancement is based on whether your child has:
- Chewing and speech difficulties because their jaws don't align
- The need for eye protection
- Breathing problems while asleep
Most often, children with sleep apnea have the surgery at 7 to 9 years old to improve breathing. If your child has severe breathing problems, they may need a Le Fort III distraction as early as 3 years old.
Protecting eyesight
An eye doctor (ophthalmologist) will check your child regularly for problems with their eyesight.
- If your child has very shallow eye sockets, their eyes may stick out (proptosis) and be at greater risk of injury.
- Your child’s eyes may not align in the typical way because of the position or shape of the eye sockets. This can affect their vision.
- Increased pressure inside the skull can also cause problems with your child’s eyesight.
- Some children with Crouzon syndrome cannot close their eyes completely. That can irritate the covering on the front of their eyes (corneas). The condition is called keratitis. To avoid harm to their vision, your child may need surgery before they are 2.
The best procedure to protect their eyes depends on your child:
- Le Fort III midface advancement to move the middle part of the face forward. It creates larger cheekbones that protect the eyes. This is done at age 8 to 10.
- Fronto-orbital advancement to bring the forehead and upper eye sockets forward to help protect the eyes. This surgery also may be done if your child cannot close their eyes completely.
- Monobloc frontofacial advancement to expand the skull and advance the midface. This is not used often, but it is the best choice for young children whose cheekbones and brow bones are behind their eyes and who have breathing problems.
Dental care and bite alignment
Good mouth care (oral hygiene) is important for all children, but especially those with craniofacial disorders. Our team includes pediatric dentists and orthodontists who specialize in craniofacial conditions.

A pediatric dentist will check your child’s general dental health and look to see if their teeth are too crowded. As your child grows, a craniofacial team orthodontist will check how your child’s upper and lower teeth fit together (occlusion).

Learn more about craniofacial orthodontics.

Your child may need:
- Upper teeth removed to relieve crowding because of their small upper jaw.
- Orthodontic braces to expand the roof of their mouth (palate) and align their teeth.
- Braces or appliances to put the teeth in the best position before jaw surgery.
- Braces to hold the teeth in the correct position for at least 6 months after jaw surgery.
- Surgery to move the upper jaw forward (Le Fort I maxillary advancement) if their teeth do not fit together well when their face bones have finished growing. Usually, this happens at 16 years for a girl and 18 years for a boy. Many children with Crouzon syndrome need this adjustment as teenagers even though their upper jaw was enlarged as a result of Le Fort III midface advancement when they were younger.
Managing hearing and speech problems
Many children with Crouzon syndrome have hearing loss.

Usually, your baby’s hearing is screened in the hospital soon after birth or within a few days. If problems are found, we do further testing.

As your child grows, they will have a range of hearing tests based on their hearing status and ear health. A specialist trained to test hearing in infants and children (audiologist) will perform the tests.

Our ear, nose and throat specialist (otolaryngologist) and audiologist will talk with you and your child about hearing management, such as:
- If hearing aids can help your child
- The best place to sit in the classroom when your child reaches school age
Hearing problems can lead to speech difficulties. Your child may have problems with speech if the roof of their mouth or the inside of their nose has an unusual shape or size.

If needed, a speech and language pathologist (SLP) will check your child and recommend speech therapy or other treatment.

Why choose Seattle Children’s for Crouzon syndrome treatment?

Each year, Seattle Children’s Craniofacial Center cares for hundreds of children with craniosynostosis, including rare syndromes like Crouzon. Our team has more experience caring for children with craniosynostosis than any other center in the United States. We have experts in every field your child might need.

Many of our patients are newly diagnosed babies. We also care for children who received their initial care at another hospital. We treat children, teens and young adults up to age 21 and sometimes beyond.

The right treatment at the right time: Our experience helps us find problems early and take steps to prevent or treat them. We watch carefully for problems with your child’s breathing, feeding, vision, hearing, speech and development and recommend care for any issues they have.
Better results with fewer surgeries: Our approach helps children with Crouzon syndrome get the most benefit from treatment early in childhood. As a result, later in life, some surgeries are either not needed or are less extensive. Often, we combine surgery to cut a bone with distraction osteogenesis, which lets us make bigger changes in bone position.
Team-based care from experts: We’ll bring together all the team members your child needs, such as a craniofacial ; nurse; ; ; craniofacial ; pediatric ; ; ear, nose and throat specialist (); ; and .
Support for your whole family: A diagnosis of Crouzon syndrome can be scary. We take time to explain your child’s condition. We help you understand your child’s treatment options and make the choices that are right for your family. Every family is offered social work support.
Research to improve care: Our team created a clinical care pathway to guide treatment for craniosynostosis based on research about what is most effective. Our research is helping us to:
- Better understand the genetic causes of craniosynostosis
- Study how the condition affects learning and behavior
- Build a library of 3-D images to better measure the results of surgery and improve treatments