Bone, Joint and Muscle Conditions

Craniosynostosis

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    • If you are a provider, fax a New Appointment Request Form (NARF) (PDF) (DOC) to 206-985-3121 or 866-985-3121 (toll-free).
    • Instead of fax, you can use eReferral, an online method for submitting referrals and tracking referral status. If you don’t have an eReferral login, learn more.
    • No pre-referral work-up is required, but it is helpful to receive clinical information (such as head circumference and a description of head shape). Fax any clinic notes along with the NARF.
    • If imaging studies (CT, MRI, X-rays) have been done, please upload to eMix or mail a disc to:
      Seattle Children’s Craniofacial Center
      P.O. Box 5371/OB.9.520
      Seattle, WA 98145-5005
    • View our complete Craniofacial Center Referral Information (PDF).

What is craniosynostosis?

Craniosynostosis is when 1 or more of the soft fibrous seams (sutures) in a baby's skull close earlier than normal. It is pronounced crane-eo-sin-ost-O-sis.

The sutures are between the bone plates in a baby's skull. Sutures allow the baby's head to come through the birth canal. After birth, they let the skull get bigger and make room for brain growth.

Over time, the sutures close and connect the skull bones. The different sutures close at different times, starting at about 3 to 9 months. The process continues until adulthood.

Craniosynostosis Drawn by Raymond Sze
This diagram shows the sutures most often affected. Craniosynostosis of the sagittal suture is the most common type.

When these seams close (fuse) too early, it changes the shape of baby's skull and it can't grow right. This can increase pressure in the skull and hurt brain development. A baby can have 1 or more fused sutures.

Doctors do not know exactly what causes craniosynostosis. Babies are often born with it (congenital). You are likely to notice an unusual shape to your baby's head at birth or soon after.

  • In this video, Dr. Carrie Heike explains how the different types of craniosynostosis affect a baby’s skull. The shape of the skull depends on which suture is closed.

  • Most children with craniosynostosis have only 1 fused suture (single-suture craniosynostosis). Otherwise, they are healthy.

    If a child has more than 1 fused suture, their craniosynostosis may be part of a genetic condition, such as Crouzon syndrome, Apert syndrome, Saethre-Chotzen syndrome, Muenke syndrome or Pfeiffer syndrome.

    Children with these syndromes have other medical conditions besides craniosynostosis. A syndrome is a disease or disorder that has a pattern of features.

Craniosynostosis at Seattle Children's

Most families have never heard of craniosynostosis before their child is born with it. But we treat hundreds of children with this condition each year. Our team has more experience caring for children with craniosynostosis than any other center in the United States.

Many of our patients are babies who are newly diagnosed. We also care for children who received their initial treatment at another hospital.

Please call the Craniofacial Center at 206-987-2208 for more information, a second opinion or to make an appointment.

  • Each year we care for more than 400 children with craniosynostosis. That includes more than 150 children with complex craniosynostosis conditions.

    Depending on what is right for your child, we do open remodeling surgery or endoscopic strip craniectomy.

    In 2015, U.S. News & World Report ranked our Neurosurgery program number 1 on the West Coast, and number 10 in the country.

    See Statistics and Outcomes for details on the patients we care for and procedures we perform each year.

  • Our center has experts in every field your child might need. These 50 specialists work together to diagnose and care for our patients. We will create a personalized plan of care for your child.

    Our team meets weekly to discuss complex patient cases and decide on the best care for each child. We work together, and with you and your family’s doctor, to coordinate your child’s care.

    Your child’s treatment team is managed by a “hub” consisting of a craniofacial pediatrician, craniofacial nurse specialist, family service coordinator and social worker.

    Your child’s team also will include a craniofacial plastic surgeon, pediatric neurosurgeon and social worker. If needed, your child may receive care from other experts in our center, including a surgical orthodontist, orthotist, dietitian, ophthalmologist and otolaryngologist.

  • A diagnosis of craniosynostosis can be scary. We take time to explain your child’s condition and answer all your questions.

    We help you fully understand your treatment options and make the choice that is right for your family. Our social workers support your child and family through the challenges of this condition.

    Seattle Children’s provides craniofacial care for children living in an area comprising one-fourth of the continental United States. We also care for patients from across the globe. This experience has helped us develop systems to provide outstanding, personalized care at a great distance.

  • Our team has created a clinical care pathway that guides your child’s treatment from diagnosis onward.

    It is based on the research evidence about what is most effective. This gives doctors a blueprint for giving the best care and makes it easier to improve that care.

    We receive more research funding from the National Institutes of Health (NIH) than any other craniofacial center in the United States.

    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 find out which surgical approaches lead to the most improvement for a specific syndrome

    Read more about:

Symptoms of Craniosynostosis

Craniosynostosis usually is present when your baby is born (congenital). But in mild cases, you and your doctor may not notice it right away.

The first sign of craniosynostosis is an unusual head shape. The shape depends on which soft fibrous seam (suture) in the skull is closed.

Other signs and symptoms include:

  • The soft spot (fontanelle) on baby's head disappears early or bulges
  • A raised ridge develops along the fused sutures in the skull

Other problems happen depending on which of the sutures grow together. For example, a fused coronal suture may cause a baby's face to appear slightly twisted.

Diagnosing Craniosynostosis

Sometimes, craniosynostosis is diagnosed before a baby is born. In rare cases the fused suture may be visible during a routine ultrasound. Learn more about how prenatal counseling can help you prepare.

More often, doctors identify craniosynostosis in the first weeks of life. The doctor will feel your child's head during a physical exam. The shape of their head will help the doctor tell which sutures have grown together.

The doctor also will measure your baby's head. The physical exam and measurement may be all that is needed to diagnose your baby.

A CT (computed tomography) scan of your child's head confirms the diagnosis. This also helps us plan the best treatment for your child.

  • In this video, our expert doctors share how craniosynostosis is diagnosed. They also explain our team approach to the best care for your child.

  • Your child may also have testing to find out if they have one of the genetic causes of craniosynostosis. These include Crouzon syndrome, Apert syndrome, Saethre-Chotzen syndrome, Muenke syndrome, Pfeiffer syndrome and several other rare conditions. Knowing if your baby’s craniosynostosis is part of a syndrome helps us give your child the best care.

    Our Craniofacial Genetics Clinic helps identify syndromes. Our geneticists and genetic counselors can advise you about the possible benefits of genetic testing and explain the results of genetic tests. Understanding your child’s condition can help you make informed decisions about their treatment.

Treatments for Craniosynostosis

The main treatment for craniosynostosis is surgery to make sure your child’s brain has enough room to grow.

Surgeons open the fused fibrous seams (sutures) in your child’s skull. Surgery helps the skull grow into a more typical shape and prevents a buildup of pressure on the brain.

Our doctors carefully assess your baby and explain the different surgeries. We work with you to decide the best option.

Surgery recommendation depends on suture and baby’s age

Our recommended treatment depends on which suture in your child’s skull is affected. Their age also matters. We tailor care to your child and family.

Each surgery involves an experienced craniofacial plastic surgeon and a neurosurgeon working as a team in the operating room.

If only 1 suture is fused, most children need only 1 surgery. Rarely, a child needs a second surgery because their head returns to its original shape. This is uncommon, but can happen with any type of craniosynostosis.

  • For babies younger than 4 months with a fusion of the sagittal or lambdoid suture, we provide 2 options:

    See how open remodeling and endoscopic procedures compare.

  • We recommend open remodeling surgery for babies older than 4 months with a fusion of the sagittal or lambdoid suture.

    Starting about 5 months of age, skull growth slows and helmet therapy to reshape the skull is less effective. That is why open surgery is best for older babies.

  • We often recommend open fronto-orbital surgery for babies with a fused metopic or coronal suture. This surgery takes place when your baby is 9 to 12 months, depending on which suture is fused.

    Endoscopic surgery may also be an option for babies 4 months or younger with a single fused metopic or coronal suture. But there is a greater chance that they will need open surgery later. We partner with you to make a decision that is best for your family.

  • Babies with multiple fused sutures or those with genetic syndromes usually need a series of operations to repair the fused suture and treat other health conditions.

    These syndromes include Apert syndrome, Crouzon syndrome, Saethre-Chotzen syndrome, Muenke syndrome and Pfeiffer syndrome.

  • During this operation, surgeons use an endoscope. This is a thin, lighted tube with a camera and small tools attached. Surgeons remove bone but do not change the shape of the skull.

    • The craniofacial plastic surgeon makes 2 small incisions in the top of your baby's head to separate the skin and other soft tissue from the bone underneath. Each incision is about 1 inch (3 cm).
    • While protecting the brain, the neurosurgeon makes small holes in the skull or enters the skull through your baby's soft spot.
    • Using a small tool on the endoscope, the neurosurgeon separates the skull bone from the protective covering of the brain.
    • The neurosurgeon and plastic surgeon remove the fused suture on the top of your baby’s head. They remove 2 small strips of bone on each side of the skull near the ears.
    • The strips are cut into pieces and then put back into places where bone was removed. This helps in healing. Over time, the pieces of bone will grow together to protect your baby’s brain.
    • Surgeons stitch the incisions closed. The stitches dissolve over time.
    • After the surgery, most babies spend 2 nights in the hospital: 1 night in the intensive care unit (ICU) and 1 night in a regular hospital room.
    • Your baby wears a helmet for several months. The helmet molds their head to a shape that allows for normal brain growth.

    See details on helmet therapy and endoscopic strip craniectomy.

  • To repair a fused sagittal suture, open remodeling surgery is most often done when a baby is 3 to 6 months old. Surgery on the lambdoid suture happens when a baby is 9 to 12 months old.

    • Open remodeling surgery starts by making a zig-zag incision over the top of baby’s head. The incision goes from one side of the temple to the other.
    • The plastic surgeon separates the skin and other soft tissue from the bone underneath.
    • Next, the plastic surgeon marks a pattern of bone cuts that will allow the skull shape to change as the brain grows.
    • The neurosurgeon creates small holes in the skull. The surgeons carefully separate the coating of the brain (dura) from the bone.
    • Sometimes they remove bone. That lets the plastic surgeon reshape and place it in a better position.
    • In other cases, the cut bone remains attached to the skull. The plastic surgeon reshapes it with special instruments.
    • Sometimes plates and screws are used to hold the shape during healing. These dissolve completely in 1 to 2 years. Rarely, small surgical wires are used for added strength. These do not dissolve.

    After surgery, the brain has more room to grow and the skull has a more typical shape. Your child does not need a helmet to shape their head after surgery.

  •  

    In this video, Dr. Richard Hopper compares open cranial remodeling and endoscopic strip craniectomy.

    Both options are available for babies who are 4 months old or younger and have a single fused sagittal or lambdoid suture.

    Open Remodeling Surgery Endoscopic Strip Craniectomy
    3 to 5 hours in surgery. 1 to 2 hours in surgery.
    Likely to need a blood transfusion. Less likely to need a blood transfusion.
    Hospital stay of 3 to 5 days, including 1 in intensive care unit (ICU). Hospital stay of 2 days, including 1 in ICU.
    Larger scar, but it is usually completely covered by hair within months. Less scarring.
    No need for a helmet after surgery. After surgery, baby wears a soft helmet for up to 1 year to change their head shape.
      Frequent check-ups with the helmet molding team to adjust
    helmet for up to a year. We work with a molding company that
    has offices across the United States.
  • This surgery takes place when your baby is 9 to 12 months old, depending on which suture is fused.

    • A plastic surgeon and a neurosurgeon work together to move and reshape your baby’s forehead and the upper part of their eye sockets.
    • The operation creates more space for your baby’s brain and more protection for their eyes.
    • To hold the bone in its new shape until it heals, the surgeon may use screws and plates that absorb into the body over time.
    • The operation takes 4 to 5 hours.
    • Most babies stay in the hospital 3 to 4 days, including 1 day in the intensive care unit.

     

    Fronto-orbital surgery for metopic and unilateral coronal synostosis

    In this video, Dr. Richard Hopper explains how fronto-orbital surgery can repair a metopic suture or a coronal suture on 1 side of a baby’s head (unilateral coronal).

What to Expect on Surgery Day

While your child is in the operating room, you will get updates every couple of hours. We give you a pager to carry while you are waiting. When surgery is done, the surgeon will talk to you in person.

See details on how to prepare, what to expect and how to care for your child after surgery.

  •  

    In this video, Nurse Kim Davis and Dr. Richard Hopper explain what to expect on your child's craniofacial surgery day. They also describe how we make the process easier for our patients and their families.

Contact Us

Contact the Craniofacial Center at 206-987-2208 for an appointment, a second opinion or more information.