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Craniosynostosis

  • In the midst of all of the joy you feel as a new parent, learning that your child has a craniofacial deformity—especially one like craniosynostosis that may require surgery—can be overwhelming. Your list of questions may seem endless: What exactly is craniosynostosis?How serious is it? Is my child at risk for developmental problems? How will this affect his daily life? 

    Learning the basics about craniosynostosis is a good first step. 

    • Craniosynostosis is a condition in which the fibrous joints, called sutures, between the plates of the skull fuse too early during a child’s development.
    • A child with craniosynostosis may have problems with brain and skull growth, possibly leading to developmental delays and cognitive impairment.
    • Some children with craniosynostosis develop abnormally high pressure inside the skull, called intracranial pressure. This can cause serious neurological complications if left untreated.
    • Craniosynostosis can cause a misshapen appearance of the head and skull.
    • Craniosynostosis is often noticeable at birth, but can also emerge in older infants.
    • Sometimes, it runs in families—but most often, it appears to occur randomly.
    • Craniosynostosis affects more boys than girls.
    • Many infant skull shape irregularities are actually due to a condition called plagiocephalywhich is related to the baby’s position during sleep—and not craniosynostosis. Plagiocephaly is a very different disorder, and does not require surgery to treat.
    • Surgery is the recommended treatment option for most types of craniosynostosis, in order to help reduce intracranial pressure and correct the deformity. 


  • Learning more about craniosynostosis can help you better understand what to expect during your child’s treatment. A good starting point is to visualize the basic structures in a newborn’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 craniosynostosis:

    • The sutures close too early.
    • The pressure inside the skull, or intracranial pressure, can become elevated.
    • The skull and facial bones may become visibly deformed or asymmetrical.
    • The brain and skull may develop abnormally. 

    If untreated, craniosynostosis can lead to serious problems, including:

    • developmental delays
    • problems with learning, memory, speech and communication
    • severe nausea and vomiting
    • seizures

    Craniosynostosis is a potentially progressive condition, meaning that, if it is not treated, symptoms can continue, and possibly worsen, over time. As a result, surgery is recommended for most children with craniosynostosis.

    Did you know?

    Boston Children’s neurosurgery, neurology programs are ranked #1 by U.S. News & World Report

    Signs and symptoms

    What are the symptoms of craniosynostosis?
    Symptoms of craniosynostosis may vary, depending upon:

    • the type of craniosynostosis
    • the age of the child at diagnosis
    • which sutures in the skull are involved
    • how advanced the condition is when discovered 

    The most common signs that a child may have craniosynostosis include:

    • an unusually shaped head or face
    • asymmetrical appearance of the head or skull (one side of the face or top of the head looks different than the other)
    • an enlarged appearance of the head or skull
    • a bulging fontanelle ( the “soft spot” at the top of a newborn’s skull)
    • bulging eyes 

    You should seek treatment from a qualified medical professional right away if you notice several of these warning signs in your child. Early intervention is key to successfully treating craniosynostosis and preventing serious problems.

    Types

    There are several different forms of craniosynostosis, namely: 

    • sagittal suture craniosynostosis; this is the most common type. Babies with this form of craniosynostosis experience premature closing of the sagittal suture, which divides the parietal bones (the bones that make up the top and sides of the head). This causes a narrowed, elongated head appearance, called scaphalocephaly.

       
    • unilateral coronal synostosis, which causes fused skull bones along what is known as the coronal suture. This suture runs from the ear to the baby’s fontanelle, or “soft spot,” on either the right or left side. Unilateral coronal synostosis prevents the forehead and brow bone from developing normally.

       
    • trigonocephaly, which involves the fusion of the metopic suture (running from the top of the head down the forehead to the top of the nose).  Children with this type of craniosynostosis may have a prominent ridge along their foreheads, and their skulls may have a pointed, slightly triangular appearance in front.

    Causes

    What causes craniosynostosis?

    Craniosynostosis often occurs randomly—meaning there’s no clear reason why a child develops the disorder. 

    In some cases, craniosynostosis is caused by an inherited genetic problem. When craniosynostosis is inherited, typically:

    • One parent has what is called anautosomal dominant gene for craniosynostosis. This means that there is a 50 percent chance of having a child with craniosynostosis, even if the parent is not directly affected by the condition.

    Frequently Asked Questions (FAQ)

    Q: Will my child be OK?
    A: The severity of craniosynostosis can vary widely, depending on:

    • the type of craniosynostosis
    • the specific sutures and parts of the skull involved
    • the age of your child at diagnosis
    • the overall health of your child
    • the symptoms already present at diagnosis 

    Some forms of craniosynostosis—particularly in children who are diagnosed when the condition is already pronounced—can cause elevated intracranial pressure (and delays in brain development, leading to problems with learning, speech and other functions). 

    The key factor in successfully treating the condition is early detection and prompt, medical attention. The earlier craniosynostosis is diagnosed and treated, the better the general outlook for the child. 

    Q: How common is craniosynostosis?
    A: It affects about one in every 2,500 children. 

    Q: At what age does craniosynostosis tend to develop?
    A: Physicians can sometimes detect craniosynostosis in fetuses as early as the 12th week of a woman’s pregnancy, but prenatal diagnosis is very uncommon. Craniosynostosis is almost always noticeable at birth, although some children’s conditions may not be detected until later in infancy.

    Q: Is my baby going to need surgery?
    A: Most children with craniosynostosis will need surgery to prevent further problems with their brain and skull growth and to correct deformities in their skulls’ structures. Here at Children’s Hospital Boston, our clinicians have extensive experience performing surgeries for all types of craniosynostosis, and most children who have these procedures go on to lead fulfilling, active adult lives. 

    Q: What do I need to look out for once my child has been diagnosed with craniosynostosis?
    A:
    Parents of children with craniosynostosis should always be watchful for changes in their child’s:

    • head shape and size
    • movement
    • activity levels
    • temperament
    • appetite
    • cognitive functioning (memory, learning, speech and language) 

    Q: Is there a cure for craniosynostosis?
    A: Craniosynostosis can be a progressive condition, meaning that, if it is not treated, symptoms can continue—and possibly worsen—over time. As a result, for most children with craniosynostosis, surgery will eventually become necessary.

    The good news is that the surgical techniques used at Children’s have an excellent success rate for correcting the deformities of the head and skull in children with craniosynostosis, and can help prevent further problems with brain and skull growth.

    Did you know?

    Boston Children’s offers counseling for families with known, suspected inherited conditions

    Questions to ask your doctor

    You and your family play an essential role in your child’s treatment for craniosynostosis. 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 craniosynostosis. 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?
    • What type of craniosynostosis does my child have?
    • How advanced is my child’s craniosynostosis?
    • Is my child's craniosynostosis possibly inherited? If so, can you refer me to genetic counseling services for my family?
    • What is the long-term outlook for my child?
    • Is surgery necessary at this time?
    • Do I need to restrict my child’s physical activity?
    • Does my child need to wear a protective helmet?
    • Do I need to make any other 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?
  • How is craniosynostosis diagnosed?

    Many children with craniosynostosis display visible symptoms—namely, a misshapen, enlarged or asymmetrical head and skull—at birth. Other times, a child’s craniosynostosis is diagnosed during a routine physical examination. 

    Once an initial finding of craniosynostosis 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 x-rays, pictures taken with small doses of radiation
    • ordering a computed tomography (CT) scan, a non-invasive procedure using x-ray equipment and powerful computers to create detailed images of particular parts of the body

    Boston Children’s Fetal-Neonatal Neurology Program helps newborns and infants with neurological problems.

  • Boston Children's Hospital has been a worldwide innovator in diagnosing and treating children with craniosynostosis for decades. Clinicians in our Departments of Neurosurgery and Plastic Surgery have extensive experience in treating children, adolescents and adults with all forms of the condition, and can even diagnose craniosynostosis in utero in many cases. 

    And we always use minimally invasive techniques—medical and surgical procedures that use small incisions and miniaturized cameras and tools—whenever we can.

    Surgery

    Most children with craniosynostosis will require surgical intervention. 

    Surgery for craniosynostosis:

    • is designed to reduce intracranial pressure and 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)
    • may need to be repeated over time, depending on the seriousness of the condition, as the child continues to grow 

    Before the operation, your child's surgeon will:

    • explain the reasons to consider surgery
    • explain the different types of surgery and if your child is a candidate for the minimally invasive 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, 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 craniosynostosis, 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, Children's has several other resources designed to give your family comfort, support and guidance:
     

    Patient and family resources at Children's

    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 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.

    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

    Children's 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 Children's 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.

    Children's International Center 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 01-617-355-5209 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 Children's unreservedly endorse 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
  • 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. Boston Children’s scientific research program is one of the largest and most active of any pediatric hospital in the world.

    In particular, our neurosurgical and plastic surgery researchers are making new inroads in understanding the causes and development of craniofacial disorders, paving ground for promising new treatments. Among our current research projects with promise for treating 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. 


    Recent staff publications

    Boston Children's Hospital clinicians are noted in bold type.

    Abbott MM, Rogers GF, Proctor MR, Busa K, Meara JG. The cost of treating sagittal synostosis in the first year of life: Endoscopically-assisted suturectomy and postoperative helmet therapy vs. open cranial vault remodeling. Journal of Craniofacial Surgery. Accepted for publication.

    Berry-Candelario J, Ridgway EB, Grondin RT, Rogers G, Proctor MR. Endoscope-Assisted Strip Craniectomy and Post-Operative Helmet Therapy for Treatment of Craniosynostosis. Neurosurgical Focus. 2011 Aug;31(2):E5.

    McCarthy JG, Warren SM, Bernstein JM, Burnett W, Cunningham ML, Edmond JC, Figueroa AA, Kapp-Simon KA, Labow B, Peterson-Falzone S, Proctor M, Rubin M, Sze RW, Yemen T.  Parameters of Care for Craniosynostosis. The Cleft Palate-Craniofacial Journal. 2011 Aug 17.

    Clinical trials

    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.

    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 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.

  • Miles Chernov (r) traveled to Boston Children's Hospital with his father Gabe (l) to undergo minimally invasive surgery for craniosynostosis.

    Miles’ Story

    The biggest problems most doctors in summer camp deal with are sunburns, bug bites and minor scrapes. Fortunately for Gabe and Erin Chernov, who own the Birch Trail summer camp for girls in northern Wisconsin, their camp doctor is a practicing neurosurgeon, a doctor who helped them realize that their son Miles was born with a rare disease called craniosynostosis.

    Affecting about one in 2,500 children, craniosynostosis is a disease in which the bone plates in a baby’s head fuse too early. Untreated, this can lead to excess pressure in the skull and learning disabilities, in addition to cosmetic deformity. But treatment usually involves open surgery.

    “It scared the heck out of us…they open the whole head, a huge scar from ear to ear,” Gabe said after hearing the open surgery details.

    After considering who they could talk to about this, they turned to the neurosurgeon in their camp, who recommended another neurosurgeon in Milwaukee and a neurosurgeon at Boston Children’s Hospital. The neurosurgeon in Milwaukee recommended open surgery where the problem suture is removed. Open surgery is the usual treatment for this condition, but it’s an invasive four-to-six-hour operation and three to four days in intensive care. This procedure would include a long recovery time with a guaranteed blood transfusion. The Chernovs would also have to wait until Miles was 8 months old.

    They then met with Boston Children’s neurosurgeon Mark Proctor, MD, who is an expert in the condition. Procter has a great deal of experience with minimally invasive endoscopic techniques for this condition, which he has been doing since 2004 at Boston Children’s. He is now one of only a few surgeons in the country who is experienced with the procedure. In fact, Proctor has authored several key papers about minimally invasive approaches to the treatment of craniosynostosis. In one of these papers, he was the lead neurosurgeon involved in developing formal parameters of care for the condition. 

    The minimally invasive craniectomy that Proctor proposed for Miles Chernov is designed to allow for normal brain growth and development and correct deformities in the facial and skull bones. It works best in children who are less than 3-4 months old, since the bones are still soft and pliable. It’s usually an hour long procedure with no need for a blood transfusion, and only one night in the hospital. Fortunately for 3-month-old Miles, he was a good candidate and he had the minimally invasive procedure in July, 2012.

    As Miles continues to grow, he wears a helmet, and he visits Proctor for a follow-up exam every two months for a year. His last visit was such a success, he may even be able to stop wearing the helmet after just three months. It’s also likely that Miles won’t have any long–term problems because of the craniosynostosis.

    A journey that started in a northern Wisconsin summer camp, ended a few weeks later in a Boston Children’s operating room. Talking of their experience at Boston Children’s Gabe said, “In a heartbeat we would recommend this to other families.” Gabe was pleased to report that Miles will now be “a healthy kid for the rest of his life.”

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