Craniosynostosis is a condition in which the fibrous connections, between the bones of the skull, called sutures, grow together (fuse) too early during a child’s development. It most commonly affects only one of the sutures, but it can also occur in more than one. This fusion can cause problems with brain and skull growth. In some cases, it can lead to developmental delays and cognitive problems, especially if more than one suture is involved. Some children with craniosynostosis can also develop high pressure inside the skull, called increased intracranial pressure.
Craniosynostosis is often noticeable at birth, but can also be diagnosed in older children. This condition sometimes runs in families, but most often it occurs randomly.
John Meara, MD, DMD, MBA, Plastic Surgeon-in-Chief and Mark Proctor, MD, Neurosurgeon-in-Chief
There are four main types of craniosynostosis:
JR has received care from Boston Children's Hospital for more than 30 years to treat a rare form of craniosynostosis, befriending a team of providers who have addressed all aspects of his care, including dentistry, neurosurgery, plastic and oral surgery, and psychiatry.
Symptoms of craniosynostosis may vary, depending on which sutures in the skull are involved and how advanced the condition is when it’s diagnosed.
The most common signs of craniosynostosis include:
If untreated, craniosynostosis can cause more serious symptoms in some children, including:
Many infant skull shape irregularities are actually due to a condition called plagiocephaly, which is caused by a baby’s position during sleep. Plagiocephaly is a common disorder and does not require surgery. Your clinician can help diagnose which condition your child has.
Experts often cannot identify what causes craniosynostosis. In most cases, it appears to happen randomly. Increasingly, a genetic cause is identified.
Many children with craniosynostosis have visible symptoms — such as a misshapen or asymmetrical head — at birth. Other times, a child’s craniosynostosis is diagnosed during a routine physical examination.
Your clinician may diagnose craniosynostosis based on his or her symptoms along with a detailed patient history and a full exam that includes a careful assessment of the shape of the skull.
In rare cases, craniosynostosis may be diagnosed before birth by a prenatal ultrasound or magnetic resonance imaging (MRI).
To diagnose craniosynostosis, often no additional tests are needed other than assessment by an expert. Your child’s clinician may also use one or more of the following tests:
Most, but not all, children with craniosynostosis need surgery to relieve pressure on the brain and correct the shape of the head. In some mild cases, observation might be the best treatment alternative.
Surgery for craniosynostosis often works best in children under age 1, since the bones are still soft and pliable, making them easier to move. Older babies and children can also have surgery, but it may be more complex. In rare cases, the surgery may need to be repeated over time, as the child grows.
The surgeon may use:
At Boston Children’s Hospital, our clinicians in Neurosurgery and Plastic Surgery have extensive experience diagnosing and treating craniosynostosis in infants and children of all ages — using minimally invasive procedures whenever possible.
The severity of craniosynostosis can vary widely, depending on the type of craniosynostosis your child has and how many sutures are affected. The good news is that with early detection and prompt treatment, most children don’t have any significant long-term problems.
This condition affects about one in every 2,500 children.
Craniosynostosis is almost always noticeable at birth, although mild conditions may not be found until later in infancy or childhood. Physicians can sometimes detect craniosynostosis during pregnancy, but this is not common.
Craniosynostosis is often a progressive condition. This means that if it is not treated, the symptoms may get worse over time. As a result, most children with craniosynostosis need surgery to prevent problems with brain and skull growth and to correct skull deformities. Your clinician can help you decide if surgery is the right treatment for your child.
