The skull is made up of 8bones and joints where the bones of the skull meet (sutures). These
sutures allow the skull to grow as an infant grows and develops. Over time, the sutures
close and the bones fuse together. This forms the skull into a solid piece of bone.
Craniosynostosis is a condition
or more of the sutures close too early. This may cause:
- Problems with normal brain and skull growth
- More pressure than normal inside the head
- Skull or facial bones to become
irregular in shape
The problem occurs in 1 in every 2,000 live births. It affects boys slightly more
often than girls.
Craniosynostosis most often happens
by chance. But it may occur more often in some families. It may be passed down
Autosomal recessive. This means that both parents carry
the gene that causes the condition. Carrier parents have a 1 in 4 chance to have a
child with craniosynostosis with each pregnancy.
Autosomal dominant. This means that only
parent must carry the gene. The parents have a 1 in 2 chance that each child will
have the condition.
The condition may also be part of many other genetic syndromes.
There are many types of craniosynostosis. Different names are given to the various
types. The types are named by which suture or sutures are involved. They are also
named by how the shape of the skull is affected. Common types of craniosynostosis are
This refers to a twisted skull
shape. The forehead and the brow on the affected side are flat. The forehead on the
opposite side may stick out. The back of the head may also be flat. The eye on the
affected side may also have a different shape. This typecan
be caused by an early fusion of the coronal or
But in the most common form of
none of the sutures are fused.
It is not
with any early suture closure. Rather, it
always lies on the same area
the back of
head. Some babies
have a motor disorder that
position when they are
This means a "triangle-like"
shape skull in which a ridge may stick out from the forehead. The eyes may be close
together, and the forehead may look pointed and narrow. It is caused by
fusion of the forehead (metopic) suture. This suture runs from the
top of the head down the middle of the forehead, toward the nose.
This means a skull that is long
and narrow from front to back and narrow from ear to ear. It is caused
fusion of the sagittal suture. This suture runs front to back, down
the middle of the top of the head. This is the most common type of
The first and only symptoms may be changes in the shape of your baby’s head and face.
A child may also have:
- One side of the face that is not the same when compared with the other side
- Small or absent soft spot on the top of the head (anterior fontanelle)
- Higher pressure
normal in the skull. But this isn’t common. It is especially true when more than one
Symptoms of increased pressure in the skull include:
- Full or bulging soft spot on the top
of the head (anterior)
- Sleepiness or baby is less alert than
- Very noticeable scalp veins
- Increased irritability or
- High-pitched cry
- Poor feeding
- Projectile vomiting
- Increasing head size
- Bulging eyes or trouble looking
- Poor development
- Trouble breathing (in certain cases when multiple sutures are
The symptoms of craniosynostosis
may look like other health conditions. Make sure your baby sees
provider for a diagnosis.
Craniosynostosis may be present at
birth (congenital). Or it may be found later, during a physical exam. The diagnosis
is based on a physical exam.
includes measuring your child’s skull. During the exam, your child’s healthcare
provider will ask a lot of questions about your pregnancy and your child’s birth.
may be asked if you have a family history of head or face defects. You may also be
about your child’s development, because the condition can cause delays. Developmental
delays may need more medical follow-ups.
Diagnostic tests are no longer
routinely done for this condition. But your baby may need these tests to confirm the
diagnosis of craniosynostosis:
- X-rays of the head
- CT scan of the
shows more detail than
An ultrasound during pregnancy can sometimes diagnose craniosynostosis before the
The key to treating
craniosynostosis is early detection and treatment. Treatment will depend on your child’s
symptoms, age, and general health. It will also depend on how severe the condition
When the diagnosis is made before a
child is age 1, surgery is usually the recommended treatment. The goal of surgery
fix the deformities of the face and skull bones. It can also ease pressure in the
if it is present.
It is usually best for your baby to
have the surgery before
reach age 1.
This is because the bones are still very soft. It is easier to fix the deformities
that time. Your baby may need surgery at a much earlier age if the condition is more
serious. Most surgeries are done between ages 3 and 8 months old. Before surgery,
child's healthcare provider will explain the procedure.
When the diagnosis is made after 1 year of age, nonsurgical treatment
is often recommended if the skull's shape appears close to normal.
is usually not treated surgically. Parents are instructed to increase the amount of
tummy time when the baby is awake. The baby should continue to sleep on
back. A helmet can be an option.
Some forms of craniosynostosis can affect the development of the brain.
Most children who have surgery early live healthy lives. But long-term complications
may occur. A child with craniosynostosis needs regular medical checkups to make sure
that the skull, facial bones, and brain are developing normally. Your child’s healthcare
provider, surgeon, and other specialists will work with you and your child. The medical
team will work with your family to provide education and guidance.
Your child’s provider may recommend genetic counseling. This is to check for genetic
conditions that may be present in your family.
- Craniosynostosis is a condition where
or more of the sutures of the skull close too early.
causes problems with normal brain and skull growth.
- Craniosynostosis usually occurs by chance.
- The first and only symptoms are usually changes in the shape of the baby’s head and
- Surgery is usually the recommended treatment.
- Most children who have surgery early
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- At the visit, write down the name of a
and any new medicines, treatments, or tests. Also write down any new instructions
your provider gives you for your child.
- Know why a new medicine or treatment is prescribed and how it will help your child.
Also know what the side effects are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose
for that visit.
- Know how you can contact your child’s provider after office hours. This is important
if your child becomes ill and you have questions or need advice.