The skull is made up of bones 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
where one 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
(inherited) in one of two ways:
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 one parent must
carry the gene. The parents have a 1 in 2 chance that each child will have the
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 listed below.
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. Plagiocephaly is often caused by the
head resting on one side for too long without any anomaly of the sutures. In some
cases, it is caused by an early fusion of the coronal or lambdoidal sutures.
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 by 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 craniosynostosis.
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 that normal in the skull. But this isn’t common. It is especially true when more than one suture fuses.
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 his or her healthcare 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. This 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. You may be asked if you have a family history of head or face defects. You may also be asked 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:
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 is to
fix the deformities of the face and skull bones. It can also ease pressure in the skull,
if it is present.
It is usually best for your baby to
have the surgery before he or she reaches age 1. This is because the bones are still
very soft. It is easier to fix the deformities at 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, your child's healthcare provider will explain
When the diagnosis is made after 1 year of age, nonsurgical treatment
is often recommended if the skull's shape appears close to normal.
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 one or more of the sutures of the skull close too early. This 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 face.
- Surgery is usually the recommended treatment.
- Most children who have surgery early live healthy lives.
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 new diagnosis, 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.